WorldWideScience

Sample records for cardiomyopathy dilated

  1. Dilated cardiomyopathy

    Science.gov (United States)

    Cardiomyopathy - dilated ... The most common causes of dilated cardiomyopathy are: Heart disease caused by a narrowing of the arteries Poorly controlled high blood pressure There are many other causes of dilated ...

  2. Dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Radionuclide techniques are easily obtainable, noninvasive examinations that provide useful information in the evaluation, diagnosis and management of patients with dilated cardiomyopathy. The gated blood pool scan allows the assessment of ventricular size, configuration, and wall and septal thickness. These data allow the functional class of the cardiomyopathy (congestive, restrictive or hypertrophic) to be defined. Often THallium-201 myocardial perfusion imaging adds further information and is particularly useful in distinguishing congestive cardiomyopathy from severe coronary artery disease and in depicting septal abnormalities in hipertrophic cardiomyopathy. Useful as these techniques are, they are not substitutes for conventional approaches to diagnosis. Careful history taking and physical examination, as well as scrutiny of the electrocardiogram, chest X-ray and echocardiogram should be standard practice for the evaluation of patients with suspected cardiomyopathy. Judicious use of noninvasive techniques may obviate the need for cardiac catheterization in many patients

  3. 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...

  4. Dilated cardiomyopathy following trastuzumab chemotherapy

    Directory of Open Access Journals (Sweden)

    Saurabh Karmakar

    2012-01-01

    Cardiotoxicity manifesting as dilated cardiomyopathy is a rarely reported adverse effect of trastuzumab. We hereby report a case of dilated cardiomyopathy, which occurred following trastuzumab chemotherapy in a 32-year-old female. The patient responded to discontinuation of trastuzumab and standard medical treatment. Extensive search of Indian literature revealed no reported case of dilated cardiomyopathy occurring due to trastuzumab.

  5. Severity of dilated cardiomyopathy (DCM)

    International Nuclear Information System (INIS)

    In order to assess the severity of dilated cardiomyopathy, thallium scan was performed in 60 cases. In 16 of all, serial thallium scan was performed in the periods of average 26 months. In these cases, extension of perfusion defect was observed from apical to inferoposterior regions. Therefore, we classified dilated cardiomyopathy into three groups by thallium scan; (I) dilated left ventricular type (n = 16), (II) apical hypoperfusion type (n = 12), (III) inferoposterior perfusion defect type (n = 32). These groups were correlated with hemodynamic findings. All patients had also cardiac catheterization and gated blood pool scan. As results, group III had high incidence of right ventricular and lung thallium uptake and patchy pattern compared to other groups. Group III had also high incidence of dyspnoea on exertion, S3 and ECG abnormalities. In hemodynamics, end-diastolic ventricular volume index and end-systolic ventricular volume index increased and, right ventricular ejection fraction and left ventricular ejection fraction decreased according to the severity of dilated cardiomyopathy from group I to III. In addition, the incidence of mitral regurgitation and dyskinesis was observed highly in group III. In conclusion, perfusion defect was frequently demonstrated in dilated cardiomyopathy without coronary artery stenosis. And right and left ventricular function was depressed according to the extension of perfusion defect. (author)

  6. 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 ...

  7. 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...

  8. 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)....

  9. Differentiation of ischemic cardiomyopathy from idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    To determine whether ischemic cardiomyopathy (ischemic DCM) could be distinguished from idiopathic dilated cardiomyopathy (idiopathic DCM) by radionuclide cardiac imaging, seven patients with ischemic DCM and eight patients with idiopathic DCM were studied with thallium scanning and gated cardiac blood pool imaging at rest and during exercise. The resting ejection fraction of the two groups were similar (ischemic DCM 20.7+-9.0%, idiopathic DCM 26.0+-13.4%, ns), and the change in the ejection fraction during exercise were also similar. Both groups showed no increment of ejection fraction during exercise. Thallium scan showed perfusion defect in 14 of 15 patients. The perfusion defects were extensive in ischemic DCM than idiopathic DCM (defect score 49.6+-5.7 vs. 21.1+-13.2%, p>0.001). Moreover, the distribution of the defects were different; in ischemic DCM the defects were distributed according to the territory of coronary arteries, but in idiopathic DCM, defects were seen frequently in the apex or at the base of left ventricle. In conclusion, resting thallium scan was most reliable imaging technique to distinguish ischemic DCM from idiopathic DCM. (author)

  10. 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.

  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. A Rare Occurance with Epidermolysis Bullosa Disease: Dilated Cardiomyopathy

    OpenAIRE

    Derya Cimen

    2014-01-01

    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 diagn...

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    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)

  17. 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...

  18. 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)

  19. 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.

  20. Simultaneous onset of idiopathic dilated cardiomyopathy in identical middle-aged twins

    OpenAIRE

    Sutton, A.; Somasundram, U; Hall, J

    1999-01-01

    Idiopathic dilated cardiomyopathy is a primary myocardial disease which is characterised by left ventricular, or biventricular, dilatation and impaired contractility. The precise aetiology is unknown and the relative contribution of genetic and environmental factors is debated. We report two identical male twins of Caucasian origin with idiopathic dilated cardiomyopathy who presented within a few months of each other.


Keywords: idiopathic dilated cardiomyopathy; twins

  1. 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 these......, leading to 99.1% coverage of the target region with at least 50-fold and a mean read depth of 2415. In this well characterized cohort, we find the highest number of known cardiomyopathy mutations in plakophilin-2, myosin-binding protein C-3, and desmoplakin. When we include yet unknown but predicted...... disease variants, we find titin, plakophilin-2, myosin-binding protein-C 3, desmoplakin, ryanodine receptor 2, desmocollin-2, desmoglein-2, and SCN5A variants among the most commonly mutated genes. The overlap between DCM, hypertrophic cardiomyopathy (HCM), and channelopathy causing mutations is...

  2. Mutations of Presenilin Genes in Dilated Cardiomyopathy and Heart Failure

    OpenAIRE

    Li, Duanxiang ; Parks, Sharie B. ; Kushner, Jessica D. ; Nauman, Deirdre ; Burgess, Donna ; Ludwigsen, Susan ; Partain, Julie ; Nixon, Randal R. ; Allen, Charles N. ; Irwin, Robert P. ; Jakobs, Petra M. ; Litt, Michael ; Hershberger, Ray E. 

    2006-01-01

    Two common disorders of the elderly are heart failure and Alzheimer disease (AD). Heart failure usually results from dilated cardiomyopathy (DCM). DCM of unknown cause in families has recently been shown to result from genetic disease, highlighting newly discovered disease mechanisms. AD is the most frequent neurodegenerative disease of older Americans. Familial AD is caused most commonly by presenilin 1 (PSEN1) or presenilin 2 (PSEN2) mutations, a discovery that has greatly advanced the fiel...

  3. 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...

  4. Idiopathic dilated cardiomyopathy: possible triggers and treatment strategies

    OpenAIRE

    Hazebroek, M.; Dennert, R; Heymans, S.

    2012-01-01

    Despite recent advances in the management of patients with heart failure, morbidity and mortality rates remain high. Common causes of heart failure are ischaemic heart disease, uncontrolled hypertension and valvular disease. However, in up to 50 % of the cases its exact cause remains initially unknown; this condition is called idiopathic dilated cardiomyopathy (DCM). Improved diagnostic methods, most notably the advancements in molecular and immunohistological biopsy techniques and genetic re...

  5. Superior vena thrombosis with peripartum dilated cardiomyopathy

    International Nuclear Information System (INIS)

    A 30 years multiparous female with history of emergency caesarean section 10 days back was referred to us with cough, severe breathlessness at rest, orthopnea with pain in neck and arms. Clinical examination revealed signs of heart failure. Echocardiography showed ejection fraction of 15%, with no right ventricular strain. A diagnosis of peripartum cardiomyopathy was made. Doppler ultrasound of neck veins showed bilateral internal jugular vein thrombosis. Subsequent multislice CT examination showed thrombosis of superior vena cava and both internal jugular veins (with collateral formation) and pulmonary embolism. There were no mediastinal abnormalities on the CT scan. Her thrombophilia screen and CT scan brain was normal. She was managed in collaboration with cardiologist. Following treatment with subcutaneous enoxaparin therapy and warfarin her symptoms of upper limb pain improved. She responded very well to medical therapy for heart failure with marked improvement of NYHA functional class. (author)

  6. Clinical significance of normal cardiac silhouette in dilated cardiomyopathy

    International Nuclear Information System (INIS)

