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  1. Incidence of dilated cardiomyopathy

    OpenAIRE

    Abelmann, Walter H.

    1985-01-01

    Full reliable data on the incidence and prevalence of dilated cardiomyopathy are not available. In the United States, at least 0.7% of cardiac deaths are attributable to cardiomyopathy. Dilated cardiomyopathy probably contributes the great majority of these cases. The mortality rate for cardiomyopathy in males is twice that of females, and for blacks it is 2.4 times that of whites. Cardiomyopathy was diagnosed in 0.67% of patients discharged from hospitals in 1979 with diagnoses of disease of...

  2. Genetics Home Reference: dilated cardiomyopathy with ataxia syndrome

    Science.gov (United States)

    ... dilated cardiomyopathy with ataxia syndrome dilated cardiomyopathy with ataxia syndrome Enable Javascript to view the expand/collapse ... Open All Close All Description Dilated cardiomyopathy with ataxia (DCMA) syndrome is an inherited condition characterized by ...

  3. The Mutations Associated with Dilated Cardiomyopathy

    OpenAIRE

    Ruti Parvari; Aviva Levitas

    2012-01-01

    Cardiomyopathy is an important cause of heart failure and a major indication for heart transplantation in children and adults. This paper describes the state of the genetic knowledge of dilated cardiomyopathy (DCM). The identification of the causing mutation is important since presymptomatic interventions of DCM have proven value in preventing morbidity and mortality. Additionally, as in general in genetic studies, the identification of the mutated genes has a direct clinical impact for the f...

  4. Related factors of dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Guangyong Huang; Hang Gao; Xiangang Meng; Zhonghua Yan; Xiangquan Kong; Lexin Wang

    2009-01-01

    Objective To investigate the etiology and relative factors of dilated cardiomyopathy (DCM) in Chinese patients. Methods A case-control study was conducted to compare 233 patients with DCM in high-incidence areas (case group) and 150 patients with stable angina pectoris (control group). Life styles and history of diseases information was collected by questionaire; human anti-myocardial antibody IgG (AMA- IgG), human Coxsackie B virus IgG (CBV- IgG) and human adenovirus antibody IgG (ADV- lgG) were measured with ELISA. General chemical and toxicological indicators in drink water from high and low prevalence areas and serum trace elements also were compared. Results 1 ) Compared with the control group, the case group had more farmers (P < 0.01), with low average incomes (P < 0.01), higher alcohol consumption (P < 0.01) and higher incidence of the history of myocarditis (P < 0.01 ). 2) AMA-IgG, CBV-IgG and ADV-IgG levels were low and the positive rates ofAMA-IgG, CBV-IgG and ADV-IgG of patients with DCM were respectively 7.78%, 6.67% and 6.67%, no statistical significance comparing with those in the control group. 3) The content of iron (1.36±2.18 vs 0.39±0.67 mg/L, P<0.05) and manganese (0.384±0.35 vs 0.15±0.14, P<0.01 ) in drinking water of high-incidence areas was significantly higher than that in low-incidence areas. 4) The content of serum iron (69.14±57.8 vs 20.04±17.5 μ mol/L, P<0.01 ) and copper (25.74±4.2 vs 19.7±4.5 μmol/L, P<0.01) in the case group evidently exceeded the normal range and obviously higher than that in the control group. Conclusions 1) The incidence of some DCM is related with low incomes, high alcohol consumption and myocarditis. 2) These data do not support that DCM is related with persistent virus infection and autoimmunization; 3) Iron and manganese contents exceeding standards in drinking water and the high content of serum iron and copper is comparatively related with the incidence of DCM.

  5. Peripartum cardiomyopathy and dilated cardiomyopathy: different at heart.

    Science.gov (United States)

    Bollen, Ilse A E; Van Deel, Elza D; Kuster, Diederik W D; Van Der Velden, Jolanda

    2014-01-01

    Peripartum cardiomyopathy (PPCM) is a severe cardiac disease occurring in the last month of pregnancy or in the first 5 months after delivery and shows many similar clinical characteristics as dilated cardiomyopathy (DCM) such as ventricle dilation and systolic dysfunction. While PPCM was believed to be DCM triggered by pregnancy, more and more studies show important differences between these diseases. While it is likely they share part of their pathogenesis such as increased oxidative stress and an impaired microvasculature, discrepancies seen in disease progression and outcome indicate there must be differences in pathogenesis as well. In this review, we compared studies in DCM and PPCM to search for overlapping and deviating disease etiology, pathogenesis and outcome in order to understand why these cardiomyopathies share similar clinical features but have different underlying pathologies. PMID:25642195

  6. Peripartum cardiomyopathy and dilated cardiomyopathy: different at heart

    Directory of Open Access Journals (Sweden)

    Ilse Anne Elise Bollen

    2015-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a severe cardiac disease occurring in the last month of pregnancy or in the first 5 months after delivery and shows many similar clinical characteristics as dilated cardiomyopathy (DCM such as ventricle dilation and systolic dysfunction. While PPCM was believed to be DCM triggered by pregnancy, more and more studies show important differences between these diseases. While it is likely they share part of their pathogenesis such as increased oxidative stress and an impaired microvasculature, discrepancies seen in disease progression and outcome indicate there must be differences in pathogenesis as well. In this review, we compared studies in DCM and PPCM to search for overlapping and deviating disease etiology, pathogenesis and outcome in order to understand why these cardiomyopathies share similar clinical features but have different underlying pathologies.

  7. ACE I/D polymorphism in Indian patients with hypertrophic cardiomyopathy and dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Rai, Taranjit Singh; Dhandapany, Perundurai Subramaniam; Ahluwalia, Tarun Veer Singh;

    2008-01-01

    The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM).......The study was carried to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) polymorphism with the risk of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM)....

  8. Psoriasis and dilated cardiomyopathy: coincidence or associated diseases?

    Science.gov (United States)

    Eliakim-Raz, Noa; Shuvy, Mony; Lotan, Chaim; Planer, David

    2008-01-01

    Psoriasis is a common immune-mediated disease which affects 1-3% of the population. The etiology of psoriasis is unknown. Idiopathic dilated cardiomyopathy is probably the end result of a variety of toxic, metabolic or infectious agents. During a computerized search for cardiomyopathy among all patients hospitalized with psoriasis in the Hadassah University Hospital since 1980 we found an increased prevalence of cardiomyopathy, and specifically dilated cardiomyopathy. We present 4 patients who suffer from both conditions. In accordance with previous data, an association between preexisting psoriasis and dilated cardiomyopathy is suggested. We suggest that the genetic risk factors of dilated cardiomyopathy are shared by psoriasis, and more specifically psoriatic arthritis. Alternatively, the immune reaction that is triggered in dilated cardiomyopathy leading to the progression of the disease might be enhanced in patients with psoriasis or psoriatic arthritis. Chronic inflammation and persistent secretion of proinflammatory cytokines may be considered a potential pathway, triggering the initiation and progression of dilated cardiomyopathy in psoriatic patients. Further investigation of the genetic and immune risk factors involved in dilated cardiomyopathy and in psoriasis may lead to a better understanding of the pathogenesis and treatment of dilated cardiomyopathy.

  9. The Mutations Associated with Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ruti Parvari

    2012-01-01

    Full Text Available Cardiomyopathy is an important cause of heart failure and a major indication for heart transplantation in children and adults. This paper describes the state of the genetic knowledge of dilated cardiomyopathy (DCM. The identification of the causing mutation is important since presymptomatic interventions of DCM have proven value in preventing morbidity and mortality. Additionally, as in general in genetic studies, the identification of the mutated genes has a direct clinical impact for the families and population involved. Identifying causative mutations immediately amplifies the possibilities for disease prevention through carrier screening and prenatal testing. This often lifts a burden of social isolation from affected families, since healthy family members can be assured of having healthy children. Identification of the mutated genes holds the potential to lead to the understanding of disease etiology, pathophysiology, and therefore potential therapy. This paper presents the genetic variations, or disease-causing mutations, contributing to the pathogenesis of hereditary DCM, and tries to relate these to the functions of the mutated genes.

  10. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.

    Science.gov (United States)

    Ware, James S; Li, Jian; Mazaika, Erica; Yasso, Christopher M; DeSouza, Tiffany; Cappola, Thomas P; Tsai, Emily J; Hilfiker-Kleiner, Denise; Kamiya, Chizuko A; Mazzarotto, Francesco; Cook, Stuart A; Halder, Indrani; Prasad, Sanjay K; Pisarcik, Jessica; Hanley-Yanez, Karen; Alharethi, Rami; Damp, Julie; Hsich, Eileen; Elkayam, Uri; Sheppard, Richard; Kealey, Angela; Alexis, Jeffrey; Ramani, Gautam; Safirstein, Jordan; Boehmer, John; Pauly, Daniel F; Wittstein, Ilan S; Thohan, Vinay; Zucker, Mark J; Liu, Peter; Gorcsan, John; McNamara, Dennis M; Seidman, Christine E; Seidman, Jonathan G; Arany, Zoltan

    2016-01-21

    Background Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin. Methods In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls. Results We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P=1.3×10(-7)) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P=0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P=2.7×10(-10)); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P=0.005). Conclusions The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder.

  11. Patient with Eating Disorder, Carnitine Deficiency and Dilated Cardiomyopathy.

    Science.gov (United States)

    Fotino, A Domnica; Sherma, A

    2015-01-01

    Dilated cardiomyopathy is characterized by a dilated and poorly functioning left ventricle and can result from several different etiologies including ischemic, infectious, metabolic, toxins, autoimmune processes or nutritional deficiencies. Carnitine deficiency-induced cardiomyopathy (CDIM) is an uncommon cause of dilated cardiomyopathy that can go untreated if not considered. Here, we describe a 30-year-old woman with an eating disorder and recent percutaneous endoscopic gastrotomy (PEG) tube placement for weight loss admitted to the hospital for possible PEG tube infection. Carnitine level was found to be low. Transthoracic echocardiogram (TTE) revealed ejection fraction 15%. Her hospital course was complicated by sepsis from a peripherally inserted central catheter (PICC). She was discharged on a beta-blocker and carnitine supplementation. One month later her cardiac function had normalized. Carnitine deficiency-induced myopathy is an unusual cause of cardiomyopathy and should be considered in adults with decreased oral intake or malabsorption who present with cardiomyopathy. PMID:27159507

  12. Genetics Home Reference: DMD-associated dilated cardiomyopathy

    Science.gov (United States)

    ... on PubMed Cohen N, Muntoni F. Multiple pathogenetic mechanisms in X linked dilated cardiomyopathy. Heart. 2004 Aug; ... with a qualified healthcare professional . About Genetics Home Reference Site Map Contact Us Selection Criteria for Links ...

  13. Molecular genetics of dilated cardiomyopathy in the Dobermann dog

    NARCIS (Netherlands)

    Stabej, Polona

    2005-01-01

    Canine dilated cardiomyopathy (DCM) is a disease of the myocardium associated with dilatation and impaired contraction of the ventricles. It primarily affects large and giant breed dogs with Dobermanns being one of the most frequently affected. The high prevalence of DCM in specific breeds suggests

  14. Titin gene mutations are common in families with both peripartum cardiomyopathy and dilated cardiomyopathy

    NARCIS (Netherlands)

    van Spaendonck-Zwarts, Karin Y.; Posafalvi, Anna; van den Berg, Maarten P.; Hilfiker-Kleiner, Denise; Bollen, Ilse A. E.; Sliwa, Karen; Alders, Marielle; AlMomani, Rowida; van Langen, Irene M.; van der Meer, Peter; Sinke, Richard J.; van der Velden, Jolanda; Van Veldhuisen, Dirk J.; van Tintelen, J. Peter; Jongbloed, Jan D. H.

    2014-01-01

    Aim Peripartum cardiomyopathy (PPCM) can be an initial manifestation of familial dilated cardiomyopathy (DCM). We aimed to identify mutations in families that could underlie their PPCM and DCM. Methods and results We collected 18 families with PPCM and DCM cases from various countries. We studied th

  15. X-Linked Dilated Cardiomyopathy: A Cardiospecific Phenotype of Dystrophinopathy

    Directory of Open Access Journals (Sweden)

    Akinori Nakamura

    2015-06-01

    Full Text Available X-linked dilated cardiomyopathy (XLDCM is a distinct phenotype of dystrophinopathy characterized by preferential cardiac involvement without any overt skeletal myopathy. XLDCM is caused by mutations of the Duchenne muscular dystrophy (DMD gene and results in lethal heart failure in individuals between 10 and 20 years. Patients with Becker muscular dystrophy, an allelic disorder, have a milder phenotype of skeletal muscle involvement compared to Duchenne muscular dystrophy (DMD and sometimes present with dilated cardiomyopathy. The precise relationship between mutations in the DMD gene and cardiomyopathy remain unclear. However, some hypothetical mechanisms are being considered to be associated with the presence of some several dystrophin isoforms, certain reported mutations, and an unknown dystrophin-related pathophysiological mechanism. Recent therapy for Duchenne muscular dystrophy, the severe dystrophinopathy phenotype, appears promising, but the presence of XLDCM highlights the importance of focusing on cardiomyopathy while elucidating the pathomechanism and developing treatment.

  16. A Rare Occurance with Epidermolysis Bullosa Disease: Dilated Cardiomyopathy

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    Derya Cimen

    2014-02-01

    Full Text Available Epidermolysis bullosa is a congenital and herediter vesiculobullous disease. Dystrophic form of this disease is characterized by severe malnutrition, failure to thrive, adhesions at fingers, joint contractures related with the formation of scar tissues, carcinoma of the skin, anemia, hipoalbuminemia, wound enfections and sepsis. Rarely, mortal dilated cardiomyopathy may occur in patients. In this report we present a 13 year-old pediatric patient with dilated cardiomyopathy, clinically diagnosed with Epidermolysis bullosa as well as a review of recent related literature.

  17. Dilated cardiomyopathy in an American cocker spaniel with taurine deficiency.

    Science.gov (United States)

    Gavaghan, B J; Kittleson, M D

    1997-12-01

    An American Cocker Spaniel with low plasma taurine concentration (taurine. Improvement in all echocardiographic indices were noted over a 22 week follow-up, most notably an increase in left ventricular shortening fraction to 20%, a decrease of E-point septal separation from 14 mm to 7 mm and marked left ventricular remodelling. This degree of improvement in myocardial function may represent a direct link between dilated cardiomyopathy in the American Cocker Spaniel and plasma taurine deficiency. Alternatively, this response may reflect a breed-related cardiomyopathy with a natural history and therapeutic response not commonly seen in the more common large breed cardiomyopathy presentations.

  18. Atlas of the clinical genetics of human dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Haas, Jan; Frese, Karen S; Peil, Barbara;

    2015-01-01

    AIM: Numerous genes are known to cause dilated cardiomyopathy (DCM). However, until now technological limitations have hindered elucidation of the contribution of all clinically relevant disease genes to DCM phenotypes in larger cohorts. We now utilized next-generation sequencing to overcome thes...

  19. Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yabe, Toshikazu; Furuno, Takashi; Kitaoka, Hiroaki; Matsumura, Yoshihisa; Yamasaki, Naohito; Doi, Yoshinori [Kochi Medical School, Nankoku (Japan)

    2002-11-01

    This study evaluated the significance of perfusion defects demonstrated by thallium-201 and age in the prognosis of patients with idiopathic dilated cardiomyopathy. Seventy-four dilated cardiomyopathy patients underwent thallium scintigraphy as well as clinical and hemodynamic examination. Abnormal perfusion defects were present in 23 of 38 patients aged <60 years (61%) and in 26 of 36 elderly patients aged {>=}60 years (72%; NS). Univariate analysis showed that such perfusion defects were a significant predictor of cardiac death only in patients aged <60 years (p=0.015). Stepwise discriminant analysis also revealed that perfusion defects were a significant predictor in patients aged <60 years (Wilks' lambda 0.499, chi-square test 20.2, p=0.003). Perfusion defects were not more important than the history of syncope or stroke in elderly dilated cardiomyopathy patients. Twenty-one patients died of disease-related causes during 58{+-}43 months. The five-year survival rate was better in patients aged <60 years without than in those with perfusion defects (100% vs 58.4%, respectively), but not affected in patients aged {>=}60 years (66.7% vs 62.2%). Thallium scintigraphy is valuable for the prognosis of patients with dilated cardiomyopathy aged <60 years who are usually candidates for heart transplantation. Absence of thallium perfusion defects may indicate good long-term prognosis. (author)

  20. Linkage of familial dilated cardiomyopathy to chromosome 9

    Energy Technology Data Exchange (ETDEWEB)

    Krajinovic, M.; Vatta, M.; Milasin, J. [Univ. of Trieste (Italy)] [and others

    1995-10-01

    Idiopathic dilated cardiomyopathy is a heart muscle disease of unknown etiology, characterized by impaired myocardial contractility and ventricular dilatation. The disorder is an important cause of morbidity and mortality and represents the chief indication for heart transplantation. Familial transmission is often recognized (familial dilated cardiomyopathy, or FDC), mostly with autosomal dominant inheritance. In order to understand the molecular genetic basis of the disease, a large six-generation kindred with autosomal dominant FDC was studied for linkage analysis. A genome-wide search was undertaken after a large series of candidate genes were excluded and was then extended to two other families with autosomal dominant pattern of transmission and identical clinical features. Coinheritance of the disease gene was excluded for >95% of the genome, after 251 polymorphic markers were analyzed. Linkage was found for chromosome 9q13-q22, with a maximum multipoint lod score of 4.2. There was no evidence of heterogeneity. The FDC locus was placed in the interval between loci D9S153 and D9S152. Several candidate genes for causing dilated cardiomyopathy map in this region. 33 refs., 3 figs., 1 tab.

  1. X-Linked Dilated Cardiomyopathy: A Cardiospecific Phenotype of Dystrophinopathy

    OpenAIRE

    Akinori Nakamura

    2015-01-01

    X-linked dilated cardiomyopathy (XLDCM) is a distinct phenotype of dystrophinopathy characterized by preferential cardiac involvement without any overt skeletal myopathy. XLDCM is caused by mutations of the Duchenne muscular dystrophy (DMD) gene and results in lethal heart failure in individuals between 10 and 20 years. Patients with Becker muscular dystrophy, an allelic disorder, have a milder phenotype of skeletal muscle involvement compared to Duchenne muscular dystrophy (DMD) and sometime...

  2. Oxidative Stress in Dilated Cardiomyopathy Caused by MYBPC3 Mutation

    Directory of Open Access Journals (Sweden)

    Thomas L. Lynch

    2015-01-01

    Full Text Available Cardiomyopathies can result from mutations in genes encoding sarcomere proteins including MYBPC3, which encodes cardiac myosin binding protein-C (cMyBP-C. However, whether oxidative stress is augmented due to contractile dysfunction and cardiomyocyte damage in MYBPC3-mutated cardiomyopathies has not been elucidated. To determine whether oxidative stress markers were elevated in MYBPC3-mutated cardiomyopathies, a previously characterized 3-month-old mouse model of dilated cardiomyopathy (DCM expressing a homozygous MYBPC3 mutation (cMyBP-C(t/t was used, compared to wild-type (WT mice. Echocardiography confirmed decreased percentage of fractional shortening in DCM versus WT hearts. Histopathological analysis indicated a significant increase in myocardial disarray and fibrosis while the second harmonic generation imaging revealed disorganized sarcomeric structure and myocyte damage in DCM hearts when compared to WT hearts. Intriguingly, DCM mouse heart homogenates had decreased glutathione (GSH/GSSG ratio and increased protein carbonyl and lipid malondialdehyde content compared to WT heart homogenates, consistent with elevated oxidative stress. Importantly, a similar result was observed in human cardiomyopathy heart homogenate samples. These results were further supported by reduced signals for mitochondrial semiquinone radicals and Fe-S clusters in DCM mouse hearts measured using electron paramagnetic resonance spectroscopy. In conclusion, we demonstrate elevated oxidative stress in MYPBC3-mutated DCM mice, which may exacerbate the development of heart failure.

  3. Subtle abnormalities in contractile function are an early manifestation of sarcomere mutations in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Lakdawala, Neal K; Thune, Jens J; Colan, Steven D;

    2012-01-01

    Sarcomere mutations cause both dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM); however, the steps leading from mutation to disease are not well described. By studying mutation carriers before a clinical diagnosis develops, we characterize the early manifestations of sarcomere ...

  4. Comprehensive evaluation of radionuclide techniques in differentiating dilated cardiomyopathy and ischemic cardiomyopathy

    International Nuclear Information System (INIS)

    This study analyzed significance of radionuclide technique in differentiating dilated cardiomyopathy (DCM) from ischemic cardiomyopathy (CAD-CM). The patients with DCM usually show mild perfusion abnormalities and do not have perfusion defects. Perfusion imaging and metabolic imaging is concordant in most patients and reduced wall motion is typically diffuse in DCM. The majority of patients with CAD-CM have perfusion defects and distributed as segmental. The perfusion imaging and metabolic imaging of patients with CAD-CM generally show mismatch and the wall motion abnormality is segmental

  5. HLA-DQA1 Polymorphism in Idiopathic Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    刘巍; 李为民; 孙宁玲; 闫征; 何培英

    2003-01-01

    To determine whether the possession of certain HLA-DQA1 alleles was associated with the risk of developing idiopathic dilated cardiomyopathy (IDC) and to substantiate the role of an autoimmunologic pathogenesis in IDC. Type the alleles of HLA-DQA1 by polymerase chain reaction with sequence-specific primers (PCR-SSP) technique in 38 patients of idiopathic dilated cardiomyopathy (7 women and 31 men), aged from 17 to 56 years old with diagnosis being according to World Health Organization criteria (IDC group), in 50 patients of end-stage heart failure of known etiology (18 women and 32men), with ages ranging from 34 to 72( HF group), and in the control group consisting of presumably 100 healthy subjects(39 women and 61 men) from the health survey, aged from 30 to 59 years old. The frequency of HLA-DQA1 * 0501 in the DCM patients was significantly elevated than that in the HF and the control group. Molecular analysis of the DQA1 gene polymorphism performed in the three subgroups shows an increased frequency of DQA1 * 0501 among patients with less EF.The HF group carries a high frequency of HLA-DQA1 * 0301. An increased frequency of DQA1 * 0201 and DQA1 * 0103 was found in the control group. HLA-DQA1 * 0501 is an associated gene of idiopathic dilated cardiomyopathy and the possession of DQA1 * 0301 may be indicative of the known etiologic heart failure, suggesting that the mechanisms involved in the pathogenesis of IDC and otherwise heart failure are different. Immunologic abnormalities may be a major contributor to the susceptibility of developing of IDC.

  6. Stroke and dilated cardiomyopathy associated with celiac disease

    Institute of Scientific and Technical Information of China (English)

    Murat; Dogan; Erdal; Peker; Eren; Cagan; Sinan; Akbayram; Mehmet; Acikgoz; Huseyin; Caksen; Abdurrahman; Uner; Yasar; Cesur

    2010-01-01

    Celiac disease(CD) is manifested by a variety of clinical signs and symptoms that may begin either in childhood or adult life.Neurological symptoms without signs of malabsorption have been observed for a long time in CD.In this report,an 8-year-old girl with CD presented with rarely seen dilated cardiomyopathy and stroke.The girl was admitted with left side weakness.Her medical history indicated abdominal distention,chronic diarrhea,failure to thrive,and geophagia.On physical examination,short stature,pale ...

  7. Dilated cardiomyopathy as part of familial dystrophia myotonica

    DEFF Research Database (Denmark)

    Gadgaard, Tenna; Eiskjær, Hans; Jensen, Peter Kjestrup Axel;

    2014-01-01

    Dilated cardiomyopathy (DCM) is a condition characterized by non-ischaemic heart failure and is often hereditary. We present a family in which the proband had DCM in isolation while several relatives presented with myotonia, hypotonia, poly-hydramnion during pregnancy or a mental handicap....... The disease presentation and subsequent genetic investigations were consistent with a diagnosis of dystrophia myotonica. This case presentation illustrate that DCM may be part of a systemic condition and that familial investigations may have important implications for correct diagnosis, treatment...... and counseling....

  8. A Family History of Dilated Cardiomyopathy Induced by Viral Myocarditis

    Directory of Open Access Journals (Sweden)

    Thomas Cognet

    2012-01-01

    Full Text Available Myocarditis can lead to acute heart failure, cardiogenic shock, or sudden death and later, dilated cardiomyopathy (DCM with chronic heart failure. We report the cases of two DCM induced by acute and past myocarditis in the same family and expressed by its two main complications within few weeks: an hemodynamic presentation as a fulminant myocarditis rapidly leading to cardiac tranplantation and a rythmologic presentation as an electrical storm leading to catheter ablation of ventricular tachycardia. These cases ask the question of the family predisposition to viral myocarditis leading to DCM.

  9. Molecular profiling of dilated cardiomyopathy that progresses to heart failure

    Science.gov (United States)

    Burke, Michael A.; Chang, Stephen; Wakimoto, Hiroko; Gorham, Joshua M.; Conner, David A.; Christodoulou, Danos C.; Parfenov, Michael G.; DePalma, Steve R.; Eminaga, Seda; Konno, Tetsuo; Seidman, Jonathan G.; Seidman, Christine E.

    2016-01-01

    Dilated cardiomyopathy (DCM) is defined by progressive functional and structural changes. We performed RNA-seq at different stages of disease to define molecular signaling in the progression from pre-DCM hearts to DCM and overt heart failure (HF) using a genetic model of DCM (phospholamban missense mutation, PLNR9C/+). Pre-DCM hearts were phenotypically normal yet displayed proliferation of nonmyocytes (59% relative increase vs. WT, P = 8 × 10–4) and activation of proinflammatory signaling with notable cardiomyocyte-specific induction of a subset of profibrotic cytokines including TGFβ2 and TGFβ3. These changes progressed through DCM and HF, resulting in substantial fibrosis (17.6% of left ventricle [LV] vs. WT, P = 6 × 10–33). Cardiomyocytes displayed a marked shift in metabolic gene transcription: downregulation of aerobic respiration and subsequent upregulation of glucose utilization, changes coincident with attenuated expression of PPARα and PPARγ coactivators -1α (PGC1α) and -1β, and increased expression of the metabolic regulator T-box transcription factor 15 (Tbx15). Comparing DCM transcriptional profiles with those in hypertrophic cardiomyopathy (HCM) revealed similar and distinct molecular mechanisms. Our data suggest that cardiomyocyte-specific cytokine expression, early fibroblast activation, and the shift in metabolic gene expression are hallmarks of cardiomyopathy progression. Notably, key components of these profibrotic and metabolic networks were disease specific and distinguish DCM from HCM. PMID:27239561

  10. Dobutamine stress echocardiographyin distinguishing ischemic from nonischemic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Miloradović Vladimir

    2005-01-01

    Full Text Available Introduction The aim of this study was to evaluate the diagnostic accuracy of dobutamine stress echocardiography for detection of coronary artery disease in patients with dilated cardiomyopathy. Detection of regional wall motion abnormalities at rest does not reliably distinguish ischemic from nonischemic cardiomyopathy. Material and methods To distinguish between ischemic and nonischemic dilated cardiomyopathy (DCM, we studied 50 patients with left ventricular dysfunction (20 ischemic and 30 nonischemic, detected by coronary angiography using dobutamine stress echocardiography. Echocardiographic images were obtained at baseline, low and paek dose of dobutamine. Rest and stress left ventricular wall motion scores were derived from analysis of regional wall motion. Results Dobutamine infusion was terminated after achievement of the target heart rate or maximal protocol dose in 16 (80% patients with ischemic heart disease and in 23 (73.3% patients with nonischemic heart disease. At rest, there were more normal segments (p<0.001 and a trend toward more akinetic segments (p, not significant per ischemic than per nonischemic DCM patients. However, either at rest or with low-dose dobutamine, individual data largely overlapped. At peak dose, in ischemic DCM, regional contraction worsened in many normal or dyssinergic regions at rest (in some cases after inprovement with low-dose dobutamine; in contrast, in nonischemic DCM, further mild impovement was observed in a variable number of left ventricular areas. Thus, with peak-dose dobutamine, more akinetic and less normal segments were present per ishemic than per nonischemic DCM patient (both, p<0.001. A value of six or more akinetic segments was 90% sensitive and 98% specific for ischemic DCM. Conclusions Our data show that analysis of regional contraction by dobutamine stress echocardiography can distinguish between.

  11. Magnetic resonance imaging of dilated cardiomyopathy; MRT bei dilatativen Kardiomyopathien

    Energy Technology Data Exchange (ETDEWEB)

    D' Anastasi, M. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Greif, M. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Medizinische Klinik und Poliklinik I, Muenchen (Germany); Reiser, M.F.; Theisen, D. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Deutsches Zentrum fuer Herzkreislaufforschung (DZHK), Muenchen (Germany)

    2013-01-15

    Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy with a prevalence of 1 out of 2,500 in adults. Due to mild clinical symptoms in the early phase of the disease, the true prevalence is probably even much higher. Patients present with variable clinical symptoms ranging from mild systolic impairment of left ventricular function to congestive heart failure. Even sudden cardiac death may be the first clinical symptom of DCM. The severity of the disease is defined by the degree of impairment of global left ventricular function. Arrhythmias, such as ventricular or supraventricular tachycardia, atrioventricular (AV) block, ventricular extrasystole and atrial fibrillation are common cardiac manifestations of DCM. Magnetic resonance imaging (MRI) plays an important role in the exact quantification of functional impairment of both ventricles and in the evaluation of regional wall motion abnormalities. With its excellent ability for the assessment of myocardial structure, it is becoming increasingly more important for risk stratification and therapy guidance. (orig.) [German] Die dilatative Kardiomyopathie (DCM) ist die haeufigste Form der Kardiomyopathie mit einer Praevalenz von 1/2500 Erwachsenen. Aufgrund der zunaechst milden klinischen Symptomatik ist jedoch von einer relativ hohen Dunkelziffer auszugehen. Die klinische Praesentation ist variabel, die Schwere der Erkrankung wird vom Ausmass der systolischen Funktionseinschraenkung bestimmt. Herzrhythmusstoerungen, wie ventrikulaere oder supraventrikulaere Tachykardien, AV-Blockierungen, ventrikulaere Extrasystolen und Vorhofflimmern sind moegliche klinische Manifestationen. Bei manchen Patienten ist der ploetzliche Herztod die erste klinische Manifestation der Erkrankung. Die kardiale MRT spielt eine bedeutende Rolle fuer die Beurteilung des Ausmasses der ventrikulaeren Dilatation, Dysfunktion und fuer die Beurteilung regionaler Wandbewegungsstoerungen. Darueber hinaus kann sie zur Anwendung kommen

  12. [Right ventricular dysplasia and dilated cardiomyopathy observed by radionuclide images].

    Science.gov (United States)

    Takamura, I; Ando, J; Miyamoto, A; Kobayashi, T; Sakamoto, S; Yasuda, H

    1985-12-01

    Four cases of right ventricular dysplasia (RVD) and 28 cases of dilated cardiomyopathy (DCM) were studied. RVD was characterized clinically by syncope, sustained recurrent ventricular tachycardia with left bundle branch block patterns on the surface electrocardiogram, and right heart failure. Furthermore, moderate to severe dilatation of the right ventricle and depressed right ventricular function were apparent on radionuclide angiography. However, left ventricular dilatation and depressed left ventricular function were documented in DCM. Right ventricular volume was proportional to left ventricular volume in DCM, however, right ventricular volume was disproportionately greater in RVD. On the T1-201 perfusion image, left ventricular perfusion defects were delineated in 10 of 26 patients with DCM, and in one of four RVD patients. During two to eight year follow-up periods, six patients died suddenly five of whom had left ventricular perfusion defects. However, in 19 patients without left ventricular perfusion defects, only one sudden death was observed. A connecting link between sudden death and left ventricular perfusion defect is suggested. PMID:3841888

  13. Dilated aortic root and severe aortic regurgitation causing dilated cardiomyopathy in classic Ehlers-Danlos syndrome.

    Science.gov (United States)

    Zainal, Abir; Hamad, Mahmoud Nidal; Naqvi, Syed Yaseen

    2016-01-01

    Ehlers-Danlos syndrome (EDS) is a group of heritable disorders characterised by vast clinical heterogeneity ranging from the classic constellation of symptoms including skin hyperextensibility, joint hypermobility and skin fragility to the exceedingly critical consequences of arterial rupture and visceral perforation. We describe the case of a 65-year-old male with a history of classic EDS who reported of dyspnoea on exertion, orthopnoea, fatigue and palpitations. He was found to have dilated cardiomyopathy with an ejection fraction of 35%, aortic root dilation and severe aortic valve regurgitation. The authors intend to draw attention to the rare cardiac manifestations of this condition and the therapeutic challenges involved in managing such patients. PMID:27413024

  14. Cardiovascular genetics: technological advancements and applicability for dilated cardiomyopathy.

    Science.gov (United States)

    Kummeling, G J M; Baas, A F; Harakalova, M; van der Smagt, J J; Asselbergs, F W

    2015-07-01

    Genetics plays an important role in the pathophysiology of cardiovascular diseases, and is increasingly being integrated into clinical practice. Since 2008, both capacity and cost-efficiency of mutation screening of DNA have been increased magnificently due to the technological advancement obtained by next-generation sequencing. Hence, the discovery rate of genetic defects in cardiovascular genetics has grown rapidly and the financial threshold for gene diagnostics has been lowered, making large-scale DNA sequencing broadly accessible. In this review, the genetic variants, mutations and inheritance models are briefly introduced, after which an overview is provided of current clinical and technological applications in gene diagnostics and research for cardiovascular disease and in particular, dilated cardiomyopathy. Finally, a reflection on the future perspectives in cardiogenetics is given.

  15. Kidney infarction in Friedreich's ataxia with dilated cardiomyopathy.

    Science.gov (United States)

    Evangelopoulos, Dimitrios Stergios; Pirvu, Tatiana Nataly; Exadaktylos, Aristomenis; Kohl, Sandro

    2012-09-30

    A 37-year-old man with advanced Friedreich's ataxia was referred to our emergency department with acute exacerbated abdominal pain of unclear aetiology. Laboratory tests showed slightly increased inflammatory parameters, elevated troponin and B-type natriuretic peptide, as well as minimal proteinuria. Transthoracic echocardiography revealed a pre-existing dilated cardiomyopathy. Abdominal sonography showed no pathological alterations. Owing to persistent pain under analgesia, a contrast-enhanced CT-abdomen was performed, which revealed a non-homogeneous perfusion deficit of the right kidney, although neither abdominal vascular alteration, cardiac thrombus, deep vein thrombosis nor a patent foramen ovale could be detected. Taking all clinical and radiological results into consideration, the current incident was diagnosed as a thromboembolic kidney infarction. As a consequence, lifelong oral anticoagulation was initiated.

  16. Pathomorphological Changes of the Myocardium in Canine Dilated Cardiomyopathy (DCM

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    Janus Izabela

    2015-04-01

    Full Text Available The study was conducted on ventricular and atrial wall preparations from 11 dogs with clinically diagnosed dilated cardiomyopathy. After fixation, the specimens were stained with haematoxylin and eosin and Masson-Goldner trichrome technique. Parenchymal changes (fibrosis and fatty infiltration, vascular changes (congestion and coronary vessel wall hypertrophy, degenerative changes (loss of striation, changes in cardiomycyte and nuclei structure, and presence of inflammatory infiltrates (mononuclear and polynuclear were estimated. Complex histological changes in both ventricular and atrial muscles were shown. It was not determined whether the processes occurring in the myocardium have a primary character, or are a consequence of developing heart failure. Such issues will be put under further and more detailed examination.

  17. Epidemiology and genetics of dilated cardiomyopathy in the Indian context

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    Ushasree B

    2009-07-01

    Full Text Available Background: Dilated cardiomyopathy (DCM still remains to be a poorly understood and less analyzed group of cardiac-muscle disorders when compared to hypertrophic cardiomyopathy (HCM. Also, the vast clinical heterogeneity among the patients has rendered the small and isolated kindred studies less informative on the genetics and epidemiology of DCM. Aim of the study: The study aimed at understanding the epidemiology and genetics of DCMs in the Indian context. Materials and methods/ Statistical analysis: One hundred seven DCM patients and 105 healthy individuals were included in the study for epidemiological and genetic risk factor identification and to fit the possible mode of inheritance. Single′s ascertainment methodology for segregation analysis and Penrose frequency estimates were followed to evaluate for the role of specific epidemiological factors in the disease etiology. Chi-square analysis was carried out to interpret the results statistically. Results and Conclusion: Our study suggests that epidemiological factors like gender, age at onset and vegetarian diet in conjunction with sarcomere gene mutations may play a role in the disease expression. Similarly, segregation analysis for the possible mode of inheritance showed a deviation from the autosomal dominant mode of inheritance, strengthening the underlying genetic heterogeneity of DCM.

  18. 扩张型心肌病%Dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    张健

    2012-01-01

    The term "dilated cardiomyopathy" ( DCM) has been referred to as a spectrum of heterogeneous myocardial disorders that are characterized of ventricular dilatation and suppressed myocardial contractility despite absence of abnormal loading conditions or factors contributing to reduced contractility as a result of ischemia. In addition to diverse treatment outcomes and prognoses, the challenges in making diagnosis and differential diagnosis could have arisen from various etiologies and similar clinical manifestations. These critiques were herein reviewed in a briefly manner.%扩张型心肌病是一组心肌的异质性生疾病,其特点是在没有异常负荷状态或没有导致心肌收缩力下降的缺血因素存在的前提下,表现为心室扩大和心肌收缩力下降.扩张型心肌病病因复杂多样,临床表现近似,诊断和鉴别诊断困难,治疗效果差异较大,预后有别.

  19. Cell therapy in dilated cardiomyopathy: from animal models to clinical trials

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    C. del Corsso

    2011-05-01

    Full Text Available Dilated cardiomyopathy can be the end-stage form and common denominator of several cardiac disorders of known cause, such as hypertensive, ischemic, diabetic and Chagasic diseases. However, some individuals have clinical findings, such as an increase in ventricular chamber size and impaired contractility (classical manifestations of dilated cardiomyopathy even in the absence of a diagnosed primary disease. In these patients, dilated cardiomyopathy is classified as idiopathic since its etiology is obscure. Nevertheless, regardless of all of the advances in medical, pharmacological and surgical procedures, the fate of patients with dilated cardiomyopathy (of idiopathic or of any other known cause is linked to arrhythmic episodes, severe congestive heart failure and an increased risk of sudden cardiac death. In this review, we will summarize present data on the use of cell therapies in animal models of dilated cardiomyopathies and will discuss the few clinical trials that have been published so far involving patients affected by this disease. The animal models discussed here include those in which the cardiomyopathy is produced by genetic manipulation and those in which disease is induced by chemical or infectious agents. The specific model used clearly creates restrictions to translation of the proposed cell therapy to clinical practice, insofar as most of the clinical trials performed to date with cell therapy have used autologous cells. Thus, translation of genetic models of dilated cardiomyopathy may have to wait until the use of allogeneic cells becomes more widespread in clinical trials of cell therapies for cardiac diseases.

  20. Terapia celular na cardiomiopatia dilatada Cell therapy in dilated cardiomyopathy

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    Helena Furtado Martino

    2006-05-01

    Full Text Available Homem de 41 anos em insuficiência cardíaca sistólica, CF III NYHA, estágio clínico C, por cardiomiopatia dilatada, foi submetido ao transplante autólogo da fração mononuclear da medula óssea, via sistema arterial coronariano, através de cateterismo cardíaco. Dois meses após o procedimento, houve diminuição do BNP plasmático, diminuição da área cardíaca ao estudo radiológico do tórax e à ressonância nuclear magnética. O ecocardiograma demonstrou diminuição do fluxo regurgitante secundário a dilatação do anel mitral. Na ergoespirometria houve melhor desempenho, com aumento do consumo máximo de oxigênio, sendo possível redução da terapêutica medicamentosa. A ausência de eventos adversos caracterizados por: instabilidade clínica/hemodinâmica, alteração enzimática ou eletrocardiográfica apontam para segurança e exeqüibilidade deste procedimento realizado e descrito com pioneirismo na cardiomiopatia dilatada.A forty-one-year-old male with systolic heart failure, FC-III NYHA, clinical stage C due to dilated cardiomyopathy was submitted to an autologous transplant of the mononuclear fraction of bone marrow via coronary artery system through heart catheterism. Two months after the procedure, there was a decrease in plasma BNP and cardiac area reduction at the thorax X-ray and nuclear magnetic resonance. The echocardiogram showed decrease of the secondary regurgitation and mitral ring dilatation. There was a better performance at the ergospirometry, with increase of the maximum oxygen consumption and consequent reduction in drug therapy. The absence of adverse events characterized by clinical/hemodynamic instability, enzymatic alteration or electrocardiogram demonstrate the safety and feasibility of this procedure carried out and described with pioneering spirit in dilated cardiomyopathy.

  1. [Studies on the genetic susceptibility to dilated cardiomyopathy].

    Science.gov (United States)

    Li, Y Y; Zhang, J N; Ma, W Z

    1993-01-01

    HLA-DQB1,-DRB1 genes of 27 Chinese patients with dilated cardiomyopathy (DCM), 7 high risk individuals in a DCM kindred and 17 normal control subjects were analysed with the use of restriction fragment length polymorphisms (RFLP) with full length DQB1 and DRB1 cDNA probes according to the standard and nomenclature of the Xth International Histocompatibility Workshop. The resulting restriction patterns allowed genotyping of HLA-DR and HLA-DQw. D-DQw8 frequency increased significantly in patients with DCM as compared with that of the controls (P DQw4 also increased in patients although no statistical significance was shown when Chi-square value was corrected with Yate's correction, whereas D-DQw5 overrepresented in controls (P DQw8 and D-DQw4. These results support the hypothesis that HLA class II genes were associated with an increased risk for DCM, HLA-DQB rather than -DRB may confer genetic susceptibility to DCM. PMID:8104770

  2. Deception in simplicity: hereditary phospholamban mutations in dilated cardiomyopathy.

    Science.gov (United States)

    Young, Howard S; Ceholski, Delaine K; Trieber, Catharine A

    2015-02-01

    The sarcoplasmic reticulum (SR) calcium pump (SERCA) and its regulator phospholamban are required for cardiovascular function. Phospholamban alters the apparent calcium affinity of SERCA in a process that is modulated by phosphorylation via the β-adrenergic pathway. This regulatory axis allows for the dynamic control of SR calcium stores and cardiac contractility. Herein we focus on hereditary mutants of phospholamban that are associated with heart failure, such as Arg(9)-Cys, Arg(9)-Leu, Arg(9)-His, and Arg(14)-deletion. Each mutant has a distinct effect on PLN function and SR calcium homeostasis. Arg(9)-Cys and Arg(9)-Leu do not inhibit SERCA, Arg(14)-deletion is a partial inhibitor, and Arg(9)-His is comparable to wild-type. While the mutants have distinct functional effects on SERCA, they have in common that they cannot be phosphorylated by protein kinase A (PKA). Arg(9) and Arg(14) are required for PKA recognition and phosphorylation of PLN. Thus, mutations at these positions eliminate β-adrenergic control and dynamic cardiac contractility. Hydrophobic mutations of Arg(9) cause more complex changes in function, including loss of PLN function and dominant negative interaction with SERCA in heterozygous individuals. In addition, aberrant interaction with PKA may prevent phosphorylation of wild-type PLN and sequester PKA from other local subcellular targets. Herein we consider what is known about each mutant and how the synergistic changes in SR calcium homeostasis lead to impaired cardiac contractility and dilated cardiomyopathy. PMID:25563649

  3. Genetic Modifiers of Duchenne Muscular Dystrophy and Dilated Cardiomyopathy.

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    Andrea Barp

    Full Text Available Dilated cardiomyopathy (DCM is a major complication and leading cause of death in Duchenne muscular dystrophy (DMD. DCM onset is variable, suggesting modifier effects of genetic or environmental factors. We aimed to determine if polymorphisms previously associated with age at loss of independent ambulation (LoA in DMD (rs28357094 in the SPP1 promoter, rs10880 and the VTTT/IAAM haplotype in LTBP4 also modify DCM onset.A multicentric cohort of 178 DMD patients was genotyped by TaqMan assays. We performed a time-to-event analysis of DCM onset, with age as time variable, and finding of left ventricular ejection fraction 70 mL/m2 as event (confirmed by a previous normal exam < 12 months prior; DCM-free patients were censored at the age of last echocardiographic follow-up.Patients were followed up to an average age of 15.9 ± 6.7 years. Seventy-one/178 patients developed DCM, and median age at onset was 20.0 years. Glucocorticoid corticosteroid treatment (n = 88 untreated; n = 75 treated; n = 15 unknown did not have a significant independent effect on DCM onset. Cardiological medications were not administered before DCM onset in this population. We observed trends towards a protective effect of the dominant G allele at SPP1 rs28357094 and recessive T allele at LTBP4 rs10880, which was statistically significant in steroid-treated patients for LTBP4 rs10880 (< 50% T/T patients developing DCM during follow-up [n = 13]; median DCM onset 17.6 years for C/C-C/T, log-rank p = 0.027.We report a putative protective effect of DMD genetic modifiers on the development of cardiac complications, that might aid in risk stratification if confirmed in independent cohorts.

  4. A Case Report of Cardiac Amyloidosis Initially Managed as Dilated Cardiomyopathy: Missing the obvious!

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    Yerramareddy Vijaya Chandra

    2016-06-01

    Full Text Available Amyloidosis is a rare disorder with uncertain incidence; however, in UK and US population, AL amyloidosis, the most frequently diagnosed type, has an annual incidence of 6 to 10 cases per million. [1] The deposition of amyloid, the extracellular proteinaceous material, in the tissues results in a group of disorders called amyloidoses. [2] The most commonly deposited amyloid material in various organ systems including heart are light chains, transthyretin and serum amyloid A. [2] One of the challenges in diagnosing amyloidosis early is that it commonly manifests with nonspeci c symptoms of fatigue and weight loss. The diagnosis is generally considered only when symptoms are traceable to a speci c organ. [3] Cardiac amyloidosis presents initially with mild LV diastolic dysfunction, progressing to classical restrictive cardiomyopathy and nally even dilated cardiomyopathy like stage with end-stage heart failure. The disease can be mistaken in the early stages with hypertrophic cardiomyopathy and hypertensive heart disease and in the late stages with the common-garden variety of dilated cardiomyopathy. Here, we describe a case of cardiac amyloidosis, initially diagnosed and managed as dilated cardiomyopathy with inadequate response to management. Amyloidosis; 2D ECHO; Dilated Cardiomyopathy

  5. CLINICAL PROFILE OF PATIENTS WITH DILATED CARDIOMYOPATHY IN A TERTIARY CARE CENTER IN NORTH EAST INDIA

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    Naruttam

    2014-07-01

    Full Text Available BACKGROUND: Dilated cardiomyopathy (DCM is a severe illness with high mortality in the young adult population in resource limited settings. In north-east part of India, clinicians still continue to face the challenges of identifying and treating the dilated cardiomyopathy to improve quality of life due to various reasons. In India, there have been few investigations focusing on the dilated cardiomyopathy patients. But from this part of the country, there is paucity of data. Therefore, the present study was undertaken to examine the clinical characteristics of dilated cardiomyopathy patients who presented to our hospital. MATERIALS AND METHODS: This prospective observational study was carried out in the Department of General Medicine, Jorhat Medical College and Hospital, Jorhat, Assam, from January 2013 to December 2013. Total 31 consecutive patients fulfilling the criteria of dilated cardiomyopathy were studied. Dilated Cardiomyopathy was diagnosed if enlarged left ventricle with decreased systolic function as measured by left ventricular ejection fraction characterized dilated cardiomyopathy. Patients were excluded from the study if they have one or more of the following; systemic hypertension (>160/100 mm Hg, evidence of coronary artery diseases, pericardial diseases, congenital heart disease, valvular heart diseases, cor pulmonale and rapid, sustained supraventricular tachycardia. Detailed inform consent from the patients and ethical permission from the concerned authorities were taken. RESULTS: Thirty one patients were diagnosed with dilated cardiomyopathy during the study period who fulfilled the inclusion and exclusion criteria of the study. Twenty-nine (92% of 31 patients presented with clinical features of congestive heart failure as their initial presentation and majority presented with dyspnea (70.97%, followed by palpitation (64.52%. The mean cardio thoracic ratio was (0.61. Majority of the patients (70.97% were in sinus rhythm and

  6. Frequency of echocardiographic complications of dilated cardiomyopathy at a tertiary care hospital

    International Nuclear Information System (INIS)

    Dilated cardiomyopathy can lead to a variety of complications recognisable on clinical, echocardiographic, electrocardiographic and radiographic assessment. Among this, transthoracic echocardiography has the dual advantage of being helpful in making the diagnosis of dilated cardiomyopathy as well as an effective tool in early recognition of certain complications for timely management to improve the quality of life of these patients. Methods: This descriptive (case series) study was undertaken at departments of medicine, cardiology, paediatrics and obs/gyn, Ayub Teaching Hospital, Abbottabad from July to December, 2008. fifty patients of dilated cardiomyopathy without age and gender discrimination were selected by convenience sampling. Those with hypertrophic and restrictive cardiomyopathies, valvular and congenital heart disease, hypertension and ischemic heart disease were excluded. Results: mean age was 47.12 +- 17.9 year with male predominance (males=34, females=16). Mean ejection fraction was 30.6 +- 6.9%. complications revealed on echocardiography were intracardiac thrombi (5, 10%), spontaneous echo contrast (5, 10%), pericardial effusion (6, 12%), mitral regurgitation (46, 92%), tricuspid (25, 50%), aortic (5, 10%), pulmonary (2, 4%) multi-valvular regurgitation (28, 56%), and left atrial dilatation (36, 72%). Conclusion: lv systolic dysfunction, cardiac thrombi, spontaneous echo contrast, mitral and tricuspid regurgitation and left atrial enlargement are important complications of dilated cardiomyopathy. echocardiography is important tool towards identification of these complications. (author)

  7. Application of radionuclide techniques in evaluation of dilated cardiomyopathy and ischemic cardiomyopathy

    International Nuclear Information System (INIS)

    Objective: To assess the clinical significance of radionuclide techniques in differentiating dilated cardiomyopathy (DCM) from ischemic cardiomyopathy (CAD-CM). Methods: 28 patients (pts) with DCM and 55 pts with CAD-CM were studied. All pts underwent 99Tcm-MIBI myocardial perfusion SPECT and 18F-FDG myocardial metabolic PET. 73 pts had 99Tcm-RBC radionuclide ventriculography and 68 pts had coronary angiography. Results: 23 pts (82%) with DCM showed perfusion abnormalities with mild and not segmental distribution. 52 pts (95%) with CAD-CM showed perfusion abnormalities that distributed along the coronary vessel territories. Perfusion defects were found in 4 pts (14%) with DCM and 45 pts (82%) with CAD-CM (P<0.01). The average perfusion score was 4.5 +- 2.6 in DCM and 9.5 +- 2.9 in CAD-CM, the area of perfusion diminished uptake was significantly smaller in DCM than in CAD-CM (P < 0.001). 2 pts with DCM and 18 pts with CAD-CM had metabolic defect. The patterns of perfusion/metabolic imaging showed mismatch in most pts with CAD-CM but match in pts with DCM. The LVEF in pts with DCM and CAD-CM was decreased but no significant difference between DCM and CAD-CM was observed. The RVEF in pts with DCM was significantly lower than that in pts with CAD-CM (32.4% +- 13.9% vs 40.9% +- 15.4%, P < 0.05). Conclusions: The radionuclide techniques showed to be helpful for distinguishing DCM from CAD-CM. The discriminate analysis revealed that segmental perfusion abnormality and RVEF were the most important factors for differentiation of DCM from CAD-CM

  8. A negative screen for mutations in calstabin 1 and 2 genes in patients with dilated cardiomyopathy

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    Biagi Diogo G

    2012-01-01

    Full Text Available Abstract Background Calstabins 1 and 2 bind to Ryanodine receptors regulating muscle excitation-contraction coupling. Mutations in Ryanodine receptors affecting their interaction with calstabins lead to different cardiac pathologies. Animal studies suggest the involvement of calstabins with dilated cardiomyopathy. Results We tested the hypothesis that calstabins mutations may cause dilated cardiomyopathy in humans screening 186 patients with idiopathic dilated cardiomyopathy for genetic alterations in calstabins 1 and 2 genes (FKBP12 and FKBP12.6. No missense variant was found. Five no-coding variations were found but not related to the disease. Conclusions These data corroborate other studies suggesting that mutations in FKBP12 and FKBP12.6 genes are not commonly related to cardiac diseases.

  9. Tafazzin gene mutations are uncommon causes of dilated cardiomyopathy in adults

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    Matthew Taylor

    2011-07-01

    Full Text Available Barth syndrome is an X-linked genetic condition featuring neutropenia, skeletal myopathy, and dilated cardiomyopathy in boys due to tafazzin (TAZ mutations. Pure dilated cardiomyopathy without other features of Barth syndrome may also result from TAZ mutations and survival into adulthood has been described. Although TAZ testing is routinely included in dilated cardiomyopathy panels in adults, the prevalence of TAZ mutations in the adult population, including women who may be at risk to develop later onset disease due to TAZ mutations, has not been measured. We screened 292 families with dilated cardiomyopathy (209 male and 83 female probands for TAZ mutations using denaturing high-performance liquid chromatography and sequence analysis. Putative mutations were evaluated based on standard criteria including screening available relatives and healthy controls and for effects on splicing efficiency in the case of one intronic variant. Two variants suspicious for being pathogenic were found in two unrelated families (c.387T>C, Phe128Ser and c.507C>T, Leu169Leu. The Phe128Ser variant had been previously reported as a pathogenic mutation; however we determined that this variant is instead a rare polymorphism restricted to African Americans. The Leu169Leu variant was detected in a male patient and altered RNA processing in our minigene assay supporting a pathogenic role. No mutations in female subjects were detected. Tafazzin mutations were rare in our population of adults with dilated cardiomyopathy and none were found in females. Our findings indicate that genetic testing for tafazzin should not be routinely performed in dilated cardiomyopathy as suggested by current guidelines. Furthermore, the Phe128Ser variant is not pathogenic, but likely represents a benign polymorphism in persons of African American ancestry.

  10. Haplotypes of NOS3 gene polymorphisms in dilated cardiomyopathy.

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    Lova Satyanarayana Matsa

    Full Text Available Dilated Cardiomyopathy (DCM is characterized by systolic dysfunction, followed by heart failure necessitating cardiac transplantation. The genetic basis is well established by the identification of mutations in sarcomere and cytoskeleton gene/s. Modifier genes and environmental factors are also considered to play a significant role in the variable expression of the disease, hence various mechanisms are implicated and one such mechanism is oxidative stress. Nitric Oxide (NO, a primary physiological transmitter derived from endothelium seems to play a composite role with diverse anti-atherogenic effects as vasodilator. Three functional polymorphisms of endothelial nitric oxide synthase (NOS3 gene viz., T-786C of the 5' flanking region, 27bp VNTR in intron4 and G894T of exon 7 were genotyped to identify their role in DCM. A total of 115 DCM samples and 454 controls were included. Genotyping was carried out by PCR -RFLP method. Allelic and genotypic frequencies were computed in both control & patient groups and appropriate statistical tests were employed. A significant association of TC genotype (T-786C with an odds ratio of 1.74, (95% CI 1.14 - 2.67, p = 0.01 was observed in DCM. Likewise the GT genotypic frequency of G894T polymorphism was found to be statistically significant (OR 2.10, 95% CI 1.34-3.27, p = 0.0011, with the recessive allele T being significantly associated with DCM (OR 1.64, 95% CI 1.18 - 2.30, p = 0.003. The haplotype carrying the recessive alleles of G894T and T-786C, C4bT was found to exhibit 7 folds increased risk for DCM compared to the controls. Hence C4bT haplotype could be the risk haplotype for DCM. Our findings suggest the possible implication of NOS3 gene in the disease phenotype, wherein NOS3 may be synergistically functioning in DCM associated heart failure via the excessive production of NO in cardiomyocytes resulting in decreased myocardial contractility and systolic dysfunction, a common feature of DCM

  11. Pathogenesis of dilated cardiomyopathy: molecular, structural, and population analyses in tropomodulin-overexpressing transgenic mice.

    Science.gov (United States)

    Sussman, M A; Welch, S; Gude, N; Khoury, P R; Daniels, S R; Kirkpatrick, D; Walsh, R A; Price, R L; Lim, H W; Molkentin, J D

    1999-12-01

    Dilated cardiomyopathy is characterized by decreased contractile function and loss of myofibril organization. Previously unexplored structural and molecular events that precede and initiate dilation can now be studied in tropomodulin-overexpressing transgenic (TOT) mice exhibiting progressive dilated cardiomyopathy. Onset of dilation did not correspond to a change in transgene expression levels, which were more than threefold above normal at birth and remained elevated throughout postnatal life. Similarly, mitogen-activated protein kinase activation (p38, ERK1/ERK2, JNK1/JNK2) was not associated with dilation. In contrast, calcineurin was activated before dilation, presumably due to doubling of intracellular diastolic calcium levels in TOT cardiomyocytes. Amplitude of systolic calcium transients was greatly increased as well, demonstrating the novel and unique calcium handling profile of TOT cardiomyocytes. Loss of myofibril organization was not apparent by confocal microscopy until over 1 week after birth, although neonatal sarcomeric abnormalities were revealed by ultrastructural analysis. Rapid postnatal increases in heart:body weight ratio at 1.5 weeks were followed by two waves of mortality between 2 and 3 weeks after birth coincident with maturational stress. Ultimately, TOT pathogenesis is a compensatory response to altered sarcomeric structure driven by calcineurin activation within days after birth, making TOTs an excellent paradigm for studying the role of calcium overload in dilated cardiomyopathy. PMID:10595939

  12. Non-invasive evaluation of arrhythmic risk in dilated cardiomyopathy:From imaging to electrocardiographic measures

    Institute of Scientific and Technical Information of China (English)

    Massimo; Iacoviello; Francesco; Monitillo

    2014-01-01

    Malignant ventricular arrhythmias are a major adverse event and worsen the prognosis of patients affected by ischemic and non-ischemic dilated cardiomyopathy.The main parameter currently used to stratify arrhythmic risk and guide decision making towards the implantation of a cardioverter defibrillator is the evaluation of the left ventricular ejection fraction.However,this strategy is characterized by several limitations and consequently additional parameters have been suggested in order to improve arrhythmic risk stratification.The aim of this review is to critically revise the prognostic significance of non-invasive diagnostic tools in order to better stratify the arrhythmic risk prognosis of dilated cardiomyopathy patients.

  13. Canine candidate genes for dilated cardiomyopathy: annotation of and polymorphic markers for 14 genes

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    van Oost Bernard A

    2007-10-01

    Full Text Available Abstract Background Dilated cardiomyopathy is a myocardial disease occurring in humans and domestic animals and is characterized by dilatation of the left ventricle, reduced systolic function and increased sphericity of the left ventricle. Dilated cardiomyopathy has been observed in several, mostly large and giant, dog breeds, such as the Dobermann and the Great Dane. A number of genes have been identified, which are associated with dilated cardiomyopathy in the human, mouse and hamster. These genes mainly encode structural proteins of the cardiac myocyte. Results We present the annotation of, and marker development for, 14 of these genes of the dog genome, i.e. α-cardiac actin, caveolin 1, cysteine-rich protein 3, desmin, lamin A/C, LIM-domain binding factor 3, myosin heavy polypeptide 7, phospholamban, sarcoglycan δ, titin cap, α-tropomyosin, troponin I, troponin T and vinculin. A total of 33 Single Nucleotide Polymorphisms were identified for these canine genes and 11 polymorphic microsatellite repeats were developed. Conclusion The presented polymorphisms provide a tool to investigate the role of the corresponding genes in canine Dilated Cardiomyopathy by linkage analysis or association studies.

  14. Dilated Cardiomyopathy in a Rio Grande Wild Turkey (Meleagris gallopavo intermedia) in Southern Utah, USA, 2013.

    Science.gov (United States)

    Frame, David D; Kelly, E Jane; Van Wettere, Arnaud

    2015-07-01

    A male Rio Grande Wild Turkey (Meleagris gallopavo intermedia) living in semidomestication was submitted for necropsy. Emaciation, a greatly enlarged heart, and chronic passive congestion of the liver were present. Dilated cardiomyopathy occurs in domestic turkey flocks but has not been reported in Wild Turkeys.

  15. Immunohistological evidence for a chronic intramyocardial inflammatory process in dilated cardiomyopathy.

    OpenAIRE

    Kühl, U.; Noutsias, M; Seeberg, B.; Schultheiss, H P

    1996-01-01

    OBJECTIVE: To determine whether immunohistochemical analysis of cardiac biopsies from patients presenting clinically as dilated cardiomyopathy (DCM) show a chronic inflammatory process. DESIGN: Comparative case control study. SETTING: Tertiary referral centre. PATIENTS: Biopsies from 170 patients with DCM and 85 control patients with other cardiac diseases. RESULTS: Nine patients had sufficient interstitial inflammatory cells to be called borderline myocarditis on conventional histology, leav...

  16. [Clinical case of the month. Cardiac complications of acromegaly: a rare cause of dilated cardiomyopathy].

    Science.gov (United States)

    Devoitille, A; Beckers, A; Piérard, L A

    2012-04-01

    Acromegaly is a disease characterized by chronic growth hormone hypersecretion. Cardiovascular complications represent the main cause of death. We present here a rare case of dilated cardiomyopathy whose diagnosis revealed an acromegaly. This will provide the opportunity to review an uncommon disease and its recently reassessed prevalence.

  17. CARDIAC TRANSPLANTATION IN YOUNG PATIENT WITH DILATED CARDIOMYOPATHY AND SECONDARY ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    E. V. Shlyakhto

    2013-01-01

    Full Text Available Patients with congestive heart failure have an increased incidence of thromboembolic events. The choice of me- dical management in patients with antiphospholipid antibodies and generalized thrombosis due to hypercoagula- bility is complex issue. We report heart transplant outcome in 15 years old patient with dilated cardiomyopathy and secondary anti-phospholipid syndrome. 

  18. Evaluation of pulsatile plasma concentrations of growth hormone in healthy dogs and dogs with dilated cardiomyopathy

    NARCIS (Netherlands)

    Beijerink, N.J.; Lee, W.M.; Stokhof, A.A.; Voorhout, G.; Mol, J.A.; Kooistra, H.S.

    2011-01-01

    Abstract OBJECTIVE: To evaluate plasma concentrations of growth hormone (GH) and insulin-like growth factor I (IGF-I) in healthy dogs and large-breed dogs with dilated cardiomyopathy (DCM). ANIMALS: 8 dogs with DCM and 8 healthy control dogs of comparable age and body weight. PROCEDURES: Blood sampl

  19. Association between LMNA mutation and familial and idiopathic dilated cardiomyopathy patients in Xinjiang

    Institute of Scientific and Technical Information of China (English)

    孙帅

    2014-01-01

    Objective To explore the association between LMNA gene mutation and familiar dilated cardiomyopathy(DCM)(FDCM)and idiopathic DCM(IDCM)in Uygurs and Han people in Xinjiang area.Methods Peripheral blood samples were collected from 28 family member with FDCM and 123 sporadic patients with IDCM(56 Uygur patients and 67 Han patients),80 Uygur

  20. Reversal of Dilated Cardiomyopathy After Successful Radio-Frequency Ablation of Frequent Atrial Premature Beats, a New Cause for Arrhythmia-Induced Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Paul Louis Vervueren, MD

    2012-12-01

    Full Text Available Incessant atrial premature beats as a potential cause for tachycardia-induced cardiomyopathy was suspected in a patient presenting with dilated non-ischemic cardiomyopathy and severely altered left ventricular ejection fraction. The elimination of a left atrial focus by percutaneous RF ablation led to normalization of the clinical status, of atrial and ventricular dimensions and left ventricular systolic function.

  1. Comparison of Calcitonin Gene Related Peptide Level between Children with Dilated Cardiomyopathy and Control Group

    Directory of Open Access Journals (Sweden)

    Noormohammad Noori

    2015-06-01

    Full Text Available Background: Dilated cardiomyopathy is revealed with left ventricular dilatation and systolic dysfunction. Objectives: This study aimed to compare the children with dilated cardiomyopathy and control group regarding the level of Calcitonin Gene Related Peptide (CGRP and its relationship with echocardiography findings Patients and Methods: This case-control study was conducted on 37 children with dilated cardiomyopathy and free of any clinical symptoms and 37 healthy age- and sex-matched children referring to Ali-e-Asghar and Ali Ebne Abitaleb hospitals in Zahedan, Iran. After taking history, echocardiography was performed for both groups. The data were analyzed using the SPSS statistical software and appropriate statistical tests. Results: The two groups were significantly different regarding most of the echocardiographic parameters (P < 0.05. Also, a significant difference was found between the two groups concerning the mean CGRP levels (P = 0.001. Among echocardiographic parameters, CGRP was directly related to Interventricular Septal dimension in Systole (IVSS (P = 0.022, R = 0.375. However, no significant relationship was observed between CGRP level and Ross classification. Conclusions: The findings of this study showed an increase in CGRP serum levels in the case group. Besides, a direct correlation was observed between CGRP level and IVSS.

  2. Familial Dilated Cardiomyopathy Caused by a Novel Frameshift in the BAG3 Gene.

    Directory of Open Access Journals (Sweden)

    Rocio Toro

    Full Text Available Dilated cardiomyopathy, a major cause of chronic heart failure and cardiac transplantation, is characterized by left ventricular or biventricular heart dilatation. In nearly 50% of cases the pathology is inherited, and more than 60 genes have been reported as disease-causing. However, in 30% of familial cases the mutation remains unidentified even after comprehensive genetic analysis. This study clinically and genetically assessed a large Spanish family affected by dilated cardiomyopathy to search for novel variations.Our study included a total of 100 family members. Clinical assessment was performed in alive, and genetic analysis was also performed in alive and 1 deceased relative. Genetic screening included resequencing of 55 genes associated with sudden cardiac death, and Sanger sequencing of main disease-associated genes. Genetic analysis identified a frame-shift variation in BAG3 (p.H243Tfr*64 in 32 patients. Genotype-phenotype correlation identified substantial heterogeneity in disease expression. Of 32 genetic carriers (one deceased, 21 relatives were clinically affected, and 10 were asymptomatic. Seventeen of the symptomatic genetic carriers exhibited proto-diastolic septal knock by echocardiographic assessment.We report p.H243Tfr*64_BAG3 as a novel pathogenic variation responsible for familial dilated cardiomyopathy. This variation correlates with a more severe phenotype of the disease, mainly in younger individuals. Genetic analysis in families, even asymptomatic individuals, enables early identification of individuals at risk and allows implementation of preventive measures.

  3. NT-proBNP as a biomarker in risk stratification of patients with dilated cardiomyopathy

    OpenAIRE

    Pazurek, Kattrin Rosa

    2012-01-01

    This trial analysis is based on the data of 155 patients with dilated cardiomyopathy and is intended to show the prognostic importance of NT-proBNP as a risk factor for cardiac mortality in non-ischemic cardiomyopathy. Coronary heart disease was excluded as a diagnosis for these patients via coronary angiography. NT-proBNP has established itself in the diagnosis of chronic heart failure in the past few years. Most of the studies on this subject have included patients with ischemic cardiomyopa...

  4. Prevalence of cardiac dyssynchrony and correlation with atrio-ventricular block and QRS width in dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Anzouan-Kacou, J B; Ncho-Mottoh, M P; Konin, C;

    2012-01-01

    Cardiac dyssynchrony causes disorganised cardiac contraction, delayed wall contraction and reduced pumping efficiency. We aimed to assess the prevalence of different types of dyssynchrony in patients with dilated cardiomyopathy (DCM), and to establish the correlation between atrio-ventricular blo...

  5. CLINICAL SIGNIFICANCE OF COMPLETE LEFT BUNDLE BRANCH BLOCK IN DILATED CARDIOMYOPATHY

    Institute of Scientific and Technical Information of China (English)

    黄秀惠; 沈卫峰; 龚兰生

    1995-01-01

    Clinical,electrocardiographic and echocardiographic findings in 64 patients with dilated cardiomyopa-thy were retrospectively studied.Compared with 51 patients without complete left bundle branch block (CLBBB) ,13 patients with CLBBB had higher New York Heart Association (NYHA) functional class (P<0.05),increased left ventricular end-diastolic and end-systolic diameters (P<0.002) and myocardial mass (P<0.02),severe mitral regurgitation (P<0.01) and higher mortality rate (P<0.04).Multivari-ate stepwise regrassion analysis revealed that the presence of CLBBB was an independent prognostic factor for patients with dilated cardinomyopathy.

  6. Sheehan's syndrome with reversible dilated cardiomyopathy: A case report and brief overview.

    Science.gov (United States)

    Islam, A K M Monwarul; Hasnat, Mohammad A; Doza, Fatema; Jesmin, Humayra

    2014-04-01

    Sheehan's syndrome is a rare condition characterized by post-partal panhypopituitarism due to necrosis of adenohypophysis resulting from severe post-partum hemorrhage. Lethargy, amenorrhea and failure of lactation are the usual presenting features. Cardiac involvement in Sheehan's syndrome is rare. The case presented here describes dilated cardiomyopathy in a 36-year-old lady who failed to respond adequately to the standard anti-failure treatment. Further investigation revealed the diagnosis of Sheehan's syndrome. Besides other manifestations, cardiac function reverted to normal after giving replacement therapy with glucocorticoid, levothyroxine and sex hormone. Physicians, specially those in developing countries, should have high index of suspicion for the diagnosis of Sheehan's syndrome while dealing with a case of 'peripartal dilated cardiomyopathy'. Persistent amenorrhea and failure of lactation may be important clues in this context. Timely diagnosis and appropriate treatment can lessen the sufferings of the patients. PMID:24719543

  7. TRANSURETHRAL RESECTION OF PROSTRATE IN DILATED CARDIOMYOPATHY PATIENT WITH LOW EJECTION FRACTION

    Directory of Open Access Journals (Sweden)

    Prabhavathi

    2014-06-01

    Full Text Available Dilated cardiomyopathy (DCM patient undergoing non-cardiac surgery poses a challenge for the anesthesiologist to manage it efficiently. DCM is usually accompanied by progressive congestive cardiac failure (CCF and life threatening arrhythmias. The anesthesiologist must have the idea of its haemodynamics, diagnostic evaluations, treatment modalities and more so regarding various drug interactions during anesthesia. We managed this case with combined low dose spinal epidural anesthesia with dexmeditomedine as additive.

  8. QT dispersion on ECG Holter monitoring and risk of ventricular arrhythmias in patients with dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Polyxeni Garyfallidis

    2010-05-01

    Full Text Available Background. QT dispersion (QTd is increased in patients with dilated cardiomyopathy. Increased QTd has been associated with the risk of sudden death. We studied: a the relation between QTd on 12-lead ECG and QTd-ECG Holter; b the relation between QTd apex (QTda and QTd end (QTde on ECG Holter and the risk of ventricular arrhythmias in patients with dilated cardiomyopathy. Methods and Results. 65 patients with dilated cardiomyopathy (33 idiopathic and 32 post-ischemic etiology; NYHA II-III were studied. We divided the patients into: Group A -patients with not-sustained ventricular arrhythmias-; and Group B -patients without arrhythmias-. A significant direct correlation between QTd calculated from 12-lead ECG and from ECG Holter was found in all patients. QTda/24h was not significantly different in the two groups (Gr.A 59.9±7.8 msec vs Gr.B 53.6±8.4 msec p=ns while QTde/24h was significantly higher in Group A (Gr.A 81.9±5.9 msec vs Gr.B 44.5±6.8 msec; p<0.005. In post-ischemic etiology (32 pts; 17 with arrhythmias the correlation between QTde/24h and ventricular arrhythmias was confirmed (Gr.A 81.4±7.8 msec vs Gr.B 42.6±6.2 msec p<0.002. Conclusions. ECG Holter recordings can evaluate QTd as well as the QTd on 12-lead ECG. An increased QTde/24h seems to be correlated with the occurence of ventricular arrhythmias in patients with dilated cardiomyopathy and can then be a useful tool to select patients at high risk for sudden death.

  9. MLP and CARP are linked to chronic PKCα signaling in dilated cardiomyopathy

    OpenAIRE

    Ehler, Elisabeth

    2016-01-01

    MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signaling processes is still enigmatic. Elevated PKCα signaling activity is known to be an important contributor to heart failure. Here we show that MLP directly inhibits the activity of PKCα. In end-stage DCM, PKCα is concentrated at the intercalated disk of cardiomyocytes, where it is sequestered by the adaptor protein CARP in a multiprotein compl...

  10. MLP and CARP are linked to chronic PKCα signalling in dilated cardiomyopathy

    OpenAIRE

    Gehmlich, K; S. Lange(Justus Liebig-Universität Gie\\ssen II Physikalisches Institut, Germany); Lun, AS; Blondelle, J; HOOPER, C.; Dalton, ND; Alvarez, EA; Zhang, X.; Bang, M-L; Abassi, YA; dos Remedios, CG; Peterson, KL; Chen, J.; Ehler, E.

    2016-01-01

    MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signalling processes is still enigmatic. Elevated PKCasignalling activity is known to be an important contributor to heart failure. Here we show that MLP directly inhibits the activity of PKCa. In end-stage DCM, PKCa is concentrated at the intercalated disc of cardiomyocytes, where it is sequestered by the adaptor protein CARP in a multiprotein comp...

  11. Successful treatment of cardiac electrical storm in dilated cardiomyopathy using esmolol: A case report

    OpenAIRE

    Li, Li; Zhou, Yuan-Li; Zhang, Xue-Jing; Wang, Hua-ting

    2016-01-01

    The present study reports a case of electrical storm occurring in a 43-year-old woman with dilated cardiomyopathy. The patient suffered from a cardiac electrical storm, with 98 episodes of ventricular tachycardia rapidly degenerating to ventricular fibrillation in hospital. The patient was converted with a total of 120 defibrillations. Recurrent ventricular tachycardia/fibrillation was initiated by premature ventricular beats. The patient did not respond to the use of amiodaronum. However, th...

  12. Extracellular matrix alterations in cardiomyopathy: The possible crucial role in the dilative form

    OpenAIRE

    Kapelko, Valeri I

    2001-01-01

    The collagen network, part of the myocardial extracellular matrix (EM), and other EM proteins transmit mechanical forces generated by cardiomyocytes to cardiac cavities. Network rearrangement and enlargement – fibrosis – is an essential component of cardiac remodelling at various pathological stages. In particular, similarly abundant fibrosis occurs in dilated, hypertrophic and restrictive cardiomyopathy, and it is unclear how this relates to respective changes in ventricular cavities and siz...

  13. Diagnosis and treatment of congestive heart failure secondary to dilated cardiomyopathy in a hedgehog.

    Science.gov (United States)

    Delk, K W; Eshar, D; Garcia, E; Harkin, K

    2014-03-01

    A one-year-old African pygmy hedgehog (Atelerix albiventris) was evaluated for severe respiratory distress. Physical examination findings included marked dyspnoea, cyanosis and tachypnoea. Radiographic findings included an enlarged heart and pulmonary oedema, and dilated cardiomyopathy was confirmed via echocardiogram. The patient was treated for congestive heart failure because of dilated cardiomyopathy with furosemide, enalapril, pimobendan and l-carnitine. Within 24 hours of treatment, the pulmonary oedema and cyanosis had resolved. Following discharge, clinical improvement was noted by the owner and echocardiogram confirmed improved fractional shortening. Cardiomyopathy has been reported at post-mortem examination in hedgehogs, but there are no reports of ante-mortem diagnosis and treatment. Performing baseline cardiac assessment in hedgehogs is recommended, and treatment with l-carnitine and pimobendan may improve outcome, as carnitine deficiency is a possible cause of cardiomyopathy in hedgehogs. Successful emergency treatment of congestive heart failure in the hedgehog of this report may be effective for other hedgehogs presented with similar clinical signs. PMID:24372164

  14. Peripartum cardiomyopathy

    Science.gov (United States)

    Cardiomyopathy - peripartum ... Cardiomyopathy occurs when there is damage to the heart. As a result, the heart muscle becomes weak ... the lungs, liver, and other body systems. Peripartum cardiomyopathy is a form of dilated cardiomyopathy in which ...

  15. An Upgrade on the Rabbit Model of Anthracycline-Induced Cardiomyopathy: Shorter Protocol, Reduced Mortality, and Higher Incidence of Overt Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Jesús Talavera

    2015-01-01

    Full Text Available Current protocols of anthracycline-induced cardiomyopathy in rabbits present with high premature mortality and nephrotoxicity, thus rendering them unsuitable for studies requiring long-term functional evaluation of myocardial function (e.g., stem cell therapy. We compared two previously described protocols to an in-house developed protocol in three groups: Group DOX2 received doxorubicin 2 mg/kg/week (8 weeks; Group DAU3 received daunorubicin 3 mg/kg/week (10 weeks; and Group DAU4 received daunorubicin 4 mg/kg/week (6 weeks. A cohort of rabbits received saline (control. Results of blood tests, cardiac troponin I, echocardiography, and histopathology were analysed. Whilst DOX2 and DAU3 rabbits showed high premature mortality (50% and 33%, resp., DAU4 rabbits showed 7.6% premature mortality. None of DOX2 rabbits developed overt dilated cardiomyopathy; 66% of DAU3 rabbits developed overt dilated cardiomyopathy and quickly progressed to severe congestive heart failure. Interestingly, 92% of DAU4 rabbits showed overt dilated cardiomyopathy and 67% developed congestive heart failure exhibiting stable disease. DOX2 and DAU3 rabbits showed alterations of renal function, with DAU3 also exhibiting hepatic function compromise. Thus, a shortened protocol of anthracycline-induced cardiomyopathy as in DAU4 group results in high incidence of overt dilated cardiomyopathy, which insidiously progressed to congestive heart failure, associated to reduced systemic compromise and very low premature mortality.

  16. Potential genetic predisposition for anthracycline-associated cardiomyopathy in families with dilated cardiomyopathy

    NARCIS (Netherlands)

    Wasielewski, Marijke; van Spaendonck-Zwarts, Karin Y; Westerink, Nico-Derk L; Jongbloed, Jan D H; Postma, Aleida; Gietema, Jourik A; van Tintelen, J Peter; van den Berg, Maarten P

    2014-01-01

    OBJECTIVE: Anthracyclines are successfully used in cancer treatment, but their use is limited by their cardiotoxic side effects. Several risk factors for anthracycline-associated cardiomyopathy (AACM) are known, yet the occurrence of AACM in the absence of these known risk factors suggests that othe

  17. Inherited infantile dilated cardiomyopathy in dogs: genetic, clinical, biochemical, and morphologic findings.

    Science.gov (United States)

    Alroy, J; Rush, J E; Freeman, L; Amarendhra Kumar, M S; Karuri, A; Chase, K; Sarkar, S

    2000-11-01

    Dilated cardiomyopathy, a lethal disease characterized by left ventricular dilation and systolic dysfunction, is relatively common in humans and other mammals. Idiopathic dilated cardiomyopathy (IDCM) is a primary myocardial disease of unknown cause and can be a familial disorder. This report describes autosomal recessive IDCM in dogs. It occurs in Portuguese Water Dog (PWD) pups and is manifested by acute, vague clinical signs and sudden death. Affected pups have progressive reduction of fractional shortening that can be demonstrated by echocardiography prior to the development of clinical signs. Furthermore, these pups have low plasma taurine levels when consuming certain diets. Affected pups had dilation of the left ventricle and alterations in the sarcomere appearance, while immunohistochemical and biochemical studies demonstrate an increase in desmin, a cytoskeleton protein. The clinical and morphologic findings of IDCM in PWDs are distinct from those reported in adult IDCM. Finally, the clinical and echocardiographic manifestations were reversible in some pups following oral taurine supplementation for 2 months. These results suggest that IDCM in PWDs is correlated with low plasma taurine levels.

  18. A novel mitochondrial tRNA gene mutation in a chinese family with dilated cardiomyopathy and sensorineural deafness

    Institute of Scientific and Technical Information of China (English)

    Xianghong Wu; Xiumei Xie; Guotian Ma; Guoju Sun; Xiaobin Chen

    2006-01-01

    Objective: To determine whether a mutation of mitochondrial DNA induces familial dilated cardiomyopathy in Chinese families with cardiomyopathy, and analyzed the correlation between the genotype and phenotype. Methods: Affected members in three Chinese families of the familial dilated cardiomyopathy underwent clinical evaluation and DNA analysis. Polymerase chain reaction and direct DNA sequencing were used to screen for mitochondrial DNA mutation. The type of mtDNA vairations and clinical situation were analysed on the patients with mitochondrial DNA mutation. Results: The mitochondrial A3434G mutation was identified in one of the three families,the 3434 th nucleotide A was replaced by G, which led to change of amino acid. No mutations were identified in the clinically unaffected members of the family and all members of the other two families.Conclusion: This study indicates that the mitochondrial A3434G mutation maybe related with familial dilated cardiomyopathy and deafness.

  19. Dilated cardiomyopathy - approach made simplified with myocardial perfusion scintigraphy and cardiac viability studies

    International Nuclear Information System (INIS)

    Full text: To differentiate the ischemic vs nonischemic dilated cardiomyopathy and to assess myocardial viability in the ischemic cardiomyopathy. Materials and Methods: 34 patients (24 males and 10 females) with dilated cardiomyopathy diagnosed on echocardiography with reduced left ventricular ejection fraction and global hypokinesia were included between the period of Jan 2009 and July 2010. All the patients underwent rest myocardial perfusion scintigraphy first; 45 minutes after intravenous injection of 7 mCi of 99mTc MIBI. The stress myocardial perfusion imaging (after physical stress or gm/kg/min; 6 min infusion) was pharmacological stress with adenosine; 140 performed in the patients with normal perfusion at rest. The 18F FDG (5 mCi) cardiac viability studies were performed in patient with abnormal rest myocardial perfusion. The images were acquired on GE Infinia systems and processed on Emory toolbox (ECT) to study the ischemia and viability. Results: The mean left ventricular ejection fraction was found to be 27.38% at rest. The stress and rest perfusion scintigraphy was carried out in 20/34(58%) patients in whom 9(45%) patients underwent pharmacological stress with Inj adenosine and 11(55%) patients underwent physical stress. The stress induced ischemia was diagnosed in 12(60%) patients and infarct in 2(10%) patients with mixed ischemia and infarct pattern in 2(10%) patients. The nonischemic cause was diagnosed in 4 patients. The cardiac viability study was carried out in 14/34 (42%) patients with 6(42%) viable, 5(35%) nonviable and 3(21%) mixed viable and nonviable patterns were identified. Conclusion: The myocardial perfusion scintigraphy was the simplified approach for differentiating ischemic verses nonischemic dilated cardiomyopathy with addition of cardiac viability study (18FDG) made it one stop shop for the complete work-up of patients for further management

  20. Feasibility of Bone Marrow Stromal Cells Autologous Transplantation for Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    ZHOU Cheng; YANG Chenyuan; XIAO Shiliang; FEI Hongwen

    2007-01-01

    The feasibility of bone marrow stromal cells autologous transplantation for rabbit model of dilated cardiomyopathy induced by adriamycin was studied. Twenty rabbits received 2 mg/kg of adriamycin intravenously once a week for 8 weeks (total dose, 16 mg/kg) to induce the cardiomyopathy model with the monitoring of cardiac function by transthoracic echocardiography. Marrow stromal cells were isolated from cell-transplanted group rabbits and were culture-expanded on the 8th week. On the 10th week, cells were labeled with 4,6-diamidino-2-phenylindole (DAPI), and then injected into the myocardium of the same rabbits. The results showed that viable cells labeled with DAPI could be identified in myocardium at 2nd week after transplantation. Histological findings showed the injury of the myocardium around the injection site was relieved with less apoptosis and more expression of bcl-2. The echocardiography found the improvement of local tissue movement from (2.12±0.51) cm/s to (3.81±0.47) cm/s (P<0.05) around the inject site, but no improvement of heart function as whole. It was concluded bone marrow stromal cells transplantation for dilated cardiomyopathy was feasibe. The management of cells in vitro, the quantity and the pattern of the cells transplantation and the action mechanism still need further research.

  1. Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Peuhkurinen Keijo

    2011-06-01

    Full Text Available Abstract Background The purpose of this study was to identify early features of lamin A/C gene mutation related dilated cardiomyopathy (DCM with cardiovascular magnetic resonance (CMR. We characterise myocardial and functional findings in carriers of lamin A/C mutation to facilitate the recognition of these patients using this method. We also investigated the connection between myocardial fibrosis and conduction abnormalities. Methods Seventeen lamin A/C mutation carriers underwent CMR. Late gadolinium enhancement (LGE and cine images were performed to evaluate myocardial fibrosis, regional wall motion, longitudinal myocardial function, global function and volumetry of both ventricles. The location, pattern and extent of enhancement in the left ventricle (LV myocardium were visually estimated. Results Patients had LV myocardial fibrosis in 88% of cases. Segmental wall motion abnormalities correlated strongly with the degree of enhancement. Myocardial enhancement was associated with conduction abnormalities. Sixty-nine percent of our asymptomatic or mildly symptomatic patients showed mild ventricular dilatation, systolic failure or both in global ventricular analysis. Decreased longitudinal systolic LV function was observed in 53% of patients. Conclusions Cardiac conduction abnormalities, mildly dilated LV and depressed systolic dysfunction are common in DCM caused by a lamin A/C gene mutation. However, other cardiac diseases may produce similar symptoms. CMR is an accurate tool to determine the typical cardiac involvement in lamin A/C cardiomyopathy and may help to initiate early treatment in this malignant familiar form of DCM.

  2. Improvement of cardiac function after kidney transplantation with dilated cardiomyopathy and long dialysis vintage.

    Science.gov (United States)

    Mimura, Imari; Kawarazaki, Hiroo; Momose, Toshimitsu; Shibagaki, Yugo; Fujita, Toshiro

    2009-12-01

    Patients with long dialysis vintage have low cardiac output for various reasons. Although kidney transplantation is known to improve cardiac mortality, patients are sometimes evaluated as contraindicated for transplantation because of cardiac risk. We successfully performed kidney transplantation for a patient with a long dialysis vintage and dilated cardiomyopathy. Sequential (123)I-metaiodobenzylguanidine ((123)I-MIBG) scintigraphy suggested that amelioration of uraemia improved cardiac function. Kidney transplantation for patients with severely impaired cardiac function is safe and effective under careful perioperative monitoring irrespective of dialysis vintage. Sequential (123)I-MIBG scintigraphy can be used as an evaluation tool for the improvement in cardiac function.

  3. Investigation of HSP60 gene expression in mRNA level in heart at dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Riabenko D. V.

    2009-02-01

    Full Text Available The expression of HSP60 in the mRNA level in human hearts at the end-stage of dilated cardiomyopathy (DCM as well as in the hearts of mice with disease model similar to human DCM was investigated. We observed a significant increase in the Hsp60 mRNA level at the beginning of the disease and decrease to a normal level at the end stage. As the Hsp60 level was increased during the disease up to the end stage we can presume some changes in the regulation of Hsp60 synthesis or its degradation at DCM progression

  4. QT Dispersion Level and Its Clinical Significance.in Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To evaluate the clinical significance of QT dis persion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12-lead electrocardiograms ( ECGs) in 60 DCM patients and cormpared with 60 healthy subjects. Results The values of QTd and QTcd in DCM were significantly higher than those in control group ( P 110 ms and QTd≤110 ms(P0.05). Conclusion The values of QTd and QTcd increased in DCM patients were susceptive index for monitoring maligant VA in DCM, also important prognostic markers of CSD. QTd was correlated with NY HA functional class but not with EF and FS.

  5. De novo RRAGC mutation activates mTORC1 signaling in syndromic fetal dilated cardiomyopathy.

    Science.gov (United States)

    Long, Pamela A; Zimmermann, Michael T; Kim, Maengjo; Evans, Jared M; Xu, Xiaolei; Olson, Timothy M

    2016-08-01

    Idiopathic dilated cardiomyopathy (DCM) is a heritable, genetically heterogeneous disorder with variable age-dependent penetrance. We sought to identify the genetic underpinnings of syndromic, sporadic DCM in a newborn female diagnosed in utero. Postnatal evaluation revealed ventricular dilation and systolic dysfunction, bilateral cataracts, and mild facial dysmorphisms. Comprehensive metabolic and genetic testing, including chromosomal microarray, mitochondrial DNA and targeted RASopathy gene sequencing, and clinical whole exome sequencing for known cardiomyopathy genes was non-diagnostic. Following exclusion of asymptomatic DCM in the parents, trio-based whole exome sequencing was carried out on a research basis, filtering for rare, predicted deleterious de novo and recessive variants. An unreported de novo S75Y mutation was discovered in RRAGC, encoding Ras-related GTP binding C, an essential GTPase in nutrient-activated mechanistic target of rapamycin complex 1 (mTORC1) signaling. In silico protein modeling and molecular dynamics simulation predicted the mutation to disrupt ligand interactions and increase the GDP-bound state. Overexpression of RagC(S75Y) rendered AD293 cells partially insensitive to amino acid deprivation, resulting in increased mTORC1 signaling compared to wild-type RagC. These findings implicate mTORC1 dysregulation through a gain-of-function mutation in RagC as a novel molecular basis for syndromic forms of pediatric heart failure, and expand genotype-phenotype correlation in RASopathy-related syndromes. PMID:27234373

  6. Oral Chinese Herbal Medicine for Treatment of Dilated Cardiomyopathy: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Zhu, Yu-Shuo; Li, Yun-Lun; Ju, Jian-Qing; Du, Feng; Zang, Yan-Ping; Wang, Xiao-Bing; Sheng, Jie

    2016-01-01

    Dilated cardiomyopathy (DCM) is one of the main causes of heart failure and could increase death, hospitalization, and rehospitalization rate. The effect of conventional medicine treatment (CMT) is limited; meanwhile, the combination of CMT and Oral Chinese Herbal Medicine (OCHM) represents exciting adjunctive therapies. In this study, we ascertained the therapeutic effect of OCHM in combination with CMT for dilated cardiomyopathy by using meta-analysis methods for controlled clinical trials. We searched studies from five databases and extracted data from these studies. We also assessed the methodological quality of the included studies. We evaluated the following outcome measures to estimate the prognosis in patients with DCM: left ventricular ejection fraction (LVEF), left ventricular end-diastolic dimension (LVEDD), stroke volume (SV), brain natriuretic peptide (BNP), 6-minute walk test (6MWT), and overall efficacy. The result showed that OCHM combined with CMT for the improvement of therapeutic effect in DCM patients. However, the evidence remains weak due to the small sample size, high clinical heterogeneity, and poor methodological quality of the included trials. Further, large sample size and well-designed trials are needed. PMID:27630730

  7. Lamin A/C truncation in dilated cardiomyopathy with conduction disease

    Directory of Open Access Journals (Sweden)

    Huber Jill M

    2003-07-01

    Full Text Available Abstract Background Mutations in the gene encoding the nuclear membrane protein lamin A/C have been associated with at least 7 distinct diseases including autosomal dominant dilated cardiomyopathy with conduction system disease, autosomal dominant and recessive Emery Dreifuss Muscular Dystrophy, limb girdle muscular dystrophy type 1B, autosomal recessive type 2 Charcot Marie Tooth, mandibuloacral dysplasia, familial partial lipodystrophy and Hutchinson-Gilford progeria. Methods We used mutation detection to evaluate the lamin A/C gene in a 45 year-old woman with familial dilated cardiomyopathy and conduction system disease whose family has been well characterized for this phenotype 1. Results DNA from the proband was analyzed, and a novel 2 base-pair deletion c.908_909delCT in LMNA was identified. Conclusions Mutations in the gene encoding lamin A/C can lead to significant cardiac conduction system disease that can be successfully treated with pacemakers and/or defibrillators. Genetic screening can help assess risk for arrhythmia and need for device implantation.

  8. Myocarditis Leading to Severe Dilated Cardiomyopathy in a Patient with Dengue Fever

    Directory of Open Access Journals (Sweden)

    Hassan Tahir

    2015-01-01

    Full Text Available Background. Majority of dengue fever cases follow a benign self-limiting course but recently rare presentations and complications are increasingly seen due to rising burden of disease. Cardiac involvement in dengue fever with fatal outcome is a very rare complication. We report a case of 44-year-old patient who presented with symptoms of severe acute congestive heart secondary to myocarditis induced cardiomyopathy caused by dengue virus infection. Case Presentation. A 44-year-old man presented to ER with the complaints of high fever, fatigue, and shortness of breath. Patient was lethargic and blood pressure was low when he was brought to the ER. CXR showed cardiomegaly with pulmonary congestion and echocardiography revealed dilated left ventricle and ejection fraction of 10%. Patient condition worsened and he got admitted to the ICU because of acute hypoxic respiratory failure. Despite aggressive measures, patient died on day 5. Conclusion. Dilated cardiomyopathy is a rare complication of dengue myocarditis. Early recognition of acute DCM caused by dengue myocarditis is imperative in the management of dengue fever as early detection and management of cardiac failure can improve the survival of patient.

  9. Oral Chinese Herbal Medicine for Treatment of Dilated Cardiomyopathy: A Systematic Review and Meta-Analysis

    Science.gov (United States)

    Zhu, Yu-Shuo; Ju, Jian-Qing; Du, Feng; Zang, Yan-Ping; Wang, Xiao-Bing; Sheng, Jie

    2016-01-01

    Dilated cardiomyopathy (DCM) is one of the main causes of heart failure and could increase death, hospitalization, and rehospitalization rate. The effect of conventional medicine treatment (CMT) is limited; meanwhile, the combination of CMT and Oral Chinese Herbal Medicine (OCHM) represents exciting adjunctive therapies. In this study, we ascertained the therapeutic effect of OCHM in combination with CMT for dilated cardiomyopathy by using meta-analysis methods for controlled clinical trials. We searched studies from five databases and extracted data from these studies. We also assessed the methodological quality of the included studies. We evaluated the following outcome measures to estimate the prognosis in patients with DCM: left ventricular ejection fraction (LVEF), left ventricular end-diastolic dimension (LVEDD), stroke volume (SV), brain natriuretic peptide (BNP), 6-minute walk test (6MWT), and overall efficacy. The result showed that OCHM combined with CMT for the improvement of therapeutic effect in DCM patients. However, the evidence remains weak due to the small sample size, high clinical heterogeneity, and poor methodological quality of the included trials. Further, large sample size and well-designed trials are needed.

  10. Characterization of the human myocardial proteome in inflammatory dilated cardiomyopathy by label-free quantitative shotgun proteomics of heart biopsies

    NARCIS (Netherlands)

    Hammer, E.; Goritzka, M.; Ameling, S.; Darm, K.; Steil, L.; Klingel, K.; Trimpert, C.; Herda, L.R.; Dorr, M.; Kroemer, H.K.; Kandolf, R.; Staudt, A.; Felix, S.B.; Volker, U.

    2011-01-01

    Dilated cardiomyopathy (DCM) is characterized by contractile dysfunction leading to heart failure. The molecular changes in the human heart associated with this disease have so far mostly been addressed at the gene expression level and only a few studies have analyzed global changes in the myocardia

  11. Severe reversible dilated cardiomyopathy associated with a large left ventricular thrombus in a young child with middle aortic syndrome.

    Science.gov (United States)

    Ponniah, U; Overholt, E

    2014-01-01

    We report a case of a seven-year girl who presented with severe dilated cardiomyopathy (DCM) associated with a large thrombus in the left ventricle (LV). She had a long segment stenosis of the lower thoracic descending aorta, possibly due to non-specific aortitis and underwent successful stent angioplasty. The LV thrombus resolved after heparin without sequelae.

  12. Severe reversible dilated cardiomyopathy associated with a large left ventricular thrombus in a young child with middle aortic syndrome

    OpenAIRE

    Ponniah, U.; Overholt, E.

    2014-01-01

    this article reports a case of a seven-year girl who presented with severe dilated cardiomyopathy (DCM) associated with a large thrombus in the left ventricle (LV). She had a long segment stenosis of the lower thoracic descending aorta, possibly due to non-specific aortitis and underwent successful stent angioplasty. The LV thrombus resolved after heparin without sequelae.

  13. Our Experience with Autologous Bone Marrow Stem Cell Application in Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Mukund K

    2009-01-01

    Full Text Available Background - Use of autologous bone marrow stem cell is a newly evolving treatment modality for end stage cardiac failure as reported in the literature. We report our experience with two patients with dilated cardiomyopathy who underwent this treatment after failure of maximal conventional therapy. Methods - A 29 year old Male patient with history of orthopnea and PND, with a diagnosis of dilated cardiomyopathy and echocardiographic evidence of severe LV dysfunction was referred for further treatment. His echo on admission showed EF of 17% and no other abnormal findings except elevated bilirubin levels. He was in NYHA functional class IV. He received intracoronary injection of autologous bone marrow stem cells in January 2009. 254X106 cells were injected with a CD34+ of 0.20%. His clinical condition stabilized and he was discharged home. He received a second injection of 22X106 in vitro expanded stem cells with a CD34+ of 0.72% in Aug 2009. He is now in NYHA class II-III with EF 24%. A 31year old Male patient with history of increasing shortness of breath, severe over the past 3-4 days was admitted for evaluation and treatment. His echo on admission showed EF of 20% and was in NYHA functional class IV. Coronary angiogram was normal and he was stabilized on maximal anti failure measures. He received intracoronary autologous bone marrow stem cell injection of 56X106 with a CD34+ of 0.53% in August 2009. His clinical condition stabilized over the next 10 days and he was discharged home. Conclusions - In our experience of two cases of dilated cardiomyopathy, safety of intracoronary injection of autologous bone marrow stem cells both isolated and in vitro expanded has been proven in both the cases with efficacy proven in one of the cases. Long term follow-up of these two cases and inclusion of more number of similar cases where all available conventional therapies have not resulted in significant improvement for such studies are planned.

  14. Anaesthetic management of a patient with dilated cardiomyopathy posted for non-cardiac surgery

    Directory of Open Access Journals (Sweden)

    Madhusudan M

    2016-07-01

    Full Text Available Dilated cardiomyopathy (DCM is characterized by impaired ventricular contractility and causes concern in anaesthetic management as it may sometimes predispose to malignant arrhythmias. A 77-year-old woman diagnosed to have irreducible umbilical hernia, was posted for emergency laparotomy and hernia repair. On examination, she belonged to American Society of Anesthesiologists (ASA physical status grade III with a functional status of 4 metabolic equivalents (METs. She was also suffering from DCM with severe left ventricular (LV dysfunction (LV ejection fraction 25%. This patient was successfully managed by administering general anaesthesia with ProSeal laryngeal mask airway. We report the detailed anaesthetic management of this patient who underwent emergency laparotomy and hernia repair.

  15. MLP and CARP are linked to chronic PKCα signalling in dilated cardiomyopathy.

    Science.gov (United States)

    Lange, Stephan; Gehmlich, Katja; Lun, Alexander S; Blondelle, Jordan; Hooper, Charlotte; Dalton, Nancy D; Alvarez, Erika A; Zhang, Xiaoyu; Bang, Marie-Louise; Abassi, Yama A; Dos Remedios, Cristobal G; Peterson, Kirk L; Chen, Ju; Ehler, Elisabeth

    2016-01-01

    MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signalling processes is still enigmatic. Elevated PKCα signalling activity is known to be an important contributor to heart failure. Here we show that MLP directly inhibits the activity of PKCα. In end-stage DCM, PKCα is concentrated at the intercalated disc of cardiomyocytes, where it is sequestered by the adaptor protein CARP in a multiprotein complex together with PLCβ1. In mice deficient for both MLP and CARP the chronic PKCα signalling chain at the intercalated disc is broken and they remain healthy. Our results suggest that the main role of MLP in heart lies in the direct inhibition of PKCα and that chronic uninhibited PKCα activity at the intercalated disc in the absence of functional MLP leads to heart failure. PMID:27353086

  16. Review on CFD simulation in heart with dilated cardiomyopathy and myocardial infarction.

    Science.gov (United States)

    Chan, Bee Ting; Lim, Einly; Chee, Kok Han; Abu Osman, Noor Azuan

    2013-05-01

    The heart is a sophisticated functional organ that plays a crucial role in the blood circulatory system. Hemodynamics within the heart chamber can be indicative of exert cardiac health. Due to the limitations of current cardiac imaging modalities, computational fluid dynamics (CFD) have been widely used for the purposes of cardiac function assessment and heart disease diagnosis, as they provide detailed insights into the cardiac flow field. An understanding of ventricular hemodynamics and pathological severities can be gained through studies that employ the CFD method. In this research the hemodynamics of two common myocardial diseases, dilated cardiomyopathy (DCM) and myocardial infarction (MI) were investigated, during both the filling phase and the whole cardiac cycle, through a prescribed geometry and fluid structure interaction (FSI) approach. The results of the research indicated that early stage disease identification and the improvement of cardiac assisting devices and therapeutic procedures can be facilitated through the use of the CFD method. PMID:23428371

  17. RESIDENT PROGENITOR CARDIAC CELLS IN PATIENTS WITH DILATED CARDIOMYOPATHY AND CONGESTIVE HEART FAILURE

    Directory of Open Access Journals (Sweden)

    T. G. Kulikova

    2014-01-01

    Full Text Available Aim. To study content of resident progenitor cardiomyocytes in endomyocardial biopsy samples of patients with dilated cardiomyopathy (DCM and heart failure (HF at different disease stages and relate it to patient clinical characteristics.Material and methods. Resident progenitor cardiomyocytes were studied in endomyocardial biopsy samples from 14 patients (age from 26 to 52 years old with DCM and HF by immunofluorescence method. Results were analyzed individually for each patient.Results. Resident progenitor cardiomyocytes expressing simultaneously stem cell markers c-kit, MDR-1 and early cardiomyocyte differentiation markers GATA-4 and Nkx2.5 were found in endomyocardial biopsy samples from patients with DCM and HF. Resident progenitor cardiomyocytes detected by these cell markers were found in all patients at all disease stages.Conclusion. Results show that the myocardial regenerative processes exist at all stages of the disease progression.

  18. RESIDENT PROGENITOR CARDIAC CELLS IN PATIENTS WITH DILATED CARDIOMYOPATHY AND CONGESTIVE HEART FAILURE

    Directory of Open Access Journals (Sweden)

    T. G. Kulikova

    2015-09-01

    Full Text Available Aim. To study content of resident progenitor cardiomyocytes in endomyocardial biopsy samples of patients with dilated cardiomyopathy (DCM and heart failure (HF at different disease stages and relate it to patient clinical characteristics.Material and methods. Resident progenitor cardiomyocytes were studied in endomyocardial biopsy samples from 14 patients (age from 26 to 52 years old with DCM and HF by immunofluorescence method. Results were analyzed individually for each patient.Results. Resident progenitor cardiomyocytes expressing simultaneously stem cell markers c-kit, MDR-1 and early cardiomyocyte differentiation markers GATA-4 and Nkx2.5 were found in endomyocardial biopsy samples from patients with DCM and HF. Resident progenitor cardiomyocytes detected by these cell markers were found in all patients at all disease stages.Conclusion. Results show that the myocardial regenerative processes exist at all stages of the disease progression.

  19. Epidemiological study of dilated cardiomyopathy from eastern India with special reference to left atrial size

    Directory of Open Access Journals (Sweden)

    Rudrajit Paul, Saumen Nandi, Pradip K Sinha

    2014-07-01

    Full Text Available Dilated cardiomyopathy (DCM is a common cause of emergency visit in our country. The disease is often misdiagnosed and mistreated. There are very few studies on DCM from India. We undertook a small study on DCM patients from Eastern India to find the demographic and echocardiographic characteristics. Patients and methods: We under took this study in a tertiary care Medical College of Eastern India. All patients coming to the emergency with dyspnea were evaluated for cardiac dysfunction. Emergency echocardiography was done to diagnose dilated cardiomyopathy. Patients with DCM were then evaluated as per protocol. After stabilization, echocardiography was repeated to note the study parameters like left atrial diameter. Standard statistical tests were used. Results: we had a total of 70 patients in our study with a male: female ratio of 43:27. Most patients were aged over 40 years. Patients with COPD, history of radiation, malignancy or drug abuse were excluded. Most patients (47% were on NYHA stage 3 at the time of presentation. In our patient cohort, 24% were alcoholic and 46% were smokers. Atrial fibrillation was present in 15.7% of the patients and right and left bundle branch block had been present in 8 and 15 patients respectively. In echocardiography, increased left atrial (LA size (>40 mm was found in 45 patients. Many patients had valvular regurgitation, mitral, aortic or tricuspid. LA size was positively correlated with left ventricular systolic diameter (r=0.403 and negatively correlated with ejection fraction (r= -0.23. Analysis and conclusion: different ECG abnormalities like bundle branch block and arrhythmias like atrial fibrillation are quite common in DCM. In echocardiography, left atrial size is an important prognostic marker and correlates with left ventricular function.

  20. Adverse events in families with hypertrophic or dilated cardiomyopathy and mutations in the MYBPC3 gene

    Directory of Open Access Journals (Sweden)

    Lehrke Stephanie

    2008-10-01

    Full Text Available Abstract Background Mutations in MYBPC3 encoding myosin binding protein C belong to the most frequent causes of hypertrophic cardiomyopathy (HCM and may also lead to dilated cardiomyopathy (DCM. MYBPC3 mutations initially were considered to cause a benign form of HCM. The aim of this study was to examine the clinical outcome of patients and their relatives with 18 different MYBPC3 mutations. Methods 87 patients with HCM and 71 patients with DCM were screened for MYBPC3 mutations by denaturing gradient gel electrophoresis and sequencing. Close relatives of mutation carriers were genotyped for the respective mutation. Relatives with mutation were then evaluated by echocardiography and magnetic resonance imaging. A detailed family history regarding adverse clinical events was recorded. Results In 16 HCM (18.4% and two DCM (2.8% index patients a mutation was detected. Seven mutations were novel. Mutation carriers exhibited no additional mutations in genes MYH7, TNNT2, TNNI3, ACTC and TPM1. Including relatives of twelve families, a total number of 42 mutation carriers was identified of which eleven (26.2% had at least one adverse event. Considering the twelve families and six single patients with mutations, 45 individuals with cardiomyopathy and nine with borderline phenotype were identified. Among the 45 patients, 23 (51.1% suffered from an adverse event. In eleven patients of seven families an unexplained sudden death was reported at the age between 13 and 67 years. Stroke or a transient ischemic attack occurred in six patients of five families. At least one adverse event occurred in eleven of twelve families. Conclusion MYBPC3 mutations can be associated with cardiac events such as progressive heart failure, stroke and sudden death even at younger age. Therefore, patients with MYBPC3 mutations require thorough clinical risk assessment.

  1. Arginylation regulates myofibrils to maintain heart function and prevent dilated cardiomyopathy

    Science.gov (United States)

    Kurosaka, Satoshi; Leu, N. Adrian; Pavlov, Ivan; Han, Xuemei; Ribeiro, Paula Aver Bretanha; Xu, Tao; Bunte, Ralph; Saha, Sougata; Wang, Junling; Cornachione, Anabelle; Mai, Wilfried; Yates, John R; Rassier, Dilson E.; Kashina, Anna

    2012-01-01

    Protein arginylation mediated by arginyltransferase (ATE1) is essential for heart formation during embryogenesis, however its cell-autonomous role in cardiomyocytes and the differentiated heart muscle has never been investigated. To address this question, we generated cardiac muscle-specific Ate1 knockout mice, in which Ate1 deletion was driven by α-myosin heavy chain promoter (αMHC-Ate1 mouse). These mice were initially viable, but developed severe cardiac contractility defects, dilated cardiomyopathy, and thrombosis over time, resulting in high rates of lethality after 6 months of age. These symptoms were accompanied by severe ultrastructural defects in cardiac myofibrils, seen in the newborns and far preceding the onset of cardiomyopathy, suggesting that these defects were primary and likely underlay the development of the future heart defects. Several major sarcomeric proteins were arginylated in vivo. Moreover, Ate1 deletion in the hearts resulted in a significant reduction of active and passive myofibril forces, suggesting that arginylation is critical for both myofibril structural integrity and contractility. Thus, arginylation is essential for maintaining the heart function by regulation of the major myofibril proteins and myofibril forces, and its absence in the heart muscle leads to progressive heart failure through cardiomyocyte-specific defects. PMID:22626847

  2. A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.

    Science.gov (United States)

    Galant, Damien; Gaborit, Bénédicte; Desgrouas, Camille; Abdesselam, Ines; Bernard, Monique; Levy, Nicolas; Merono, Françoise; Coirault, Catherine; Roll, Patrice; Lagarde, Arnaud; Bonello-Palot, Nathalie; Bourgeois, Patrice; Dutour, Anne; Badens, Catherine

    2016-01-01

    ZMPSTE24 encodes the only metalloprotease, which transforms prelamin into mature lamin A. Up to now, mutations in ZMPSTE24 have been linked to Restrictive Dermopathy (RD), Progeria or Mandibulo-Acral Dysplasia (MAD). We report here the phenotype of a patient referred for severe metabolic syndrome and cardiomyopathy, carrying a mutation in ZMPSTE24. The patient presented with a partial lipodystrophic syndrome associating hypertriglyceridemia, early onset type 2 diabetes, and android obesity with truncal and abdominal fat accumulation but without subcutaneous lipoatrophy. Other clinical features included acanthosis nigricans, liver steatosis, dilated cardiomyopathy, and high myocardial and hepatic triglycerides content. Mutated fibroblasts from the patient showed increased nuclear shape abnormalities and premature senescence as demonstrated by a decreased Population Doubling Level, an increased beta-galactosidase activity and a decreased BrdU incorporation rate. Reduced prelamin A expression by siRNA targeted toward LMNA transcripts resulted in decreased nuclear anomalies. We show here that a central obesity without subcutaneous lipoatrophy is associated with a laminopathy due to a heterozygous missense mutation in ZMPSTE24. Given the high prevalence of metabolic syndrome and android obesity in the general population, and in the absence of familial study, the causative link between mutation and phenotype cannot be formally established. Nevertheless, altered lamina architecture observed in mutated fibroblasts are responsible for premature cellular senescence and could contribute to the phenotype observed in this patient.

  3. A young man with hemoptysis: Rare association of idiopathic pulmonary hemosiderosis, celiac disease and dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Gopi C Khilnani

    2015-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare cause of recurrent diffuse alveolar hemorrhage (DAH with no specific treatment. Herein, we discuss a case of hemoptysis, who had IPH and other rare associations. A 19-year-old man presented with recurrent hemoptysis, generalized weakness and progressive dyspnea for 3 years. Earlier, he was diagnosed with anemia and was treated with blood transfusions and hematinics. On examination he had pallor, tachycardia and was underweight. Investigations revealed low level of hemoglobin (7.8 g/dl and iron deficiency. An electrocardiography (ECG showed sinus tachycardia, interventricular conduction delay and T-wave inversion. Echocardiography revealed dilated cardiomyopathy with left ventricular dysfunction. Computed tomography of the chest demonstrated bilateral diffuse ground glass opacity suggestive of pulmonary hemorrhage. Pulmonary function tests showed restrictive pattern with increased carbon monoxide diffusion. Bronchoalveolar lavage and transbronchial lung biopsy showed hemosiderin-laden macrophages. Patient could recall recurrent episodes of diarrhea in childhood. Serum antitissue transglutamase antibodies were raised (291.66 IU/ml, normal <30 IU/ml. Duodenal biopsy showed subtotal villous atrophy consistent with celiac disease. He was started on gluten-free diet, beta blockers and diuretics. After two years of treatment, he has been showing consistent improvement. Screening for CD is important in patients with IPH. Cardiomyopathy forms rare third association. All three show improvement with gluten-free diet.

  4. Right ventricular bifocal stimulation in the treatment of dilated cardiomyopathy with heart failure

    Directory of Open Access Journals (Sweden)

    José Carlos Pachón Mateos

    1999-12-01

    Full Text Available OBJECTIVE: To describe a new more efficient method of endocardial cardiac stimulation, which produces a narrower QRS without using the coronary sinus or cardiac veins. METHODS: We studied 5 patients with severe dilated cardiomyopathy, chronic atrial fibrillation and AV block, who underwent definitive endocardial pacemaker implantation, with 2 leads, in the RV, one in the apex and the other in the interventricular septum (sub pulmonary, connected, respectively, to ventricular and atrial bicameral pacemaker outputs. Using Doppler echocardiography, we compared, in the same patient, conventional (VVI, high septal ("AAI" and bifocal ("DDT" with AV interval ~ 0 stimulation. RESULTS: The RV bifocal stimulation had the best results with an increase in ejection fraction and cardiac output and reduction in QRS duration, mitral regurgitation and in the left atrium area (p <= 0.01. The conventional method of stimulation showed the worst result. CONCLUSION: These results suggest that, when left ventricular stimulation is not possible, right ventricular bifocal stimulation should be used in patients with severe cardiomyopathy where a pacemaker is indicated.

  5. Multiomics approach to identify novel biomarkers for dilated cardiomyopathy: Proteome and transcriptome analyses of 4C30 dilated cardiomyopathy mouse model.

    Science.gov (United States)

    Nishigori, Mitsuhiro; Yagi, Hiroaki; Mochiduki, Akikazu; Minamino, Naoto

    2016-11-01

    Dilated cardiomyopathy (DCM) is an intractable disease, without any radical treatment option other than cardiac transplantation. Additionally, biomarkers to determine progressive staging are not yet available. Irrespective of the diversity of causative gene mutations, the phenotype of DCM is rather common. Therefore, it is plausible to determine DCM staging in terms of variations in protein and mRNA levels. In this study, we performed proteome and transcriptome analysis of the left ventricle of 4C30 DCM model mice showing mild and severe phenotypes at 12 and 24 weeks (wk) after birth, respectively. Proteomic analyses results showed 109 proteins that increased and 133 others that decreased among 1874 detected proteins. We selected biomarker candidates by confirming consistent alterations in protein levels at 12 and 24 wk, and mRNA levels at 12 wk, and narrowed these down based on the requirement that they should be detectable in blood. Finally, we selected six biomarker candidates based on sustained or augmented alteration at 24 wk and confirmed their definite alterations in the left ventricle by quantitative polymerase chain reaction, western blot analysis, and multiple reaction monitoring (MRM). To assess the validity of this strategy, we measured plasma concentrations of the six candidates by MRM method and identified two proteins (FTL1 and GRP78) that demonstrated significant elevation in the 4C30 mice plasma. Taken together, a multiomics strategy comparing tissue expression levels of proteins and mRNAs between diseased and control groups, with appropriate confirmation, is a promising approach for the discovery of new biomarkers. © 2016 Wiley Periodicals, Inc. Biopolymers (Pept Sci) 106: 491-502, 2016. PMID:26799926

  6. Becker Muscular Dystrophy (BMD) caused by duplication of exons 3-6 of the dystrophin gene presenting as dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Tsai, A.C.; Allingham-Hawkins, D.J.; Becker, L. [Univ. of Toronto, Ontario (Canada)] [and others

    1994-09-01

    X-linked dilated cardiomyopathy (XLCM) is a progressive myocardial disease presenting with congestive heart failure in teenage males without clinical signs of skeletal myopathy. Tight linkage of XLCM to the DMD locus has been demonstrated; it has been suggested that, at least in some families, XLCM is a {open_quotes}dystrophinopathy.{close_quotes} We report a 14-year-old boy who presented with acute heart failure due to dilated cardiomyopathy. He had no history of muscle weakness, but physical examination revealed pseudohypertrophy of the calf muscles. He subsequently received a heart transplantation. Family history was negative. Serum CK level at the time of diagnosis was 10,416. Myocardial biopsy showed no evidence of carditis. Dystrophin staining of cardiac and skeletal muscle with anti-sera to COOH and NH{sub 2}termini showed a patchy distribution of positivity suggestive of Becker muscular dystrophy. Analysis of 18 of the 79 dystrophin exons detected a duplication that included exons 3-6. The proband`s mother has an elevated serum CK and was confirmed to be a carrier of the same duplication. A mutation in the muscle promotor region of the dystrophin gene has been implicated in the etiology of SLCM. However, Towbin et al. (1991) argued that other 5{prime} mutations in the dystrophin gene could cause selective cardiomyopathy. The findings in our patient support the latter hypothesis. This suggests that there are multiple regions in the dystrophin gene which, when disrupted, can cause isolated dilated cardiomyopathy.

  7. Effects of candesartan on electrical remodeling in the hearts of inherited dilated cardiomyopathy model mice.

    Directory of Open Access Journals (Sweden)

    Fuminori Odagiri

    Full Text Available Inherited dilated cardiomyopathy (DCM is characterized by dilatation and dysfunction of the ventricles, and often results in sudden death or heart failure (HF. Although angiotensin receptor blockers (ARBs have been used for the treatment of HF, little is known about the effects on postulated electrical remodeling that occurs in inherited DCM. The aim of this study was to examine the effects of candesartan, one of the ARBs, on cardiac function and electrical remodeling in the hearts of inherited DCM model mice (TNNT2 ΔK210. DCM mice were treated with candesartan in drinking water for 2 months from 1 month of age. Control, non-treated DCM mice showed an enlargement of the heart with prolongation of QRS and QT intervals, and died at t1/2 of 70 days. Candesartan dramatically extended the lifespan of DCM mice, suppressed cardiac dilatation, and improved the functional parameters of the myocardium. It also greatly suppressed prolongation of QRS and QT intervals and action potential duration (APD in the left ventricular myocardium and occurrence of ventricular arrhythmia. Expression analysis revealed that down-regulation of Kv4.2 (Ito channel protein, KChIP2 (auxiliary subunit of Kv4.2, and Kv1.5 (IKur channel protein in DCM was partially reversed by candesartan administration. Interestingly, non-treated DCM heart had both normal-sized myocytes with moderately decreased Ito and IKur and enlarged cells with greatly reduced K+ currents (Ito, IKur IK1 and Iss. Treatment with candesartan completely abrogated the emergence of the enlarged cells but did not reverse the Ito, and IKur in normal-sized cells in DCM hearts. Our results indicate that candesartan treatment suppresses structural remodeling to prevent severe electrical remodeling in inherited DCM.

  8. Aberrant sialylation causes dilated cardiomyopathy and stress-induced heart failure.

    Science.gov (United States)

    Deng, Wei; Ednie, Andrew R; Qi, Jianyong; Bennett, Eric S

    2016-09-01

    Dilated cardiomyopathy (DCM), the third most common cause of heart failure, is often associated with arrhythmias and sudden cardiac death if not controlled. The majority of DCM is of unknown etiology. Protein sialylation is altered in human DCM, with responsible mechanisms not yet described. Here we sought to investigate the impact of clinically relevant changes in sialylation on cardiac function using a novel model for altered glycoprotein sialylation that leads to DCM and to chronic stress-induced heart failure (HF), deletion of the sialyltransferase, ST3Gal4. We previously reported that 12- to 20-week-old ST3Gal4 (-/-) mice showed aberrant cardiac voltage-gated ion channel sialylation and gating that contribute to a pro-arrhythmogenic phenotype. Here, echocardiography supported by histology revealed modest dilated and thinner-walled left ventricles without increased fibrosis in ST3Gal4 (-/-) mice starting at 1 year of age. Cardiac calcineurin expression in younger (16-20 weeks old) ST3Gal4 (-/-) hearts was significantly reduced compared to WT. Transverse aortic constriction (TAC) was used as a chronic stressor on the younger mice to determine whether the ability to compensate against a pathologic insult is compromised in the ST3Gal4 (-/-) heart, as suggested by previous reports describing the functional implications of reduced cardiac calcineurin levels. TAC'd ST3Gal4 (-/-) mice presented with significantly reduced systolic function and ventricular dilation that deteriorated into congestive HF within 6 weeks post-surgery, while constricted WT hearts remained well-adapted throughout (ejection fraction, ST3Gal4 (-/-) = 34 ± 5.2 %; WT = 53.8 ± 7.4 %; p stress-induced HF. PMID:27506532

  9. Postpartum dilated cardiomyopathy in a patient with systemic lupus erythematosus, nephritis and lupus anticoagulant: a diagnostic dilemma

    Science.gov (United States)

    Hall, Daniel; New, David; Kelly, Teresa

    2011-01-01

    A 32-year-old Caucasian woman presented with shortness of breath four weeks postpartum. She was known to suffer from systemic lupus erythematosus with cutaneous, joint and minor renal involvement. During pregnancy, the patient had developed nephrotic syndrome for which she was managed with prophylactic anticoagulation and corticosteroid therapy. A leg deep vein thrombosis had arisen following caesarean section following antepartum haemorrhage. Examination revealed a heart murmur, and pulmonary signs. Computed tomography pulmonary angiogram showed cardiomegaly and bilateral pleural effusions but no pulmonary embolus. Echocardiogram demonstrated dilated cardiomyopathy. An initial diagnosis of peripartum cardiomyopathy was considered, with lupus myocarditis and coronary in situ thrombosis among the differential diagnoses.

  10. Impaired cardiac adrenergic innervation assessed by MIBG imaging as a predictor of treatment response in childhood dilated cardiomyopathy

    OpenAIRE

    Acar, P; Merlet, P.; Iserin, L; Bonnet, D.; Sidi, D; Syrota, A; Kachaner, J

    2001-01-01

    OBJECTIVE—To evaluate the prognostic value of metaiodobenzylguanidine (MIBG) imaging in childhood cardiomyopathy.
DESIGN—Prospective cohort study.
SETTING—Tertiary referral centre.
PATIENTS—40 children (21 boys, 19 girls; mean (SD) age, 7.0 (5.6) years) with heart failure resulting from idiopathic dilated cardiomyopathy (n = 23) or various other disorders (n = 17).
METHODS—At the initial examination, cardiac 123I-MIBG uptake and release, circulating noradrenaline (norepinephrine) concentratio...

  11. Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice: a position statement of the ESC working group on myocardial and pericardial diseases.

    Science.gov (United States)

    Pinto, Yigal M; Elliott, Perry M; Arbustini, Eloisa; Adler, Yehuda; Anastasakis, Aris; Böhm, Michael; Duboc, Denis; Gimeno, Juan; de Groote, Pascal; Imazio, Massimo; Heymans, Stephane; Klingel, Karin; Komajda, Michel; Limongelli, Giuseppe; Linhart, Ales; Mogensen, Jens; Moon, James; Pieper, Petronella G; Seferovic, Petar M; Schueler, Stephan; Zamorano, Jose L; Caforio, Alida L P; Charron, Philippe

    2016-06-14

    In this paper the Working Group on Myocardial and Pericardial Disease proposes a revised definition of dilated cardiomyopathy (DCM) in an attempt to bridge the gap between our recent understanding of the disease spectrum and its clinical presentation in relatives, which is key for early diagnosis and the institution of potential preventative measures. We also provide practical hints to identify subsets of the DCM syndrome where aetiology directed management has great clinical relevance. PMID:26792875

  12. Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-Thalassemia Major; a Double-Blind Randomized Controlled Trial

    OpenAIRE

    Gholam-Hossein Ajami; Hamid Amoozgar; Mohammad Borzouee; Mehran Karimi; Farah Piravian; Afsaneh Ashrafi; Zahra Kheirandish

    2010-01-01

    Objective: Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy.Methods: During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. A...

  13. Prognostic value of late gadolinium enhancement in dilated cardiomyopathy patients: a meta-analysis

    International Nuclear Information System (INIS)

    Aim: To evaluate the association between late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) and major adverse events in dilated cardiomyopathy (DCM) patients. Materials and methods: Databases, including PubMed, Ovid, and EMBASE, were searched for studies evaluating LGE at CMR in DCM patient prognostication. Clinical outcomes were analysed using fixed-effects models or, in cases of significant heterogeneity, random-effects models. Results: In the meta-analysis of 13 studies on 1675 DCM patients with a mean follow-up of 3 years, LGE is associated with all-cause mortality (pooled odds ratio, 3.43 [95% confidence interval, 2.26–5.22], p<0.00001), cardiac death/transplantation (3.65 [1.80–7.40], p=0.0003), hospitalisation for heart failure (2.87 [1.53–5.39], p=0.001), major arrhythmia events (sudden cardiac death, sustained ventricular tachycardia or fibrillation, appropriate implantable cardioverter–defibrillator (ICD) discharge/pacing, and syncope: 4.24 [2.95–6.08], p<0.00001), and sudden cardiac death (3.33 [1.80–6.17], p=0.0001). Conclusion: LGE in DCM patients appears to be associated with mortality and major cardiac events, underscoring its potential as an independent index for risk stratification and treatment guidance. -- Highlights: •Cardiac magnetic resonance imaging is a flourishing technique in the field of cardiomyopathy examination for its high space resolution. •Late gadolinium enhancement appears associated with increased risk for all-cause mortality and major cardiavascular events. •Late gadolinium enhancement by cardiac magnetic resonance might guide treatment and clinical care of heart failure patients

  14. Clinical Profile and Prognosis of Patients with Right Ventricular Dilated Cardiomyopathy: Results of a Prospective Study

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    Ya.R. Akhmatov

    2015-12-01

    Full Text Available The aim of our study was to investigate the clinical prevalence of dilated cardiomyopathy (DCM with predominantly failure of the right-side heart (right ventricular DCM, RV-DCM, and features of the clinical course and prognosis of the disease compared to DCM with biventricular heart failure (BV-HF. The study design suggests a prospective observation of 300 patients with idiopathic DCM between 2000 and 2012. Herewith, we followed the criteria of the WHO/ISFC Task Force (1995 on the Definationa and Classification of Cardiomyopathies. All patients underwent a comprehensive examination. Two groups were formed for further comparative analysis. Group 1 included 22 patients (mean age 42.9±14.3 years, male/female 5/17 with RV-DCM. Group 2 included 38 patients (mean age 43.6±13.8, male/female 29/9 with DCM and BV-HF. The groups were matched for age, sex, NYHA class II-III, and disease duration. According to our aim, we studied 5-year survival prognosis and analyzed the incidence and causes of deaths, as well as the occurrence of nonfatal complications of the disease. Medical therapy for DCM patients was performed according to the CHF therapy guidelines (ACC/AHA 2001, 2005. The results of our investigations during many years of research have shown that the clinical incidence of RV-DCM was 7.3% among all forms of DCM. The study of life prognosis in patients with 2 forms of DCM showed that 5-year mortality of patients was about 50%. Herewith, we detected the differences in causes of death depending on the type of heart damage, primarily development of fatal pulmonary embolism.

  15. Role of coxsackievirus and adenovirus receptor in the pathogenesis of dilated cardiomyopathy and its influencing factor

    Institute of Scientific and Technical Information of China (English)

    ZHANG Shuo; JIA Hai-bo; GONG Bin-sheng; ZHANG Shao-jun; LI Xia; YU Bo

    2008-01-01

    Background Although clinical treatment for heart failure and sudden death has been improved over the last few decades, the morbidity and mortality of dilated cardiomyopathy (DCM) have increased. So a better understanding of the underlying molecular events leading to DCM is urgent. Persistent viral infection (especially coxsackievirus group B) of the myocardium in viral myocarditis and DCM has never been neglected by experts. Recent data indicate that the up-regulation of coxsackievirus and adenovirus receptor (CAR) in viral cardiomyopathy contributes to viral infection as a key factor in the pathogenesis of this disease. This study aimed to investigate the role and regulatory mechanism of CAR in DCM by the bioinformatic method.Methods We identified the clusters of genes co-expressed with CAR by clustering algorithm based on the public available microarray dataset of DCM (Kittleson, et al. 2005), and mapped these genes into the protein-protein interaction networks to investigate the interaction relationship to each other at the protein level after confirming that the samples are characterized by the cluster of genes in correctly partitioning.Results The gene cluster GENESET 11 containing 33 genes including CAR with similar expression pattem was identified by cluster algorithm, of which 19 genes were found to have interaction information of the protein encoded by them in the current human protein interaction database. Especially, 12 genes present as critical nodes (called HUB node) at the protein level are involved in energy metabolism, signal transduction, viral infection, immuno-response, cell apoptosis, cell proliferation, tissue repair, etc.Conclusions The genes in GENESET 11 together with CAR may play a pathogenic role in the development of DCM, mainly involved in the mechanism of energy metabolism, signal transduction, viral infection, immuno-response, cell apoptosis and tissue repair.

  16. Cardiac-specific deletion of the microtubule-binding protein CENP-F causes dilated cardiomyopathy

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    Ellen Dees

    2012-07-01

    CENP-F is a large multifunctional protein with demonstrated regulatory roles in cell proliferation, vesicular transport and cell shape through its association with the microtubule (MT network. Until now, analysis of CENP-F has been limited to in vitro analysis. Here, using a Cre-loxP system, we report the in vivo disruption of CENP-F gene function in murine cardiomyocytes, a cell type displaying high levels of CENP-F expression. Loss of CENP-F function in developing myocytes leads to decreased cell division, blunting of trabeculation and an initially smaller, thin-walled heart. Still, embryos are born at predicted mendelian ratios on an outbred background. After birth, hearts lacking CENP-F display disruption of their intercalated discs and loss of MT integrity particularly at the costamere; these two structures are essential for cell coupling/electrical conduction and force transduction in the heart. Inhibition of myocyte proliferation and cell coupling as well as loss of MT maintenance is consistent with previous reports of generalized CENP-F function in isolated cells. One hundred percent of these animals develop progressive dilated cardiomyopathy with heart block and scarring, and there is a 20% mortality rate. Importantly, although it has long been postulated that the MT cytoskeleton plays a role in the development of heart disease, this study is the first to reveal a direct genetic link between disruption of this network and cardiomyopathy. Finally, this study has broad implications for development and disease because CENP-F loss of function affects a diverse array of cell-type-specific activities in other organs.

  17. Different effects of bisoprolol on heart rate in patients with ischemic or idiopathic dilated cardiomyopathy (A 24-hour Holter substudy of the Cardiac Insufficiency Bisoprolol Study [CIBIS])

    NARCIS (Netherlands)

    Anthonio, RL; Brouwer, J; Lechat, P; Haaksma, J; van der Ven, L; van Veldhuisen, DJ; Crijns, HJGM; van Gilst, WH

    1999-01-01

    The effect of beta blockade on heart rate in patients with either idiopathic or ischemic cardiomyopathy was studied. It was found that beta blockade reduced the early morning increase in heart rate to a greater extent in patients with idiopathic dilated cardiomyopathy than in those with ischemic dil

  18. Autoantibodies in dilated cardiomyopathy induce vascular endothelial growth factor expression in cardiomyocytes

    Energy Technology Data Exchange (ETDEWEB)

    Saygili, Erol, E-mail: erol.saygili@med.uni-duesseldorf.de [Division of Cardiology, Pulmonology, and Vascular Medicine, University Hospital Düsseldorf, Moorenstrasse 5, D-40225 Düsseldorf (Germany); Noor-Ebad, Fawad; Schröder, Jörg W.; Mischke, Karl [Department of Cardiology, University RWTH Aachen, Pauwelsstr. 30, D-52074 Aachen (Germany); Saygili, Esra [Clinic for Gastroenterology, Hepatology and Infectious Diseases, Heinrich-Heine-University, Moorenstrasse 5, D-40225 Düsseldorf (Germany); Rackauskas, Gediminas [Department of Cardiovascular Medicine, Vilnius University Hospital Santariskiu Klinikos, Vilnius University (Lithuania); Marx, Nikolaus [Department of Cardiology, University RWTH Aachen, Pauwelsstr. 30, D-52074 Aachen (Germany); Kelm, Malte; Rana, Obaida R. [Division of Cardiology, Pulmonology, and Vascular Medicine, University Hospital Düsseldorf, Moorenstrasse 5, D-40225 Düsseldorf (Germany)

    2015-09-11

    Background: Autoantibodies have been identified as major predisposing factors for dilated cardiomyopathy (DCM). Patients with DCM show elevated serum levels of vascular endothelial growth factor (VEGF) whose source is unknown. Besides its well-investigated effects on angiogenesis, evidence is present that VEGF signaling is additionally involved in fibroblast proliferation and cardiomyocyte hypertrophy, hence in cardiac remodeling. Whether autoimmune effects in DCM impact cardiac VEGF signaling needs to be elucidated. Methods: Five DCM patients were treated by the immunoadsorption (IA) therapy on five consecutive days. The eluents from the IA columns were collected and prepared for cell culture. Cardiomyocytes from neonatal rats (NRCM) were incubated with increasing DCM-immunoglobulin-G (IgG) concentrations for 48 h. Polyclonal IgG (Venimmun N), which was used to restore IgG plasma levels in DCM patients after the IA therapy was additionally used for control cell culture purposes. Results: Elevated serum levels of VEGF decreased significantly after IA (Serum VEGF (ng/ml); DCM pre-IA: 45 ± 9.1 vs. DCM post–IA: 29 ± 6.7; P < 0.05). In cell culture, pretreatment of NRCM by DCM-IgG induced VEGF expression in a time and dose dependent manner. Biologically active VEGF that was secreted by NRCM significantly increased BNP mRNA levels in control cardiomyocytes and induced cell-proliferation of cultured cardiac fibroblast (Fibroblast proliferation; NRCM medium/HC-IgG: 1 ± 0.0 vs. NRCM medium/DCM-IgG 100 ng/ml: 5.6 ± 0.9; P < 0.05). Conclusion: The present study extends the knowledge about the possible link between autoimmune signaling in DCM and VEGF induction. Whether this observation plays a considerable role in cardiac remodeling during DCM development needs to be further elucidated. - Highlights: • Mechanisms of remodeling in dilated cardiomyopathy (DCM) are not fully understood. • Autoantibodies have been identified as major predisposing factors

  19. Interaction between Hsp60 and Bax in normal human myocardium and in myocardium affected by dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Tykhonkova I. O.

    2009-04-01

    Full Text Available The main functional compartments of molecular chaperone Hsp60 are mitochondria and cytoplasm. Up to 30 % of Hsp60 are located in cytoplasm of cardiomyocytes. The interaction between molecular chaperone Hsp60 and proapoptotic Bax protein in the cytoplasmic fraction from normal human heart tissue has been revealed by co-immunoprecipitation in contrast to myocardium affected by dilated cardiomyopathy, where this interaction has not been observed

  20. Anaesthetic management of laparoscopic surgery for rectal cancer in patients of dilated cardiomyopathy with poor ejection fraction: a case report

    Science.gov (United States)

    Wu, Yao-Hua; Hu, Liang; Xia, Jin; Hao, Quan-Shui; Feng, Li; Xiang, Hong-Bing

    2015-01-01

    A patient with dilated cardiomyopathy with poor ejection fraction posted for laparoscopic surgery for rectal cancer which was successfully performed under general anesthesia with endotracheal intubation and mechanical ventilation was reported. Our observations strongly indicate that detailed preoperative assessment, watchful intraoperative monitoring, and skillful optimization of fluid status and hemodynamic play important role in the high risk patient under general anesthesia with endotracheal intubation and mechanical ventilation. PMID:26309623

  1. Anomalous origin of the left coronary artery from the pulmonary artery presenting as dilated cardiomyopathy: a case report

    OpenAIRE

    Gribaa, Rym; Slim, Mehdi; Ben Salem, Helmi; Neffati, Elyes; Boughzela, Essia

    2014-01-01

    Introduction Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly and one of the causes of myocardial ischemia. The usual clinical course is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. Case presentation We report the case of a 6-month-old Tunisian girl who presented with dilated cardiomyopathy. Echocardiography suspected anomalous origin of the left coronary artery. The definitive dia...

  2. Connexin 43 remodeling induced by LMNA gene mutation Glu82Lys in familial dilated cardiomyopathy with atrial ventricular block

    Institute of Scientific and Technical Information of China (English)

    SUN Li-ping; WANG Lin; WANG Hui; ZHANG Yin-hui; PU Jie-lin

    2010-01-01

    Background Mutations in the lamin A/C gene (LMNA) may cause familial dilated cardiomyopathy (dilated cardiomyopathy) characterized by early onset atrio-ventricular block (A-V block) before the manifestation of dilated cardiomyopathy and high risk of sudden death due to ventricular arrhythmia, which is very similar to the phenotype of gap junction related heart disease. This study aimed to determine the expression and localization of connexins in neonatal myocytes transfected with wild-type (WT) or mutant LMNA to elucidate how these mutations cause heart diseases. Methods We studied the connexin 43 (Cx43) and connexin 40 (Cx40) expression in cultured neonatal myocytes transfected with wild-type (WT) or mutant LMNA (Glu82Lys (E82K) and Arg644Cys (R644C) using confocal imaging and Western blotting analysis.Results Cx43 protein expression was reduced by 40% in cells transfected with LMNA E82K than that in cells transfected with WT LMNA cDNA. Confocal imaging showed that the Cx43 located inside the cells by LMNA E82K. By contrast, LMNA E82K mutation had no effect on expression and localization of Cx40. LMNA R644C transfection did not show any significant effects on gap junctions at all.Conclusions Our findings suggest that LMNA E82K significantly reduced the Cx43 expression and altered its localization which may be one of the pathological mechanisms underlying LMNA-related heart disease.

  3. A longitudinal study on BIO14.6 hamsters with dilated cardiomyopathy: micro-echocardiographic evaluation

    Directory of Open Access Journals (Sweden)

    Belfiore Maria

    2011-12-01

    Full Text Available Abstract Background In recent years, several new technologies for small-animal imaging have been developed. In particular, the use of ultrasound in animal imaging has focused on the investigation of accessible biological structures such as the heart, of which it provides a morphological and functional assessment. The purpose of this study was to investigate the role of micro-ultrasonography (μ-US in a longitudinal study on BIO14.6 cardiomyopathic hamsters treated with gene therapy. Methods Thirty hamsters were divided into three groups (n = 10: Group I, untreated BIO 14.6 hamsters; Group II, BIO 14.6 hamsters treated with gene therapy; Group III, untreated wild type (WT hamsters. All hamsters underwent serial μ-US sessions and were sacrificed at predetermined time points. Results μ-US revealed: in Group I, progressive dilation of the left ventricle with a change in heart morphology from an elliptical to a more spherical shape, altered configuration of the mitral valve and subvalvular apparatus, and severe reduction in ejection fraction; in Group II, mild decrease in contractile function and ejection fraction; in Group III, normal cardiac chamber morphology and function. There was a negative correlation between the percentage of fibrosis observed at histology and the ejection fraction obtained on μ-echocardiography (Spearman r: -0.839; p Conclusions Although histological examination remains indispensable for a conclusive diagnosis, high-frequency μ-echocardiography, thanks to the high spatial and contrast resolution, can be considered sufficient for monitoring therapeutic efficacy and/or the progression of dilated cardiomyopathy, providing an alternative tool for repeatable and noninvasive evaluation.

  4. Serum lipidomics meets cardiac magnetic resonance imaging: profiling of subjects at risk of dilated cardiomyopathy.

    Science.gov (United States)

    Sysi-Aho, Marko; Koikkalainen, Juha; Seppänen-Laakso, Tuulikki; Kaartinen, Maija; Kuusisto, Johanna; Peuhkurinen, Keijo; Kärkkäinen, Satu; Antila, Margareta; Lauerma, Kirsi; Reissell, Eeva; Jurkko, Raija; Lötjönen, Jyrki; Heliö, Tiina; Orešič, Matej

    2011-01-20

    Dilated cardiomyopathy (DCM), characterized by left ventricular dilatation and systolic dysfunction, constitutes a significant cause for heart failure, sudden cardiac death or need for heart transplantation. Lamin A/C gene (LMNA) on chromosome 1p12 is the most significant disease gene causing DCM and has been reported to cause 7-9% of DCM leading to cardiac transplantation. We have previously performed cardiac magnetic resonance imaging (MRI) to LMNA carriers to describe the early phenotype. Clinically, early recognition of subjects at risk of developing DCM would be important but is often difficult. Thus we have earlier used the MRI findings of these LMNA carriers for creating a model by which LMNA carriers could be identified from the controls at an asymptomatic stage. Some LMNA mutations may cause lipodystrophy. To characterize possible effects of LMNA mutations on lipid profile, we set out to apply global serum lipidomics using Ultra Performance Liquid Chromatography coupled to mass spectrometry in the same LMNA carriers, DCM patients without LMNA mutation and controls. All DCM patients, with or without LMNA mutation, differed from controls in regard to distinct serum lipidomic profile dominated by diminished odd-chain triglycerides and lipid ratios related to desaturation. Furthermore, we introduce a novel approach to identify associations between the molecular lipids from serum and the MR images from the LMNA carriers. The association analysis using dependency network and regression approaches also helped us to obtain novel insights into how the affected lipids might relate to cardiac shape and volume changes. Our study provides a framework for linking serum derived molecular markers not only with clinical endpoints, but also with the more subtle intermediate phenotypes, as derived from medical imaging, of potential pathophysiological relevance.

  5. Serum lipidomics meets cardiac magnetic resonance imaging: profiling of subjects at risk of dilated cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Marko Sysi-Aho

    Full Text Available Dilated cardiomyopathy (DCM, characterized by left ventricular dilatation and systolic dysfunction, constitutes a significant cause for heart failure, sudden cardiac death or need for heart transplantation. Lamin A/C gene (LMNA on chromosome 1p12 is the most significant disease gene causing DCM and has been reported to cause 7-9% of DCM leading to cardiac transplantation. We have previously performed cardiac magnetic resonance imaging (MRI to LMNA carriers to describe the early phenotype. Clinically, early recognition of subjects at risk of developing DCM would be important but is often difficult. Thus we have earlier used the MRI findings of these LMNA carriers for creating a model by which LMNA carriers could be identified from the controls at an asymptomatic stage. Some LMNA mutations may cause lipodystrophy. To characterize possible effects of LMNA mutations on lipid profile, we set out to apply global serum lipidomics using Ultra Performance Liquid Chromatography coupled to mass spectrometry in the same LMNA carriers, DCM patients without LMNA mutation and controls. All DCM patients, with or without LMNA mutation, differed from controls in regard to distinct serum lipidomic profile dominated by diminished odd-chain triglycerides and lipid ratios related to desaturation. Furthermore, we introduce a novel approach to identify associations between the molecular lipids from serum and the MR images from the LMNA carriers. The association analysis using dependency network and regression approaches also helped us to obtain novel insights into how the affected lipids might relate to cardiac shape and volume changes. Our study provides a framework for linking serum derived molecular markers not only with clinical endpoints, but also with the more subtle intermediate phenotypes, as derived from medical imaging, of potential pathophysiological relevance.

  6. Serum Uric Acid Correlation with Echocardiographic Indices in Children with Dilated Cardiomyopathy

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    Shahrokh Rajaei

    2009-12-01

    Full Text Available Background: Dilated cardiomyopathy (DCMP is a myocardial disease characterized by dilated left ventricle or both ventricles and reduced contractility of the myocardium. In patients suffering from DCMP, the serum level of uric acid may increase. This research was designed to evaluate the effect of the serum level of uric acid on systolic and diastolic functions in patients with DCMP.Methods: This case-control study was performed on 30 patients with DCMP aged between 1 month and 12 years who were consistent with a control group in terms of age and gender. Patients suffering from congenital and acquired cardiac, renal, metabolic, endocrine, musculoskeletal, neurologic, vascular, and hematologic diseases were excluded. After physical examination, chest X-ray, and electrocardiography, systolic and diastolic parameters were measured via echocardiography, and fasting serum uric acid level was measured. The data were analyzed using the t-test and Pearson correlation coefficient.Results: The average age of the patients in the case and control groups was 7.28 and 7.13 years, respectively. There were 15 boys, and the rest were girls. The serum uric acid level in the case and control groups was 6.22 and 3.31 mg/dl, respectively; the difference was statistically significant (P value <0.01. There was a significant correlation between serum uric acid level and left ventricular isovolumic contraction, interventricular septal diameter, left ventricular septal diameter in diastole, and fractional shortening (P value <0.05.Conclusion: In children with DCMP, the serum level of uric acid increases significantly and this increase is significantly correlated with some of left heart echocardiographic parameters. This test is of predictive value for disease progression.

  7. Usefulness of running wheel for detection of congestive heart failure in dilated cardiomyopathy mouse model.

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    Masami Sugihara

    Full Text Available BACKGROUND: Inherited dilated cardiomyopathy (DCM is a progressive disease that often results in death from congestive heart failure (CHF or sudden cardiac death (SCD. Mouse models with human DCM mutation are useful to investigate the developmental mechanisms of CHF and SCD, but knowledge of the severity of CHF in live mice is necessary. We aimed to diagnose CHF in live DCM model mice by measuring voluntary exercise using a running wheel and to determine causes of death in these mice. METHODOLOGY/PRINCIPAL FINDINGS: A knock-in mouse with a mutation in cardiac troponin T (ΔK210 (DCM mouse, which results in frequent death with a t(1/2 of 70 to 90 days, was used as a DCM model. Until 2 months of age, average wheel-running activity was similar between wild-type and DCM mice (approximately 7 km/day. At approximately 3 months, some DCM mice demonstrated low running activity (LO: 5 km/day. In the LO group, the lung weight/body weight ratio was much higher than that in the other groups, and the lungs were infiltrated with hemosiderin-loaded alveolar macrophages. Furthermore, echocardiography showed more severe ventricular dilation and a lower ejection fraction, whereas Electrocardiography (ECG revealed QRS widening. There were two patterns in the time courses of running activity before death in DCM mice: deaths with maintained activity and deaths with decreased activity. CONCLUSIONS/SIGNIFICANCE: Our results indicate that DCM mice with low running activity developed severe CHF and that running wheels are useful for detection of CHF in mouse models. We found that approximately half of ΔK210 DCM mice die suddenly before onset of CHF, whereas others develop CHF, deteriorate within 10 to 20 days, and die.

  8. Abnormal Glucose Tolerance Is Associated with a Reduced Myocardial Metabolic Flexibility in Patients with Dilated Cardiomyopathy.

    Science.gov (United States)

    Tricò, Domenico; Baldi, Simona; Frascerra, Silvia; Venturi, Elena; Marraccini, Paolo; Neglia, Danilo; Natali, Andrea

    2016-01-01

    Dilated cardiomyopathy (DCM) is characterized by a metabolic shift from fat to carbohydrates and failure to increase myocardial glucose uptake in response to workload increments. We verified whether this pattern is influenced by an abnormal glucose tolerance (AGT). In 10 patients with DCM, 5 with normal glucose tolerance (DCM-NGT) and 5 with AGT (DCM-AGT), and 5 non-DCM subjects with AGT (N-AGT), we measured coronary blood flow and arteriovenous differences of oxygen and metabolites during Rest, Pacing (at 130 b/min), and Recovery. Myocardial lactate exchange and oleate oxidation were also measured. At Rest, DCM patients showed a reduced nonesterified fatty acids (NEFA) myocardial uptake, while glucose utilization increased only in DCM-AGT. In response to Pacing, glucose uptake promptly rose in N-AGT (from 72 ± 21 to 234 ± 73 nmol/min/g, p equivalents, p metabolism and the reduced myocardial metabolic flexibility in response to an increased workload. This trial is registered with ClinicalTrial.gov NCT02440217.

  9. Afterload reduction: a comparison of captopril and nifedipine in dilated cardiomyopathy.

    Science.gov (United States)

    Agostoni, P G; De Cesare, N; Doria, E; Polese, A; Tamborini, G; Guazzi, M D

    1986-01-01

    Nifedipine and captopril are potent vasodilators and may be expected to help left ventricular failure by reducing afterload. Nifedipine (20 mg three times a day) and captopril (50 mg three times a day) were added to an optimal regimen of digitalis and diuretics in a double blind crossover trial in 18 cases of dilated cardiomyopathy. New York Heart Association functional class rating symptoms and exercise tolerance times improved on captopril but not on nifedipine. The reduction in pulmonary capillary wedge pressure and the increase of cardiac output on captopril indicated that the augmented functional capacity may have resulted in part from an improved performance of the left ventricle. Although there were comparable decreases in systemic vascular resistance and presumably in impedence to ejection by the left ventricle on both drugs, the dimensions of the ventricular cavity were found to be reduced by captopril and augmented by nifedipine, and only captopril reduced the afterload (wall stress). In addition, the force-length relation (between left ventricular end systolic stress and end systolic diameter) was shifted to the left of baseline by captopril and to the right by nifedipine, suggesting that muscle contractility was reduced by nifedipine and not by captopril. These results suggest that nifedipine and captopril have different effects on afterload and contractility and these may account for the different effects of these drugs on the performance of the heart and clinical responses. PMID:3516187

  10. Candidate gene expression analysis of toxin-induced dilated cardiomyopathy in the turkey (Meleagris gallopavo).

    Science.gov (United States)

    Lin, K-C; Gyenai, K; Pyle, R L; Geng, T; Xu, J; Smith, E J

    2006-12-01

    Dilated cardiomyopathy (DCM), a heart disease, affects many vertebrates including humans and poultry. The disease can be either idiopathic (IDCM) or toxin-induced (TIDCM). Although genetic and other studies of IDCM are extensive, the specific etiology of TIDCM is still unknown. In this study, we compared mRNA levels of cardiac troponin T (cTnT) and phospholamban (PLN) in turkeys affected and unaffected by TIDCM. Cardiac TnT and PLN were chosen because their altered expression has been observed in IDCM-affected birds. A total of 72 birds, 44 affected and 28 unaffected with TIDCM, were used. Differences in the mRNA levels of cTnT and PLN between affected and unaffected turkeys were significant only for cTnT. The sequence of the turkey PLN showed significant similarity at the nucleotide level to the reference chicken sequence and to those of other species. In addition to implicating cTnT in TIDCM, the present work describes a partial turkey PLN coding sequence that could be useful for future studies.

  11. Prognostic significance of radionuclide-assessed right ventricular function in dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ohno, Akira; Nishimura, Tsunehiko; Uehara, Toshiisa; Shimonagata, Tsuyoshi; Kumita, Shinichiro; Ogawa, Youji; Nagata, Seiki; Miyatake, Kunio (National Cardiovascular Center, Suita, Osaka (Japan))

    1991-09-01

    To assess the prognostic significance of right ventricular function in dilated cardiomyopathy (DCM), we studied consecutive 57 DCM patients. There were 41 men and 16 women, whose mean age was 48 years (range 3-68 years). The mean left ventricular ejection fraction (LVEF) in all patients was 29{+-}11%, and the mean interval from the onset of symptom of cardiac failure (CHF history) was 4 years (range 0-33 years). With follow-up of 3.8 years, five patients had died until the first year, and 14 had died until the third year. By using multivariate regression analysis, there were no prognostic significance in clinical parameters such as age, CHF history, sex, atrial fibrillation, except for NYHA class, and medication at the third year. In survival curves according to Kaplan-Meier method, right ventricular ejection fraction (RVEF) and mean pulmonary artery (PA) had predictive value (p<0.05), while LVEF did not. The patients with RVEF<45% had poor survival rate compared to those with RVEF{>=}45%. The patients with RVEF<45% showed lower LVEF and left ventricular end-systolic volume index. RVEF may offer prognostic predictive value through the effect of not only mean PA but also left ventricular parameter. In conclusion, radionuclide assessment of right ventricular function should be valuable for the prognostic evaluation of DCM patients. (author).

  12. Functional Class in Children with Idiopathic Dilated Cardiomyopathy. A pilot Study

    Science.gov (United States)

    Tavares, Aline Cristina; Bocchi, Edimar Alcides; Guimarães, Guilherme Veiga

    2016-01-01

    Background Idiopathic dilated cardiomyopathy (IDCM), most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF) and low functional capacity (FC). FC is never self reported by children. Objective The aims of this study were (i) To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv) To evaluate whether there was correlation between VO2 max and Weber's classification. Method Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF) were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification. Results Chi-square test showed association between a CFm and CFp (1, n = 31) = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31) = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31) = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19) = 11.8; p = 0.003, to CFp (1, n = 19) = 20.4; p = 0.0001and CFc (1, n = 19) = 6.4; p = 0.04). Conclusion Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification. PMID:27168472

  13. Preliminary Survey on the Prevalence Rate of Hypertension in Patients with Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    To investigate the prevalence of hypertension and its primary risk factors in patients with dilated cardiomyopathy (DCM). Methods Three hundred and sixty-two patients with DCM (DCM group)and 401 age-matched residents (control group) were enrolled randomly in the study, the hypertensive prevalence rate were calculated respectively in the two groups and were compared with each other; the patients in the DCM group were divided into two subgroups (hypertension subgroup and non-hypertension subgroup) according to whether the patients have hypertension;the clinical data related to blood pressure was compared between the two subgroups. Results The prevalence of hypertension in DCM group was significantly higher than that in the control group ( 32. 8% vs. 20. 1%, P< 0.01 ) ; There were no significant differences on the age, gender, occupation and left ventricular ejection fraction (LVEF) between the two subgroups, but the mean heart rate and the percentage of patients who had family history of hypertension were significantly higher in the hypertension subgroup than that in the non-hypertension subgroup ( P<0.05 and P<0. 01 ).Conclusions The prevalence of hypertension in patients with DCM was high; The increased activity of sympathetic nervous system and the hypertensive genetic factor may be the main risk factors of hypertension in patients with DCM.

  14. Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

    Science.gov (United States)

    Sano, Makoto; Satoh, Hiroshi; Suwa, Kenichiro; Saotome, Masao; Urushida, Tsuyoshi; Katoh, Hideki; Hayashi, Hideharu; Saitoh, Takeji

    2016-01-01

    Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative, and patients with cardiac sarcoidosis (CS) who have impaired left ventricular (LV) systolic function are sometimes diagnosed with dilated cardiomyopathy (DCM). Late gadolinium enhancement (LE) in magnetic resonance imaging is now a critical finding in diagnosing CS, and the novel Japanese guideline considers myocardial LE to be a major criterion of CS. This article describes the value of LE in patients with CS who have impaired LV systolic function, particularly the diagnostic and clinical significance of LE distribution in comparison with DCM. LE existed at all LV segments and myocardial layers in patients with CS, whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM. Transmural (nodular), circumferential, and subepicardial and subendocardial LE distribution were highly specific in patients with CS, whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM. Since sarcoidosis patients with LE have higher incidences of heart failure symptoms, ventricular tachyarrhythmia and sudden cardiac death, the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS. PMID:27721933

  15. EFFECT OF AROTINOLOL ON LEFT VENTRICULAR FUNCTION IN PATIENTS WITH IDIOPATHIC DILATED CARDIOMYOPATHY

    Institute of Scientific and Technical Information of China (English)

    Chao-mei Fan; Xiu-qing Du; Na-qiang Lu; Hong Yang; Yi-shi Li; Li Xu; Ke-fei DOU; Jing-lin Zhao; Xian-qi Yuan; Yan-fen Zhao; Rong-fang Shi

    2007-01-01

    To evaluate the efficacy and safety of long-term treatment with arotinolol in patients with idiopathic dilated cardiomyopathy (IDCM).Methods Sixty-three patients with IDCM were evaluated at baseline and after 12-month therapy with arotinolol.The conventional therapy for congestive heart failure was continued throughout the study with arotinolol as the only β-blocker. Left ventricular function was assessed with the New York Heart Association functional class and two-dimensional echocardiography.Results After 12-month arotinolol treatment, there was a significant improvement in left ventricular systolic function. Left ventricular end-systolic dimension significantly decreased from 59. 52 ± 8. 83 mm to 50. 89 ± 8.17 mm (P <0.001). Left ventricular ejection fraction significantly increased from 27.39% ±7.94% to 41.13% ±9.45% (P <0.001). Left ventricular mass index decreased from 150. 47 ± 42. 42 g/m2 to 141.58 ± 34.36 g/m2 ( P<0.01). No adverse events leading to premature discontinuation of study drug occurred.Conclusion In this preliminary study, 12-month arotinolol treatment has a favorable effect on left ventricular function in patients with IDCM, and it is safe and well tolerated.

  16. Effect of arotinolol on right ventricular function in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective Dilated cardiomyopathy (DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study, we evaluated the effect of arotinolol on right ventricular function in patients with DCM. Methods Right ventricular ejection fraction (RVEF) and right ventricular diameter (RVD) were measured by two-dimensional echocardiography (2-DE) in 33 DCM patients; RVEF measured by first-pass radionuclide angiography (FPRA) was compared with that by 2-DE. Results The treatment with arotinolol for one year resulted in a reduction in the right ventricular diameter (baseline, 23.0 ± 8.3 mm vs after one-year treatment, 20.7 ± 5.4 mm; P=0.004 ) and an associated increase in ejection fraction (baseline, 36.9 ± 10.3% vs after one-year treatment, 45.8 ± 9.6%; P < 0.001 ); there is a high correlation between the 2-DE method and radionuclide ventriculographic method. The correlation coefficient is 0.933 (P<0.001). Conclusion Arotinolol therapy could not only improve left ventricular function, but also improve right ventricular function in DCM patients.

  17. Involvement of mast cells in the development of fibrosis in rats with postmyocarditis dilated cardiomyopathy.

    Science.gov (United States)

    Palaniyandi Selvaraj, Suresh; Watanabe, Kenichi; Ma, Meilei; Tachikawa, Hitoshi; Kodama, Makoto; Aizawa, Yoshifusa

    2005-11-01

    Dilated cardiomyopathy (DCM) is a major cause of morbidity and mortality. Occurrence of myocardial fibrosis is an important event in the ventricular remodeling process, which takes place during DCM. Mast cells are well known inflammatory cells implicated in various biological phenomena. The involvement of mast cells in the development of myocardial fibrosis of DCM in rats after autoimmune myocarditis remains unknown. Nine-week-old male Lewis rats were immunized with cardiac myosin and divided into vehicle treated (group V) and disodium cromoglycate (DSCG), a mast cell stabilizer (24 mg/kg i.p.) treated (group DSCG) groups. The animals were sacrificed after 60 d of immunization. The myocardium was excised and preserved for histopathology and protein analysis. Myocardial levels of transforming growth factor (TGF) beta1 and collagen-III were quantified. Staining of mast cells was performed by toluidine blue. A significant correlation was obtained between myocardial fibrosis and cardiac mast cell density. DSCG reduced myocardial fibrosis besides preventing infiltration and degranulation of mast cells. Our findings confirm the active participation of mast cells in the progression of myocardial fibrosis in rats with postmyocarditis DCM. PMID:16272703

  18. Association of HLA-DQ with Idiopathic Dilated Cardiomyopathy in a Northern Chinese Han Population

    Institute of Scientific and Technical Information of China (English)

    WeiLiu; WeiminLi; NinglingSun

    2004-01-01

    Autoimmune mechanisms are likely involved in the pathogenesis of idiopathic dilated cardiomyopathy (IDC) and components of MHC may serve as markers for the propensity to develop immune-mediated myocardial damage. This study was conducted to investigate the possible association between HLA-DQA1, -DQB1 alleles and IDC in Han population from northern China by using PCR-based sequence-specific primer (PCR-SSP) technique for HLA genotyping. Among 68 unrelated IDC patients, 4 probands of IDC pedigrees and 100 healthy controls, we found that the alleles of HLA-DQAI*0501 and HLA-DQBI*0303 conferred susceptibility to IDC while HLA-DQAI*0201 and HLA-DQBI*0502, *0504 alleles were in negative association with IDC. The serine at position 57 (SERs7) in the exon of HLA-DQBI*0502 and *0504 was confirmed in our experiment as a marker for resistance to IDC. The results suggest that HLA-DQ polymorphism may be involved in the pathogenesis of IDC. Cellular & Molecular Immunology.

  19. Association of HLA-DQ with Idiopathic Dilated Cardiomyopathy in a Northern Chinese Han Population

    Institute of Scientific and Technical Information of China (English)

    Wei Liu; Weimin Li; Ningling Sun

    2004-01-01

    Autoimmune mechanisms are likely involved in the pathogenesis of idiopathic dilated cardiomyopathy (IDC) and components of MHC may serve as markers for the propensity to develop immune-mediated myocardial damage. This study was conducted to investigate the possible association between HLA-DQA1, -DQB1 alleles and IDC in Han population from northern China by using PCR-based sequence-specific primer (PCR-SSP) technique for HLA genotyping. Among 68 unrelated IDC patients, 4 probands of IDC pedigrees and 100 healthy controls, we found that the alleles of HLA-DQA1*0501 and HLA-DQB1*0303 conferred susceptibility to IDC while HLA-DQA1*0201 and HLA-DQB1*0502, *0504 alleles were in negative association with IDC. The serine at position 57 (SER57) in the exon of HLA-DQB1*0502 and *0504 was confirmed in our experiment as a marker for resistance to IDC. The results suggest that HLA-DQ polymorphism may be involved in the pathogenesis of IDC.