    It is generally believed that patients with dilated cardiomyopathy have a large cardiac silhouette on chest roentgenography. Contrary to this general belief, we have recently examined several patients with a dilated left ventricle (LV) on echocardiography but in whom the cardio-thoracic ratio (CTR) was within normal limits. To investigate this apparent discrepancy, we evaluated the relationship between LV dimensions, measured on M-mode echocardiography, and CTR in 49 patients with dilated cardiomyopathy. Among these patients, 11 (22%) had a CTR less than 50% and 38 (78%) had a CTR greater than 50%. The spacial orientation (cardiac rotation) of the LV within the thorax was evaluated by magnetic resonance imaging (MRI) in 5 patients with a CTR less than 50% and in 7 patients with a CTR greater than 50%, in comparison with 7 normal controls. In each of these patients, cardiac rotation was assessed from both a transverse and a frontal MRI section. In both groups, LV end-diastolic dimension was greater than 5 cm. Transverse cardiac rotation was 32±8 degrees in patients with a CTR less than 50%. This was significantly lower than in the 7 normal controls (43±7 degrees) (p<0.05). In patients with a CTR greater than 50%, however, transverse cardiac rotation (55±5 degrees) was significantly greater than in normal controls (p<0.01). No differences in frontal cardiac rotation were observed between the 2 groups. These data indicate that a normal cardiac silhouette in patients with dilated cardiomyopathy can be explained on the basis of a counterclockwise transverse rotation of the heart within the thorax, and it cannot always rule out the dilatation of the LV. (author)

  7. Differentiating cardiomyopathy of coronary artery disease from nonischemic dilated cardiomyopathy utilizing positron emission tomography

    International Nuclear Information System (INIS)

    To determine if imaging of blood flow (using N-13 ammonia) and glucose metabolism (using F-18 2-deoxyglucose) with positron emission tomography can distinguish cardiomyopathy of coronary artery disease from nonischemic dilated cardiomyopathy, 21 patients with severe left ventricular dysfunction who were evaluated for cardiac transplantation were studied. The origin of left ventricular dysfunction had been previously determined by coronary angiography to be ischemic (11 patients) or nonischemic (10 patients). Images were visually analyzed by three observers on a graded scale in seven left ventricular segments and revealed fewer defects in dilated cardiomyopathy compared with ischemic cardiomyopathy for N-13 ammonia (2.7 +/- 1.6 versus 5 +/- 0.6; p less than 0.03) and F-18 deoxyglucose (2.8 +/- 2.1 versus 4.6 +/- 1.1; p less than 0.03). An index incorporating extent and severity of defects revealed more homogeneity with fewer and less severe defects in subjects with nonischemic than in those with ischemic cardiomyopathy as assessed by imaging of flow (2.8 +/- 1.8 versus 9.2 +/- 3; p less than 0.001) and metabolism (3.8 +/- 3.3 versus 8.5 +/- 3.6; p less than 0.005). Diagnostic accuracy for distinguishing the two subgroups by visual image analysis was 85%. Using previously published circumferential count profile criteria, patients with dilated cardiomyopathy had fewer ischemic segments (0.4 +/- 0.8 versus 2.5 +/- 2 per patient; p less than 0.01) and infarcted segments (0.1 +/- 0.3 versus 2.4 +/- 1.4 per patient; p less than 0.001) than did patients with cardiomyopathy of coronary artery disease. The sensitivity for differentiating the two clinical subgroups using circumferential profile analysis was 100% and the specificity 80%

  8. 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 ...

  9. 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.

  10. Serological and molecular evidence of enterovirus infection in patients with end-stage dilated cardiomyopathy.

    OpenAIRE

    Muir, P; Nicholson, F; Illavia, S. J.; McNeil, T. S.; Ajetunmobi, J. F.; Dunn, H; Starkey, W. G.; Reetoo, K. N.; Cary, N R; Parameshwar, J; Banatvala, J E

    1996-01-01

    OBJECTIVE: To study the relative diagnostic value of enterovirus-specific molecular biological and serological assays in patients with end-stage dilated cardiomyopathy, and to investigate the possible role of other cardiotropic viruses in dilated cardiomyopathy. DESIGN: Analysis of recipient myocardial tissue and serum from patients with dilated cardiomyopathy and controls undergoing cardiac transplantation for end-stage cardiac disease. SETTING: University virology department and transplanta...

  11. 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

  12. 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.

  13. 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 ...

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

    Science.gov (United States)

    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.

  15. Frequency and echocardiographic study of dilated cardiomyopathy in children presenting with cardiac failure

    International Nuclear Information System (INIS)

    Objective: To evaluate the role of echocardiography in diagnosis of dilated cardiomyopathy as a cause of cardiac failure in children. Design: This was descriptive study. Children presenting with cardiac failure from indoor patients were selected and echocardiography along with chest X- ray, ECG, cardiac enzymes and ASO titre was performed in all patients. Subject: Fifty hospitalized patients with congestive heart failure were selected consecutively from hospitalized patients. Main Outcome: Role of echocardiography in the diagnosis of dilated cardiomyopathy in children presenting with cardiac failure. Results: Out of fifty patients admitted with cardiac failure 27 (54%) cases were found to be dilated cardiomyopathy while congenital heart disease, myocarditis and rheumatic heart disease were found in 12 (24%), 8 (16%) and 3 (6%) cases respectively. Conclusion: Dilated cardiomyopathy is an important cause of cardiac failure in children and echocardiography is an important tool to diagnose and differentiate dilated cardiomyopathy from other causes of cardiac failure. (author)

  16. 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.

  17. Echocardiographic characterization of dilatation cardiomyopathy in the English Cocker Spaniel

    International Nuclear Information System (INIS)

    The echocardiographic characterization of a dilatation cardiomyopathy in small-breed dogs is reported. Twelve clinically healthy adult English Cocker Spaniel dogs (between 2 and 9 years old and weighing 11.5 to 15.4 kg [mean 12.9 +/- 1.00 kg]) from a kennel population with a history of cardiomyopathy were assessed, using M-mode echocardiography. The dogs were selected on ECG and/or radiographic evidence of ventricular enlargement. Nine dogs had R-wave amplitude in lead 11 of greater than 3.0 mV. Two dogs had an unusual right-axis deviation, the result of deep Q waves in the limb leads and deep S waves in chest leads CV6LL and CV6LU, indicating that there was right ventricular enlargement. All dogs had increased end-systolic dimensions (mean 3.0 +/- 0.6 cm). End-diastolic dimensions were increased in 9 dogs (mean 4.0 +/- 0.5 cm), and there was a decrease of left ventricular (LV) function as measured by fractional shortening in 8 dogs. Mean fractional shortening for the 12 dogs was 25.4 +/- 5.7%. There was significant correlation between LV dimensions and age at echocardiographic assessment, indicating that LV dilatation was progressive. Three of the oldest dogs had severe dilatation of the LV, and in 2 of these, LV function was severely decreased. Left ventricular function in the 3rd dog, however, was within the acceptable range. Fractional shortening and thickness of the LV caudal wall and interventricular septum were significantly correlated (P less than 0.01 for interventricular system and P less than 0.05 for LV caudal wall)

  18. 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

  19. [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

  20. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  2. A young man with acute dilated cardiomyopathy associated with methylphenidate

    Directory of Open Access Journals (Sweden)

    Tor-Bjarne Nymark

    2008-05-01

    Full Text Available Tor-Bjarne Nymark1, A Hovland2, H Bjørnstad2, E W Nielsen1,31Section for Emergency Medicine; 2Department of Cardiology, Nordland Hospital, Bodø, Norway; 3University of Tromsø, Tromsø, NorwayAbstract: An 18-year-old obese man with a body mass index of 40, diagnosed with attention-deficit hyperactivity disorder and treated with methylphenidate (Concerta® was acutely admitted to hospital with hypoxia and dyspnoea. On investigation signs of liver-, renal-, and heart-failure were found. Noradrenalin infusion was started. Echocardiography showed dilated left ventricle and an ejection fraction (EF of 25%. Liver function improved, noradrenalin and dobutamine were tapered, but three days after admission a new echocardiography showed an EF of 10%. The patient was transferred to the National Hospital (Rikshospitalet, Oslo, where intensified treatment including intra aortic balloon pump (IABP was instituted. Cardiac function improved, and 3 weeks later the IABP was disconnected. EF at this point was 15%. The patient was denied heart transplantation due to various cofactors. The investigation concluded with a probable relationship between his cardiomyopathy and the use of methylphenidate (Concerta.Keywords: cardiomyopathy, heart failure, ADHD, methylphenidate

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Echocaridography, electrocardiography, and radiography of cats with dilatation cardiomyopathy, hypertrophic cardiomyopathy, and hyperyroidism

    International Nuclear Information System (INIS)