  20. Genetic analysis of dilated cardiomyopathy--HLA and immunoglobulin genes may confer susceptibility.

    Science.gov (United States)

    Nishi, H; Kimura, A; Fukuta, S; Kusukawa, R; Kawamura, K; Nimura, Y; Nagano, M; Yasuda, H; Kawai, C; Sugimoto, T

    1992-10-01

    To identify genetic factors in the immune system which control the susceptibility to dilated cardiomyopathy (DCM), HLA class II DNA typing was performed in 61 Japanese patients, using PCR/SSO probe analyses. The frequencies of HLA-DQB1*0503 (15% vs 5%; RR = 3.06, chi 2 = 7.19) and DQB1*0604 (21% vs 10%; RR = 2.41, chi 2 = 6.20) were significantly increased and that of HLA-DQB1*0502 (RR = 1.74) was slightly increased in the DCM patients. The frequency of DQB1*0303 (16% vs 31%; RR = 0.44, chi 2 = 5.16) was significantly decreased in the patients. The increased HLA-DQB1 alleles have a histidine residue in common at the 30th codon for the HLA-DQ beta chain. Among the genetic markers studied by Southern blot analyses, IGLV (immunoglobulin lambda light chain, pV3.3) showed a strong association with DCM, i.e. A2/A2 genotype was found in 37.7% of patients whereas it was observed in only 18.9% of the control subjects (RR = 2.6, chi 2 = 7.77). The frequency of this genotype was higher in patients under age 45 years at the time of diagnosis (45.5%, RR = 3.6, chi 2 = 10.02). These results suggest that HLA and immunoglobulin genes are closely linked to susceptibility to DCM.

  1. Risk factor of sudden death in dilated cardiomyopathy patients: A retrospective follow-up study

    Institute of Scientific and Technical Information of China (English)

    LIU Ping; MA Ai-qun; LIU Yu; ZHANG Yan-hui

    2005-01-01

    Objective: To discuss the related risk factors of sudden death in dilated cardiomyopathy(DCM)patients. Methods: A retrospective survey of DCM patients was conducted, ail patients were chosen at random from Xi'an city and 8 adjacent counties. One hundred and fifty patients were reinvestigated after 3. 1 ± 1.5 years. Binary multivariate logistic regression analyses and one way analysis of variance(ANOVA) were used to identify risk factors of the sudden death in DCM patients. Results: Risk factors of sudden death in 150 DCM patients were frequently ventricular premature beats (OR=11. 617), paroxysmalventricular tachycardia (OR=6.305), hypertension (OR= 5.689), EF (OR=0.977). The serum sodium concentration (P= 0. 023) and left ventricular diastolic dimension (LVDD)(P= 0. 039) were significant difference between the sudden death group and the survival group in one way ANOVA, LVDD was not a risk factor in multivariate analysis controlling for possible confounding. Conclusion: The present study identified some risk factors of sudden death in DCM patients, including frequently ventricular premature beats, paroxysmal ventricular tachycardia, hypertension and low EF value.

  2. Traditional Chinese Medicine Tongxinluo Improves Cardiac Function of Rats with Dilated Cardiomyopathy

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    Fang-Fang Shen

    2014-01-01

    Full Text Available The study aimed at testing the hypothesis that tongxinluo capsule might exert its cardioprotective effect by preventing ventricular remodeling and improving coronary microvascular function in a rat model of doxorubicin-induced dilated cardiomyopathy (DCM. Rats that survived DCM induction were randomly divided into three groups to be given 1.5 g·kg−1·day−1 (TXL-H, n=9 or 0.15 g·kg−1·day−1 (TXL-L, n=10 of tongxinluo, or normal saline at the same volume (DCM-C, n=10 intragastrically. Age matched normal rats treated with normal saline were used as normal controls (NOR-C, n=9. After four weeks of treatment, the DCM-C, TXL-H, and TXL-L groups exhibited significant cardiac dysfunction, left ventricular remodeling, and coronary microvascular dysfunction, compared with the NOR-C rats. However, myocardial functional parameters were significantly improved and microvascular density (MVD increased in the TXL-H group compared with the DCM-C group (all P<0.01. Left ventricular remodeling was prevented. There were close linear relationships between CVF and LVEF (r=-0.683, P<0.05, MVD and LVEF (r=0.895, P<0.05, and MVD and CVF (r=-0.798, P<0.05. It was indicated that high-dose tongxinluo effectively improved cardiac function in rat model of DCM.

  3. A Study on Changes of the Plasma Endothelin Level in Patients with Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    黄凯; 朱树雄; 尹瑞兴

    2003-01-01

    Objectives To evaluate the changes of the plasma endothelin level in patients with dilated cardiomyopathy, and to investigate the relationship between the plasma endothelin level and the severity of heart failure, heart size, left ventricular function and with or not with pulmonary arterial hypertension; the changes of the plasma endothelin level before and after treatment. Methods The plasma endothelin level of 30 patients with DCM, 30 healthy control subjects, and their LVEF, PAP, heart size, plasma endothelin level before and after treatment were determined.Results The plasma endothelin level in DCM group was significantly higher than that in control group (135.93 ± 70. 65pg/mL) vs(43.65 ± 12.07pg/mL),P<0.05; there was a correlation between ET level and heart size ( r = 0. 4580, P = 0. 0109); there was a significant negative correlation between LVEF of DCM and ET level(r = -0. 6922,P =0. 0021 ) ;and it was a significant positive correlation between ET level and pulmonary arterial hypertension (r = 0. 8974, P <0. 0005). After treatment, the ET level decreased significantly than before in DCM group (129.15 ±43.93pg/mL) vs (63.12 ± 22.20pg/mL), P <0.05. Conclusions Plasma ET level may be a new parameter of evaluating severity of heart failure, efficacy of therapy and prognosis.

  4. Cardiac magnetic resonance imaging in dilated cardiomyopathy in adults - towards identification of myocardial inflammation

    Energy Technology Data Exchange (ETDEWEB)

    Voigt, Antje; Beling, Mark; Stangl, Karl [Charite-Universitaetsmedizin Berlin, Department of Cardiology, Campus Mitte, Berlin (Germany); Elgeti, Thomas; Durmus, Tahir; Idiz, Merve Ece; Schilling, Rene; Taupitz, Matthias; Wagner, Moritz [Charite-Universitaetsmedizin Berlin, Department of Radiology, Campus Mitte, Berlin (Germany); Butler, Craig [University of Alberta, Mazankowski Alberta Heart Institute, Edmonton (Canada); Klingel, Karin; Kandolf, Reinhard [University Hospital, Department of Molecular Pathology, Tuebingen (Germany); Kivelitz, Dietmar [Asklepios Klinik St. Georg, Department of Radiology, Hamburg (Germany)

    2011-05-15

    To assess active myocardial inflammation by cardiovascular magnetic resonance (CMR) and endomyocardial biopsy (EMB) amongst adult patients with dilated cardiomyopathy (DCM). We evaluated 23 adults with chronic DCM, who had successfully undergone both CMR and EMB within 3.5 {+-} 2.6 days. EMB was considered the gold standard. CMR assessment of myocardial inflammation used the following parameters as recommended by the recently published ''Lake Louise Criteria'': global myocardial oedema, global relative enhancement (RE), and late gadolinium enhancement (LGE). According to ''Lake Louise Criteria'', myocardial inflammation was diagnosed if two or more of the three above-mentioned parameters were positive. Myocardial inflammation was confirmed by immunohistology in 12 patients (52.2%). Sensitivity, specificity, and diagnostic accuracy of CMR to detect immunohistologically confirmed myocardial inflammation were 75.0%, 72.7%, and 73.9%, respectively. Sensitivity, specificity, and diagnostic accuracy of the individual CMR parameters to detect myocardial inflammation were as follows: global myocardial oedema, 91.7%, 81.8%, and 87.0%, respectively; global RE, 58.3%, 63.6%, and 60.9%, respectively; LGE, 58.3%, 45.4%, and 52.2%, respectively. Global myocardial oedema was identified as a promising CMR parameter for assessment of myocardial inflammation in patients with DCM. In these patients, global myocardial oedema yielded superior diagnostic performance compared to ''Lake Louise Criteria''. (orig.)

  5. QT Dispersion Level and Its Clinical Significance.in Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To evaluate the clinical significance of QT dis persion (QTd, QTcd) in dilated cardiomyopathy (DCM). Methods QTd and QTcd were measured on simultaneously recording 12-lead electrocardiograms ( ECGs) in 60 DCM patients and cormpared with 60 healthy subjects. Results The values of QTd and QTcd in DCM were significantly higher than those in control group ( P < 0. 01 ). With subgroup analysis, QTd and QTcd in patients uith cardiac sudden death (CSD) were longer than those in survivors and those died of progressive heart failure ( P < 0. 05), patients with ventricular tachycardia (VT) or with severe heart failure than those without (compared uith patients with ventricular premature beats [V PB], P<0.05, compared with patients without ventricular arrhythmia [VA], P<0. 01) or with mild heart failure (P<0. 01). The values of QTd and QTcd in patients with VPB were greater than those in patients without VA( P< 0. 05). There were significant differences in the rates of VT, CSD and heart failure between the groups of QTd> 110 ms and QTd≤110 ms(P<0. 01 or P<0.05), in contrast to ejection fraction (EF) and fractional shortening (FS)( P>0.05). Conclusion The values of QTd and QTcd increased in DCM patients were susceptive index for monitoring maligant VA in DCM, also important prognostic markers of CSD. QTd was correlated with NY HA functional class but not with EF and FS.

  6. Viscoelastic behavior of human lamin A proteins in the context of dilated cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Avinanda Banerjee

    Full Text Available Lamins are intermediate filament proteins of type V constituting a nuclear lamina or filamentous meshwork which lines the nucleoplasmic side of the inner nuclear membrane. This protein mesh provides a supporting scaffold for the nuclear envelope and tethers interphase chromosome to the nuclear periphery. Mutations of mainly A-type lamins are found to be causative for at least 11 human diseases collectively termed as laminopathies majority of which are characterised by aberrant nuclei with altered structural rigidity, deformability and poor mechanotransduction behaviour. But the investigation of viscoelastic behavior of lamin A continues to elude the field. In order to address this problem, we hereby present the very first report on viscoelastic properties of wild type human lamin A and some of its mutants linked with Dilated cardiomyopathy (DCM using quantitative rheological measurements. We observed a dramatic strain-softening effect on lamin A network as an outcome of the strain amplitude sweep measurements which could arise from the large compliance of the quasi-cross-links in the network or that of the lamin A rods. In addition, the drastic stiffening of the differential elastic moduli on superposition of rotational and oscillatory shear stress reflect the increase in the stiffness of the laterally associated lamin A rods. These findings present a preliminary insight into distinct biomechanical properties of wild type lamin A protein and its mutants which in turn revealed interesting differences.

  7. Abnormal Glucose Tolerance Is Associated with a Reduced Myocardial Metabolic Flexibility in Patients with Dilated Cardiomyopathy

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    Domenico Tricò

    2016-01-01

    Full Text Available Dilated cardiomyopathy (DCM is characterized by a metabolic shift from fat to carbohydrates and failure to increase myocardial glucose uptake in response to workload increments. We verified whether this pattern is influenced by an abnormal glucose tolerance (AGT. In 10 patients with DCM, 5 with normal glucose tolerance (DCM-NGT and 5 with AGT (DCM-AGT, and 5 non-DCM subjects with AGT (N-AGT, we measured coronary blood flow and arteriovenous differences of oxygen and metabolites during Rest, Pacing (at 130 b/min, and Recovery. Myocardial lactate exchange and oleate oxidation were also measured. At Rest, DCM patients showed a reduced nonesterified fatty acids (NEFA myocardial uptake, while glucose utilization increased only in DCM-AGT. In response to Pacing, glucose uptake promptly rose in N-AGT (from 72 ± 21 to 234 ± 73 nmol/min/g, p<0.05, did not change in DCM-AGT, and slowly increased in DCM-NGT. DCM-AGT sustained the extra workload by increasing NEFA oxidation (from 1.3 ± 0.2 to 2.9 ± 0.1 μmol/min/gO2 equivalents, p<0.05, while DCM-NGT showed a delayed increase in glucose uptake. Substrate oxidation rates paralleled the metabolites data. The presence of AGT in patients with DCM exacerbates both the shift from fat to carbohydrates in resting myocardial metabolism and the reduced myocardial metabolic flexibility in response to an increased workload. This trial is registered with ClinicalTrial.gov NCT02440217.

  8. Prognostic value of sympathetic innervation and cardiac asynchrony in dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Manrique, Alain; Hitzel, Anne; Vera, Pierre [Rouen University Hospital - Henri Becquerel Center, Nuclear Medicine, Rouen (France); Bernard, Mathieu; Bauer, Fabrice [Rouen University Hospital, Cardiology, Rouen (France); Menard, Jean-Francois [Rouen University Hospital, Biostatistics, Rouen (France); Sabatier, Remi [Caen University Hospital, Cardiology, Caen (France); Jacobson, Arnold [GE Healthcare, Princeton, NJ (United States); Agostini, Denis [Caen University Hospital, Nuclear Medicine, Caen (France)

    2008-11-15

    The purpose of the study is to examine prognostic values of cardiac I-123 metaiodobenzylguanidine (MIBG) uptake and cardiac dyssynchrony in patients with dilated cardiomyopathy (DCM). Ninety-four patients with non-ischemic DCM underwent I-123 MIBG imaging for assessing cardiac sympathetic innervation and equilibrium radionuclide angiography. Mean phase angles and SD of the phase histogram were computed for both right ventricular (RV) and left ventricular (LV). Phase measures of interventricular (RV-LV) and intraventricular (SD-RV and SD-LV) asynchrony were computed. Most patients were receiving beta-blockers (89%) and angiotensin-converting enzyme inhibitors (88%). One patient (1%) was lost to follow-up, six had cardiac death (6.4%), eight had heart transplantation (8.6%), and seven had unplanned hospitalization for heart failure (7.5%; mean follow-up: 37 {+-} 16 months). Patients with poor clinical outcome were older, had higher The New York Heart Association functional class, impaired right ventricular ejection fraction and left ventricular ejection fraction, and impaired cardiac I-123 MIBG uptake. On multivariate analysis, I-123 MIBG heart-to-mediastinum (H/M) uptake ratio <1.6 was the only predictor of both primary (cardiac death or heart transplantation, RR = 7.02, p < 0.01) and secondary (cardiac death, heart transplantation, or recurrent heart failure, RR = 8.10, p = 0.0008) end points. In patients receiving modern medical therapy involving beta-blockers, I-123 MIBG uptake, but not intra-LV asynchrony, was predictive of clinical outcome. The impact of beta-blockers on the prognostic value of ventricular asynchrony remains to be clarified. (orig.)

  9. Computer-based assessment of left ventricular wall stiffness in patients with ischemic dilated cardiomyopathy

    Science.gov (United States)

    Su, Y.; Teo, S. K.; Tan, R. S.; Lim, C. W.; Zhong, L.

    2013-02-01

    Ischemic dilated cardiomyopathy (IDCM) is a degenerative disease of the myocardial tissue accompanied by left ventricular (LV) structural changes such as interstitial fibrosis. This can induce increased passive stiffness of the LV wall. However, quantification of LV passive wall stiffness in vivo is extremely difficult, particularly in ventricles with complex geometry. Therefore, we sought to (i) develop a computer-based assessment of LV passive wall stiffness from cardiac magnetic resonance (CMR) imaging in terms of a nominal stiffness index (E*); and (ii) investigate whether E* can offer an insight into cardiac mechanics in IDCM. CMR scans were performed in 5 normal subjects and 5 patients with IDCM. For each data sample, an in-house software was used to generate a 1-to-1 corresponding mesh pair of the LV from the ED and ES phases. The E* values are then computed as a function of local ventricular wall strain. We found that E* in the IDCM group (40.66 - 215.12) was at least one order of magnitude larger than the normal control group (1.00 - 6.14). In addition, the IDCM group revealed much higher inhomogeneity of E* values manifested by a greater spread of E* values throughout the LV. In conclusion, there is a substantial elevated ventricular stiffness index in IDCM. This would suggest that E* could be used as discriminator for early detection of disease state. The computational performance per data sample took approximately 25 seconds, which demonstrates its clinical potential as a real-time cardiac assessment tool.

  10. Thiamin, selenium, and copper levels in patients with idiopathic dilated cardiomyopathy taking diuretics

    Directory of Open Access Journals (Sweden)

    Sérgio da Cunha

    2002-11-01

    Full Text Available OBJECTIVE: To analyze the association of thiamin, selenium, and copper serum levels with cardiac function in patients with idiopathic dilated cardiomyopathy using diuretics, and also to compare them with levels in control patients with no evidence of disease. METHODS: The study comprised 30 patients with heart disease and 30 healthy control individuals. Thiamin was analyzed by measuring the activity of erythrocytic transketolase and the effect of thiamin pyrophosphate. Selenium and copper serum levels were measured by hydride generation and flame atomic absorption spectrophotometry, respectively. RESULTS: Thiamin deficiency was observed in 10% of the control individuals and in 33% of the patients with heart disease (p=0.02. The mean selenium and copper serum levels in control individuals and patients with heart disease were, respectively, 73.2±9.9 µg/L (56.5 to 94.5 µg/L and 72.3±14.3 µg/L (35.5 to 94 µg/L (p=0.77; 1.1±0.4mg/L (0.6 to 1.8mg/L and 1.2± 0.4mg/L (0.6 to 2.2mg/L (p=0.27. No association between the levels of these nutrients and cardiac function was observed. CONCLUSION: Thiamin deficiency was significantly more frequent in patients with heart disease. No significant difference was observed between the mean selenium and copper serum levels in control individuals and in patients with heart disease. The results suggest possible benefits with thiamin replacement in patients taking diuretics.

  11. AN ASSESSMENT OF ECHOCARDIOGRAPHY AS A DIAGNOSTIC TOOL FOR DILATED CARDIOMYOPATHY IN TURKEY (MELEAGRIS GALLOPAVO

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    Kwaku Gyenai

    2012-01-01

    Full Text Available Our understanding of the etiology of Dilated Cardiomyopathy (DCM, which affects about 5% of turkeys, is limited. This limitation may be due to the lack of an easy-to-use diagnostic tool with well-defined parameters and does not involve necropsy. This lack of a widely tested non-necropsy method makes it difficult for a large-scale study of the genetic factors that underlie DCM. Here, we Evaluated Echocardiography (ECHO for its ease and reliability for identifying DCM-affected turkeys from hatch to four weeks-of-age. The parameters evaluated included Left Ventricular Internal-Diastolic (LVIDd, Internal-Systolic Dimension (LVISd, Interventricular Septum End-Diastolic (IVSEd, Interventricular Septum End-Systolic (IVSEs, Left Ventricular Wall End-Systolic (LVWEs and Left Ventricular Wall End-Diastolic (LVWEd. To induce DCM, feed containing 700 ppm of Furazolidone (Fz was fed to turkey poults from one to 28 days-of-age. The LVIDd and LVISd were the most consistent indicators of DCM. Both parameters revealed differences between control and treatment poults of between 25 and 326% at the 4 ages at which ECHO measurements were taken. The average difference in LVIDd between control and poults fed Fz-containing diets ranged from 25% in one week-old to 80% in 4-week-old poults. At similar ages, average differences between control and poults fed Fz-containing diets in LVISd were 74 and 326% respectively. Necropsy of poults that survived to the end of the 4-week Fz-treatment confirmed these ECHO measurements in treatment and normal poults. Our data suggest that using LVIDd and LVISd as parameters make ECHO a reliable tool for identifying DCM in turkeys. "

  12. Neopterin and Beta-2 Microglobulin Relations to Immunity and Inflammatory Status in Nonischemic Dilated Cardiomyopathy Patients

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    Celina Wojciechowska

    2014-01-01

    Full Text Available Background. The aim of the study was to assess the relationships among serum neopterin (NPT, β2-microglobulin (β2-M levels, clinical status, and endomyocardial biopsy results of dilated cardiomyopathy patients (DCM. Methods. Serum NPT and β-2 M were determined in 172 nonischaemic DCM patients who underwent right ventricular endomyocardial biopsy and 30 healthy subjects (ELISA test. The cryostat biopsy specimens were assessed using histology, immunohistology, and immunochemistry methods (HLA ABC, HLA DR expression, CD3 + lymphocytes, and macrophages counts. Results. The strong increase of HLA ABC or HLA DR expression was detected in 27.2% patients—group A—being low in 72.8% patients—group B. Neopterin level was increased in patients in group A compared to healthy controls 8.11 (4.50–12.57 versus 4.99 (2.66–8.28 nmol/L (P<0.05. β-2 microglobulin level was higher in DCM groups A (2.60 (1.71–3.58 and B (2.52 (1.51–3.72 than in the control group 1.75 (1.28–1.96 mg/L, P<0.001. Neopterin correlated positively with the number of macrophages in biopsy specimens (P<0.05 acute phase proteins: C-reactive proteins (P<0.05; fibrinogen (P<0.01; and NYHA functional class (P<0.05 and negatively with left ventricular ejection fraction (P<0.05. Conclusions. Neopterin but not β-2 microglobulin concentration reflected immune response in biopsy specimens. Neopterin correlated with acute phase proteins and stage of heart failure and may indicate a general immune and inflammatory activation in heart failure.

  13. Left Atrial Volume Determinants in Patients with Non-Ischemic Dilated Cardiomyopathy

    Science.gov (United States)

    Mancuso, Frederico José Neves; Moisés, Valdir Ambrósio; Almeida, Dirceu Rodrigues; Poyares, Dalva; Storti, Luciana Julio; Oliveira, Wércules Antonio; Brito, Flavio Souza; de Paola, Angelo Amato Vincenzo; Carvalho, Antonio Carlos Camargo; Campos, Orlando

    2015-01-01

    Background Left atrial volume (LAV) is a predictor of prognosis in patients with heart failure. Objective We aimed to evaluate the determinants of LAV in patients with dilated cardiomyopathy (DCM). Methods Ninety patients with DCM and left ventricular (LV) ejection fraction ≤ 0.50 were included. LAV was measured with real-time three-dimensional echocardiography (eco3D). The variables evaluated were heart rate, systolic blood pressure, LV end-diastolic volume and end-systolic volume and ejection fraction (eco3D), mitral inflow E wave, tissue Doppler e´ wave, E/e´ ratio, intraventricular dyssynchrony, 3D dyssynchrony index and mitral regurgitation vena contracta. Pearson´s coefficient was used to identify the correlation of the LAV with the assessed variables. A multiple linear regression model was developed that included LAV as the dependent variable and the variables correlated with it as the predictive variables. Results Mean age was 52 ± 11 years-old, LV ejection fraction: 31.5 ± 8.0% (16-50%) and LAV: 39.2±15.7 ml/m2. The variables that correlated with the LAV were LV end-diastolic volume (r = 0.38; p < 0.01), LV end-systolic volume (r = 0.43; p < 0.001), LV ejection fraction (r = -0.36; p < 0.01), E wave (r = 0.50; p < 0.01), E/e´ ratio (r = 0.51; p < 0.01) and mitral regurgitation (r = 0.53; p < 0.01). A multivariate analysis identified the E/e´ ratio (p = 0.02) and mitral regurgitation (p = 0.02) as the only independent variables associated with LAV increase. Conclusion The LAV is independently determined by LV filling pressures (E/e´ ratio) and mitral regurgitation in DCM. PMID:25993483

  14. Clinical significance and pathogenic role of anti-cardiac myosin autoantibody in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Objective In order to explore the possible roles played by the autoimmune mechanism in the progression of myocarditis into dilated cardiomyopathy (DCM) using an animal model, we investigated whether autoimmune myocarditis might develop into DCM. Methods Experimental Balb/C mice (n=20) were immunized with cardiac myosin with Freund's complete adjuvant at days 0, 7 and 30. The control Balb/C mice (n=10) were immunized with Freund's complete adjuvant in the same mannere. Serum and myocardium samples were collected after the first immunization at days 15, 21 and 120. The anti-myosin antibody was examined by enzyme-linked immunosorbent assay and immunoblotting.Results Pathological findings demonstrated that there was myocardial necrosis or inflammatory infiltration during acute stages and fibrosis mainly in the late phase of experimental group, but the myocardial lesions were not found in the control group. Autoimmunity could induce myocarditis and DCM in the absence of viral infection. High titer anti-myosin IgG antibodies were found in the experimental group, but not in the control group. Furthermore, the anti-myosin heavy chain (200 KD) antibody was positive in 21 of 48 patients with DCM and viral myocarditis, but only 4 of 20 patients with coronary heart disease, including 1 case and 3 cases that reacted with heavy and light chains (27.5 KD), respectively. The antibodies were not detected in healthy donors.Conclusion Cardiac myosin might be an autoantigen that provokes autoimmunity and leads to the transformation of myocarditis into DCM. Detection of anti-myosin heavy chain antibody might contribute to diagnosis for DCM and viral myocarditis.

  15. Antifailure Therapy Including Spironolactone Improves Left Ventricular Energy Supply‐Demand Relations in Nonischemic Dilated Cardiomyopathy

    Science.gov (United States)

    Bell, Susan P.; Adkisson, Douglas W.; Lawson, Mark A.; Wang, Li; Ooi, Henry; Sawyer, Douglas B.; Kronenberg, Marvin W.

    2014-01-01

    Background Left ventricular (LV) energy supply‐demand imbalance is postulated to cause “energy starvation” and contribute to heart failure (HF) in nonischemic dilated cardiomyopathy (NIDCM). Using cardiac magnetic resonance (CMR) and [11C] acetate positron emission tomography (PET), we evaluated LV perfusion and oxidative metabolism in NIDCM and the effects of spironolactone on LV supply‐demand relations. Methods and Results Twelve patients with NIDCM underwent CMR and PET at baseline and after ≥6 months of spironolactone therapy added to a standard HF regimen. The myocardial perfusion reserve index (MPRI) was calculated after gadolinium injection during adenosine, as compared to rest. The monoexponential clearance rate of [11C] acetate (kmono) was used to calculate the work metabolic index (WMI), an index of LV mechanical efficiency, and kmono/RPP (rate‐pressure product), an index of energy supply/demand. At baseline, the subendocardium was hypoperfused versus the subepicardium (median MPRI, 1.63 vs. 1.80; P<0.001), but improved to 1.80 (P<0.001) after spironolactone. The WMI increased (P=0.001), as did kmono/RPP (P=0.003). These improvements were associated with reverse remodeling, increased LV ejection fraction, and decreases in LV mass and systolic wall stress (all P<0.002). Conclusions NIDCM is associated with subendocardial hypoperfusion and impaired myocardial oxidative metabolism, consistent with energy starvation. Antifailure therapy improves parameters of energy starvation and is associated with augmented LV performance. Clinical Trial Registration URL: http://www.clinicaltrials.gov/ Unique identifier: ID NCT00574119. PMID:25164945

  16. Long-term prognostic value of restitution slope in patients with ischemic and dilated cardiomyopathies.

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    Marc Dorenkamp

    Full Text Available BACKGROUND: An action potential duration (APD restitution curve with a steep slope ≥1 has been associated with increased susceptibility for malignant ventricular arrhythmias. We aimed to evaluate the "restitution hypothesis" and tested ventricular APD restitution slope as well as effective refractory period (ERP/APD ratio for long-term prognostic value in patients with ischemic (ICM or dilated cardiomyopathy (DCM. METHODOLOGY/PRINCIPAL FINDINGS: Monophasic action potentials were recorded in patients with ICM (n = 32 and DCM (n = 42 undergoing routine programmed ventricular stimulation (PVS. Left ventricular ejection fraction was 32±7% and 28±9%, respectively. APD and ERP were measured at baseline stimulation (S(1 and upon introduction of one to three extrastimuli (S(2-S(4. ERP/APD ratios and the APD restitution curve were calculated and the maximum restitution slope was determined. After a mean follow-up of 6.1±3.0 years, the combined end-point of mortality and and/or implantable cardioverter-defibrillator shock was not predicted by restitution slope or ERP/APD ratios. Comparing S(2 vs. S(3 vs. S(4 extrastimuli for restitution slope (1.5±0.6 vs. 1.4±0.4 vs. 1.3±0.5; p = NS, additional extrastimuli did not lead to a steepening restitution slope. ERP/APD ratio decreased with additional extrastimuli (0.98±0.09 [S(1] vs. 0.97±0.10 [S(2] vs. 0.93±0.11 [S(3]; p = 0.03 S(1 vs. S(3. Positive PVS was strongly predictive of outcome (p = 0.006. CONCLUSIONS/SIGNIFICANCE: Neither ventricular APD restitution slope nor ERP/APD ratios predict outcome in patients with ICM or DCM.

  17. Changes of Left Ventricular Geometry Shape and Left Ventricular Regional Function in Patients With Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Liang-yu WANG; Ming-xing XIE; Qing-bo LI; Ping CHEN; Zhi-xiong CAI; Zhi-dan ZHU

    2009-01-01

    Objectives To assess the left ventricle regional systolic and diastolic function, left ventricle geometry and left venti-tie sphericity indexes in patients with dilated cardiomyopathy (DCM) by quantitative tissue velocity imaging (QTVI). Methods Thirty normal subjects and 52 DCM patients underwent QTVI and colour Doppler flow imaging study in or-der to measure the left ventricular regional function along left ventricle apical long-axis view and the left ventricle geom-etry. Peak tissue velocities of left venticle regional muscular tissue during systole (Vs), systolic acceleration (a), ear-ly diastole(Ve) and left atrium contraction(Va) along left venticle apical long axis view were measured. The indexes of left ventdcular regional systolic and diastolic function were mearsured at the same time. The left ventricle geometry shape was reflected from the systolic and diastolic sphericity index (Sis and Sid), the left ventricular ejection fraction (LVEF) and D wave/A wave (PVd/Pva) of pulmonary veins flowing spectrum reflected the global left ventricular systolic and diastolic function. The Vs, Ve, Va, a, PVd/Pva ratio, LVEF, Sis, Sid and their correlations between normal subjects and patients with DCM were compared and analyzed. Results Vs, Ve, Va, a, PVd/Pva, Sis and Sid in patients with DCM were lower than those in normal persons. There were significant relations between Sis and a (r=0.6142, P<0.05), Ve/Va and Sid (r=0.6271, P<0.05). Conclusions QTVI offer a newer method which has a higher sensitivity and accuracy in evaluating the left venticle regional systolic and diastolic function in DCM patients. There was significant relation between regional cardiac function and left venticle sphericity.

  18. Increased stromal-cell-derived factor 1 enhances the homing of bone marrow derived mesenchymal stem cells in dilated cardiomyopathy in rats

    Institute of Scientific and Technical Information of China (English)

    ZHOU Yan-li; Michael Fu; ZHANG Hai-feng; LI Xin-li; DI Ruo-min; YAO Wen-ming; LI Dian-fu; FENG Jian-lin; HUANG Jun; CAO Ke-jiang

    2010-01-01

    Background Stem cell transplantation has been shown to have beneficial effects on dilated cardiomyopathy. However,mechanism for stem cell homing to cardiac tissue in dilated cardiomyopathy has not yet been elucidated.Methods Mesenchymal stem cells were obtained from rat bone marrow, expanded in vitro, and labeled with 99mTc.Cardiomyopathy model was induced by doxorubicin in rats. 99mTc labeled cells were infused into the left ventricles in cardiomyopathy and control rats. Sixteen hours after injection, animals were sacrificed and different tissues were harvested to measure specific radioactivity. By use of real-time polymerase chain reaction and immunohistochemistry,Mrna and protein expressions for stromal-cell-derived factor 1 in cardiac tissue were measured.Results Labeling efficiency of mesenchymal stem cells was (70.0±11.2)%. Sixteen hours after mesenchymal stem cell transplantation, the heart-to-muscle radioactivity ratio was increased significantly in cardiomyopathy hearts as compared to control hearts. Both Mrna and rotein expressions of stromal-cell-derived factor 1 were up-regulated in cardiomyopathy hearts as compared with control hearts.Conclusion In dilated cardiomyopathy induced by doxorubicin up-regulated expression of stromal-cell-derived factor 1in heart may induce mesenchymal stem cells home to the heart.

  19. Cardiomyopathy

    Science.gov (United States)

    Cardiomyopathy is the name for diseases of the heart muscle. These diseases enlarge your heart muscle or ... tissue. Some people live long, healthy lives with cardiomyopathy. Some people don't even realize they have ...

  20. An Indian family with an Emery-Dreifuss myopathy and familial dilated cardiomyopathy due to a novel LMNA mutation

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    Khushal B Jadhav

    2012-01-01

    Full Text Available Emery-Dreifuss myopathy can be associated with a cardiomyopathy and cardiac dysrhythmias. The inheritance pattern of Emery-Dreifuss muscular dystrophy (EDMD is X linked, whereas EDMD2 is autosomal dominant. EDMD2 is caused by lamin A/C gene (LMNA mutations that produce alterations in the lamin proteins that are integral to nuclear and cell integrity. A 53-year-old man was brought to us with a right internal carotid artery dissection. Detailed work-up of the patient and family members revealed some unusual features, and genetic sequencing of the LMNA gene was undertaken. A novel mutation was identified in two of the samples sent for analysis. We present the first Indian family of EDMD2 with familial dilated cardiomyopathy and cardiac dysrhythmias in whom LMNA gene sequencing was performed. A novel mutation was identified and additional unusual clinical features were described.

  1. Immunohistological Detection of Parvovirus B19 (B19V) Capsid Proteins and B19V Specific Antibody Profiles in Patients with Acute Myocarditis and Dilated Cardiomyopathy

    OpenAIRE

    Sabi, Titus Mbah

    2010-01-01

    Inflammatory cardiomyopathy (DCMi) is a complex disease which can be induced and sustained by a multiplicity of different etiologies. The clinical course can be acute or chronic. A progression from acute Myokarditis (AMC) to chronic dilated cardiomyopathy (DCM) is possible, although the detailed mechanisms are not well known. Acute Infection with cardiotropic viruses and virus persistence seem to play a vital role. Parvovirus B19 (B19V) has been identified as the most common...

  2. Genetic association study identifies HSPB7 as a risk gene for idiopathic dilated cardiomyopathy.

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    Klaus Stark

    2010-10-01

    Full Text Available Dilated cardiomyopathy (DCM is a structural heart disease with strong genetic background. Monogenic forms of DCM are observed in families with mutations located mostly in genes encoding structural and sarcomeric proteins. However, strong evidence suggests that genetic factors also affect the susceptibility to idiopathic DCM. To identify risk alleles for non-familial forms of DCM, we carried out a case-control association study, genotyping 664 DCM cases and 1,874 population-based healthy controls from Germany using a 50K human cardiovascular disease bead chip covering more than 2,000 genes pre-selected for cardiovascular relevance. After quality control, 30,920 single nucleotide polymorphisms (SNP were tested for association with the disease by logistic regression adjusted for gender, and results were genomic-control corrected. The analysis revealed a significant association between a SNP in HSPB7 gene (rs1739843, minor allele frequency 39% and idiopathic DCM (p = 1.06 × 10⁻⁶, OR  = 0.67 [95% CI 0.57-0.79] for the minor allele T. Three more SNPs showed p < 2.21 × 10⁻⁵. De novo genotyping of these four SNPs was done in three independent case-control studies of idiopathic DCM. Association between SNP rs1739843 and DCM was significant in all replication samples: Germany (n =564, n = 981 controls, p = 2.07 × 10⁻³, OR = 0.79 [95% CI 0.67-0.92], France 1 (n = 433 cases, n = 395 controls, p =3.73 × 10⁻³, OR  = 0.74 [95% CI 0.60-0.91], and France 2 (n = 249 cases, n = 380 controls, p = 2.26 × 10⁻⁴, OR  = 0.63 [95% CI 0.50-0.81]. The combined analysis of all four studies including a total of n = 1,910 cases and n = 3,630 controls showed highly significant evidence for association between rs1739843 and idiopathic DCM (p = 5.28 × 10⁻¹³, OR= 0.72 [95% CI 0.65-0.78]. None of the other three SNPs showed significant results in the replication stage.This finding of the HSPB7 gene from a genetic search for idiopathic DCM using

  3. Relationship between HLA-DQA1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    刘巍; 李为民; 孙宁玲

    2004-01-01

    Background Autoimmune mechanisms are likely to participate in the pathogenesis of subgroup of idiopathic dilated cardiomyopathy (IDC), and components of the major histocompatibility complex may serve as markers for the propensity to develop immune-mediated myocardial damage. Human leukocyte antigen (HLA) class Ⅱ genes, especially highly polymorphic HLA-DQ genes, play an important role in the activation of immune responses, and thus control the predisposition for or protect from IDC. This study was conducted to investigate the HLA-DQA1 allele polymorphisms in IDC patients and to explore the underlying immunological mechanism and the hereditary susceptibility to IDC.Methods The polymerase chain reaction sequence-specific primers (PCR-SSP) technique was used to analyze the polymorphisms in the second exon of DQA1 in three groups: 72 IDC patients diagnosed according to the criteria of World Health Organization (IDC group); 100 end-stage heart failure patients suffering from a disease of known etiology (HF group); and 100 healthy subjects enrolled for the study during a routine health survey (control group). Patients in the IDC group were stratified according to ejection fraction (EF). Those with EF values were higher than 35% were placed into subgroup 1; subgroup 2 included patients with an EF value of 15%-35%; and subgroup 3 consisted of those whose EF values less than 15%. Results The frequency of HLA-DQA1*0501 alleles was significantly higher in the IDC group (0.39) than that in the HF group (0.12) and the control group (0.09) (both P<0.05). Further analysis of the three IDC subgroups showed a higher frequency of DQA1*0501 among patients with lower EF values (both P<0.05, compared with subgroups 1 and 2). The frequency of DQA1*0201 was higher in the control group than that in the IDC group (P<0.05).Conclusions The HLA-DQA1*0501 allele confers susceptibility to IDC, while the DQA1*0201 allele confers protection against it, which indicates that genetic background

  4. Changes in myocardial collagen content before and after left ventricular assist device application in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    LIANG Hong 梁红; Roland Hetzer; Johannes Müller; WENG Yu-guo 翁渝国; Gerd Wallukat; FU Ping 付平; LIN Han-sheng 林汉生; Sabina Bartel; Christoph Knosalla; Reinhard Pregla

    2004-01-01

    Background The purposes of this study were to confirm the changes in myocardial collagen level after left ventricular assist device (LVAD) support in dilated cardiomyopathy (DCM), find the relation between these changes and prognosis, and test a practical method to assess the level of myocardial collagen.Methods Left ventricular samples were collected from DCM patients with different prognosis (transplanted group n=8, weaning group n=10) at the time when the LVADs were implanted and again during cardiac transplantation (n=8). The level of neutral salt soluble collagen (NSC) and acid soluble collagen (ASC) was measured by Sircol collagen assay, and that of total collagen and insoluble collagen (ISC) by quantification of hydroxyproline (Hyp). Serum samples were collected from a portion of these patients (transplanted group, n=6; weaning group n=7) at the time the LVADs were implanted, 1 month after implantation and on explantation. Circulating concentration of carboxy-terminal propeptide of type Ⅰ procollagen (PⅠCP), amino-terminal propeptide of type Ⅰ procollagen (PⅠNP), amino-terminal propeptide of type Ⅲ procollagen (PⅢNP) and type Ⅰ collagen telopeptide (ⅠCTP) were measured by the equilibrium type radioimmunoassay. Results Before LVAD implantation the level of NSC and ISC in the weaning group was higher but ASC in the transplanted group was lower than in the controls (P<0.05). After LVAD support, the level of total collagen was higher, but ASC was also lower in the transplanted group than in the controls (P<0.05). In comparison of the pre- and post-LVAD subgroups of the transplanted and weaning groups, all collagen fraction levels before LVAD implantation were lower in the transplanted group than in the weaning group (P<0.05); but this difference disappeared after LVAD support. Comparison of the pre- and post-LVAD subgroups of the transplanted group showed increased level of NSC and total collagen after LVAD support. The changes of serum peptide

  5. The Relationship between Left Atrial Volume and Ventricular Arrhythmias in the Patients with Dilated Cardiomyopathy

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    Abdullah Kaplan

    2014-03-01

    Full Text Available Background:: The present study aimed to investigate the relationship between Left Atrial Volume (LAV, a marker of diastolic dysfunction, and the frequency of malignant ventricular arrhythmia in the patients with left ventricular dysfunction and a previously implanted Implantable Cardioverter Defibrillator (ICD device. Methods:: This cross-sectional study was conducted on 32 patients with ischemic or idiopathic dilated cardiomyopathy, each having had an ICD device implanted at least 1 year beforehand. The ventricular arrhythmia episodes which were detected and stored by the device were retrieved and evaluated. In addition to routine echocardiographic measurements, all the patients had their LAV and LAV indexes calculated. After all, student’s t-test, Mann-Whitney U test, and Pearson correlation were used to analyze the data. Besides, P value < 0.05 was considered as statistically significant. Results:: This study was conducted on 4 female and 28 male patients with the mean age of 58.41 ± 9.97 years. Among the study patients, 21 had at least one previous myocardial infarction. In addition, 17 patients had experienced sustained VT or VF within the last year. No significant difference was found between the patients with and without malignant ventricular arrhythmias (sustained VT or VF regarding LAV (17 patients with arrhythmia (68 + 23.39 mL vs. 15 patients without arrhythmia (55.13 ± 20.41 mL; P = 0.100. However, the LAV index was significantly higher in the patients with arrhythmia compared to those without arrhythmia (39.27 ± 12.19 mL / m2 vs. 25.18 ± 7.45 mL / m2; P = 0.004. Both LAV (73.33 ± 17.64 mL and 57.52 ± 23.15 mL, respectively; P = 0.040 and LAV index (40.86 ± 8.47 mL / m2 and 28.20 ± 11.77 mL / m2, respectively; P = 0.010 were significantly greater in the patients with ICD shock therapy within the last year compared to the others. However, both groups were similar regarding Left Ventricular Volume (LVV, LVV index, and

  6. Multicenter study on hepatitis C virus infection in patients with dilated cardiomyopathy. North Italy Transplant Program (NITP).

    Science.gov (United States)

    Prati, D; Poli, F; Farma, E; Picone, A; Porta, E; De Mattei, C; Zanella, A; Scalamogna, M; Gamba, A; Gronda, E; Faggian, G; Livi, U; Puricelli, C; Viganò, M; Sirchia, G

    1999-06-01

    Preliminary epidemiological and histological studies from Japan suggested that hepatitis C virus (HCV) infection has a role in the development of dilated cardiomyopathy (DCM). This multicenter study was conducted to verify this hypothesis on a large cohort of Italian patients with end-stage heart failure. Antibodies to HCV were determined in the 752 consecutive patients (608 males and 144 females; age, 53 +/- 13 years) who entered the waiting list for cardiac transplantation from 1995 to 1997 at the six cardiac surgery centers participating in the North Italy Transplant program. Three hundred and nine patients (41%) had dilated, 9 (1%) restrictive, and 4 (0.5%) hypertrophic cardiomyopathy; 284 patients (38%) had ischemic, 65 (9%) valvular, and 22 (3%) congenital heart disease; 5 patients (0.5%) had primary pulmonary hypertension; 54 patients (7%) had other or nonspecified heart disease. Overall, 41 of 752 patients (5.4%) resulted anti-HCV-reactive. Serological evidence of HCV infection was found in 12 of 309 patients with DCM (3.9%; 95% CI, 1.7-6.0), and in 29 of 443 without DCM (6.5%; 95% CI, 4.2-8.8), without statistical difference (difference of prevalence rate: 2.6%; 95% CI, -4.9 to 5.8). In conclusion, HCV does not seem to have a primary role in the pathogenesis of DCM. However, since our findings are in disagreement with those obtained in smaller series of patients of other ethnicity, large studies from different countries should be conducted.

  7. Left ventricular assist for pediatric patients with dilated cardiomyopathy using the Medos VAD cannula and a centrifugal pump.

    Science.gov (United States)

    Huang, Shu-Chien; Chi, Nai-Hsin; Chen, Chun-An; Chen, Yih-Sharng; Chou, Nai-Kuan; Ko, Wen-Je; Wang, Shoei-Shen

    2009-11-01

    Ventricular assist devices for small pediatric patients are expensive and commercially unavailable in Taiwan. We used the Medos ventricular assist device cannula (Medos, Aachen, Germany) and a centrifugal pump to support pediatric patients with dilated cardiomyopathy and decompensated heart failure. From January 2007 to December 2008, three pediatric patients with dilated cardiomyopathy were supported using a centrifugal pump as the left ventricular assist device. The Medos arterial cannula was sutured to the ascending aorta, and the Apex cannula was fixed into the left ventricular apex. When the patient was weaned off of cardiopulmonary bypass, the left ventricular assist device pump was started. The pump flow was gradually titrated according to the filling status of the left ventricle. All the left ventricular assist devices were successfully implanted and functioned well. Two patients on extracorporeal membrane oxygenation had severe lung edema before left ventricular assist device implantation. Both patients required extracorporeal membrane oxygenation for the postoperative period until the pulmonary edema was resolved. Among the three patients, two successfully bridged to heart transplantation after support for 6 and 11 days, respectively. The first patient (10 kg) expired due to systemic emboli 30 days after left ventricular assist device support. In summary, these results suggest that the Medos ventricular assist device cannula and a centrifugal pump is an option for temporary left ventricular assist device support in patients with intractable heart failure and as a bridge to heart transplantation.

  8. Regional evidence of modulation of cardiac adiponectin level in dilated cardiomyopathy: pilot study in a porcine animal model

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    Caselli Chiara

    2012-11-01

    Full Text Available Abstract Background The role of systemic and myocardial adiponectin (ADN in dilated cardiomyopathy is still debated. We tested the regulation of both systemic and myocardial ADN and the relationship with AMP-activated protein kinase (AMPK activity in a swine model of non-ischemic dilated cardiomyopathy. Methods and results Cardiac tissue was collected from seven instrumented adult male minipigs by pacing the left ventricular (LV free wall (180 beats/min, 3 weeks, both from pacing (PS and opposite sites (OS, and from five controls. Circulating ADN levels were inversely related to global and regional cardiac function. Myocardial ADN in PS was down-regulated compared to control (p Conclusions Paradoxically, circulating ADN did not show any cardioprotective effect, confirming its role as negative prognostic biomarker of heart failure. Myocardial ADN was reduced in PS compared to control in an AMPK-independent fashion, suggesting the occurrence of novel mechanisms by which reduced cardiac ADN levels may regionally mediate the decline of cardiac function.

  9. Determinants of Atrial Electromechanical Delay in Patients with Functional Mitral Regurgitation and Non-ischemic Dilated Cardiomyopathy

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    Bengi Bakal Ruken

    2014-12-01

    Full Text Available Introduction: Atrial conduction time has important hemodynamic effects on ventricular filling and is accepted as a predictor of atrial fibrillation. In this study we assessed atrial conduction time in patients with non ischemic dilated cardiomyopathy (NIDCMP and functional mitral regurgitation (MR and aimed to determine factors predicting atrial conduction time prolongation. Methods: Sixty five patients with non ischemic dilated cardiomyopathy who have moderate to severe MR and 60 control subjects were included in the study. In addition to conventional echocardiographic measures used to asses left ventricle and MR, atrial electromechanical coupling (time interval from the onset of P wave on surface electrocardiogram [ECG] to the beginning of A wave interval with tissue Doppler echocardiography [PA], intra- and interatrial electromechanical delay (intra and inter AEMD were measured. Results: The correlations between inter AEMD and left atrial (LA size, MR volume, isovolumetric relaxation time (IVRT, deceleration time (DT, systolic pulmonary artery pressure (PAPs, E/A ratio and E/e’ were very poor. Similarly, intra AEMD was not correlated to LA size , MR volume, IVRT, DT, PAPs, E/A ratio and E/e’. However, both inter AEMD and intra AEMD had good correlation with left ventricular mass index, tenting area (TA, tenting distance (TD, coaptation septal distance (CSD, sphericity index (SI. Conclusion: Prolongation of inter and intra AEMDs were found to be well correlated with parameters reflecting left ventricular and mitral annular remodeling.

  10. A novel mutation and first report of dilated cardiomyopathy in ALG6-CDG (CDG-Ic: a case report

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    Zagal Ahmad

    2010-04-01

    Full Text Available Abstract Congenital disorders of glycosylation (CDG are an expanding group of inherited metabolic diseases with multisystem involvement. ALG6-CDG (CDGIc is an endoplasmatic reticulum defect in N-glycan assembly. It is usually milder than PMM2-CDG (CDG-Ia and so is its natural course. It is characterized by psychomotor retardation, seizures, ataxia, and hypotonia. In contrast to PMM2-CDG (CDGIa, there is no cerebellar hypoplasia. Cardiomyopathy has been reported in a few CDG types and in a number of patients with unexplained CDG. We report an 11 year old Saudi boy with severe psychomotor retardation, seizures, strabismus, inverted nipples, dilated cardiomyopathy, and a type 1 pattern of serum transferrin isoelectrofocusing. Phosphomannomutase and phosphomannose isomerase activities were normal in fibroblasts. Full gene sequencing of the ALG6 gene revealed a novel mutation namely c.482A>G (p.Y161C and heterozygosity in the parents. This report highlights the importance to consider CDG in the differential diagnosis of unexplained cardiomyopathy.

  11. Myocardial deformation pattern in left ventricular non-compaction: Comparison with dilated cardiomyopathy

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    Olivier Huttin

    2014-12-01

    Conclusions: Compared to DCM, LVNC presented with relatively preserved apical deformation as compared to basal segments. Lower regional deformation values in compacted segments confirm the concept that LVNC is a phenotypic marker of an underlying diffuse cardiomyopathy involving both C and NC myocardium.

  12. Five-week use of a monopivot centrifugal blood pump as a right ventricular assist device in severe dilated cardiomyopathy.

    Science.gov (United States)

    Inoue, Takamichi; Kitamura, Tadashi; Torii, Shinzo; Hanayama, Naoji; Oka, Norihiko; Itatani, Keiichi; Tomoyasu, Takahiro; Irisawa, Yusuke; Shibata, Miyuki; Hayashi, Hidenori; Ono, Minoru; Miyaji, Kagami

    2014-03-01

    Right heart failure is a critical complication in patients requiring mechanical ventricular support. However, it is often difficult to provide adequate right ventricular support in the acute phase. A 41-year-old woman diagnosed with dilated cardiomyopathy with severe right heart failure underwent implantation of a paracorporeal pulsatile left ventricular assist device (LVAD, Nipro Corporation, Tokyo, Japan) and a MERA monopivot centrifugal pump (Senko Medical Instrument Manufacturing Co., Ltd., Tokyo, Japan) as a right ventricular assist device (RVAD). The patient developed ischemic enteritis 3 weeks after surgery, necessitating fasting and reversal of anticoagulation therapy. A target international normalized ratio of 1.5 was selected, and aspirin administration was discontinued. Following recovery without thromboembolic events, the patient failed the RVAD discontinuation test. Five weeks after surgery, the monopivot centrifugal pump was exchanged for a pulsatile pump. No thrombus was evident on the centrifugal pump. The patient was undergoing cardiac rehabilitation at the time of this writing and awaiting heart transplantation.

  13. Anaesthetic Management of Renal Transplant Surgery in Patients of Dilated Cardiomyopathy with Ejection Fraction Less Than 40%

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    Divya Srivastava

    2014-01-01

    Full Text Available Cardiovascular disease (CVD is an important comorbidity of chronic kidney disease, and reducing cardiovascular events in this population is an important goal for the clinicians who care for chronic kidney disease patients. The high risk for CVD in transplant recipients is in part explained by the high prevalence of conventional CVD risk factors (e.g., diabetes, hypertension, and dyslipidemia in this patient population. Current transplant success allows recipients with previous contraindications to transplant to have access to this procedure with more frequency and safety. Herein we provide a series of eight patients with dilated cardiomyopathy with poor ejection fraction posted for live donor renal transplantation which was successfully performed under regional anesthesia with sedation.

  14. Correlation between changes in diastolic dysfunction and health-related quality of life after cardiac rehabilitation program in dilated cardiomyopathy

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    Sherin H.M. Mehani

    2013-03-01

    Full Text Available Chronic heart failure (CHF is a complex syndrome characterized by progressive decline in left ventricular function, low exercise tolerance and raised mortality and morbidity. Left ventricular diastolic dysfunction plays a major role in CHF and progression of most cardiac diseases. The current recommended goals can theoretically be accomplished via exercise and pharmacological therapy so the aim of the present study was to evaluate the impact of cardiac rehabilitation program on diastolic dysfunction and health related quality of life and to determine the correlation between changes in left ventricular diastolic dysfunction and domains of health-related quality of life (HRQoL. Forty patients with chronic heart failure were diagnosed as having dilated cardiomyopathy (DCM with systolic and diastolic dysfunction. The patients were equally and randomly divided into training and control groups. Only 30 of them completed the study duration. The training group participated in rehabilitation program in the form of circuit-interval aerobic training adjusted according to 55–80% of heart rate reserve for a period of 7 months. Circuit training improved both diastolic and systolic dysfunction in the training group. On the other hand, only a significant correlation was found between improvement in diastolic dysfunction and health related quality of life measured by Kansas City Cardiomyopathy Questionnaire. It was concluded that improvement in diastolic dysfunction as a result of rehabilitation program is one of the important underlying mechanisms responsible for improvement in health-related quality of life in DCM patients.

  15. Exome sequencing identified mutations in CASK and MYBPC3 as the cause of a complex dilated cardiomyopathy phenotype.

    Science.gov (United States)

    Reinstein, Eyal; Tzur, Shay; Bormans, Concetta; Behar, Doron M

    2016-01-01

    Whole-exome sequencing for clinical applications is now an integral part of medical genetics practice. Though most studies are performed in order to establish diagnoses in individuals with rare and clinically unrecognizable disorders, due to the constantly decreasing costs and commercial availability, whole-exome sequencing has gradually become the initial tool to study patients with clinically recognized disorders when more than one gene is responsible for the phenotype or in complex phenotypes, when variants in more than one gene can be the cause for the disease. Here we report a patient presenting with a complex phenotype consisting of severe, adult-onset, dilated cardiomyopathy, hearing loss and developmental delay, in which exome sequencing revealed two genetic variants that are inherited from a healthy mother: a novel missense variant in the CASK gene, mutations in which cause a spectrum of neurocognitive manifestations, and a second variant, in MYBPC3, that is associated with hereditary cardiomyopathy. We conclude that although the potential for co-occurrence of rare diseases is higher when analyzing undefined phenotypes in consanguineous families, it should also be given consideration in the genetic evaluation of complex phenotypes in non-consanguineous families. PMID:27173948

  16. Evaluation of dilated cardiomyopathy by /sup 201/Tl myocardial single photon emission computed tomography. Morphological and quantitative analysis

    Energy Technology Data Exchange (ETDEWEB)

    Futagami, Yasuo; Makino, Katsutoshi; Ichikawa, Takehiko

    1984-08-01

    To estimate dilated cardiomyopathy (DCM)morphologically and quantitatively, /sup 201/Tl myocardial single photon emission computed tomography (SPECT) was performed in 14 DCM and 5 normal cases. Using a rotating dual-gamma camera system, resting SPECT data were collected for 6 minutes. Quantitative analysis of clinical cases was based on phantom studies. Marked spherical left ventricular (LV) dilatation (14/14), localized-diffuse low uptake or defect (12/14), and right ventricular visualization (6/14) were characteristic features in DCM. Differentiation of DCM from ischemic heart disease by SPECT was possible through the feature indicating disproportionately large LV cavity to defect size or degree. Quantitative analysis When DCM was compared with normal control (n-5), following 3 features were impressive: DCM was significantly higher in LV myocardial /sup 201/Tl uptake ratio and LV volume than normal control; DCM was significantly lower in LV myocardial /sup 201/Tl uptake ratio of unit volume (1 ml) than normal control; DCM was significantly lower in mean myocardial count/mean lung count.ratio than normal control.

  17. Cardiac-specific VLCAD deficiency induces dilated cardiomyopathy and cold intolerance

    Science.gov (United States)

    Xiong, Dingding; He, Huamei; James, Jeanne; Tokunaga, Chonan; Powers, Corey; Huang, Yan; Osinska, Hanna; Towbin, Jeffrey A.; Purevjav, Enkhsaikhan; Balschi, James A.; Javadov, Sabzali; McGowan, Francis X.; Strauss, Arnold W.

    2013-01-01

    The very long-chain acyl-CoA dehydrogenase (VLCAD) enzyme catalyzes the first step of mitochondrial β-oxidation. Patients with VLCAD deficiency present with hypoketotic hypoglycemia and cardiomyopathy, which can be exacerbated by fasting and/or cold stress. Global VLCAD knockout mice recapitulate these phenotypes: mice develop cardiomyopathy, and cold exposure leads to rapid hypothermia and death. However, the contribution of different tissues to development of these phenotypes has not been studied. We generated cardiac-specific VLCAD-deficient (cVLCAD−/−) mice by Cre-mediated ablation of the VLCAD in cardiomyocytes. By 6 mo of age, cVLCAD−/− mice demonstrated increased end-diastolic and end-systolic left ventricular dimensions and decreased fractional shortening. Surprisingly, selective VLCAD gene ablation in cardiomyocytes was sufficient to evoke severe cold intolerance in mice who rapidly developed severe hypothermia, bradycardia, and markedly depressed cardiac function in response to fasting and cold exposure (+5°C). We conclude that cardiac-specific VLCAD deficiency is sufficient to induce cold intolerance and cardiomyopathy and is associated with reduced ATP production. These results provide strong evidence that fatty acid oxidation in myocardium is essential for maintaining normal cardiac function under these stress conditions. PMID:24285112

  18. A new surgical approach for treating dilated cardiomyopathy with mitral regurgitation

    Directory of Open Access Journals (Sweden)

    Buffolo Enio

    2000-01-01

    Full Text Available OBJECTIVE: To evaluate the early outcome of mitral valve prostheses implantation and left ventricular remodeling in 23 patients with end-stage cardiomyopathy and secondary mitral regurgitation (NYHA class III and IV. METHODS: Mitral valvular prosthesis implantation with preservation of papillary muscles and chordae tendinae, and plasty of anteriun cuspid for remodeling of the left ventricle. RESULTS: The surgery was performed in 23 patients, preoperative ejection fraction (echocardiography varied from 13% to 44% (median: 30%. In 13 patients associated procedures were performed: myocardial revascularization (9, left ventricle plicature repair (3 and aortic prosthese implantation (1. Early deaths (2 occurred on the 4th PO day (cardiogenic shock and on the 20th PO day (upper gastrointestinal bleeding, and a late death in the second month PO (ventricular arrhythmia. Improvement occurred in NYHA class in 82.6% of the patients (P<0.0001, with a survival rate of 86.9% (mean of 8.9 months of follow-up. CONCLUSION: This technique offers a promising therapeutic alternative for the treatment of patients in refractory heart failure with cardiomyopathy and secondary mitral regurgitation.

  19. A Case of a Senile Systemic Amyloidosis Patient Presenting With Angina Pectoris and Dilated Cardiomyopathy

    OpenAIRE

    Kang, Gu Hyun; Ryu, Dong Ryeol; Song, Pil Sang; Song, Young Bin; Hahn, Joo-Yong; Choi, Seung-Hyuck; Gwon, Hyeon-Cheol

    2011-01-01

    A 77-year-old man visited our hospital complaining of aggravated exertional chest pain. He was diagnosed with syndrome X 7 years ago and underwent medical treatment in a regional hospital. Coronary angiography and echocardiography did not show any significant abnormalities. On the seventh in-hospital day, cardiogenic shock developed and echocardiography showed a dilated left ventricular (LV) cavity and severe LV systolic dysfunction. We thus inserted an intra-aortic balloon pump for hemodynam...

  20. A case of juvenile acromegaly that was initially diagnosed as severe congestive heart failure from acromegaly-induced dilated cardiomyopathy.

    Science.gov (United States)

    Sue, Mariko; Yoshihara, Aya; Okubo, Yoichiro; Ishikawa, Mayumi; Ando, Yasuyo; Hiroi, Naoki; Shibuya, Kazutoshi; Yoshino, Gen

    2010-01-01

    Acromegaly is characterized by chronic hypersecretion of growth hormone (GH) and is associated with increased mortality rate because of the potential complications such as cardiovascular disease, respiratory disease, or malignancy, which are probably caused by the long-term exposure of tissues to excess GH, for at least 10 years, before diagnosis and treatment. A 22-year-old man with a 2-month history of fatigue was admitted to our hospital because of chest discomfort, dyspnea, and pitting edema of the lower limbs experienced over a 1-month period. On admission, his height and body weight were 186 cm and 138.5 kg, respectively, with a BMI of 39.8 kg/m(2). He showed acromegalic features and elevated serum GH and IGF-1 levels, which were 11.5 ng/mL and 960 ng/mL, respectively. There was no GH suppression in the 75-g oral glucose tolerance test. Pituitary magnetic resonance imaging (MRI) revealed microadenoma. Chest X-ray revealed cardiomegaly, and echocardiogram showed dilated left ventricular (LV) cavity and diffuse hypokinesis with extremely decreased ejection fraction (EF). He was diagnosed as having acromegaly with congestive heart failure from diastolic cardiomyopathy. After the successful transsphenoidal resection of the pituitary adenoma, the level of GH was normalized. However, the cardiac dysfunction did not show any improvement even after the administration of β-blockers, angiotensin-converting enzyme inhibitor (ACE-I), or diuretics. The patient was re-hospitalized, and he died of cardiac failure at the age of 25 years. Patients with acromegaly have been reported to have about 30% higher mortality rate, and cardiovascular disease accounts for 60% of the deaths. We report a case of a patient with juvenile acromegaly who was diagnosed with severe cardiac failure at the time of diagnosis and failed to recover cardiac function even after the successful resection of the pituitary adenoma. Immediate diagnosis and treatment are required for better control of

  1. COMPARATIVE EFFICACY OF CHRONIC HEART FAILURE TREATMENT WITH PERINDOPRIL OR CARVEDILOL IN PATIENTS WITH ALCOHOLIC OR IDIOPATHIC DILATED CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Zhirov

    2015-09-01

    Full Text Available Aim. To study the clinical and hemodynamic efficacy of monotherapy with ACE inhibitor perindopril or beta-blocker carvedilol in patients with chronic heart failure (CHF due to dilated cardiomyopathy (DCMP of various etiology.Material and methods. Patients (n=69 with DCMP of different etiology were included into the open randomized study. Idiopathic DCMP (IDCMP was diagnosed in 26 patients and alcoholic cardiomyopathy (ACMP - in 43 patients. Patients of IDCMP and ACMP groups were randomized for treatment with perindopril (groups 1 and 3, respectively or carvedilol (groups 2 and 4, respectively. Follow-up was 6 months. End-diastolic and end-systolic left ventricular volume, stroke volume index, ejection fraction (EF and exercise capacity were determined at baseline and in 2 and 6 months of treatment. Safety of the treatments was also assessed.Results. Group 1: the average CHF class (NYHA decreased by 20.7% (p<0.01, EF increased by 18.2% (p<0.05. Group 2: the average CHF class decreased by 29.6% (p<0.01, EF increased by 18.2% (p<0.05. Group 3: the average CHF class decreased by 14.3% (p<0.01, EF increased by 19.6% (p<0.05. Group 4: the average CHF class decreased by 41.4% (p<0.001, EF increased by 32.8% (p<0.001.Conclusion. Monotherapy with carvedilol in patients with ACMP was more effective than this with perindopril. Long-term monotherapy with perindopril or carvedilol in patients with DCMP was well tolerated and safety.

  2. COMPARATIVE EFFICACY OF CHRONIC HEART FAILURE TREATMENT WITH PERINDOPRIL OR CARVEDILOL IN PATIENTS WITH ALCOHOLIC OR IDIOPATHIC DILATED CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    I. V. Zhirov

    2013-01-01

    Full Text Available Aim. To study the clinical and hemodynamic efficacy of monotherapy with ACE inhibitor perindopril or beta-blocker carvedilol in patients with chronic heart failure (CHF due to dilated cardiomyopathy (DCMP of various etiology.Material and methods. Patients (n=69 with DCMP of different etiology were included into the open randomized study. Idiopathic DCMP (IDCMP was diagnosed in 26 patients and alcoholic cardiomyopathy (ACMP - in 43 patients. Patients of IDCMP and ACMP groups were randomized for treatment with perindopril (groups 1 and 3, respectively or carvedilol (groups 2 and 4, respectively. Follow-up was 6 months. End-diastolic and end-systolic left ventricular volume, stroke volume index, ejection fraction (EF and exercise capacity were determined at baseline and in 2 and 6 months of treatment. Safety of the treatments was also assessed.Results. Group 1: the average CHF class (NYHA decreased by 20.7% (p<0.01, EF increased by 18.2% (p<0.05. Group 2: the average CHF class decreased by 29.6% (p<0.01, EF increased by 18.2% (p<0.05. Group 3: the average CHF class decreased by 14.3% (p<0.01, EF increased by 19.6% (p<0.05. Group 4: the average CHF class decreased by 41.4% (p<0.001, EF increased by 32.8% (p<0.001.Conclusion. Monotherapy with carvedilol in patients with ACMP was more effective than this with perindopril. Long-term monotherapy with perindopril or carvedilol in patients with DCMP was well tolerated and safety.

  3. Quantitative genomic and antigenomic enterovirus RNA detection in explanted heart tissue samples from patients with end-stage idiopathic dilated cardiomyopathy.

    Science.gov (United States)

    Lévêque, Nicolas; Renois, Fanny; Talmud, Déborah; Nguyen, Yohan; Lesaffre, François; Boulagnon, Camille; Bruneval, Patrick; Fornes, Paul; Andréoletti, Laurent

    2012-10-01

    Standardized one-step real-time RT-PCR assay detected enterovirus RNA in cardiac biopsy samples from 4 of 20 patients suffering from idiopathic dilated cardiomyopathy (IDCM). The median viral load was 287 copies per microgram of total extracted nucleic acids, with positive- to negative-strand RNA ratios ranging from 2 to 20. These results demonstrate enterovirus persistence in the heart of IDCM patients, characterized by low viral loads and low positive- to negative-RNA ratios. PMID:22837323

  4. Evaluation of acceleration and deceleration cardiac processes using phase-rectified signal averaging in healthy and idiopathic dilated cardiomyopathy subjects.

    Science.gov (United States)

    Bas, Rosana; Vallverdú, Montserrat; Valencia, Jose F; Voss, Andreas; de Luna, Antonio Bayés; Caminal, Pere

    2015-02-01

    The aim of the present study was to investigate the suitability of the Phase-Rectified Signal Averaging (PRSA) method for improved risk prediction in cardiac patients. Moreover, this technique, which separately evaluates acceleration and deceleration processes of cardiac rhythm, allows the effect of sympathetic and vagal modulations of beat-to-beat intervals to be characterized. Holter recordings of idiopathic dilated cardiomyopathy (IDC) patients were analyzed: high-risk (HR), who suffered sudden cardiac death (SCD) during the follow-up; and low-risk (LR), without any kind of cardiac-related death. Moreover, a control group of healthy subjects was analyzed. PRSA indexes were analyzed, for different time scales T and wavelet scales s, from RR series of 24 h-ECG recordings, awake periods and sleep periods. Also, the behavior of these indexes from simulated data was analyzed and compared with real data results. Outcomes demonstrated the PRSA capacity to significantly discriminate healthy subjects from IDC patients and HR from LR patients on a higher level than traditional temporal and spectral measures. The behavior of PRSA indexes agrees with experimental evidences related to cardiac autonomic modulations. Also, these parameters reflect more regularity of the autonomic nervous system (ANS) in HR patients. PMID:25585858

  5. DNA sequence and haplotype variation in two candidate genes for dilated cardiomyopathy in the turkey Meleagris gallopavo.

    Science.gov (United States)

    Lin, Kuan-chin; Xu, Jun; Kamara, Davida; Geng, Tuoyu; Gyenai, Kwaku; Reed, Kent M; Smith, Edward J

    2007-05-01

    Determining variation in genes is fundamental to understanding their function in the disease state. Cardiac troponin T (cTnT) and phospholamban (PLN) genes have been implicated in dilated cardiomyopathy (DCM) in human and model species. To investigate the role of these 2 candidate genes in DCM in the turkey Meleagris gallopavo, understanding sequence variants and map position distribution is necessary. To this end, a total of 1854 and 1771 bp of cTnT and PLN gene sequences, respectively, were scanned for single nucleotide polymorphisms (SNPs) in a randomly bred population. A total of 15 SNPs was identified in the cTnT and PLN genomic sequences. Nine haplotypes, 5 in cTnT and 4 in PLN, were identified. Observed heterozygosities (0.02-0.39) in the turkey population were low for both genes. Within each gene, 1 SNP corresponding to a restriction enzyme site was identified and used to develop a PCR-restriction fragment length polymorphism (RFLP) genotyping assay. The PLN gene was genetically mapped to turkey chromosome 2, equivalent to Gallus gallus chromosome 3, and cTnT mapped to a turkey microchromosome. Although limited because of the relatively small sample size of 55 birds, the data from this SNP analysis of PLN and cTnT provide a foundation from which to evaluate the function of cTnT and PLN in the turkey. Information about the distribution of the SNPs and haplotypes will facilitate future association and linkage studies.

  6. Evaluation of sympathetic activity by 123I-metaiodobenzylguanidine myocardial scintigraphy in dilated cardiomyopathy patients with sleep breathing disorder

    International Nuclear Information System (INIS)

    Because increased sympathetic nervous activity (SNA) in patients with dilated cardiomyopathy (DCM) associated with sleep breathing disorder (SBD) is known to deteriorate the prognosis of cardiac failure, 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy was used as the investigative tool in the present study. The study group comprised 53 patients (47 men, 6 women; mean age 56±3 years) with chronic stable DCM. Patients were divided into SBD(+) or SBD(-) group according to 24-h pulse oximetry results. SBD(+) was defined when the 3% oxygen desaturation index was more than 15/h during sleep. In total, 32 patients were SBD(-) and 21 were SBD(+). In both groups, pulse oximetry were performed during sleep and awakening pulse rate, and measurement of the blood levels of catecholamines and B-type natriuretic peptide was performed. MIBG myocardial scintigraphy and echocardiography were performed at the same time. No significant difference was found between the 2 groups in catecholamine levels or left ventricular ejection fraction. However, MIBG had a significantly increased washout rate and a significantly decreased delayed heart to mediastinum ratio in the SBD(+) group compared with the SBD(-) group. SNA is increased in DCM patients when associated with SBD. MIBG myocardial scintigraphy may be a sensitive method of detecting increased SNA. (author)

  7. Identification of a Novel TAZ Gene Mutation in a Family With X-Linked Dilated Cardiomyopathy Barth Syndrome

    Directory of Open Access Journals (Sweden)

    Minal Borkar PhD

    2015-02-01

    Full Text Available Mutations in the tafazzin (TAZ gene on chromosome Xq28 are responsible for the Barth syndrome (BTHS phenotype resulting in a loss of function in the protein tafazzin involved in the transacylation of cardiolipin, an essential mitochondrial phospholipid. TAZ gene was investigated in the proband in our study, who died of dilated cardiomyopathy at 8 months of age, and his family by sequencing to identify the genetic cause of BTHS. Molecular analysis revealed a novel mutation in exon 5 (c.520T>G of the TAZ gene. This novel mutation c.520T>G, pW174G, was also found in female carriers (mother and grandmother of proband in the family. Bioinformatic analysis was carried out to examine the effect of mutation in the gene and confirmed the deleterious effect of this single mutation to the protein structure. Protein modeling and 3-dimensional structure of TAZ protein demonstrated the significantly visible changes in mutated protein leading to BTHS phenotype. Prenatal diagnosis in a subsequent pregnancy showed a carrier female, and pregnancy was continued. Child is doing well at 1 year of age.

  8. Antioxidants Improve the Phenotypes of Dilated Cardiomyopathy and Muscle Fatigue in Mitochondrial Superoxide Dismutase-Deficient Mice

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    Takahiko Shimizu

    2013-01-01

    Full Text Available Redox imbalance elevates the reactive oxygen species (ROS level in cells and promotes age-related diseases. Superoxide dismutases (SODs are antioxidative enzymes that catalyze the degradation of ROS. There are three SOD isoforms: SOD1/CuZn-SOD, SOD2/Mn-SOD, and SOD3/EC-SOD. SOD2, which is localized in the mitochondria, is an essential enzyme required for mouse survival, and systemic knockout causes neonatal lethality in mice. To investigate the physiological function of SOD2 in adult mice, we generated a conditional Sod2 knockout mouse using a Cre-loxP system. When Sod2 was specifically deleted in the heart and muscle, all mice exhibited dilated cardiomyopathy (DCM and died by six months of age. On the other hand, when Sod2 was specifically deleted in the skeletal muscle, mice showed severe exercise disturbance without morphological abnormalities. These provide useful model of DCM and muscle fatigue. In this review, we summarize the impact of antioxidants, which were able to regulate mitochondrial superoxide generation and improve the phenotypes of the DCM and the muscle fatigue in mice.

  9. An Uncommon Association of Familial Partial Lipodystrophy, Dilated Cardiomyopathy, and Conduction System Disease

    Directory of Open Access Journals (Sweden)

    Ragesh Panikkath MD

    2016-07-01

    Full Text Available A 46-year-old African American woman presented with severe respiratory distress requiring intubation and was diagnosed with nonischemic cardiomyopathy. She had the typical phenotype of familial partial lipodystrophy 2 (FPLD2. Sequence analysis of LMNA gene showed a heterozygous missense mutation at exon 8 (c.1444C>T causing amino acid change, p.R482W. She later developed severe coronary artery disease requiring multiple percutaneous coronary interventions and coronary artery bypass surgery. She was later diagnosed with diabetes, primary hyperparathyroidism, and euthyroid multinodular goiter. She had sinus nodal and atrioventricular nodal disease and had an implantable cardioverter defibrillator implantation due to persistent left ventricular dysfunction. The device eroded through the skin few months after implantation and needed a re-implant on the contralateral side. She had atrial flutter requiring ablation. This patient with FPLD2 had most of the reported cardiac complications of FPLD2. This case is presented to improve the awareness of the presentation of this disease among cardiologists and internists.

  10. An Uncommon Association of Familial Partial Lipodystrophy, Dilated Cardiomyopathy, and Conduction System Disease

    Science.gov (United States)

    Panikkath, Ragesh; Panikkath, Deepa; Sanchez-Iglesias, S.; Araujo-Vilar, D; Lado-Abeal, Joaquin

    2016-01-01

    A 46-year-old African American woman presented with severe respiratory distress requiring intubation and was diagnosed with nonischemic cardiomyopathy. She had the typical phenotype of familial partial lipodystrophy 2 (FPLD2). Sequence analysis of LMNA gene showed a heterozygous missense mutation at exon 8 (c.1444C>T) causing amino acid change, p.R482W. She later developed severe coronary artery disease requiring multiple percutaneous coronary interventions and coronary artery bypass surgery. She was later diagnosed with diabetes, primary hyperparathyroidism, and euthyroid multinodular goiter. She had sinus nodal and atrioventricular nodal disease and had an implantable cardioverter defibrillator implantation due to persistent left ventricular dysfunction. The device eroded through the skin few months after implantation and needed a re-implant on the contralateral side. She had atrial flutter requiring ablation. This patient with FPLD2 had most of the reported cardiac complications of FPLD2. This case is presented to improve the awareness of the presentation of this disease among cardiologists and internists. PMID:27504462

  11. Comparação do desfecho entre a cardiopatia chagásica e a miocardiopatia dilatada idiopática Comparison of outcome between Chagas cardiomyopathy and idiopathic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Amanda Pires Barbosa

    2011-12-01

    Full Text Available FUNDAMENTO: Pouco se sabe sobre o desfecho dos pacientes com cardiopatia chagásica, em comparação aos pacientes com miocardiopatia dilatada idiopática na era contemporânea. OBJETIVO: Comparar o desfecho dos pacientes chagásicos com insuficiência cardíaca sistólica crônica decorrente da cardiopatia chagásica ao observado em pacientes com MDI na era contemporânea. MÉTODOS: Foi incluído um total de 352 pacientes (246 com cardiomiopatia chagásica e 106 com miocardiopatia dilatada idiopática, seguidos prospectivamente em nossa Instituição, de janeiro de 2000 a janeiro de 2008. Todos os pacientes receberam tratamento clínico contemporâneo padrão. RESULTADOS: Na análise multivariada com o modelo de risco proporcional de Cox, o uso da digoxina (relação de risco = 3,17; intervalo de confiança de 95%, de 1,62 a 6,18; p = 0,001 necessitou de suporte inotrópico (relação de risco = 2,08; intervalo de confiança de 95%, de 1,43 a 3,02; p BACKGROUND: Little is known about the outcome of patients with Chagas cardiomyopathy in comparison to that of patients with Idiopathic Dilated Cardiomyopathy in the contemporary era. OBJECTIVE: To compare the outcome of chagasic patients with chronic systolic heart failure secondary to Chagas cardiomyopathy with that observed in patients with IDC in the contemporary era. METHODS: A total of 352 patients (246 with Chagas cardiomyopathy, 106 with Idiopathic Dilated Cardiomyopathy prospectively followed at our Institution from January, 2000 to January, 2008 were included. All patients received standard contemporary medical therapy. RESULTS: In Cox proportional hazards model multivariate analysis, digoxin use (Hazard Ratio=3.17; 95% Confidence Interval 1.62 to 6.18; p=0.001, need of inotropic support (Hazard Ratio=2.08; 95% Confidence Interval 1.43 to 3.02; p<0.005, left ventricular ejection fraction (Hazard Ratio=0.97; 95% Confidence Interval 0.95 to 0.99; p<0.005, and Chagas cardiomyopathy etiology

  12. Types of Cardiomyopathy

    Science.gov (United States)

    ... ventricles, making it harder for the heart to pump blood. Hypertrophic cardiomyopathy also can cause stiffness of the ... Over time, the heart loses the ability to pump blood effectively. Dilated cardiomyopathy can lead to heart failure , ...

  13. Impact of shocks on mortality in patients with ischemic or dilated cardiomyopathy and defibrillators implanted for primary prevention.

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    Florian Streitner

    Full Text Available BACKGROUND: Emerging interest is seen in the paradox of defibrillator shocks for ventricular tachyarrhythmia and increased mortality risk. Particularly in patients with dilated cardiomyopathy (DCM, the prognostic importance of shocks is unclear. The purpose of this study was to compare the outcome after shocks in patients with ischemic cardiomyopathy (ICM or DCM and defibrillators (ICD implanted for primary prevention. METHODS AND RESULTS: Data of 561 patients were analyzed (mean age 68.6±10.6 years, mean left ventricular ejection fraction 28.6±7.3%. During a median follow-up of 49.3 months, occurrence of device therapies and all-cause mortality were recorded. 74 out of 561 patients (13.2% experienced ≥1 appropriate and 51 out of 561 patients (9.1% ≥1 inappropriate shock. All-cause mortality was 24.2% (136 out of 561 subjects. Appropriate shock was associated with a trend to higher mortality in the overall patient population (HR 1.48, 95% CI 0.96-2.28, log rank p = 0.072. The effect was significant in ICM patients (HR 1.61, 95% CI 1.00-2.59, log rank p = 0.049 but not in DCM patients (HR 1.03, 95% CI 0.36-2.96, log rank p = 0.96. Appropriate shocks occurring before the median follow-up revealed a much stronger impact on mortality (HR for the overall patient population 2.12, 95% CI 1.24-3.63, p = 0.005. The effect was driven by ICM patients (HR 2.48, 95% CI 1.41-4.37, p = 0.001, as appropriate shocks again did not influence survival of DCM patients (HR 0.63, 95% CI 0.083-4.75, p = 0.65. Appropriate shocks occurring after the median follow-up and inappropriate shocks occurring at any time revealed no impact on survival in any of the groups (p = ns. CONCLUSION: Appropriate shocks are associated with reduced survival in patients with ICM but not in patients with DCM and ICDs implanted for primary prevention. Furthermore, the negative effect of appropriate shocks on survival in ICM patients is only evident within the

  14. Preliminary clinical study of left ventricular myocardial strain in patients with non-ischemic dilated cardiomyopathy by three-dimensional speckle tracking imaging

    Directory of Open Access Journals (Sweden)

    Duan Fengxia

    2012-03-01

    Full Text Available Abstract Background Non-ischemic dilated cardiomyopathy (DCM is the most common cardiomyopathy worldwide, with significant mortality. Correct evaluation of the patient's myocardial function has important clinical significance in the diagnosis, therapeutic effect assessment and prognosis in non-ischemic DCM patients. This study evaluated the feasibility of three-dimensional speckle tracking imaging (3D-STE for assessment of the left ventricular myocardial strain in patients with non-ischemic dilated cardiomyopathy (DCM. Methods Apical full-volume images were acquired from 65 patients with non-ischemic DCM (DCM group and 59 age-matched normal controls (NC group, respectively. The following parameters were measured by 3D-STE: the peak systolic radial strain (RS, circumferential strain (CS, longitudinal strain (LS of each segment. Then all the parameters were compared between the two groups. Results The peak systolic strain in different planes had certain regularities in normal groups, radial strain (RS was the largest in the mid region, the smallest in the apical region, while circumferential strain (CS and longitudinal strain (LS increased from the basal to the apical region. In contrast, the regularity could not be applied to the DCM group. RS, CS, LS were significantly decreased in DCM group as compared with NC group (P Conclusions 3D-STE is a reliable tool for evaluation of left ventricular myocardial strain in patients with non-ischemic DCM, with huge advantage in clinical application.