    The echocardiographic, ECG, and radiographic findings of sequentially examined cats with dilatation cardiomyopathy (DCM, n = 7), hypertrophic cardiomyopathy (HCM, n = 8), and hyperthyroidism (HT, n = 20) were compared with those of healthy control cats (n = 11). Cats with DCM were easily differentiated from healthy cats by echocardiography and from cats with HCM and HT by a dilated left ventricle at end-diastole with a mean +/- SD of 2.20 +/- 0.36 cm, reduced fractional shortening (2.9% +/- 3.7%), reduced aortic amplitude (0.07 +/- 0.05 cm), reduced left ventricular wall amplitude (0.09 +/- 0.09 cm), and increased E-point septal separation (0.83 +/- 0.29 cm). The cats with HCM were most consistently recognized echocardiographically by increased left ventricular wall thickness at end-diastole (0.75 +/- 0.12 cm). Some cats with HT had abnormal echocardiograms with left ventricular wall hypertrophy. These cats could usually be differentiated from the cats with HCM because of normal or increased ventricular wall amplitude, aortic amplitude, or percentage of thickening of the left ventricular wall and interventricular septum. Left atrial enlargement (left atrial diameter greater than 1.57 cm or left atrium/aorta greater than 1.75) was commonly detected by the echocardiogram in cats with DCM, HCM, or HT. The echocardiogram was helpful in differentiating the type of cardiomyopathy (DCM, HCM, or HT) when plain thoracic radiographs indicated that cardiomegaly existed. The ECG may have indicated incorrectly that there was left ventricular enlargement in some cats with HT, and it did not indicate consistently that left ventricular enlargement existed when present in cats with DCM or HCM. The ECG was a poor indicator of left atrial enlargement in all cats

  6. Takayasu arteritis with dilated cardiomyopathy and aortic dissection: a rare presentation

    International Nuclear Information System (INIS)

    We report a peculiar case of TA in a young male Pakistani patient presenting with dilated cardiomyopathy (DCM) and aortic dissection. To our knowledge this is the first reported case of TA with DCM and aortic dissection simultaneously. (author)

  7. 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

    We investigated a Danish cohort of 31 unrelated patients with idiopathic dilated cardiomyopathy (IDC), to assess the role that mutations in sarcomere protein genes play in IDC. Patients were genetically screened by capillary electrophoresis single strand conformation polymorphism and subsequently...

  8. Dilated cardiomyopathy of unknown cause in young patients: risk evaluation, possible etiologies, and treatment.

    Directory of Open Access Journals (Sweden)

    Göran Wettrell

    2007-01-01

    Full Text Available Childhood dilated cardiomyopathy (DCM is characterized by a dilated left ventricle and systolic dysfunction, and in some patients also by right ventricle failure. It is a serious myocardial disease, usually idiopathic, but its infectious, metabolic and genetic aetiologies are increasingly revealed. DCM frequently results in heart failure and sometimes death. It is the most common form of cardiomyopathy and the most common reason for heart transplantation in children.

  9. High prevalence of myocardial monoclonal antimyosin antibody uptake in patients with chronic idiopathic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Monoclonal antimyosin antibody studies were undertaken to assess the presence of myocardial uptake in patients with chronic idiopathic dilated cardiomyopathy. Three groups were studied: 17 patients with chronic (greater than 12 months) idiopathic dilated cardiomyopathy, 12 patients with a large, poorly contracting left ventricle not due to dilated cardiomyopathy (control patients) and 8 normal individuals. The patients in the cardiomyopathy and control groups showed a similar degree of clinical and functional impairment. Imaging was undertaken 48 h after antimyosin injection. The heart/lung ratio of antimyosin uptake was used to assess the results. The mean ratio in the cardiomyopathy group was 1.83 +/- 0.36 (range 1.40 to 2.80), a value significantly higher than that obtained in the control patients without cardiomyopathy (mean 1.46 +/- 0.04, range 1.38 to 1.50) or normal subjects (mean 1.46 +/- 0.13, range 1.31 to 1.6) (p less than 0.01). No difference in the ratio was noted between the normal subjects and control patients. Abnormal antimyosin uptake was seen in 12 (70%) of the 17 patients with cardiomyopathy and in only 1 (8%) of the 12 control patients. Positive monoclonal antimyosin antibody studies are highly prevalent in chronic idiopathic dilated cardiomyopathy

  10. Right ventricular function in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    The characteristics and pathogenesis of right ventricular dysfunction in 14 patients with dilated cardiomyopathy (DCM) were investigated by equilibrium right ventricular blood pool scintigraphy using ultrashort-lifetime 81mKr. Thirteen patients with severe left ventricular dysfunction due to old anterior myocardial infarction (OMI) and nine normal subjects were used as controls. The right ventricular end-diastolic pressure and volume index, mean pulmonary arterial pressure, and total pulmonary vascular resistance index were almost the same in the DCM and OMI patients. The right ventricular ejection fraction was 44.2±6.0% (mean±SD) in DCM patients and 47.1±7.9% in OMI patients, both significantly lower than those in the normal subjects (54.5±5.3%), but with no difference between the two case groups. The right ventricular peak filling rate was significantly reduced in both case groups as compared with the normal subjects (2.46±0.81 EDV/sec). The reduction was significantly greater (p81mKr blood pool scintigraphy is useful in the study of the right ventricular systolic and diastolic function. The diastolic parameters are more sensitive indicators for evaluation of right ventricular function in DCM than the systolic parameters. (author)

  11. 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

  12. [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

  13. Left ventricular diastolic properties in dilated cardiomyopathy, transmural myocardial infarction, and hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Left ventricular (LV) diastolic properties in dilated cardiomyopathy (DCM), transmural myocardial infarction (TMI), and hypertrophic cardiomyopathy (HCM) were evaluated. Radionuclide angiography and M-mode echocardiography were performed for 11 cases of DCM, 40 cases of TMI, 21 cases of HCM, and nine normal control subjects. In DCM, the peak filling rate (PFR) and filling fraction (FF) were significantly reduced, but the time to the peak filling rate (TPFR) was not prolonged. In TMI, both the PFR and FF were significantly reduced. Moreover, the TPFR was significantly prolonged in TMI as compared to DCM. Although depression of the PFR in HCM was not apparent, prolongation of the TPFR in HCM was marked. In DCM, there was good correlation between the PFR and left ventricular ejection fraction (EF) (r = 0.71, p < 0.03). In TMI, there was a good correlation between the TPFR and the standard deviation of the LV phase angle histogram (SDP), indicating LV asynergy (r = 0.589, p < 0.005). In HCM, both the FF and PFR correlated inversely with the LV wall thickness (r = -0.74, p < 0.008; r = -0.581, p < 0.03, respectively). These results indicate that various factors affect LV diastolic properties in heart disease, and that radionuclide angiography is a valuable technique for evaluating LV diastolic function. (author)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  17. 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)

  18. 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

  19. Dipyridamole 201Tl myocardial SPECT imaging in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Objective: To explore the characteristics of dipyridamole 201Tl myocardial perfusion imaging (MPI) SPECT in patients with dilated cardiomyopathy. Methods: Thirty patients with dilated cardiomyopathy underwent pharmacological stress 201Tl MPI SPECT after intravenous infusion of dipyridamole (0.56 mg/kg) for 4 min. The early and delayed SPECT images were acquired respectively at 10 and 240 min after 201Tl injection. The images were analyzed and reported by two or three experienced nuclear medicine physicians. Results: All patients were found to have abnormal perfusion patterns at delay imaging, however 90.00% (27/30) were also abnormal at early images. Six patients had reverse redistribution. Conclusion: Dipyridamole 201Tl MPI SPECT imaging may be of some value for the assessment of patients with dilated cardiomyopathy. (authors)

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Chronic oral exposure to the aldehyde pollutant acrolein induces dilated cardiomyopathy

    OpenAIRE

    Ismahil, Mohamed Ameen; Hamid, Tariq; Haberzettl, Petra; Gu, Yan; Chandrasekar, Bysani; Srivastava, Sanjay; Bhatnagar, Aruni; Prabhu, Sumanth D.

    2011-01-01

    Environmental triggers of dilated cardiomyopathy are poorly understood. Acute exposure to acrolein, a ubiquitous aldehyde pollutant, impairs cardiac function and cardioprotective responses in mice. Here, we tested the hypothesis that chronic oral exposure to acrolein induces inflammation and cardiomyopathy. C57BL/6 mice were gavage-fed acrolein (1 mg/kg) or water (vehicle) daily for 48 days. The dose was chosen based on estimates of human daily unsaturated aldehyde consumption. Compared with ...

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

    Directory of Open Access Journals (Sweden)

    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.

  3. 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

  4. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  6. Difference in myocardial flow reserve between patients with dilated cardiomyopathy and those with dilated phase of hypertrophic cardiomyopathy. Evaluation by 15O-water PET

    International Nuclear Information System (INIS)

    The clinical features of patients with the dilated phase of hypertrophic cardiomyopathy (DHCM) may resemble those of patients with dilated cardiomyopathy (DCM); that is, systolic dysfunction and left ventricular dilatation. Myocardial flow reserve (MFR) is impaired in patients with nonischemic cardiomyopathy, and the reduced MFR may be related to poor prognosis. Several studies report that the mortality rate for patients with DHCM is higher than for DCM, but the difference between these 2 cardiomyopathies is still unclear. The purpose of this study was to assess the MFR of these 2 cardiomyopathies, using 15O-water positron emission tomography (PET) to elucidate their differences. In total 30 patients were investigated: 23 with DCM (Group A) and 7 with DHCM (Group B). All those who were in a stable condition underwent cardiac catheterization. Myocardial blood flow (MBF) at rest and under adenosine 5'-triphosphate (ATP) infusion was measured by 15O-water PET, and the MFR was calculated. There were no significant differences in the hemodynamics of the 2 groups. The mean MFR in DHCM was significantly lower than that in DCM (1.49±0.31 vs 2.62±1.08; p=0.042), whereas MBF at rest did not differ (DCM vs DHCM: 0.66±0.20 vs 0.49±0.05 ml·min-1·g-1; no significance (NS)). The MFR in both Group A and B was significantly decreased compared with the normal controls (MFR in normal controls: 5.15±1.64, p=0.00015, 0.00013, respectively). These results suggest that impaired vasodilatation (ie, dysfunction of the microcirculation) is more severe in patients with DHCM than in patients with DCM, even though patients' characteristics and hemodynamics do not differ. (author)

  7. New contribution to the study of ventricular remodeling and valve rings in dilated cardiomyopathy: anatomical and histological evaluation

    OpenAIRE

    Dalva, Moise; Correia, Aristides Tadeu; Jatene, Natalia de Freitas; Saldiva, Paulo Hilário Nascimento; Jatene, Fabio Biscegli

    2014-01-01

    Introduction Idiopathic dilated cardiomyopathy causes great impact but many aspects of its pathophysiology remain unknown. Objective To evaluate anatomical and histological aspects of hearts with idiopathic dilated cardiomyopathy and compare them to a control group, evaluating the behavior of the perimeters of the atrioventricular rings and ventricles and to compare the percentage of collagen and elastic fibers of the atrioventricular rings. Methods Thirteen hearts with cardiomyopathy and 13 ...

  8. Effects of Omega-3 Polyunsaturated Fatty Acids on Heart Function and Oxidative Stress Biomarkers in Pediatric Patients with Dilated Cardiomyopathy

    OpenAIRE

    Omidreza Firuzi; Nader Shakibazad; Hamid Amoozgar; MohammadBorzoee; Saeed Abtahi; Gholamhossein Ajami; Pegah Ardi; Ramin Miri

    2013-01-01

    Objectives: Dilated cardiomyopathy is the most prevalent type of cardiomyopathy in children, which results in congestive heart failure and causes significant morbidity and mortality. This study, aims to investigate the effect of supplementation with omega-3 polyunsaturated fatty acids (n-3 PUFA) on heart function and oxidative stress biomarkers in these patients. Methods: The present research was a case-control study on pediatric patients with dilated cardiomyopathy, who received n-3 PUFA...

  9. 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

  10. Initial presentation with dilated cardiomyopathy in a patient of tuberous sclerosis: A rare case report

    OpenAIRE

    Jain, Dharmendra; Kumar, Vikas; Kar, Deba P.; Prasad, Shashi R.

    2013-01-01

    A 35-year-old man presented with dilated cardiomyopathy, an unusual association with tuberous sclerosis. Clinical history and examination were consistent with tuberous sclerosis including major features of tuberous sclerosis complex (TSC) like facial angiofibroma, shagreen patch, subependymal nodules, and angiomyolipoma of kidney. The clinical manifestations, pathogenesis and evaluation of tuberous sclerosis are discussed.

  11. Dynamic functional modules in co-expressed protein interaction networks of dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Oyang Yen-Jen

    2010-10-01

    Full Text Available Abstract Background Molecular networks represent the backbone of molecular activity within cells and provide opportunities for understanding the mechanism of diseases. While protein-protein interaction data constitute static network maps, integration of condition-specific co-expression information provides clues to the dynamic features of these networks. Dilated cardiomyopathy is a leading cause of heart failure. Although previous studies have identified putative biomarkers or therapeutic targets for heart failure, the underlying molecular mechanism of dilated cardiomyopathy remains unclear. Results We developed a network-based comparative analysis approach that integrates protein-protein interactions with gene expression profiles and biological function annotations to reveal dynamic functional modules under different biological states. We found that hub proteins in condition-specific co-expressed protein interaction networks tended to be differentially expressed between biological states. Applying this method to a cohort of heart failure patients, we identified two functional modules that significantly emerged from the interaction networks. The dynamics of these modules between normal and disease states further suggest a potential molecular model of dilated cardiomyopathy. Conclusions We propose a novel framework to analyze the interaction networks in different biological states. It successfully reveals network modules closely related to heart failure; more importantly, these network dynamics provide new insights into the cause of dilated cardiomyopathy. The revealed molecular modules might be used as potential drug targets and provide new directions for heart failure therapy.

  12. Evaluation of myocardial disorders in patients with dilated cardiomyopathy and left ventricular eccentric hypertrophy

    International Nuclear Information System (INIS)

    201Tl myocardial SPECT was performed in cases of dilated cardiomyopathy and valvular heart disease with left ventricular eccentric hypertrophy, and the two groups were compared from the standpoint of the mechanism of onset of myocardial disorders. Significant coefficients of correlation were seen between the Tl score and LVDd (r=0.792, r=0.785) and Tl score and LVEF (r=-0.634, r=-0.555) in both dilated cardiomyopathy and valvular heart disease. In cases of valvular heart disease, significant correlation coefficients (r=-0.756, r=-0.720) between LVDd and r-WR (relative-washout rate), and Tl score and r-WR were observed, but no such correlation was seen in dilated cardiomyopathy. In valvular heart disease, a decrease in myocardial perfusion associated with enlargement of the left ventricle appeared, while in dilated cardiomyopathy, there was a marked decrease in LVEF in proportion to the thallium defect. Therefore, it was assumed that left ventricular wall disorders occur due to myocardial metabolic disorders and coronary microcirculation disorders. (author)

  13. 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

  14. 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. 

  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. 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.

  17. Usefulness of 123I-BMIPP scanning for distinction of ischemic from nonischemic dilated cardiomyopathy

    International Nuclear Information System (INIS)

    To determine if imaging of blood flow (using 201Tl) and fatty acid (using 123I-BMIPP) with SPECT can distinguish cardiomyopathy of coronary artery disease from nonischemic dilated cardiomyopathy, 24 patients with severe left ventricular dysfunction were evaluated. The origin of left ventricular dysfunction had been previously determined by coronary angiography to be ischemic (9 patients) or nonischemic (15 patients). Images were visually analyzed by three observers on a graded scale (score: 0, normal; 1, mild uptake reduction; 2, severe uptake reduction; 3, defect) in 20 left ventricular segments, which revealed higher defect score in ischemic cardiomyopathy (ICM) compared with nonischemic dilated cardiomyopathy (NCM) for 123I-BMIPP (35.5±14.4 versus 14.1±9.3, p201Tl (27.6±14.6 versus 12.1±7.4, p123I-BMIPP (2.25±0.52 versus 1.36±0.36, p201Tl (1.92±0.51 versus 1.24±0.42, p123I-BMIPP is helpful in distinguishing patients with severe left ventricular dysfunction secondary to coronary artery disease from those with nonischemic cardiomyopathy. (author)

  18. 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.

  19. 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...

  20. Long term cardioprotective action of trimetazidine and potential effect on the inflammatory process in patients with ischaemic dilated cardiomyopathy

    OpenAIRE

    Di Napoli, P; Taccardi, A A; Barsotti, A

    2005-01-01

    Objective: To investigate the long term effects of trimetazidine in patients with dilated ischaemic cardiomyopathy. The effects of trimetazidine on left ventricular function as well as its tolerability profile and potential anti-inflammatory effects were studied.

  1. 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 block...

  2. 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.

  3. Trimetazidine, a metabolic modulator, has cardiac and extracardiac benefits in idiopathic dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Tuunanen, H.; Engblom, E.; Naum, A.; Nagren, K.; Scheinin, M.; Hesse, B.; Airaksinen, K.E. Juhani; Nuutila, P.; Iozzo, P.; Ukkonen, H.; Opie, L.H.; Knuuti, J.