  15. HEART REMODELING AT DIFFERENT STAGES OF CHRONIC HEART FAILURE IN PATIENTS WITH POSTINFARCTION CARDIOSCLEROSIS AND DILATED CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    V. V. Mazur

    2010-01-01

    Full Text Available Aim. To study the features of cardiac remodeling in patients with dilated cardiomyopathy (DCM and postinfarction cardiosclerosis (PICS, that can be used for differential diagnosis of these diseases.Material and methods. Patients with DCM (27 men and 5 women; aged 43.1±2.3 and patients with PICS (62 men; aged 56.4±1.1 and chronic heart failure (CHF were included in the study. The diagnosis of DCM was based on clinical investigation, which also includes coronary angiography. The diagnosis of DCM in 19 patients was proven by the results of postmortem investigation. The diagnosis of PICS was based on documented history of myocardial infarction, ECG and echocardiographic sings. Echocardiography was performed in all patients and 14 healthy volunteers.Results. End-systolic size (ESS of left ventricular (LV in patients with DCM and PICS at I (respectively 7.60±0.17 and 7.94±0.18 cm, IIA (7.66±0.28 and 8.64±0.30 cm and IIB stages of CHF (8.26±0.28 and 8.94±0.15 cm was significantly more than this in healthy volunteers (6.36±0.16, all p<0.01. ESS of right ventricular (RV in DCM patients of the same CHF stages (respectively 7.21±0.22, 7.40±0.27 and 8.23±0.27 cm is also more than this in healthy volunteers (5.95±0.17, all p<0.01. ESS RV in PICS patients at I (5.40±0.11 cm and IIA (5.80±0.26 cm CHF stages did not differ from healthy volunteers, and this index risen to IIB stage (6.62±0.21 cm, but was lower than in DCM patients.The ESS LV/ESS RV ratio at any CHF stage in PICS patients was significantly higher than this in DCM patients (1.48±0.04 and 1.06±0.02, 1.50±0.05 and 1.04±0.02, 1.37±0,06 and 1.00±0.01, respectively.Conclusion. The ESS LV/ESS RV ratio can be used for differential diagnosis of dilatation in DCM and PICS patients.

  16. Comparison of the structure and function of phospholamban and the arginine-14 deficient mutant associated with dilated cardiomyopathy.

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    Eleri Hughes

    Full Text Available Phospholamban (PLB is a pentameric protein that plays an important role in regulating cardiac contractility via a reversible inhibitory association with the sarcoplasmic reticulum Ca2+ATPase (SERCA, the enzyme responsible for maintaining correct calcium homeostasis. Here we study the functional and biophysical characteristics of a PLB mutant associated with human dilated cardiomyopathy (DCM, with a deletion of arginine at position 14 (PLBR14Δ. In agreement with recent findings, we find that PLBR14Δ has a reduced inhibitory effect on SERCA compared to wild type PLB (PLBWT when reconstituted into lipid membranes. The mutation also leads to a large reduction in the protein kinase A-catalysed phosphorylation of Ser-16 in the cytoplasmic domain of PLBR14Δ. Measurements on SERCA co-reconstituted with an equimolar mixture of PLBWT and PLBR14Δ (representing the lethal heterozygous state associated with DCM indicates that the loss-of-function mutation has a dominant effect on PLBWT functionality and phosphorylation capacity, suggesting that mixed PLBWT/PLBR14Δ pentamers are formed that have characteristics typical of the mutant protein. Structural and biophysical analysis of PLBR14Δ indicates that the mutation perturbs slightly the helical structure of the PLB cytoplasmic domain and reduces its affinity for the phospholipid bilayer surface, thereby altering the orientation of the cytoplasmic domain relative to the wild-type protein. These results indicate that the structure and function consequences of the R14 deletion have profound effects on the regulation of SERCA which may contribute to the aetiology of DCM.

  17. EFFECTS OF EPCs OR b-FGF INTRAMYOCARDIAL INFUSION ON CARDIAC FUNCTION AND NEOVASCULARIZATION FOR DILATED CARDIOMYOPATHY RATS

    Institute of Scientific and Technical Information of China (English)

    ZHANG Xin; WEI Meng; YAN Xiao-yu

    2008-01-01

    Objective To compare the different effects of endothelia progenitor cells (EPCs) or basic fibroblast growth factor (b-FGF) intromyocardial infusion on cardiac function and neovascularization for dilated cardiomyopathy(DCM)rats.Methods Fifty adult female rats received inguinal subcutaneous injections of isoproterenol (ISO, 250 mg/kg) for induction of DCM. Four weeks later, the model rats were randomly divided into EPCs group, b-FGF group and control group. The 2×106 EPCs (resolved in 100 μL PBS), 100 μL b-FGF (100 μg/mL) and 100 μL PBS were evenly transplanted into the myocardium of EPCs group, b-FGF group and control group, respectively. Three months later, echocardiographic examination and regional myocardial blood flow (RMBF)measurement were performed. EPCs were traced by fluorescence in situ hybridization (FISH). The protein and mRNA expression of b-FGF in each group was measured by ELISA assay and reverse transcription-polymerase chain reaction (RT-PCR).Results Three months after transplantation, sry positive cells were detected only in EPCs group. The cardiac function as well as RMBF was significantly improved in EPCs group compared with b-FGF group or control group. There was higher capillary density in EPCs group. The protein and mRNA expression of b-FGF was stronger than b-FGF group and control group.Conclusion Transplantation of EPCs can improve cardiac function, induce neovascularization and increase RMBF for DCM rats. The treatment with EPCs has better effect than administration of b-FGF alone.

  18. Changes of myocardial gene expression and protein composition in patients with dilated cardiomyopathy after immunoadsorption with subsequent immunoglobulin substitution.

    Science.gov (United States)

    Ameling, Sabine; Bhardwaj, Gourav; Hammer, Elke; Beug, Daniel; Steil, Leif; Reinke, Yvonne; Weitmann, Kerstin; Grube, Markus; Trimpert, Christiane; Klingel, Karin; Kandolf, Reinhard; Hoffmann, Wolfgang; Nauck, Matthias; Dörr, Marcus; Empen, Klaus; Felix, Stephan B; Völker, Uwe

    2016-09-01

    Immunoadsorption with subsequent immunoglobulin substitution (IA/IgG) represents a therapeutic approach for patients with dilated cardiomyopathy (DCM). Here, we studied which molecular cardiac alterations are initiated after this treatment. Transcription profiling of endomyocardial biopsies with Affymetrix whole genome arrays was performed on 33 paired samples of DCM patients collected before and 6 months after IA/IgG. Therapy-related effects on myocardial protein levels were analysed by label-free proteome profiling for a subset of 23 DCM patients. Data were analysed regarding therapy-associated differences in gene expression and protein levels by comparing responders (defined by improvement of left ventricular ejection fraction ≥20 % relative and ≥5 % absolute) and non-responders. Responders to IA/IgG showed a decrease in serum N-terminal proBNP levels in comparison with baseline which was accompanied by a decreased expression of heart failure markers, such as angiotensin converting enzyme 2 or periostin. However, despite clinical improvement even in responders, IA/IgG did not trigger general inversion of DCM-associated molecular alterations in myocardial tissue. Transcriptome profiling revealed reduced gene expression for connective tissue growth factor, fibronectin, and collagen type I in responders. In contrast, in non-responders after IA/IgG, fibrosis-associated genes and proteins showed elevated levels, whereas values were reduced or maintained in responders. Thus, improvement of LV function after IA/IgG seems to be related to a reduced gene expression of heart failure markers and pro-fibrotic molecules as well as reduced fibrosis progression. PMID:27412778

  19. Oxidative Stress Markers and C-Reactive Protein Are Related to Severity of Heart Failure in Patients with Dilated Cardiomyopathy

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    Celina Wojciechowska

    2014-01-01

    Full Text Available Background. The aim of study was to determine relationships between functional capacity (NYHA class, left ventricle ejection fraction (LVEF, hemodynamic parameters, and biomarkers of redox state and inflammation in patients with dilated cardiomyopathy (DCM. Methods. DCM patients (n=109, aged 45.97±10.82 years, NYHA class IIV, and LVEF 2.94±7.1% were studied. Controls comprised age-matched healthy volunteers (n=28. Echocardiography and right heart catheterization were performed. Serum activities of superoxide dismutase isoenzymes (MnSOD and CuZnSOD, concentrations of uric acid (UA, malondialdehyde (MDA, and C-reactive protein (hs-CRP were measured. Results. MnSOD, UA, hs-CRP, and MDA were significantly higher in DCM patients compared to controls. Except MDA concentration, above parameters were higher in patients in III-IV NYHA class or with lower LVEF. hsCRP correlated with of MnSOD (P<0.05 and CuZnSOD activity (P<0.01. Both isoenzymes positively correlated with mPAP and pulmonary capillary wedge pressure (MnSOD, resp., P<0.01 and P<0.05 and CuZnSOD P<0.05; P<0.05. UA positively correlated with MnSOD (P<0.05, mPAP (P<0.05, and PVRI (P<0.05. The negative correlation between LVEF and UA (P<0.01 was detected. Conclusion. There are relationships among the severity of symptoms of heart failure, echocardiographic hemodynamic parameters, oxidative stress, and inflammatory activation. Increased MnSOD activity indicates the mitochondrial source of ROS in patients with advanced heart failure.

  20. Endothelial Restoration of Receptor Activity-Modifying Protein 2 Is Sufficient to Rescue Lethality, but Survivors Develop Dilated Cardiomyopathy.

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    Kechele, Daniel O; Dunworth, William P; Trincot, Claire E; Wetzel-Strong, Sarah E; Li, Manyu; Ma, Hong; Liu, Jiandong; Caron, Kathleen M

    2016-09-01

    RAMPs (receptor activity-modifying proteins) serve as oligomeric modulators for numerous G-protein-coupled receptors, yet elucidating the physiological relevance of these interactions remains complex. Ramp2 null mice are embryonic lethal, with cardiovascular developmental defects similar to those observed in mice null for canonical adrenomedullin/calcitonin receptor-like receptor signaling. We aimed to genetically rescue the Ramp2(-/-) lethality in order to further delineate the spatiotemporal requirements for RAMP2 function during development and thereby enable the elucidation of an expanded repertoire of RAMP2 functions with family B G-protein-coupled receptors in adult homeostasis. Endothelial-specific expression of Ramp2 under the VE-cadherin promoter resulted in the partial rescue of Ramp2(-/-) mice, demonstrating that endothelial expression of Ramp2 is necessary and sufficient for survival. The surviving Ramp2(-/-) Tg animals lived to adulthood and developed spontaneous hypotension and dilated cardiomyopathy, which was not observed in adult mice lacking calcitonin receptor-like receptor. Yet, the hearts of Ramp2(-/-) Tg animals displayed dysregulation of family B G-protein-coupled receptors, including parathyroid hormone and glucagon receptors, as well as their downstream signaling pathways. These data suggest a functional requirement for RAMP2 in the modulation of additional G-protein-coupled receptor pathways in vivo, which is critical for sustained cardiovascular homeostasis. The cardiovascular importance of RAMP2 extends beyond the endothelium and canonical adrenomedullin/calcitonin receptor-like receptor signaling, in which future studies could elucidate novel and pharmacologically tractable pathways for treating cardiovascular diseases. PMID:27402918

  1. Can Serum Tenascin-C Be Used as a Marker of Inflammation in Patients with Dilated Cardiomyopathy?

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    Alyaa A. Kotby

    2013-01-01

    Full Text Available Background. Tenascin-C (TN-C is an extracellular matrix glycoprotein that appears at sites of inflammation in cardiac pathologies. Aim of the Work. To evaluate the role of TN-C as a marker for active inflammation in children with dilated cardiomyopathy (DCM. Subjects and Methods. 24 consecutive patients with primary nonfamilial DCM aged 6–72 months (mean 45.19±11.03 were divided into group I, twelve patients with acute onset DCM (6 months duration, and compared to 20 healthy age- and sex-matched controls. Investigations included estimation of serum TN-C and echocardiographic evaluation using M-mode and 2D speckle tracking echocardiography (STE. Results. Serum TN-C showed a higher significant statistical elevation among patients than controls (P<0.001 and in group I than group II (P<0.001. EF was significantly decreased, and LVEDD and EDV increased in patients than controls and in GI than GII. STE showed a statistically significant difference in global peak strain longitudinal (GPSL average in patients than controls (P<0.05 and between GI and GII (P<0.001. STE wall motion scoring showed normokinesia (33.5%, hypokinesia (8.33%, and akinesia (50% in GI and hypokinesia (100% in GII. There was a statistically significant positive correlation between serum TN-C and GPSL average. Conclusions. Increased serum TN-C can be used as a marker of inflammation in DCM and is associated with the severity of heart failure and LV dysfunction as detected by STE.

  2. A proton leak current through the cardiac sodium channel is linked to mixed arrhythmia and the dilated cardiomyopathy phenotype.

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    Pascal Gosselin-Badaroudine

    Full Text Available Cardiac Na(+ channels encoded by the SCN5A gene are essential for initiating heart beats and maintaining a regular heart rhythm. Mutations in these channels have recently been associated with atrial fibrillation, ventricular arrhythmias, conduction disorders, and dilated cardiomyopathy (DCM.We investigated a young male patient with a mixed phenotype composed of documented conduction disorder, atrial flutter, and ventricular tachycardia associated with DCM. Further family screening revealed DCM in the patient's mother and sister and in three of the mother's sisters. Because of the complex clinical phenotypes, we screened SCN5A and identified a novel mutation, R219H, which is located on a highly conserved region on the fourth helix of the voltage sensor domain of Na(v1.5. Three family members with DCM carried the R219H mutation.The wild-type (WT and mutant Na(+ channels were expressed in a heterologous expression system, and intracellular pH (pHi was measured using a pH-sensitive electrode. The biophysical characterization of the mutant channel revealed an unexpected selective proton leak with no effect on its biophysical properties. The H(+ leak through the mutated Na(v1.5 channel was not related to the Na(+ permeation pathway but occurred through an alternative pore, most probably a proton wire on the voltage sensor domain.We propose that acidification of cardiac myocytes and/or downstream events may cause the DCM phenotype and other electrical problems in affected family members. The identification of this clinically significant H(+ leak may lead to the development of more targeted treatments.

  3. A Novel Murine Model of Parvovirus Associated Dilated Cardiomyopathy Induced by Immunization with VP1-Unique Region of Parvovirus B19

    Science.gov (United States)

    Šimoliūnas, Egidijus; Rinkūnaitė, Ieva; Smalinskaitė, Luka; Podkopajev, Andrej; Bironaitė, Daiva; Weis, Cleo-Aron; Marx, Alexander; Bukelskienė, Virginija; Gretz, Norbert; Grabauskienė, Virginija; Labeit, Dittmar; Labeit, Siegfried

    2016-01-01

    Background. Parvovirus B19 (B19V) is a common finding in endomyocardial biopsy specimens from myocarditis and dilated cardiomyopathy patients. However, current understanding of how B19V is contributing to cardiac damage is rather limited due to the lack of appropriate mice models. In this work we demonstrate that immunization of BALB/c mice with the major immunogenic determinant of B19V located in the unique sequence of capsid protein VP1 (VP1u) is an adequate model to study B19V associated heart damage. Methods and Results. We immunized mice in the experimental group with recombinant VP1u; immunization with cardiac myosin derived peptide served as a positive reference and phosphate buffered saline served as negative control. Cardiac function and dimensions were followed echocardiographically 69 days after immunization. Progressive dilatation of left ventricle and decline of ejection fraction were observed in VP1u- and myosin-immunized mice. Histologically, severe cardiac fibrosis and accumulation of heart failure cells in lungs were observed 69 days after immunization. Transcriptomic profiling revealed ongoing cardiac remodeling and immune process in VP1u- and myosin-immunized mice. Conclusions. Immunization of BALB/c mice with VP1u induces dilated cardiomyopathy in BALB/c mice and it could be used as a model to study clinically relevant B19V associated cardiac damage. PMID:27812527

  4. Dilated cardiomyopathy and left bundle branch block associated with ingestion of colloidal gold and silver is reversed by British antiLewisite and vitamin E: The potential toxicity of metals used as health supplements

    OpenAIRE

    Archer, Stephen Lawrence

    2008-01-01

    A case of left bundle branch block and a dilated, nonhypertrophic cardiomyopathy associated with ingestion of colloidal gold and silver as an ‘energy tonic’ is described. The cardiac disease was reversed within two months by a course of dimercaprol (Akorn Inc, USA) (British antiLewisite) and vitamin E. This is the first case of gold and silver cardiomyopathy in humans, and highlights the risks of these colloidal metal ‘health supplements’.

  5. Th17-related cytokines in systemic lupus erythematosus patients with dilated cardiomyopathies: a possible linkage to parvovirus B19 infection.

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    Der-Yuan Chen

    Full Text Available Dilated cardiomyopathies (DCM are a major cause of mortality in patients with systemic lupus erythematosus (SLE. Immune responses induced by human parvovirus B19 (B19 are considered an important pathogenic mechanism in myocarditis or DCM. However, little is known about Th17-related cytokines in SLE patients with DCM about the linkage with B19 infection. IgM and IgG against B19 viral protein, and serum levels of Th17-related cytokines were determined using ELISA in eight SLE patients with DCM and six patients with valvular heart disease (VHD. Humoral responses of anti-B19-VP1u and anti-B19-NS1 antibody were assessed using Western blot and B19 DNA was detected by nested Polymerase Chain Reaction (PCR. Levels of interleukin (IL-17, IL-6, IL-1β, and tumor necrosis factor (TNF-α were significantly higher in SLE patients with DCM (mean ± SEM, 390.99±125.48 pg/ml, 370.24±114.09 pg/ml, 36.01±16.90 pg/ml, and 183.84±82.94 pg/ml, respectively compared to healthy controls (51.32±3.04 pg/ml, p<0.001; 36.88±6.64 pg/ml, p<0.001; 5.39±0.62 pg/ml, p<0.005; and 82.13±2.42 pg/ml, p<0.005, respectively. Levels of IL-17 and IL-6 were higher in SLE patients with DCM versus those with VHD (both p<0.01. Five (62.5% of DCM patients had detectable anti-B19-NS1 IgG and four (50.0% of them had anti-B19-VP1u IgG, whereas only one (16.7% of VHD patients had detectable anti-B19-NS1 IgG and anti-B19-VP1u IgG. Serum levels of IL-17, IL-6 and IL-1β were markedly higher in SLE patients with anti-B19-VP1u IgG and anti-B19-NS1 IgG compared to those without anti-B19-VP1u IgG or anti-B19-NS1 IgG, respectively. These suggest a potential association of B19 with DCM and Th17-related cytokines implicated in the pathogenesis of DCM in SLE patients.

  6. Association of Nicotinamide Phosphoribosyltransferase (NAMPT Gene Polymorphisms and of Serum NAMPT Levels with Dilated Cardiomyopathy in a Chinese Population

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    Qingyu Dou

    2015-09-01

    Full Text Available Nicotinamide phosphoribosyltransferase (NAMPT has crucial roles for myocardial development, cardiomyocyte energy metabolism and cell death/survival by regulating NAD+-dependent sirtuin-1 (SIRT1 deacetylase. This study aimed to determine if the single nucleotide polymorphisms (SNPs of the NAMPT gene may affect the susceptibility and prognosis for patients with dilated cardiomyopathy (DCM and to describe the association of serum NAMPT levels with clinical features of DCM. Three SNPs (rs61330082, rs2505568, and rs9034 were analyzed by the polymerase chain reaction-restriction fragment length polymorphism method in a case-control study of 394 DCM patients and 395 controls from China. Serum NAMPT levels were measured by enzyme-linked immunosorbent assay kits. The homozygote for the minor allele at rs2505568 and rs9034 could not be detected in this study. Rs9034 T allele and CT genotype were associated with increased DCM risk (OR: 1.63, 95% CI = 1.16–2.27, p = 0.005 and OR: 1.72, 95% CI = 1.20–2.50, p = 0.0027, respectively. Nominally significant decreased DCM risk was found to be associated with the A allele and AT genotype of rs2505568 (OR: 0.48, 95% CI = 0.35–0.67, p < 0.0001 and OR: 0.44, 95% CI = 0.31–0.62, p < 0.0001, respectively, but it should be interpreted with caution because of Hardy-Weinberg disequilibrium in the control group. Of five haplotypes constructed, TAC (rs61330082-rs2505568-rs9034 was a protective haplotype to DCM (OR: 0.22, 95% CI = 0.13–0.39, p = 1.84 × 10−8. The Cox multivariate survival analysis indicated that the rs9034 CT genotype (hazard ratio (HR: 0.59, 95% CI = 0.37–0.96, p = 0.03 was an independently multivariate predictor for longer overall survival in DCM patients. Serum NAMPT levels were significantly higher in the DCM group than controls (p < 0.0001 and gradually increased with the increase of New York Heart Association grade in DCM patients. However, there was a lack of association of the three

  7. Multistep ion channel remodeling and lethal arrhythmia precede heart failure in a mouse model of inherited dilated cardiomyopathy.

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    Takeshi Suzuki

    Full Text Available BACKGROUND: Patients with inherited dilated cardiomyopathy (DCM frequently die with severe heart failure (HF or die suddenly with arrhythmias, although these symptoms are not always observed at birth. It remains unclear how and when HF and arrhythmogenic changes develop in these DCM mutation carriers. In order to address this issue, properties of the myocardium and underlying gene expressions were studied using a knock-in mouse model of human inherited DCM caused by a deletion mutation ΔK210 in cardiac troponinT. METHODOLOGY/PRINCIPAL FINDINGS: By 1 month, DCM mice had already enlarged hearts, but showed no symptoms of HF and a much lower mortality than at 2 months or later. At around 2 months, some would die suddenly with no clear symptoms of HF, whereas at 3 months, many of the survivors showed evident symptoms of HF. In isolated left ventricular myocardium (LV from 2 month-mice, spontaneous activity frequently occurred and action potential duration (APD was prolonged. Transient outward (I(to and ultrarapid delayed rectifier K(+ (I(Kur currents were significantly reduced in DCM myocytes. Correspondingly, down-regulation of Kv4.2, Kv1.5 and KChIP2 was evident in mRNA and protein levels. In LVs at 3-months, more frequent spontaneous activity, greater prolongation of APD and further down-regulation in above K(+ channels were observed. At 1 month, in contrast, infrequent spontaneous activity and down-regulation of Kv4.2, but not Kv1.5 or KChIP2, were observed. CONCLUSIONS/SIGNIFICANCE: Our results suggest that at least three steps of electrical remodeling occur in the hearts of DCM model mice, and that the combined down-regulation of Kv4.2, Kv1.5 and KChIP2 prior to the onset of HF may play an important role in the premature sudden death in this DCM model. DCM mice at 1 month or before, on the contrary, are associated with low risk of death in spite of inborn disorder and enlarged heart.

  8. Assessment of right ventricular oxidative metabolism by PET in patients with idiopathic dilated cardiomyopathy undergoing cardiac resynchronisation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Knuuti, Juhani; Naum, Alexandru; Stolen, Kira Q.; Kalliokoski, Riikka [University of Turku, Turku PET Centre, P.O. Box 52, Turku (Finland); Sundell, Jan [University of Turku, Turku PET Centre, P.O. Box 52, Turku (Finland); University of Turku, Department of Medicine, Turku (Finland); Engblom, Erik; Koistinen, Juhani; Airaksinen, K.E. Juhani [University of Turku, Department of Medicine, Turku (Finland); Ylitalo, Antti [Satakunta Central Hospital, Department of Medicine, Pori (Finland); Nekolla, Stephan G. [Klinikum rechts der Isar der Technischen Universitaet Muenchen, Klinik und Poliklinik fuer Nuklearmedizin, Munich (Germany); Bax, K.E. Jeroen J. [Leiden University, Department of Cardiology, Leiden (Netherlands)

    2004-12-01

    Right ventricular (RV) performance is known to have prognostic value in patients with congestive heart failure (CHF). Cardiac resynchronisation therapy (CRT) has been found to enhance left ventricular (LV) energetics and metabolic reserve in patients with heart failure. The interplay between the LV and RV may play an important role in CRT response. The purpose of the study was to investigate RV oxidative metabolism, metabolic reserve and the effects of CRT in patients with CHF and left bundle brach block. In addition, the role of the RV in the response to CRT was evaluated. Ten patients with idiopathic dilated cardiomyopathy who had undergone implantation of a biventricular pacemaker 8{+-}5 months earlier were studied under two conditions: CRT ON and after CRT had been switched OFF for 24 h. Oxidative metabolism was measured using [{sup 11}C]acetate positron emission tomography (K{sub mono}). The measurements were performed at rest and during dobutamine-induced stress (5 {mu}g/kg per minute). LV performance and interventricular mechanical delay (interventricular asynchrony) were measured using echocardiography. CRT had no effect on RV K{sub mono} at rest (ON: 0.052{+-}0.014, OFF: 0.047{+-}0.018, NS). Dobutamine-induced stress increased RV K{sub mono} significantly under both conditions but oxidative metabolism was more enhanced when CRT was ON (0.076{+-}0.026 vs 0.065{+-}0.027, p=0.003). CRT shortened interventricular delay significantly (45{+-}33 vs 19{+-}35 ms, p=0.05). In five patients the response to CRT was striking (32% increase in mean LV stroke volume, range 18-36%), while in the other five patients no response was observed (mean change +2%, range -6% to +4%). RV K{sub mono} and LV stroke volume response to CRT correlated inversely (r=-0.66, p=0.034). None of the other measured parameters, including all LV parameters and electromechanical parameters, were associated with the response to CRT. In responders, RV K{sub mono} with CRT OFF was significantly lower

  9. Virus-induced dilated cardiomyopathy is characterized by increased levels of fibrotic extracellular matrix proteins and reduced amounts of energy-producing enzymes.

    Science.gov (United States)

    Nishtala, Krishnatej; Phong, Truong Q; Steil, Leif; Sauter, Martina; Salazar, Manuela G; Kandolf, Reinhard; Kroemer, Heyo K; Felix, Stephan B; Völker, Uwe; Klingel, Karin; Hammer, Elke

    2011-11-01

    The most relevant clinical phenotype resulting from chronic enteroviral myocarditis is dilated cardiomyopathy (DCM). Mice of the susceptible mouse strain A.BY/SnJ mimick well human DCM since they develop as a consequence of persistent infection and chronic inflammation a dilation of the heart ventricle several weeks after coxsackievirus B3 (CVB3) infection. Therefore, this model is well suited for the analysis of changes in the heart proteome associated with DCM. Here, we present a proteomic survey of the dilated hearts based on differential fluorescence gel electrophoresis and liquid chromatography-mass spectrometric centered methods in comparison to age-matched non-infected hearts. In total, 101 distinct proteins, which belong to categories immunity and defense, cell structure and associated proteins, energy metabolism and protein metabolism/modification differed in their levels in both groups. Levels of proteins involved in fatty acid metabolism and electron transport chain were found to be significantly reduced in infected mice suggesting a decrease in energy production in CVB3-induced DCM. Furthermore, proteins associated with muscle contraction (MLRV, MLRc2, MYH6, MyBPC3), were present in significantly altered amounts in infected mice. A significant increase in the level of extracellular matrix proteins in the dilated hearts indicates cardiac remodeling due to fibrosis.

  10. Systematic review of pregnancy in women with inherited cardiomyopathies

    NARCIS (Netherlands)

    Krul, Sebastien P. J.; van der Smagt, Jasper J.; van den Berg, Maarten P.; Sollie, Krystyna M.; Pieper, Petronella G.; van Spaendonck-Zwarts, Karin Y.

    2011-01-01

    Pregnancy exposes women with inherited cardiomyopathies to increased risk for heart failure and arrhythmias. In this paper, we review the clinical course and management of pregnant women with the following inherited cardiomyopathies: hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogeni

  11. Genome-wide Studies of Copy Number Variation and Exome Sequencing Identify Rare Variants in BAG3 as a Cause of Dilated Cardiomyopathy

    Science.gov (United States)

    Norton, Nadine; Li, Duanxiang; Rieder, Mark J.; Siegfried, Jill D.; Rampersaud, Evadnie; Züchner, Stephan; Mangos, Steve; Gonzalez-Quintana, Jorge; Wang, Libin; McGee, Sean; Reiser, Jochen; Martin, Eden; Nickerson, Deborah A.; Hershberger, Ray E.

    2011-01-01

    Dilated cardiomyopathy commonly causes heart failure and is the most frequent precipitating cause of heart transplantation. Familial dilated cardiomyopathy has been shown to be caused by rare variant mutations in more than 30 genes but only ∼35% of its genetic cause has been identified, principally by using linkage-based or candidate gene discovery approaches. In a multigenerational family with autosomal dominant transmission, we employed whole-exome sequencing in a proband and three of his affected family members, and genome-wide copy number variation in the proband and his affected father and unaffected mother. Exome sequencing identified 428 single point variants resulting in missense, nonsense, or splice site changes. Genome-wide copy number analysis identified 51 insertion deletions and 440 copy number variants > 1 kb. Of these, a 8733 bp deletion, encompassing exon 4 of the heat shock protein cochaperone BCL2-associated athanogene 3 (BAG3), was found in seven affected family members and was absent in 355 controls. To establish the relevance of variants in this protein class in genetic DCM, we sequenced the coding exons in BAG3 in 311 other unrelated DCM probands and identified one frameshift, two nonsense, and four missense rare variants absent in 355 control DNAs, four of which were familial and segregated with disease. Knockdown of bag3 in a zebrafish model recapitulated DCM and heart failure. We conclude that new comprehensive genomic approaches have identified rare variants in BAG3 as causative of DCM. PMID:21353195

  12. Pregnancy, cardiomyopathies, and genetics

    NARCIS (Netherlands)

    Van Tintelen, J. Peter; Pieper, Petronella G.; Van Spaendonck-Zwarts, Karin Y.; Van den Berg, Maarten P.

    2014-01-01

    Although familial forms of cardiomyopathy such as hypertrophic or dilated cardiomyopathy have been recognized for decades, it is only recently that much of the genetic basis of these inherited cardiomyopathies has been elucidated. This has provided important insights into the pathophysiological mech

  13. Efficacy of Carvedilol in Patients with Dilated Cardiomyopathy due to Beta-Thalassemia Major; a Double-Blind Randomized Controlled Trial

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    Afsaneh Ashrafi

    2010-09-01

    Full Text Available Objective: Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy.Methods: During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging (TDI data were collected from each patient.Findings: Eight patients received Carvedilol (Group 1 and six received placebo (Group 2. The mean age of patients in Group1 and 2 were 16±0.7 years and 17±3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association (NYHA classification, Ejection fraction, End diastolic dimension changes, TDI systolic(S, early (Ea and late (Aa diastolic waves were not statistically significant in these two Groups (P>0.05. Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1±0.37 m/s to 1.8±0.40 m/s and from 1.34±0.30 m/s to 2.6±0.23m/s respectively (P=0.04.Conclusion: Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients.

  14. Familial dilated cardiomyopathy associated with congenital defects in the setting of a novel VCL mutation (Lys815Arg in conjunction with a known MYPBC3 variant

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    Quinn S. Wells

    2011-08-01

    Full Text Available Idiopathic dilated cardiomyopathy (DCM is a primary myocardial disorder characterized by ventricular chamber enlargement and systolic dysfunction. Twenty to fifty percent of idiopathic DCM cases are thought to have a genetic cause. Of more than 30 genes known to be associated with DCM, rare variants in the VCL and MYBPC3 genes have been reported in several cases of DCM. In this report, we describe a family with DCM and congenital abnormalities who carry a novel missense mutation in the VCL gene. More severely affected family members also possess a second missense variant in MYBPC3, raising the possibility that this variant may be a disease modifier. Intere - stingly, many of the affected individuals also have congenital defects, including two with bicuspid aortic valve with aortic regurgitation. We discuss the implications of the family history and genetic information on management of at-risk individuals with aortic regurgitation.

  15. Possible Association of HLA-DRB1 Gene with the Autoantibody against Myocardial Mitochondria ADP/ATP Carrier in Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王秋芬; 廖玉华; 龚非力; 毛焕元; 张金枝

    2002-01-01

    Summary: To probe the genetic background and immunopathogenesis of dilated cardiomyopathy (DCM) 77 patients with DCM, HLA-DRB1 gene polymorphism were analyzed by using the polymerase chain reaction /sequence specific primer (PCR/SSP) technique and autoantibody against myocardial mitochondria ADP/ATP carrier were examined by using the Immunoblot analysis. The frequency of HLA-DRB1 * 0901 allele was significantly higher in DCM patients in which autoantibody against ADP/ATP carrier of myocardial mitochondria is positive in contrast with those in which the autoantibody is negative (25.46 % vs 3.45 %, P<0.05), the relative risk (RR) being 9.56. The other frequencies of HLA-DRB1 alleles have no significant difference in the antibody positive group and negative group. It is possible that a subset of DCM patients may exist in which autoimmunity is associated with genetic factors.

  16. [New echocardiographic para-apical technic for determining the size and thickness of the wall of the right ventricle in probands and patients with dilated cardiomyopathy].

    Science.gov (United States)

    Dyczynski, J; Schweitzer, L

    1987-12-01

    To evaluate the right ventricular size and free wall thickness a new echocardiographic para-apical technique was developed. All cases were examined from the para-apical window in the extreme left lateral decubitus position, on a special mattress that allowed us to investigate the total apical region. The para-apical transducer position makes it possible to cut the right ventricle cross-sectionally in serial bread-loafing fashion from the apex to the base, like the left ventricle. In this way, using the M-mode technique, either diameters and/or right ventricular free wall thickness as well as shortening fraction could be estimated. To estimate the usefulness of the para-apical technique, a study was performed in 50 normal subjects as well as in 16 patients with dilated cardiomyopathy. In all patients, standard views and the new para-apical view were performed. In five cases, contrast echocardiography of the heart was performed to verify the right-heart anatomy. In five patients, computer-tomography of the heart was performed to see the position of the right-heart chambers in the chest and to evaluate the new para-apical ultrasound window. The standard values for the right ventricle in the control group were (in mean values): diastolic diameter, 28.61 +/- 3.19 mm; systolic diameter, 19.10 +/- 2.40 mm; free wall thickness, 4.46 +/- 1.06 mm and shortening fraction, 32.42% +/- 4.02%. The examined parameters in patients with dilated cardiomyopathy were: diastolic diameter, mean value 38.63 +/- 5.80 mm; systolic diameter, mean value 32.48 +/- 4.96 mm; free wall thickness, mean value 8.70 +/- 1.05 mm and shortening fraction, mean value 15.96 +/- 4.02%.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3439250

  17. Allogeneic cardiospheres delivered via percutaneous transendocardial injection increase viable myocardium, decrease scar size, and attenuate cardiac dilatation in porcine ischemic cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Kristine Yee

    Full Text Available Epicardial injection of heart-derived cell products is safe and effective post-myocardial infarction (MI, but clinically-translatable transendocardial injection has never been evaluated. We sought to assess the feasibility, safety and efficacy of percutaneous transendocardial injection of heart-derived cells in porcine chronic ischemic cardiomyopathy.We studied a total of 89 minipigs; 63 completed the specified protocols. After NOGA-guided transendocardial injection, we quantified engraftment of escalating doses of allogeneic cardiospheres or cardiosphere-derived cells in minipigs (n = 22 post-MI. Next, a dose-ranging, blinded, randomized, placebo-controlled ("dose optimization" study of transendocardial injection of the better-engrafting product was performed in infarcted minipigs (n = 16. Finally, the superior product and dose (150 million cardiospheres were tested in a blinded, randomized, placebo-controlled ("pivotal" study (n = 22. Contrast-enhanced cardiac MRI revealed that all cardiosphere doses preserved systolic function and attenuated remodeling. The maximum feasible dose (150 million cells was most effective in reducing scar size, increasing viable myocardium and improving ejection fraction. In the pivotal study, eight weeks post-injection, histopathology demonstrated no excess inflammation, and no myocyte hypertrophy, in treated minipigs versus controls. No alloreactive donor-specific antibodies developed over time. MRI showed reduced scar size, increased viable mass, and attenuation of cardiac dilatation with no effect on ejection fraction in the treated group compared to placebo.Dose-optimized injection of allogeneic cardiospheres is safe, decreases scar size, increases viable myocardium, and attenuates cardiac dilatation in porcine chronic ischemic cardiomyopathy. The decreases in scar size, mirrored by increases in viable myocardium, are consistent with therapeutic regeneration.

  18. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part I (Diagnostics

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2014-01-01

    Full Text Available PAim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA and the syndrome of dilated cardiomyopathy (DCM.Material and methods. Patients (n=320 with IA (n=190; 117 women, age 45.3±14.8 years and DCM (n=130, 41 women, age 46.9±12.5 years were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM, viral serology (166 and 122, multispiral computed tomography (42 and 88, cardiac magnetic resonance imaging (41 and 22, coronary angiography (19 and 54, myocardial biopsy/autopsy (19/0 and 33/9 were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% of IA and 66.7% of DCM-patients, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency  of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower, while in the main group a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patients.Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach.

  19. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part I (Diagnostics

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2015-09-01

    Full Text Available PAim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA and the syndrome of dilated cardiomyopathy (DCM.Material and methods. Patients (n=320 with IA (n=190; 117 women, age 45.3±14.8 years and DCM (n=130, 41 women, age 46.9±12.5 years were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM, viral serology (166 and 122, multispiral computed tomography (42 and 88, cardiac magnetic resonance imaging (41 and 22, coronary angiography (19 and 54, myocardial biopsy/autopsy (19/0 and 33/9 were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% of IA and 66.7% of DCM-patients, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency  of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower, while in the main group a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patients.Conclusion. Nosological cause of IA and DCM syndrome can be diagnosed in most patients by using an integrated clinical and morphological approach.

  20. Genetic linkage analysis excludes HLA and several other potential candidates as being responsible for familial dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Durand, J.B.; Bachinski, L.L.; Beiling, L. [Baylor College of Medicine, Houston, TX (United States)] [and others

    1994-09-01

    Familial dilated cardiomyopthy (FDCM), manifested by ventricular dilation and decreased systolic function, is inherited as an autosomal dominant trait. We identified a family segregating DCM with 11 affected living individuals in whom the diagnosis was confirmed by echocardiography (EF <50%, left ventricular volume >80 ml/m{sup 2}). DNA was extracted and analyzed with highly polymorphmic microsatellite markers (STRs). In view of the high frequency of antibodies to specific HLA proteins in FDCM, this region was selected as a possible candidate locus. Genes whose products are sarcomeric proteins were also selected as candidates. Genetic linkage of FDCM to these candidate genes was excluded on the basis of a LOD score of <= -2. Subsequent to the candidate gene approach we pursued random mapping and completed analysis of a total of 93 chromosomal markers excluding 1000 cM.

  1. Locally expressed IGF1 propeptide improves mouse heart function in induced dilated cardiomyopathy by blocking myocardial fibrosis and SRF-dependent CTGF induction

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    Melissa Touvron

    2012-07-01

    Cardiac fibrosis is critically involved in the adverse remodeling accompanying dilated cardiomyopathies (DCMs, which leads to cardiac dysfunction and heart failure (HF. Connective tissue growth factor (CTGF, a profibrotic cytokine, plays a key role in this deleterious process. Some beneficial effects of IGF1 on cardiomyopathy have been described, but its potential role in improving DCM is less well characterized. We investigated the consequences of expressing a cardiac-specific transgene encoding locally acting IGF1 propeptide (muscle-produced IGF1; mIGF1 on disease progression in a mouse model of DCM [cardiac-specific and inducible serum response factor (SRF gene disruption] that mimics some forms of human DCM. Cardiac-specific mIGF1 expression substantially extended the lifespan of SRF mutant mice, markedly improved cardiac functions, and delayed both DCM and HF. These protective effects were accompanied by an overall improvement in cardiomyocyte architecture and a massive reduction of myocardial fibrosis with a concomitant amelioration of inflammation. At least some of the beneficial effects of mIGF1 transgene expression were due to mIGF1 counteracting the strong increase in CTGF expression within cardiomyocytes caused by SRF deficiency, resulting in the blockade of fibroblast proliferation and related myocardial fibrosis. These findings demonstrate that SRF plays a key role in the modulation of cardiac fibrosis through repression of cardiomyocyte CTGF expression in a paracrine fashion. They also explain how impaired SRF function observed in human HF promotes fibrosis and adverse cardiac remodeling. Locally acting mIGF1 efficiently protects the myocardium from these adverse processes, and might thus represent a therapeutic avenue to counter DCM.

  2. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part II (Treatment

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2014-01-01

    Full Text Available Part I (Diagnostics, was published in the journal “Rational Pharmacotherapy in Cardiology” 2014;10(1:62-72Aim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA and the syndrome of dilated cardiomyopathy (DCM.Material and methods. Patients (n=320 with IA (n=190; 117 women, age 45.3±14.8 years and DCM (n=130, 41 women, age 46.9±12.5 years were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM, viral serology (166 and 122, multispiral computed tomography (42 and 88, cardiac magnetic resonance imaging (41 and 22, coronary angiography (19 and 54, myocardial biopsy/autopsy (19/0 and 33/9 were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% patients with IA and 66.7% patients with DCM, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower than in the main group, where a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patientsThe basic therapy (antiviral drugs, corticosteroids, hydroxychloroquine, azathioprine was performed in some patients with myocarditis. Improving of the

  3. CLINICAL AND MORPHOLOGICAL APPROACH TO DIAGNOSIS OF "IDIOPATHIC" ARRHYTHMIAS AND DILATED CARDIOMYOPATHY SYNDROME AS A BASIS FOR DIFFERENTIATED THERAPY. Part II (Treatment

    Directory of Open Access Journals (Sweden)

    O. V. Blagova

    2015-09-01

    Full Text Available Part I (Diagnostics, was published in the journal “Rational Pharmacotherapy in Cardiology” 2014;10(1:62-72Aim. To develop a comprehensive clinical and morphological approach to the nosological diagnosis and treatment of "idiopathic" arrhythmias (IA and the syndrome of dilated cardiomyopathy (DCM.Material and methods. Patients (n=320 with IA (n=190; 117 women, age 45.3±14.8 years and DCM (n=130, 41 women, age 46.9±12.5 years were included in the main group. 51 people (patients with ischemic heart disease; heart valve disease, hypertrophic cardiomyopathy, who underwent open-heart surgery; healthy volunteers were included in the control group. Along with the standard tests evaluation of the level of anti-heart antibodies (185 patients with IA and 122 with DCM, viral serology (166 and 122, multispiral computed tomography (42 and 88, cardiac magnetic resonance imaging (41 and 22, coronary angiography (19 and 54, myocardial biopsy/autopsy (19/0 and 33/9 were performed.Results. According to morphological study infectious-immune myocarditis was found in 78.9% patients with IA and 66.7% patients with DCM, arrhythmogenic right ventricular dysplasia in 5.3% and 4.8% of patients, respectively. Other genetic cardiomyopathies, including combination with myocarditis were revealed in other patients. The frequency of the viral genome detection in the myocardium in IA, DCM and the control group was 17.6%, 66.7% and 77.1%, respectively. However in the control group the incidence of myocarditis and anti-heart antibodies titers were significantly lower than in the main group, where a strong correlation between myocarditis and anti-heart antibodies titers was found. The algorithm of noninvasive nosological diagnostics was developed; it allowed to verify diagnosis in 95% of IA patients and 89% DCM patientsThe basic therapy (antiviral drugs, corticosteroids, hydroxychloroquine, azathioprine was performed in some patients with myocarditis. Improving of the

  4. Sex differences in cardiomyopathies

    NARCIS (Netherlands)

    Meyer, Sven; van der Meer, Peter; van Tintelen, J. Peter; van den Berg, Maarten P.

    2014-01-01

    Cardiomyopathies are a heterogeneous group of heart muscle diseases with a variety of specific phenotypes. According to the contemporary European Society of Cardiology classification, they are classified into hypertrophic (HCM), dilated (DCM), arrhythmogenic right ventricular (ARVC), restrictive (RC

  5. [Arrhythmic cardiomyopathy. Case report].

    Science.gov (United States)

    Streangă, Violeta; Dimitriu, A G; Iordache, C; Georgescu, G; Grecu, Mihaela

    2004-01-01

    An 11 year-old boy was admitted with incessant sinus node reentrant tachycardia and secondary dilated arrhythmic cardiomyopathy, treated by radiofrequency ablation. Two years later he was admitted with incessant automatic atrial tachycardia and arrhythmic cardiomyopathy; a second catheter ablation procedure failed, but the third one, performed four month later, was successfully and resulted in a restoration of a normal sinus rhythm and a complete regression of arrhythmic cardiomyopathy.

  6. Use of iodine-123 metaiodobenzylguanidine myocardial imaging to predict the effectiveness of β-blocker therapy in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    We studied 13 patients with dilated cardiomyopathy (DCM) and seven normal subjects. We obtained myocardial SPET images 15 min and 4 h after administration of 123I-MIBG (111 MBq). Studies were performed in the patients with DCM before and 1 and 3 months after the administration of metoprolol and in the normal subjects. We calculated the regional 123I-MIBG washout rate (r-WR) in the SPET image, and the global 123I-MIBG washout rate (g-WR) and heart-mediastinum activity ratio (H/M) using the anterior planar image. We classified patients into those showing a ≥5% increase in LV ejection fraction (LVEF) at 3 months compared with LVEF values before the treatment (group I, n=7) and those showing a 123I-MIBG SPET imaging can be used to predict the functional improvement of LVEF at 1 month of β-blocker therapy in patients with DCM. (orig./VHE). With 4 figs., 1 tab

  7. Early-progressive dilated cardiomyopathy in a family with Becker muscular dystrophy related to a novel frameshift mutation in the dystrophin gene exon 27.

    Science.gov (United States)

    Tsuda, Takeshi; Fitzgerald, Kristi; Scavena, Mena; Gidding, Samuel; Cox, Mary O; Marks, Harold; Flanigan, Kevin M; Moore, Steven A

    2015-03-01

    We report a family in which two male siblings with Becker muscular dystrophy (BMD) developed severe dilated cardiomyopathy (DCM) and progressive heart failure (HF) at age 11 years; one died at age 14 years while awaiting heart transplant and the other underwent left ventricular assist device implantation at the same age. Genetic analysis of one sibling showed a novel frameshift mutation in exon 27 of Duchenne muscular dystrophy (DMD) gene (c.3779_3785delCTTTGGAinsGG), in which seven base pairs are deleted and two are inserted. Although this predicts an amino-acid substitution and premature termination (p.Thr1260Argfs*8), muscle biopsy dystrophin immunostaining instead indicates that the mutation is more likely to alter splicing. Despite relatively preserved skeletal muscular performance, both the siblings developed progressive HF secondary to early-onset DCM. In addition, their 7-year-old nephew with delayed gross motor development, mild proximal muscle weakness and markedly elevated serum creatine kinase level (>13 000 IU l(-1)) at 16 months was recently demonstrated to have the familial DMD mutation. Here, we report a novel genotype of BMD with early-onset DCM and progressive lethal HF during early adolescence.

  8. Prognostic value of repeated {sup 123}I-metaiodobenzylguanidine imaging in patients with dilated cardiomyopathy with congestive heart failure before and after optimized treatments. Comparison with neurohumoral factors

    Energy Technology Data Exchange (ETDEWEB)

    Matsui, Toshiki; Tsutamoto, Takayoshi; Maeda, Keiko; Kusukawa, Junya; Kinoshita, Masahiko [Shiga Univ. of Medical Science, Otsu (Japan)

    2002-06-01

    The present study was undertaken to assess whether repeated measurement of cardiac {sup 123}I-metaiodobenzylguanidine (MIBG) imaging parameters before and after optimized treatments is useful for predicting the prognosis of patients with congestive heart failure (CHF) resulting from dilated cardiomyopathy (DCM). The subjects were 85 consecutive patients with DCM who had a left ventricular ejection fraction (LVEF) of less than 45%. The MIBG and the concentrations of neurohumoral factors were measured at baseline and after 6 months of optimized treatments. Cox proportional hazards analysis was performed to assess the various parameters before and after treatment. Twenty-three patients had a cardiac event (12 died; 11 hospitalized) during a mean follow-up period of 2 years. Although there was no difference between the baseline heart to mediastinum (H/M) ratio measured by MIBG between survivors and nonsurvivors, the H/M ratio was significantly decreased in nonsurvivors after 6 months. Multivariate analysis revealed that a high plasma concentration of brain natriuretic peptide level after 6 months (p=0.0049) and absolute changes in the H/M ratio (p=0.0046) were independent predictors of mortality. Comparison of the H/M ratio on MIBG imaging before and after optimized additional treatment provided useful information for predicting mortality and was independent of clinical and neurohumoral factors previously shown to be associated with poor prognosis in patients with DCM. (author)

  9. A Mitochondrial DNA A8701G Mutation Associated with Maternally Inherited Hypertension and Dilated Cardiomyopathy in a Chinese Pedigree of a Consanguineous Marriage

    Institute of Scientific and Technical Information of China (English)

    Ye Zhu; Xiang Gu; Chao Xu

    2016-01-01

    Background: Cardiovascular diseases, including dilated cardiomyopathy (DCM) and hypertension, are the leading cause of death worldwide.The role of mitochondrial DNA (mtDNA) in the pathogenesis of these diseases has not been completely clarified.In this study, we evaluate whether A8701G mutation is associated with maternally inherited hypertension and DCM in a Chinese pedigree of a consanguineous marriage.Methods: Fourteen subjects in a three-generation Han Chinese family with hypertension and DCM, in which consanguineous marriage was present in the parental generation, were interviewed.We divided all the family members into case (7 maternal members) and control group (7 nonmaternal members) for comparison.Clinical evaluations and sequence analysis ofmtDNA were obtained from all participants.Frequency differences between maternal and nonmaternal members were tested to locate the disease-associated mutations.Results: The majority of the family members presented with a maternal inheritance of hypertension and DCM.Sequence analysis of mtDNA in this pedigree identified eight mtDNA mutations.Among the mutations identified, there was only one significant mutation: A8701G (P =0.005), which is a homoplasmic mitochondrial missense mutation in all the matrilineal relatives.There was no clear evidence for any synergistic effects between A8701G and other mutations.Conclusions: A8701G mutation may act as an inherited risk factor for the matrilineal transmission of hypertension and DCM in conj unction with genetic disorders caused by consanguineous marriage.

  10. R25G mutation in exon 1 of LMNA gene is associated with dilated cardiomyopathy and limb-girdle muscular dystrophy 1B

    Institute of Scientific and Technical Information of China (English)

    YUAN Wo-liang; HUANG Wei-jun; HUANG Chun-yan; WANG Jing-feng; XIE Shuang-lun; NIE Ru-qiong; LIU Ying-mei; LIU Pin-ming; ZHOU Shu-xian; CHEN Su-qin

    2009-01-01

    Background Mutations of the LMNA gene encoding lamin A and C are associated with dilated cardiomyopathy (DCM), conduction system defects and skeletal muscle dystrophy. Here we report a family with a mutation of the LMNA gene to identify the relationship between genotype and phenotype.Methods All 30 members of the family underwent clinical and genetic evaluation. A mutation analysis of the LMNA gene was performed. All of the 12 exons of LMNA gene were extended with polymerase chain reaction (PCR) and the PCR products were screened for gene mutation by direct sequencing.Results Ten members of the family had limb-girdle muscular dystrophy (LGMD) and 6 are still alive. Two patients suffered from DCM. Cardiac arrhythmias included atrioventricular block and atrial fibrillation; sudden death occurred in 2 patients. The pattern of inheritance was autosomal dominant. Mutation c.73C>G (R25G) in exon 1 encoding the globular domains was confirmed in all of the affected members, resulting in the conversion of arginine (Arg) to glycine (Gly). Conclusions The mutation R25G in exon 1 of LMNA gene we reported here in a Chinese family had a phenotype of malignant arrhythmia and mild LGMD, suggesting that patients with familial DCM, conduction system defects and skeletal muscle dystrophy should be screened by genetic testing for the LMNA gene.

  11. Usefulness of microvolt T-wave alternans for prediction of ventricular tachyarrhythmic events in patients with dilated cardiomyopathy: results from a prospective observational study

    Science.gov (United States)

    Hohnloser, Stefan H.; Klingenheben, Thomas; Bloomfield, Daniel; Dabbous, Omar; Cohen, Richard J.

    2003-01-01

    OBJECTIVES: This study was designed to evaluate the ability of microvolt-level T-wave alternans (MTWA) to identify prospectively patients with idiopathic dilated cardiomyopathy (DCM) at risk of ventricular tachyarrhythmic events and to compare its predictive accuracy with that of conventional risk stratifiers. BACKGROUND: Patients with DCM are at increased risk of sudden death from ventricular tachyarrhythmias. At present, there are no established methods of assessing this risk. METHODS: A total of 137 patients with DCM underwent risk stratification through assessment of MTWA, left ventricular ejection fraction, baroreflex sensitivity (BRS), heart rate variability, presence of nonsustained ventricular tachycardia (VT), signal-averaged electrocardiogram, and presence of intraventricular conduction defect. The study end point was either sudden death, resuscitated ventricular fibrillation, or documented hemodynamically unstable VT. RESULTS: During an average follow-up of 14 +/- 6 months, MTWA and BRS were significant univariate predictors of ventricular tachyarrhythmic events (p < 0.035 and p < 0.015, respectively). Multivariate Cox regression analysis revealed that only MTWA was a significant predictor. CONCLUSIONS: Microvolt-level T-wave alternans is a powerful independent predictor of ventricular tachyarrhythmic events in patients with DCM.

  12. Use of RNA-seq to identify cardiac genes and gene pathways differentially expressed between dogs with and without dilated cardiomyopathy.

    Science.gov (United States)

    Friedenberg, Steven G; Chdid, Lhoucine; Keene, Bruce; Sherry, Barbara; Motsinger-Reif, Alison; Meurs, Kathryn M

    2016-07-01

    OBJECTIVE To identify cardiac tissue genes and gene pathways differentially expressed between dogs with and without dilated cardiomyopathy (DCM). ANIMALS 8 dogs with and 5 dogs without DCM. PROCEDURES Following euthanasia, samples of left ventricular myocardium were collected from each dog. Total RNA was extracted from tissue samples, and RNA sequencing was performed on each sample. Samples from dogs with and without DCM were grouped to identify genes that were differentially regulated between the 2 populations. Overrepresentation analysis was performed on upregulated and downregulated gene sets to identify altered molecular pathways in dogs with DCM. RESULTS Genes involved in cellular energy metabolism, especially metabolism of carbohydrates and fats, were significantly downregulated in dogs with DCM. Expression of cardiac structural proteins was also altered in affected dogs. CONCLUSIONS AND CLINICAL RELEVANCE Results suggested that RNA sequencing may provide important insights into the pathogenesis of DCM in dogs and highlight pathways that should be explored to identify causative mutations and develop novel therapeutic interventions. PMID:27347821

  13. Effects of carvedilol in heart failure due to dilated cardiomyopathy. Results of a double-blind randomized placebo-controlled study (CARIBE study

    Directory of Open Access Journals (Sweden)

    Paulo Roberto Chizzola

    2000-03-01

    Full Text Available OBJECTIVE: To assess the effects of carvedilol in patients with idiopathic dilated cardiomyopathy. METHODS: In a double-blind randomized placebo-controlled study, 30 patients (7 women with functional class II and III heart failure were assessed. Their ages ranged from 28 to 66 years (mean of 43±9 years, and their left ventricular ejection fraction varied from 8% to 35%. Carvedilol was added to the usual therapy of 20 patients; placebo was added to the usual therapy of 10 patients. The initial dose of carvedilol was 12.5 mg, which was increased weekly until it reached 75 mg/day, according to the patient's tolerance. Clinical assessment, electrocardiogram, echocardiogram, and radionuclide ventriculography were performed in the pretreatment phase, being repeated after 2 and 6 months of medication use. RESULTS: A reduction in heart rate (p=0.016 as well as an increase in left ventricular shortening fraction (p=0.02 and in left ventricular ejection fraction (p=0.017 occurred in the group using carvedilol as compared with that using placebo. CONCLUSION: Carvedilol added to the usual therapy for heart failure resulted in better heart function.

  14. [Peripartum cardiomyopathy].

    Science.gov (United States)

    Mouquet, Frédéric; Bouabdallaoui, Nadia

    2015-01-01

    The peripartum cardiomyopathy is a rare form of dilated cardiomyopathy resulting from alteration of angiogenesis toward the end of pregnancy. The diagnosis is based on the association of clinical heart failure and systolic dysfunction assessed by echocardiography or magnetic resonance imaging. Diagnoses to rule out are myocardial infarction, amniotic liquid embolism, myocarditis, inherited cardiomyopathy, and history of treatment by anthracycline. Risk factors are advance maternal age (>30), multiparity, twin pregnancy, African origin, obesity, preeclampsia, gestational hypertension, and prolonged tocolytic therapy. Treatment of acute phase is identical to usual treatment of acute systolic heart failure. After delivery, VKA treatment should be discussed in case of systolic function pregnancy should be discouraged, especially if systolic function did not recover.

  15. [Peripartum cardiomyopathy].

    Science.gov (United States)

    Mouquet, Frédéric; Bouabdallaoui, Nadia

    2015-01-01

    The peripartum cardiomyopathy is a rare form of dilated cardiomyopathy resulting from alteration of angiogenesis toward the end of pregnancy. The diagnosis is based on the association of clinical heart failure and systolic dysfunction assessed by echocardiography or magnetic resonance imaging. Diagnoses to rule out are myocardial infarction, amniotic liquid embolism, myocarditis, inherited cardiomyopathy, and history of treatment by anthracycline. Risk factors are advance maternal age (>30), multiparity, twin pregnancy, African origin, obesity, preeclampsia, gestational hypertension, and prolonged tocolytic therapy. Treatment of acute phase is identical to usual treatment of acute systolic heart failure. After delivery, VKA treatment should be discussed in case of systolic function <25% because of higher risk of thrombus. A specific treatment by bromocriptine can be initiated on a case-by-case basis. Complete recovery of systolic function is observed in 50% of cases. The mortality risk is low. Subsequent pregnancy should be discouraged, especially if systolic function did not recover. PMID:26160284

  16. Experimental Study ofa New Operative Procedure for Non-Ischemic Dilated Cardiomyopathy-Overlapping Cardiac Volume Reduction Operation

    Institute of Scientific and Technical Information of China (English)

    罗滨; 孟春营; 温定国; 松居喜朗; 安田庆秀

    2003-01-01

    Objectives To assess anewly devised procedure of cardiac volume reduction without resection of cardiac muscle and evaluated in experimental settings. Methods Ten beagle dogs underwent a rapid pacing leading to heart failure for 3 weeks and received the left ventricular reduction termed overlapping cardiac volume reduction operation (OLCVR) ,which consisted of a longitudinal incision in left ventricular (LV) free wall, sutures of the left marginal to the septal wall, and the right marginal to LV free wall.A slope of the linear preload recruitable stroke work relationship (Mw) , with a X - intercept (Vo) were calculated as the precise indicators of left ventricular systolic function. The constant of isovolumic pressure decay (Tau) and a peak filling rate (PFR) were also calculated as the indicators of LV diastolic function.Results LV end- diastolic dimensions was significantly reduced by OLCVR (43±2 to 25±1; mm).Fractional shortening was significantly improved by OLCVR (11±2 to 30±4;%). Mw (erg* cm-3* 103)was also significantly improved (21±2 to 33 ±3 (p<0. 001 ) ) , whereas Vo, Tau and PFR did not show significant changes. Conclusions The OLCVR significantly increased in the early LV systolic function without detrimental effects on diastolic function. This procedure may become a therapeutic option for end - stage cardiomyopathy.

  17. The LMNA mutation p.Arg321Ter associated with dilated cardiomyopathy leads to reduced expression and a skewed ratio of lamin A and lamin C proteins

    Energy Technology Data Exchange (ETDEWEB)

    Al-Saaidi, Rasha [Research Unit for Molecular Medicine, Aarhus University and Aarhus University Hospital, Aarhus (Denmark); Rasmussen, Torsten B. [Department of Cardiology, Aarhus University Hospital, Aarhus (Denmark); Palmfeldt, Johan [Research Unit for Molecular Medicine, Aarhus University and Aarhus University Hospital, Aarhus (Denmark); Nissen, Peter H. [Department of Clinical Biochemistry, Aarhus University Hospital, Aarhus (Denmark); Beqqali, Abdelaziz [Heart Failure Research Center, Academic Medical Center, Amsterdam (Netherlands); Hansen, Jakob [Department of Forensic Medicine, Bioanalytical Unit, University of Aarhus (Denmark); Pinto, Yigal M. [Heart Failure Research Center, Academic Medical Center, Amsterdam (Netherlands); Boesen, Thomas [Department of Molecular Biology and Genetics, University of Aarhus (Denmark); Mogensen, Jens [Department of Cardiology, Odense University Hospital, Odense (Denmark); Bross, Peter, E-mail: peter.bross@ki.au.dk [Research Unit for Molecular Medicine, Aarhus University and Aarhus University Hospital, Aarhus (Denmark)

    2013-11-15

    Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by cardiac chamber enlargement and reduced systolic function of the left ventricle. Mutations in the LMNA gene represent the most frequent known genetic cause of DCM associated with disease of the conduction systems. The LMNA gene generates two major transcripts encoding the nuclear lamina major components lamin A and lamin C by alternative splicing. Both haploinsuffiency and dominant negative effects have been proposed as disease mechanism for premature termination codon (PTC) mutations in LMNA. These mechanisms however are still not clearly established. In this study, we used a representative LMNA nonsense mutation, p.Arg321Ter, to shed light on the molecular disease mechanisms. Cultured fibroblasts from three DCM patients carrying this mutation were analyzed. Quantitative reverse transcriptase PCR and sequencing of these PCR products indicated that transcripts from the mutant allele were degraded by the nonsense-mediated mRNA decay (NMD) mechanism. The fact that no truncated mutant protein was detectable in western blot (WB) analysis strengthens the notion that the mutant transcript is efficiently degraded. Furthermore, WB analysis showed that the expression of lamin C protein was reduced by the expected approximately 50%. Clearly decreased lamin A and lamin C levels were also observed by immunofluorescence microscopy analysis. However, results from both WB and nano-liquid chromatography/mass spectrometry demonstrated that the levels of lamin A protein were more reduced suggesting an effect on expression of lamin A from the wild type allele. PCR analysis of the ratio of lamin A to lamin C transcripts showed unchanged relative amounts of lamin A transcript suggesting that the effect on the wild type allele was operative at the protein level. Immunofluorescence microscopy analysis showed no abnormal nuclear morphology of patient fibroblast cells. Based on these data, we propose that

  18. MR imaging in cardiomyopathies

    International Nuclear Information System (INIS)

    According to the WHO classification, cardiomyopathies are a group of diseases which are associated with myocardial dysfunction and can be classified either as primary or secondary cardiomyopathies. Genetic disorders have been identified in certain primary cardiomyopathies, however often the etiology remains unknown. The term ''secondary cardiomyopathy'' is used to specify diseases with the clinical indications of a cardiomyopathy, but can be attributed to a certain pathophysiological mechanism such as exposure to toxic substances, metabolic syndromes or systemic diseases. Based on morphological and functional criteria, primary cardiomyopathies are divided into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM). During the last two decades MR imaging has emerged to a well established diagnostic tool for the understanding and treatment of cardiomyopathies. Morphological and functional information can be achieved with a high level of accuracy and reproducibility. Tissue alteration of the myocardium can be detected assessing regional contrast enhancement, T1- and T2-signal intensities and chemical shift phenomena. This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies. (orig.)

  19. 心脏磁共振成像技术在扩张型心肌病中的应用及研究进展%Application and Research Progress of Cardiac Magnetic Resonance Imaging in Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    张颖雪(综述); 赵新湘; 孙勇(审校)

    2016-01-01

    扩张型心肌病是心肌病中最常见的类型,其病因复杂、病情呈进行性加重,预后较差。如何对其潜在的风险进行预测,对临床具有重要的意义,而心脏磁共振技术已成为一种有效的“一站式”心脏检查手段,可对此提供明确有效的帮助。现就多种心脏磁共振技术在扩张型心肌病中的研究进展做一综述。%Dilated cardiomyopathy is the most common type of cardiomyopathy , the cause of which is complicated , the disease is pro-gressive and the prognosis is poor .How to predict the potential risk has important significance to the clinic .Cardiac magnetic resonance ima-ging technology has become an effective “one-stop” method for the diagnosis of heart disease ,which can provide a clear and effective help . The research progress of multiple cardiac magnetic resonance imaging technology in dilated cardiomyopathy is summarized in this article .

  20. Evaluation of Proximal Aortic Elastic Properties in Non-Ischemic Dilated Cardiomyopathy by Trans-Thoracic Echocardiography: A Case-Control Study

    Directory of Open Access Journals (Sweden)

    Alireza Moaref

    2015-06-01

    Full Text Available Background: Stiffening of the large vessels, especially aorta, plays a significant role in increasing the left ventricular work and aggravating the cardiovascular diseases by attenuating the vascular wall distensibility and its buffering effect. Objectives: This study aimed to compare the stiffness of proximal aorta in the patients with established diagnosis of Non-Ischemic Dilated Cardiomyopathy (NIDC and the normal population. Patients and Methods: This case-control study aimed to compare 50 patients with established diagnosis of NIDC and 50 individuals with no history of significant cardiovascular diseases in the outpatient echocardiography clinics affiliated to Shiraz University of Medical Sciences, Shiraz, Iran during a three-month period in 2011. The patients with aortic valve disorders, coronary artery disease, congenital heart disease, diabetes mellitus, hypertension, and renal failure were excluded from the study. Stiffness indices were determined using the data gathered via echocardiography (left ventricular ejection fraction, end-systolic and end-diastolic volumes, and internal aortic diameter and blood pressure measurement. Ordinal and quantitative data were analyzed by Chi-square and independent T-test, respectively using the SPSS statistical software, version 16.0. Besides, P < 0.05 was considered as statistically significant. Results: The results of this study revealed significantly lower aortic strain (0.05 ± 0.02 in the NIDC patients versus 0.20 ± 0.46 in the controls, P < 0.001 and brachial pulse pressure (40.50 ± 9.00 in the NIDC patients versus 45.90 ± 8.08 in the controls, P < 0.001 in the NIDC patients. Conclusions: Aortic stiffness is capable of deteriorating the cardiovascular function by augmenting the afterload. Thus, it should be seriously considered as an issue while approaching NIDC patients since they demonstrate lower aortic distensibility.

  1. Relationship between myocardial extracellular space expansion estimated with post-contrast T1 mapping MRI and left ventricular remodeling and neurohormonal activation in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Ji Hyun; Son, Jung Woo; Chung, Hye Moon [Cardiology Division, Dept. of Internal Medicine, Yonsei University College of Medicine, Seoul (Korea, Republic of); and others

    2015-10-15

    Post-contrast T1 values are closely related to the degree of myocardial extracellular space expansion. We determined the relationship between post-contrast T1 values and left ventricular (LV) diastolic function, LV remodeling, and neurohormonal activation in patients with dilated cardiomyopathy (DCM). Fifty-nine patients with DCM (mean age, 55 ± 15 years; 41 males and 18 females) who underwent both 1.5T magnetic resonance imaging and echocardiography were enrolled. The post-contrast 10-minute T1 value was generated from inversion time scout images obtained using the Look-Locker inversion recovery sequence and a curve-fitting algorithm. The T1 sample volume was obtained from three interventricular septal points, and the mean T1 value was used for analysis. The N-Terminal pro-B-type natriuretic peptide (NT-proBNP) level was measured in 40 patients. The mean LV ejection fraction was 24 ± 9% and the post-T1 value was 254.5 ± 46.4 ms. The post-contrast T1 value was significantly correlated with systolic longitudinal septal velocity (s'), peak late diastolic velocity of the mitral annulus (a'), the diastolic elastance index (Ed, [E/e']/stroke volume), LV mass/volume ratio, LV end-diastolic wall stress, and LV end-systolic wall stress. In a multivariate analysis without NT-proBNP, T1 values were independently correlated with Ed (β = -0.351, p = 0.016) and the LV mass/volume ratio (β = 0.495, p = 0.001). When NT-proBNP was used in the analysis, NT-proBNP was independently correlated with the T1 values (β = -0.339, p = 0.017). Post-contrast T1 is closely related to LV remodeling, diastolic function, and neurohormonal activation in patients with DCM.

  2. Does pretreatment of bone marrow mesenchymal stem cells with 5-azacytidine or double intravenous infusion improve their therapeutic potential for dilated cardiomyopathy?

    Science.gov (United States)

    Yang, Sirui; Piao, Jinhua; Jin, Lianhua; Zhou, Yan

    2013-01-01

    Background This study was designed to investigate whether pretreatment of bone marrow mesenchymal stem cells (BMSCs) with 5-azacytidine (5-aza) or double intravenous infusion could enhance their therapeutic potential for dilated cardiomyopathy (DCM). Material/Methods BMSCs were cultured for 2 weeks in the presence or absence of 5-aza and DCM serum. The cultured BMSCs (Groups 1 and 2), 5-aza-induced BMSCs (Groups 3 and 4), and medium alone (model control) were transplanted into 80 female Wistar rats by intravenous tail vein injection. Double infusion of BMSCs with 1-day time-interval was carried out in Groups 2 and 4. Postmortem histological analysis and evaluation of heart function were performed at 4 weeks post-transplantation. Results Some transplanted BMSCs engrafted into myocardial tissue and were positive for cardiac marker troponin T. The hearts containing transplanted BMSCs secreted a larger amount of vascular endothelial growth factor. Cardiac function parameters and serum level of brain natriuretic peptide (BNP) did not differ among Groups 1, 3, and the model control. As compared with model control, BMSC transplantation in Groups 2 and 4 significantly decreased the serum level of BNP and improved cardiac contractile function, as evidenced by reduced left ventricular end-diastolic and end-systolic diameter, elevated ejection fraction, and fractional shortening. Conclusions BMSC transplantation is a promising strategy for the treatment of DCM. Pretreatment of BMSCs with 5-aza and DCM serum does not enhance their therapeutic efficacy, and the double intravenous BMSC infusion method is superior to single infusion for preserving cardiac contractile function in a rat model of DCM. PMID:23314418

  3. Relationship between cardiac {sup 123}I-Metaiodobenzylguanidine imaging and the transcardiac gradient of neurohumoral factors in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Matsui, Toshiki; Tsutamoto, Takayoshi; Kinoshita, Masahiko [Shiga Univ. of Medical Science, Otsu (Japan)

    2001-12-01

    Cardiac sympathetic nervous function is altered in congestive heart failure (CHF) and the uptake and washout rate of cardiac {sup 123}I-metaiodobenzylguanidine (MIBG) are useful markers for evaluating the severity of it. To assess what parameters predict decreased uptake or increased washout rate of MIBG, the concentrations of neurohumoral factor in both the aorta (Ao) and coronary sinus (CS) were measured, as well as hemodynamic parameters by catheterization, in patients with dilated cardiomyopathy (DCM). MIBG imaging was performed within 1 week of cardiac catheterization. Regarding MIBG parameters, the correlation with the transcardiac gradient of norepinephrine (NE), brain natriuretic peptide (BNP) and hemodynamics was investigated. Stepwise multivariate regression analysis was used to determine which variables closely correlated with cardiac MIBG parameters. There was a significant increase in the NE level between the Ao (446 pg/ml) and the CS (727 pg/ml). According to stepwise multivariate regression analysis, the heart/mediastinum (H/M) ratio independently correlated with the transcardiac gradient of BNP (r=-0.480, p<0.01), and the washout rate independently correlated with the transcardiac gradient of NE (r=0.481, p<0.01). These findings indicate that the H/M ratio may reflect the transcardiac gradient of BNP, which implies the degree of left ventricular dysfunction and/or damage and the washout rate may reflect altered cardiac sympathetic nerve terminal in DCM patients with CHF, suggesting that both the H/M ratio and washout rate provide important information about the failing ventricle. (author)

  4. Comparison of cardiac magnetic resonance imaging features of isolated left ventricular non-compaction in adults versus dilated cardiomyopathy in adults

    Energy Technology Data Exchange (ETDEWEB)

    Cheng, H. [Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Zhao, S., E-mail: cjrzhaoshihua2009@163.com [Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Jiang, S.; Lu, M.; Yan, C.; Ling, J.; Zhang, Y.; Liu, Q.; Ma, N.; Yin, G.; Wan, J. [Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Yang, Y. [Department of Cardiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Li, L. [Department of Pathology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China); Jerecic, R. [MR Research and Development, Siemens Medical Solutions, Chicago, IL (United States); He, Z. [Department of Nuclear Medicine, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037 (China)

    2011-09-15

    Aim: To compare cardiac magnetic resonance imaging (MRI) features between isolated left ventricular non-compaction (IVNC) and dilated cardiomyopathy (DCM) in adults. Materials and methods: A consecutive series of 50 patients with IVNC from a single institution were reviewed. During the same period, 50 patients with DCM who had prominent trabeculations, who were matched for age, gender, and body surface area, were prospectively included. Left ventricular (LV) morphology and function were assessed using cardiac MRI. Results: Compared with patients with DCM, patients with IVNC had a significantly lower LV sphericity index and end-diastolic volume index (LVEDVI) and a greater LV ejection fraction (LVEF), number of trabeculated segments, and ratio of non-compacted to compacted myocardium (NC/C ratio). There were no significant differences in stroke volume index, cardiac output, and cardiac index between the two patient groups. In patients with IVNC, the number of trabeculated segments and the NC/C ratio correlated positively with LVEDVI (r = 0.626 and r = 0.559, respectively) and negatively with LVEF (r = -0.647 and r = -0.521, respectively, p < 0.001 for all). In patients with DCM, the number of non-compacted segments and the NC/C ratio had no correlation with either the LVEDVI (r = -0.082 and r = -0.135, respectively) or the LVEF (r = 0.097 and r = 0.205, respectively). Conclusion: There are demonstrable morphological and functional differences between IVNC and DCM at LV assessment using cardiac MRI. The occurrence of trabeculated myocardium might be due to a different pathophysiological mechanism.

  5. 正五聚蛋白3与扩张型心肌病的研究进展%Relationship between PTX3 and Idiopathic Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    张艳春

    2013-01-01

    Myocarditis is a disease which causes inflammation of the cardiac muscle. There are two classifications of myocarditis梡rimary and secondary. Myocarditis is classed as secondary when there is a systemic disease at the time of diagnosis, otherwise it is classed as primary. Idiopathic dilated cardiomyopathy ( DCM ) accounts for 70 to 80 percent of primary myocarditis cases. Possible pathogenic factors of DCM are viral infection, autoimmune response and genetic factor. The inflammatory reaction is the critical factor in DCM. Pentranxin 3 ( PTX3 ) is a new inflammatory marker. This article examines the potential use of PTX3 in DCM.%原发性心肌病是一类原因不明、以心功能障碍为主要特征的心肌病变.扩张型心肌病约占原发性心肌病的70%~80%.目前扩张型心肌病发病机制分为三类:病毒感染、自身免疫炎症反应、遗传因素.炎症反应是介导扩张型心肌病发生的重要机制,正五聚蛋白3(PTX3)是一种新的炎症标志物,与C反应蛋白属于PTX家族.现主要针对穿透素3这种新的炎症标志物与扩张型心肌病关系的研究进展做一综述.

  6. Mitochondrial-related gene expression profiles suggest an important role of PGC-1alpha in the compensatory mechanism of endemic dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    He, Shu-Lan [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Tan, Wu-Hong, E-mail: tanwh@mail.xjtu.edu.cn [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Zhang, Zeng-Tie; Zhang, Feng [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Qu, Cheng-Juan [Institute of Biomedicine, University of Eastern Finland, Kuopio (Finland); Lei, Yan-Xia; Zhu, Yan-He [Key Laboratory of Environment and Gene Related Diseases, Xi' an Jiaotong University, Ministry Education, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Key Laboratory of Trace Elements and Endemic Diseases, Xi' an Jiaotong University, Ministry of Health, No. 76 Yanta West Road, Xi' an, Shaanxi 710061 (China); Yu, Han-Jie [Department of Biotechnology, Northwest University, Xi' an, Shaanxi 710069 (China); Xiang, You-Zhang [Shandong Institute for prevention and Treatment of Endemic Disease, Jinan, Shandong 250014 (China); and others

    2013-10-15

    Keshan disease (KD) is an endemic dilated cardiomyopathy with unclear etiology. In this study, we compared mitochondrial-related gene expression profiles of peripheral blood mononuclear cells (PBMCs) derived from 16 KD patients and 16 normal controls in KD areas. Total RNA was isolated, amplified, labeled and hybridized to Agilent human 4×44k whole genome microarrays. Mitochondrial-related genes were screened out by the Third-Generation Human Mitochondria-Focused cDNA Microarray (hMitChip3). Quantitative real-time PCR, immunohistochemical and biochemical parameters related mitochondrial metabolism were conducted to validate our microarray results. In KD samples, 34 up-regulated genes (ratios≥2.0) were detected by significance analysis of microarrays and ingenuity systems pathway analysis (IPA). The highest ranked molecular and cellular functions of the differentially regulated genes were closely related to amino acid metabolism, free radical scavenging, carbohydrate metabolism, and energy production. Using IPA, 40 significant pathways and four significant networks, involved mainly in apoptosis, mitochondrion dysfunction, and nuclear receptor signaling were identified. Based on our results, we suggest that PGC-1alpha regulated energy metabolism and anti-apoptosis might play an important role in the compensatory mechanism of KD. Our results may lead to the identification of potential diagnostic biomarkers for KD in PBMCs, and may help to understand the pathogenesis of KD. Highlights: • Thirty-four up-regulated genes were detected in KD versus health controls. • Forty pathways and four networks were detected in KD. • PGC-1alpha regulated energy metabolism and anti-apoptosis in KD.