    2008-01-01

    BACKGROUND: The anti-ischemic agent trimetazidine improves ejection fraction in heart failure that is hypothetically linked to inhibitory effects on cardiac free fatty acid (FFA) oxidation. However, FFA oxidation remains unmeasured in humans. We investigated the effects of trimetazidine on cardiac...... perfusion, efficiency of work, and FFA oxidation in idiopathic dilated cardiomyopathy. METHODS AND RESULTS: Nineteen nondiabetic patients with idiopathic dilated cardiomyopathy on standard medication were randomized to single-blind trimetazidine (n=12) or placebo (n=7) for 3 months. Myocardial perfusion......, FFA, and total oxidative metabolism were measured using positron emission tomography with [(15)O]H(2)O, [(11)C]acetate, and [(11)C]palmitate. Cardiac function was assessed echocardiographically; insulin sensitivity was assessed by the homeostasis model assessment index. Trimetazidine increased...

  4. 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

  5. Comparison of Immune Profiles in Fetal Hearts with Idiopathic Dilated Cardiomyopathy, Maternal Autoimmune-Associated Dilated Cardiomyopathy and the Normal Fetus.

    Science.gov (United States)

    Nield, Lynne E; von Both, Ingo; Popel, Najla; Strachan, Kate; Manlhiot, Cedric; Shannon, Patrick; McCrindle, Brian W; Atkinson, Adelle; Miner, Steven E S; Jaeggi, Edgar T; Taylor, Glenn P

    2016-02-01

    The etiology of idiopathic dilated cardiomyopathy (iDCM) remains unknown. Immune therapies have improved outcome in fetuses with DCM born to mothers with autoimmune disease (aDCM). The purpose of this retrospective study was to compare the myocardial B and T cell profiles in fetuses and neonates with idiopathic DCM (iDCM) versus autoimmune-mediated DCM (aDCM) and to describe the normal cell maturation within the human fetal myocardium. Of 60 fetal autopsy cases identified from institutional databases, 10 had aDCM (18-38 weeks), 12 iDCM (19-37 weeks) and 38 had normal hearts (11-40 weeks). Paraffin-embedded myocardium sections were stained for all lymphocyte (CD45), B cells (CD20, CD79a), T cells (CD3, CD4, CD7, CD8) and monocyte (CD68) surface markers. Two independent, blinded cell counts were performed. Normal hearts expressed all B and T cell markers in a bimodal fashion, with peaks at 22 and 37 weeks of gestation. The aDCM cohort was most distinct from normal hearts, with less overall T cell markers [EST -9.1 (2.6) cells/mm(2), p = 0.001], CD4 [EST -2.0 (0.6), p = 0.001], CD3 [EST -3.9 (1.0), p development of the heart and evolution of dilated cardiomyopathy. PMID:26481221

  6. 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.

  7. 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...

  8. 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...

  9. 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.

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

    OpenAIRE

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

    2015-01-01

    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 Re...

  11. 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...

  12. 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...

  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. Characterization of cardiac function and metabolism in human dilated cardiomyopathy with MR imaging and spectroscopy

    International Nuclear Information System (INIS)

    This paper reports thirteen patients with dilated cardiomyopathy and eight normal volunteers studied with P-31 MR spectroscopy and cine H-1 MR imaging at 1.5 T. A double-tuned (H-1-P-31) surface coil was used to both transmit and receive radio-frequency signals. Prominent peaks in the phosphodiester (PDE) and phosphomonoester regions were observed in cardiomyopathic patients. The ratio of adenosine triphosphate to phosphocreatine (PCr/β-ATP) was not significantly lower (1.51 ± 0.09 vs 1.54 ± 0.04), but PDE/PCr (0.80 ± 0.07 vs 0.54 ± 0.10)(P ≤ .01) and PDE/β -ATP (1.19 ± 0.10 vs 0.84 ± 0.08)(P ≤ .05) were significantly higher in patients with dilated cardiomyopathy (ejection fraction [EF], 24% ± 3%) compared with normal volunteers (EF, 62% ± 3%). Thus, localized, gated P-31 MR spectroscopy combined with cine H-1 MR imaging identifies abnormal myocardial phosphate metabolism associated with abnormal ventricular function, which might be useful in characterizing patients with dilated cardiomyopathy

  15. 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 ...

  16. Left ventricular end-systolic wall stress is a potent prognostic variable in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Dilated cardiomyopathy is an important cause of morbidity and mortality among patients with heart failure. Left ventricular dilation is viewed as a compensatory response to maintain stroke volume, and left ventricular dilation is directly related to the increase of wall stress. However, only a few studies have examined whether wall stress can be a prognostic variable in patients with dilated cardiomyopathy. This study was designed to elucidate whether left ventricular systolic wall stress was related to the prognosis in patients with dilated cardiomyopathy. Twenty-five normal control subjects and 68 patients with dilated cardiomyopathy participated in this study. Hemodynamic parameters and left ventricular systolic wall stress were determined using echocardiography. In addition, the extent score determined by thallium-201 myocardial scintigraphy was measured as an index of cumulative loss of myocardium. During the 53-month follow-up period, 13 patients died of cardiac events. In a stepwise multivariable analysis, end-systolic wall stress and fractional shortening were significant predictors of survival. The extent score was markedly greater in the patients who died than in alive patients. There was a significant correlation between end-systolic wall stress and extent score (r=0.501, p=0.0001). Left ventricular end-systolic wall stress is an important predictor of mortality in patients with dilated cardiomyopathy. (author)

  17. 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.

  18. 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

  19. 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.

  20. Three-dimensional MR microscopy of a transgenic mouse model of dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Background. Scientists are now able to alter the genetics of vertebrate embryos routinely to produce animal models of human developmental diseases. However, our understanding of structural changes in these animal models is limited by current methodologies. Histological techniques, although providing great anatomic detail, display only ''static'' data (one time point only) in two dimensions. Ultrasound may be used to generate continuous time course data, but is limited by interobserver variation, limited acoustic windows, and relatively low resolution. Objective. To apply the high resolution, non-destructive, and three-dimensional acquisition capabilities of magnetic resonance (MR) microscopy to compare the hearts of normal mice versus an established transgenic mouse model of dilated cardiomyopathy. Materials and methods. Transgenic mice exhibiting dilated cardiomyopathy were developed via the introduction of a mutated, heart-specific gene (myosin light chain). Post-mortem cardiac imaging was performed on the transgenic mice and normal controls. MR imaging was performed on a Bruker 3T imaging magnet using a custom radiofrequency coil following contrast perfusion of the atrial and ventricular chambers. Image resolution was 156 μm isotropic voxels. MR images were compared to gross pathologic specimens. Imaging data were post-processed using custom software to calculate the volumes of the atria and ventricles and to display the three-dimensional morphology of the chambers and myocardium. Results. Of the seven mice scanned, four exhibited normal right atrial (average = 14.8 μl ± 1.4), left atrial (average = 8.5 μl ± 0.3), right ventricular (average = 12.9 μl ± 2.7), and left ventricular (average 3.3 μl ± 0.5) volumes. Three mice exhibited dilatation of the right and left cardiac chambers (RA average = 23.9 μl ± 5.6; LA average = 15.9 μl ± 4.8; RV average = 32.5 μl ± 6.8; LV average 24.0 μl ± 1.4). The gross morphology was verified upon autopsy of the

  1. Intravenous Cardiac Stem Cell-Derived Exosomes Ameliorate Cardiac Dysfunction in Doxorubicin Induced Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Adam C. Vandergriff

    2015-01-01

    Full Text Available Despite the efficacy of cardiac stem cells (CSCs for treatment of cardiomyopathies, there are many limitations to stem cell therapies. CSC-derived exosomes (CSC-XOs have been shown to be responsible for a large portion of the regenerative effects of CSCs. Using a mouse model of doxorubicin induced dilated cardiomyopathy, we study the effects of systemic delivery of human CSC-XOs in mice. Mice receiving CSC-XOs showed improved heart function via echocardiography, as well as decreased apoptosis and fibrosis. In spite of using immunocompetent mice and human CSC-XOs, mice showed no adverse immune reaction. The use of CSC-XOs holds promise for overcoming the limitations of stem cells and improving cardiac therapies.