  7. 小儿扩张型心肌病病因及临床分析%Etiology and clinical analysis of dilated cardiomyopathy in children

    Institute of Scientific and Technical Information of China (English)

    彭静; 邓淑珍; 王瑞耕; 刘玲; 孙东明; 夏琨

    2013-01-01

    目的 探讨小儿扩张型心肌病(dilated cardiomyopathy,DCM)的发病年龄、病因、心功能状况及心律失常的特点.方法 对我院2002年1月至2011年12月收治的112例DCM患儿的发病年龄、病因、临床特点进行分析,并按心功能Ⅰ、Ⅱ、Ⅲ、Ⅳ级分为4组,比较各组心律失常的发生率及类型.结果 112例DCM患儿中,<1岁确诊的52例(46.42%);诊断时有心力衰竭107例(95.53%);能明确病因的25例(22.32%),其中心肌炎16例(14.28%),家族性单纯DCM 5例(4.46%),原发性肉碱缺乏症2例(1.78%),Duchenne肌营养不良1例(0.89%),药物毒性反应(阿霉素)所致DCM 1例(0.89%);96例(85.71%)患儿出现各种心律失常.按心功能Ⅰ、Ⅱ、Ⅲ、Ⅳ级分级的4组中,各组出现1种心律失常的发生率分别为20.00%、31.25%、36.17%和42.85%,出现2种以上心律失常者的发生率分别为0、18.75%、38.29%和50.00%,出现恶性心律失常的发生率分别为0、12.50%、40.42%和67.85%.心功能Ⅱ、Ⅲ、Ⅳ级组间比较差异有统计学意义(P<0.05).结论 小儿DCM在1岁以内发病率最高;其主要病因有心肌炎、家族性、代谢缺陷及神经肌肉疾病等;心力衰竭常为患儿的首发症状及主要临床表现;DCM的心律失常发生率高且多样,常为多种心律失常并存,且心律失常的发生与患儿心功能密切相关;小儿DCM各系统栓塞罕见.%Objective To determine the etiology and clinical characteristics of children with dilated cardiomyopathy.Methods 112 children with DCM from January 2002 to December 2011 were studied.The age,cause and the clinical manifestations were analyzed.According to the children with heart function,they were divided into four groups,the incidence and type of arrhythmia were compared.Results In 112 cases of DCM in children,< 1 year old diagnosed 52 cases (46.42%),107 cases (95.53%) with heart failure,25 cases (22.32%) with chear causes,96

  8. Rescue of hereditary form of dilated cardiomyopathy by rAAV-mediated somatic gene therapy: Amelioration of morphological findings, sarcolemmal permeability, cardiac performances, and the prognosis of TO-2 hamsters

    OpenAIRE

    Kawada, Tomie; Nakazawa, Mikio; Nakauchi, Sakura; YAMAZAKI, Ken; Shimamoto, Ryoichi; Urabe, Masashi; Nakata, Jumi; Hemmi, Chieko; Masui, Fujiko; Nakajima, Toshiaki; Suzuki, Jun-ichi; Monahan, John; Sato, Hiroshi; Masaki, Tomoh; Ozawa, Keiya

    2002-01-01

    The hereditary form comprises ≈1/5 of patients with dilated cardiomyopathy (DCM) and is a major cause of advanced heart failure. Medical and socioeconomic settings require novel treatments other than cardiac transplantation. TO-2 strain hamsters with congenital DCM show similar clinical and genetic backgrounds to human cases that have defects in the δ-sarcoglycan (δ-SG) gene. To examine the long-term in vivo supplement of normal δ-SG gene driven by cytomegalovirus promoter, we analyzed the pa...

  9. Alcoholic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Gonzalo; Guzzo-Merello; Marta; Cobo-Marcos; Maria; Gallego-Delgado; Pablo; Garcia-Pavia

    2014-01-01

    Alcohol is the most frequently consumed toxic substance in the world. Low to moderate daily intake of alcohol has been shown to have beneficial effects on the cardiovascular system. In contrast, exposure to high levels of alcohol for a long period could lead to progressive cardiac dysfunction and heart failure. Cardiac dysfunction associated with chronic and excessive alcohol intake is a specific cardiac disease known as alcoholic cardiomyopathy(ACM). In spite of its clinical importance, data on ACM and how alcohol damages the heart are limited. In this review, we evaluate available evidence linking excessive alcohol consumption with heart failure and dilated cardiomyopathy. Additionally, we discuss the clinical presentation, prognosis and treatment of ACM.

  10. Assessment of central chemosensitivity and cardiac sympathetic nerve activity using I-123 MIBG imaging in central sleep apnea syndrome in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Iodine-123 m-iodobenzylguanidine (MIBG) imaging has been used to study cardiac sympathetic function in various cardiac diseases. Central sleep apnea syndrome (CSAS) occurs frequently in patients with chronic heart failure (CHF) and is reported to be associated with a poor prognosis. One of the mechanisms of its poor prognosis may be related to impaired cardiac sympathetic activity. However, the relationship between chemosensitivity to carbon dioxide, which is reported to correlate with the severity of CSAS, and cardiac sympathetic activity has not been investigated. Therefore, this study was undertaken to assess cardiac sympathetic function and chemosensitivity to carbon dioxide in CHF patients. The oxygen desaturation index (ODI) was evaluated in 21 patients with dilated cardiomyopathy (male/female: 19/2, left ventricular ejection fraction (LVEF)5 times/h underwent polysomnography. Patients with an apnea hypopnea index >15/h but without evidence of obstructive apnea were defined as having CSAS. Early (15 min) and delayed (4 hr) planar MIBG images were obtained from these patients. The mean counts in the whole heart and the mediastinum were obtained. The heart-to-mediastinum count ratio of the delayed image (H/M) and the corrected myocardial washout rate (WR) were also calculated. The central chemoreflex was assessed with the rebreathing method using a hypercapnic gas mixture (7% CO2 and 93% O2). Ten of the 21 patients had CSAS. The H/M ratio was similar in patients both with and without CSAS (1.57±0.18 vs. 1.59±0.14, p=0.82). However, the WR was higher in patients with CSAS than in patients without CSAS (40±8% vs. 30±12%, p<0.05). ODI significantly correlated with central chemosensitivity to carbon dioxide. Moreover, there was a highly significant correlation between WR and central chemosensitivity (r=0.65, p<0.05). However, there was no correlation between ODI and the WR (r=0.36, p=0.11). Cardiac sympathetic nerve activity in patients with CHF and CSAS is

  11. Monitorização eletrocardiográfica ambulatorial por 24-horas em cães com cardiomiopatia dilatada idiopática Twenty-four-hour ambulatory electrocardiographic monitoring in dogs with idiopathic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    F.L. Yamaki

    2007-12-01

    Full Text Available Caracterizou-se monitorização eletrocardiográfica ambulatorial por 24 horas (ou monitorização Holter em cães com cardiomiopatia dilatada idiopática, visando principalmente à detecção de arritmias ventriculares não detectadas pela eletrocardiografia convencional (de repouso. Para tanto, avaliaram-se 40 pacientes com diagnóstico de cardiomiopatia dilatada idiopática, por meio de exame físico e mensuração indireta da pressão arterial, além de exames eletrocardiográfico, ecocardiográfico, radiográfico de tórax e da monitorização Holter. Extra-sístoles ventriculares foram detectadas, por monitorização Holter, em 97,5% dos animais e taquicardia ventricular, em 45%. Não houve correlação entre o número de extra-sístoles ventriculares e a fração de encurtamento. Considerando as manifestações clínicas, apenas houve associação entre presença de taquicardia ventricular e histórico de síncopes. Conclui-se que a incidência de arritmias ventriculares em cães com cardiomiopatia dilatada idiopática é bastante alta, sendo a taquicardia ventricular relativamente freqüente, ocorrendo mais sob a forma não sustentada.This study aimed to characterize 24-hour ambulatory electrocardiographic monitoring (Holter monitoring in dogs with idiopathic dilated cardiomyopathy. Physical examination and indirect (Doppler blood pressure measurement, and also electrocardiography, thoracic radiography, echocardiography, and 24-hour ambulatory electrocardiographic exams were performed in 40 dogs with idiopathic dilated cardiomyopathy. Ventricular extrasystoles were detected in 97.5% of the animals, and ventricular tachycardia in 45%. No correlation between the number of ventricular extrasystoles and the shortening fraction was observed. Concerning the clinical symptoms, there was only association between the presence of ventricular tachycardia and past report of syncope. It was concluded that the incidence of ventricular arrhythmias is

  12. DNA analysis in inherited cardiomyopathies : Current status and clinical relevance

    NARCIS (Netherlands)

    Van Spaendonck-Zwarts, Karin Y.; Van den Berg, Maarten P.; Van Tintelen, J. Peter

    2008-01-01

    Most hypertrophic cardiomyopathies and a subset of dilated and arrhythmogenic right ventricular cardiomyopathies are familial diseases. They generally show an autosomal dominant pattern of inheritance and have underlying mutations in genes encoding sarcomeric, cytoskeletal, nuclear envelope, and des

  13. 多普勒超声心动图评价小儿扩张型心肌病%Evaluation of Doppler echocardiograms on dilated cardiomyopathy in children English Column

    Institute of Scientific and Technical Information of China (English)

    梁光明

    2002-01-01

    Objective To evaluate the appraisal of Doppler echocardiograms on dilated cardiomyopathy(DCM) in children. Methods 26 patients with DCM were tested by Doppler echocardiograms. The left ventricular diastolic function, systolic function, and pulmonary artery systolic pressure (PASP)were tested before and after treatment. Results After treatment , the systolic function still changed, but situation got well obviously. Diastolic function was mostly false normal type (52.6% ), 3 patients returned to normal. Each index parameter (except E/A) of diastolie function was much different from the normal value(P<0.001). Serious continuous pulmonary hypertension is a prognosticate index for the children with DCM .Conclusions Doppler echocardiograms can be used to assess myocardium rehabilitation for the children with DCM.

  14. Restrictive cardiomyopathy

    Science.gov (United States)

    Cardiomyopathy - restrictive; Infiltrative cardiomyopathy ... In a case of restrictive cardiomyopathy, the heart muscle is normal size or slightly enlarged. Most of the time, it also pumps normally. However, it does not ...

  15. Charcot-Marie-Tooth disease and dilated cardiomyopathy. A rare combination. Enfermedad de Charcot-Marie-Tooth y miocardiopatía dilatada. Una rara asociación.

    Directory of Open Access Journals (Sweden)

    Rafael Pila Pérez

    2011-07-01

    Full Text Available

    Se presenta el caso de un paciente de 50 años de edad, con 14 años de evolución de manifestaciones clínicas, destacándose las alteraciones musculoesqueléticas de los cuatro miembros con atrofia de las prominencias tenar e hipotenar y de la musculatura de ambas piernas. Se destacó la presencia de alteraciones sensitivas en miembros inferiores con distribución en calcetín, atrofia, atonía, arreflexia y marcha equina. Desde el punto de vista cardiaco, el paciente presentaba un fibriloaleteo. La radiografía de tórax mostró un aumento marcado del área cardiaca y la ecocardiografía puso de manifiesto una miocardiopatía dilatada. El estudio histopatológico confirmó la presencia de la enfermedad de Charcot-Marie-Tooth asociada a miocardiopatía dilatada. El diagnóstico se basó en las características clínicas, la velocidad de conducción motora, y el estudio histopatológico, que demostró desmielinización con lesiones en “cebolla”, si bien faltaron los estudios genéticos. La enfermedad de Charcot-Marie-Tooth es una enfermedad rara; aproximadamente un 60 % de los pacientes que la padecen, son portadores de una duplicación del cromosoma 17. Por ello, se consideró oportuno transmitir la experiencia de este caso.

    The case of a 50 years old male patient is presented. Along 14 years of clinical evolution, four limbs musculoskeletal disorders with atrophy of the thenar and hypothenar prominences and muscles of both legs had been emphasized. The presence of sensory impairment in lower limbs with stocking distribution, atrophy, weakness, areflexia and equine gait were very peculiar in this case. From the cardiac point of view, the patient presented a fibrillation/flutter. Chest radiography showed a marked increase in the cardiac area and echocardiography revealed dilated cardiomyopathy. Histopathological examination confirmed the presence of Charcot-Marie-Tooth disease associated with dilated cardiomyopathy. While genetic

  16. O uso da L-carnitina como adjuvante no tratamento da miocardiopatia dilatada em criança com Aids Usage of L-carnitine as adjuvant in the treatment of dilated cardiomyopathy in a child with Aids

    Directory of Open Access Journals (Sweden)

    Lourdes Zélia Zanoni

    2011-06-01

    Full Text Available OBJETIVO: Apresentar a resposta cardiovascular à L-carnitina de um paciente com insuficiência cardíaca congestiva decorrente de miocardiopatia dilatada pelo vírus da imunodeficiência humana. DESCRIÇÃO DO CASO: Criança com quadro clínico de insuficiência cardíaca congestiva grave devido à miocardiopatia dilatada pela síndrome de imunodeficiência adquirida. O tratamento para as manifestações clínicas foi instituído, com pouca resposta clínica. Com objetivo de melhorar o desempenho energético/metabólico dos cardiomiócitos, foi instituída terapia com L-carnitina. Observou-se significativa melhora clínica do paciente, em relação ao desempenho cardíaco, mesmo antes do início do tratamento com os fármacos antirretrovirais. COMENTÁRIOS: A L-carnitina é um composto que facilita o transporte dos ácidos graxos de cadeia longa para dentro da mitocôndria. Nesse caso, o uso da L-carnitina parece ser clinica e bioquimicamente justificado.OBJECTIVE: To present the cardiovascular response to L-carnitine of a patient with congestive heart failure caused by dilated cardiomyopathy and human immunodeficiency virus. CASE DESCRIPTION: Child with a clinical history of severe congestive heart failure due to dilated cardiomyopathy caused by acquired immunodeficiency syndrome. The treatment for the symptoms resulted in a poor clinical response. In order to improve the energetic performance/metabolism of cardiomyocytes, therapy with L-carnitine was established. There was significant clinical improvement of the cardiac performance of the patient, even before starting the treatment with antiretroviral drugs. COMMENTS: L-carnitine is a compound that facilitates the transport of long-chain fatty acids into the mitochondria. In this case the administration of L-carnitine appears to be clinically and biochemical justified.

  17. An acute coronary intervention to an AMI patient caused by embolism with dilated cardiomyopathy%栓塞致扩张型心肌病患者 AMI 急诊干预一例

    Institute of Scientific and Technical Information of China (English)

    敬锐; 林文华

    2013-01-01

    Acute coronary embolism caused by acute myocardial infarction ( AMI ) is common in clinical work However , it often be misdiagnosed due to its non-specific clinical symptoms.The most frequently misdiagnosis is ischemic heart disease complicated AMI.In this article, we described an old female patient , who has an clinic history of dilated cardiomyopathy and atrial fibrillation , and meet an acute chest pain which is diagnosed non-ST-elevator myocardial infarction ( NSTEMI ).She was diagnosed as ischemic heart disease combined with AMI at first time.Then, she was diagnosed as Dilated cardiomyopathy with acute coronary artery thrombosis after the coronary intervention.We further analyzed and elaborated how to identify and intervene this class of patients.The case prompted the emergency interventional doctor should take account the possibility of coronary embolism when meet an AMI patient with high risks of embolism.%冠状动脉急性栓塞导致的AMI在临床工作中并不少见,但常因临床症状的非特异性而导致误诊,最常误诊为缺血性心肌病合并AMI。该文报道1例因突发胸痛就诊于急诊的老年女性,急性非ST段抬高型心肌梗死( NSTEMI )诊断明确,合并心脏扩大、心房颤动。初诊为缺血性心肌病合并AMI,后经急诊介入干预,修正诊断为扩张型心肌病合并冠状动脉急性血栓栓塞。并根据其临床及介入干预特点,对该类患者如何识别及如何干预进行了分析和阐述。该例提示急诊介入医生,对于栓塞高危患者发生AMI时,应考虑到冠状动脉栓塞的可能性。

  18. 疑似扩张型心肌病的遗传性出血性毛细血管扩张症%Hereditary Hemorrhagic Telangiectasis in the Similitude of Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    周国; 王磌; 张奇志; 张迅英

    2012-01-01

    Objective To explore the clinical characteristics of hereditary hemorrhagic telangiectasis (HHT) in order to improve the diagnosis rate. Methods Retrospective analysis of one case of HHT suspected as dilated cardiomyopathy was made. Results The patient was admitted for convulsivum dyspnea, short breath for five years and aggravation for nine days. The patient was diagnosed as having heart failure in a local hospital, and was treated with diuretics and the symptom was temporarily relieved. The patient had a long history of spontaneous rhinorrhagia and anaemia, and manifested telangiectasis in skin and mucous membrane. The patient was diagnosed as having HHT combined with cardiac dysfunction concomitantly by the results of laboratory examination, ECG, UCG and CTA. She was discharged after proper treatment. Conclusion Clinicians should caution against HHT in patients with clinical manifestations of suspected dilated cardiomyopathy, accompanied with chronic hemorrhage, telangiectasis in skin and mucous membrane and internal organ injuries.%目的 探讨遗传性出血性毛细血管扩张症(hereditary hemorrhagic telangiectasis,HHT)的临床特点,以提高诊断率.方法 回顾分析1例疑似扩张型心肌病的HHT临床资料.结果 本例因阵发性胸闷、气短5年,加重9d人院.当地医院曾诊断为心力衰竭予利尿剂治疗,症状可暂时缓解.患者有长期自发性鼻出血史及贫血史,皮肤、黏膜有毛细血管扩张表现,结合实验室、心电图、超声及肺动脉血管造影检查结果,确诊为HHT并心功能不全.予相应治疗后病情明显好转出院.结论 对临床表现类似扩张型心肌病,且有慢性出血、皮肤黏膜毛细血管扩张及内脏损害者,应警惕HHT可能,及时行相关检查.

  19. Therapeutic effect of co-enzyme Q10 on idiopathic dilated cardiomyopathy: assessment by iodine-123 labelled 15-(p-iodophenyl)-3(R,S)-methylpentadecanoic acid myocardial single-photon emission tomography

    International Nuclear Information System (INIS)

    It has been reported that myocardial mitochondrial function can be improved by the administration of co-enzyme Q10 (CoQ10). Recently, iodine-123 labelled 15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) was developed for metabolic imaging using single-photon emission tomography (SPET). This study was conducted to determine whether the therapeutic effects of CoQ10 on idiopathic dilated cardiomyopathy can be evaluated by BMIPP myocardial SPET. Fifteen patients, comprising 14 men and one woman (mean age: 64±12 years), were examined. CoQ10 was administered at 30 mg/day for a period of 35.7±12.4 days. BMIPP myocardial SPET was carried out before and after CoQ10 treatment. The count ratio of the heart (H) to the upper mediastinum (M) (H/M ratio) was calculated using a region of interest method with anterior planar imaging. Representative short-axis tomograms were divided into 27 segments (three slices x nine segments). Each segmental score was analysed semiquantitatively using a four-point scoring system (normal=0, mild low uptake=1, severe low uptake=2, defect=3). The H/M ratio showed a significant improvement, from 2.39±0.39 to 2.54±0.47, after treatment (P<0.05). The BMIPP total defect score after CoQ10 treatment was significantly decreased to 10.1±4.3, compared to 13.9±4.5 without CoQ10 treatment (P<0.001). However, the percent fractional shortening measured using echocardiography was not significantly different before and after CoQ treatment (19.2±8.1 vs 19.7±7.1). BMIPP myocardial SPET was confirmed to be sensitive in evaluating the therapeutic effects of CoQ10 in patients with idiopathic dilated cardiomyopathy. This method is unique, since the therapeutic effects can be estimated from the perspective of metabolic SPET imaging. (orig.). With 5 figs., 1 tab

  20. Loss of αT-catenin alters the hybrid adhering junctions in the heart and leads to dilated cardiomyopathy and ventricular arrhythmia following acute ischemia.

    Science.gov (United States)

    Li, Jifen; Goossens, Steven; van Hengel, Jolanda; Gao, Erhe; Cheng, Lan; Tyberghein, Koen; Shang, Xiying; De Rycke, Riet; van Roy, Frans; Radice, Glenn L

    2012-02-15

    It is generally accepted that the intercalated disc (ICD) required for mechano-electrical coupling in the heart consists of three distinct junctional complexes: adherens junctions, desmosomes and gap junctions. However, recent morphological and molecular data indicate a mixing of adherens junctional and desmosomal components, resulting in a 'hybrid adhering junction' or 'area composita'. The α-catenin family member αT-catenin, part of the N-cadherin-catenin adhesion complex in the heart, is the only α-catenin that interacts with the desmosomal protein plakophilin-2 (PKP2). Thus, it has been postulated that αT-catenin might serve as a molecular integrator of the two adhesion complexes in the area composita. To investigate the role of αT-catenin in the heart, gene targeting technology was used to delete the Ctnna3 gene, encoding αT-catenin, in the mouse. The αT-catenin-null mice are viable and fertile; however, the animals exhibit progressive cardiomyopathy. Adherens junctional and desmosomal proteins were unaffected by loss of αT-catenin, with the exception of the desmosomal protein PKP2. Immunogold labeling at the ICD demonstrated in the αT-catenin-null heart a preferential reduction of PKP2 at the area composita compared with the desmosome. Furthermore, gap junction protein Cx43 was reduced at the ICD, including its colocalization with N-cadherin. Gap junction remodeling in αT-catenin-knockout hearts was associated with an increased incidence of ventricular arrhythmias after acute ischemia. This novel animal model demonstrates for the first time how perturbation in αT-catenin can affect both PKP2 and Cx43 and thereby highlights the importance of understanding the crosstalk between the junctional proteins of the ICD and its implications for arrhythmogenic cardiomyopathy.

  1. Dystrophin-Deficient Cardiomyopathy.

    Science.gov (United States)

    Kamdar, Forum; Garry, Daniel J

    2016-05-31

    Dystrophinopathies are a group of distinct neuromuscular diseases that result from mutations in the structural cytoskeletal Dystrophin gene. Dystrophinopathies include Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy, as well as DMD and BMD female carriers. The primary presenting symptom in most dystrophinopathies is skeletal muscle weakness. However, cardiac muscle is also a subtype of striated muscle and is similarly affected in many of the muscular dystrophies. Cardiomyopathies associated with dystrophinopathies are an increasingly recognized manifestation of these neuromuscular disorders and contribute significantly to their morbidity and mortality. Recent studies suggest that these patient populations would benefit from cardiovascular therapies, annual cardiovascular imaging studies, and close follow-up with cardiovascular specialists. Moreover, patients with DMD and BMD who develop end-stage heart failure may benefit from the use of advanced therapies. This review focuses on the pathophysiology, cardiac involvement, and treatment of cardiomyopathy in the dystrophic patient. PMID:27230049

  2. Arrhythmogenic cardiomyopathy.

    Science.gov (United States)

    Pilichou, Kalliopi; Thiene, Gaetano; Bauce, Barbara; Rigato, Ilaria; Lazzarini, Elisabetta; Migliore, Federico; Perazzolo Marra, Martina; Rizzo, Stefania; Zorzi, Alessandro; Daliento, Luciano; Corrado, Domenico; Basso, Cristina

    2016-01-01

    -functional abnormalities. The main differential diagnoses are idiopathic right ventricular outflow tract tachycardia, myocarditis, sarcoidosis, dilated cardiomyopathy, right ventricular infarction, congenital heart diseases with right ventricular overload and athlete heart. A positive genetic test in the affected AC proband allows early identification of asymptomatic carriers by cascade genetic screening of family members. Risk stratification remains a major clinical challenge and antiarrhythmic drugs, catheter ablation and implantable cardioverter defibrillator are the currently available therapeutic tools. Sport disqualification is life-saving, since effort is a major trigger not only of electrical instability but also of disease onset and progression. We review the current knowledge of this rare cardiomyopathy, suggesting a flowchart for primary care clinicians and geneticists. PMID:27038780

  3. 血管紧张素转换酶抑制剂和血管紧张素受体拮抗剂对扩张型心肌病内皮功能的影响%Effects of Angiotensin Converting Enzyme Inhibitor or Angiotensin Receptor Blocker to Endothelium Function in Idiopathic Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    陈嫦娥

    2014-01-01

    目的:探讨血管紧张素转换酶抑制剂(angiotension converting enzyme inhibitor,ACEI)达爽和血管紧张素受体拮抗剂(angiotension receptor blocker,ARB)安博维对扩张型心肌病患者内皮功能的影响。方法:采用超声肱动脉内径测量法,对20例正常人和24例扩张型心肌病患者服用达爽或安博维治疗前后肱动脉内径在反应性充血后和含服硝酸甘油后的百分变化率进行比较,分析ACE-I和ARB对扩张型心肌病内皮功能的影响。结果:治疗前扩张型心肌病患者肱动脉内径在反应性充血前后变化较小,但与对照组比较差异均有统计学意义(P0.05)。结论:ACE-I和ARB均可改善扩张型心肌病的内皮功能,两者在治疗效果上差异不明显。%Objective:To evaluate the effects of angiotension converting enzyme inhibitor(ACEI) imidapril or angiotension receptor blocker (ARB)irbesartan on the endothelial function in patients with idiopathic dilated cardiomyopathy by transthoracic echocardiography.Method:Twenty-four patients with idiopathic dilated cardiomyopathy and 20 healthy subjects were included in this study. Brachial artery endothelium dependent dilation function [flow-mediated dilation(FMD)] and nitroglycerin-induced percent changes in the brachial artery diameter were measured by two-dimensional ultrasound before and after the treatment of imidapril or irbesartan and the effect of ACE-I and ARB on endothelial function in dicated cordiomyopathy were analyzed. Result:The treatment of dilated cardiomyopathy patients in the brachial artery diameter had smaller changes before and after the reactive hyperemia,but with the control group had significant difference(P0.05).Conclusion:ACE-I and ARB can improve the endothelial function in patients with dilated cardiomyopathy,the treatment effect is not obvious difference.

  4. Hipotiroidismo, miocardiopatía dilatada y síndrome nefrótico durante el embarazo Hypothyroidism, dilated cardiomyopathy and nephrotic syndrome during pregnancy

    Directory of Open Access Journals (Sweden)

    Ariel K. Saad

    2011-02-01

    Full Text Available El hipotiroidismo en el embarazo es infrecuente, pero cuando ocurre suele asociarse con complicaciones maternas y fetales. Se presenta el caso de una mujer joven sin antecedentes de enfermedad cardiovascular que consulta por ortopnea, dolor torácico y edema de miembros inferiores. Los exámenes pusieron en evidencia la existencia de insuficiencia cardíaca, hipotiroidismo, síndrome nefrótico e insuficiencia renal. El eco-Doppler mostró dilatación de las cuatro cavidades cardíacas con deterioro grave de la función sistólica. El tratamiento con levotiroxina por vía intravenosa mejoró el cuadro clínico y los parámetros de laboratorio. Se analizan los efectos de la hormona tiroidea sobre el aparato cardiovascular y se comentan los mecanismos fisiopatológicos de la insuficiencia cardíaca en el embarazo.Hypothyroidism during pregnancy is infrequent, but its presence is associated with maternal and fetal complications. We present the case of a young pregnant woman with no previous history of cardiovascular disease, who consulted for orthopnea, chest pain and edema in both legs. Laboratory tests demonstrated a hypothyroid condition and a nephrotic syndrome with renal failure. The echo-Doppler exam showed a four chamber dilatation with systolic dysfunction. Treatment with intravenous levothyroxine improved her medical condition. We analyze the effects of thyroid hormone on the heart and vascular system and discuss the pathophysiologic mechanisms of heart failure during pregnancy.

  5. Como o ecocardiograma pode ser útil em predizer a morte em crianças com cardiomiopatia dilatada idiopática? How can the echocardiogram be useful for predicting death in children with idiopathic dilated cardiomyopathy?

    Directory of Open Access Journals (Sweden)

    Vitor Manuel Pereira Azevedo

    2004-06-01

    Full Text Available OBJETIVO: Determinar os fatores preditores ecocardiográficos de morte em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo retrospectivo de 148 crianças com cardiomiopatia dilatada idiopática diagnosticadas entre setembro/1979 a março/2003. Critérios para inclusão: insuficiência cardíaca e redução da contratilidade no ecocardiograma, na ausência de cardiopatia congênita ou secundária. Foram analisados 470 exames, durante 244,8 meses de evolução. Parâmetros analisados: dimensão do átrio esquerdo (DAE, relação átrio esquerdo/aorta (DAE/Ao, dimensão sistólica (DSVE e diastólica (DDVE do VE, massa do VE (massa, dimensão do ventrículo direito (DVD, fração de ejeção do VE (FE, percentagem de encurtamento do VE (%EC, gravidade da insuficiência das valvas atrioventriculares e da valva pulmonar e medida da pressão sistólica (PsVD e diastólica (PdVD do VD. Foi considerado significativo erro alfa OBJECTIVE: To determine the echocardiographic predicting factors of death in children with idiopathic dilated cardiomyopathy. METHODS: A retrospective study of 148 children with idiopathic dilated cardiomyopathy diagnosed between September 1979 and March 2003 was carried out. The inclusion criteria were as follows: heart failure and a reduction in contractility on the echocardiogram in the absence of congenital or secondary heart disease. Four hundred and seventy examinations during a period of 244.8 months of evolution were analyzed. The following parameters were assessed: left atrial dimension (LAD; left atrium/aorta ratio (LAD/Ao; left ventricular systolic (LVSD and diastolic (LVDD dimensions; left ventricular mass (LVmass; right ventricular dimension (RVD; left ventricular ejection fraction (LVEF; left ventricular shortening fraction (% SH; severity of the insufficiency of the atrioventricular and pulmonary valves; and right ventricular systolic (RVSP and diastolic (RVDP pressures. The significance level

  6. Usefulness of {sup 123}I-metaiodobenzylguanidine myocardial scintigraphy for predicting the effectiveness of {beta}-blockers in patients with dilated cardiomyopathy from the standpoint of long-term prognosis

    Energy Technology Data Exchange (ETDEWEB)

    Fujimoto, Shinichiro; Inoue, Aritomo; Hisatake, Shinji; Yamashina, Shohei; Yamashina, Hisayo; Nakano, Hajime; Yamazaki, Junichi [Toho University School of Medicine, Division of Cardiovascular Medicine, Department of Internal Medicine, Ohmori Hospital, Tokyo (Japan)

    2004-10-01

    The usefulness of {sup 123}I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy in predicting the effectiveness of {beta}-blocker therapy in dilated cardiomyopathy (DCM) was investigated from the standpoint of long-term prognosis. The subjects were 53 DCM patients in whom {beta}-blockers had been successfully introduced and used for 6 months or longer. When symptoms were stable before the introduction of {beta}-blockers and for up to 1 year thereafter, MIBG myocardial single-photon emission computed tomography was performed and the images analysed to obtain the extent score (EXT), severity score (SEV) and washout rate (WR). At the same time, echocardiography was performed to measure left ventricular ejection fraction (LVEF). Thereafter, patients were placed under observation for an average of 1,314{+-}986 days, with the occurrence of cardiac events as the endpoint. The degree of improvement in WR after introduction of {beta}-blockers was a significant predictor of cardiac events. In fact, none of the patients whose improvement in WR was valued at 10 or higher had cardiac events. Accordingly, using improvement in WR of 10 as the cut-off value, the patients were divided into two groups, ''improved'' and ''unimproved''. There were significant differences between the groups in respect of early EXT, early SEV and WR before the introduction of {beta}-blockers. As regards predictors of WR improvement, multivariate logistic regression analysis demonstrated that early EXT, WR and LVEF were significant predictors. This study shows that, from the standpoint of long-term prognosis, DCM patients who would benefit the most from {beta}-blocker therapy are those with low early EXT and early SEV and high WR before {beta}-blocker introduction regardless of LVEF values. (orig.)

  7. Survival and echocardiographic evaluation of dogs with idiopathic dilated cardiomyopathy treated with carvedilol Avaliação ecocardiográfica e de sobrevida de cães com cardiomiopatia dilatada idiopática tratados com carvedilol

    Directory of Open Access Journals (Sweden)

    E.C. Soares

    2010-06-01

    Full Text Available Sixty dogs with idiopathic dilated cardiomyopathy were randomly treated with traditional therapy - digitalis, diuretics, angiotensin-converting inhibitors - (group A or treated with these drugs plus carvedilol (group B. Echocardiographic variables were measured before and after 3, 13, 26, and 52 weeks of treatment or until death. Comparisons between groups and time were performed. No significant differences between groups were found in the most of the echocardiographic variables. The left ventricular end-systolic diameter indexed to body surface area (LVESDi increased significantly in the group A dogs compared to the group B animals. The survival of groups A and B dogs were not different (P-value=0.1137. In conclusion, the stability of the LVESDi observed in the group treated with carvedilol may represent the beneficial effect over the ventricular remodeling.Sessenta cães com cardiomiopatia dilatada idiopática receberam, aleatoriamente, tratamento convencional - digitálicos, diuréticos, inibidores da enzima conversora de angiotensina - (grupo A ou esses fármacos mais carvedilol (grupo B. As variáveis ecocardiográficas foram avaliadas antes e depois de três, 13, 26 e 52 semanas de tratamento ou até o óbito. Não foram encontradas diferenças significativas entre os grupos de animais quanto à maioria das variáveis ecocardiográficas. O diâmetro sistólico final do ventrículo esquerdo indexado à superfície corpórea (DSVEi aumentou de forma significativa no grupo A quando comparado ao grupo B. Não se observou diferença na sobrevida dos grupos A e B (P=0,1137. Concluiu-se que a estabilização do DSVEi no grupo tratado com carvedilol pode representar o efeito benéfico deste fármaco sobre o remodelamento ventricular.

  8. Evolving Approaches to Genetic Evaluation of Specific Cardiomyopathies.

    Science.gov (United States)

    Teo, Loon Yee Louis; Moran, Rocio T; Tang, W H Wilson

    2015-12-01

    The understanding of the genetic basis of cardiomyopathy has expanded significantly over the past 2 decades. The increasing availability, shortening diagnostic time, and lowering costs of genetic testing have provided researchers and physicians with the opportunity to identify the underlying genetic determinants for thousands of genetic disorders, including inherited cardiomyopathies, in effort to improve patient morbidities and mortality. As such, genetic testing has advanced from basic scientific research to clinical application and has been incorporated as part of patient evaluations for suspected inherited cardiomyopathies. Genetic evaluation framework of inherited cardiomyopathies typically encompasses careful evaluation of family history, genetic counseling, clinical screening of family members, and if appropriate, molecular genetic testing. This review summarizes the genetics, current guideline recommendations, and evidence supporting the genetic evaluation framework of five hereditary forms of cardiomyopathy: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM), and left ventricular noncompaction (LVNC). PMID:26472190

  9. Importance of genetic evaluation and testing in pediatric cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Muhammad; Tariq; Stephanie; M; Ware

    2014-01-01

    Pediatric cardiomyopathies are clinically heterogeneous heart muscle disorders that are responsible for significant morbidity and mortality. Phenotypes include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, left ventricular noncompaction and arrhythmogenic right ventricular cardiomyopathy. There is substantial evidence for a genetic contribution to pediatric cardiomyopathy. To date, more than 100 genes have been implicated in cardiomyopathy, but comprehensive genetic diagnosis has been problematic because of the large number of genes, the private nature of mutations, and difficulties in interpreting novel rare variants. This review will focus on current knowledge on the genetic etiologies of pediatric cardiomyopathy and their diagnostic relevance in clinical settings. Recent developments in sequencing technologies are greatly impacting the pace of gene discovery and clinical diagnosis. Understanding the genetic basis for pediatric cardiomyopathy and establishing genotypephenotype correlations may help delineate the molecular and cellular events necessary to identify potential novel therapeutic targets for heart muscle dysfunction in children.

  10. 扩张型心肌病患者血尿酸水平与心房颤动的关系%Correlation between uric acid and atrial fibrillation in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    甘华文; 唐其柱; 周恒; 袁园; 纵静

    2012-01-01

    Objective To investigate the association between uric acid and atrial fibrillation in patients with dilated cardiomyopathy.Methods Patients with dilated cardiomyopathy were screened.Age,serum uric acid,NYHA classes,left atrial dimension (LAD),left ventricular end-diastolic dimension (LVEDD),left ventricular ejection fraction (LVEF),interventricular septal thickness (IVST) and left ventricular posterior wall thickness( LVPWT) were compared between the dilated cardiomyopathy patients with atrial fibrillation and those without atrial fibrillation.The incidence of atrial fibrillation was compared between elevated uric acid level patients and normal ones.The effect of serum uric acid on the initiation and perpetuation of atrial fibrillation was also analyzed.Results After univariate analysis,age [ (64 ± 13 )years vs (59 ± 15 )years,P < 0.05 ],uric acid [ (491 ± 135 )μmol/L vs (424 ±121 ) μmol/L,P < 0.01 ],LAD [ (48 ± 8 ) mm vs (43 ± 5 ) mm,P < 0.01 ] were significantly increased in patients with atrial fibrillation compared with non-atrial fibrillation patients.There was a higher incidence of atrial fibrillation in the elevated uric acid group than in the normal uric acid group[ 34.1% (30/88) vs 20.2% (18/89),P <0.05].After multivariate logistic regression analysis,the independent predictors of atrial fibrillation were uric acid ( OR:1.003,95 % CI:1.000-1.006,P < 0.05 ) and LAD ( OR:1.147,95 % CI:1.076-1.224,P < 0.01 ).Conclusion There is an independent association between elevated serum uric acid levels and atrial fibrillation in patients with DCM,and the uric acid levels might be a useful predictor of atrial fibrillation.%目的 探讨扩张型心肌病(DCM)患者血尿酸水平与心房颤动(房颤)发生的关系以及临床意义.方法 回顾性分析2010年1月至2011年12月在我院住院治疗的DCM患者的临床资料,比较房颤组(48例)与非房颤组(129例)年龄、心功能分级、血尿酸水平、左心房内径、左

  11. Calcium Ions in Inherited Cardiomyopathies.

    Science.gov (United States)

    Deftereos, Spyridon; Papoutsidakis, Nikolaos; Giannopoulos, Georgios; Angelidis, Christos; Raisakis, Konstantinos; Bouras, Georgios; Davlouros, Periklis; Panagopoulou, Vasiliki; Goudevenos, John; Cleman, Michael W; Lekakis, John

    2016-01-01

    Inherited cardiomyopathies are a known cause of heart failure, although the pathways and mechanisms leading from mutation to the heart failure phenotype have not been elucidated. There is strong evidence that this transition is mediated, at least in part, by abnormal intracellular Ca(2+) handling, a key ion in ventricular excitation, contraction and relaxation. Studies in human myocytes, animal models and in vitro reconstituted contractile protein complexes have shown consistent correlations between Ca(2+) sensitivity and cardiomyopathy phenotype, irrespective of the causal mutation. In this review we present the available data about the connection between mutations linked to familial hypertrophic (HCM), dilated (DCM) and restrictive (RCM) cardiomyopathy, right ventricular arrhythmogenic cardiomyopathy/dysplasia (ARVC/D) as well as left ventricular non-compaction and the increase or decrease in Ca(2+) sensitivity, together with the results of attempts to reverse the manifestation of heart failure by manipulating Ca(2+) homeostasis. PMID:26411603

  12. Morphometric Documentation of a High Prevalence of Left Ventricular Dilated Cardiomyopathy in Both Clinically Normal and Cyanotic Mature Commercial Broiler Breeder Roosters with Comparisons to Market-Age Broilers.

    Science.gov (United States)

    Wilson, Floyd D; Magee, Danny L; Jones, Kelli H; Baravik-Munsell, Erica; Cummings, Timothy S; Wills, Robert W; Pace, Lanny W

    2016-09-01

    Previous studies documented the common occurrence of transitory cyanosis and echocardiographic aortic insufficiency in mature commercial broiler breeder roosters. During further investigations, we observed a high prevalence of hearts exhibiting extensive dilation of the left ventricle chamber compatible with dilated left ventricular cardiomyopathy present in both cyanotic and normal subpopulations. We conducted quantitative studies focused on documentation of cardiac ventricle parameters by using simple gross morphometric methods performed on formalin-fixed hearts obtained from both clinically normal roosters and those exhibiting variable transitory cyanosis, echocardiographic aortic insufficiency, or both. A high prevalence of often dramatic left ventricular dilation reflected in enlarged left ventricular chamber areas and elevated left ventricle-to-total ventricle area ratios was morphometrically documented. However, no statistically significant differences in the occurrence of ventricular abnormalities were observed between normal and cyanotic roosters. Age-associated changes were also demonstrated by comparative morphometric studies on hearts from normal market-age broilers (average age of 7 wk) and those of mature roosters (average age of 42 wk). Elevation in both left and right ventricular weight-to-total heart weight ratios dramatically increased with aging. In addition, values (average ± SD) for the left ventricle chamber area-to-total ventricle area ratios increased from 3.2 ± 2.0% in broilers up to 10.0 ± 8.8% in roosters. None of the normal broilers studied demonstrated left ventricular volume ratios above 10%, whereas 33% of the roosters had left ventricular volume ratios above 10%, including 13% with ratios of 20% or higher. However, the left ventricle wall area-to-body weight ratios were much closer for the two age groups (0.85 ± 0.18 cm(2)/kg in broilers and 0.79 ± 0.13 cm(2)/kg in roosters). Also, the standard right ventricle-to-total ventricle

  13. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Wiese, Signe; Hove, Jens D; Bendtsen, Flemming;

    2014-01-01

    biomarkers could improve the diagnostic assessm+ent. Cirrhotic cardiomyopathy contributes to various complications in cirrhosis, especially as an important factor in the development of hepatic nephropathy. Additionally, cirrhotic cardiomyopathy seems to be associated with the development of heart failure...... in relation to invasive procedures such as shunt insertion and liver transplantation. Current pharmacological treatment is nonspecific and directed towards left ventricular failure, and liver transplantation is currently the only proven treatment with specific effect on cirrhotic cardiomyopathy....

  14. Tratamento de insuficiência cardíaca com benazepril em cães com cardiomiopatia dilatada e endocardiose Treatment of congestive heart failure with benazepril in dogs with dilated cardiomyopathy and endocardiosis

    Directory of Open Access Journals (Sweden)

    P.M. Pereira

    2005-09-01

    Full Text Available Foram avaliados os efeitos clínicos do benazepril, um inibidor da enzima de conversão da angiotensina de ação prolongada, em cães com insuficiência cardíaca congestiva (ICC secundária à endocardiose de mitral ou cardiomiopatia dilatada. O medicamento foi administrado na dose de 0,25 a 0,5mg/kg/dia. Diuréticos, digitálicos e f��rmacos antiarrítmicos foram usados de acordo com a necessidade de cada paciente. Exames físico, radiográfico e eletrocardiográfico foram realizados nos dias 0, 7, 28 e 56. A gasometria arterial e a bioquímica sérica foram avaliadas nos dias 0 e 56. Os sinais de dispnéia e o estado geral dos pacientes melhoraram em todos os cães após o início do tratamento. Houve diminuição na freqüência da tosse e não houve alterações no eletrocardiograma, exceto pela diminuição na amplitude e na duração da onda P. Nenhum efeito colateral foi observado. Conclui-se que o benazepril é um inibidor da enzima de conversão da angiotensina, eficaz e bem tolerado no tratamento da ICC no cão.Clinical effects of benazepril, a long acting angiotensin-converting enzyme (ACEi, in dogs with naturally-occurring congestive heart failure (CHF caused by mitral endocardiosis or dilated cardiomyopathy were studied. The drug was given orally at a dose of 0.25 to 0.5mg/kg/day. Diuretics, digitalics, and antiarrhtyhmic drugs were given as needed. Physical, radiographic, and eletrocardiographic examination were performed at days 0, 7, 28, and 56. Serum biochemistry and arterial blood gases were obtained at days 0 and 56. Signs of dyspnea and general condition improved in all dogs. Cough decreased in frequency. The electrocardiogram did not change with benazepril use except for a decrease in P wave amplitude and duration. No adverse effects related to the use of benazepril were observed. Benazepril is an effective and well tolerated ACEi for the treatment of CHF in dogs.

  15. Relationship between late ventricular potentials and myocardial {sup 123}I-metaiodobenzylguanidine scintigraphy in patients with dilated cardiomyopathy with mild to moderate heart failure: results of a prospective study of sudden death events

    Energy Technology Data Exchange (ETDEWEB)

    Kasama, Shu [Gunma University Graduate School of Medicine, Department of Medicine and Biological Science (Cardiovascular Medicine), Gunma (Japan); Cardiovascular Hospital of Central Japan (Kitakanto Cardiovascular Hospital), Department of Cardiovascular Medicine, Gunma (Japan); Toyama, Takuji; Kaneko, Yoshiaki; Kurabayashi, Masahiko [Gunma University Graduate School of Medicine, Department of Medicine and Biological Science (Cardiovascular Medicine), Gunma (Japan); Iwasaki, Toshiya; Sumino, Hiroyuki; Kumakura, Hisao; Minami, Kazutomo; Ichikawa, Shuichi [Cardiovascular Hospital of Central Japan (Kitakanto Cardiovascular Hospital), Department of Cardiovascular Medicine, Gunma (Japan); Matsumoto, Naoya [Nihon University School of Medicine, Department of Cardiology, Tokyo (Japan); Sato, Yuichi [Health Park Clinic, Department of Imaging, Gunma (Japan)

    2012-06-15

    Late ventricular potentials (LPs) are considered to be useful for identifying patients with heart failure at risk of developing ventricular arrhythmias. {sup 123}I-metaiodobenzylguanidine (MIBG) scintigraphy, which is used to evaluate cardiac sympathetic activity, has demonstrated cardiac sympathetic denervation in patients with malignant ventricular tachyarrhythmias. This study was undertaken to clarify the relationship between LPs and {sup 123}I-MIBG scintigraphy findings in patients with dilated cardiomyopathy (DCM). A total of 56 patients with DCM were divided into an LP-positive group (n = 24) and an LP-negative group (n = 32). During the compensated period, the delayed heart/mediastinum count (H/M) ratio, delayed total defect score (TDS), and washout rate (WR) were determined from {sup 123}I-MIBG images and plasma brain natriuretic peptide (BNP) concentrations were measured. Left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV), and left ventricular ejection fraction (LVEF) were simultaneously determined by echocardiography. LVEDV, LVESV, LVEF and plasma BNP concentrations were similar in the two groups. However, TDS was significantly higher (35 {+-} 8 vs. 28 {+-} 6, p < 0.005), the H/M ratio was significantly lower (1.57 {+-} 0.23 vs. 1.78 {+-} 0.20, p < 0.005), and the WR was significantly higher (60 {+-} 14% vs. 46 {+-} 12%, p < 0.001) in the LP-positive than in the LP-negative group. The average follow-up time was 4.5 years, and there were nine sudden deaths among the 56 patients (16.1%). In logistic regression analysis, the incidences of sudden death events were similar in those LP-negative with WR <50%, LP-negative with WR {>=}50% and LP-positive with WR <50% (0%, 10.0% and 14.3%, respectively), but was significantly higher (41.2%) in those LP-positive with WR {>=}50% (p < 0.01, p < 0.05, and p < 0.05, respectively). The present study demonstrated that the values of cardiac {sup 123}I-MIBG scintigraphic parameters

  16. Assessment of cardiac neuronal function with iodine-123 MIBG scintigraphy in children with idiopathic dilated cardiomyopathy; Evaluation de la fonction neuronale cardiaque par la scintigraphie a l'iode-123 MIBG dans les myocardiopathies dilatees idiopahiques de l'enfant

    Energy Technology Data Exchange (ETDEWEB)

    Maunoury, Ch.; Sebahoun, St.; Hallaj, I.; Barritault, L.; Acar, Ph.; Sidi, D.; Kachaner, J. [Hopital Necker-Enfants-Malades, 75 - Paris (France); Agostini, D.; Bouvard, G. [Centre Hospitalier Universitaire Cote de Nacre, 14 - Caen (France)

    2000-03-01

    The I-123 MIBG cardiac scintigraphy can assess norepinephrine uptake. It has been shown that cardiac adrenergic neuronal function was impaired in adults with dilated cardiomyopathy. The aim of this prospective study was to assess cardiac neuronal function in children with idiopathic dilated cardiomyopathy (DCM) and to compare cardiac uptake of I-123 MIBG with left ventricular ejection fraction (LVEF). We studied 26 consecutive patients with idiopathic DCM, aged 44 {+-} 50 months, and 12 controls, aged 49 {+-}65 months. A planar scintigraphy was performed in all children 4 hours after intravenous injection of 20 to 75 MBq of I-123 MIBG. A static anterior view was acquired for 10 minutes. Cardiac uptake of I-123 MIBG was expressed as the heart to mediastinum count ratio (HMR). Equilibrium radionuclide angiography was performed following a standard protocol. Cardiac uptake of I-123 MIBG was significantly decreased in patients with idiopathic DCM when compared with cardiac uptake in controls (172{+-}34% vs 277{+-}14%, P<0.0001). There was a good correlation between RCM and LVEF in patients with idiopathic DCM (y = 2.5 x +113.3, r = 0.80, P < 0.0001). In conclusion, cardiac neuronal function was impaired in children with idiopathic DCM and related to impairment of left ventricular function. (author)

  17. Genetics Home Reference: familial dilated cardiomyopathy

    Science.gov (United States)

    ... As a result, the heart is unable to pump blood as efficiently as usual. Eventually, all four chambers ... thin and weakened, it is less able to pump blood. Over time, this condition results in heart failure. ...

  18. Insights into restrictive cardiomyopathy from clinical and animal studies

    OpenAIRE

    Jean-Charles, Pierre-Yves; Li, Yue-Jin; Nan, Chang-Long; Huang, Xu-Pei

    2011-01-01

    Cardiomyopathies are diseases that primarily affect the myocardium, leading to serious cardiac dysfunction and heart failure. Out of the three major categories of cardiomyopathies (hypertrophic, dilated and restrictive), restrictive cardiomyopathy (RCM) is less common and also the least studied. However, the prognosis for RCM is poor as some patients dying in their childhood. The molecular mechanisms behind the disease development and progression are not very clear and the treatment of RCM is...

  19. Magnetic resonance imaging in familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion and cardiac enzymes

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, Tsunehiko; Nagata, Seiki; Sakakibara, Hiroshi

    1988-05-01

    Gated magnetic resonance imaging (MRI) was performed in 6 patients with familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion, and 12 patients with ordinary hypertrophic cardiomyopathy. The patients with ordinary hypertrophic cardiomyopathy and abnormal thickening of the septal wall and normal left ventricular dimensions, while the patients with familial hypertrophic cardiomyopathy had focal wall thinning (usually involving the apical-septal wall) and dilated left ventricle in addition to hypertrophied heart. The quantitative measurement for cardiac dimensions using MRI was similar to that found on echocardiography in all cases. In addition, inhomogeneous signal intensities at left ventricular wall were observed in 3 cases of familial hypertrophic cardiomyopathy, which may suggest the existence of myocardial fibrosis. Gated MRI should be performed for early detection and follow-up of hypertrophic cardiomyopathy, since some patients will progress from hypertrophic cardiomyopathy to dilated cardiomyopathy.

  20. What Causes Cardiomyopathy?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Causes Cardiomyopathy? Cardiomyopathy can be acquired or inherited. “Acquired” means ... case when the disease occurs in children. Hypertrophic Cardiomyopathy Hypertrophic cardiomyopathy usually is inherited. It’s caused by ...

  1. Logistic Analysis of Risk Factor About Dilated Cardiomyopathy with Ventricular Congestive Heart Failure%扩张型心肌病合并充血性心力衰竭的相关因素Logistic分析

    Institute of Scientific and Technical Information of China (English)

    谢进; 李欣; 许臣洪; 胡沛; 卢洪涛

    2012-01-01

    目的:寻找扩张型心肌病(DCM)合并充血性心力衰竭(CHF)的独立危险因素,为临床预防和治疗扩心病患者的病情提供依据.方法:选择我院收治的扩张型心肌病患者125例,按照NYHA分级分组,将心功能Ⅱ-Ⅳ级同时左心室射血分数(LVEF)<40%的患者91例定义为观察组;将心功能Ⅰ级同时左心室射血分数≥40%的患者34例定义为对照组.在比较两组临床资料的基础上,采用logistic多因素分析方法确立扩张型心肌病合并心力衰竭的独立危险因素.结果:单因素分析结果发现,观察组中的“房颤”及“脉压≥70mmHg”这两个参数比对照组的数量明显增多,差异有统计学意义(P<0.05).logistic多因素分析发现“脉压≥70mmHg”、“房颤”是扩心病合并心力衰竭的独立危险因素.结论:“脉压≥70mmHg”、“房颤”是扩张型心肌病合并充血性心力衰竭的独立危险因素,需要在临床治疗扩心病患者时警惕这两项指标的异常.%Objective: To find the independent risk of factor of dilated cardiomyopathy (DCM) with ventricular congestive heart failure (CHF). Methods: 125 patients from our hospital were involved in this investigation. Patients were divided by NYHA. 91 patients with NYHA grade II -IV and LVEF<40% were selected as observation group, and the rest 34 patients with NYHA grade I with LVEF ≥40% were selected as control group. On the base of analyses of clinical data, logistic analyses were used to establish its independent risk factors. Results: Univariate analyses showed that "atrial fibrillation"and "pulse pressure ≥ 70mmHg" in the observation group were increased significantly than that in the control group (P<0.05). Logistic analyses showed that "pulse pressure≥ 70mmHg" and "atrial fibrillation"were independent factors. Conclusion: "pulse pressure ≥70mmHg" and "atrial fibrillation" were independent factors of DCM with CHF. It is necessary to be alert when these

  2. 扩张型心肌病患者心外膜脂肪厚度与左室重构的相关性研究%Association between epicardial adipose tissue thickness and left ventricular remodeling in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    马晶

    2014-01-01

    Objective To explore the association between epicardial adipose tissue (EAT) thickness and left ventricular remodeling,left ventricular dysfunction in dilated cardiomyopathy (DCM).Methods One hundred and sixteen patients with DCM (DCM group) and 76 healthy subjects (control group) were examined by ultrasoundcardiogram.Left ventricular end-systolic diameter (LVESD),left ventricular end-diastolic diameter (LVEDD),left ventricular end-systolic volume (LVESV),left ventricular end-diastolic volume (LVEDV),left ventricular end-systolic volume index (LVESVI),left ventficular end-diastolic volume index (LVEDVI),left ventricular ejection fraction (LVEF) and EAT thickness were measured or calculated,and the relationship was evaluated.Results EAT thickness in DCM group was significantly lower than that in control group [(4.7 ± 1.2) mm vs.(7.6 ± 2.1) mm],and there was statistical difference (P < 0.05).The linear correlation analysis results showed there was positive correlation between EAT thickness and LVESD,LVEDD,LVESV,LVEDV,LVEDVI,LVESVI (r =0.236,0.220,0.245,0.256,0.282,0.279,P < 0.05),and there was no relationship between EAT thickness and LVEF (r =0.134,P >0.05).Conclusions In patients with DCM,there is a correlation between EAT thickness and ventricular remodeling.There is no correlation between EAT thickness and left ventricular dysfunction.%目的 探讨扩张型心肌病(DCM)患者的心外膜脂肪(EAT)厚度与左室重构及左室功能不全的关系.方法 116例DCM患者(DCM组)和76例健康体检者(对照组)均行超声心动图检查,测量或计算左室收缩末期内径(LVESD)、左室舒张末期内径(LVEDD)、左室收缩末期容积(LVESV)、左室舒张末期容积(LVEDV)、左室收缩末期容积指数(LVESVI)、左室舒张末期容积指数(LVEDVI)、左室射血分数(LVEF)、EAT厚度等,并进行比较分析.结果 DCM组EAT厚度为(4.7±1.2) mm,显著低于对照组的(7.6±2.1) mm,差异有统计学意义(P<0.05).经线性相

  3. 扩张型心肌病患儿血清periostin蛋白的检测及意义%Expression of serum periostin in children with dilated cardiomyopathy and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    吴岚; 孙景辉; 张春艳; 王朝霞

    2014-01-01

    目的 探讨血清periostin蛋白水平与扩张型心肌病(DCM)患儿病情严重程度的相关性.方法 选取2009年1月至2013年6月于吉林大学第一医院儿科诊断扩张型心肌病患儿32例为DCM组,根据ROSS评分标准分为0~2分组(6例)、3~6分组(7例)、7~9分组(11例)及10~12分组(8例);选取于吉林大学第一医院同期体检的健康儿童20例为健康对照组.酶联免疫法测定各组儿童血清periostin蛋白水平,免疫抑制法测定肌酸激酶同工酶(CK-MB)水平,心脏彩超测定左心室射血分数(LVEF)及左心室舒张末期内径(LVEDD),比较各组间上述指标的差异,采用直线相关分析法分析periostin与ROSS评分及LVEF的相关性.结果 1.与健康对照组比较,DCM组血清periostin蛋白水平显著增高,差异有统计学意义(P=0.00);且DCM患儿随着ROSS评分的增高血清periostin蛋白水平逐渐增高,不同ROSS评分组间差异有统计学意义(P均<0.05).2.与健康对照组比较,DCM组CK-MB水平显著增高,差异有统计学意义(P=0.00);不同ROSS评分组间CK-MB水平比较差异无统计学意义(P均>0.05).3.与健康对照组比较,DCM组LVEF显著降低,差异有统计学意义(P=0.00);且DCM患儿随着ROSS评分的增高LVEF逐渐降低,不同ROSS评分组间差异有统计学意义(P均<0.05).4.与健康对照组比较,DCM组LVEDD显著增大,差异有统计学意义(P=0.00);不同ROSS评分组间LVEDD比较差异无统计学意义(P均>0.05).5.血清periostin蛋白水平与ROSS评分呈正相关(r=0.742,P< 0.001),与CK-MB水平无相关性(r=0.247,P>0.05),与LVEF呈负相关(r=-0.424,P<0.01).结论 血清periostin蛋白水平在DCM患儿中显著增高,其水平与ROSS评分呈正相关,与LVEF呈负相关,可作为DCM患儿病情评估的新指标之一.%Objective To investigate the correlation of serum periostin and severe degree of dilated cardiomyopathy(DCM) in children.Methods Thirty-two children with DCM from Jan.2009 to Jun

  4. 自体骨髓单个核细胞移植治疗心肌病疗效及安全性评估%Effect and safety of autologous intracoronary bone marrow mononuclear cells transplantation in dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王悦喜; 阿荣; 张迎军; 董莉; 李婧; 杨振华; 任保军

    2014-01-01

    Objective To investigate the effect and safety of autologous bone marrow mononuclear cells(BMMNCs) transplantation in dilated cardiomyopathy.Methods 20 patients aged 18-67 years with dilated cardiomyopathy,who suffered from New York Heart Association class Ⅲ to Ⅳ heart failure,left ventricular ejection fraction(LVEF) <35%,were randomized to transplantation group (intracoronary injection of autologous BMMNCs 1.80 × 109~ 5.90 × 109/L,combined with drug treatment,n=8) or the control group(ordinary drugs treatment,n=12).The left ventricular end diastolic diameter(LVEDD),ejection fraction(EF),6-minute walk test and myocardial metabolism detected by emission computed tomography(ECT) were observed after 6 months of treatment.Results There were significant differences in LVEDD,EF,6-minute walk test between the transplantation group and the control group after 6 months of treatment [(50.3 ± 4.2) mm vs.(55.4 ±3.7) mm,(45.4±5.2)% vs.(39.2±6.3)%,(76.6±5.8) m vs.(69.7±8.6) m,t=2.93,3.21,2.96,respectively,all P<0.05].After 6 months of treatment,LVEDD was shorted from(57.2± 6.5) mm to(50.3±4.2) mm(t=5.60,P<0.01) and EF was increased from(30.4±6.7) % to(45.4 ±5.2) %(t=6.30,P<0.01) in the transplantation group,and EF was increased from(31.1±5.9) % to(39.2±6.3) %(t=3.60,P<0.05) in the control group.Compared with pre-treatment,the 6-minute walk distance were increased in the two group after 6 months of treatment [transplantation group:(76.6±5.8) m vs.(54.0±6.2) m,P<0.05; control group:(69.7±8.6) m vs.(55.0±5.7) m,P<0.05].Myocardialmetabolism density of radioactive 18-fluorodeoxyglucose(18-FDG) in the same segment was significantly increased,and the metabolic density of radioactive FDG in sparse segment was significantly reduced in transplantation group after 6 months of treatment as compared with pre-treatment.No serious complications associated with BMMNCs injection,including ventricular arrhythmia and death,were observed in

  5. O papel do acúmulo de colágeno no interstício miocárdico na sobrevida dos pacientes com cardiomiopatia dilatada idiopática e chagásica The role of storage of interstitial myocardial collagen on the overlife rate of patients with idiopathic and chagasic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Vera Lopes Nunes

    2006-12-01

    Full Text Available OBJETIVO: Avaliar a correlação entre um marcador estrutural do miocárdio e a sobrevida dos pacientes com cardiomiopatia dilatada. MÉTODOS: Mediante realização da biópsia endomiocárdica e exame ecocardiográfico foram estudados 9 indivíduos sem doença estrutural miocárdica (controle e 45 pacientes com cardiomiopatia dilatada grave de etiologia idiopática (MCDI e chagásica (MCDC. Foi analisada a correlação entre a quantidade de colágeno miocárdico intersticial (FVCI e a sobrevida desses pacientes, se a FVCI diferia entre as etiologias, e se a fibrose interferia na função e geometria do miocárdio. RESULTADOS: Foi observado que a FVCI foi 15 vezes maior nos cardiomiopatas em relação ao grupo-controle, mas não diferiu em relação às MCDI e MCDC (*p OBJECTIVE: To find out whether there is a correlation between a myocardial structural marker and the overlife rate of patients with dilated cardiomyopathy. METHODS: Using endomyocardial biopsy and 2D-echocardiogram, we studied nine patients with no changes in myocardial structure (control and 45 patients with severe dilated cardiomyopathy of idiopathic etiology (IDCM and of Chagasic etiology (CDCM. We analyzed the correlation between the quantity of interstitial myocardial collagen (ICVF and the overlife rates of these patients. We also evaluated the difference in ICVF between these groups and whether fibrosis interfered on the geometry and function of the myocardium. RESULTS: We observed that ICVF was 15 times higher in cardiomyopathy patients than in the control group, but there was no difference in ICVF between CDCM and IDCM (*p < 0.001 patients. There was no correlation between ICVF and the overlife rate in cardiomyopathy patients (IDCM p = 0.249, and CDCM p = 0.587. We observed a significant correlation between ICVF and left ventricular ejection fraction (LVEF only for IDCM. There was no correlation between ICVF and left ventricular diastolic diameter in either etiology

  6. Molecular genetics and pathogenesis of cardiomyopathy.

    Science.gov (United States)

    Kimura, Akinori

    2016-01-01

    Cardiomyopathy is defined as a disease of functional impairment in the cardiac muscle and its etiology includes both extrinsic and intrinsic factors. Cardiomyopathy caused by the intrinsic factors is called as primary cardiomyopathy of which two major clinical phenotypes are hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). Genetic approaches have revealed the disease genes for hereditary primary cardiomyopathy and functional studies have demonstrated that characteristic functional alterations induced by the disease-associated mutations are closely related to the clinical types, such that increased and decreased Ca(2+) sensitivities of muscle contraction are associated with HCM and DCM, respectively. In addition, recent studies have suggested that mutations in the Z-disc components found in HCM and DCM may result in increased and decreased stiffness of sarcomere, respectively. Moreover, functional analysis of mutations in the other components of cardiac muscle have suggested that the altered response to metabolic stresses is associated with cardiomyopathy, further indicating the heterogeneity in the etiology and pathogenesis of cardiomyopathy. PMID:26178429

  7. Celiac disease with pulmonary haemosiderosis and cardiomyopathy

    OpenAIRE

    Işikay, Sedat; Yilmaz, Kutluhan; Kilinç, Metin

    2012-01-01

    Celiac disease or pulmonary haemosiderosis can be associated with several distinguished conditions. Pulmonary haemosiderosis is a rare, severe and fatal disease characterised by recurrent episodes of alveolar haemorrhage, haemoptysis and anaemia. Association of pulmonary haemosiderosis and celiac disease is extremely rare. We describe a case of celiac disease presented with dilated cardiomyopathy and pulmonary haemosiderosis without gastrointestinal symptoms of celiac disease. In addition, vi...

  8. 卡维地洛对扩张型心肌病心力衰竭患者心功能及运动耐量的改善作用%Improving effect of carvedilol on cardiac function and exercise tolerance in patients with congestive heart failure of dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    吴大庆; 杨永健

    2002-01-01

    Objective To observe the improving effect of carvedilol on cardiac function in patients with congestive heart failure(CHF) of dilated cardiomyopathy(DCM).Methods Total 60 patients with chronic heart failure secondary to DCM were divided into two groups randomly, namely carvedilol group additionally treated with carvedilol (the test group), conventional group receiving placebo (the controls). The left ventricular fraction shortening (FS), ejection fraction (EF), stroke volume (SV), left ventricular diastolic dimension (LVDD) were measured with echocardiography, were measured before and after 4 month treatment. Results After treated for 4 months , the cardiac function improved greatly in both groups.In the test group, LVDD, EF and 6 minute walking distance improved more signicantly compared to the controls. Conclusion Carvedilol can improve cardiac function and exercise tolerance in the patients with CHF of DCM obviously.

  9. Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?

    Science.gov (United States)

    Arbustini, Eloisa; Favalli, Valentina; Narula, Nupoor; Serio, Alessandra; Grasso, Maurizia

    2016-08-30

    Left ventricular noncompaction (LVNC) describes a ventricular wall anatomy characterized by prominent left ventricular (LV) trabeculae, a thin compacted layer, and deep intertrabecular recesses. Individual variability is extreme, and trabeculae represent a sort of individual "cardioprinting." By itself, the diagnosis of LVNC does not coincide with that of a "cardiomyopathy" because it can be observed in healthy subjects with normal LV size and function, and it can be acquired and is reversible. Rarely, LVNC is intrinsically part of a cardiomyopathy; the paradigmatic examples are infantile tafazzinopathies. When associated with LV dilation and dysfunction, hypertrophy, or congenital heart disease, the genetic cause may overlap. The prevalence of LVNC in healthy athletes, its possible reversibility, and increasing diagnosis in healthy subjects suggests cautious use of the term LVNC cardiomyopathy, which describes the morphology but not the functional profile of the cardiomyopathy. PMID:27561770

  10. [Ventricular tachyarrhythmias in patients with cardiomyopathy

    DEFF Research Database (Denmark)

    Henningsen, K.; Christensen, A.H.; Svendsen, Jesper Hastrup

    2008-01-01

    INTRODUCTION: The purpose of this study was to determine the number and distribution of cardiomyopathies as the aetiology of ventricular tachyarrhythmias among patients discharged from the Department of Cardiology, Rigshospitalet. MATERIALS AND METHODS: The study was a retrospective review of...... patients discharged with the diagnostic codes ventricular tachycardia, ventricular fibrillation or premature ventricular contractions with cardiomyopathy as the presumed aetiology. Patients discharged during a period of 6 years and 5 months were included in the study. The patients were characterized by...... disease, gender, age, previous cardiac arrest and treatment with implantable cardioverter-defibrillator (ICD). RESULTS: 993 patients were screened and 128 patients with cardiomyopathy were identified, corresponding to 13% of the screened patients. 58 (45%) of the patients had dilated cardiomyopathy (DCM...

  11. Substituição da valva mitral com tração dos músculos papilares em pacientes com miocardiopatia dilatada Mitral valve replacement with chordae tendineae preservation, traction and fixation in end-stage dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fabio Antonio Gaiotto

    2007-03-01

    Full Text Available OBJETIVO: Avaliar a geometria e a função do ventrículo esquerdo (VE após a troca mitral com tração e fixação dos papilares, em portadores de insuficiência cardíaca terminal com insuficiência mitral secundária. MÉTODO: Dos 20 pacientes avaliados, 70% eram homens, com idade média de 50,2 anos e 55% recebiam inotrópicos. A fração de ejeção (FEVE foi menor que 30% em todos; 85% estavam em classe funcional (CF IV. Dezoito receberam próteses de pericárdio bovino e dois, mecânicas. Os períodos considerados foram: 3, 6, 12 e 18 meses. As variáveis consideradas: volume sistólico do VE (VS, a FEVE, os diâmetros sistólico e diastólico finais (DSF e DDF e os volumes sistólico e diastólico finais (VSF e VDF. No estudo estatístico, empregou-se da análise de variância (AV e o teste de Friedmann (F. A sobrevida foi aferida pelo método de Kaplan-Meyer. RESULTADOS: Dois (10% faleceram no período imediato. A sobrevida no primeiro ano foi de 85%, no segundo, 44%, no terceiro, 44%, no quarto, 44% e no quinto, 44%. A comparação entre pré e 3 meses, empregando-se a AV, não revelou alteração significativa para o VS (p=0,086. Houve acréscimo da FEVE (p=0,008 e decréscimo do DDF (p=0,038; do DSF (p=0,008; do VDF (p=0,029 e do VSF (p=0,009. Os momentos pré, 3 e 6 meses, com o teste F, não revelaram alterações. Entre os momentos pré, 3 meses e final, empregando-se a AV, não houve significância. CONCLUSÃO: Há melhora da FEVE, dos VDF, VSF, DDF e DSF; até o terceiro mês. A partir de então, as variáveis permanecem estáveis.OBJECTIVE: This study aimed at evaluating results of mitral valve replacement using a new technique of complete chordae tendineae adjustment for left ventricular remodeling. METHODS: Twenty end-stage idiopathic dilated cardiomyopathy patients with severe functional mitral valve regurgitation underwent mitral valve replacement. Seventeen (85% were in functional class IV. Both anterior and posterior

  12. Clínica de cães com cardiomiopatia dilatada idiopática, tratados ou não com carvedilol Clinic of dogs with dilated cardiomyopathy (DCM treated or not by carvedilol

    Directory of Open Access Journals (Sweden)

    Moacir Leomil Neto

    2011-04-01

    -arrhythmics. Carvedilol is a third generation non-selective β-blocker which blocks equally and competitively (β1, β2 and α1 receptors. Produces an evident peripheral vasodilation, exerts anti-oxidative effects, removing free radicals of oxygen and preventing lipidic peroxydation of cardiac membranes, and the loss of myocytes and arrhythmias, as well as reducing mortality rate in human patients. The aim of the present study was to evaluate by physical examination, electrocardiography, radiography, and echocardiography the evolution of dogs with dilated cardiomyopathy (DCM treated by conventional therapy associated to carvedilol. Forty-nine dogs with DCM were divided in two groups: group NT: treated with conventional therapy, and group T: treated with conventional therapy associated to carvedilol. The animals were submitted to clinical and complementary examinations during one year. The results demonstrated that carvedilol therapy presented good tolerability on the dose of 0.3mg kg-1 each 12 hours, prolonged lifetime of the dogs in 30.9%, did not alter systolic or diastolic pressure, reduced heart frequency after three weeks of treatment, significantly enhanced shortening and ejection fractions after six months of treatment, did not promote radiographic or E-septum distance alterations, decreased patients letality, as demonstrated by improvement of clinical score and functional class (heart failure according to NYHA of the animals, obtained three weeks after the beginning of cavedilol therapy.

  13. Sincronia ventricular em portadores de miocardiopatia dilatada e indivíduos normais: avaliação através da ventriculografia radioisotópica Ventricular synchrony in patients with dilated cardiomyopathy and normal individuals: assessment by radionuclide ventriculography

    Directory of Open Access Journals (Sweden)

    Simone Cristina S. Brandão

    2007-05-01

    Full Text Available OBJETIVO: Estabelecer parâmetros de sincronia intra- e interventricular em indivíduos normais e compará-los aos de pacientes com miocardiopatia dilatada com e sem distúrbios de condução ao eletrocardiograma (ECG. MÉTODOS: Três grupos de pacientes foram incluídos no estudo: 18 indivíduos (G1 sem cardiopatia e com ECG normal (52+/-12 anos, 29% masculinos; 50 portadores de miocardiopatia dilatada e disfunção ventricular esquerda grave, sendo 20 pacientes (G2 com QRS 120 ms (57+/-12 anos, 60% masculinos. Todos foram submetidos à ventriculografia radioisotópica (VR. Para avaliar dissincronia intraventricular esquerda foi estudada a largura do histograma de fase e para avaliar dissincronia interventricular foi medida a diferença da média do ângulo de fase entre o ventrículo direito e o esquerdo (DifDE. RESULTADOS: As frações de ejeção do ventrículo esquerdo (FEVEs foram: 62±6% (G1, 27±6% (G2 e 22±7% (G3 e do VD foram: 46 ± 4% (G1, 38±9%(G2 e 37±9% (G3. A avaliação da largura do histograma de fase foi de: 89±18 ms (G1, 203±54 ms (G2 e 312±130 ms (G3, pOBJECTIVE: To establish the parameters of intra- and interventricular synchrony in normal individuals and to compare them with patients with dilated cardiomyopathy with and without conduction disorders shown in the electrocardiogram (ECG examination. METHODS: Three groups of patients were included in this study: 18 individuals (G1 with no cardiomyopathy and with a normal ECG (52±12 years, 29% male; 50 patients with dilated cardiomyopathy and severe left ventricular dysfunction, with 20 patients (G2 presenting QRS 120ms (57±12 years, 60% male. All patients underwent RV. Evaluation of left intraventricular dyssynchrony was carried out with the measurement of the phase histogram width and interventricular dyssynchrony was evaluated by the difference of the mean phase angle between the right and left ventricles (RLDif. RESULTS: Left ventricle ejection fractions (LVEFs were

  14. Dilatations Revisited

    CERN Document Server

    La, H S

    1996-01-01

    Dilatation, i.e. scale, symmetry in the presence of the dilaton in Minkowski space is derived from diffeomorphism symmetry in curved spacetime, incorporating the volume-preserving diffeomorphisms. The conditions for scale invariance are derived and their relation to conformal invariance is examined. In the presence of the dilaton scale invariance automatically guarantees conformal invariance due to diffeomorphism symmetry. Low energy scale-invariant phenomenological Lagrangians are derived in terms of dilaton-dressed fields, which are identified as the fields satisfying the usual scaling properties. The notion of spontaneous scale symmetry breaking is defined in the presence of the dilaton. In this context, possible phenomenological implications are advocated and by computing the dilaton mass the idea of PCDC (partially conserved dilatation current) is further explored.

  15. Cirrhotic cardiomyopathy.

    Science.gov (United States)

    Milani, A; Zaccaria, R; Bombardieri, G; Gasbarrini, A; Pola, P

    2007-06-01

    Decompensated liver cirrhosis is characterized by a peripheral vasodilation with a low-resistance hyperdynamic circulation. The sustained increase of cardiac work load associated with such a condition may result in an inconstant and often subclinical series of heart abnormalities, constituting a new clinical entity known as "cirrhotic cardiomyopathy". Cirrhotic cardiomyopathy is variably associated with baseline increase in cardiac output, defective myocardial contractility and lowered systo-diastolic response to inotropic and chronotropic stimuli, down-regulated beta-adrenergic function, slight histo-morphological changes, and impaired electric "recovery" ability of ventricular myocardium. Cirrhotic cardiomyopathy is usually clinically latent or mild, likely because the peripheral vasodilation significantly reduces the left ventricle after-load, thus actually "auto-treating" the patient and masking any severe manifestation of heart failure. In cirrhotic patients, the presence of cirrhotic cardiomyopathy may become unmasked and clinically evident by certain treatment interventions that increase the effective blood volume and cardiac pre-load, including surgical or transjugular intrahepatic porto-systemic shunts, peritoneo-venous shunts (LeVeen) and orthotopic liver transplantation. Under these circumstances, an often transient overt congestive heart failure may develop, with increased cardiac output as well as right atrial, pulmonary artery and capillary wedge pressures. PMID:17383244

  16. Takotsubo cardiomyopathy

    DEFF Research Database (Denmark)

    Nielsen, Lene Hüche; Munk, Kim; Goetzsche, Ole;

    2009-01-01

    INTRODUCTION: Sparse information with regard to the electrocardiographic (ECG) changes in Takotsubo cardiomyopathy (TC) is available. The purpose of this study was to describe the clinical characteristics and electrocardiographic changes in a Danish cohort of patients with TC. We discuss the...

  17. Exome Sequencing Identifies a Novel LMNA Splice-Site Mutation and Multigenic Heterozygosity of Potential Modifiers in a Family with Sick Sinus Syndrome, Dilated Cardiomyopathy, and Sudden Cardiac Death.

    Science.gov (United States)

    Zaragoza, Michael V; Fung, Lianna; Jensen, Ember; Oh, Frances; Cung, Katherine; McCarthy, Linda A; Tran, Christine K; Hoang, Van; Hakim, Simin A; Grosberg, Anna

    2016-01-01

    The goals are to understand the primary genetic mechanisms that cause Sick Sinus Syndrome and to identify potential modifiers that may result in intrafamilial variability within a multigenerational family. The proband is a 63-year-old male with a family history of individuals (>10) with sinus node dysfunction, ventricular arrhythmia, cardiomyopathy, heart failure, and sudden death. We used exome sequencing of a single individual to identify a novel LMNA mutation and demonstrated the importance of Sanger validation and family studies when evaluating candidates. After initial single-gene studies were negative, we conducted exome sequencing for the proband which produced 9 gigabases of sequencing data. Bioinformatics analysis showed 94% of the reads mapped to the reference and identified 128,563 unique variants with 108,795 (85%) located in 16,319 genes of 19,056 target genes. We discovered multiple variants in known arrhythmia, cardiomyopathy, or ion channel associated genes that may serve as potential modifiers in disease expression. To identify candidate mutations, we focused on ~2,000 variants located in 237 genes of 283 known arrhythmia, cardiomyopathy, or ion channel associated genes. We filtered the candidates to 41 variants in 33 genes using zygosity, protein impact, database searches, and clinical association. Only 21 of 41 (51%) variants were validated by Sanger sequencing. We selected nine confirmed variants with minor allele frequencies G, a novel heterozygous splice-site mutation as the primary mutation with rare or novel variants in HCN4, MYBPC3, PKP4, TMPO, TTN, DMPK and KCNJ10 as potential modifiers and a mechanism consistent with haploinsufficiency.

  18. Exome Sequencing Identifies a Novel LMNA Splice-Site Mutation and Multigenic Heterozygosity of Potential Modifiers in a Family with Sick Sinus Syndrome, Dilated Cardiomyopathy, and Sudden Cardiac Death.

    Directory of Open Access Journals (Sweden)

    Michael V Zaragoza

    Full Text Available The goals are to understand the primary genetic mechanisms that cause Sick Sinus Syndrome and to identify potential modifiers that may result in intrafamilial variability within a multigenerational family. The proband is a 63-year-old male with a family history of individuals (>10 with sinus node dysfunction, ventricular arrhythmia, cardiomyopathy, heart failure, and sudden death. We used exome sequencing of a single individual to identify a novel LMNA mutation and demonstrated the importance of Sanger validation and family studies when evaluating candidates. After initial single-gene studies were negative, we conducted exome sequencing for the proband which produced 9 gigabases of sequencing data. Bioinformatics analysis showed 94% of the reads mapped to the reference and identified 128,563 unique variants with 108,795 (85% located in 16,319 genes of 19,056 target genes. We discovered multiple variants in known arrhythmia, cardiomyopathy, or ion channel associated genes that may serve as potential modifiers in disease expression. To identify candidate mutations, we focused on ~2,000 variants located in 237 genes of 283 known arrhythmia, cardiomyopathy, or ion channel associated genes. We filtered the candidates to 41 variants in 33 genes using zygosity, protein impact, database searches, and clinical association. Only 21 of 41 (51% variants were validated by Sanger sequencing. We selected nine confirmed variants with minor allele frequencies G, a novel heterozygous splice-site mutation as the primary mutation with rare or novel variants in HCN4, MYBPC3, PKP4, TMPO, TTN, DMPK and KCNJ10 as potential modifiers and a mechanism consistent with haploinsufficiency.

  19. Takotsubo cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Sénior, Juan Manuel

    2015-04-01

    Full Text Available Takotsubo cardiomyopathy or stress-induced cardiomyopathy is often diagnosed as an acute coronary syndrome in postmenopausal women, because its clinical presentation may mimic an acute myocardial infarction: anginal chest pain, changes in the ST segment and T wave in precordial leads and elevated cardiac biomarkers of necrosis. It is characterized by systolic dysfunction with transient ballooning of the apical and middle portions of the left ventricle in the absence of significant coronary disease. Prognosis is good and complete recovery occurs in days to weeks. We report three cases of postmenopausal women with initial diagnosis of acute myocardial infarction; no significant coronary lesions were found in the coronary angiography; apical ballooning, characteristic of this syndrome, was observed on left ventriculography. On follow-up, the three patients had complete recovery of systolic function at six weeks.