  2. Clinical assessment of serum myosin light chain I in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Serum cardiac myosin light chain I (LCI) levels were quantitated using a radioimmunoassay kit in patients suspected of dilated cardiomyopathy (DCM). In this study, 55 patients were evaluated between 1986 and 1991. They were composed of 40 males and 15 females, and their age was 27-75 years (51±11 years). The patients with renal dysfunction were excluded due to their serum creatinine levels (>2.0 mg/dl). After cardiac catheterization, endomyocardial biopsy and echocardiography, 44 patients were diagnosed as DCM, 2 as ischemic heart disease, 2 as chronic myocarditis, 1 as restrictive cardiomyopathy, 1 as dilated hypertrophic cardiomyopathy, 1 as cardiac amyloidosis, 2 as myopathy, 1 as polymyositis and 1 as hypothyroidism. Only two patients with DCM had elevated LCI. Besides, two patients with myopathy or hypothyroidism had elevated LCI. In the follow-up, one patient died suddenly 6 months later and another showed normal value of LCI four years later. LCI elevation in DCM was not related to either the severity of heart failure or cardiac function and it showed no finding of 201Tl myocardial defect or elevated CPK. The mechanism for elevated LCI in myopathy is related to a crossreaction with myosin light chain in the skeletal muscle. In hypothyroidism, it may be related to decreased clearance of normal LCI concentration or increased myosin light chain from damaged skeletal muscle. In conclusion, it is evident that the measurement of LCI is not helpful in clinical assessment of patients with DCM, but may be useful in detection of secondary cardiomyopathy. (author)

  3. Myocardial damage assessed by indium-111-antimyosin: correlation with persistent enteroviral ribonucleic acid in dilated cardiomyopathy

    International Nuclear Information System (INIS)

    The persistence of enteroviral ribonucleic acid (RNA) in the myocardium has been implicated as a pathogenetic factor in idiopathic dilated cardiomyopathy. Enteroviral persistence may lead to myocardial cell membrane damage, resulting in increased uptake of antimyosin antibodies. To further evaluate this hypothesis, a direct comparison of myocardial antimyosin uptake with the presence of enteroviral RNA was performed in ten patients (one female, nine male; 53±8 years) with chronic dilated cardiomyopathy. Planar antimyosin images were obtained 48 h after the injection of indium-111-labelled antimyosin Fab. Using a region of interest technique, the heart to lung uptake ratio (HLR) was calculated as a semiquantitative parameter of myocardial tracer uptake. Cardiac catheterization was performed to assess left ventricular function and to obtain myocardial biopsy samples. In the biopsy samples, gene amplification by polymerase chain reaction (PCR) was used to specifically detect enteroviral RNA. In the ten patients, the left ventricular ejection fraction was 39%±11% and the end-diastolic volume 131±46 ml/m2. The HLR was 1.72±0.21 and showed no correlation with functional parameters. In two patients with a positive PCR consistent with persisting enteroviral RNA, the HLR was not higher than that in eight patients with a negative PCR (1.46±0.18 vs 1.78±0.18, respectively). These results suggest that increased uptake of 111In-antimyosin in chronic idiopathic dilated cardiomyopathy cannot be explained by pure persistence of enteroviral RNA. Other pathogenetic factors such as myocardial autoantibodies or microvascular spasm may be responsible for myocyte membrane damage detected by antimyosin. (orig.). With 3 figs., 1 tab

  4. Nuclear magnetic resonance imaging in patients with hypertrophic and dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Patients with hypertrophic and dilated cardiomyopathy and normal subjects were investigated with nuclear magnetic resonance imaging. To evaluate the NMR scanner possibilities, the results were compared with the echocardiographic investigation of the same patients. The capabilities of NMR imaging to provide information about intracardiac anatomy are emphasized. This study is preceded by a description of the physical principles underlying the phenomenon of nuclear magnetic resonance and of the techniques used to obtain NMR images and a review of the clinical use of NMR imaging for cardiac diagnosis

  5. Endogenous plasma endothelin concentrations and coronary circulation in patients with mild dilated cardiomyopathy

    OpenAIRE

    Mundhenke, M; Schwartzkopff, B; Kostering, M; Deska, U; Klein, R.; Strauer, B.

    1999-01-01

    OBJECTIVE—To determine whether increased plasma concentrations of endothelin-1 (ET-1) and big endothelin (BET) play a role in the regulation of coronary circulation in patients with idiopathic dilated cardiomyopathy (IDCM).
SETTING—Tertiary referral centre for cardiac diseases. 
PATIENTS—Fourteen patients (eight male/six female; mean (SD) age 59 (9) years) with IDCM (ejection fraction 36 (9)%) and five normotensive subjects (two male/three female; age 52 (7) years) serving as controls were st...

  6. Transgenic expression of replication-restricted enteroviral genomes in heart muscle induces defective excitation-contraction coupling and dilated cardiomyopathy.

    OpenAIRE

    Wessely, R; Klingel, K; L. F. Santana; Dalton, N.; Hongo, M; Jonathan Lederer, W; Kandolf, R; Knowlton, K U

    1998-01-01

    Numerous studies have implicated Coxsackievirus in acute and chronic heart failure. Although enteroviral nucleic acids have been detected in selected patients with dilated cardiomyopathy, the significance of such persistent nucleic acids is unknown. To investigate the mechanisms by which restricted viral replication with low level expression of Coxsackieviral proteins may be able to induce cardiomyopathy, we generated transgenic mice which express a replication-restricted full-length Coxsacki...

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

    Science.gov (United States)

    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, the administration of esmolol stabilized the patient's heart rhythm. A moderate dose of the β-blocker esmolol, administered as an 0.5-mg intravenous bolus injection followed by an infusion at a rate of 0.15 mg/kg/min, inhibited the recurrence of ventricular fibrillation and normalized the electrocardiographic pattern. The results suggest that esmolol may be able to improve the survival rate of patients with electrical storm in dilated cardiomyopathy and should be considered as a primary therapy in the management of cardiac electrical storms. PMID:27347024

  8. 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.

  9. 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

  10. 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.

  11. 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.

  12. Characteristics of myocardial 18F-fluorodeoxyglucose positron emission computed tomography in dilated cardiomyopathy and ischemic cardiomyopathy

    International Nuclear Information System (INIS)

    Myocardial 18F-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) has been used to assess myocardial ischemia and viability, but few studies have conducted on FDG-PET for dilated cardiomyopathy (DCM). We investigated myocardial FDG uptake in patients with DCM in comparison with ischemic cardiomyopathy (ICM). Twenty-four patients with heart failure were included in this study. Fourteen of them were diagnosed as DCM and the other 10 were ICM. All of them underwent myocardial FDG-PET at fasting and after glucose loading the same day. FDG uptake was quantified by the ratio of the counts at the heart to those at the liver (H/L ratio). Left ventricular (LV) function was measured by echocardiography. We classified FDG distribution patterns in the myocardium in the fasting state into 3 types (faint uptake, regional uptake and diffuse uptake). In DCM patients, 5 had faint uptake, 7 had regional uptake, and the other 2 had diffuse uptake. On the other hand, all ICM patient had regional uptake (p<0.05). In DCM, there were no significant relationships between the patterns and LV functions. On the other hand, there were close correlation between the H/L ratio after glucose loading and the left ventricular ejection fraction (r=0.680, p<0.01). The changes in PET images caused by glucose loading were classified into 2 types (non-reversing and reversing patterns). DCM significantly showed a non-reversing pattern (86%, 12 of 14 patients) whereas ICM showed mainly a reversing pattern (70%, 7 of 10 patients; p<0.05). In conclusion, myocardial FDG uptake after glucose loading may indicate a myocardial viable mass although FDG uptake at fasting was not evidently related to LV function. The change in the pattern of the FDG image from fasting to glucose loading may be useful in differentiating DCM form ICM. (author)

  13. Evaluation of myocardial disorders in patients with dilated cardiomyopathy and left ventricular eccentric hypertrophy; By sup 201 Tl myocardial SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Yamazaki, Junichi; Ohsawa, Hidefumi; Uchi, Takashi (Toho Univ., Tokyo (Japan). School of Medicine) (and others)

    1992-03-01

    {sup 201}Tl myocardial SPECT was performed in cases of dilated cardiomyopathy and valvular heart disease with left ventricular eccentric hypertrophy, and the two groups were compared from the standpoint of the mechanism of onset of myocardial disorders. Significant coefficients of correlation were seen between the Tl score and LVDd (r=0.792, r=0.785) and Tl score and LVEF (r=-0.634, r=-0.555) in both dilated cardiomyopathy and valvular heart disease. In cases of valvular heart disease, significant correlation coefficients (r=-0.756, r=-0.720) between LVDd and r-WR (relative-washout rate), and Tl score and r-WR were observed, but no such correlation was seen in dilated cardiomyopathy. In valvular heart disease, a decrease in myocardial perfusion associated with enlargement of the left ventricle appeared, while in dilated cardiomyopathy, there was a marked decrease in LVEF in proportion to the thallium defect. Therefore, it was assumed that left ventricular wall disorders occur due to myocardial metabolic disorders and coronary microcirculation disorders. (author).

  14. Assessment of myocardial damage in dilated-phase hypertrophic cardiomyopathy by using indium-111-antimyosin Fab myocardial scintigraphy

    International Nuclear Information System (INIS)

    For the detection of myocardial cell damage, an 111In-antimyosin Fab study was carried out on seven patients (Group A) in the dilated phase of hypertrophic cardiomyopathy, seven patients (Group B) with dilated cardiomyopathy, and eight control patients (Group C). Imaging was done 48 hr after intravenous injection of 74 MBq of 111In-antimyosin Fab. Myocardial antimyosin uptake was visually graded as 0, +1, +2 or +3. A score of +2 or +3 was considered positive. The heart/lung ratio of antimyosin uptake (antimyosin index) also was determined. Antimyosin uptake was positive in seven (100%), nine (90%) and no (0%) patients in Groups A, B, and C, respectively. The antimyosin index in Groups A and B was 2.46 ± 0.49 and 2.04 ± 0.24, respectively, findings were significantly higher than that in Group C (1.51 ± 0.13) (p less than 0.01). Positive biopsy findings were noted in only two patients in Group A. Thus, antimyosin uptake was increased in dilated phase hypertrophic cardiomyopathy and dilated cardiomyopathy, which suggests ongoing necrotic changes in these patients

  15. Markers of immune activation and apoptosis in endomyocardial biopsies of individuals with recent-onset dilated cardiomyopathy

    Czech Academy of Sciences Publication Activity Database

    Kubánek, M.; Sramko, M.; Malušková, J.; Chudičková, Milada; Holáň, Vladimír; Kautzner, J.

    2013-01-01

    Roč. 34, č. 1 (2013), s. 211-211. ISSN 0195-668X. [Congress of the European-Society-of- Cardiology (ESC). 31.08.2013-,04.09.2013, Amsterdam] Institutional support: RVO:68378041 Keywords : dilated cardiomyopathy * immune activation and apoptosis Subject RIV: EC - Immunology

  16. 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.

  17. Collagen content, but not the ratios of collagen type III/I mRNAs, differs among hypertensive, alcoholic, and idiopathic dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    H.N. Soufen

    2008-12-01

    Full Text Available Cardiac interstitial fibrosis may contribute to ventricular dysfunction and the prognosis of patients with dilated cardiomyopathy. The objective of the present study was to determine if total myocardial collagen content and collagen type III/I (III/I ratio mRNAs differ in hypertensive, alcoholic, and idiopathic dilated cardiomyopathy subjects. Echocardiography and exercise cardiopulmonary testing were performed in patients with idiopathic (N = 22, hypertensive (N = 12, and alcoholic (N = 11 dilated cardiomyopathy. Morphometric analysis of collagen was performed in fragments obtained by endomyocardial biopsy with picrosirius red staining. The collagen III/I ratio was determined by reverse transcription polymerase chain reaction. Samples of controls (N = 10 were obtained from autopsy. Echocardiographic variables and maximal oxygen uptake were not different among dilated cardiomyopathy groups. Collagen was higher in all dilated cardiomyopathy groups (idiopathic, hypertensive and alcoholic, 7.36 ± 1.09% versus controls (1.12 ± 0.18%, P < 0.05. Collagen was lower in idiopathic dilated cardiomyopathy (4.97 ± 0.83% than hypertensive (8.50 ± 1.11% and alcoholic (10.77 ± 2.09% samples (P < 0.005 for both. The collagen III/I ratio in all samples from dilated cardiomyopathy patients was higher compared to that in controls (0.29 ± 0.04, P < 0.05 but was the same in the samples from idiopathic (0.77 ± 0.07, hypertensive (0.75 ± 0.07, and alcoholic (0.81 ± 0.16 dilated cardiomyopathy groups. Because of the different physical properties of the types of collagen, the higher III/I ratio may contribute to progressive ventricular dilation and dysfunction in dilated cardiomyopathy patients.

  18. 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.

  19. 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

  20. 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

  1. 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.

  2. Gallium-67 imaging in patients with dilated cardiomyopathy and biopsy-proven myocarditis

    International Nuclear Information System (INIS)

    Current standards for detection of myocarditis in a clinical setting rely on endomyocardial biopsy for accurate diagnosis. With this technique a subset of patients with dilated cardiomyopathy show unsuspected myocarditis histologically. Endomyocardial biopsy, despite its specificity, may lack sensitivity due to sampling error if the inflammation is patchy or focal. Therefore, inflammation-sensitive radioisotopic imaging may be a useful adjunct in the diagnosis of myocarditis. This study was designed to evaluate the applicability of gallium-67 (67Ga) myocardial imaging as an adjunct to endomyocardial biopsy in the diagnosis of myocarditis. Sixty-eight consecutive patients referred for evaluation of dilated cardiomyopathy underwent 71 parallel studies with 67Ga imaging and biopsies that served as the basis of comparison for this study. Histologic myocarditis was identified in 8% of biopsy specimens. Clinical and hemodynamic parameters could not be used to predict the presence of myocarditis. Five of six biopsy samples (87%) with myocarditis showed dense 67Ga uptake, whereas only nine of 65 negative biopsy samples (14%) were paired with equivocally positive 67Ga scans. The single patient with myocarditis and no myocardial 67Ga uptake had dense mediastinal lymph node uptake that may have obscured cardiac uptake. The incidence of myocarditis on biopsy with a positive 67Ga scan was 36% (5/14); however, the incidence of myocarditis with a negative 67Ga scan was only 1.8% (1/57). Follow-up scans for three patients showed close correlation of 67Ga uptake with myocarditis on biopsy. In conclusion 67Ga may be a useful screening test for identifying patients with a high yield of myocarditis on biopsy, and serial scans may eliminate the need for frequent biopsies in patients with proven myocarditis

  3. Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis

    Directory of Open Access Journals (Sweden)

    Inas Abdullsattar Saad

    2007-01-01

    Full Text Available Dilated cardiomyopathy is the most common type of heart muscle disease in children with idiopathic etiology in the majority of cases. Idiopathic dilated cardiomyopathy (IDCM is a severe illness which carries a high mortality rate in the pediatric population. In order to characterize IDCM evolution and identify prognostic predictors in our pediatric cardiology center in the western province of Saudi Arabia, 55 patients with IDCM were evaluated clinically and by echocardiography. They were followed for a minimum of one year and a maximum of four and a half years. Patients less than two years of age represented 69% of the cohort. Gender distribution revealed 65.5% female and 35.5% male. Outcomes were divided into four groups: 25 patients (45.5% improved (Group I, 17 patients (31% had a stationary course (Group II, 13 patients (23.6% deteriorated (Group III, and eventually 11 patients (from Group III died. Survival rate was 80% with a mean follow-up period of 36.2 ± 22.1 months. The older the age at presentation, the worse the prognosis, with P value= 0.029.In this study, we found a significant correlation of prognosis with end diastolic volume (EDV (P=0.05 as well as stroke volume (SV (P=0.04 on presentation. However, fractional shortening of ejection fraction on presentation could not be correlated statistically to the prognosis. Also results suggested that higher z-score of intraventricular septum & Left ventricular posterior wall dimensions in diastole significantly correlated to favorable outcomes and higher z-score of Left ventricular end diastolic dimension (LVEDD was significantly related to unfavorable outcome. We concluded that further multi-center studies are necessary to verify predictors of outcome in IDCM patients. Identification of markers affecting early myocardial function is essential to achieving improvements in treatments and consequently outcomes in this pediatric population.

  4. 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.

  5. 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.

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

    Science.gov (United States)

    Khilnani, Gopi C; Jain, Neetu; Tiwari, Pavan; Hadda, Vijay; Singh, Lavleen

    2015-01-01

    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. PMID:25624603

  7. 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. PMID:27120622

  8. 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.

  9. 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

  10. Clinical usefulness of Tc-99m hexakis 2-methoxybutyl isonitrile gated SPECT in patients with dilated cardiomyopathy: retrospective analysis

    International Nuclear Information System (INIS)

    Background: In Dilated cardiomyopathy the heart is enlarged and ventricles are dilated. Gated myocardial perfusion single photon emission computed tomography is considered state of the art for myocardial perfusion imaging. A retrospective analysis was conducted to evaluate patients with dilated cardiomyopathy with Tc-99m sestamibi gated myocardial perfusion single photon emission computed tomography to evaluate its clinical utility. Methods: A 10 year retrospective medical record review was done from 1991 to 2001 at Wake Forest University, North Carolina, USA. Eligibility criteria included a diagnosis of dilated cardiomyopathy and availability of coronary angiography and Tc-99m sestamibi cardiac imaging results. 26 cases were selected for the final review and inclusion in the study. The study was done with standard protocols for cardiac sestamibi imaging. Results: A total of 26 cases were included in the final analysis. Cases were divided into two main groups. Group-A included 16 patients with no correlation between Tc-99m sestamibi and cardiac catheterisation reports. Group-B included 10 patients with good correlation between the above tests. There were no significant differences between the left ventricular ejection fraction, angina history, sex distribution and diabetic status between the two groups. We applied Wilcoxon Signed Rank Test and z-test to quantify the difference between the two groups. Data was tabulated and z-test was performed. The calculated p value was <0.0001. This is significantly less than the tabulated p-value at 5% level of significance, i.e., 1.96. Significant differences exist between Group-A and Group-B. Conclusion: Tc-99m sestamibi is an excellent agent for investigating myocardial perfusion in dilated cardiomyopathy. The reversible and fixed perfusion defects (small to medium sized) seen in dilated cardiomyopathy after performance of Tc-99m sestamibi gated single photon emission computed tomography imaging may not be due to coronary

  11. 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.