  20. Evaluation of left ventricular systolic function in patients with dilated cardiomyopathy by real-time three-dimensional speckle tracking echocardiography%实时三维超声心动图斑点追踪技术评价扩张型心肌病患者左室收缩功能

    Institute of Scientific and Technical Information of China (English)

    李阳; 邓又斌; 黄润青; 孙杰; 刘琨; 汤乔颖

    2013-01-01

    目的 应用实时三维超声心动图斑点追踪技术评价扩张型心肌病(DCM)患者左室收缩功能.方法 应用实时三维超声斑点追踪技术分别测量24例DCM患者(DCM组)和19例健康成人志愿者(对照组)左室收缩期纵向、圆周向、径向以及面积应变峰值,比较两组左室心肌基底部、中间部及心尖部局部应变和总体应变的差异,并分析总体应变与左室射血分数的相关性.结果 DCM组左室心肌纵向、圆周向、径向及面积的基底部、中间部、心尖部局部应变和心肌总体应变均明显小于对照组对应节段,差异均有统计学意义(均P<0.01).左室心肌纵向、圆周向、径向以及面积总体应变均与左室射血分数有良好的相关性(r=0.873、0.862、0.885及0.894,均P<0.01).结论 实时三维超声心动图斑点追踪技术可以为DCM的诊断、疗效评估以及预后判断提供较好的检测手段,具有较大的临床价值.%Objective To evaluate the systolic function of the left ventricle in patients with dilated cardiomyopathy by real-time three-dimensional speckle tracking echocardiography.Methods The peak systolic longitudinal train,circumferential stain,radial strain and area strain of left ventricle were measued by real time three-dimensional speckle tracking echocardiography technology in 24 patients with dilated cardiomyopathy (DCM group) and 19 healthy volunteers (control group).The difference of regional myocardial strain of basal,middle,apical level and global myocardial strain were compared between the two groups.The correlation between global myocardial strain in all directions and left ventricular ejection fraction was analyzed.Results The global and each level longitudinal strain,circumferential strain,radial strain and area strain in DCM group were significantly lower than those in control group(P<0.01).The global myocardial longitudinal train,circumferential stain,radial strain and area strain were

  1. Takotsubo cardiomyopathy or broken heart syndrome: A review article

    Directory of Open Access Journals (Sweden)

    Allahyar Golabchi

    2011-01-01

    Full Text Available Stress-induced cardiomyopathy or Takotsubo cardiomyopathy is a recently increasing diagnosed disease showed by transient apical or mid left ventricular dilation and dysfunction. This sign is similar to acute myocardial infarction but without significant coronary artery stenosis and intra coronary clots. On the other hand there are important and essential differences in their management. Consequently, our physicians should know about its pathophysiology, diagnosis and treatment.

  2. Challenges and opportunities in dystrophin-deficient cardiomyopathy gene therapy

    OpenAIRE

    Duan, Dongsheng

    2006-01-01

    The last decade has evidenced unprecedented progress in gene therapy of Duchenne and Becker muscular dystrophy (DMD and BMD) skeletal muscle disease. Cardiomyopathy is a leading cause of morbidity and mortality in both patients and carriers of DMD, BMD and X-linked dilated cardiomyopathy. However, there is little advance in heart gene therapy. The gene, the vector, vector delivery, the target tissue and animal models are five fundamental components in developing an effective gene therapy. Int...

  3. Acute peritonitis as the first presentation of valvular cardiomyopathy.

    LENUS (Irish Health Repository)

    Higgins, Nikki

    2012-02-01

    Valvular cardiomyopathy can present a diagnostic challenge in the absence of overt cardiac symptoms. This report describes the case of a 46-year-old woman who presented with acute peritonitis associated with vomiting and abdominal distension. Subsequent abdominal computed tomography and ultrasound revealed bibasal pleural effusions, ascites, and normal ovaries. An echocardiogram revealed that all cardiac chambers were dilated with a global decrease in contractility and severe mitral, tricuspid, and aortic regurgitation. A diagnosis of cardiomyopathy with acute heart failure, secondary to valvular heart disease, was secured. Acute peritonitis as the presenting feature of valvular cardiomyopathy is a rare clinical entity.

  4. Dilating Eye Drops

    Science.gov (United States)

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Dilating Eye Drops En Español Read in Chinese What are dilating eye drops? Dilating eye drops contain medication to enlarge ( ...

  5. 磁共振成像对扩张型心肌病转基因模型小鼠左右心室对比分析%Comparative analysis of left and right ventricular dilated cardiomyopathy in transgenic mice by magnetic resonance imaging

    Institute of Scientific and Technical Information of China (English)

    朱皓; 吕丹; 高凯; 张连峰

    2013-01-01

    目的 对cTnTR141W扩张型心肌病转基因模型小鼠左、右心室进行对比分析,研究cTnTR141W转基因小鼠作为右心室心肌病的动物模型的可行性.方法 利用7.0T高场强磁共振成像(MRI)技术,定量分析了2、4、6和8月龄对照组及cTnTR141W转基因模型小鼠左、右心室的舒张末容积(EDV)、收缩末容积(ESV)和射血分数(EF)的变化情况,同时对6月龄对照组cTnTR141W转基因模型小鼠心肌组织进行组织学分析.结果 转基因阴性对照小鼠相比,cTnTR141W转基因小鼠左、右心室的容积在2月龄时已有增大趋势,而射血分数有减小趋势.右心室射血分数减小出现最早也最显著(P<0.05).随年龄增加,cTnTR141W转基因小鼠与转基因阴性对照小鼠相比,右心室的结构和功能的病理生理变化与左心室同时趋于严重.该小鼠左、右心室在4月龄后表现典型的扩张型心肌病表型.结论 cTnTR141W转基因模型小鼠左心室和右心室的扩张性心肌病表型同时出现,该小鼠可作为右室性心肌病等右心室功能下降相关疾病研究的动物模型.%Objective To compare the left and right ventricular function of cTnTR141wtransgenic mice with dilated cardiomyopathy, and to assess whether cTnT transgenic mice can be used as an animal model of right ventricular cardiomyopathy. Methods The structural and functional changes of left and right ventricular myocardium in non-transgenic mice ( NTG) and cTnTR141w transgenic mice at 2, 4 , 6 and 8 months of age were assessed and analyzed by 7. 0 T high-field magnetic resonance imaging technology. Changes of the left and right ventricular end-diastolic volume ( EDV) , end-systolic volume (ESV) , and ejection fraction (EF) were quantitatively analyzed with ages. Pathologic changes of myocardial tissue from NTG and cTnTR141w transgenic mice at 6 month of age were analyzed. Results Compared with the NTG mice, the left and right ventricular volume of cTnTR141w transgenic

  6. 二维斑点追踪超声心动图评价扩张型心肌病左心功能的研究进展%Progress of Two-dimensional Speckle Tracking Echocardiography in Evaluating Left Heart Function of Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王银荣

    2012-01-01

    二维斑点追踪超声心动图(STE)是近年发展起来的一种超声新技术,是应变/应变率成像的一种方法.这种非侵入性诊断方法能够区分节段心肌的主动和被动运动,量化心肌内的收缩不同步,评估局部心肌功能,具有广阔的应用前景.现简要阐述STE的基本概念及其在定量评价扩张型心肌病的临床应用,并讨论该技术的局限性和发展前景.%Two-dimentional speckle tracking echocardiography( STE )is a new ultrasound method developed in recent years,which is a method of strain/strain rate imaging. This noninvasive diagnostic method can distinguish active myocardial movement from passive movement, quantify myocardial dyssynchrony, and assess partial myocardial function,which has promising application field. Here is to elaborate the basic concept of STE and its clinical application in evaluating dilated cardiomyopathy quantitatively, and discuss the limitations and development prospects of the technology.

  7. Study on Left Ventricular Systolic Function in Patients with Dilated Cardiomyopathy Using Doppler Tissue Imaging%多普勒组织成像对扩张型心肌病患者左室收缩功能的研究

    Institute of Scientific and Technical Information of China (English)

    白文伟; 章克信; 陆映珠; 汪丽琼; 冯震霞

    2004-01-01

    目的探讨多普勒组织成像(Doppler tissue imaging,DTI)评价扩张型心肌病(dilated cardiomyopathy,DCM)患者左室收缩功能的应用价值.方法脉冲DTI测定左室长轴切面室间隔(IVS)左室面、右室面及左室后壁(LVPW)内膜下心肌、外膜下心肌的收缩期运动峰值速度(Peak-S).计算左室面与右室面,内膜下心肌与外膜下心肌间的速度差(△V=peakS1-peakS2);心尖四腔切面测定二尖瓣环外侧收缩期运动峰值速度(Sa峰值速度).结果 DCM患者室壁收缩期运动峰值速度(peak-S)、内外膜间峰值速度差(△V)及Sa峰值速度较正常人明显降低,(P<0.01),二尖瓣侧环Sa峰值速度与LVE F高度相关(r=0.79,P<0.01).结论 DTI可作为评价DCM患者左室局部及整体收缩功能的新方法.

  8. Fatores prognósticos e evolução da função ventricular em 5 anos de seguimento da ventriculectomia parcial esquerda no tratamento da cardiomiopatia dilatada Prognostic factors in the follow-up of patients with idiopathic dilated cardiomyopathy submitted to partial left ventriculectomy

    Directory of Open Access Journals (Sweden)

    Luiz Felipe P. MOREIRA

    2001-12-01

    Full Text Available OBJETIVO: Nesta investigação, os resultados tardios da ventriculectomia parcial esquerda, associada à correção da insuficiência das valvas atrioventriculares, foram estudados em 43 pacientes portadores de cardiomiopatia dilatada. CASUÍSTICA E MÉTODOS: Os pacientes estavam em classe funcional III (18 ou IV (25 no pré-operatório, sendo que 7 pacientes foram operados na vigência de choque cardiogênico. A redução cirúrgica do volume do ventrículo esquerdo (VE foi associada à anuloplastia mitral em 32 pacientes e à substituição daquela valva em 3. Em 10 pacientes, também foi realizada plastia de valva tricúspide. Doze pacientes foram submetidos ao implante de desfibriladores automáticos. RESULTADOS: Ocorreram 9 (20,9% óbitos hospitalares. O tempo de seguimento pós-operatório variou entre dois e 68 meses, com média de 34,2 meses. Aos seis meses de seguimento, 8 pacientes estavam em classe funcional I, 13 em classe II, 3 em classe III e 1 em classe IV (pOBJECTIVE: Partial left ventriculectomy has been performed in patients with severe cardiomyopathies. The purpose of this investigation is to document the clinical effects of this procedure, associated with mitral insufficiency correction, in 43 patients with idiopathic dilated cardiomyopathy. METHODS: Eighteen patients were in New York Heart Association class III and 25 were in persistent class IV. Seven of these patients were operated on in cardiogenic shock. The procedure was associated with mitral anuloplasty in 32 patients and with mitral replacement in three. Ten patients were also submitted to De Vega tricuspid valve anuloplasty. Automatic cardioverter-defibrillators were implanted in 12 patients. RESULTS: Nine (20.9% patients died during the hospital period. The follow-up time ranged from two to 57 months, with a mean of 28.3 months. At six months of follow-up, eight patients were in functional class I, 13 patients in class II, three patients in class III e one patient

  9. Evaluation of dilated cardiomyopathy myocardial ’s motion function using two-dimensional speckle tracking displacement ima-ging%二维斑点追踪位移成像评价扩张型心肌病心肌运动时效性的研究

    Institute of Scientific and Technical Information of China (English)

    张文军; 郭智宇

    2014-01-01

    Objective To investigate clinical diagnosis value of dilated cardiomyopathy myocardial motion functions using two‐dimensional speckle tracking displacement imaging .Methods We quantitated longitudinal displacement and time to peak longitudinal displacement of left ventricular 17 segment through 45 normal persons and 32 patients with dilated car‐diomyopathy myocardial using GE Vivid E9 ultrasound unit and Echopac advanced workstation .Results LV longitudinal displacement was significantly decreasing from basal to apical segments in the normal group .The left ventricular segment displacement was greater in the normal group than DCM group with statistically significant difference .LV time to peak longitudinal displacement was located before AVC (aortic vavle colsed)in the normal group ,and after AVC in the DCM group;LV time to peak longitudinal displacement was more significantly delayed in the normal group than DCM group and there was a statistically significant difference between groups .Conclusion Two‐dimensional speckle tracking displacement imaging can provide new parameter for clinical evaluation of dilated cardiomyopathy myocardial motion functions .%目的:探讨二维斑点追踪位移成像在临床诊断扩张型心肌病心肌运动时效性中的价值。方法应用GE Vivid E9超声诊断仪及Echopac高级心血管分析工作站对45例正常人及32例扩张型心肌病患者的左心室心肌17节段长轴位移及位移达峰时间进行定量分析。结果正常组左心室长轴基底段位移最大,心尖段最小,差异有统计学意义;左心室长轴各节段位移达峰时间均位于AVC(主动脉瓣膜关闭)之前。扩心病组左心室长轴各节段位移均减低,心尖段位移小于基底段差异有统计学意义;左心室长轴大部分节段位移达峰时间均位于AVC之后。正常组左心室长轴位移均大于扩心病组并有统计学差异;两组长轴节段位移达峰时间均有统计学差

  10. Novel Phenotype–Genotype Correlations of Restrictive Cardiomyopathy With Myosin-Binding Protein C (MYBPC3) Gene Mutations Tested by Next-Generation Sequencing

    OpenAIRE

    Wu, Wei; Lu, Chao-Xia; Wang, Yi-Ning; Liu, Fang; Chen, Wei; Liu, Yong-Tai; Han, Ye-Chen; Cao, Jian; Zhang, Shu-Yang; Zhang, Xue

    2015-01-01

    Background MYBPC3 dysfunctions have been proven to induce dilated cardiomyopathy, hypertrophic cardiomyopathy, and/or left ventricular noncompaction; however, the genotype–phenotype correlation between MYBPC3 and restrictive cardiomyopathy (RCM) has not been established. The newly developed next-generation sequencing method is capable of broad genomic DNA sequencing with high throughput and can help explore novel correlations between genetic variants and cardiomyopathies. Methods and Results ...

  11. Inherited cardiomyopathies caused by troponin mutations

    Institute of Scientific and Technical Information of China (English)

    Qun-Wei Lu; Xiao-Yan Wu; Sachio Morimoto

    2013-01-01

    Genetic investigations of cardiomyopathy in the recent two decades have revealed a large number of mutations in the genes encoding sarcomeric proteins as a cause of inherited hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), or restrictive cardiomyopathy (RCM). Most functional analyses of the effects of mutations on cardiac muscle contraction have revealed significant changes in the Ca2+-regulatory mechanism, in which cardiac troponin (cTn) plays important structural and functional roles as a key regulatory protein. Over a hundred mutations have been identified in all three subunits of cTn, i.e., cardiac troponins T, I, and C. Recent studies on cTn mutations have provided plenty of evidence that HCM- and RCM-linked mutations increase cardiac myofilament Ca2+ sensitivity, while DCM-linked mutations decrease it. This review focuses on the functional consequences of mutations found in cTn in terms of cardiac myofilament Ca2+ sensitivity, ATPase activity, force generation, and cardiac troponin I phosphorylation, to understand potential molecular and cellular pathogenic mechanisms of the three types of inherited cardiomyopathy.

  12. Role of hepatitis C virus in myocarditis and cardiomyopathies

    Institute of Scientific and Technical Information of China (English)

    Akira Matsumori

    2004-01-01

    Recent nationwide clinico-epidemiological surveys in Japan showed that the occurrence of cardiomyopathies was most frequently seen in the age of sixties, and that cardiomyopathies are important causes of heart failure in the elderly. Viral infection was conventionally considered to cause myocarditis, which resulted in the development of dilated cardiomyopathy. Recent studies suggest that hepatitis C virus (HCV) is involved in the development of dilated cardiomyopathy, hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy in addition to myocarditis. Furthermore, left ventricular aneurysm represents the same morbid state not only after myocardial infarction but also after myocarditis. There were wide variations in the frequency of detection of HCV genomes in cardiomyopathy in different regions and in different populations. Major histocompatibility complex class Ⅱ genes may play a role in the susceptibility to HCV infection, and may influence the development of different phenotypes of cardiomyopathy. If in fact the myocardial damage is caused by HCV, it might be expected that interferon (IFN) administration would be useful for its treatment. Hepatitis patients receiving IFN treatment for hepatitis were screened by thallium myocardial scintigraphy, and an abnormality was discovered in half of the patients. Treatment with IFN resulted in a disappearance of the image abnormality. It has thus been suggested that mild myocarditis and myocardial damage may be cured with IFN. We have recently found that high concentrations of circulating cardiac troponin T are a specific marker of cardiac involvement in HCV infection. By measuring cardiac troponin T in patients with HCV infection, the prevalence of cardiac involvement in HCV infection will be clarified. We are proposing a collaborative work on a global network on myocarditis/cardiomyopathies due to HCV infection. (J Geriatr Cardiol 2004;1(2):83-89. )

  13. Children's Cardiomyopathy Foundation

    Science.gov (United States)

    Search The Children's Cardiomyopathy Foundation (CCF) is a national, non-profit organization focused on pediatric cardiomyopathy, a chronic disease of the heart muscle. CCF is dedicated to accelerating the search for ...

  14. A Treatable Cause of Cardiomyopathy: Vitamin D Deficiency

    Directory of Open Access Journals (Sweden)

    Erdal Eren

    2015-08-01

    Full Text Available Dilated cardiomyopathy is an important cause of heart failure in children. Medical therapy rarely results in complete improvement of the disease, treatment of which usually requires transplantation. Herein, we present a patient with cardiomyopathy and rickets. Case report: A 3-month-old boy was referred to Pediatric Endocrinology Clinic due to low calcium level. On his physical examination, enlarged wrists and large anterior fontanel were remarkable. Results of laboratory analyses revealed a calcium level of 6.8 mg/dL, phosphorus level of 4.9 mg/dL, alkaline phosphatase level of 1637 U/L, parathyroid hormone level of 191.2 pg/ mL, and 25-hydroxyvitamin D level of 5.7 ng/mL. Hand-wrist radiograph revealed signs consistent with rickets. Echocardiogram revealed dilated left ventricle, hypokinetic myocardium, an ejection fraction of 42%, and fractional shortening by 20%. Oral calcium lactate was started and then vitamin D treatment was added. At the 3rd month of the therapy, laboratory tests completely returned to normal and signs of rickets disappeared. Echocardiogram findings returned to normal. Since cardiac functions began to improve after the therapy, dilated cardiomyopathy associated with vitamin D deficiency was considered. Vitamin D deficiency should be considered while evaluating dilated cardiomyopathy in the regions that are endemic for nutritional rickets and it should be kept in mind that the therapy may provide dramatic improvement

  15. 联合使用培哚普利和万爽力辅助治疗扩张性心肌病心衰患者的疗效观察%Clinical Observation on Combination of Perindopril and Vasorel in Adjuvant Treatment of Dilated Cardiomyopathy with Heart Failure

    Institute of Scientific and Technical Information of China (English)

    周乃菁; 蒲鹏; 江洪

    2011-01-01

    目的 观察培哚普利、万爽力辅助治疗扩张性心肌病心力衰竭患者的临床疗效.方法 将75例患者随机分为3组,对照组(n=25)给予常规治疗,治疗组Ⅰ(n=25)在常规治疗的基础上加用培哚普利,治疗组Ⅱ(n=25)在治疗组Ⅰ的基础上再加用万爽力.结果 对照组总有效率为52%,治疗组Ⅰ总有效率为80%(P<0.05),治疗组Ⅱ总有效率为88%(P<0.01);治疗组Ⅰ、Ⅱ在治疗后左室收缩末期内径(LVSD)、舒张末期内径(LVDD)、左房内径(LAD)、室间隔厚度(IVST)均明显减小,左室射血系数(LVEF)显著增加,P<0.05,差异均有统计学意义.结论 联合使用培哚普利、万爽力辅助治疗可明显缓解扩张性心肌病心衰竭患者临床症状,还可显著改善左心结构与功能.%[ Objective ] To investigate the clinical effects of combination of Perindopril and Vasorel in adjuvant treatment of dilated cardiomyopathy with heart failure. [ Methods] 75 patients were randomly divided into three groups, the control group (n = 25) was treated with routine therapy, the treatment group Ⅰ ( n = 25) was given additional Perindopril on the basis of routine therapy, and the treatment group Ⅱ (n = 25) was given additional Vasorel on the basis of the treatment group Ⅰ therapy. [ Results] The total effective rate of the control group was 52% , that of the treatment group Ⅰ was 80% ( P < 0. 05 ), and that of the treatment group Ⅱ was 88%( P <0. 01 ). After treatment, the levels of left ventricular end-systolic dimension ( LVSD) , left ventricular end-diastolic diameter ( LVDD), left atrium diameter (LAD) and interventricular septal thickness (IVST) reduced significantly, level of left ventricular ejection fraction ( LVEF) increased significantly, and all of the differences were significant ( P < 0. 05 ). [ Conclusion ] The combination of Perindopril and Vasorel alleviate can significantly alleviate clinical symptoms of dilated cardiomyopathy with heart

  16. Assessment of radial movement of left ventricle with velocity vector imaging in patients with dilated cardiomyopathy%速度向量成像技术评价扩张型心肌病患者径向室壁运动

    Institute of Scientific and Technical Information of China (English)

    王玮; 施仲伟; 胡厚达; 许燕; 张凤如

    2010-01-01

    Objective To assess the radial systolic function of left ventricle(LV) in patients with dilated cardiomyopathy(DCM) by velocity vector imaging(VVI).Methods Sixteen patients with DCM and twenty control subjects were detected by VVI.VVI data were collected from the six basal segments and six mid segments in parastenal LV short axis views.The radial systolic velocity(V) ,strain(ε) ,strain rate(SR),the time to peak systolic velocity(PTV) and the time to maximum strain(PTε) were measured with special software.The differene of the earliest and the latest time to peak velocity(T-max) and the standard deviation of time to peak velocity(T-SD) of 12 segments were calculated.Results Compared to the controlled group,patients with DCM had significantly lower radial V,ε and SR (P <0.01) in all the 12 segments,significantly longer PTV and PTε (P < 0.05) in most segments, and significantly larger T-max and T-SD (P <0.05).Conclusions VVI is useful to assess the abnormalities in LV radial movement in patients with DCM and could provide more information about regional cardiac function.%目的 应用速度向量成像技术(velocity vector imaging,VVI)评价扩张型心肌病(dilated cardiomyopathy,DCM)径向局部心肌收缩功能和同步性.方法 16例DCM患者和20例对照者进行超声心动图检查,脱机分析左室短轴观中6个基底节段和6个中间节段共12个节段的径向收缩期峰值速度(V)、应变(ε)、应变率(SR)、径向速度达峰时间(PTV)、应变达峰时间(PTε),计算12节段的最早与最晚速度达峰时间差值(T-max)及速度达峰时间标准差(T-SD).结果 ①DCM组各节段的V、ε、SR的平均值均显著低于对照组相应节段(P<0.01);②DCM组的PTV除乳头肌水平前间隔及后间隔外,其余节段均大于对照组(P<0.05),PTε除前间隔二尖瓣水平、乳头肌水平和后间隔二尖瓣水平、乳头肌水平外,其余节段均显著延长(P<0.05);③DCM组的T-max

  17. Echomorphology of cardiomyopathy: review of 217 cases from 1999 to 2010

    International Nuclear Information System (INIS)

    Objective: To study echocardiogram features of different types of cardiomyopathy presenting over a 12 year period at a single centre in Peshawar. Methods: The series comprised a retrospective review of 13,788 consecutive echocardiograms carried out at the Muhammadi Hospital International Medical Research Centre, Hayatabad, Peshawar, from January 1999 to December 2010. Patients were split into two: Group I with paediatric and adolescent cases (0-18 years) and Group II with adults (>18 years). In the adult group, women with peripartum cardiomyopathy were subdivided into two groups of 18-30 years and 30 to 44 years. Standard Echo B and M modes and Doppler parameters were recorded to ascertain the diagnoses of common primary and secondary cardiomyopathies. Patients with myocarditis with chambers dilatation and global dysfunction, and cardiopathy associated with major cardiovascular diseases were excluded. SPSS 14 was used for statistical analysis. Results: Cardiomyopathy was diagnosed in 217 (1.57%) cases. There were 144 (66%) cases of dilated cardiomyopathy with a mean age of 13+-14.8 years; 17 (8%) cases of hypertrophic cardiomyopathy with a mean age of 12+-11.5 years; and 7 (3%) cases of restrictive cardiomyopathy with a mean age of 31+-7.8 years. Primary cardiac amyloidosis was confirmed in 9 (4%) cases, and peripartum cardiomyopathy in 25 (11%) females. Rare subtypes were found in 15 (7%) cases. Conclusion: DCM was the most frequently diagnosed subtype of cardiomyopathy followed by HCM in both the adult and paediatric age groups. (author)

  18. Use of I-123 MIBG cardiac scintigraphy to assess the impact of carvedilol on cardiac adrenergic neuronal function in childhood dilated cardiomyopathy; Interet de la scintigraphie cardiaque a l'I-123 MIBG pour evaluer l'impact du carvedilol sur la fonction neuronale adrenergique cardiaque dans les myocardiopathies dilatees de l'enfant

    Energy Technology Data Exchange (ETDEWEB)

    Maunoury, C. [Hopital Europeen Georges Pompidou (HEGP), Dept. de Physiologie et Radio-Isotopes, 75 - Paris (France); Acar, P. [Centre Hospitalier Universitaire, Service de Cardiologie Pediatrique, Hopital des Enfants, 31 - Toulouse (France); Sidi, D. [Centre Hospitalier Universitaire Necker-Enfants-Malades, 75 - Paris (France)

    2006-04-15

    I-123 MIBG cardiac scintigraphy is a useful tool to assess cardiac adrenergic neuronal function, which is impaired in children with dilated cardiomyopathy (DCM). In adults with DCM, long-term treatment with carvedilol improves both cardiac adrenergic neuronal function and left ventricular function. The aim of this prospective study was to evaluate the impact of carvedilol on cardiac adrenergic neuronal function and on left ventricular function in seventeen patients (11 female, 6 male, mean age 39 {+-} 57 months, range 1 - 168 months) with DCM. All patients underwent I-123 MIBG cardiac scintigraphy and equilibrium radio-nuclide angiography before and after a 6 month period of carvedilol therapy. A static anterior view of the chest was acquired 4 hours after intravenous injection of 20 to 75 MBq of I-123 MIBG. Cardiac neuronal uptake of I-123 MIBG was measured using the heart to mediastinum count ratio (HMR). Radionuclide left ventricular ejection fraction (LVEF) was assessed following a standard protocol. There was no major cardiac events (death or transplantation) during the follow-up period. I-123 MIBG cardiac uptake and left ventricular function respectively increased by 38% and 65% after 6 months of treatment with carvedilol (HMR 223 {+-} 49% vs 162 {+-} 26%, p < 0.0001 and LVEF = 43 {+-} 17% vs 26 {+-} 11%, p < 0.0001). Carvedilol can improve cardiac adrenergic neuronal function and left ventricular function in children with DCM. Further studies are needed to assess the relationship between improvement in I-123 MIBG cardiac uptake and the beneficial effects of carvedilol on morbidity and mortality. (authors)

  19. Smooth muscle LDL receptor-related protein-1 deletion induces aortic insufficiency and promotes vascular cardiomyopathy in mice.

    Directory of Open Access Journals (Sweden)

    Joshua E Basford

    Full Text Available Valvular disease is common in patients with Marfan syndrome and can lead to cardiomyopathy. However, some patients develop cardiomyopathy in the absence of hemodynamically significant valve dysfunction, suggesting alternative mechanisms of disease progression. Disruption of LDL receptor-related protein-1 (Lrp1 in smooth muscle cells has been shown to cause vascular pathologies similar to Marfan syndrome, with activation of smooth muscle cells, vascular dysfunction and aortic aneurysms. This study used echocardiography and blood pressure monitoring in mouse models to determine whether inactivation of Lrp1 in vascular smooth muscle leads to cardiomyopathy, and if so, whether the mechanism is a consequence of valvular disease. Hemodynamic changes during treatment with captopril were also assessed. Dilation of aortic roots was observed in young Lrp1-knockout mice and progressed as they aged, whereas no significant aortic dilation was detected in wild type littermates. Diastolic blood pressure was lower and pulse pressure higher in Lrp1-knockout mice, which was normalized by treatment with captopril. Aortic dilation was followed by development of aortic insufficiency and subsequent dilated cardiomyopathy due to valvular disease. Thus, smooth muscle cell Lrp1 deficiency results in aortic dilation and insufficiency that causes secondary cardiomyopathy that can be improved by captopril. These findings provide novel insights into mechanisms of cardiomyopathy associated with vascular activation and offer a new model of valvular cardiomyopathy.

  20. Evolução clínica e capacidade funcional de pacientes com cardiomiopatia dilatada após quatro anos do transplante Clinical and functional capacity of patients with dilated cardiomyopathy after four years of transplantation

    Directory of Open Access Journals (Sweden)

    Daniela Gardano Bucharles Mont'Alverne

    2012-12-01

    Full Text Available OBJETIVO: Avaliar a evolução do paciente miocardiopata após transplante (Tx cardíaco, analisando sua sobrevida, complicações trans e pós-operatórias e respostas cardiovasculares após cerca de quatro anos do procedimento cirúrgico. MÉTODOS: A pesquisa foi realizada no período de fevereiro a maio de 2011, com pacientes submetidos a Tx cardíaco no Hospital Dr. Carlos Alberto Studart Gomes - Hospital de Messejana (HDM. A amostra foi composta de todos os pacientes transplantados no ano de 2007 no referido hospital. Inicialmente, foi aplicada uma ficha de avaliação, coletando dados dos prontuários, sobre a evolução do paciente no período trans e pós-operatório até a alta hospitalar. Após a coleta dessas informações, os pacientes foram submetidos ao teste da caminhada dos seis minutos (TC6. Os valores encontrados na distância percorrida foram comparados aos valores de referência esperados para a população utilizando a equação de Enright e Sherrill. RESULTADOS: Do total de 24 pacientes que realizaram Tx cardíaco no HDM no ano de 2007, 14 foram avaliados e 10 excluídos do estudo. Com relação às complicações, no período transoperatório, a mais evidenciada foi a disfunção do ventrículo direito (64,3% e, no pós-operatório, quadro de taquicardia (64,3%. Analisando o TC6 observou-se diminuição de 11,6% na distância percorrida quando comparada à distância estimada (486 ± 55 m, 550 ± 59 m, respectivamente. CONCLUSÃO: Os resultados obtidos neste estudo perante o TC6 evidenciam que as respostas cardiovasculares dos pacientes avaliados estão abaixo do estimado, contudo dentro da faixa de normalidade estabelecida.OBJECTIVE: To evaluate patient with cardiomyopathy's progress after cardiac transplant, by analyzing his survival, complications and cardiovascular responses after nearly four years of surgery. METHODS: The survey was conducted from February to May 2011, with patients undergoing cardiac transplantation

  1. O papel da L-carnitina no estado nutricional e na evolução ecocardiográfica da cardiomiopatia dilatada idiopática da infância The role of L-carnitine in nutritional status and echocardiographic parameters in idiopathic dilated cardiomyopathy in children

    Directory of Open Access Journals (Sweden)

    Vitor M. P. Azevedo

    2005-10-01

    Full Text Available OBJETIVO: A desnutrição é marcadora independente de óbito na cardiomiopatia dilatada idiopática. Foi analisada a repercussão da introdução da L-carnitina nos parâmetros nutricionais e ecocardiográficos em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo prospectivo aberto de 11 crianças, comparadas com 40 controles, pareados para sexo e idade. Foi administrada L-carnitina oral (100 mg/kg/dia, além do tratamento padrão. Foram realizadas 118 pesagens no grupo L-carnitina e 264 nos controles, além de 65 ecocardiogramas no grupo L-carnitina e 144 nos controles. Análise estatística: qui-quadrado, teste t de Student, ANOVA e correlação de Pearson. Foi utilizado alfa = 0,05. RESULTADOS: Grupo L-carnitina: idade = 3,82 anos, 72,7% (p = 0,033 menores de 2 anos e do sexo feminino, e 90,9% (p = 0,001 em classe funcional III e IV. Não ocorreram óbitos no período. Não houve diferença no percentil de peso inicial (31,2±8,74 vs. 19,6±21,2 (p = 0,29 nem no índice z (-0,68±1,05 vs. -1,16±0,89 (p = 0,24. Ocorreu aumento do percentil (p = 0,026 e do índice z (p = 0,033 após a L-carnitina. Não houve diferença na fração de ejeção na apresentação (54,9%±3,8 vs. 49,3%±6,6 (p = 0,19, porém a massa VE/SC foi superior no grupo L-carnitina (169,12 g/m²±26,24 vs. 110,67 g/m²±15,62 (p = 0,0005. Após a L-carnitina, a ANOVA demonstrou aumento da fração de ejeção (48,3±7 para 67,2±7 (p = 0,044, e a massa do VE/SC foi reduzida (164,29g/m²±28,14 para 110,88g/m²±28,88, porém sem significância estatística (p = 0,089. CONCLUSÃO: Na cardiomiopatia dilatada idiopática na infância, a suplementação com L-carnitina pode auxiliar na recuperação nutricional e na melhora da fração de ejeção, facilitando a reversão do quadro de caquexia e da insuficiência cardíaca.OBJECTIVES: Malnutrition is an independent predictor of death in idiopathic dilated cardiomyopathy. An analysis was performed of the

  2. Clinical Effect of Combined Treatment of Kato Pury and Digoxin for Heart Failure Caused by Dilated Cardiomyopathy%卡托普利与地高辛联用治疗扩张型心肌病心衰的临床分析

    Institute of Scientific and Technical Information of China (English)

    万俊茹

    2015-01-01

    Objective To investigate the clinical effect of combined treatment of Kato Pury and digoxin for heart failure caused by dilated cardiomyopathy. Methods 40 patients with heart failure caused by dilated cardiomyopathy admitted to our hospital from November 2007 to November 2013 were selected as the research object and were randomly divided into experimental group and control group. Patients in the experiment group the combined treatment of Kato Pury and digoxin, while those in the control group were still given digoxin therapy. Clinical effects of the two groups were compared and analyzed. Results After treatment, in the ex-perimental group, the treatment is significant effect in 8 cases (40%), and effect in 10 cases (50%), and the total effective rate was 90%, while in the control group, the treatment is significant effect in 4 cases (20%) and effect in 10 cases (50%), and the total ef-fective rate was 70%, and the clinical effect of experimental group was obviously superior to that of the control group. After a course of treatment, the clinical n indexes of systolic blood pressure, diastolic blood pressure, heart rate and ejection fraction in the experimental group (112 ± 10.2) mmHg and (76 ± 8.6) mmHg and (84 ± 12.3) /min and (51 ± 4.6)% was superior to those in the control group, and there statistically significant difference between the two groups (P<0.05). Conclusion The curative effect of com-bined treatment of Kato Pury and digoxin in the treatment of patients with heart failure induced by dilated cardiomyopathy is obvi-ously superior than that of the single use of digoxin.%目的:探讨卡托普利与地高辛联用治疗扩张型心肌病致心衰的临床疗效。方法随机选择2007年11月—2013年11月收治的40例扩张型心肌病致心衰患者为实验对象,将患者随机分为实验组和对照组两组,实验组患者接受卡托普利与地高辛联用治疗,对照组仅接受地高辛治疗,对两组患者的临床治疗效

  3. 曲美他嗪对扩张型心肌病患者心房颤动发生率的影响%The influence to atrial fibrillation occurs of long -term use of trimetazidine in treatment of dilated cardiomy-opathy

    Institute of Scientific and Technical Information of China (English)

    匡素清; 姜燕飞; 迟增磊

    2015-01-01

    目的:探讨长期服用曲美他嗪对扩张型心肌病(DCM)患者心房颤动发生率的影响。方法收集扩张型心肌病患者90例,按照数字表法随机分为观察组和对照组,每组45例。对照组患者根据其心功能情况进行常规的抗心力衰竭治疗,观察组患者在进行常规的抗心力衰竭治疗的基础上,同时口服曲美他嗪,随访6个月后,比较两组患者治疗后左室射血分数(LVEF)、左室舒张末内径(LVEDD)、心功能改善情况,以及心房颤动发生率。结果治疗后,观察组心房颤动发生率为2.27%,明显低于对照组的11.11%(χ2=10.74, P <0.05);观察组心功能改善率为95.56%,明显高于对照组的82.22%(χ2=9.62,P <0.05);观察组 LVEDD为(52±9)mm,明显低于对照组的(63±11)mm(t =6.83,P <0.05);观察组 LVEF 为(54±7)%,明显高于对照组的(41±6)%(t =8.72,P <0.05)。结论扩张型心肌病患者长期服用曲美他嗪,可明显降低心房颤动发生率。%Objective To investigate the influence to atrial fibrillation occurs of long -term use of trimetazi-dine in treatment of dilated cardiomyopathy.Methods 90 cases with dilated cardiomyopathy (DCM)were averagely divided into the observation group and the control group according to a random number table,45 cases in each group. The patients of the control group were treated with conventional anti -heart failure according to their heart function, the patients of the observation group were given oral trimetazidine on the bases of conventional anti -heart failure. After follow -up six months,the left ventricular ejection fraction (LVEF),left ventricular end diastolic diameter (LVEDD),improve heart function,as well as the incidence of atrial fibrillation after treatment of two groups were compared.Results The rate of occurrence after treatment of the observation group atrial

  4. Assessment of the Left Ventricular Systolic Function in Dilated Cardiomyopathy Patients by Two -dimensional Speckle Tracking Imaging( 2D - STI)%二维斑点追踪成像技术评价扩张型心肌病患者左心室心肌纵向收缩功能

    Institute of Scientific and Technical Information of China (English)

    黄俊; 胡元平; 宋樟伟; 杨炜宇; 徐瑞; 倪显达

    2012-01-01

    Objective To assess the left ventricular systolic function in patients with dilated cardiomyopathy ( DCM ) . Methods 35 healthy subjects and 39 dilated cardiomyopathy patients underwent conventional echocardiography examination. Left atrial ( LA) diameter were measured by M - mode echocardiography, left ventricular( LV) end - systolic volume, end - diastolic volume and left ventricular ejection fraction (LVEF) were calculated by bi -plane Simpson's method. The peak velocity during early diastole(Ve) and late diastole (Va) of anterior mitral valve were measured by pulse -waved doppler, and the ratio Ve/Va was calculated. We acquired the apical four - chamber, two - chamber and the long - axis views of the left ventricular images in these patients with GE - Vivid7 - dimension. Then the peak longitudinal velocity, strain and strain rate in systolic period were measured and recorded. Results The values of LAD, LVESV and LVEDV in DCM patients were significantly higher than those of healthy subjects (P 0. 05 ) . The peak velocity in systolic period of the base and middle LV segments in DCM patients were lower than those of the healthy subjects (P < 0. 05). The peak longitudinal strain and strain rate were significantly lower than healthy subjects (P < 0. 01). The peak velocity of the healthy subjects and the DCM patients were descent from the base to the apex. Conclusion The peak velocity, stain and strain rate of regional myocardial function in long - axis of left ventricular can be analyzed by 2D - STI, and it is a feasible technique for the assessment of cardiac longitudinal systolic function in DCM patients, and it can be widely used in the cardiac examination.%目的 评价二维斑点追踪成像技术(2D - STI)在扩张型心肌病(dilated cardiomyopathy,DCM)患者的左心室心肌纵向收缩功能应用价值.方法 对39例DCM患者和35例正常对照组行常规超声心动图检查得到左心房内径(LAD)、左心室射血分数(LVEF)、过二尖瓣口

  5. RARE CASES OF HYPERTROPHIC CARDIOMYOPATHY: VARIANTS AND CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    V. Yu. Zimina

    2015-09-01

    Full Text Available Hypertrophic cardiomyopathy belongs to a group of hereditary diseases due to sarcomere gene mutation. This abnormality is characterized by the development of symmetric or asymmetric hypertrophy of left ventricular myocardium with its normal contractile function or hypercontractility. Authors provide a brief overview of variants of hypertrophic cardiomyopathy and phenocopies of this disease, when structural changes in the heart are not the result of classic sarcomere gene mutation. In patients with some phenocopies concentric left ventricular hypertrophy can transform into its dilatation with reduced contractility. Such variant of hypertrophic cardiomyopathy is presented in the first clinical observation. The second case shows that hypertrophic cardiomyopathy can be one of the symptoms of the disease with other reasons for poor outcome.

  6. Analysis of risk factor about ventricular remodeling of dilated cardiomyopathy with congestive heart failure%扩张型心肌病心室重构伴充血性心力衰竭的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    刘源; 唐其柱; 倪健

    2011-01-01

    To investigate the risk factor of ventricular remodeling (VRM) after dilated cardiomyop athy (DCM) with ventricular congestive heart failure (CHF). Method: 96 patients NYHA grade II-IIV with LVEF<40% were selected as observation group, and the rest 35 patients NYHA grade T with LVEF≥40% were selected as control group. Clinical data was compared. On the base of the single factor analysis, Logistic stepwise analysis was used to establish its independent risk factors. Result; Univariate analysis showed that com pared with the control group, male prevalence, atrial fibrillation, pulse pressureare ≥70 mmHg were significantly increased in observation group (P<0.01). Logistic stepwise analysis displayed that pulse pressureare ≥70 mmHg and atrial fibrillation were independent factors in observation group. Conclusion: Pulse pressureare ≥70 mmHg and atrial fibrillation are independent risk factors of VRM after DCM with CHF.%目的:探讨扩张型心肌病(DCM)后心室重构(VRM)伴充血性心力衰竭(CHF)的危险因素.方法:选择DCM患者NYHA分级Ⅱ~Ⅳ级,且左心室射血分数(LVEF)<40%的96例为观察组;心功能Ⅰ级,LVEF≥40%的35例患者为对照组,比较2组临床资料,在单因素分析基础上,采用Logistic多因素逐步分析方法确立DCM后VRM伴CHF的独立危险因素.结果:单因素分析结果显示,观察组中男性患病率、心房颤动(房颤)及脉压≥70 mmHg(1 mmHg=0.133 kPa)者较对照组高,差异有统计学意义(P<0.01).Logistic逐步回归分析脉压≥70 mmHg、房颤为DCM后VRM伴CHF的独立危险因素.结论:脉压≥70 mmHg、房颤是DCM后VRM伴CHF的独立危险因素.

  7. X-linked cardiomyopathy is heterogeneous

    Energy Technology Data Exchange (ETDEWEB)

    Wilson, M.J.; Sillence, D.O.; Mulley, J.C. [and others

    1994-09-01

    Two major loci of X-linked cardiomyopathy have been mapped by linkage analysis. The gene for X-linked dilated cardiomyopathy (XLCM) is mapped to the dystrophin locus at Xp21, while Barth syndrome has been localised to distal Xq28. XLCM usually presents in juvenile males with no skeletal disease but decreased dystrophin in cardiac muscle. Barth syndrome most often presents in infants and is characterized by skeletal myopathy, short stature and neutropenia in association with cardiomyopathy of variable severity. Prior to carrier or prenatal diagnosis in a family, delineation of the cardiomyopathy locus involved is essential. We report the linkage mapping of a large kindred in which several male infants have died with hypertrophic cardiomyopathy. There is a family history of unexplained death of infant males less than 6 months old over 4 generations. Features of Barth syndrome such as short stature, skeletal myopathy and neutropenia have not been observed. Genotyping at 10 marker loci in Xq28 has revealed significant pairwise lod scores with the cardiomyopathy phenotype at DXS52 (Z=2.21 at {theta}=0.0), at markers p26 and p39 near DXS15 (Z=2.30 at {theta}=0.0) and at F8C (Z=2.24 at {theta}=0.0). A recombinant detected with DXS296 defines the proximal limit to the localization. No recombinants were detected at any of the loci distal to DXS296. The most distal marker in Xq28, DXS1108, is within 500 kb of the telomere. As the gene in this family is localized to Xq28, it is possible that this disorder is an allelic variant at the Barth syndrome locus.

  8. Cardiomyopathy in congenital and acquired generalized lipodystrophy: a clinical assessment.

    Science.gov (United States)

    Lupsa, Beatrice C; Sachdev, Vandana; Lungu, Andreea O; Rosing, Douglas R; Gorden, Phillip

    2010-07-01

    Lipodystrophy is a rare disorder characterized by loss of adipose tissue and low leptin levels. This condition is characterized by severe dyslipidemia, insulin resistance, diabetes mellitus, and steatohepatitis. Another phenotypic feature that occurs with considerable frequency in generalized lipodystrophy is cardiomyopathy. We report here the cardiac findings in a cohort of patients with generalized congenital and acquired lipodystrophy, and present a literature review of the cardiac findings in patients with generalized lipodystrophy. We studied 44 patients with generalized congenital and acquired lipodystrophy, most of them enrolled in a clinical trial of leptin therapy. Patients underwent electrocardiograms and transthoracic echocardiograms to evaluate their cardiac status. We followed these patients for an extended time period, some of them up to 8 years. Evaluation of our cohort of patients with generalized lipodystrophy shows that cardiomyopathy is a frequent finding in this population. Most of our patients had hypertrophic cardiomyopathy, and only a small number had features of dilated cardiomyopathy. Hypertrophic cardiomyopathy was more frequent in patients with seipin mutation, a finding consistent with the literature. The underlying mechanism for cardiomyopathy in lipodystrophy is not clear. Extreme insulin resistance and the possibility of a "lipotoxic cardiomyopathy" should be entertained as possible explanations.

  9. Mutation Analysis of Mitochondrial tRNA Gene in Patients with Primary Dilated Cardiomyopathy%原发性扩张型心肌病的线粒体tRNA基因突变分析

    Institute of Scientific and Technical Information of China (English)

    李红超; 舒红英; 李晓杰; 倪斌; 谢海龙; 周海燕; 倪崖

    2015-01-01

    To identify the potential pathogenic mutations of mitochondrial tRNA in patients with primary dilated cardio-myopathy(DCM) and the possible association of the mutations with DCM.Paraffin-embedded myocardial tissues from two patients with DCM and 10 healthy controls,which were discarded after forensic examination,were used for the study.PCR amplification wasperformed for the mitochondrial tRNA genes and direct sequencing was conducted.Sequencing re-sults showed no variation for mitochondrial tRNA genes in the normal myocardial tissues.The tRNAVal G1664A variation and tRNAMet T4454C variation were identified in patients with DCM.These two variations were previously reported as polymorphism in MitoMap.There was no pathogenic mutation detected in mitochondrial tRNA genes of the two patients with DCM.A patient pool of large sample size is expected for analysis of the pathogenic mutations,polymorphism loci and haplogroup that might be associated with DCM.%为寻找原发性扩张型心肌病病例是否存在已知以及未知的线粒体tRNA致病性突变,以探讨扩张型心肌病可能的发病原因.收集2例原发性扩张型心肌病患者和10例正常对照尸检心肌组织石蜡标本,针对22种线粒体tRNA基因分别设计一对引物,PCR扩增后并测序分析线粒体tRNA基因突变情况.结果在对照样本中未检测到线粒体tRNA变异位点,在1例患者中检测到了tRNAVal基因G1664A变异,Mitomap已有报道为多态性位点;于另1例患者中检测到tRNAMetT4454C变异,有文章报道该位点与线粒体功能障碍有关,Mitomap报道为多态性位点.本研究中2例病例中未检测到线粒体tRNA致病性突变位点,可能与病例个体的心衰程度有关,有必要扩大样本量深入研究线粒体tRNA以及mtDNA其他基因突变与原发性扩张型心肌病之间的关系,以寻找可能的致病突变位点、易感的多态性位点或者单倍体群,为认识原发性扩张型心肌病的发病机制进一步提供理论基础和依据.

  10. Cardiovascular magnetic resonance in hypertrophic cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Shiozaki, Afonso Akio; Parga, Jose Rodrigues; Arteaga, Edmundo; Rochitte, Carlos Eduardo [Sao Paulo Univ. (USP), SP (Brazil). Instituto do Coracao. Setor de Tomografia Computarizada e Ressonancia Magnetica Cardiovascular]. E-mail: rochitte@incor.usp.br; Kim, Raymond J. [Duke Cardiovascular Magnetic Resonance Center, Durham, NC (United States); Tassi, Eduardo Marinho [Diagnosticos da America S.A., Rio de Janeiro, RJ (Brazil). Sector of Cardiovascular Magnetic Resonance and Computed Tomography

    2007-03-15

    Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiac disease that causes sudden death in young people, with an incidence of 1:500 adults. The routinely used criteria for worst prognosis have limited sensitivity and specificity. Thus, the estimated risk of evolving to dilated cardiomyopathy or sudden death is somewhat inaccurate, leading to management uncertainty of HCM patients. Therefore, an accurate noninvasive method for the diagnosis of HCM with prognostic value is of great importance. In the last years, Cardiovascular Magnetic Resonance (CMR) emerged not only as a diagnostic tool, but also as a study with prognostic values, by characterizing myocardial fibrosis with great accuracy in HCM patients. Additionally, CMR identifies the types of hypertrophy, analyses the ventricular function, estimates the intraventricular gradient and allows the determination of differential diagnosis. Moreover, CMR can uniquely access myocardial fibrosis in HCM. (author)

  11. Clinical Characteristics and Treatment of Cardiomyopathies in Children.

    Science.gov (United States)

    Price, Jack F; Jeewa, Aamir; Denfield, Susan W

    2016-01-01

    Cardiomyopathies are diseases of the heart muscle, a term introduced in 1957 to identify a group of myocardial diseases not attributable to coronary artery disease. The definition has since been modified to refer to structural and or functional abnormalities of the myocardium where other known causes of myocardial dysfunction, such as systemic hypertension, valvular disease and ischemic heart disease, have been excluded. In this review, we discuss the pathophysiology, clinical assessment and therapeutic strategies for hypertrophic, dilated and hypertrophic cardiomyopathies, with a particular focus on aspects unique to children.

  12. Unclassifiable arrhythmic cardiomyopathy associated with Emery-Dreifuss caused by a mutation in FHL1.

    Science.gov (United States)

    San Román, I; Navarro, M; Martínez, F; Albert, L; Polo, L; Guardiola, J; García-Molina, E; Muñoz-Esparza, C; López-Ayala, J M; Sabater-Molina, M; Gimeno, J R

    2016-08-01

    Emery-Dreifuss muscular dystrophy (EDMD) is a heterogeneous genetic disorder characterized by peripheral muscular weakness often associated with dilated cardiomyopathy. We characterize clinically a large family with a mutation in FHL1 gene (p.Cys255Ser). Penetrance was 44%, 100% for males and 18% for females. The heart was the main organ involved. Affected adult males had mild hypertrophy, systolic dysfunction and restriction with non-dilated ventricles. Carriers had significant QTc prolongation. The proband presented with resuscitated cardiac arrest. There were two transplants. Pathological study of explanted heart showed fibrofatty replacement and scarring consistent with arrhythmogenic cardiomyopathy and prominent left ventricular trabeculations. Myopathic involvement was evident in all males. Females had no significant neuromuscular disease. Mutations in FHL1 cause unclassifiable cardiomyopathy with coexisting EDMD. Prognosis is poor and systolic impairment and arrhythmias are frequent. Thrombopenia and raised creatine phosphokinase should raise suspicion of an FHL-1 disorder in X-linked cardiomyopathy. PMID:26857240

  13. Cardiomyopathy induced by pulmonary sequestration in a 50-year-old man.

    Science.gov (United States)

    Chatelain, Shaun; Comp, Robert A; Grace, R Randal; Sabbath, Adam M

    2015-02-01

    A 50-year-old black man presented at the emergency department with midsternal, nonradiating chest pressure and chronic dyspnea on exertion. Four years before the current admission, he had been diagnosed with nonischemic cardiomyopathy at another facility. After our complete evaluation, we suspected that his symptoms arose from left-to-left shunting in association with pulmonary sequestration, a congenital malformation. Our preliminary diagnosis of secondary dilated cardiomyopathy was confirmed by normalization of the patient's ventricular size and function after lobectomy. To our knowledge, this patient is the oldest on record to present with cardiomyopathy consequent to pulmonary sequestration. His case is highly unusual because of his age and the rapid resolution of his symptoms after lobectomy. We believe that pulmonary sequestration should be included in the differential diagnosis of dilated cardiomyopathy.

  14. A mutation in the human phospholamban gene, deleting arginine 14, results in lethal, hereditary cardiomyopathy

    OpenAIRE

    Haghighi, Kobra; Kolokathis, Fotis; Gramolini, Anthony O.; Waggoner, Jason R.; Pater, Luke; Lynch, Roy A.; Fan, Guo-Chang; Tsiapras, Dimitris; Parekh, Rohan R.; Dorn, Gerald W., II; MacLennan, David H.; Kremastinos, Dimitrios Th; Kranias, Evangelia G.

    2006-01-01

    The sarcoplasmic reticulum Ca2+-cycling proteins are key regulators of cardiac contractility, and alterations in sarcoplasmic reticulum Ca2+-cycling properties have been shown to be causal of familial cardiomyopathies. Through genetic screening of dilated cardiomyopathy patients, we identified a previously uncharacterized deletion of arginine 14 (PLN-R14Del) in the coding region of the phospholamban (PLN) gene in a large family with hereditary heart failure. No homozygous individuals were ide...

  15. Cardiomyopathy responsive to gluten withdrawal in a patient with coeliac disease.

    Science.gov (United States)

    McGrath, Samuel; Thomas, Angharad; Gorard, David Angelo

    2016-01-01

    A 57-year-old man with iron deficiency anaemia developed general malaise, exertional dyspnoea and features of cardiac failure out of proportion to his anaemia (haemoglobin 120 g/L). Investigations showed a severely dilated left ventricle with an ejection fraction of 15%, due to dilated cardiomyopathy. He was treated with high-dose diuretics, ACE inhibitors and β-blocker therapy. Subsequent investigation into his iron deficiency anaemia revealed a new diagnosis of coeliac disease. After starting a gluten-free diet, his cardiac function improved markedly, with ejection fraction reaching 70%, allowing his cardiac medications to be withdrawn. This case suggests a link between coeliac disease and cardiomyopathy. PMID:26976850

  16. Cardiovascular genetics : Technological advancements and applicability for dilated cardiomyopathy

    NARCIS (Netherlands)

    Kummeling, G. J M; Baas, A. F.; Harakalova, M.; van der Smagt, J. J.; Asselbergs, F. W.

    2015-01-01

    Genetics plays an important role in the pathophysiology of cardiovascular diseases, and is increasingly being integrated into clinical practice. Since 2008, both capacity and cost-efficiency of mutation screening of DNA have been increased magnificently due to the technological advancement obtained

  17. Dilated Cardiomyopathy and Myocardial Infarction Secondary to Congenital Generalized Lipodystrophy

    OpenAIRE

    Khalife, Wissam I.; Mourtada, Manal C.; Khalil, Jihad

    2008-01-01

    Congenital generalized lipodystrophy, also known as Berardinelli-Seip syndrome, is a very rare hereditary syndrome that is characterized by an almost complete absence of adipose tissue from birth. Cardiac involvement seems to have substantial influence in the long-term prognosis.

  18. RBM20, a gene for hereditary cardiomyopathy, regulates titin splicing

    OpenAIRE

    Guo, Wei; Schafer, Sebastian; Greaser, Marion L.; Radke, Michael H.; Liss, Martin; Govindarajan, Thirupugal; Maatz, Henrike; Schulz, Herbert; Li, Shijun; Parrish, Amanda M.; Dauksaite, Vita; Vakeel, Padmanabhan; Klaassen, Sabine; Gerull, Brenda; Thierfelder, Ludwig

    2012-01-01

    Alternative splicing plays a major role in the adaptation of cardiac function exemplified by the isoform switch of titin, which adjusts ventricular filling. We previously identified a rat strain deficient in titin splicing. Using genetic mapping, we found a loss-of-function mutation in RBM20 as the underlying cause for the pathological titin isoform expression. Mutations in human RBM20 have previously been shown to cause dilated cardiomyopathy. We showed that the phenotype of Rbm20 deficient ...

  19. Review of cardiomyopathy imaging

    Energy Technology Data Exchange (ETDEWEB)

    Gunaratnam, Kughan, E-mail: Kughan@hotmail.com [Monash Medical Centre, Southern Health, Melbourne (Australia); Wong, Lok Hun, E-mail: nuhkol@hotmail.com [Monash Medical Centre, Southern Health, Melbourne (Australia); Nasis, Arthur, E-mail: arthur.nasis@southernhealth.org.au [Monash Medical Centre, Southern Health, Melbourne (Australia); Ellims, Andris, E-mail: aellims@hotmail.com [The Alfred Hospital, 55 Commercial Road, Melbourne, VIC 3004 (Australia); Nandurkar, Dee, E-mail: nandurkar.dee@gmail.com [Monash Medical Centre, Southern Health, Melbourne (Australia); Soo, Geoffrey, E-mail: geoffrey.soo@southernhealth.org.au [Monash Medical Centre, Southern Health, Melbourne (Australia); Cameron, James, E-mail: james.cameron@monash.edu.au [Monash Medical Centre, Southern Health, Melbourne (Australia); Troupis, John, E-mail: john.troupis@southernhealth.org.au [Monash Medical Centre, Southern Health, Melbourne (Australia)

    2013-10-01

    Cardiomyopathies are increasingly being detected on both routine and non-routine imaging. Furthermore, the diagnosis of cardiomyopathy is changing from the traditional method of clinical presentation and cardiac morphology to a quantifiable method based on both cardiac morphology and function. With cardiac magnetic resonance imaging, coronary computed tomography and nuclear medicine increasingly being utilized along with echocardiography in the diagnostic process, it is important for the radiologist to be aware of the relevant criteria in formulating a diagnosis. We aim to provide an overview of the imaging characteristics of the most commonly encountered cardiomyopathies.

  20. Interação entre especialidades: miocardiopatia dilatada e neoplasia de mama HER2 positiva Interacción entre especialidades: miocardiopatía dilatada y neoplasia de mama HER2 positiva Interaction between specialties: dilated cardiomyopathy and HER2-positive breast Cancer

    Directory of Open Access Journals (Sweden)

    Solange Moraes Sanches

    2010-01-01

    en la miocardiopatía dilatada, de una forma muy interesante.Basic research may result in unexpected benefit in terms of progress in the understanding of mechanisms responsible for different diseases and their potential treatment alternatives. This is seen, for instance, when a specific situation, defined in clinical practice, may be translated into laboratory findings which suggest a new therapy for an unrelated disease, representing the inverse of the more usual bench-to-bedside path. During the past few years, the use of the monoclonal antibody trastuzumab, in the adjuvant and therapeutic context, has become of fundamental importance in the treatment of breast cancer with amplification/overexpression of HER2, resulting in significant increase in survival rates. The observation that trastuzumab also induces cardiotoxicity, and the identification of mechanisms involved in this side effect, have allowed the investigation of these factors as a therapeutic alternative for dilated cardiomyopathy, in a highly interesting fashion.

  1. 内皮祖细胞和碱性成纤维生长因子对扩张型心肌病大鼠心功能和血管新生的影响%The different effects of endothelial progenitor cells and bFGF on cardiac function and angiogenesis in dilated cardiomyopathy rats

    Institute of Scientific and Technical Information of China (English)

    张昕; 王德强; 郑玉云; 郭晓华

    2011-01-01

    Objective To compare the different effects of endothelial progenitor cells(EPCs) transplantation and basic fibroblast growth factor(bFGF) intramyocardial infusion on cardiac function of dilated cardiomyopathy(DCM) rats. Methods Fifty adult female rats received subcutaneous injection of isoprenaline for induction of DCM. Four weeks later,the model rats were randomly divided into EPCs group, bFGF group and control group of 12 rats each. Three months later , echocardiographic examination and regional myocardial blood flow(RMFM) measurement were performed. EPCs were traced by fluorescence in situ hybridization. The protein and mRNA expression of bFGF in each group was measured by ELISA assay and reverse transcription-polymerase chain reaction respectively. Results Three months after intramyocardial transplantation of EPCs,Sry gene positive cells were detected only in EPCs group,these cells were present in walls of new and original vessels. The cardiac function as well as RMFM were significantly improved in EPCs group and bFGF group,the improvement was more significant in EPCs group. There was higher capillary density,expression of protein and mRNA of bFGF in EPCs group compared with bFGF group and control group (P < 0.05). Conclusions Transplantation of EPCs in myocardium can improve cardiac function,induce neovascularization and increase RMFM in DCM rats. The treatment with EPCs has better efficacy than administration of bFGF alone.%目的 比较心肌内移植内皮祖细胞(EPCs)和注射碱性成纤维生长因子(bFGF)对扩张型心肌病大鼠心功能改善的影响.方法 50只雌性SD大鼠皮下注射异丙肾上腺素,制作扩张型心肌病模型.4周后,36只模型鼠随机分为EPCs组,bFGF组和对照组,每组12只.3个月后,心脏彩色超声评估心功能情况;彩色微球技术评估局部心肌血流量(RMFM);Y染色体原位杂交示踪移植的EPCs;采用逆转录PCR分析bFGF mRNA的表达,ELISA分析心肌组织

  2. The clinical study of relationship among different bundle bronch conduction with Doppler echocardiography ventricular arrhythmia in dilated cardiomyopathy%扩张型心肌病束支传导阻滞与心脏多普勒 超声指标、室性心律失常相关关系的研究

    Institute of Scientific and Technical Information of China (English)

    吴敏; 马守国; 曹玉珍; 张效东; 南静; 尹军; 袁慧敏; 石文芳

    2001-01-01

    Objective To study the relationship among different bundle bronch conduction with Doppler echocardiography,ventricular arrhythmia(VA) and other clinical materials in dilated cardiomyopathy(DCM).Methods 140 patients were divided into five groups according to different bundle bronch conduction in 12-leads electrocardiograme(groups CLBBB,CRBBB,CRBBB+LAFB,LAFB and normal bundle bronch conduction NBBC).By receivel clinical materials and measured parameters of Doppler echocardiography,24 hours ambulatory electrocardiograme before drug treatment in every group.The correlation between these data were assessed.Results Using statistic analyses showed that among different bundle bronch conduction groups in patients with DCM,the sick duration,the NYHA functional class,the heart thorax ratio,ther LV end-diastotic and end -systolic volume,the LV ejection fraction,the peak E velocity,E/A ratio and above I owns Ⅱ degree VA in groups CLBBB,CRBBB,CRBBB+LAFB were more serious than those in groups LAFB and NBBC.Conclusion Our data provide relevant information among different bundle bronch conduction groups,CLBBB、 CRBBB and CRBBB+LAFB are associated with the risk parameters of Doppler echocardiography,ventricular arrhythmia.%目的探讨扩张型心肌病(DCM)病人束支传导功能状态与心脏多普勒超声指标、室性心律失常的关系。方法根据140例DCM病人十二导体表心电图表现分为束支传导功能异常和束支传导功能正常5组,采集临床资料和心脏多普勒超声显像、24 h动态心电图、X线心脏摄片等检查结果进行组间统计学处理,评价束支传导功能状态与心脏多普勒超声指标、室性心律失常及其它临床指标的关系。结果 DCM束支传导异常组(CLBBB、CRBBB、CRBBB+LAFB)较束支传导功能正常组和单纯LAFB组NYHA心功能分级、X线心胸比、病程年限、心脏多普勒超声指标、Lown氏Ⅲ级以上室性心律失常发生率有显著统计学差异。

  3. Diurnal variation features of PR intervals in patients with dilated cardiomyopathy and heart failure%原发性扩张型心肌病心衰患者心电图PR间期昼夜变化特性的研究

    Institute of Scientific and Technical Information of China (English)

    汪丽; 蔡尚郎

    2012-01-01

    Objective: To study diurnal variation features of PR interval in dilated cardiomyopathy (DCM) patients with sinus rhythm and heart failure to provide evidence for reasonable regulating A - V interval and optimizing cardiac resynchronization therapy (CRT). Methods: A total of 58 DCM patients were enrolled as DCM group, including 20 cases with NYHA cardiac function class II , 19 cases with class IE and 19 cases with class W ; Another 20 normal subjects undergoing physical examination were regard as normal control group. Indexes of 24h dynamic electrocardiogram were measured in two groups. Results: Compared with normal control group, there were significant increase in daytime PR interval [ (142 ± 40. 33) ms vs. (163 ± 48. 9) ms] and nighttime PR interval [ (186 ± 49. 91) ms vs. (195 ± 51. 72) ms], and significant decrease in difference of PR interval between day and night [(44± 15. 37) ms vs. (32 ± 15. 72) ms] in DCM group (NYHA class I group as example), P<0. 05 all; As cardiac function aggravated, there were significant increase in daytime and nighttime PR interval, and significant decrease in difference of PR interval between day and night (P<0. 05 or <0. 01). Conclusion: In DCM patients with sinus rhythm and heart failure, there are significant increase in daytime and nighttime PR interval, significant decrease in difference of PR interval between day and night and they were more significant as cardiac function aggravates, indicating that in this time vagus nerve damage is more severe, optimizing CRT may significantly improve curative effects in these patients with NYHA class Ⅳ.%目的:探讨扩张型心肌病(DCM)窦性心律心衰患者PR间期昼夜变化规律及其意义,为A-V间期合理调整及心脏再同步化治疗(CRT)的优化方案提供依据.方法:入选原发性DCM患者58例(DCM组),其中NY-HAⅡ级20例、Ⅲ级19例、Ⅳ19例;另选体检正常者20例(正常对照组),测量各组24h动态心电图指标.结果:与正常对照

  4. V-plane显像与组织多普勒技术评价扩张型心肌病患者左心室收缩同步性的一致性研究%Evaluation of consistency between V-plane imaging and tissue Doppler imaging for systolic synchronicity in patients with dilated cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    宋宏宁; 周青; 秦真英; 陈金玲; 郭瑞强

    2014-01-01

    目的 应用V-plane技术观察扩张型心肌病(DCM)患者左心室收缩不同步显像,并与临床常用的组织多普勒显像(TDI)技术进行对比,评价其相关性与一致性.方法 对20例DCM患者及20例健康对照者进行超声检查,获取二维图像、V-plane双平面图像和TDI图像,测量左室12个节段TDI脉冲频谱达峰时间,并计算其标准差(TDI_SD),测量V-plane显像中12节段收缩位移达峰时间,并计算其标准差(V-plane_SD).结果 与对照组相比,DCM组的TDI SD和V-plane_SD增大,TDI达峰时间延迟,V-plane达峰时间缩短,差异有统计学意义(P<0.01);DCM组与对照组12节段中各节段V-plane收缩达峰时间均显著高于TDI收缩达峰时间,差异有统计学意义(P<0.01);V-plane_SD与TDI_SD具有显著相关性(r=0.925,P<0.001),Bland-Ahman分析显示TDI SD与V-plane_SD比值的95%数据点在一致性界限内,其比值的一致性界限为(0.50,1.36).结论 在评价DCM患者左室收缩同步性时V-plane技术与TDI技术具有较高的相关性及一致性,V-plane能克服TDI不能在同一心动周期显示12节段的局限性.%Objective To evaluate left ventricular systolic synchronicity in patients with dilated cardiomyopathy by V-plane imaging and compare with clinical commonly used tissue Doppler imaging (TDI),evaluate the relevance and consistency between these two parameters.Methods 20 patients diagnosed with DCM and 20 healthy controls were enrolled,2D images,V-plane imaging and TDI waveform were acquired.Time to peak velocity of left ventricular 12 segments were measured by TDI and the standard deviation (TDI_SD) were calculated.Displacement time to peak were measured by V-plane and the standard deviation (V-plane_SD) were calculated.Results Compared with control group,TDI_SD and V-plane_SD increased significantly (P < 0.01),TDI time to peak increased and V-plane time to peak decreased significantly(P <0.01).In the two group,12 segment time to peak measured by V

  5. Biventricular Takotsubo Cardiomyopathy

    Science.gov (United States)

    Daoko, Joseph; Rajachandran, Manu; Savarese, Ronald; Orme, Joseph

    2013-01-01

    Biventricular takotsubo cardiomyopathy is associated with more hemodynamic instability than is isolated left ventricular takotsubo cardiomyopathy; medical management is more invasive and the course of hospitalization is longer. In March 2011, a 62-year-old woman presented at our emergency department with abdominal pain, nausea, and vomiting. On hospital day 2, she experienced chest pain. An electrocardiogram and cardiac enzyme levels suggested an acute myocardial infarction. She underwent cardiac angiography and was found to have severe left ventricular systolic dysfunction involving the mid and apical segments, which resulted in a left ventricular ejection fraction of 0.10 to 0.15 in the absence of obstructive coronary artery disease. Her hospital course was complicated by cardiogenic shock that required hemodynamic support with an intra-aortic balloon pump and dobutamine. A transthoracic echocardiogram revealed akinesis of the mid-to-distal segments of the left ventricle and mid-to-apical dyskinesis of the right ventricular free wall characteristic of biventricular takotsubo cardiomyopathy. After several days of medical management, the patient was discharged from the hospital in stable condition. To the best of our knowledge, this is the first review of the literature on biventricular takotsubo cardiomyopathy that compares its hemodynamic instability and medical management requirements with those of isolated left ventricular takotsubo cardiomyopathy. Herein, we discuss the case of our patient, review the pertinent medical literature, and convey the prevalence and importance of right ventricular involvement in patients with takotsubo cardiomyopathy. PMID:23914028

  6. [Cardiomyopathies and pregnancy--how often, when to decide to terminate?].

    Science.gov (United States)

    Nowalany-Kozielska, Ewa

    2015-01-01

    Cardiomyopathies are a small percentage of heart disease in pregnant women, but usually cause severe complications. It is not know the exact incidence of cardiomyopathy associated with pregnancy in Europe. In these patients, there is a higher probability of death due to changes in hemodynamic, metabolic and hemostatic that occur in pregnancy. Pregnant mortality is 2.4% vs. 0.007%--in the normal population. The most common cause of severe maternal complications is peripartum cardiomyopathy (PPCM). In dilated cardiomyopathy (DCM) and restrictive (RCM) is usually observed significant clinical deterioration during pregnancy. On the other hand, in patients with hypertrophic cardiomyopathy (HCM), pregnancy and childbirth are often associated with a low risk of complications. There is a greater risk in women presenting symptoms before and on pregnancy and in women with large inrtaventricular and subaortic pressure gradient. There are only a few case reports of pregnancy in patients with rare storage diseases and infiltrative phenotype of hypertrophic cardiomyopathy. For these patients the pregnancy is contraindicated. In patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is sometimes the severity of arrhythmia in the third trimester of pregnancy, and childbirth (natural or cesarean section) is usually safe. Remember to informing women with various cardiomyopathies both the risks of pregnancy and about the possibility of transferring the disease to offspring. Contraception should be advised in many cases. PMID:26455023

  7. Animal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins.

    Science.gov (United States)

    Bang, Marie-Louise

    2017-01-01

    The cardiac Z-line at the boundary between sarcomeres is a multiprotein complex connecting the contractile apparatus with the cytoskeleton and the extracellular matrix. The Z-line is important for efficient force generation and transmission as well as the maintenance of structural stability and integrity. Furthermore, it is a nodal point for intracellular signaling, in particular mechanosensing and mechanotransduction. Mutations in various genes encoding Z-line proteins have been associated with different cardiomyopathies, including dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, restrictive cardiomyopathy, and left ventricular noncompaction, and mutations even within the same gene can cause widely different pathologies. Animal models have contributed to a great advancement in the understanding of the physiological function of Z-line proteins and the pathways leading from mutations in Z-line proteins to cardiomyopathy, although genotype-phenotype prediction remains a great challenge. This review presents an overview of the currently available animal models for Z-line and Z-line associated proteins involved in human cardiomyopathies with special emphasis on knock-in and transgenic mouse models recapitulating the clinical phenotypes of human cardiomyopathy patients carrying mutations in Z-line proteins. Pros and cons of mouse models will be discussed and a future outlook will be given. J. Cell. Physiol. 232: 38-52, 2017. © 2016 Wiley Periodicals, Inc. PMID:27171814

  8. Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies

    International Nuclear Information System (INIS)

    To analyse the value of cardiovascular magnetic resonance (CMR)-derived myocardial parameters to differentiate left ventricular non-compaction cardiomyopathy (LVNC) from other cardiomyopathies and controls. We retrospectively analysed 12 patients with LVNC, 11 with dilated and 10 with hypertrophic cardiomyopathy and compared them to 24 controls. LVNC patients had to fulfil standard echocardiographic criteria as well as additional clinical and imaging criteria. Cine steady-state free precession and late gadolinium enhancement (LGE) imaging was performed. The total LV myocardial mass index (LV-MMI), compacted (LV-MMIcompacted), non-compacted (LV-MMInon-compacted), percentage LV-MMnon-compacted, ventricular volumes and function were calculated. Data were compared using analysis of variance and Dunnett's test. Additionally, semi-quantitative segmental analyses of the occurrence of increased trabeculation were performed. Total LV-MMInon-compacted and percentage LV-MMnon-compacted were discriminators between patients with LVCN, healthy controls and those with other cardiomyopathies with cut-offs of 15 g/m2 and 25 %, respectively. Furthermore, trabeculation in basal segments and a ratio of non-compacted/compacted myocardium of ≥3:1 were criteria for LVNC. A combination of these criteria provided sensitivities and specificities of up to 100 %. None of the LVNC patients demonstrated LGE. Absolute CMR quantification of the LV-MMInon-compacted or the percentage LV-MMnon-compacted and increased trabeculation in basal segments allows one to reliably diagnose LVNC and to differentiate it from other cardiomyopathies. (orig.)

  9. New perspectives in Hypertrophic Cardiomyopathy

    NARCIS (Netherlands)

    M.J.M. Kofflard (Marcel)

    1998-01-01

    textabstractHypertrophic cardiomyopathy is a primary cardiac disorder with a heterogeneous expression. Although relatively uncommon, the disease has been studied extensively as appears from the numerous studies that have explored specific facets of hypertrophic cardiomyopathy. This review will focus

  10. Treatment of Chagas Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fernando A. Botoni

    2013-01-01

    Full Text Available Chagas' disease (ChD, caused by the protozoa Trypanosoma cruzi (T. cruzi, was discovered and described by the Brazilian physician Carlos Chagas in 1909. After a century of original description, trypanosomiasis still brings much misery to humanity and is classified as a neglected tropical disease prevalent in underdeveloped countries, particularly in South America. It is an increasing worldwide problem due to the number of cases in endemic areas and the migration of infected subjects to more developed regions, mainly North America and Europe. Despite its importance, chronic chagas cardiomyopathy (CCC pathophysiology is yet poorly understood, and independently of its social, clinical, and epidemiological importance, the therapeutic approach of CCC is still transposed from the knowledge acquired from other cardiomyopathies. Therefore, the objective of this review is to describe the treatment of Chagas cardiomyopathy with emphasis on its peculiarities.

  11. Tako-tsubo cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Yan Zhuang; Di Xu

    2009-01-01

    Tako-tsubo cardiomyopathy(TC) is a recently described acute cardiac syndrome, which the latest cardiomyopathy classification of the European Society of Cardiology describes as an unclassified cardiomyopathy. TC mimics acute myocardial infarction(AMI) and is characterised by ischaemic chest symptoms, an elevated electrocardiogram ST-segment, and moderately increased levels of cardiac disease markers. However, patients with TC have no coronary angiogram-detectable or non-obstructive coronary arterial disease(CAD), and left ventriculography documents transient left apical and middle ventricular wall dysfunction. In this review, we describe TC and evaluate epidemiological, clinical and instrumental features, pathophysiological mechanisms, therapy and prognosis of this syndrome, with a view to raising awareness of the disease.

  12. Cardiac MRI in a Patient with Coincident Left Ventricular Non-Compaction and Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Zahra Alizadeh-Sani

    2011-12-01

    Full Text Available Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults. Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular non-compaction and establish prognostic factors. We report a 47-year-old woman with a history of dilated cardiomyopathy with unknown etiology. Echocardiography showed mild left ventricular enlargement with severe systolic dysfunction (EF = 20-25%. According to cardiac magnetic resonance imaging findings non-compaction left ventricle with hypertrophic cardiomyopathy was considered, and right ventricular septal biopsy was recommended. Right ventricular endomyocardial biopsy showed moderate hypertrophy of cardiac myocytes with foci of myocytolysis and moderate interstitial fibrosis. No evidence of infiltrative deposition was seen.

  13. Arrhythmogenic right ventricular cardiomyopathy in two cats.

    Science.gov (United States)

    Harvey, A M; Battersby, I A; Faena, M; Fews, D; Darke, P G G; Ferasin, L

    2005-03-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease characterised by infiltration of the myocardium by adipose and fibrous tissue. The disease is an important cause of sudden death in humans, but has rarely been described in animals. This report describes ARVC in two cats with right-sided congestive heart failure. One cat had also experienced previous episodes of syncope. Standard six-lead and 24-hour (Holter) electrocardiogram recording revealed complete atrioventricular block and multiform ventricular ectopics in both cats, with the addition of ventricular tachycardia, ventricular bigeminy and R-on-T phenomenon in one of them. On echocardiography, the right ventricle and atrium were massively dilated and hypokinetic. The survival times of the cats were three days and 16 days following diagnosis. Histopathology in one case revealed fibro-fatty infiltration of the myocardium, predominantly affecting the right ventricular free wall. PMID:15789811

  14. 重组人生长激素对幼年扩张型心肌病模型大鼠心脏结构及功能的改善作用%Improvement in cardiac morphology and function in young rats with dilated cardiomyopathy by recombinant human growth hormone

    Institute of Scientific and Technical Information of China (English)

    成胜权; 强欢; 付蓉; 潘凯丽

    2015-01-01

    目的:观察重组人生长激素(rhGH)对幼年扩张型心肌病(DCM)模型大鼠左心室形态及功能的影响,以及其对DCM的改善效果及安全性。方法将60只Sprague-Dawley雄性大鼠随机分为空白对照组、DCM组和生长激素治疗组(rhGH组),每组20只。呋喃唑酮(0.25 mg/g)灌胃12周制作DCM模型;rhGH组大鼠给予rhGH腹腔注射(0.15 u/kg),每日1次,共12周,DCM组行等量生理盐水替代腹腔注射;空白对照组不采取任何措施。多普勒超声心动图检测心脏指数,ELISA法检测血清生化指标水平,多导生理仪检测血流动力学指标,普通光镜和电镜下观察各组大鼠心脏病理组织学改变,苦味酸-天狼猩红偏振光法染色检测心肌胶原蛋白水平。结果与对照组相比,DCM组大鼠均表现出明显心腔扩大,室壁厚度明显变薄,缩短分数(FS)和射血分数(EF)下降(P0.05)。DCM组大鼠各血清生化指标和血流动力学指标水平与对照组相比差异均有统计学意义(P0.05)。DCM组心肌胶原蛋白ColⅠ/ColⅢ比值高于对照组和rhGH组(P0.05)。结论 rhGH对幼年DCM模型大鼠左心室形态及心功能改善具有一定的作用。%ObjectiveTo investigate the effects of recombinant human growth hormone (rhGH) on the morphology and function of the left cardiac ventricle in young rats with dilated cardiomyopathy (DCM), and to evaluate the efifcacy and safety of rhGH in the treatment of DCM.MethodsSixty male Sprague-Dawley rats were randomly and equally assigned to control group, DCM group, and rhGH group. Furazolidone (0.25 mg/g) was given by gavage for 12 weeks to prepare the DCM model. Rats in the rhGH group received an intraperitoneal injection of rhGH (0.15 U/kg) once per day for 12 weeks, while rats in the DCM group received an equal volume of normal saline instead. Rats in the control group did not receive any treatment. Cardiac indices, serum

  15. Relationship among heart rate turbulence, QT dispersion and heart function in patients with dilated cardiomyopathy%扩张型心肌病窦性心率震荡及QT离散度与心功能变化的相关性

    Institute of Scientific and Technical Information of China (English)

    郭海鹏; 唐其柱; 邓伟; 周恒; 丘天翼; 严玲; 沈涤非

    2010-01-01

    Objective To explore the relationship among heart rate turbulence (HRT), QT dispersion (QTd) and heart function in patients with dilated cardiomyopathy (DCM) and assess its clinical value. Methods A total of 81 DCM patients with ventricular premature contraction (VPC) were divided into two groups according to heart function: Group A (NYHA class Ⅰ -Ⅱ , n = 34) and Group B (NYHA class Ⅲ-V, n =47). Thirty out-patient control cases were chosen from those undergoing regular physical examination. 24hour holter was performed to monitor turbulence onset ( TO ) and turbulence slope (TS).Electrocardiogram (ECG) was used to assess QTd. Meanwhile left ventricular ejection fraction ( LVEF),left ventricular end-diastolic dimension ( LVEDD), E and A-wave peak velocities, E/A were measured by echocardiogram. After a comparison of all indicators in each group, an investigation was conducted to discern the relationship among HRT, QTd and heart function. Results Compared with normal group, TO significantly increased in DCM A and B group: [0. 38 ( - 0.99 ~ 1.85 ) % vs 1.82 ( 0. 02 ~ 3.92 ) % vs ( -4. 03 ± 3.48 )%, P < 0. 01]. TS significantly decreased while QTd increased. The trend of QTd addition was apparent along with heart failure. TO was negatively correlated with LVEF ( r = -0. 701, P <0. 05 ) but positively correlated with LVEDD ( r =0. 621, P <0. 05 ). There was no correlation with E and A-wave peak velocities. TS and QTd also had an obvious correlation with LVEF and LVEDD ( all P < 0. 05 ) .Conclusions HRT is dramatically blunted in DCM patients and has a certain correlation with cardiac dysfunction. A combined test of HRT and QTd is a sensitive and indirect index in assessing autonomic nerve functions. It has a high clinical value of predicting the prognosis.%目的 探讨扩张型心肌病(DCM)患者窦性心率震荡(HRT)及QT离散度(QTa)与心功能变化的相关性及临床价值.方法 81例临床诊断DCM伴室性早搏患者,按心功能分级

  16. Cardiomyopathy Following Latrodectus Envenomation

    Directory of Open Access Journals (Sweden)

    Levine, Michael

    2010-12-01

    Full Text Available Latrodectus envenomations are common throughout the United States and the world. While many envenomations can result in catecholamine release with resultant hypertension and tachycardia, myocarditis is very rare. We describe a case of a 22- year-old male who sustained a Latrodectus envenomation complicated by cardiomyopathy. [West J Emerg Med. 2010; 11(5:521-523.

  17. Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes.

    Science.gov (United States)

    Akdis, Deniz; Brunckhorst, Corinna; Duru, Firat; Saguner, Ardan M

    2016-08-01

    This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes. At later stages, heart failure may occur. Diagnosis is established with the 2010 Task Force Criteria (TFC). Modern imaging tools are crucial for ACM diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting functional and structural alternations. Of note, structural findings often become visible after electrical alterations, such as premature ventricular beats, ventricular fibrillation (VF) and ventricular tachycardia (VT). 12-lead ECG is important to assess for depolarisation and repolarisation abnormalities, including T-wave inversions as the most common ECG abnormality. Family history and the detection of causative mutations, mostly affecting the desmosome, have been incorporated in the TFC, and stress the importance of cascade family screening. Differential diagnoses include idiopathic right ventricular outflow tract (RVOT) VT, sarcoidosis, congenital heart disease, myocarditis, dilated cardiomyopathy, athlete's heart, Brugada syndrome and RV infarction. Therapeutic strategies include restriction from endurance and competitive sports, β-blockers, antiarrhythmic drugs, heart failure medication, implantable cardioverter-defibrillators and endocardial/epicardial catheter ablation. PMID:27617087

  18. Phidippides cardiomyopathy: a review and case illustration.

    Science.gov (United States)

    Trivax, Justin E; McCullough, Peter A

    2012-02-01

    Phidippides was a Greek messenger who experienced sudden death after running more than 175 miles in two days. In today's world, marathon running and other endurance sports are becoming more popular and raising concern about sudden deaths at these events. Once etiologies such has hypertrophic cardiomyopathy, anomalous coronary arteries, and coronary atherosclerosis have been excluded, there is now an additional consideration termed Phidippides cardiomyopathy. Because endurance sports call for a sustained increase in cardiac output for several hours, the heart is put into a state of volume overload. It has been shown that approximately one-third of marathon runners experience dilation of the right atrium and ventricle, have elevations of cardiac troponin and natriuretic peptides, and in a smaller fraction later develop small patches of cardiac fibrosis that are the likely substrate for ventricular tachyarrhythmias and sudden death. Cardiac magnetic resonance imaging is emerging as the diagnostic test of choice for this condition. This review and case report summarizes the key features of this newly appreciated disorder.

  19. [Plasma selenium and peripartum cardiomyopathy in Bamako, Mali].

    Science.gov (United States)

    Cénac, A; Touré, K; Diarra, M B; Sergeant, C; Jobic, Y; Sanogo, K; Dembele, M; Fayol, V; Simonoff, M

    2004-01-01

    Peripartum heart failure due to unexplained dilated cardiomyopathy is a common disorder as Savannak-Sahelian Africa. One of the many suspected risk factors identified is selenium deficiency. The purpose of this study was to measure plasma selenium levels in patients with peripartum heart failure due to cardiomyopathy in Bamako, Republic of Mali and compare data with healthy Sahalian women with the same obstetrical status. Plasma selenium was measured in a patient group consisting of 28 Malian women presenting peripartum heart failure and in a control group of 28 healthy breast-feeding Nigerien women of comparable age. The criteria for matching the two groups was parity (similar number of deliveries) since multiparity is a risk factor for peripartum cardiomyopathy. The Wilcoxon test (nonparametric) was used to compare the 2 groups considering up value < 0.05 as significant. Plasma selenium was significantly lower in patients from Mali than in controls from Niger (65 +/- 17 ng/ml vs. 78 +/- 17 ng/ml, p = 0.01). The results of this study showing lower plasma selenium in Bamako patients with peripartum cardiomyopathy than in a matching healthy control population confirms the previous data from the Niamey study. PMID:15460143

  20. Genetics Home Reference: familial restrictive cardiomyopathy

    Science.gov (United States)

    ... Home Health Conditions familial restrictive cardiomyopathy familial restrictive cardiomyopathy Enable Javascript to view the expand/collapse boxes. ... All Open All Close All Description Familial restrictive cardiomyopathy is a genetic form of heart disease. For ...

  1. Dilation and Curettage (D&C)

    Science.gov (United States)

    ... For Patients About ACOG Dilation and Curettage (D&C) Home For Patients Search FAQs Dilation and Curettage ( ... February 2016 PDF Format Dilation and Curettage (D&C) Special Procedures What is dilation and curettage (D& ...

  2. Saw-tooth cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Karatza Ageliki A

    2009-12-01

    Full Text Available Abstract We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV, dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interventricular septum and lateral LV wall, along with areas of hypokinesis at the LV septum and apex in a noncoronary distribution, without any late gadolinium enhancement. We have termed this condition saw-tooth cardiomyopathy because of the very characteristic appearance.

  3. Saw-tooth cardiomyopathy

    OpenAIRE

    Karatza Ageliki A; Danias Peter G; Davlouros Periklis A; Kiaffas Maria G; Alexopoulos Dimitrios

    2009-01-01

    Abstract We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV), dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interve...

  4. Correction of human phospholamban R14del mutation associated with cardiomyopathy using targeted nucleases and combination therapy

    NARCIS (Netherlands)

    Karakikes, Ioannis; Stillitano, Francesca; Nonnenmacher, Mathieu; Tzimas, Christos; Sanoudou, Despina; Termglinchan, Vittavat; Kong, Chi-Wing; Rushing, Stephanie; Hansen, Jens; Ceholski, Delaine; Kolokathis, Fotis; Kremastinos, Dimitrios; Katoulis, Alexandros; Ren, Lihuan; Cohen, Ninette; Gho, Johannes M. I. H.; Tsiapras, Dimitrios; Vink, Aryan; Wu, Joseph C.; Asselbergs, Folkert W.; Li, Ronald A.; Hulot, Jean-Sebastien; Kranias, Evangelia G.; Hajjar, Roger J.

    2015-01-01

    A number of genetic mutations is associated with cardiomyopathies. A mutation in the coding region of the phospholamban (PLN) gene (R14del) is identified in families with hereditary heart failure. Heterozygous patients exhibit left ventricular dilation and ventricular arrhythmias. Here we generate i

  5. The mitochondrial DNA T16189C polymorphism and HIV-associated cardiomyopathy: a genotype-phenotype association study

    Directory of Open Access Journals (Sweden)

    Poulton Joanna

    2009-04-01

    Full Text Available Abstract Background The mitochondrial DNA (mtDNA T16189C polymorphism, with a homopolymeric C-tract of 10–12 cytosines, is a putative genetic risk factor for idiopathic dilated cardiomyopathy in the African and British populations. We hypothesized that this variant may predispose to dilated cardiomyopathy in people who are infected with the human immunodeficiency virus (HIV. Methods A case-control study of 30 HIV-positive cases with dilated cardiomyopathy and 37 HIV-positive controls without dilated cardiomyopathy was conducted. The study was confined to persons of black African ancestry to minimize confounding of results by population admixture. HIV-positive patients with an echocardiographically confirmed diagnosis of dilated cardiomyopathy and HIV-positive controls with echocardiographically normal hearts were studied. Patients with secondary causes of cardiomyopathy (such as hypertension, diabetes, pregnancy, alcoholism, valvular heart disease, and opportunistic infection were excluded from the study. DNA samples were sequenced for the mtDNA T16189C polymorphism with a homopolymeric C-tract in the forward and reverse directions on an ABI3100 sequencer. Results The cases and controls were well matched for age (median 35 years versus 34 years, P = 0.93, gender (males 60% vs 53%, P = 0.54, and stage of HIV disease (mean CD4 T cell count 260.7/μL vs. 176/μL, P = 0.21. The mtDNA T16189C variant with a homopolymeric C-tract was detected at a frequency of 26.7% (8/30 in the HIV-associated cardiomyopathy cases and 13.5% (5/37 in the HIV-positive controls. There was no significant difference between cases and controls (Odds Ratio 2.33, 95% Confidence Interval 0.67–8.06, p = 0.11. Conclusion The mtDNA T16189C variant with a homopolymeric C-tract is not associated with dilated cardiomyopathy in black African people infected with HIV.

  6. Framings and dilations

    OpenAIRE

    Larson, David R.; Szafraniec, Franciszek Hugon

    2013-01-01

    The notion of framings, recently emerging in P. G. Casazza, D. Han, and D. R. Larson, Frames for Banach spaces, in {\\em The functional and harmonic analysis of wavelets and frames} (San Antonio, TX, 1999), {\\em Contemp. Math}. {\\bf 247} (1999), 149-182 as generalization of the reconstraction formula generated by pairs of dual frames, is in this note extended substantially. This calls on refining the basic dilation results which still being in the flavor of {\\em th\\'eor\\`eme principal} of B. S...

  7. [The clinical practice guidelines of the Sociedad Española de Cardiología on cardiomyopathies and myocarditis].

    Science.gov (United States)

    Galve Basilio, E; Alfonso Manterola, F; Ballester Rodés, M; Castro Beiras, A; Fernández de Soria Pantoja, R; Penas Lado, M; Sánchez Domínguez, J

    2000-03-01

    Myocardial diseases are a extraordinarily heterogeneous group of processes that only have in common the fact that they involve heart muscle and that they cause a wide spectrum of myocardial dysfunction. The approach of the management and treatment of the cardiomyopathies is a continuous matter of discussion because the vast majority of alternatives in this field have not been based on the best scientific possible evidence and, since except for the case of heart failure associated with dilated cardiomyopathy. The majority of different options have not been studied by means of large (or even small) randomized trials. Nevertheless, this chapter has tried to provide the reader with different approaches on how to deal with important clinical problems in dilated, hypertrophic and restrictive cardiomyopathies, and in myocarditis as well. For this, we have utilized the most relevant information found coupled with our best clinical judgment, although we admit that many of the clinical recommendations can be controversial.

  8. Clinical Features and Echocardiographic Findings in Children with Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Cristina Blesneac

    2013-12-01

    Full Text Available Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%. Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background

  9. Dilated cardiomiopathy associated with celiac disease: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Milisavljević Nemanja

    2012-01-01

    Full Text Available Introduction. Celiac disease is an inflammatory condition of the small intestinal mucosa induced by gluten consumption in genetically susceptible individuals, leading to a spectrum of gastrointestinal presentation. A number of autoimmune and other disorders are highly associated with celiac disease. Cardiomyopathy associated with celiac disease has been rarely reported in the literature. Case Outline. We present a case of a 27-year-old male with one month history of diarrhea, weight loss, fatigue, dyspeptic symptoms, peripheral edema, and cardiac palpitations. After positive serological screening with immunoglobulin A anti-tissue transglutaminase antibody test, the diagnosis of celiac disease was confirmed with histopathology examination of duodenal biopsy specimen. Echocardiographic findings were consistent with acute myocarditis. After common causes of myocarditis had been excluded, probable celiac disease-associated autoimmune myocarditis was diagnosed. The patient was recommended to undergo a strict life-long gluten-free diet. IgA anti-transglutaminase antibodies, and anti-gliadin antibodies, were both significantly elevated during the 6-, 12- and 18-month follow-up. Low compliance to gluten-free diet in our patient led to progressive worsening of the left ventricular ejective fraction and other serious cardiac complications which warranted invasive cardiac interventions. Conclusion. Dilated cardiomyopathy associated with celiac disease is a serious condition which requires multidisciplinary approach involving gastroenterologist and cardiologist. Compliance with gluten-free diet is mandatory if patients are to avoid progression of cardiomyopathy. Screening of patients with idiopathic dilated cardiomyopathy for celiac disease is advisable.

  10. The Emerging Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Metabolic Cardiomyopathies.

    Science.gov (United States)

    Mavrogeni, S; Markousis-Mavrogenis, G; Markussis, V; Kolovou, G

    2015-08-01

    The aim of this review is to discuss the role of Cardiovascular Magnetic Resonance (CMR) in the diagnosis, risk stratification, and follow-up of metabolic cardiomyopathies. The classification of myocardial diseases, proposed by WHO/ISFC task force, distinguished specific cardiomyopathies, caused by metabolic disorders, into 4 types: 1) endocrine disorders, 2) storage or infiltration disorders (amyloidosis, hemochromatosis and familial storage disorders), 3) nutritional disorders (Kwashiorkor, beri-beri, obesity, and alcohol), and 4) diabetic heart. Thyroid disease, pheochromocytoma, and growth hormone excess or deficiency may contribute to usually reversible dilated cardiomyopathy. Glucogen storage diseases can be presented with myopathy, liver, and heart failure. Lysosomal storage diseases can provoke cardiac hypertrophy, mimicking hypertrophic cardiomyopathy and arrhythmias. Hereditary hemochromatosis, an inherited disorder of iron metabolism, leads to tissue iron overload in different organs, including the heart. Cardiac amyloidosis is the result of amyloid deposition in the heart, formed from breakdown of normal or abnormal proteins that leads to increased heart stiffness, restrictive cardiomyopathy, and heart failure. Finally, nutritional disturbances and metabolic diseases, such as Kwashiorkor, beri-beri, obesity, alcohol consumption, and diabetes mellitus may also lead to severe cardiac dysfunction. CMR, through its capability to reliably assess anatomy, function, inflammation, rest-stress myocardial perfusion, myocardial fibrosis, aortic distensibility, iron and/or fat deposition can serve as an excellent tool for early diagnosis of heart involvement, risk stratification, treatment evaluation, and long term follow-up of patients with metabolic cardiomyopathies. PMID:26197853

  11. Role of left ventricular twist mechanics in cardiomyopathies, dance of the helices

    Institute of Scientific and Technical Information of China (English)

    Floris; Kauer; Marcel; Leonard; Geleijnse; Bastiaan; Martijn; van; Dalen

    2015-01-01

    Left ventricular twist is an essential part of left ventricular function. Nevertheless, knowledge is limited in "the cardiology community" as it comes to twist mechanics. Fortunately the development of speckle tracking echocardiography, allowing accurate, reproducible and rapid bedside assessment of left ventricular twist, has boosted the interest in this important mechanical aspect of left ventricular deformation. Although the fundamental physiological role of left ventricular twist is undisputable, the clinical relevance of assessment of left ventricular twist in cardiomyopathies still needs to be established. The fact remains; analysis of left ventricular twist mechanics has already provided substantial pathophysiological understanding on a comprehensive variety of cardiomyopathies. It has become clear that increased left ventricular twist in for example hypertrophic cardiomyopathy may be an early sign of subendocardial(microvascular) dysfunction. Furthermore, decreased left ventricular twist may be caused by left ventricular dilatation or an extensive myocardial scar. Finally, the detection of left ventricular rigid body rotation in noncompaction cardiomyopathy may provide an indispensible method to objectively confirm this difficult diagnosis. All this endorses the value of left ventricular twist in the field of cardiomyopathies and may further encourage the implementation of left ventricular twist parameters in the "diagnostic toolbox" for cardiomyopathies.

  12. Bootstrapping Time Dilation Decoherence

    Science.gov (United States)

    Gooding, Cisco; Unruh, William G.

    2015-10-01

    We present a general relativistic model of a spherical shell of matter with a perfect fluid on its surface coupled to an internal oscillator, which generalizes a model recently introduced by the authors to construct a self-gravitating interferometer (Gooding and Unruh in Phys Rev D 90:044071, 2014). The internal oscillator evolution is defined with respect to the local proper time of the shell, allowing the oscillator to serve as a local clock that ticks differently depending on the shell's position and momentum. A Hamiltonian reduction is performed on the system, and an approximate quantum description is given to the reduced phase space. If we focus only on the external dynamics, we must trace out the clock degree of freedom, and this results in a form of intrinsic decoherence that shares some features with a proposed "universal" decoherence mechanism attributed to gravitational time dilation (Pikovski et al in Nat Phys, 2015). We note that the proposed decoherence remains present in the (gravity-free) limit of flat spacetime, emphasizing that the effect can be attributed entirely to proper time differences, and thus is not necessarily related to gravity. Whereas the effect described in (Pikovski et al in Nat Phys, 2015) vanishes in the absence of an external gravitational field, our approach bootstraps the gravitational contribution to the time dilation decoherence by including self-interaction, yielding a fundamentally gravitational intrinsic decoherence effect.

  13. Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies

    Energy Technology Data Exchange (ETDEWEB)

    Grothoff, Matthias; Lehmkuhl, Lukas; Gutberlet, Matthias [University of Leipzig - Heart Center, Department of Diagnostic and Interventional Radiology, Leipzig (Germany); Pachowsky, Milena [Klinik fuer Strahlenheilkunde, Charite, Campus Virchow-Klinikum, Berlin (Germany); Hoffmann, Janine [University of Leipzig, Department of Obstetrics, Leipzig (Germany); Posch, Maximilian [Department of Cardiothoracic Surgery, Deutsches Herzzentrum Berlin, Berlin (Germany); Klaassen, Sabine [Experimental and Clinical Research Center, Charite Medical Faculty and Max Delbrueck Center for Molecular Medicine, Berlin (Germany)

    2012-12-15

    To analyse the value of cardiovascular magnetic resonance (CMR)-derived myocardial parameters to differentiate left ventricular non-compaction cardiomyopathy (LVNC) from other cardiomyopathies and controls. We retrospectively analysed 12 patients with LVNC, 11 with dilated and 10 with hypertrophic cardiomyopathy and compared them to 24 controls. LVNC patients had to fulfil standard echocardiographic criteria as well as additional clinical and imaging criteria. Cine steady-state free precession and late gadolinium enhancement (LGE) imaging was performed. The total LV myocardial mass index (LV-MMI), compacted (LV-MMI{sub compacted}), non-compacted (LV-MMI{sub non-compacted}), percentage LV-MM{sub non-compacted}, ventricular volumes and function were calculated. Data were compared using analysis of variance and Dunnett's test. Additionally, semi-quantitative segmental analyses of the occurrence of increased trabeculation were performed. Total LV-MMI{sub non-compacted} and percentage LV-MM{sub non-compacted} were discriminators between patients with LVCN, healthy controls and those with other cardiomyopathies with cut-offs of 15 g/m{sup 2} and 25 %, respectively. Furthermore, trabeculation in basal segments and a ratio of non-compacted/compacted myocardium of {>=}3:1 were criteria for LVNC. A combination of these criteria provided sensitivities and specificities of up to 100 %. None of the LVNC patients demonstrated LGE. Absolute CMR quantification of the LV-MMI{sub non-compacted} or the percentage LV-MM{sub non-compacted} and increased trabeculation in basal segments allows one to reliably diagnose LVNC and to differentiate it from other cardiomyopathies. (orig.)

  14. Brain Abscess after Esophageal Dilatation

    DEFF Research Database (Denmark)

    Gaïni, S; Grand, M; Michelsen, J

    2007-01-01

    Brain abscess formation is a serious disease often seen as a complication to other diseases and to procedures. A rare predisposing condition is dilatation therapy of esophageal strictures. A case of brain abscess formation after esophageal dilatations is presented. A 59-year-old woman was admitted...... with malaise, progressive lethargy, fever, aphasia and hemiparesis. Six days before she had been treated with esophageal dilatation for a stricture caused by accidental ingestion of caustic soda. The brain abscess was treated with surgery and antibiotics. She recovered completely. This clinical case...... illustrates the possible association between therapeutic esophageal dilatation and the risk of brain abscess formation....

  15. Effects of mesenchymal stem cell transplantatiOn on cardiac function and structure and eIectrophysiology in rabbits with dilated cardiomyopathy%同种异体骨髓间充质干细胞移植对扩张型心肌病模型兔心功能与结构及电生理的影响

    Institute of Scientific and Technical Information of China (English)

    余更生; 沈兴; 田杰; 白永虹; 朱静; 刘官信; 陈沅

    2008-01-01

    :细胞移植组实验兔左室收缩末期内径较模型组减小,左室射血分数,左室短轴缩短率均高于模型组,差异有统计学意义(P<0.05).②心电生理检测结果:细胞移植组实验兔MAPDS0、MAPD90短于模型组,差异有统计学意义(P<0.05).③心肌组织学观察:模型组可见心肌细胞旱广泛的水肿和显著的空泡变性改变,电镜示心肌细胞线粒体肿胀,增生,肌节长短不一,出现异常收缩带,可见局部心肌溶解,细胞移植组移植区组织与之相比,各种受损表现较轻,且移植细胞有肌钙蛋白T和缝隙连接蛋白43的表达.结论:同种异体体内移植骨髓间充质干细胞后在一定程度上可改善扩张型心肌病模型兔心功能,减轻病理损害,并可能抑制心电紊乱的进一步发展.%BACKGROUND: The study of cell transplantation to repair injured cardiac muscle and improve cardiac function of dilated cardiomyopathy (DCM) has become a hotspot in recent years. However, the effect of cardiac electrophysiology following transplantation is still unknown.OBJECTIVE: To explore the influence of ailogenic implanted mesenchymal stem cells (MSCs) on cardiac function, structure and electrophysiology of rabbits with DCM.DESIGN, TIME AND SETTING: Randomized controlled animal trial was performed at Electrophysiology Laboratory of Institute of Pediatrics, Chongqing Medical University between January 2004 and May 2006.MATERIALS: Forty-three New Zealand whiterabbits, weighing 3.0-3.5 kg, irrespective of gender, were selected. Adriamycin was produced by Haizheng Medical Products Company of Zhejiang Province, China, No. 050307. The Ultrasonograph SSD-5000 came from Aloka, Japan, and RM6240 multiplying channel electrophysiolograph of Chengdu Instrument Company was applied.METHODS: All animals were randomized into normal group (n=12), cell transplantation group (n=13), and DCM model group (n=13). Except the normal group, adriamycin was applied to create rabbit DCM model, I mg/kg, twice a week for

  16. Role of neuropeptides in cardiomyopathies.

    Science.gov (United States)

    Dvorakova, Magdalena Chottova; Kruzliak, Peter; Rabkin, Simon W

    2014-11-01

    The role of neuropeptides in cardiomyopathy-associated heart failure has been garnering more attention. Several neuropeptides--Neuropeptide Y (NPY), vasoactive intestinal peptide (VIP), calcitonin gene related peptide (CGRP), substance P (SP) and their receptors have been studied in the various types of cardiomyopathies. The data indicate associations with the strength of the association varying depending on the kind of neuropeptide and the nature of the cardiomyopathy--diabetic, ischemic, inflammatory, stress-induced or restrictive cardiomyopathy. Several neuropeptides appear to alter regulation of genes involved in heart failure. Demonstration of an association is an essential first step in proving causality or establishing a role for a factor in a disease. Understanding the complexity of neuropeptide function should be helpful in establishing new or optimal therapeutic strategies for the treatment of heart failure in cardiomyopathies.

  17. Hypertrophic obstructive cardiomyopathy (HOCM

    Directory of Open Access Journals (Sweden)

    B. Pojskić

    2006-02-01

    Full Text Available Hypertrophic cardiomyopathy (HOCM is an autosomal dominant inherited disease of myocardium. We have presented a case of an asymptomatic female patient with HOCM discovered during family screening for HOCM. At the first examination she had all criteria for HOCM, including echocardiography. The next examination was performed two years later and clear echocardiography progression of disease was found. Pathological findings, including increasing interventricular septum thickness, mitral insufficiency, left ventricular outflow obstruction gradient and narrowing of LV, encouraged us to start the drug the treatment, although patient was clinicaly asymptomatic.

  18. FAST WATERSHED-BASED DILATION

    OpenAIRE

    Jakub Smołka

    2014-01-01

    A watershed-based region growing image segmentation algorithm requires a fast watershed-based dilation implementation for effective operation. This paper presents a new way for watershed image representation and uses this representation for effective implementation of dilation. Methods for improving the algorithm speed are discussed. Presented solutions may also be used for solving other problems where fast set summation is required.

  19. Coronary artery ectasia and systolic flow cessation in a patient with hypertrophic cardiomyopathy: a case report.

    Science.gov (United States)

    Zografos, Theodoros; Kokladi, Maria; Katritsis, Demosthenes

    2010-12-01

    Coronary artery ectasia (CAE) is characterized by diffuse or localized inappropriate dilation of coronary arteries and is often associated with slow coronary blood flow. Although CAE has been described to coexist with several clinical entities there are only three reports of CAE in the presence of hypertrophic cardiomyopathy (HCM). We report a case of CAE and slow coronary flow with systolic flow cessation in a 61-year old male with coronary artery disease and HCM.

  20. Nesprin-1 mutations in human and murine cardiomyopathy

    Science.gov (United States)

    Puckelwartz, Megan J.; Kessler, Eric J.; Kim, Gene; DeWitt, Megan M.; Zhang, Yuan; Earley, Judy U.; Depreux, Frederic F.S.; Holaska, James; Mewborn, Stephanie K.; Pytel, Peter; McNally, Elizabeth M.

    2009-01-01

    Mutations in LMNA, the gene encoding the nuclear membrane proteins, lamins A and C, produce cardiac and muscle disease. In the heart, these autosomal dominant LMNA mutations lead to cardiomyopathy frequently associated with cardiac conduction system disease. Herein, we describe a patient with the R374H missense variant in nesprin-1α, a protein that binds lamin A/C. This individual developed dilated cardiomyopathy requiring cardiac transplantation. Fibroblasts from this individual had increased expression of nesprin-1α and lamins A and C, indicating changes in the nuclear membrane complex. We characterized mice lacking the carboxy-terminus of nesprin-1 since this model expresses nesprin-1 without its carboxy-terminal KASH domain. These Δ/Δ KASH mice have a normally assembled but dysfunctional nuclear membrane complex and provide a model for nesprin-1 mutations. We found that Δ/Δ KASH mice develop cardiomyopathy with associated cardiac conduction system disease. Older mutant animals were found to have elongated P wave duration, elevated atrial and ventricular effective refractory periods indicating conduction defects in the myocardium, and reduced fractional shortening. Cardiomyocyte nuclei were found to be elongated with reduced heterochromatin in the Δ/Δ KASH hearts. These findings mirror what has been described from lamin A/C gene mutations and reinforce the importance of an intact nuclear membrane complex for a normally functioning heart. PMID:19944109

  1. Arrhythmogenic right ventricular cardiomyopathy, clinical manifestations, and diagnosis.

    Science.gov (United States)

    Haugaa, Kristina H; Haland, Trine F; Leren, Ida S; Saberniak, Jørg; Edvardsen, Thor

    2016-07-01

    This review aims to give an update on the pathogenesis, clinical manifestations, and diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). Arrhythmogenic right ventricular cardiomyopathy is mainly an autosomal dominant inherited disease linked to mutations in genes encoding desmosomes or desmosome-related proteins. Classic symptoms include palpitations, cardiac syncope, and aborted cardiac arrest due to ventricular arrhythmias. Heart failure may develop in later stages. Diagnosis is based on the presence of major and minor criteria from the Task Force Criteria revised in 2010 (TFC 2010), which includes evaluation of findings from six different diagnostic categories. Based on this, patients are classified as having possible, borderline, or definite ARVC. Imaging is important in ARVC diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting structural and functional abnormalities, but importantly these findings may occur after electrical alterations and ventricular arrhythmias. Electrocardiograms (ECGs) and signal-averaged ECGs are analysed for depolarization and repolarization abnormalities, including T-wave inversions as the most common ECG alteration. Ventricular arrhythmias are common in ARVC and are considered a major diagnostic criterion if originating from the RV inferior wall or apex. Family history of ARVC and detection of an ARVC-related mutation are included in the TFC 2010 and emphasize the importance of family screening. Electrophysiological studies are not included in the diagnostic criteria, but may be important for differential diagnosis including RV outflow tract tachycardia. Further differential diagnoses include sarcoidosis, congenital abnormalities, myocarditis, pulmonary hypertension, dilated cardiomyopathy, and athletic cardiac adaptation, which may mimic ARVC. PMID:26498164

  2. Microfibrillar cardiomyopathy: A rare case

    Directory of Open Access Journals (Sweden)

    Narender Kumar

    2011-01-01

    Full Text Available Microfibrillar cardiomyopathy is a very rare cause of restrictive cardiomyopathy (RCM. The index case was a male patient who presented with shortness of breath and pedal edema. Further clinical investigations favored a clinical diagnosis of RCM. An endomyocardial biopsy revealed subendocardial and interstitial hyaline eosinophillic material resembling amyloid that did not stain with Congo red. An electron microscopic examination showed that this material was composed of twisted linear and bundles of tangled microfibrils. The etiology of the microfibrillar deposition is currently unknown. The pathologists should entertain the diagnosis of microfibrillar cardiomyopathy in suspected cases of amyloidosis that are negative for Congo red.

  3. A Review of the Giant Protein Titin in Clinical Molecular Diagnostics of Cardiomyopathies.

    Science.gov (United States)

    Gigli, Marta; Begay, Rene L; Morea, Gaetano; Graw, Sharon L; Sinagra, Gianfranco; Taylor, Matthew R G; Granzier, Henk; Mestroni, Luisa

    2016-01-01

    Titin (TTN) is known as the largest sarcomeric protein that resides within the heart muscle. Due to alternative splicing of TTN, the heart expresses two major isoforms (N2B and N2BA) that incorporate four distinct regions termed the Z-line, I-band, A-band, and M-line. Next-generation sequencing allows a large number of genes to be sequenced simultaneously and provides the opportunity to easily analyze giant genes such as TTN. Mutations in the TTN gene can cause cardiomyopathies, in particular dilated cardiomyopathy (DCM). DCM is the most common form of cardiomyopathy, and it is characterized by systolic dysfunction and dilation of the left ventricle. TTN truncating variants have been described as the most common cause of DCM, while the real impact of TTN missense variants in the pathogenesis of DCM is still unclear. In a recent population screening study, rare missense variants potentially pathogenic based on bioinformatic filtering represented only 12.6% of the several hundred rare TTN missense variants found, suggesting that missense variants are very common in TTN and are frequently benign. The aim of this review is to understand the clinical role of TTN mutations in DCM and in other cardiomyopathies. Whereas TTN truncations are common in DCM, there is evidence that TTN truncations are rare in the hypertrophic cardiomyopathy (HCM) phenotype. Furthermore, TTN mutations can also cause arrhythmogenic right ventricular cardiomyopathy (ARVC) with distinct clinical features and outcomes. Finally, the identification of a rare TTN missense variant cosegregating with the restrictive cardiomyopathy (RCM) phenotype suggests that TTN is a novel disease-causing gene in this disease. Clinical diagnostic testing is currently able to analyze over 100 cardiomyopathy genes, including TTN; however, the size and presence of extensive genetic variation in TTN presents clinical challenges in determining significant disease-causing mutations. This review discusses the current

  4. Bootstrapping Time Dilation Decoherence

    CERN Document Server

    Gooding, Cisco

    2015-01-01

    We present a general relativistic model of a spherical shell of matter with a perfect fluid on its surface coupled to an internal oscillator, which generalizes a model recently introduced by the authors to construct a self-gravitating interferometer [1]. The internal oscillator evolution is defined with respect to the local proper time of the shell, allowing the oscillator to serve as a local clock that ticks differently depending on the shell's position and momentum. A Hamiltonian reduction is performed on the system, and an approximate quantum description is given to the reduced phase space. If we focus only on the external dynamics, we must trace out the clock degree of freedom, and this results in a form of intrinsic decoherence that shares some features with a proposed "universal" decoherence mechanism attributed to gravitational time dilation [2]. We show that the proposed decoherence remains present in the (gravity-free) limit of flat spacetime, indicating that the effect can be attributed entirely to ...

  5. New insights into cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Søren; Hove, Jens D; Dixen, Ulrik;

    2013-01-01

    Cirrhotic cardiomyopathy designates a cardiac dysfunction, which includes reduced cardiac contractility with systolic and diastolic dysfunction, and presence of electrophysiological abnormalities in particular prolongation of the QT interval. Several pathophysiological mechanisms including reduce...

  6. Tachycardia-induced Cardiomyopathy (Tachycardiomyopathy

    Directory of Open Access Journals (Sweden)

    Hassan A. Mohamed

    2007-01-01

    Full Text Available The term tachycardia-induced cardiomyopathy or tachycardiomyopathy refers to impairment in left ventricular function secondary to chronic tachycardia, which is partially or completely reversible once the tachyarrhythmia is controlled. Tachycardia-induced cardiomyopathy has been shown to occur both in experimental models and in patients with incessant tachyarrhythmia.Data from several studies and from case reports have shown that rate control by means of cardioversion, negative chronotropic agents, and surgical or catheter-based atrioventricular node ablation, resulted in significant improvement of systolic function.The diagnosis of tachycardia-induced cardiomyopathy is usually made following observation of marked improvement in systolic function after normalization of heart rate. Clinicians should be aware that patients with unexplained systolic dysfunction may have tachycardia-induced cardiomyopathy, and that controlling the arrhythmia may result in improvement of systolic function.

  7. Heart Failure with Multi-organ Thrombosis: A Case of Antiphospholipid Syndrome Co-existing with Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Xueqi Li and Shipeng Wei

    2012-09-01

    Full Text Available Antiphospholipid Syndrome (APS is an autoimmune disease featured by venous or arterial thrombosis, fetal losses and thrombocytopenia in the presence of antiphospholipid antibodies. Here we reported one case of antiphospholipid syndrome together with dilated cardiomyopathy. A 46-year-old female patient complaining short of breath was found enlargement of atrial and ventricular compartments. The ecletrocardiogram and blood test revealed anteroseptal myocardial infarction, while no pulmonary thrombosis was present and therefore diagnosis of dilated cardiomyopathy was made. There were also thrombi formed in the cardial chambers and deep venous. During hospitalization, there was an onset of ischemic brain stroke and head MRI showed newly developed small infarctions. An elevation of anticardiolipin immunoglobulin A (ACAIGA was detected from the blood sample. The patient was discharged after being treated with anticoagulant, corticosteroid and other medicines for improving heart function. In our case, APS is the basic cause leading to multi-organ thrombosis and heart failure is mainly due to dilated cardiomyopathy, thus independent of APS. So this is the first time that cardiomyopathy co-existing with APS was reported.

  8. Vessel dilatation in coronary angiograms

    International Nuclear Information System (INIS)

    Amongst 166 patients with aneurysms, ectasia or megaloarteries shown on coronary angiograms, 86.1% had dilated vessels as part of generalised coronary sclerosis (usually in patients with three-vessel disease). In 9%, dilatation was of iatrogenic origin and in 4.8% it was idiopathic. One patient had Marfan's syndrome. Amongst 9 000 patients, there were eight with megalo-arteries without stenosis; six of these had atypical angina and three suffered an infarct. Patients with definite dilatation of the coronary artery and stagnation of contrast flow required treatment. (orig.)

  9. Vessel dilatation in coronary angiograms

    Energy Technology Data Exchange (ETDEWEB)

    Hinterauer, L.; Goebel, N.

    1983-11-01

    Amongst 166 patients with aneurysms, ectasia or megaloarteries shown on coronary angiograms, 86.1% had dilated vessels as part of generalised coronary sclerosis (usually in patients with three-vessel disease). In 9%, dilatation was of iatrogenic origin and in 4.8% it was idiopathic. One patient had Marfan's syndrome. Amongst 9 000 patients, there were eight with megalo-arteries without stenosis; six of these had atypical angina and three suffered an infarct. Patients with definite dilatation of the coronary artery and stagnation of contrast flow required treatment.

  10. Familial neurofibromatosis and hypertrophic cardiomyopathy.

    OpenAIRE

    Fitzpatrick, A. P.; Emanuel, R W

    1988-01-01

    Two siblings from a family in which neurofibromatosis was inherited as an autosomal dominant had hypertrophic cardiomyopathy and neurofibromatosis. Idiopathic hypertrophic cardiomyopathy may have occurred by chance in two first degree relatives with neurofibromatosis. An alternative explantation is that these diseases are both manifestations of a common hereditary defect of neural crest tissue. Another possibility is that abnormalities of catecholamine metabolism and nerve growth factor in ne...

  11. Microvascular dysfunction in nonfailing arrhythmogenic right ventricular cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Paul, Matthias [University Hospital Muenster, Department of Cardiology and Angiology, Muenster (Germany); University Hospital Muenster, Institute for Genetics of Heart Diseases, Muenster (Germany); Rahbar, Kambiz; Kies, Peter; Schober, Otmar [University Hospital Muenster, Department of Nuclear Medicine, Muenster (Germany); Gerss, Joachim [University of Muenster, Institute of Biostatistics and Clinical Research, Muenster (Germany); Schaefers, Klaus; Schaefers, Michael [University of Muenster, European Institute for Molecular Imaging - EIMI, Muenster (Germany); Breithardt, Guenter [University Hospital Muenster, Department of Cardiology and Angiology, Muenster (Germany); Schulze-Bahr, Eric [University Hospital Muenster, Institute for Genetics of Heart Diseases, Muenster (Germany); Wichter, Thomas [Marienhospital Osnabrueck, Department of Cardiology, Niels-Stensen-Kliniken, Osnabrueck (Germany)

    2012-03-15

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a nonischaemic cardiomyopathy and leading cause of sudden death in the young. It has been shown that microvascular dysfunction reflected by an impaired myocardial blood flow (MBF) response to stress is present in patients with other forms of nonischaemic cardiomyopathy, e.g. dilated cardiomyopathy, and that the reduced MBF may be related to a poor prognosis. Therefore, we quantified MBF, coronary flow reserve and coronary vascular resistance in patients with nonfailing ARVC using H{sub 2}{sup 15} O and PET. In ten male patients with ARVC (mean age 49 {+-} 14 years), MBF was quantified at rest and during adenosine-induced hyperaemia using H{sub 2}{sup 15} O PET. Results were compared with those obtained in 20 age-matched healthy male control subjects (mean age 46 {+-} 14 years). Resting MBF was not significantly different between patients with ARVC and controls (MBF{sub rest} 1.19 {+-} 0.29 vs. 1.12 {+-} 0.20 ml/min/ml). However, hyperaemic MBF was significantly lower in patients with ARVC than in controls (2.60 {+-} 0.96 vs. 3.68 {+-} 0.84 ml/min/ml; p = 0.005). Consequently, patients with ARVC had a significantly lower coronary flow reserve than control subjects (2.41 {+-} 1.34 vs. 3.39 {+-} 0.93; p = 0.030). In addition, hyperaemic coronary vascular resistance was increased in patients with ARVC (36.79 {+-} 12.91 vs. 26.31 {+-} 6.49 mmHg x ml{sup -1} x min x ml; p = 0.007), but was found to be unchanged at rest. In this small well-characterized cohort of patients with nonfailing ARVC, we found a significantly reduced hyperaemic MBF and increased coronary vascular resistance. Further studies are necessary to corroborate this potential new functional aspect of the pathophysiological mechanisms underlying ARVC. (orig.)

  12. MELAS syndrome and cardiomyopathy: linking mitochondrial function to heart failure pathogenesis.

    Science.gov (United States)

    Hsu, Ying-Han R; Yogasundaram, Haran; Parajuli, Nirmal; Valtuille, Lucas; Sergi, Consolato; Oudit, Gavin Y

    2016-01-01

    Heart failure remains an important clinical burden, and mitochondrial dysfunction plays a key role in its pathogenesis. The heart has a high metabolic demand, and mitochondrial function is a key determinant of myocardial performance. In mitochondrial disorders, hypertrophic remodeling is the early pattern of cardiomyopathy with progression to dilated cardiomyopathy, conduction defects and ventricular pre-excitation occurring in a significant proportion of patients. Cardiac dysfunction occurs in approximately a third of patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, a stereotypical example of a mitochondrial disorder leading to a cardiomyopathy. We performed unique comparative ultrastructural and gene expression in a MELAS heart compared with non-failing controls. Our results showed a remarkable increase in mitochondrial inclusions and increased abnormal mitochondria in MELAS cardiomyopathy coupled with variable sarcomere thickening, heterogeneous distribution of affected cardiomyocytes and a greater elevation in the expression of disease markers. Investigation and management of patients with mitochondrial cardiomyopathy should follow the well-described contemporary heart failure clinical practice guidelines and include an important role of medical and device therapies. Directed metabolic therapy is lacking, but current research strategies are dedicated toward improving mitochondrial function in patients with mitochondrial disorders. PMID:26712328

  13. MELAS syndrome and cardiomyopathy: linking mitochondrial function to heart failure pathogenesis.

    Science.gov (United States)

    Hsu, Ying-Han R; Yogasundaram, Haran; Parajuli, Nirmal; Valtuille, Lucas; Sergi, Consolato; Oudit, Gavin Y

    2016-01-01

    Heart failure remains an important clinical burden, and mitochondrial dysfunction plays a key role in its pathogenesis. The heart has a high metabolic demand, and mitochondrial function is a key determinant of myocardial performance. In mitochondrial disorders, hypertrophic remodeling is the early pattern of cardiomyopathy with progression to dilated cardiomyopathy, conduction defects and ventricular pre-excitation occurring in a significant proportion of patients. Cardiac dysfunction occurs in approximately a third of patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, a stereotypical example of a mitochondrial disorder leading to a cardiomyopathy. We performed unique comparative ultrastructural and gene expression in a MELAS heart compared with non-failing controls. Our results showed a remarkable increase in mitochondrial inclusions and increased abnormal mitochondria in MELAS cardiomyopathy coupled with variable sarcomere thickening, heterogeneous distribution of affected cardiomyocytes and a greater elevation in the expression of disease markers. Investigation and management of patients with mitochondrial cardiomyopathy should follow the well-described contemporary heart failure clinical practice guidelines and include an important role of medical and device therapies. Directed metabolic therapy is lacking, but current research strategies are dedicated toward improving mitochondrial function in patients with mitochondrial disorders.

  14. Dilatancy in Slow Granular Flows

    Science.gov (United States)

    Kabla, Alexandre J.; Senden, Tim J.

    2009-06-01

    When walking on wet sand, each footstep leaves behind a temporarily dry impression. This counterintuitive observation is the most common illustration of the Reynolds principle of dilatancy: that is, a granular packing tends to expand as it is deformed, therefore increasing the amount of porous space. Although widely called upon in areas such as soil mechanics and geotechnics, a deeper understanding of this principle is constrained by the lack of analytical tools to study this behavior. Using x-ray radiography, we track a broad variety of granular flow profiles and quantify their intrinsic dilatancy behavior. These measurements frame Reynolds dilatancy as a kinematic process. Closer inspection demonstrates, however, the practical importance of flow induced compaction which competes with dilatancy, leading more complex flow properties than expected.

  15. Multisensory signalling enhances pupil dilation

    OpenAIRE

    Silvia Rigato; Gerulf Rieger; Vincenzo Romei

    2016-01-01

    Detecting and integrating information across the senses is an advantageous mechanism to efficiently respond to the environment. In this study, a simple auditory-visual detection task was employed to test whether pupil dilation, generally associated with successful target detection, could be used as a reliable measure for studying multisensory integration processing in humans. We recorded reaction times and pupil dilation in response to a series of visual and auditory stimuli, which were prese...

  16. Post-earthquake dilatancy recovery

    Science.gov (United States)

    Scholz, C. H.

    1974-01-01

    Geodetic measurements of the 1964 Niigata, Japan earthquake and of three other examples are briefly examined. They show exponentially decaying subsidence for a year after the quakes. The observations confirm the dilatancy-fluid diffusion model of earthquake precursors and clarify the extent and properties of the dilatant zone. An analysis using one-dimensional consolidation theory is included which agrees well with this interpretation.

  17. Genetics Home Reference: arrhythmogenic right ventricular cardiomyopathy

    Science.gov (United States)

    ... Genetics Home Health Conditions ARVC arrhythmogenic right ventricular cardiomyopathy Enable Javascript to view the expand/collapse boxes. ... Open All Close All Description Arrhythmogenic right ventricular cardiomyopathy ( ARVC ) is a form of heart disease that ...

  18. Mitochondrial 12S Ribosomal RNA A1555G Mutation Associated with Cardiomyopathy and Hearing Loss following High-Dose Chemotherapy and Repeated Aminoglycoside Exposure

    DEFF Research Database (Denmark)

    Skou, Anne-Sofie; Tranebjærg, Lisbeth; Jensen, Tim;

    2014-01-01

    A 19-month-old girl with the A1555G mitochondrial mutation in the 12S ribosomal RNA gene and acute myelogenous leukemia developed dilated cardiomyopathy and bilateral sensorineural hearing loss before undergoing allogeneic stem cell transplantation. She had received gentamicin during episodes of ...

  19. [Levosimendan for septic shock with takotsubo cardiomyopathy].

    Science.gov (United States)

    Schlürmann, C-N; Reinöhl, J; Kalbhenn, J

    2016-01-01

    As a stress-induced disease, takotsubo cardiomyopathy can also occur in septic syndromes; however, the hemodynamic management is fundamentally different from the treatment approaches for classical septic cardiomyopathy, as beta mimetics can increase the heart failure symptoms in takotsubo cardiomyopathy. This article reports the case of an 82-year-old female patient who presented with acute abdomen due to adhesion ileus and takotsubo cardiomyopathy, developed severe septic shock with peritonitis and could be successfully hemodynamically stabilized with levosimendan.

  20. Endothelial function in pre-pubertal children at risk of developing cardiomyopathy: a new frontier

    Directory of Open Access Journals (Sweden)

    Aline Cristina Tavares

    2012-01-01

    Full Text Available Although it is known that obesity, diabetes, and Kawasaki's disease play important roles in systemic inflammation and in the development of both endothelial dysfunction and cardiomyopathy, there is a lack of data regarding the endothelial function of pre-pubertal children suffering from cardiomyopathy. In this study, we performed a systematic review of the literature on pre-pubertal children at risk of developing cardiomyopathy to assess the endothelial function of pre-pubertal children at risk of developing cardiomyopathy. We searched the published literature indexed in PubMed, Bireme and SciELO using the keywords 'endothelial', 'children', 'pediatric' and 'infant' and then compiled a systematic review. The end points were age, the pubertal stage, sex differences, the method used for the endothelial evaluation and the endothelial values themselves. No studies on children with cardiomyopathy were found. Only 11 papers were selected for our complete analysis, where these included reports on the flow-mediated percentage dilatation, the values of which were 9.80±1.80, 5.90±1.29, 4.50±0.70, and 7.10±1.27 for healthy, obese, diabetic and pre-pubertal children with Kawasaki's disease, respectively. There was no significant difference in the dilatation, independent of the endothelium, either among the groups or between the genders for both of the measurements in children; similar results have been found in adolescents and adults. The endothelial function in cardiomyopathic children remains unclear because of the lack of data; nevertheless, the known dysfunctions in children with obesity, type 1 diabetes and Kawasaki's disease may influence the severity of the cardiovascular symptoms, the prognosis, and the mortality rate. The results of this study encourage future research into the consequences of endothelial dysfunction in pre-pubertal children.

  1. Miocardiopatia dilatada em pacientes com infecção chagásica crônica: relato de dois casos fatais autóctones do Rio Negro, Estado do Amazonas Dilated cardiomyopathy in patients with chronic chagasic infection: report of two fatal autochthonous cases from Rio Negro, State of Amazonas, Brazil

    Directory of Open Access Journals (Sweden)

    Pedro Viñas Albajar

    2003-06-01

    Full Text Available Dois casos fatais de miocardiopatia chagásica crônica dilatada são relatados pela primeira vez em pacientes autóctones do rio Negro, Estado do Amazonas. Ambos os casos, um homem de 45 anos de idade e uma mulher de 44, nasceram e viveram toda a vida na região do Rio Negro, no norte do estado do Amazonas, tendo sido picados numerosas vezes por triatomineos silvestres em piaçabais da área. Os pacientes que tiveram as reações sorológicas positivas para anticorpos anti-Trypanosoma cruzi (imunofluorescência, ELISA e Wertern blot desenvolveram nos últimos 5-7 anos um quadro de insuficiência cardíaca progressivo, com aumento global da área cardíaca, bloqueios atrioventricular e de ramo esquerdo e extrassístoles ventriculares, faleceram de insuficiência cardíaca irreversível. Um dos casos em que foi feita a biópsia cardíaca cirúrgica pós-mortem, mostrou na histopatologia, miocardite crônica com infiltrado mononuclear difuso, com áreas de adensamento celular, fibrose, dissociação, fragmentação e hialinização de fibras cardíacas, sugestivo de miocardite chagásica crônica e o PCR in situ foi positivo para Trypanosoma cruzi.Two fatal cases of human chronic chagasic cardiomyopathy are reported, for the first time, in autochthonous patients from Rio Negro, Amazonas state. Both cases, (45 year old man and 44 year old woman who were born and lived their whole lives in the Rio Negro region, in the northern part of the state of Amazonas, reported having been bitten several times by triatomine bugs in their camping huts while gathering piaçava fibers. The patients, who had confirmed positive serology for Trypanosoma cruzi antibodies (indirect immunofluorescence, ELISA and Western blot, developed in the last 5-7 years a progressive cardiac failure, with global enlargement of the heart, atrioventricular and left bundle branch block, ventricular extrasystoles, both dying from irreversible cardiac insufficiency. The histopathology of

  2. Pathogenesis of Arrhythmogenic Cardiomyopathy.

    Science.gov (United States)

    Asimaki, Angeliki; Kleber, Andre G; Saffitz, Jeffrey E

    2015-11-01

    Arrhythmogenic cardiomyopathy (ACM) is a primary myocardial disease. It is characterized by frequent ventricular arrhythmias and increased risk of sudden cardiac death typically arising as an early manifestation before the onset of significant myocardial remodelling. Myocardial degeneration, often confined to the right ventricular free wall, with replacement by fibrofatty scar tissue, develops in many patients. ACM is a familial disease but genetic penetrance can be low and disease expression is highly variable. Inflammation might promote disease progression. It also appears that exercise increases disease penetrance and accelerates its development. More than 60% of probands harbour mutations in genes that encode desmosomal proteins, which has raised the possibility that defective cell-cell adhesion might play a role in disease pathogenesis. Recent advances have implicated changes in the canonical wingless-type mouse mammary tumour virus integration site (Wnt)/β-catenin and Hippo signalling pathways and defects in forwarding trafficking of ion channels and other proteins to the intercalated disk in cardiac myocytes. In this review we summarize the current understanding of the pathogenesis of ACM and highlight future research directions. PMID:26199027

  3. [Specific dilated myocardiopathy. Chronic chagasic cardiopathy at the National Institute of Cardiology Ignacio Chávez].

    Science.gov (United States)

    Monteón-Padilla, Víctor Manuel; Vargas-Alarcón, Gilberto; Vallejo-Allende, Maité; Reyes, Pedro A

    2002-01-01

    Cardiomyopathies are a heterogenous group of heart ailments. Some of them are primary myocardial diseases and are classified as dilated, hypertrophic, restrictive and arryhithmogenic. Dilated cardiomyopathies (DCs) are the most common. Sometimes it is possible to identify an etiologic agent, in that case we talk about a specific dilated cardiomyopathy. Here in, we review one of these specific DCs, the so called Chronic Chagasic Cardiopathy (CCC) from the point of view of our personal experience at the Instituto Nacional de Cardiología "Ignacio Chávez". Chagas' disease is present in Mexico, therefore CCC is also present. We estimate that 5,000 people, suffer CCC with severe symptoms. In Mexico, Chagas' disease occurs below the Tropic of Cancer and between 2,000-2,500 m above sea level, in this area there is a real risk for vectorial infection, mainly in rural villages. Clinical diagnosis should be supported by epidemiological and seroepidemiological confirmatory data. There is not appropriate therapy yet for this condition.

  4. Genetic biomarkers in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Coats, Caroline J; Elliott, Perry M

    2013-08-01

    Hypertrophic cardiomyopathy is a common inherited heart muscle disorder associated with sudden cardiac death, arrhythmias and heart failure. Genetic mutations can be identified in approximately 60% of patients; these are commonest in genes that encode proteins of the cardiac sarcomere. Similar to other Mendelian diseases these mutations are characterized by incomplete penetrance and variable clinical expression. Our knowledge of this genetic diversity is rapidly evolving as high-throughput DNA sequencing technology is now used to characterize an individual patient's disease. In addition, the genomic basis of several multisystem diseases associated with a hypertrophic cardiomyopathy phenotype has been elucidated. Genetic biomarkers can be helpful in making an accurate diagnosis and in identifying relatives at risk of developing the condition. In the clinical setting, genetic testing and genetic screening should be used pragmatically with appropriate counseling. Here we review the current role of genetic biomarkers in hypertrophic cardiomyopathy, highlight recent progress in the field and discuss future challenges.

  5. Peripartum cardiomyopathy coexistent with human immunodeficiency virus: A substantial obstetric jeopardy

    Directory of Open Access Journals (Sweden)

    Debasmita Mandal

    2013-01-01

    Full Text Available Peripartum cardiomyopathy (PPCM is a rare cause of pregnancy-related heart failure, which affects a woman during the last months of pregnancy or first months of parturition. Its etiopathogenesis is still unclear. Coexistence of PPCM with human immunodeficiency virus (HIV has been scarcely analyzed. A low CD4 count is proposed to be one of the predictors of dilated cardiomyopathy in HIV. Here, a pregnant woman with HIV presented with signs of congestive heart failure for the first time during her last trimester. Echocardiography revealed a dilated cardiomyopathy with ejection fraction of 34% which proved the diagnosis of PPCM. She underwent cesarean section for impending previous scar rupture. Her status deteriorated subsequently in spite of all efforts and she succumbed due to ventricular tachycardia. This case necessitates an awareness regarding coexistence of HIV with PPCM and dreaded clinical sequences. Patients suffering from HIV should be treated well and their CD4 count should be improved before conception to avoid such complications in pregnancy.

  6. Insights into restrictive cardiomyopathy from clinical and animal studies

    Institute of Scientific and Technical Information of China (English)

    Pierre-Yves Jean-Charles; Yue-Jin Li; Chang-Long Nan; Xu-Pei Huang

    2011-01-01

    Catdiomyopathies are diseases that primarily affect the myocardium,leading to serious cardiac dysfimction and heart failure.Out of the three major categories of candiomyopathies(hypertrophic,dilated and restrictive),restrictive cardiomyopathy(RCM)is less common and also the least studied However,the prognosis for RCM is poor as some patients dying in their childhood The molecular mechanisms behind the disease development and progression are not very clear and the treatment of RCM is very difficult and often ineffective.In this article,we reviewed the recent progress in RCM research from the clinical studies and the translational studies done on diseased transgenic animal models.This will help for a better understanding of tare mechanisms underlying the etiology and development of RCM and for the design of better treatments for the disease.

  7. Disease Rescue and Increased Lifespan in a Model of Cardiomyopathy and Muscular Dystrophy by Combined AAV Treatments

    OpenAIRE

    Carmen Vitiello; Stefania Faraso; Nicolina Cristina Sorrentino; Giovanni Di Salvo; Edoardo Nusco; Gerardo Nigro; Luisa Cutillo; Raffaele Calabrò; Alberto Auricchio; Vincenzo Nigro

    2009-01-01

    BACKGROUND: The BIO14.6 hamster is an excellent animal model for inherited cardiomyopathy, because of its lethal and well-documented course, due to a spontaneous deletion of delta-sarcoglycan gene promoter and first exon. The muscle disease is progressive and average lifespan is 11 months, because heart slowly dilates towards heart failure. METHODOLOGY/PRINCIPAL FINDINGS: Based on the ability of adeno-associated viral (AAV) vectors to transduce heart together with skeletal muscle following sy...

  8. Polymorphism in the Alpha Cardiac Muscle Actin 1 Gene Is Associated to Susceptibility to Chronic Inflammatory Cardiomyopathy

    OpenAIRE

    Amanda Farage Frade; Priscila Camilo Teixeira; Barbara Maria Ianni; Cristina Wide Pissetti; Bruno Saba; Lin Hui Tzu Wang; Andréia Kuramoto; Luciana Gabriel Nogueira; Paula Buck; Fabrício Dias; Helene Giniaux; Agnes Llored; Sthefanny Alves; Andre Schmidt; Eduardo Donadi

    2013-01-01

    AIMS: Chagas disease, caused by the protozoan Trypanosoma cruzi is endemic in Latin America, and may lead to a life-threatening inflammatory dilated, chronic Chagas cardiomyopathy (CCC). One third of T. cruzi-infected individuals progress to CCC while the others remain asymptomatic (ASY). A possible genetic component to disease progression was suggested by familial aggregation of cases and the association of markers of innate and adaptive immunity genes with CCC development. Since mutations i...

  9. Correction of human phospholamban R14del mutation associated with cardiomyopathy using targeted nucleases and combination therapy

    OpenAIRE

    Karakikes, Ioannis; Stillitano, Francesca; Nonnenmacher, Mathieu; Tzimas, Christos; Sanoudou, Despina; Termglinchan, Vittavat; Kong, Chi-Wing; Rushing, Stephanie; Hansen, Jens; Ceholski, Delaine; Kolokathis, Fotis; Kremastinos, Dimitrios; Katoulis, Alexandros; Ren, Lihuan; Cohen, Ninette

    2015-01-01

    International audience A number of genetic mutations is associated with cardiomyopathies. A mutation in the coding region of the phospholamban (PLN) gene (R14del) is identified in families with hereditary heart failure. Heterozygous patients exhibit left ventricular dilation and ventricular arrhyth-mias. Here we generate induced pluripotent stem cells (iPSCs) from a patient harbouring the PLN R14del mutation and differentiate them into cardiomyocytes (iPSC-CMs). We find that the PLN R14del...

  10. Simultaneous interstitial pneumonitis and cardiomyopathy induced by venlafaxine

    Directory of Open Access Journals (Sweden)

    Pedro Gonçalo Ferreira

    2014-06-01

    Full Text Available Venlafaxine is a serotonin-norepinephrine reuptake inhibitor used as an antidepressant. Interindividual variability and herb-drug interactions can lead to drug-induced toxicity. We report the case of a 35-year-old female patient diagnosed with synchronous pneumonitis and acute cardiomyopathy attributed to venlafaxine. The patient sought medical attention due to dyspnea and dry cough that started three months after initiating treatment with venlafaxine for depression. The patient was concomitantly taking Centella asiatica and Fucus vesiculosus as phytotherapeutic agents. Chest CT angiography and chest X-ray revealed parenchymal lung disease (diffuse micronodules and focal ground-glass opacities and simultaneous dilated cardiomyopathy. Ecocardiography revealed a left ventricular ejection fraction (LVEF of 21%. A thorough investigation was carried out, including BAL, imaging studies, autoimmune testing, right heart catheterization, and myocardial biopsy. After excluding other etiologies and applying the Naranjo Adverse Drug Reaction Probability Scale, a diagnosis of synchronous pneumonitis/cardiomyopathy associated with venlafaxine was assumed. The herbal supplements taken by the patient have a known potential to inhibit cytochrome P450 enzyme complex, which is responsible for the metabolization of venlafaxine. After venlafaxine discontinuation, there was rapid improvement, with regression of the radiological abnormalities and normalization of the LVEF. This was an important case of drug-induced cardiopulmonary toxicity. The circumstantial intake of inhibitors of the CYP2D6 isoenzyme and the presence of a CYP2D6 slow metabolism phenotype might have resulted in the toxic accumulation of venlafaxine and the subsequent clinical manifestations. Here, we also discuss why macrophage-dominant phospholipidosis was the most likely mechanism of toxicity in this case.

  11. Left Ventricular Non-compaction Cardiomyopathy - A Case Report

    Directory of Open Access Journals (Sweden)

    Timea Szakacs Xantus

    2015-06-01

    Full Text Available Background: Left non-compaction cardiomyopathy (LVNC or “spongy myocardium” is a relatively rare primary genetic cardiomyopathy, characterized by prominent wall trabeculations and intertrabecular recesses which communicate with the ventricular cavity. It appears in isolated form or coexists with other congenital heart diseases and/or systemic abnormalities. Material and method: A 28-year-old woman was admitted with exertional dyspnoea, palpitations, non-specific chest pain and progressive fatigue on exertion. In her family history sudden cardiac-related deaths at young age are present. Cardiovascular system examination revealed tachycardia, intermittent extrabeats. The rest EKG showed sinusal tachycardia (105 bpm, negative T-waves in DII, DIII, aVF, V4-V6. Consecutive 24 hours Holter EKG monitoring revealed nonsustained ventricular tachycardia, paroxysmal atrial fibrillation, isolated ventricular extrasystoles. Echocardiography showed left ventricular systolic dysfunction (LVEF:30-35%, slight LV enlargement, normal right ventricle and small left ventricle (LV trabeculae in the apical area. Cardiac MRI demonstrated dilated LV and the presence of the trabeculations of LV walls suggestive for non-compaction cardiomyopathy. A combined treatment for heart failure and cardiac arrhythmias was initiated with good clinical results. Patient was scheduled for an implantable cardioverter defibrillator “life-saving”. Conclusions: The symptoms of heart failure and cardiac arrhythmias should be considered important in apparently healthy young patients. Besides intensive medical treatment is indicated the implantation of an ICD “life-saving” and in advanced cases heart transplantation. Even if the electrocardiographic findings are non specific for noncompaction, a complete diagnostic evaluation is important, including sophisticated imaging techniques, a screening of first-degree relatives, and an extensive clinical, and genetic appreciation by a

  12. Left Atrial Function in Patients with Chronic Chagasic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Claudia da Silva Fragata

    2015-01-01

    Full Text Available Background: Chagas disease is a cause of dilated cardiomyopathy, and information about left atrial (LA function in this disease still lacks. Objective: To assess the different LA functions (reservoir, conduit and pump functions and their correlation with the echocardiographic parameters of left ventricular (LV systolic and diastolic functions. Methods: 10 control subjects (CG, and patients with Chagas disease as follows: 26 with the indeterminate form (GI; 30 with ECG alterations (GII; and 19 with LV dysfunction (GIII. All patients underwent M-mode and two-dimensional echocardiography, pulsed-wave Doppler and tissue Doppler imaging. Results: Reservoir function (Total Emptying Fraction: TEF: (p <0.0001, lower in GIII as compared to CG (p = 0.003, GI (p <0.001 and GII (p <0.001. Conduit function (Passive Emptying Fraction: PEF: (p = 0.004, lower in GIII (GIII and CG, p = 0.06; GI and GII, p = 0.06; and GII and GIII, p = 0.07. Pump function (Active Emptying Fraction: AEF: (p = 0.0001, lower in GIII as compared to CG (p = 0.05, GI (p<0.0001 and GII (p = 0.002. There was a negative correlation of E/e’ average with the reservoir and pump functions (TEF and AEF, and a positive correlation of e’ average with s’ wave (both septal and lateral walls and the reservoir, conduit and pump LA functions. Conclusion: An impairment of LA functions in Chagas cardiomyopathy was observed.

  13. Pneumatic dilatation for childhood achalasia.

    Science.gov (United States)

    Babu, R; Grier, D; Cusick, E; Spicer, R D

    2001-09-01

    Treatment of achalasia by pneumatic balloon dilatation (PBD) is well established in adults. Due to limited experience and the rarity of the condition in children, there are relatively few reports in the paediatric literature. Although PBD has been reported as a primary method of treatment, there are no reports of secondary PBD for childhood achalasia. Between 1995 and 1999, five patients underwent treatment for achalasia (age: 9-14 years, M:F = 4:1). The presenting symptoms were dysphagia (5). vomiting episodes (2), aspiration (1), food-bolus obstruction (1), and failure to thrive (1). In all patients a barium swallow and manometry were used to confirm the diagnosis. Three underwent primary PBD. Two who had previously undergone surgical myotomy underwent secondary PBD for recurrence of symptoms. Dilatation was performed using a 35-mm balloon with the child under general anaesthesia. Technical success was defined as demonstration of a waist under screening at lower pressures followed by abolition of the waist at higher pressures. In addition to reviewing our results, a systematic review of the literature was performed (Medline, Cochrane Library, Pubmed, Embase). Three patients (primary dilatation) showed excellent improvement after a single dilatation. In two cases (secondary dilatation) three and five attempts were required. No complications were encountered. The mean follow-up period was 2 years (1-3.5 years) and four patients remained asymptomatic, an overall success rate of 80%. The literature review revealed similar good results in most of the recent reports. Thus, PBD as a primary treatment for childhood achalasia has a success rate of 70%-90% with minimal side effects, short hospital stay, and good patient acceptability over an operation. We have also established the usefulness of this method as a secondary treatment when symptoms recur after surgery.

  14. Risk of Cardiomyopathy in Younger Persons With a Family History of Death from Cardiomyopathy

    DEFF Research Database (Denmark)

    Ranthe, Mattis F; Carstensen, Lisbeth; Øyen, Nina;

    2015-01-01

    incidence rate ratios for cardiomyopathy by family history of premature death. Premature cardiomyopathy deaths in first- and second-degree relatives were associated with 29- and 6-fold increases in the rate of cardiomyopathy, respectively. If the first-degree relative died aged years, the rate......BACKGROUND: Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (years) aggregates in families...... at the population level is unclear. In a nationwide cohort, we examined the risk of cardiomyopathy by family history of premature death (years) from cardiomyopathy. METHODS AND RESULTS: By linking Danish national register data, we constructed a cohort of 3.9 million persons born from 1950 to 2008. We...

  15. Oversampling of wavelet frames for real dilations

    DEFF Research Database (Denmark)

    Bownik, Marcin; Lemvig, Jakob

    2012-01-01

    We generalize the Second Oversampling Theorem for wavelet frames and dual wavelet frames from the setting of integer dilations to real dilations. We also study the relationship between dilation matrix oversampling of semi-orthogonal Parseval wavelet frames and the additional shift invariance gain...

  16. Anabolic steroid-induced cardiomyopathy underlying acute liver failure in a young bodybuilder

    Institute of Scientific and Technical Information of China (English)

    Miguel Bispo; Ana Valente; Rosário Maldonado; Rui Palma; Helena Glória; Jo(a)o Nóbrega; Paula Alexandrino

    2009-01-01

    Heart failure may lead to subclinical circulatory disturbances and remain an unrecognized cause of ischemic liver injury. We present the case of a previously healthy 40-year-old bodybuilder, referred to our Intensive-Care Unit of Hepatology for treatment of severe acute liver failure, with the suspicion of toxic hepatitis associated with anabolic steroid abuse. Despite the absence of symptoms and signs of congestive heart failure at admission, an anabolic steroid-induced dilated cardiomyopathy with a large thrombus in both ventricles was found to be the underlying cause of the liver injury. Treatment for the initially unrecognized heart failure rapidly restored liver function to normal. To our knowledge, this is the first reported case of severe acute liver failure due to an unrecognized anabolic steroid-induced cardiomyopathy. Awareness of this unique presentation will allow for prompt treatment of this potentially fatal cause of liver failure.

  17. Keshan disease and mitochondrial cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    YANG; Fuyu

    2006-01-01

    Keshan disease (KD) is a potentially fatal form of cardiomyopathy (disease of the heart muscle) endemic in certain areas of China. From 1984 to 1986, a national comprehensive scientific investigation on KD in Chuxiong region of Yunnan Province in the southwest China was conducted. The investigation team was composed of epidemiologists, clinic doctors, pathologists, biochemists, biophysicists and specialists in ecological environment. Results of pathological, biochemical and biophysical as well as clinical studies showed: an obvious increase of enlarged and swollen mitochondria with distended crista membranes in myocardium from patients with KD; significant reductions in the activity of oxidative phosphorylation (succinate dehydrogenase, cytochrome oxidase, succinate oxidase, H+-ATPase) of affected mitochondria; decrease in CoQ, cardiolipin, Se and GSHPx activity, while obvious increase in the Ca2+ content. So, it was suggested that mitochondria are the predominant target of the pathogenic factors of KD. Before Chuxiong KD survey only a few cases of mitochondrial cardiomyopathy were studied. During the multidisciplinary scientific investigation on KD in Chuxiong a large amount of samples from KD cases and the positive controls were examined. On the basis of the results obtained it was suggested that KD might be classified as a "Mitochondrial Cardiomyopathy" endemic in China. This is one of the achievements in the three years' survey in Chuxiong and is valuable not only to the deeper understanding of pathogenic mechanism of KD but also to the study of mitochondrial cardiomyopathy in general.Keshan disease is not a genetic disease, but is closely related to the malnutrition (especially microelement Se deficiency). KD occurs along a low Se belt, and Se supplementation has been effective in prevention of such disease. The incidence of KD has sharply decreased along with the steady raise of living standard and realization of preventive measures. At present, patients of

  18. Distribution of late gadolinium enhancement in various types of cardiomyopathies:Significance in differential diagnosis, clinical features and prognosis

    Institute of Scientific and Technical Information of China (English)

    Hiroshi; Satoh; Makoto; Sano; Kenichiro; Suwa; Takeji; Saitoh; Mamoru; Nobuhara; Masao; Saotome; Tsuyoshi; Urushida; Hideki; Katoh; Hideharu; Hayashi

    2014-01-01

    The recent development of cardiac magnetic resonance(CMR)techniques has allowed detailed analyses of cardiac function and tissue characterization with high spatial resolution.We review characteristic CMR features in ischemic and non-ischemic cardiomyopathies(ICM and NICM),especially in terms of the location and distribution of late gadolinium enhancement(LGE).CMR in ICM shows segmental wall motion abnormalities or wall thinning in a particular coronary arterial territory,and the subendocardial or transmural LGE.LGE in NICM generally does not correspond to any particular coronary artery distribution and is located mostly in the mid-wall to subepicardial layer.The analysis of LGE distribution is valuable to differentiate NICM with diffusely impaired systolic function,including dilated cardiomyopathy,end-stage hypertrophic cardiomyopathy(HCM),cardiac sarcoidosis,and myocarditis,and those with diffuse left ventricular(LV)hypertrophy including HCM,cardiac amyloidosis and Anderson-Fabry disease.A transient low signal intensity LGE in regions of severe LV dysfunction is a particular feature of stress cardiomyopathy.In arrhythmogenic right ventricular cardiomyopathy/dysplasia,an enhancement of right ventricular(RV)wall with functional and morphological changes of RV becomes apparent.Finally,the analyses of LGE distribution have potentials to predict cardiac outcomes and response to treatments.

  19. Multisensory signalling enhances pupil dilation.

    Science.gov (United States)

    Rigato, Silvia; Rieger, Gerulf; Romei, Vincenzo

    2016-01-01

    Detecting and integrating information across the senses is an advantageous mechanism to efficiently respond to the environment. In this study, a simple auditory-visual detection task was employed to test whether pupil dilation, generally associated with successful target detection, could be used as a reliable measure for studying multisensory integration processing in humans. We recorded reaction times and pupil dilation in response to a series of visual and auditory stimuli, which were presented either alone or in combination. The results indicated faster reaction times and larger pupil diameter to the presentation of combined auditory and visual stimuli than the same stimuli when presented in isolation. Moreover, the responses to the multisensory condition exceeded the linear summation of the responses obtained in each unimodal condition. Importantly, faster reaction times corresponded to larger pupil dilation, suggesting that also the latter can be a reliable measure of multisensory processes. This study will serve as a foundation for the investigation of auditory-visual integration in populations where simple reaction times cannot be collected, such as developmental and clinical populations. PMID:27189316

  20. Mitochondrial Dysfunction in Diabetic Cardiomyopathy

    OpenAIRE

    Jennifer G. Duncan

    2011-01-01

    Cardiovascular disease is common in patients with diabetes and is a significant contributor to the high mortality rates associated with diabetes. Heart failure is common in diabetic patients, even in the absence of coronary artery disease or hypertension, an entity known as diabetic cardiomyopathy. Evidence indicates that myocardial metabolism is altered in diabetes, which likely contributes to contractile dysfunction and ventricular failure. The mitochondria are the center of metabolism, and...

  1. Takotsubo cardiomyopathy: a historical note

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    @@ To the Editor: I read with interest the case report of Takotsubo cardiomyopathy in a Chinese woman.1 Unfortunately, the authors mistakenly attributed the original description of this syndrome to Tsuchihashi et al in 2001.2Actually Dote et al3 first described this syndrome in 1991, ten years before Tsuchihashi et al did.2 Incidentally, the authors did make a reference in their case report to the article by Dote et al3 which was cited as reference 1.

  2. Abnormal atrial activation is common in patients with arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Platonov, Pyotr G; Christensen, Alex H; Holmqvist, Fredrik;

    2011-01-01

    INTRODUCTION: Structural right atrial abnormalities have been described in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, little is known about electrocardiographic signs of atrial involvement in ARVC because no systematic studies have been conducted. METHODS: P......%, whereas 15 patients (37%) had atypical P-wave positive in all 3 leads (P right ventricular abnormality. CONCLUSIONS: Patients with ARVC commonly demonstrate deteriorated...... atrial activation expressed either as prolonged P-wave duration or abnormal P-wave morphology. The P-wave abnormalities were not secondary to right ventricular dilatation. These findings show that atrial involvement is common in ARVC and may represent yet another manifestation of the disease...

  3. Skeletal muscle involvement in cardiomyopathies.

    Science.gov (United States)

    Limongelli, Giuseppe; D'Alessandro, Raffaella; Maddaloni, Valeria; Rea, Alessandra; Sarkozy, Anna; McKenna, William J

    2013-12-01

    The link between heart and skeletal muscle disorders is based on similar molecular, anatomical and clinical features, which are shared by the 'primary' cardiomyopathies and 'primary' neuromuscular disorders. There are, however, some peculiarities that are typical of cardiac and skeletal muscle disorders. Skeletal muscle weakness presenting at any age may indicate a primary neuromuscular disorder (associated with creatine kinase elevation as in dystrophinopathies), a mitochondrial disease (particularly if encephalopathy, ocular myopathy, retinitis, neurosensorineural deafness, lactic acidosis are present), a storage disorder (progressive exercise intolerance, cognitive impairment and retinitis pigmentosa, as in Danon disease), or metabolic disorders (hypoglycaemia, metabolic acidosis, hyperammonaemia or other specific biochemical abnormalities). In such patients, skeletal muscle weakness usually precedes the cardiomyopathy and dominates the clinical picture. Nevertheless, skeletal involvement may be subtle, and the first clinical manifestation of a neuromuscular disorder may be the occurrence of heart failure, conduction disorders or ventricular arrhythmias due to cardiomyopathy. ECG and echocardiogram, and eventually, a more detailed cardiovascular evaluation may be required to identify early cardiac involvement. Paediatric and adult cardiologists should be proactive in screening for neuromuscular and related disorders to enable diagnosis in probands and evaluation of families with a focus on the identification of those at risk of cardiac arrhythmia and emboli who may require specific prophylactic treatments, for example, pacemaker, implantable cardioverter-defibrillator and anticoagulation. PMID:24149064

  4. [Cirrhotic cardiomyopathy: a specific entity].

    Science.gov (United States)

    Brondex, A; Arlès, F; Lipovac, A-S; Richecoeur, M; Bronstein, J-A

    2012-04-01

    Cirrhosis is a frequent and severe condition, which is the late stage of numerous chronic liver diseases. It is associated with major hemodynamic alterations characteristic of hyperdynamic circulation and with a series of structural, functional, electrophysiological and biological heart abnormalities termed cirrhotic cardiomyopathy. The pathogenesis of this syndrome is multifactorial. It is usually clinically latent or mild, likely because the peripheral vasodilatation significantly reduces the left ventricle afterload. However, sudden changes of hemodynamic state (vascular filling, surgical or transjugular intrahepatic porto-systemic shunts, peritoneo-venous shunts and orthotopic liver transplantation) or myocardial contractility (introduction of beta-blocker therapy) can unmask its presence, and sometimes convert latent to overt heart failure. Cirrhotic cardiomyopathy may also contribute to the pathogenesis of hepatorenal syndrome. This entity has been described recently, and its diagnostic criteria are still under debate. To date, current management recommendations are empirical, nonspecific measures. Recognition of cirrhotic cardiomyopathy depends on a high level of awareness for the presence of this syndrome, particularly in patients with advanced cirrhosis who undergo significant surgical, pharmacological or physiological stresses. PMID:22115174

  5. Cardiomyopathies and the Armed Forces.

    Science.gov (United States)

    Holdsworth, D A; Cox, A T; Boos, C; Hardman, R; Sharma, S

    2015-09-01

    Cardiomyopathies are a group of heterogeneous myocardial diseases that are frequently inherited and are a recognised cause of premature sudden cardiac death in young individuals. Incomplete expressions of disease and the overlap with the physiological cardiac manifestations of regular intensive exercise create diagnostic challenges in young athletes and military recruits. Early identification is important because sudden death in the absence of prodromal symptoms is a common presentation, and there are several therapeutic strategies to minimise this risk. This paper examines the classification and clinical features of cardiomyopathies with specific reference to a military population and provides a detailed account of the optimum strategy for diagnosis, indications for specialist referral and specific guidance on the occupational significance of cardiomyopathy. A 27-year-old Lance Corporal Signaller presents to his Regimental medical officer (RMO) after feeling 'light-headed' following an 8 mile unloaded run. While waiting to see the RMO, the medical sergeant records a 12-lead ECG. The ECG is reviewed by the RMO immediately prior to the consultation and shows voltage criteria for left ventricular (LV) hypertrophy and inverted T-waves in II, III, aVF and V1-V3 (Figure 1). This Lance Corporal is a unit physical training instructor and engages in >10 h of aerobic exercise per week. He is a non-smoker and does not have any significant medical history. PMID:26246349

  6. ABNORMAL LEFT VENTRICULAR SYSTOLIC AND DIASTOLIC FUNCTIONAL RESPONSE TO ISOMETRIC EXERCISE IN IDIOPATHIC DILATED CARDIOMY-OPATHY:BENEFICIAL EFFECT OF CAPTOPRIE

    Institute of Scientific and Technical Information of China (English)

    沈卫峰; 张宪; 胡厚达; 龚兰生

    1995-01-01

    In 19 patients with idiopathic dilated cardiomyopathy and symptoms of congetive haert failure,left ventricular (LV) systolic performance and diastolic velocity profiles were assessed by two-dimensional e-chocardiography and pulsed wave Doppler at rest and during handgrip exercise before and ninety minutes after administration of captopril (mean dose 25±12mg);range 12.5-50mg).Although heart rate and blood pressure increased similarly during handgrip exercise before and after captopril treatment,both were lower with handgrip exercise during captopril treatment.The results from this study indicated that acute angiotensin converting enzyme inhibition with captopril reduces preload and afterload and ameliorates hand-grip exercise-induced LV systolic and diastolic filling dysfunction in patients with congestive bheart failure secondary to idiopathic dilated cardiomyopathy.

  7. Antarctic Analog for Dilational Bands on Europa

    Science.gov (United States)

    Hurford, T. A.; Brunt, K. M.

    2014-01-01

    Europa's surface shows signs of extension, which is revealed as lithospheric dilation expressed along ridges, dilational bands and ridged bands. Ridges, the most common tectonic feature on Europa, comprise a central crack flanked by two raised banks a few hundred meters high on each side. Together these three classes may represent a continuum of formation. In Tufts' Dilational Model ridge formation is dominated by daily tidal cycling of a crack, which can be superimposed with regional secular dilation. The two sources of dilation can combine to form the various band morphologies observed. New GPS data along a rift on the Ross Ice Shelf, Antarctica is a suitable Earth analog to test the framework of Tufts' Dilational Model. As predicted by Tufts' Dilational Model, tensile failures in the Ross Ice Shelf exhibit secular dilation, upon which a tidal signal can be seen. From this analog we conclude that Tufts' Dilational Model for Europan ridges and bands may be credible and that the secular dilation is most likely from a regional source and not tidally driven.

  8. Restrictive Cardiomyopathy: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Bilal Bin Abdullah*, Mehboob.M.Kalburgi, Sahana Shetty and Satyasrinivas

    2011-04-01

    Full Text Available We report a 28 years old male presenting with heart failure. A thorough clinical evaluation directed us towards restrictive heart disease. Doppler echocardiographic study was used as a main modality of diagnosis and cardiac catheterization confirmed the diagnosis of idiopathic restrictive cardiomyopathy. We express the contribution of clinical findings and appropriate diagnostic measures in approaching a case of Restrictive cardiomyopathy (RCM.

  9. Cardiomyopathy in becker muscular dystrophy: Overview

    Science.gov (United States)

    Ho, Rady; Nguyen, My-Le; Mather, Paul

    2016-01-01

    Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed. PMID:27354892

  10. Cardiomyopathy in becker muscular dystrophy: Overview.

    Science.gov (United States)

    Ho, Rady; Nguyen, My-Le; Mather, Paul

    2016-06-26

    Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed. PMID:27354892

  11. PERIPARTUM CARDIOMYOPATHY--REPORT OF 16 CASES

    Institute of Scientific and Technical Information of China (English)

    杨佳欣; 刘俊涛; 边旭明

    2002-01-01

    Objective.To analyze the clinical characteristics of peripartum cardiomyopathy and to evaluate the different factors that influence the prognosis of the peripartum cardiomyopathy.Method.A retrospective review was undertaken on records of women who were diagnosed with peripartum cardiomyopathy at Peking Union Medical College Hospital between Jan.1983 and May 1999.Results.During the research period,only 16 pregnant women were documented as peripartum cardiomyopathy.Some of the women undertook ultrasonic cardiographic (UCG) examination that showed decreased systolic function.Seven women were complicated with pregnancy induced hypertension.Three died of disseminated intravascular coagulation,embolism and cardiogenic shock respectively.Conclusion.Early diagnosis of the peripartum cardiomyopathy is extremely important.The UCG can provide helpful information on disease progression or regression.

  12. [Monitoring cervical dilatation by impedance].

    Science.gov (United States)

    Salvat, J; Lassen, M; Sauze, C; Baud, S; Salvat, F

    1992-01-01

    Several different physics procedures have been tried to mechanize the recording of partograms. Can a measure of impedance of tissue Z using potential difference V, according to Ohm's law V = Z1, and 1 is a constant, be correlated with a measure of cervical dilatation using vaginal examination? This was our hypothesis. The tissue impedance meter was made to our design and applied according to a bipolar procedure. Our work was carried out on 28 patients. 10 patients were registered before labour started in order to test the apparatus and to record the impedance variations without labour taking place, and 18 patients were registered in labour to see whether there was any correlation. The level of impedance in the cervix without labour was 302.7 Ohms with a deviation of 8.2. Using student's t tests it was found that there was a significant correlation (p less than 0.001) in four measurements between the impedance measure and measures obtained by extrapolating the degrees of dilatation calculated from vaginal examination. This is a preliminary study in which we have defined the conditions that are necessary to confirm these first results and to further develop the method. PMID:1401774

  13. Ergotamine-Induced Takotsubo Cardiomyopathy.

    Science.gov (United States)

    Ozpelit, Ebru; Ozpelit, Mehmet E; Akdeniz, Bahri; Göldeli, Özhan

    2016-01-01

    Takotsubo cardiomyopathy (TC) is a recently increasing diagnosed disease showed by transient apical or mid-apical left ventricular dysfunction. It is known as a disease of postmenopausal women, which is usually triggered by emotional or physical stress. Although the trigger is mostly endogenous, some drugs have also been reported as the cause. Published case reports of TC associated with drug usage consist of sympathomimetic drugs, inotropic agents, thyroid hormone, cocaine, and 5-fluorouracil. We present an unusual case of TC in which the possible trigger is ergotamine toxicity. PMID:25099482

  14. T1 mapping in differentiation of diffuse myocardial disease in hypertrophic and dilative cardiomyopathy

    NARCIS (Netherlands)

    Puntmann, V.O.; Pastor, A.; Chen, Z.; Voigt, T.; Karim, R.; Rhode, K.; Razavi, R. S.; Schaeffter, T.; Nagel, E.

    2013-01-01

    T1 mapping was proposed as potentially valuable in quantitative assessment of diffuse myocardial fibrosis. We aimed to determine its role in differentiation of healthy myocardium from diffuse fibrosis in clinical setting. Conclusions: We demonstrate that nativeand post-contrast T1 values and their r

  15. Ivabradine Improves Heart Rate Variability in Patients with Nonischemic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ertugrul Kurtoglu

    2014-10-01

    Full Text Available Background: Ivabradine is a novel specific heart rate (HR-lowering agent that improves event-free survival in patients with heart failure (HF. Objectives: We aimed to evaluate the effect of ivabradine on time domain indices of heart rate variability (HRV in patients with HF. Methods: Forty-eight patients with compensated HF of nonischemic origin were included. Ivabradine treatment was initiated according to the latest HF guidelines. For HRV analysis, 24-h Holter recording was obtained from each patient before and after 8 weeks of treatment with ivabradine. Results: The mean RR interval, standard deviation of all normal to normal RR intervals (SDNN, the standard deviation of 5-min mean RR intervals (SDANN, the mean of the standard deviation of all normal-to-normal RR intervals for all 5-min segments (SDNN index, the percentage of successive normal RR intervals exceeding 50 ms (pNN50, and the square root of the mean of the squares of the differences between successive normal to normal RR intervals (RMSSD were low at baseline before treatment with ivabradine. After 8 weeks of treatment with ivabradine, the mean HR (83.6 ± 8.0 and 64.6 ± 5.8, p < 0.0001, mean RR interval (713 ± 74 and 943 ± 101 ms, p < 0.0001, SDNN (56.2 ± 15.7 and 87.9 ± 19.4 ms, p < 0.0001, SDANN (49.5 ± 14.7 and 76.4 ± 19.5 ms, p < 0.0001, SDNN index (24.7 ± 8.8 and 38.3 ± 13.1 ms, p < 0.0001, pNN50 (2.4 ± 1.6 and 3.2 ± 2.2 %, p < 0.0001, and RMSSD (13.5 ± 4.6 and 17.8 ± 5.4 ms, p < 0.0001 substantially improved, which sustained during both when awake and while asleep. Conclusion: Our findings suggest that treatment with ivabradine improves HRV in nonischemic patients with HF.

  16. The role of sarcomere gene mutations in patients with idiopathic dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Daniel Vega; Andersen, Paal Skytt; Hedley, Paula;

    2009-01-01

    by bidirectional DNA sequencing of conformers in the coding regions of MYH7, MYBPC3, TPM1, ACTC, MYL2, MYL3, TNNT2, CSRP3 and TNNI3. Eight probands carried disease-associated genetic variants (26%). In MYH7, three novel mutations were found; in MYBPC3, one novel variant and two known mutations were found...

  17. From the risk-stratification of patients with dilated cardiomyopathy to the optimal treatment strategy

    Directory of Open Access Journals (Sweden)

    Frolov A.V.

    2016-03-01

    Conclusions. Application of the original model of risk stratification will allow to optimize the general management in DCM and the strategy of timely selection of potential candidates for implantation of cardioverter- defibrillator for the primary prevention of SCD.

  18. LETTER: A commentary on “A young man with acute dilated cardiomyopathy associated with methylphenidate”

    Directory of Open Access Journals (Sweden)

    Ahmad Ghanizadeh

    2009-06-01

    Full Text Available Ahmad Ghanizadeh1,21Associate Professor of Child and Adolescent Psychiatry, 2Research Center for Psychiatry and Behavioral Sciences, Shiraz University of Medical Sciences, Hafez Hospital, Shiraz, IranWith reference to the report by Nymark and colleagues,1 there are some points that should be highlighted. There is certainly a debate over whether methylphenidate is truly safe regarding possible cardiac side effects.

  19. Charcot-Marie-Tooth disease and dilated cardiomyopathy. A rare combination.

    Directory of Open Access Journals (Sweden)

    Rafael Pila Pérez

    2011-07-01

    Full Text Available Se presenta el caso de un paciente de 50 años de edad, con 14 años de evolución de manifestaciones clínicas, destacándose las alteraciones musculoesqueléticas de los cuatro miembros con atrofia de las prominencias tenar e hipotenar y de la musculatura de ambas piernas. Se destacó la presencia de alteraciones sensitivas en miembros inferiores con distribución en calcetín, atrofia, atonía, arreflexia y marcha equina. Desde el punto de vista cardiaco, el paciente presentaba un fibriloaleteo. La radiografía de tórax mostró un aumento marcado del área cardiaca y la ecocardiografía puso de manifiesto una miocardiopatía dilatada. El estudio histopatológico confirmó la presencia de la enfermedad de Charcot-Marie-Tooth asociada a miocardiopatía dilatada. El diagnóstico se basó en las características clínicas, la velocidad de conducción motora, y el estudio histopatológico, que demostró desmielinización con lesiones en “cebolla”, si bien faltaron los estudios genéticos. La enfermedad de Charcot-Marie-Tooth es una enfermedad rara; aproximadamente un 60 % de los pacientes que la padecen, son portadores de una duplicación del cromosoma 17. Por ello, se consideró oportuno transmitir la experiencia de este caso.

  20. Human phospholamban null results in lethal dilated cardiomyopathy revealing a critical difference between mouse and human

    OpenAIRE

    Haghighi, Kobra; Kolokathis, Fotis; Pater, Luke; Lynch, Roy A.; Asahi, Michio; Gramolini, Anthony O.; Fan, Guo-Chang; Tsiapras, Dimitris; Hahn, Harvey S.; Adamopoulos, Stamatis; Liggett, Stephen B.; Dorn, Gerald W., II; MacLennan, David H.; Kremastinos, Dimitrios T.; Kranias, Evangelia G.

    2003-01-01

    In human disease and experimental animal models, depressed Ca2+ handling in failing cardiomyocytes is widely attributed to impaired sarcoplasmic reticulum (SR) function. In mice, disruption of the PLN gene encoding phospholamban (PLN) or expression of dominant-negative PLN mutants enhances SR and cardiac function, but effects of PLN mutations in humans are unknown. Here, a T116G point mutation, substituting a termination codon for Leu-39 (L39stop), was identified in two families with heredita...

  1. A Case Report of Cardiac Amyloidosis Initially Managed as Dilated Cardiomyopathy: Missing the obvious!

    OpenAIRE

    Yerramareddy Vijaya Chandra; Vellore Satyanarayanan Yogeeswari

    2016-01-01

    Amyloidosis is a rare disorder with uncertain incidence; however, in UK and US population, AL amyloidosis, the most frequently diagnosed type, has an annual incidence of 6 to 10 cases per million. [1] The deposition of amyloid, the extracellular proteinaceous material, in the tissues results in a group of disorders called amyloidoses. [2] The most commonly deposited amyloid material in various organ systems including heart are light chains, transthyretin and serum amyloid A. [2] One of th...

  2. Persistent scarring and dilated cardiomyopathy suggest incomplete regeneration of the apex resected neonatal mouse myocardium

    DEFF Research Database (Denmark)

    Andersen, Ditte Caroline; Jensen, Charlotte Harken; Baun, Christina;

    2016-01-01

    exhibited decreased ejection fraction and wall motion with increased end-diastolic and systolic volumes compared to sham-operated mice. Scarring with collagen accumulation was still substantial, with increased heart size, while cardiomyocyte size was unaffected. In conclusion, these data thus show that apex......Heart damage in mammals is generally considered to result in scar formation, whereas zebrafish completely regenerate their hearts following an intermediate and reversible state of fibrosis after apex resection (AR). Recently, using the AR procedure, one-day-old mice were suggested to have full...... capacity for cardiac regeneration as well. In contrast, using the same mouse model others have shown that the regeneration process is incomplete and that scarring still remains 21days after AR. The present study tested the hypothesis that like in zebrafish, fibrosis in neonatal mammals could...

  3. A case of Becker muscular dystrophy with early manifestation of cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ki Hyun Doo

    2012-09-01

    Full Text Available An 18-year-old boy was admitted with chest discomfort, nausea, and dyspnea at rest. At the age of 3 years, he underwent muscle biopsy and dystrophin gene analysis owing to an enlarged calf muscle and elevated serum kinase level (6,378 U/L without overt weakness; based on the results, Becker muscular dystrophy (BMD was diagnosed. The dystrophin gene showed deletion of exons 45 to 49. He remained ambulant and could step upstairs without significant difficulties. A chest roentgenogram showed cardiomegaly (cardiothoracic ratio, 54%, and his electrocardiogram (ECG showed abnormal ST-T wave, biatrial enlargement, and left ventricular hypertrophy. The 2-dimensional and M-mode ECGs showed a severely dilated left ventricular cavity with diffuse hypokinesis. The systolic indices were reduced, including fractional shortening (9% and ejection fraction (19%. Despite receiving intensive medical treatment, he died from congestive heart failure 5 months after the initial cardiac symptoms. We report a case of BMD with early-onset dilated cardiomyopathy associated with deletion of exons 45 to 49. Early cardiomyopathy can occur in BMD patients with certain genotypes; therefore, careful follow-up is required even in patients with mild phenotypes of BMD.

  4. On turbulence in dilatant dispersions

    Science.gov (United States)

    Baumert, Helmut Z.; Wessling, Bernhard

    2016-07-01

    This paper presents a new theory on the behaviour of shear-thickening (dilatant) fluids under turbulent conditions. The structure of a dilatant colloidal fluid in turbulent motion may be characterized by (at least) four characteristic length scales: (i) the ‘statistically largest’ turbulent scale, {λ }0, labeling the begin of the inertial part of the wavenumber spectrum; (ii) the energy-containing scale, { L }; (iii) Kolmogorov’s micro-scale, {λ }{ K }, related with the size of the smallest vortices existing for a given kinematic viscosity and forcing; (iv) the inner (‘colloidal’) micro-scale, {λ }i, typically representing a major stable material property of the colloidal fluid. In particular, for small ratios r={λ }i/{λ }{ K }∼ { O }(1), various interactions between colloidal structures and smallest turbulent eddies can be expected. In the present paper we discuss particularly that for ρ ={λ }0/{λ }{ K }\\to { O }(1) turbulence (in the narrow, inertial sense) is strangled and chaotic but less mixing fluid motions remain. We start from a new stochastic, micro-mechanical turbulence theory without empirical parameters valid for inviscid fluids as seen in publications by Baumert in 2013 and 2015. It predicts e.g. von Karman’s constant correctly as 1/\\sqrt{2 π }=0.399. In its generalized version for non-zero viscosity and shear-thickening behavior presented in this contribution, it predicts two solution branches for the steady state: The first characterizes a family of states with swift (inertial) turbulent mixing and small {λ }{ K }, potentially approaching {λ }i. The second branch characterizes a state family with ρ \\to { O }(1) and thus strangled turbulence, ρ ≈ { O }(1). Stability properties and a potential dynamic commuting between the two solution branches had to be left for future research.

  5. A mutation in the mitochondrial fission gene Dnm1l leads to cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Houman Ashrafian

    2010-06-01

    Full Text Available Mutations in a number of genes have been linked to inherited dilated cardiomyopathy (DCM. However, such mutations account for only a small proportion of the clinical cases emphasising the need for alternative discovery approaches to uncovering novel pathogenic mutations in hitherto unidentified pathways. Accordingly, as part of a large-scale N-ethyl-N-nitrosourea mutagenesis screen, we identified a mouse mutant, Python, which develops DCM. We demonstrate that the Python phenotype is attributable to a dominant fully penetrant mutation in the dynamin-1-like (Dnm1l gene, which has been shown to be critical for mitochondrial fission. The C452F mutation is in a highly conserved region of the M domain of Dnm1l that alters protein interactions in a yeast two-hybrid system, suggesting that the mutation might alter intramolecular interactions within the Dnm1l monomer. Heterozygous Python fibroblasts exhibit abnormal mitochondria and peroxisomes. Homozygosity for the mutation results in the death of embryos midway though gestation. Heterozygous Python hearts show reduced levels of mitochondria enzyme complexes and suffer from cardiac ATP depletion. The resulting energy deficiency may contribute to cardiomyopathy. This is the first demonstration that a defect in a gene involved in mitochondrial remodelling can result in cardiomyopathy, showing that the function of this gene is needed for the maintenance of normal cellular function in a relatively tissue-specific manner. This disease model attests to the importance of mitochondrial remodelling in the heart; similar defects might underlie human heart muscle disease.

  6. Restrictive cardiomyopathy and hypertrophic cardiomyopathy overlap: the importance of the phenotype

    Directory of Open Access Journals (Sweden)

    Juan Pablo Kaski

    2012-10-01

    Full Text Available Restrictive cardiomyopathy (RCM is defined on the basis of the haemodynamic finding of restrictive ventricular physiology. However, restrictive ventricular pathophysiology is also a feature of other subtypes of cardiomyopathy, including hypertrophic cardiomyopathy (HCM. Clinically and aetiologically, there is an overlap between RCM and HCM with restrictive physiology. However, the clinical distinction between these two entities can be an important pointer towards the underlying aetiology. This review highlights the importance of the recognition of the clinical phenotype as the first step in the classification of cardiomyopathies.

  7. Outpatient experience with oesophageal endoscopic dilation.

    Science.gov (United States)

    Jani, P G; Mburugu, P G

    1998-07-01

    Between March 1990 and August 1997, outpatient endoscopic balloon dilation was performed for oesophageal strictures which developed secondary to malignancies, peptic strictures, post surgical narrowing, achalasia cardia, corrosive ingestion and other causes. A total of 169 dilations were performed in the 92 cases with an average of 1.8 dilation/case (Range 1 to 8). Dilation was possible in all 92 cases without the need for fluoroscopic monitoring. Twenty three (13.6%) of the dilations were performed using pneumatic balloon while in 146(86.4%) cases wire guided metal olives were used. There were nine minor complications which were treated with medication on an outpatient basis and four major complications which required inpatient care. Three of these had perforation of the oesophagus and one died. One other patient developed aspiration pneumonia and subsequently died.

  8. Radionuclide evaluation of renal artery dilatation

    Energy Technology Data Exchange (ETDEWEB)

    Born, M.L.; Gerlock, A.J. Jr.; Goncharenko, V.; Hollifield, J.W.; MacDonell, R.C. Jr.

    1981-01-01

    Radionuclide studies were used in three patients to evaluate renal perfusion and function within 24 hours following transluminal dilatation. In one patient, technetium-99 m pertechnetate showed good renal perfusion one and 12 hours after a post-dilatation arteriogram had shown a renal artery intimal defect. Improved clearance of iodine-131 ortho-iodohippurate from the blood demonstrated an increase in renal function 18 hours following dilatation of a stenosis at a renal allograft anastomosis in the second patient, while technetium-99 m-labeled DTPA showed an improved total glomerular filtration rate 24 hours after dilatation of a saphenous vein bypass graft in the third patient. It was concluded that renal radionuclide studies are of benefit in evaluating patients in the immediate post-dilatation period.

  9. Unveiling nonischemic cardiomyopathies with cardiac magnetic resonance.

    Science.gov (United States)

    Aggarwal, Niti R; Peterson, Tyler J; Young, Phillip M; Araoz, Philip A; Glockner, James; Mankad, Sunil V; Williamson, Eric E

    2014-02-01

    Cardiomyopathy is defined as a heterogeneous group of myocardial disorders with mechanical or electrical dysfunction. Identification of the etiology is important for accurate diagnosis, treatment and prognosis, but continues to be challenging. The ability of cardiac MRI to non-invasively obtain 3D-images of unparalleled resolution without radiation exposure and to provide tissue characterization gives it a distinct advantage over any other diagnostic tool used for evaluation of cardiomyopathies. Cardiac MRI can accurately visualize cardiac morphology and function and also help identify myocardial edema, infiltration and fibrosis. It has emerged as an important diagnostic and prognostic tool in tertiary care centers for work up of patients with non-ischemic cardiomyopathies. This review covers the role of cardiac MRI in evaluation of nonischemic cardiomyopathies, particularly in the context of other diagnostic and prognostic imaging modalities. PMID:24417294

  10. Hypertrophic cardiomyopathy screening in young athletes

    Energy Technology Data Exchange (ETDEWEB)

    Rappoport, W.J. [Arizona Heart Inst., Phoenix, AZ (United States); Steingard, P.M. [Phoenix Suns, Phoenix, AZ (United States)

    2006-07-01

    Hypertrophic cardiomyopathy is the leading cause of sudden death during vigorous exercise. Early identification of this abnormality by ECG screening of high-school athletes before they participate in competitive sports helps save lives. (orig.)

  11. Diagnostic work-up in cardiomyopathies

    DEFF Research Database (Denmark)

    Rapezzi, Claudio; Arbustini, Eloisa; Caforio, Alida L P;

    2013-01-01

    a framework for the clinical approach to diagnosis in cardiomyopathies based on the recognition of diagnostic 'red flags' that can be used to guide rational selection of specialized tests including genetic analysis. The basic premise is that the adoption of a cardiomyopathy-specific mindset which combines...... conventional cardiological assessment with non-cardiac and molecular parameters increases diagnostic accuracy and thus improves advice and treatment for patients and families....

  12. Takotsubo Cardiomyopathy: A New Perspective in Asthma

    Directory of Open Access Journals (Sweden)

    Fady Y. Marmoush

    2015-01-01

    Full Text Available Takotsubo cardiomyopathy (TCM is an entity of reversible cardiomyopathy known for its association with physical or emotional stress and may mimic myocardial infarction. We report an exceedingly rare case of albuterol-induced TCM with moderate asthma exacerbation. An interesting association that may help in understanding the etiology of TCM in the asthmatic population. Although the prognosis of TCM is excellent, it is crucial to recognize beta agonists as a potential stressor.

  13. RATIONAL PHARMACOTHERAPY IN TAKOTSUBO CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    S. Marchev

    2012-01-01

    Full Text Available Rational pharmacotherapy in Takotsubo cardiomyopathy is based on clinical picture and data of functional and laboratory investigations of concrete patient. In patients with hypotension and moderate-to-severe left ventricle outflow tract obstruction inotropic agents must not to be used because they can worsen the degree of obstruction. In these patients beta blockers can improve hemodynamics by causing resolution of the obstruction. If intraventricular thrombus is detected, anticoagulation for at least 3 months is recommended. The duration of anticoagulant therapy may be modified depending on the extent of cardiac function recovery and thrombus resolution. For patients without thrombus but with severe left ventricular dysfunction, anticoagulation is recommended until the akinesis or dyskinesis has resolved but not more than 3 months.

  14. Stimulant-related Takotsubo cardiomyopathy.

    Science.gov (United States)

    Butterfield, Mike; Riguzzi, Christine; Frenkel, Oron; Nagdev, Arun

    2015-03-01

    Takotsubo cardiomyopathy (TC) is a rare but increasingly recognized mimic of acute coronary syndrome. Patients present with angina,ST-segment changes on electrocardiogram (both elevations and depressions),and rapid rises in cardiac biomarkers. Many kinds of stressful events have been associated with TC, but only a handful of drug-related cases have previously been reported. We describe the case of a 58-year-old woman who developed TC 2 days after crack cocaine use, a diagnosis first suggested as bedside echocardiography in the emergency department.Recognition of the classic echocardiographic appearance of TC—apical hypokinesis causing “ballooning” of the left ventricle during systole—may greatly assist providers in the early identification of this condition. PMID:25308824

  15. Decay law and time dilatation

    CERN Document Server

    Giacosa, Francesco

    2015-01-01

    We study the decay law for a moving unstable particle. The usual time-dilatation formula states that the decay width for an unstable state moving with a momentum $p$ and mass $M$ is $\\tilde{\\Gamma}_{p}=\\Gamma M/\\sqrt{p^{2}+M^{2}}$ with $\\Gamma$ being the decay width in the rest frame. In agreement with previous studies, we show that in the context of QM as well as QFT this equation is \\textit{not} correct provided that the quantum measurement is performed in a reference frame in which the unstable particle has momentum $p$ (note, a momentum eigenstate is \\textit{not} a velocity eigenstate in QM). We then give, to our knowledge for the first time, an analytic expression of an improved formula and we show that the deviation from $\\tilde{\\Gamma}_{p}$ has a maximum for $p/M=\\sqrt{2/3},$ but is typically \\textit{very} small. Then, the result can be easily generalized to a momentum wave packet. As a next step, we show that care is needed when one makes a boost of an unstable state with zero momentum/velocity: namel...

  16. Clinical significance of main pancreatic duct dilation on computed tomography: Single and double duct dilation

    Institute of Scientific and Technical Information of China (English)

    Mark D Edge; Maarouf Hoteit; Amil P Patel; Xiaoping Wang; Deborah A Baumgarten; Qiang Cai

    2007-01-01

    AIM: To study the patients with main pancreatic duct dilation on computed tomography (CT) and thereby to provide the predictive criteria to identify patients at high risk of significant diseases, such as pancreatic cancer, and to avoid unnecessary work up for patients at low risk of such diseases.METHODS: Patients with dilation of the main pancreatic duct on CT at Emory University Hospital in 2002 were identified by computer search. Clinical course and ultimate diagnosis were obtained in all the identified patients by abstraction of their computer database records.RESULTS: Seventy-seven patients were identified in this study. Chronic pancreatitis and pancreatic cancer were the most common causes of the main pancreatic duct dilation on CT. Although the majority of patients with isolated dilation of the main pancreatic duct (single duct dilation) had chronic pancreatitis, one-third of patients with single duct dilation but without chronic pancreatitis had pancreatic malignancies, whereas most of patients with concomitant biliary duct dilation (double duct dilation) had pancreatic cancer.CONCLUSION: Patients with pancreatic double duct dilation need extensive work up and careful follow up since a majority of these patients are ultimately diagnosed with pancreatic cancer. Patients with single duct dilation, especially such patients without any evidence of chronic pancreatitis, also need careful follow-up since the possibility of pancreatic malignancy, including adenocarcinoma and intraductal papillary mucinous tumors, is still high.

  17. Cardiomyopathy

    Science.gov (United States)

    ... Textbook of Cardiovascular Medicine . 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 65. McKenna WJ, Elliott P. ... eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 60. McMurray JJV, Pfeffer MA. ...

  18. Diastolic filling in a physical model of obstructive hypertrophic cardiomyopathy

    Science.gov (United States)

    Schovanec, Joseph; Samaee, Milad; Lai, Hong Kuan; Santhanakrishnan, Arvind

    2015-11-01

    Hypertrophic Cardiomyopathy (HCM) is an inherited heart disease that affects as much as one in 500 individuals, and is the most common cause of sudden death in young athletes. The myocardium becomes abnormally thick in HCM and deforms the internal geometry of the left ventricle (LV). Previous studies have shown that a vortex is formed during diastolic filling, and further that the dilated LV morphology seen in systolic heart failure results in altering the filling vortex from elliptical to spherical shape. We have also previously shown that increasing LV wall stiffness decreases the filling vortex circulation. However, alterations to intraventricular filling fluid dynamics due to an obstructive LV morphology and locally elevated wall stiffness (in the hypertrophied region) have not been previously examined from a mechanistic standpoint. We conducted an experimental study using an idealized HCM physical model and compared the intraventricular flow fields obtained from 2D PIV to a baseline LV physical model with lower wall stiffness and anatomical geometry. The obstruction in the HCM model leads to earlier breakdown of the filling vortex as compared to the anatomical LV. Intraventricular filling in both models under increased heart rates will be discussed.

  19. An Erupted Dilated Odontoma: A Rare Presentation.

    Science.gov (United States)

    Sharma, Gaurav; Nagra, Amritpreet; Singh, Gurkeerat; Nagpal, Archna; Soin, Atul; Bhardwaj, Vishal

    2016-01-01

    A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.

  20. An Erupted Dilated Odontoma: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Gaurav Sharma

    2016-01-01

    Full Text Available A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.

  1. An Erupted Dilated Odontoma: A Rare Presentation

    Science.gov (United States)

    Sharma, Gaurav; Nagra, Amritpreet; Singh, Gurkeerat; Nagpal, Archna; Soin, Atul; Bhardwaj, Vishal

    2016-01-01

    A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature. PMID:26989523

  2. Arrhythmogenic cardiomyopathy : diagnosis, genetic background, and risk management

    NARCIS (Netherlands)

    Groeneweg, J. A.; van der Heijden, J. F.; Dooijes, D.; van Veen, T. A. B.; van Tintelen, J. P.; Hauer, R. N.

    2014-01-01

    Arrhythmogenic cardiomyopathy (AC), also known as arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), is a hereditary disease characterised by ventricular arrhythmias, right ventricular and/or left ventricular dysfunction, and fibrofatty replacement of cardiomyocytes. Patients with A

  3. [The dilatation of auditive tube (Bougirage tubaire) by dilators in XIX century].

    Science.gov (United States)

    Kierzek, Andrzej

    2006-01-01

    The treatment of auditiory tube chronic catarrh in XIX century by pharmagological drugs, by special apparatuses of surprising technical sophistication, by electrisation and faradisation is discussed first of all. The dilatation of auditory tube by dilators was the diagnostical and therapeutical methods, especially preferenced by Victor Urbantschitsch, Hermann Schwartze, Jean P. Bonnafonte, Albert Calmettes and Bronisław Taczanowski, Teodor Heiman and Samuel Meyerson. The dilators were building of celluloid, whale-bone, silver and were absorbed by argentum nitrate, vaseline. The technique, difficulties and effects of dilatation are described in more detail. The complications of this operation is presented finally. PMID:17131853

  4. DILATE: a 2-d structural program for the dilation response of hexagonal ducts

    Energy Technology Data Exchange (ETDEWEB)

    Chan, D.P.

    1980-02-01

    An analytical method is introduced for determining the dilation of hexagonal ducts in fast reactors. The method, which is valid for temperatures where creep is linearly dependent on stress, was implemented in a fast-running computer called DILATE. A bench-mark program is presented, which shows the results of the DILATE program in close agreement with the results of the finite element program MARC-CDC. User instructions for the DILATE program are described in detail and a listing of the program is included.

  5. DILATE: a 2-d structural program for the dilation response of hexagonal ducts

    International Nuclear Information System (INIS)

    An analytical method is introduced for determining the dilation of hexagonal ducts in fast reactors. The method, which is valid for temperatures where creep is linearly dependent on stress, was implemented in a fast-running computer called DILATE. A bench-mark program is presented, which shows the results of the DILATE program in close agreement with the results of the finite element program MARC-CDC. User instructions for the DILATE program are described in detail and a listing of the program is included

  6. High Frequency QRS ECG Accurately Detects Cardiomyopathy

    Science.gov (United States)

    Schlegel, Todd T.; Arenare, Brian; Poulin, Gregory; Moser, Daniel R.; Delgado, Reynolds

    2005-01-01

    High frequency (HF, 150-250 Hz) analysis over the entire QRS interval of the ECG is more sensitive than conventional ECG for detecting myocardial ischemia. However, the accuracy of HF QRS ECG for detecting cardiomyopathy is unknown. We obtained simultaneous resting conventional and HF QRS 12-lead ECGs in 66 patients with cardiomyopathy (EF = 23.2 plus or minus 6.l%, mean plus or minus SD) and in 66 age- and gender-matched healthy controls using PC-based ECG software recently developed at NASA. The single most accurate ECG parameter for detecting cardiomyopathy was an HF QRS morphological score that takes into consideration the total number and severity of reduced amplitude zones (RAZs) present plus the clustering of RAZs together in contiguous leads. This RAZ score had an area under the receiver operator curve (ROC) of 0.91, and was 88% sensitive, 82% specific and 85% accurate for identifying cardiomyopathy at optimum score cut-off of 140 points. Although conventional ECG parameters such as the QRS and QTc intervals were also significantly longer in patients than controls (P less than 0.001, BBBs excluded), these conventional parameters were less accurate (area under the ROC = 0.77 and 0.77, respectively) than HF QRS morphological parameters for identifying underlying cardiomyopathy. The total amplitude of the HF QRS complexes, as measured by summed root mean square voltages (RMSVs), also differed between patients and controls (33.8 plus or minus 11.5 vs. 41.5 plus or minus 13.6 mV, respectively, P less than 0.003), but this parameter was even less accurate in distinguishing the two groups (area under ROC = 0.67) than the HF QRS morphologic and conventional ECG parameters. Diagnostic accuracy was optimal (86%) when the RAZ score from the HF QRS ECG and the QTc interval from the conventional ECG were used simultaneously with cut-offs of greater than or equal to 40 points and greater than or equal to 445 ms, respectively. In conclusion 12-lead HF QRS ECG employing

  7. Cardiomyopathy in captive African hedgehogs (Atelerix albiventris).

    Science.gov (United States)

    Raymond, J T; Garner, M M

    2000-09-01

    From 1994 to 1999, 16 captive African hedgehogs (Atelerix albiventris), from among 42 necropsy cases, were diagnosed with cardiomyopathy. The incidence of cardiomyopathy in this study population was 38%. Fourteen of 16 hedgehogs with cardiomyopathy were males and all hedgehogs were adult (>1 year old). Nine hedgehogs exhibited 1 or more of the following clinical signs before death: heart murmur, lethargy, icterus, moist rales, anorexia, dyspnea, dehydration, and weight loss. The remaining 7 hedgehogs died without premonitory clinical signs. Gross findings were cardiomegaly (6 cases), hepatomegaly (5 cases), pulmonary edema (5 cases), pulmonary congestion (4 cases), hydrothorax (3 cases), pulmonary infarct (1 case), renal infarcts (1 case), ascites (1 case), and 5 cases showed no changes. Histologic lesions were found mainly within the left ventricular myocardium and consisted primarily of myodegeneration, myonecrosis, atrophy, hypertrophy, and disarray of myofibers. All hedgehogs with cardiomyopathy had myocardial fibrosis, myocardial edema, or both. Other common histopathologic findings were acute and chronic passive congestion of the lungs, acute passive congestion of the liver, renal tubular necrosis, vascular thrombosis, splenic extramedullary hematopoiesis, and hepatic lipidosis. This is the first report of cardiomyopathy in African hedgehogs. PMID:11021439

  8. Peripartum cardiomyopathy – case series

    Science.gov (United States)

    Prasad, Gowri Sayi; Bhupali, Ashok; Prasad, Sayi; Patil, Ajit N.; Deka, Yashodhan

    2014-01-01

    Objectives To study the pattern of presentation, course of disease and outcome of pregnancy in Peripartum Cardiomyopathy. Methods A prospective study of sixteen cases of PPCM was conducted at Apple Saraswati Multispecialty Hospital and Dr. D.Y. Patil Medical College and Hospital, Kolhapur, Maharashtra, India from January 2006 to December 2012. Data included age distribution, parity, gestational age, symptoms and risk factors. Medical management and pregnancy outcome were documented. Serial echocardiography data was compiled for a period of one year. Results In our study 9/16 (56%) were primigravidae, 4/16 (25%) had pre-eclamsia and 6/16 (35%) had co-existing hypertension. The difference in Echocardiography parameters observed between recovered and non-recovered patients was significant: Left Ventricular End diastolic dimension (5.6 cm vs 6.06 cm), Left Ventricular Ejection Fraction (28.7% vs 22.4%) and Left Ventricular fractional shortening (17.5% vs 13.4%). Thirteen out of sixteen patients were followed up for a period of one year out of which 61% (8/13) patients recovered completely. There was one mortality. Conclusion PPCM is a diagnosis of exclusion. Majority were young primigravidae presenting postnatally. Pre-eclampsia and hypertension were risk factors. ECHO parameters were reliable predictors of recovery. Future pregnancies are better avoided. PMID:24814122

  9. Cardiac MRI in restrictive cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, A. [Department of Cardiovascular Radiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India); Singh Gulati, G., E-mail: gulatigurpreet@rediffmail.com [Department of Cardiovascular Radiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India); Seth, S. [Department of Cardiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India); Sharma, S. [Department of Cardiovascular Radiology, All India Institute of Medical Sciences, Ansari Nagar, Delhi (India)

    2012-02-15

    Restrictive cardiomyopathy (RCM) is a specific group of heart muscle disorders characterized by inadequate ventricular relaxation during diastole. This leads to diastolic dysfunction with relative preservation of systolic function. Although short axis systolic function is usually preserved in RCM, the long axis systolic function may be severely impaired. Confirmation of diagnosis and information regarding aetiology, extent of myocardial damage, and response to treatment requires imaging. Importantly, differentiation from constrictive pericarditis (CCP) is needed, as only the latter is managed surgically. Echocardiography is the initial cardiac imaging technique but cannot reliably suggest a tissue diagnosis; although recent advances, especially tissue Doppler imaging and spectral tracking, have improved its ability to differentiate RCM from CCP. Cardiac catheterization is the reference standard, but is invasive, two-dimensional, and does not aid myocardial characterization. Cardiac magnetic resonance (CMR) is a versatile technique providing anatomical, morphological and functional information. In recent years, it has been shown to provide important information regarding disease mechanisms, and also been found useful to guide treatment, assess its outcome and predict patient prognosis. This review describes the CMR features of RCM, appearances in various diseases, its overall role in patient management, and how it compares with other imaging techniques.

  10. Treatment of Achalasia by Pneumatic Dilatation & Esophagomyotomy

    Directory of Open Access Journals (Sweden)

    M. H. Emami

    2008-01-01

    Full Text Available Background/Objective: Esophagomyotomy and pneumatic dilatation are routine treatments of achalasia. The aim of this study is determine treat-ment effects of these methods."nPatients and Methods: This historical cohort study was done in Esfahan city in 2006. Sixty-six patients with achalasia that treated with pneumatic dilatation (n=46 and esophagomyotomy (n=20 were studied. The data of age, clinical recovery, complications, and satisfaction were collected. Data were analyzed with T-test and x2 and p<0.05 was different significantly."nResults: Clinical recovery was 70% with esophagomyotomy vs 80.5% with pneumatic dilatation (p>0.05. Mean of satisfaction was 8.5±2.37 in esophagomyotomy group Vs 7.68±2.49 in pneumatic dilatation group (P>0.05. Complications were not different significantly in two groups. Radiologic recovery was 66/6% in esophagomyotomy group vs 23.5% in dilatation group (P<0.5."nConclusion: Clinical recovery complications and sat-isfaction in two groups were not different significantly. This study shows need for long-term follow up in two groups.

  11. Multimodality imaging in apical hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Rosario; Parisi; Francesca; Mirabella; Gioel; Gabrio; Secco; Rossella; Fattori

    2014-01-01

    Apical hypertrophic cardiomyopathy(AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the firstline imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease.

  12. Universal decoherence due to gravitational time dilation

    CERN Document Server

    Pikovski, Igor; Costa, Fabio; Brukner, Caslav

    2013-01-01

    Phenomena inherent to quantum theory on curved space-time, such as Hawking radiation, are typically assumed to be only relevant at extreme physical conditions: at high energies and in strong gravitational fields. Here we consider low-energy quantum mechanics in the presence of weak gravitational time dilation and show that the latter leads to universal decoherence of quantum superpositions. Time dilation induces a universal coupling between internal degrees-of-freedom and the centre-of-mass of a composite particle and we show that the resulting entanglement causes the particle's position to decohere. We derive the decoherence timescale and show that the weak time dilation on Earth is already sufficient to decohere micro-scale objects. No coupling to an external environment is necessary, thus even completely isolated composite systems will decohere on curved space-time. In contrast to gravitational collapse models, no modification of quantum theory is assumed. General relativity therefore can account for the e...

  13. Cystic dilation of the ventriculus terminalis

    Science.gov (United States)

    Kawanishi, Masahiro; Tanaka, Hidekazu; Yokoyama, Kunio; Yamada, Makoto

    2016-01-01

    The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. We presented a case with the cystic dilation of the ventriculus terminalis (CDVT) in a symptomatic patient. A 66-year-old female suffered from disturbance while walking evolving for the past 2 years. An MR image revealed a cystic dilatation of ventriculus terminalis. The patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst-subarachnoid shunt. Treatment for asymptomatic patients seems to be the best conducted conservatively, whereas patients with focal neurological deficits seem to be best handled surgically.

  14. Understanding the dilation and dilation relaxation behavior of graphite-based lithium-ion cells

    Science.gov (United States)

    Bauer, Marius; Wachtler, Mario; Stöwe, Hendrik; Persson, Jon V.; Danzer, Michael A.

    2016-06-01

    The dilation of lithium-ion cells is sensitive towards swelling phenomena caused by both graphite staging processes and lithium plating on graphite anodes. In this work, the dilation behavior of graphite/NMC pouch cells is studied with a focus on relaxation phenomena occurring after current pulses. In order to prevent misleading interpretations due to thermal effects, thermal expansion is quantified and a method for the thermal compensation of dilation data is developed. Dilation data are recorded for quasi-equilibrium cycling as well as for current pulses at high rates. In the quasi-equilibrium case, the staging behavior is characterized based on dilation and voltage data. By comparison with a graphite half-cell measurement, the major effects in full cell dilation are confirmed to be anode related. In the high rate case, the dilation responses to the actual pulse and the subsequent relaxation phases are recorded systematically. Positive and negative relaxation phenomena are observed depending on the SOC. They are ascribed to both graphite staging and lithium plating processes. A model is presented explaining the unexpected relaxation effects by a temporary coexistence of three or more staging compounds during high rate lithiation and delithiation. Our data thereby confirm the shrinking annuli model introduced by Heβ and Novák.

  15. AAV micro-dystrophin gene therapy alleviates stress-induced cardiac death but not myocardial fibrosis in >21-m-old mdx mice, an end-stage model of Duchenne muscular dystrophy cardiomyopathy

    OpenAIRE

    Bostick, Brian; Shin, Jin-Hong; Yue, Yongping; Wasala, Nalinda B.; Lai, Yi; Duan, Dongsheng

    2012-01-01

    Duchenne muscular dystrophy (DMD) is a fatal genetic disease caused by the absence of the sarcolemmal protein dystrophin. Dilated cardiomyopathy leading to heart failure is a significant source of morbidity and mortality in DMD. We recently demonstrated amelioration of DMD heart disease in 16 to 20-m-old dystrophin-null mdx mice using adeno-associated virus (AAV) mediated micro-dystrophin gene therapy. DMD patients show severe heart disease near the end of their life expectancy. Similarly, md...

  16. Scintigraphic assessment of cardiac sympathetic innervation with I-123-metaiodobenzylguanidine in cardiomyopathy. Special reference to cardiac arrhythmia

    Energy Technology Data Exchange (ETDEWEB)

    Asano, Takahisa; Otsuka, Nobuaki; Sone, Teruki; Mimura, Hiroaki; Yanagimoto, Shinichi; Tomomitsu, Tatsushi; Fukunaga, Masao [Kawasaki Medical School, Kurashiki, Okayama (Japan); Morita, Koichi

    1999-07-01

    Cardiac sympathetic imagings with I-123-metaiodobenzylguanidine (MIBG) were carried out in 5 cases with dilated cardiomyopathy (DCM), 26 cases with hypertrophic cardiomyopathy (HCM), and 4 cases without cardiac disease as a control to assess cardiac sympathetic innervation qualitatively and quantitatively, and to clarify the relation of MIBG accumulation to arrhythmia. MIBG scintigraphy was performed at 15 min. (early image) and 4 hr. (delayed image) after intravenous injection of MIBG 111 MBq. The MIBG uptake ratio of mediastinum (H/M) and the cardiac washout rate (WR) from early to delayed images were calculated. On both early and delayed SPECTs, MIBG uptake was assessed by defect scores (DSs). Regarding the cases with HCM, the MIBG uptake ratio, WR, and DS were also compared in cases with and without arrhythmia. In DCM, the MIBG uptake on delayed SPECT was markedly low, the H/M ratio was significantly lower, and the DS was significantly higher than in the control (all p<0.05). As for the WR, there was no significant difference between HCM, DCM and the control. In HCM, significantly reduced MIBG uptake was observed in cases with ventricular techycardia (VT) and in cases with atrial fibrillation (Af), as compared with cases without arrhythmia (all p<0.05). There results suggest that MIBG scintigraphy might be a useful tool in the assessment of cardiac sympathetic abnormalities in cardiomyopathy, especially in cases with arrhythmia. (author)

  17. Disease rescue and increased lifespan in a model of cardiomyopathy and muscular dystrophy by combined AAV treatments.

    Directory of Open Access Journals (Sweden)

    Carmen Vitiello

    Full Text Available BACKGROUND: The BIO14.6 hamster is an excellent animal model for inherited cardiomyopathy, because of its lethal and well-documented course, due to a spontaneous deletion of delta-sarcoglycan gene promoter and first exon. The muscle disease is progressive and average lifespan is 11 months, because heart slowly dilates towards heart failure. METHODOLOGY/PRINCIPAL FINDINGS: Based on the ability of adeno-associated viral (AAV vectors to transduce heart together with skeletal muscle following systemic administration, we delivered human delta-sarcoglycan cDNA into male BIO14.6 hamsters by testing different ages of injection, routes of administration and AAV serotypes. Body-wide restoration of delta-SG expression was associated with functional reconstitution of the sarcoglycan complex and with significant lowering of centralized nuclei and fibrosis in skeletal muscle. Motor ability and cardiac functions were completely rescued. However, BIO14.6 hamsters having less than 70% of fibers recovering sarcoglycan developed cardiomyopathy, even if the total rescued protein was normal. When we used serotype 2/8 in combination with serotype 2/1, lifespan was extended up to 22 months with sustained heart function improvement. CONCLUSIONS/SIGNIFICANCE: Our data support multiple systemic administrations of AAV as a general therapeutic strategy for clinical trials in cardiomyopathies and muscle disorders.

  18. Update in cardiomyopathies and congestive heart failure

    Directory of Open Access Journals (Sweden)

    The Heart Hospital, London, UK and Monaldi Hospital, Naples, Italy

    2012-05-01

    Full Text Available This abstract book contains four reports and all abstracts presented to the Joint Meeting: Update in cardiomyopathies and congestive heart failure, 22-23 September 2011 - Naples, Italy, endorsed by the Working Group on Myocardial and Pericardial Diseases (WG 21 of the European Society of Cardiology (ESC.

  19. Contractile Dysfunction in Sarcomeric Hypertrophic Cardiomyopathy.

    Science.gov (United States)

    MacIver, David H; Clark, Andrew L

    2016-09-01

    The pathophysiological mechanisms underlying the clinical phenotype of sarcomeric hypertrophic cardiomyopathy are controversial. The development of cardiac hypertrophy in hypertension and aortic stenosis is usually described as a compensatory mechanism that normalizes wall stress. We suggest that an important abnormality in hypertrophic cardiomyopathy is reduced contractile stress (the force per unit area) generated by myocardial tissue secondary to abnormalities such as cardiomyocyte disarray. In turn, a progressive deterioration in contractile stress provokes worsening hypertrophy and disarray. A maintained or even exaggerated ejection fraction is explained by the increased end-diastolic wall thickness producing augmented thickening. We propose that the nature of the hemodynamic load in an individual with hypertrophic cardiomyopathy could determine its phenotype. Hypertensive patients with hypertrophic cardiomyopathy are more likely to develop exaggerated concentric hypertrophy; athletic individuals an asymmetric pattern; and inactive individuals a more apical hypertrophy. The development of a left ventricular outflow tract gradient and mitral regurgitation may be explained by differential regional strain resulting in mitral annular rotation.

  20. Prevalence of hypertrophic cardiomyopathy in China

    Institute of Scientific and Technical Information of China (English)

    Tsung O Cheng

    2004-01-01

    @@ To the Editor: I read with interest the recent article on cardiac troponin T (TNNT2) mutations in Chinese patients with hypertrophic cardiomyopathy (HCM) by Wu et al.1 The authors cited a prevalence of HCM of 0.2% in general population, but did not indicate whether it referred to the general population in China or some other countries.

  1. Clinical and molecular classification of cardiomyopathies

    Directory of Open Access Journals (Sweden)

    Franco Cecchi

    2012-07-01

    Full Text Available The term “cardiomyopathies” was used for the first time 55 years ago, in 1957. Since then awareness and knowledge of this important and complex group of heart muscle diseases have improved substantially. Over these past five decades a large number of definitions, nomenclature and schemes, have been advanced by experts and consensus panel, which reflect the fast and continued advance of the scientific understanding in the field. Cardiomyopathies are a heterogeneous group of inherited myocardial diseases, which represent an important cause of disability and adverse outcome. Although considered rare diseases, the overall estimated prevalence of all cardiomyopathies is at least 3% in the general population worldwide. Furthermore, their recognition is increasing due to advances in imaging techniques and greater awareness in both the public and medical community. Cardiomyopathies represent an ideal translational model of integration between basic and clinical sciences. A multidisciplinary approach is therefore essential in order to ensure their correct diagnosis and management. In the present work, we aim to provide a concise overview of the historical background, genetic and phenotypic spectrum and evolving concepts leading to the various attempts of cardiomyopathy classifications produced over the decades.

  2. A therapeutic anti-CD4 monoclonal antibody inhibits T cell receptor signal transduction in mouse autoimmune cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    WANG Zhao-hui; LIAO Yu-hua; YUAN Jing; ZHANG Li; WANG Min; ZHANG Jing-hui; LIU Zhong-ping; DONG Ji-hua

    2007-01-01

    Background T cell immune abnormalities in patients with dilated cardiomyopathy (DCM) has been intensively studied over the past 10 years. Our previous study has suggested that immunization of mice with the peptides derived from human adenine nucleotide translocator (ANT) result in the production of autoantibodies against the ANT and histopathological changes similar to those in human DCM. The ANT peptides can induce autoimmune cardiomyopathy like DCM in Balb/c mice. In this study we aimed to focus on the molecular mechanism of T cells in the autoimmune cardiomyopathy mouse model by detecting the expression of the two T cell signaling molecules.Methods The ANT peptides were used to cause autoimmune cardiomyopathy in Balb/c mice. Anti-L3T4 or rat anti-mouse IgG was administered to the mice (n=6 in each group) simultaneously immunized with ANT. ELISA analysis was used to detect autoantibodies against the ANT peptides and the percentages of interferon-Y and interleukin-4 producing cells among splenic CD4+ lymphocytes was determined by using flow cytometry analysis. The expression of CD45 in spleen T cells was determined by immunohistochemistry and the mRNAs of T cell signaling molecules were detected by real-time PCR.Results Treatment of ANT immunized Balb/c mice with anti-CD4 mAb caused a reduction in the gene expression of P56lck and Zap-70 and a lower level of CD45 expression by spleen T cells. Aiso, a reverse of the Th1/Th2 ratio that results in the reduced production of antibodies against ANT was found in the anti-CD4 monoclonal antibodies (mAb)group. Whereas irrelevant antibody (rat anti-mouse IgG) did not suppress T cell signaling molecules nor inhibit CD45 expression, and control-antibody mice did not show any significant differences compared with the DCM group.Conclusion The results show that anti-CD4 mAb is a powerful inhibitor of the early initiating events of T cell receptor(TCR) signal transduction in mouse autoimmune dilated cardiomyopathy.

  3. Desmosomal cadherins are decreased in explanted arrhythmogenic right ventricular dysplasia/cardiomyopathy patient hearts.

    Directory of Open Access Journals (Sweden)

    Alexia Vite

    Full Text Available AIMS: Arrhythmogenic right ventricular Dysplasia/cardiomyopathy (ARVD/C is an autosomal dominant inherited cardiomyopathy associated with ventricular arrhythmia, heart failure and sudden death. Genetic studies have demonstrated the central role of desmosomal proteins in this disease, where 50% of patients harbor a mutation in a desmosmal gene. However, clinical diagnosis of the disease remains difficult and molecular mechanisms appears heterogeneous and poorly understood. The aim of this study was to characterize the expression profile of desmosomal proteins in explanted ARVD/C heart samples, in order to identify common features of the disease. METHODS AND RESULTS: We examined plakophilin-2, desmoglein-2, desmocollin-2, plakoglobin and β-catenin protein expression levels from seven independent ARVD/C heart samples compared to two ischemic, five dilated cardiomyopathy and one healthy heart sample as controls. Ventricular and septum sections were examined by immunoblot analysis of total heart protein extracts and by immunostaining. Immunoblots indicated significant decreases in desmoglein-2 and desmocollin-2, independent of any known underlying mutations, whereas immune-histochemical analysis showed normal localization of all desmosomal proteins. Quantitative RT-PCR revealed normal DSG2 and DSC2 mRNA transcript levels, suggesting increased protein turn-over rather than transcriptional down regulation. CONCLUSION: Reduced cardiac desmoglein-2 and desmocollin-2 levels appear to be specifically associated with ARVD/C, independent of underlying mutations. These findings highlight a key role of desmosomal cadherins in the pathophysiology of ARVD/C. Whether these reductions could be considered as specific markers for ARVD/C requires replication analysis.

  4. Sex dimorphisms of crossbridge cycling kinetics in transgenic hypertrophic cardiomyopathy mice.

    Science.gov (United States)

    Birch, Camille L; Behunin, Samantha M; Lopez-Pier, Marissa A; Danilo, Christiane; Lipovka, Yulia; Saripalli, Chandra; Granzier, Henk; Konhilas, John P

    2016-07-01

    Familial hypertrophic cardiomyopathy (HCM) is a disease of the sarcomere and may lead to hypertrophic, dilated, restrictive, and/or arrhythmogenic cardiomyopathy, congestive heart failure, or sudden cardiac death. We hypothesized that hearts from transgenic HCM mice harboring a mutant myosin heavy chain increase the energetic cost of contraction in a sex-specific manner. To do this, we assessed Ca(2+) sensitivity of tension and crossbridge kinetics in demembranated cardiac trabeculas from male and female wild-type (WT) and HCM hearts at an early time point (2 mo of age). We found a significant effect of sex on Ca(2+) sensitivity such that male, but not female, HCM mice displayed a decrease in Ca(2+) sensitivity compared with WT counterparts. The HCM transgene and sex significantly impacted the rate of force redevelopment by a rapid release-restretch protocol and tension cost by the ATPase-tension relationship. In each of these measures, HCM male trabeculas displayed a gain-of-function when compared with WT counterparts. In addition, cardiac remodeling measured by echocardiography, histology, morphometry, and posttranslational modifications demonstrated sex- and HCM-specific effects. In conclusion, female and male HCM mice display sex dimorphic crossbridge kinetics accompanied by sex- and HCM-dependent cardiac remodeling at the morphometric, histological, and cellular level.

  5. Sex dimorphisms of crossbridge cycling kinetics in transgenic hypertrophic cardiomyopathy mice.

    Science.gov (United States)

    Birch, Camille L; Behunin, Samantha M; Lopez-Pier, Marissa A; Danilo, Christiane; Lipovka, Yulia; Saripalli, Chandra; Granzier, Henk; Konhilas, John P

    2016-07-01

    Familial hypertrophic cardiomyopathy (HCM) is a disease of the sarcomere and may lead to hypertrophic, dilated, restrictive, and/or arrhythmogenic cardiomyopathy, congestive heart failure, or sudden cardiac death. We hypothesized that hearts from transgenic HCM mice harboring a mutant myosin heavy chain increase the energetic cost of contraction in a sex-specific manner. To do this, we assessed Ca(2+) sensitivity of tension and crossbridge kinetics in demembranated cardiac trabeculas from male and female wild-type (WT) and HCM hearts at an early time point (2 mo of age). We found a significant effect of sex on Ca(2+) sensitivity such that male, but not female, HCM mice displayed a decrease in Ca(2+) sensitivity compared with WT counterparts. The HCM transgene and sex significantly impacted the rate of force redevelopment by a rapid release-restretch protocol and tension cost by the ATPase-tension relationship. In each of these measures, HCM male trabeculas displayed a gain-of-function when compared with WT counterparts. In addition, cardiac remodeling measured by echocardiography, histology, morphometry, and posttranslational modifications demonstrated sex- and HCM-specific effects. In conclusion, female and male HCM mice display sex dimorphic crossbridge kinetics accompanied by sex- and HCM-dependent cardiac remodeling at the morphometric, histological, and cellular level. PMID:27199124

  6. Inflammatory Cardiomyopathy: A Current View on the Pathophysiology, Diagnosis, and Treatment

    Directory of Open Access Journals (Sweden)

    Jan Krejci

    2016-01-01

    Full Text Available Inflammatory cardiomyopathy is defined as inflammation of the heart muscle associated with impaired function of the myocardium. In our region, its etiology is most often viral. Viral infection is a possible trigger of immune and autoimmune mechanisms which contributed to the damage of myocardial function. Myocarditis is considered the most common cause of dilated cardiomyopathy. Typical manifestation of this disease is heart failure, chest pain, or arrhythmias. The most important noninvasive diagnostic method is magnetic resonance imaging, but the gold standard of diagnostics is invasive examination, endomyocardial biopsy. In a significant proportion of cases with impaired left ventricular systolic function, recovery occurs spontaneously in several weeks and therefore it is appropriate to postpone critical therapeutic decisions about 3–6 months after start of the treatment. Therapy is based on standard heart failure treatment; immunosuppressive or antimicrobial treatment may be considered in some cases depending on the results of endomyocardial biopsy. If severe dysfunction of the left ventricle persists, device therapy may be needed.

  7. Inflammatory Cardiomyopathy: A Current View on the Pathophysiology, Diagnosis, and Treatment.

    Science.gov (United States)

    Krejci, Jan; Mlejnek, Dalibor; Sochorova, Dana; Nemec, Petr

    2016-01-01

    Inflammatory cardiomyopathy is defined as inflammation of the heart muscle associated with impaired function of the myocardium. In our region, its etiology is most often viral. Viral infection is a possible trigger of immune and autoimmune mechanisms which contributed to the damage of myocardial function. Myocarditis is considered the most common cause of dilated cardiomyopathy. Typical manifestation of this disease is heart failure, chest pain, or arrhythmias. The most important noninvasive diagnostic method is magnetic resonance imaging, but the gold standard of diagnostics is invasive examination, endomyocardial biopsy. In a significant proportion of cases with impaired left ventricular systolic function, recovery occurs spontaneously in several weeks and therefore it is appropriate to postpone critical therapeutic decisions about 3-6 months after start of the treatment. Therapy is based on standard heart failure treatment; immunosuppressive or antimicrobial treatment may be considered in some cases depending on the results of endomyocardial biopsy. If severe dysfunction of the left ventricle persists, device therapy may be needed. PMID:27382566

  8. Transient Myocarditis and Cardiomyopathy After Scorpion and Spider Envenomation

    Directory of Open Access Journals (Sweden)

    Mustafa Kır

    2011-08-01

    Full Text Available Introduction: Spider and scorpion stings can cause multiple clinical manifestations such as local skin reactions or multiple organ failure that can cause death. Multi-organ involvement is more frequent in children due to their lower body weight. The most important life threatening event after the sting is cardiac and lung involvement. In this case report, two cases who developed myocarditis and cardiomyopathy following scorpion and spider stings were reported.Case Report: Clinical evaluation of a ten-year-old boy with respiratory distress and tachycardia after being bitten by a spider on his right hand revealed high levels of cardiac enzymes [CK-MB: 16.5 ng/ml (N:0.0-7.2 ng/ml, troponin: 3.06 ng/ml (N:0.0-0.3ng/ml], pathological ST elevations in leads V3 and V4, and T wave negativity in leads V5 and V6. In echocardiography, left ventricular dilatation and moderate systolic dysfunction were found. Antivenom [Serum antiscorpionique (labs 50] was given (5 cc antivenom was administered intravenously following a 1:10 dilution with normal saline. With supportive treatment, all pathological findings resolved in a week. The second case was an eight-year-old boy who had been bitten by a scorpion on his foot and taken to the intensive care unit because of respiratory distress and convulsion. Two doses of antivenom were given to the case who had elevated levels of troponin and CK-MB, pathological ST depressions in ECG, and left ventricular dilatation and systolic dysfunction, as revealed by echocardiography. With dobutamin and supportive treatment, the pathological findings normalized in ten days.Conclusion: Myocarditis developing following spider or scorpion bites can threaten life due to left ventricular systolic dysfunction. This consequence has been attributed to increased catecholaminergic activity or direct effect of toxin to myocardial fibres. ECG must be performed, cardiac enzymes must be monitored and echocardiography must be done to evaluate

  9. Aortic biomechanics in hypertrophic cardiomyopathy

    Science.gov (United States)

    Badran, Hala Mahfouz; Soltan, Ghada; Faheem, Nagla; Elnoamany, Mohamed Fahmy; Tawfik, Mohamed; Yacoub, Magdi

    2015-01-01

    Background: Ventricular-vascular coupling is an important phenomenon in many cardiovascular diseases. The association between aortic mechanical dysfunction and left ventricular (LV) dysfunction is well characterized in many disease entities, but no data are available on how these changes are related in hypertrophic cardiomyopathy (HCM). Aim of the work: This study examined whether HCM alone is associated with an impaired aortic mechanical function in patients without cardiovascular risk factors and the relation of these changes, if any, to LV deformation and cardiac phenotype. Methods: 141 patients with HCM were recruited and compared to 66 age- and sex-matched healthy subjects as control group. Pulse pressure, aortic strain, stiffness and distensibility were calculated from the aortic diameters measured by M-mode echocardiography and blood pressure obtained by sphygmomanometer. Aortic wall systolic and diastolic velocities were measured using pulsed wave Doppler tissue imaging (DTI). Cardiac assessment included geometric parameters and myocardial deformation (strain and strain rate) and mechanical dyssynchrony. Results: The pulsatile change in the aortic diameter, distensibility and aortic wall systolic velocity (AWS') were significantly decreased and aortic stiffness index was increased in HCM compared to control (P < .001) In HCM AWS' was inversely correlated to age(r = − .32, P < .0001), MWT (r = − .22, P < .008), LVMI (r = − .20, P < .02), E/Ea (r = − .16, P < .03) LVOT gradient (r = − 19, P < .02) and severity of mitral regurg (r = − .18, P < .03) but not to the concealed LV deformation abnormalities or mechanical dyssynchrony. On multivariate analysis, the key determinant of aortic stiffness was LV mass index and LVOT obstruction while the role LV dysfunction in aortic stiffness is not evident in this population. Conclusion: HCM is associated with abnormal aortic mechanical properties. The severity of cardiac

  10. Polymorphism in the alpha cardiac muscle actin 1 gene is associated to susceptibility to chronic inflammatory cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Amanda Farage Frade

    Full Text Available AIMS: Chagas disease, caused by the protozoan Trypanosoma cruzi is endemic in Latin America, and may lead to a life-threatening inflammatory dilated, chronic Chagas cardiomyopathy (CCC. One third of T. cruzi-infected individuals progress to CCC while the others remain asymptomatic (ASY. A possible genetic component to disease progression was suggested by familial aggregation of cases and the association of markers of innate and adaptive immunity genes with CCC development. Since mutations in multiple sarcomeric genes, including alpha-cardiac actin (ACTC1 have been involved in hereditary dilated cardiomyopathy, we investigated the involvement of the ACTC1 gene in CCC pathogenesis. METHODS AND RESULTS: We conducted a proteomic and genetic study on a Brazilian study population. The genetic study was done on a main cohort including 118 seropositive asymptomatic subjects and 315 cases and the replication was done on 36 asymptomatic and 102 CCC cases. ACTC1 protein and mRNA levels were lower in myocardial tissue from patients with end-stage CCC than those found in hearts from organ donors. Genotyping a case-control cohort of CCC and ASY subjects for all informative single nucleotide polymorphism (SNP in the ACTC1 gene identified rs640249 SNP, located at the 5' region, as associated to CCC. Associations are borderline after correction for multiple testing. Correlation and haplotype analysis led to the identification of a susceptibility haplotype. Functional assays have shown that the rs640249A/C polymorphism affects the binding of transcriptional factors in the promoter regions of the ACTC1 gene. Confirmation of the detected association on a larger independent replication cohort will be useful. CONCLUSIONS: Genetic variations at the ACTC1 gene may contribute to progression to chronic Chagas Cardiomyopathy among T. cruzi-infected patients, possibly by modulating transcription factor binding to ACTC1 promoter regions.

  11. Pupil dilation betrays the timing of decisions

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    Wolfgang Einhauser

    2010-02-01

    Full Text Available The notion of mind-reading by carefully observing another individual's physiological responses has recently become commonplace in popular culture, particularly in the context of brain imaging. The question remains, however, whether outwardly accessible physiological signals indeed betray a decision before a person voluntarily reports it. In one experiment we asked observers to push a button at any time during a 10-second period (“immediate overt response”. In a series of three additional experiments observers were asked to select one number from five sequentially presented digits but concealed their decision until the trial’s end (“covert choice”. In these experiments observers either had to choose the digit themselves under conditions of reward and no reward, or were instructed which digit to select via an external cue provided at the time of the digit presentation. In all cases pupil dilation alone predicted the choice (timing of button response or chosen digit, respectively. Consideration of the average pupil-dilation responses, across all experiments, showed that this prediction of timing was distinct from a general arousal or reward-anticipation response. Furthermore, the pupil dilation appeared to reflect the post-decisional consolidation of the selected outcome rather than the pre-decisional cognitive appraisal component of the decision. Given the tight link between pupil dilation and norepinephrine levels during constant illumination, our results have implications beyond the tantalizing mind-reading speculations. These findings suggest that similar noradrenergic mechanisms may underlie the consolidation of both overt and covert decisions.

  12. Surface dilatational viscosity of Langmuir monolayers

    Science.gov (United States)

    Lopez, Juan; Vogel, Michael; Hirsa, Amir

    2003-11-01

    With increased interest in microfluidic systems, interfacial phenomena is receiving more attention. As the length scales of fluid problems decrease, the surface to volume ratio increases and the coupling between interfacial flow and bulk flow becomes increasingly dominated by effects due to intrinsic surface viscosities (shear and dilatational), in comparison to elastic effects (due to surface tension gradients). The surface shear viscosity is well-characterized, as cm-scale laboratory experiments are able to isolate its effects from other interfacial processes (e.g., in the deep-channel viscometer). The same is not true for the dilatational viscosity, because it acts in the direction of surface tension gradients. Their relative strength scale with the capillary number, and for cm-scale laboratory flows, surface tension effects tend to dominate. In microfluidic scale flows, the scaling favors viscosity. We have devised an experimental apparatus which is capable of isolating and enhancing the effects of dilatational viscosity at the cm scales by driving the interface harmonically in time, while keeping the interface flat. In this talk, we shall present both the theory for how this works as well as experimental measurements of surface velocity from which we deduce the dilatational viscosity of several monolayers on the air-water interface over a substantial range of surface concentrations. Anomalous behavior over some range of concentration, which superficially indicates negative viscosity, maybe explained in terms of compositional effects due to large spatial and temporal variations in concentration and corresponding viscosity.

  13. Aerated bunker discharge of fine dilating powders

    NARCIS (Netherlands)

    Ouwerkerk, C.E.D.; Molenaar, H.J.; Frank, M.J.W.

    1992-01-01

    The discharge rate of coarse powders (mean particle size 500 ¿m) from bunkers without aeration can be described by both empirical relations and theoretical models. In the case of small particles the discharge rate is largely overestimated. As the powder dilates during flow a negative pressure gradie

  14. Pupils dilate for vocal or familiar music.

    Science.gov (United States)

    Weiss, Michael W; Trehub, Sandra E; Schellenberg, E Glenn; Habashi, Peter

    2016-08-01

    Previous research reveals that vocal melodies are remembered better than instrumental renditions. Here we explored the possibility that the voice, as a highly salient stimulus, elicits greater arousal than nonvocal stimuli, resulting in greater pupil dilation for vocal than for instrumental melodies. We also explored the possibility that pupil dilation indexes memory for melodies. We tracked pupil dilation during a single exposure to 24 unfamiliar folk melodies (half sung to la la, half piano) and during a subsequent recognition test in which the previously heard melodies were intermixed with 24 novel melodies (half sung, half piano) from the same corpus. Pupil dilation was greater for vocal melodies than for piano melodies in the exposure phase and in the test phase. It was also greater for previously heard melodies than for novel melodies. Our findings provide the first evidence that pupillometry can be used to measure recognition of stimuli that unfold over several seconds. They also provide the first evidence of enhanced arousal to vocal melodies during encoding and retrieval, thereby supporting the more general notion of the voice as a privileged signal. (PsycINFO Database Record PMID:27123682

  15. The MEF2A gene is excluded as a candidate in 3 families with cardiomyopathies that are not linked to myosin

    Energy Technology Data Exchange (ETDEWEB)

    Bachinski, L.L.; Abchee, A. [Baylor College of Medicine, Houston, TX (United States); Krahe, R. [Dupont Institute, Wilmington, DE (United States)] [and others

    1994-09-01

    Inherited cardiomyopathies, as exemplified by hypertrophic cardiomyopathy (HCM) and idiopathic dilated cardiomyopathy (DCM), are genetically heterogeneous disorders of the myocardium which serve as paradigms for the growth response of the heart to most stimuli. HCM is an autosomal dominant disease shown to be caused by at least 5 different genes, but {beta}-myosin heavy chain (MYH7) is the only one of these genes to be identified to date. No loci have as yet been identified for DCM. MEF2A is one of at least 4 known MEF2 genes which are members of the MADS box family of transcription factors. MEF2A is expressed in cardiac tissue; thus MEF2A is a logical candidate gene for these disorders of the cardiac growth response. We have investigated the possibility of linkage between a trinucleotide repeat polymorphism in the MEF2A gene and these cardiomyopathies is a DCM family and 2 HCM families not linked to MYH7. MEF2A was excluded as a candidate for DCM (LOD of -9.03) and HCM (LODs of-5.43 and -2.44) in these families. No expansion of this trinucleotide repeat was seen in 100 unrelated HCM probands or in the DCM family, which appears to exhibit anticipation.

  16. 全方向M型超声心动图评价冠状动脉疾病与扩张型心肌病心力衰竭患者左心室短轴的局部收缩功能%Evaluation of regional myocardial function of left ventricular short-axis in patients with heart failure of coronary artery disease and dilated cardiomyopathy with omni-directional M-mode echocardiography

    Institute of Scientific and Technical Information of China (English)

    郭薇; 练敏

    2012-01-01

    To evaluate the regional myocardial function of left ventricular short-axis in heart failure patients with coronary artery disease (CAD) and dilated cardiomyopath (DCM)by omni-directional M-mode echocardiography (OME). Methods Totally 30 patients with DCM (DCM group), 30 patients with CAD (CAD group) and 30 healthy subjects as control group were selected , and CAD group was divided into CAD non-ischemic segment subgroup and CAD ische-mic segment subgroup according to the result of coronary angiography. The left ventricular short-axis 16 segment's curves of OME in every group were collected, and endocardial motion velocity (V), accelerated (A) and relatively force (F) of the peak systolic period were measured and compared. Results The phases of V, A, F' in DCM group were significantly lower than the corresponding 16 segments in control group (all P 0. 05), in the other segments decreased significantly (all P<0. 05), while the A, F' of 16 segments significantly decreased (all P<0.05). Conclusion A and F' are sensitive than V in evaluating the abnonmal regional systolic function. OME can evaluate regional systolic function in heart failure patients with DCM and CAD.%目的 应用全方向M型超声心动图(OME)评价冠心病(CAD)及扩张型心肌病(DCM)所致的心力衰竭患者左心室短轴的局部收缩功能.方法 以DCM组30例、CAD组30例患者及对照组30名健康志愿者作为研究对象.依据冠状动脉造影结果将CAD组分为CAD缺血节段亚组及CAD非缺血节段亚组,采集各组左心室短轴16个节段的全方向M型超声心动图曲线,分别测量收缩期内膜峰值运动速度(V)、加速度(A)、收缩期运动峰值相对力(F’)并进行对照分析.结果 与对照组16个节段相应室壁比较,DCM组的V、A、F’均明显减低(P均<0.05);CAD缺血节段亚组分别与CAD非缺血节段亚组及对照组的16个节段相应室壁比较,V除在基底段的侧壁、下壁、后间隔,乳头肌段的下壁、后

  17. CMR findings in patients with hypertrophic cardiomyopathy and atrial fibrillation

    Directory of Open Access Journals (Sweden)

    Dinter Dietmar

    2009-09-01

    Full Text Available Abstract Objectives We sought to evaluate the relation between atrial fibrillation (AF and the extent of myocardial scarring together with left ventricular (LV and atrial parameters assessed by late gadolinium-enhancement (LGE cardiovascular magnetic resonance (CMR in patients with hypertrophic cardiomyopathy (HCM. Background AF is the most common arrhythmia in HCM. Myocardial scarring is also identified frequently in HCM. However, the impact of myocardial scarring assessed by LGE CMR on the presence of AF has not been evaluated yet. Methods 87 HCM patients underwent LGE CMR, echocardiography and regular ECG recordings. LV function, volumes, myocardial thickness, left atrial (LA volume and the extent of LGE, were assessed using CMR and correlated to AF. Additionally, the presence of diastolic dysfunction and mitral regurgitation were obtained by echocardiography and also correlated to AF. Results Episodes of AF were documented in 37 patients (42%. Indexed LV volumes and mass were comparable between HCM patients with and without AF. However, indexed LA volume was significantly higher in HCM patients with AF than in HCM patients without AF (68 ± 24 ml·m-2 versus 46 ± 18 ml·m-2, p = 0.0002, respectively. The mean extent of LGE was higher in HCM patients with AF than those without AF (12.4 ± 14.5% versus 6.0 ± 8.6%, p = 0.02. When adjusting for age, gender and LV mass, LGE and indexed LA volume significantly correlated to AF (r = 0.34, p = 0.02 and r = 0.42, p Conclusion HCM patients with AF display significantly more LGE than HCM patients without AF. However, the extent of LGE is inferior to the LA size for predicting AF prevalence. LA dilation is the strongest determinant of AF in HCM patients, and is related to the extent of LGE in the LV, irrespective of LV mass.

  18. Advanced Electrocardiography Can Identify Occult Cardiomyopathy in Doberman Pinschers

    Science.gov (United States)

    Spiljak, M.; Petric, A. Domanjko; Wilberg, M.; Olsen, L. H.; Stepancic, A.; Schlegel, T. T.; Starc, V.

    2011-01-01

    Recently, multiple advanced resting electrocardiographic (A-ECG) techniques have improved the diagnostic value of short-duration ECG in detection of dilated cardiomyopathy (DCM) in humans. This study investigated whether 12-lead A-ECG recordings could accurately identify the occult phase of DCM in dogs. Short-duration (3-5 min) high-fidelity 12-lead ECG recordings were obtained from 31 privately-owned, clinically healthy Doberman Pinschers (5.4 +/- 1.7 years, 11/20 males/females). Dogs were divided into 2 groups: 1) 19 healthy dogs with normal echocardiographic M-mode measurements: left ventricular internal diameter in diastole (LVIDd . 47mm) and in systole (LVIDs . 38mm) and normal 24-hour ECG recordings (occult DCM: 11/12 dogs had increased M-mode measurements (LVIDd . 49mm and/or LVIDs . 40mm) and 5/11 dogs had also >100 VPCs/24h; 1/12 dogs had only abnormal 24-hour ECG recordings (>100 VPCs/24h). ECG recordings were evaluated via custom software programs to calculate multiple parameters of high-frequency (HF) QRS ECG, heart rate variability, QT variability, waveform complexity and 3-D ECG. Student's t-tests determined 19 ECG parameters that were significantly different (P occult DCM. For the 5 selected parameters the prediction of occult DCM was performed using a binary logistic regression model with Chi-square tested significance (P occult ECG with sensitivity 89% and specificity 83%. Results suggest that 12-lead A-ECG might improve diagnostic value of short-duration ECG in earlier detection of canine DCM as five selected ECG parameters can with reasonable accuracy identify occult DCM in Doberman Pinschers. Future extensive clinical studies need to clarify if 12-lead A-ECG could be useful as an additional screening test for canine DCM.

  19. Balloon dilation versus Amplatz dilation during ultrasound-guided percutaneous nephrolithotomy for staghorn stones

    Institute of Scientific and Technical Information of China (English)

    Ren Minghua; Zhang Cheng; Fu Weijun; Fu Yiming; Ma Li; Zhao Weiming; Xu Wanhai

    2014-01-01

    Background Amplatz dilation and balloon dilation are different methods in creating the accesses during percutaneous nephrolithotomy (PCNL).The aim of this study was to review the surgical experiences of managing staghorn calculi by Amplatz dilation and balloon dilation for 3 years.Methods We retrospectively analyzed clinical data from 125 patients (129 kidneys)with staghorn kidney stones who underwent PCNL from January 2010 to December 2012,of whom 60 patients underwent Amplatz dilation (AD group) and 65 underwent balloon dilation (BD group) during PCNL.Results The AD and BD groups were similar in age,male-female ratio,stone burden,stone type,hydronephrosis,and proportion of patients who had undergone extracorporeal lithotripsy.However,these two groups showed significant differences in terms of duration of percutaneous access (15.1±3.6) minutes vs.(10.0±3.3) minutes,one-attempt success rate of dilation via a single access 88.9% (72/81) vs.97.8% (91/93),hemoglobin drop after surgery (3.5±0.9) g/dl vs.(1.7±0.9) g/dl,number of cases requiring intraoperative and postoperative blood transfusion 27.9% (n=17) vs.13.2% (n=9),changes of central venous pressure before and after surgery (2.3±1.2) cmH2O vs.(1.2±0.7) cmH2O,number of patients who experienced postoperative fever >37.5℃ 21 (34.4%) vs.13 (19.1%) (all P <0.05).No injury of adjacent organs,including pleura,liver,spleen,or bowel,was noted in patients.Conclusions During ultrasound-guided PCNL for staghorn stones,balloon dilation and Amplatz dilation are all effective and safe.Compared with Amplatz dilation,balloon dilation is a better choice,as it has a higher access creation success rate,shorter access creation time less blood loss,and lower proportions of circulatory overload and postoperative fever.

  20. Desmin myopathy with severe cardiomyopathy in a Uruguayan family due to a codon deletion in a new location within the desmin 1A rod domain.

    Science.gov (United States)

    Vernengo, Luis; Chourbagi, Oussama; Panuncio, Ana; Lilienbaum, Alain; Batonnet-Pichon, Sabrina; Bruston, Francine; Rodrigues-Lima, Fernando; Mesa, Rosario; Pizzarossa, Carlos; Demay, Laurence; Richard, Pascale; Vicart, Patrick; Rodriguez, Maria-Mirta

    2010-03-01

    Desmin myopathy is a heterogeneous neuromuscular disorder characterized by skeletal myopathy and cardiomyopathy, inherited mostly in an autosomal dominant pattern. We report a five generation Uruguayan family with severe cardiomyopathy and skeletal myopathy. Its most striking features are: atrial dilation, arrhythmia, conduction block and sudden death due to conduction impairment. Affected skeletal muscle shows alteration of mitochondria with paracrystallin inclusions and granulofilamentous material scattered in the muscle fibres. This family carries an unusual deletion p.E114del within the 1A rod domain of desmin. Transfected cells expressing the mutated desmin show punctuated and speckled cytoplasmic aggregates. The mutation causes a local conformational change in heptads a/d residues and charge positions. These findings lead to the hypothesis that coiled-coil interactions may be impaired, resulting in severe alterations in the desmin network. This is the first time that a mutation affecting this domain in the desmin molecule is described in a desminopathy.