  12. 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.

  13. 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

  14. 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.

  15. 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...

  16. 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...

  17. Resultados da cardiomioplastia no tratamento da cardiomiopatia dilatada Cardiomyoplasty results in the treatment of dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Luiz Felipe P Moreira

    1991-08-01

    Full Text Available A cardiomioplastia tem sido proposta como uma alternativa ao transplante cardíaco no tratamento das cardiomiopatias isquémicas ou dilatadas. No período de maio de 1988 a outubro de 1990, 16 pacientes portadores de cardiomiopatia dilatada foram submetidos à cardiomioplastia no Instituto do Coração. Dez pacientes estavam em classe funcional III e seis em classe IV. Não houve óbitos no período de pós-operatório imediato. O tempo médio de seguimento foi de 16,9 ± 2,5 meses e a sobrevida atuarial foi 74% no 1º ano e 64,8% no 2º ano após a cardiomioplastia, sendo influenciada pela má evolução dos pacientes operados com diâmetro de ventrículo esquerdo maior do que 80 mm. Esses valores foram superiores, contudo, à sobrevida de um e dois anos de 39,5 e 29,6%, respectivamente, apresentada pelo grupo controle de 20 pacientes mantidos clinicamente (p = 0,06. Cinco dos 11 pacientes em seguimento após a cardiomioplastia, retornaram à classe funcional I e seis estão em classe II. Aos seis meses de pós-operatório, foi documentada a elevação da fração de ejeção do ventrículo esquerdo de 20,1 ± 3,8 para 26 ± 7,8% pelo estudo radioisotópico (p Dynamic cardiomyplasty has been proposed as an alternative surgical treatment for severe cardiomyopathies. From May 1988 to October 1990, dynamic cardiomyoplasty was performed in 16 patients with dilated or chagasic cardiomyopathy at the Heart Institute. Ten patients were in New York Heart Association (NYHA Class III and six in Class IV. There were no operative deaths. During a men follow-up of 16.9 ± 2.5 months, the actuarial survival was 74% at 1 year and 64.8% at 2 years of follow-up. This survival was influenced by the worse evolution of patients with left ventricular internal diameter more than 80 mm. These results were, however, better than the survival of 39.5 and 29.6%, presented at the same periods, respectively, by 20 patients maintained under medical therapy. Five of the 11

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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.

  20. 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

  1. Modulation of serotonin transporter function during fetal development causes dilated heart cardiomyopathy and lifelong behavioral abnormalities.

    Directory of Open Access Journals (Sweden)

    Cornelle W Noorlander

    Full Text Available BACKGROUND: Women are at great risk for mood and anxiety disorders during their childbearing years and may become pregnant while taking antidepressant drugs. In the treatment of depression and anxiety disorders, selective serotonin reuptake inhibitors (SSRIs are the most frequently prescribed drugs, while it is largely unknown whether this medication affects the development of the central nervous system of the fetus. The possible effects are the product of placental transfer efficiency, time of administration and dose of the respective SSRI. METHODOLOGY/PRINCIPAL FINDINGS: In order to attain this information we have setup a study in which these parameters were measured and the consequences in terms of physiology and behavior are mapped. The placental transfer of fluoxetine and fluvoxamine, two commonly used SSRIs, was similar between mouse and human, indicating that the fetal exposure of these SSRIs in mice is comparable with the human situation. Fluvoxamine displayed a relatively low placental transfer, while fluoxetine showed a relatively high placental transfer. Using clinical doses of fluoxetine the mortality of the offspring increased dramatically, whereas the mortality was unaffected after fluvoxamine exposure. The majority of the fluoxetine-exposed offspring died postnatally of severe heart failure caused by dilated cardiomyopathy. Molecular analysis of fluoxetine-exposed offspring showed long-term alterations in serotonin transporter levels in the raphe nucleus. Furthermore, prenatal fluoxetine exposure resulted in depressive- and anxiety-related behavior in adult mice. In contrast, fluvoxamine-exposed mice did not show alterations in behavior and serotonin transporter levels. Decreasing the dose of fluoxetine resulted in higher survival rates and less dramatic effects on the long-term behavior in the offspring. CONCLUSIONS: These results indicate that prenatal fluoxetine exposure affects fetal development, resulting in cardiomyopathy

  2. CLINICAL AND INSTRUMENTAL DATA AND SURVIVAL IN DILATED CARDIOMYOPATHY: THREE-YEAR OBSERVATION RESULTS

    Directory of Open Access Journals (Sweden)

    L. A. Zotova

    2014-07-01

    Full Text Available Aim — to assess survival of patients with dilated cardiomyopathy (DCM in 3‑year follow-up based on clinical history, clinical symptoms, indicators of instrumental methods of examination and tactics of the patients.Materials and methods. 105 patients with diagnosed cardiomyopathy were included in prospective single-center study. Follow‑up period was 3 years or until achieving primary endpoint. Complaints were collected, general clinical research, 6-minute walk test, electrocardiography in 12‑lead were performed annually. Also scale of evaluation of clinical status in patients with chronic heart failure (CHF in the modification of V.Y. Mareev and assessment of adherence were used annualy.Results. The group of patients with DCM was represented primarily by man, severe heart failure (III–IV functional class was originally diagnosed in almost 80 % of patients. During the 3 years of follow-up progression of heart failure, worsening of hemodynamic indices were identified in survivors. When evaluating recommended therapy with found that it meets the current guidelines of treatment of heart failure,however, low adherence to treatment of patients was revealed with statistically significant difference between groups of survivors and deceased patients. The annual mortality rate was 20 % and had no significant fluctuations. The main cause of death was heart failure decompensation.Conclusion. Progression of CHF in patients with DCM was confirmed .The most reliable method of assessing the progressive course of heart failure is 6‑minute walk test. The annual high mortality (20 % indicates an extremely poor prognosis for this disease. The major causes of death were progression of heart failure and sudden cardiac death.

  3. 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

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

    International Nuclear Information System (INIS)

    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

  5. 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...

  6. 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

  7. 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.

  8. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    International Nuclear Information System (INIS)

    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)

  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. Dilated Cardiomyopathy Revealing Cushing Disease: A Case Report and Literature Review.

    Science.gov (United States)

    Marchand, Lucien; Segrestin, Bérénice; Lapoirie, Marion; Favrel, Véronique; Dementhon, Julie; Jouanneau, Emmanuel; Raverot, Gérald

    2015-11-01

    Cardiovascular impairments are frequent in Cushing's syndrome and the hypercortisolism can result in cardiac structural and functional changes that lead in rare cases to dilated cardiomyopathy (DCM). Such cardiac impairment may be reversible in response to a eucortisolaemic state.A 43-year-old man with a medical past of hypertension and history of smoking presented to the emergency department with global heart failure. Coronary angiography showed a significant stenosis of a marginal branch and cardiac MRI revealed a nonischemic DCM. The left ventricular ejection fraction (LVEF) was estimated as 28% to 30%. Clinicobiological features and pituitary imaging pointed toward Cushing's disease and administration of adrenolytic drugs (metyrapone and ketoconazole) was initiated. Despite the normalization of cortisol which had been achieved 2 months later, the patient presented an acute heart failure. A massive mitral regurgitation secondary to posterior papillary muscle rupture was diagnosed as a complication of the occlusion of the marginal branch. After 6 months of optimal pharmacological treatment for systolic heart failure, as well as treatment with inhibitors of steroidogenesis, there was no improvement of LVEF. The percutaneous mitral valve was therefore repaired and a defibrillator implanted. The severity of heart failure contraindicated pituitary surgery and the patient was instead treated by stereotaxic radiotherapy.This is the first case reporting a Cushing's syndrome DCM without improvement of LVEF despite normalization of serum cortisol levels. PMID:26579807

  15. 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

  16. Clinical meaning of 123I-MIBG myocardial SPECT in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Metaiodobenzylguanidine(MIBG)-myocardial SPECT was performed on patients with dilated cardiomyopathy (DCM) undergoing treatment with β blocker (Nipradilol). The findings of MIBG-myocardial SPECT were compared with the changes in cardiac function obtained by echocardiograms. The patients enrolled in the study were seven males who has been diagnosed as definitely suffering from DCM according to diagnostic guidelines provided by the Ministry of Health and Welfare, Japan. The patients were aged 57.5±10.2 years. Following intravenous administration of 111 MBq (3 mCi) of MIBG, myocardial SPECT was taken 20 minutes, and 4 hours later. The washout rate of the left ventricular wall was higher in the unchanged group (40.7±1.2%) than in the improved group (30.3±6.1%). Both the early and delayed images showed higher extent and severity scores for the unchanged group than for the improved group. A correlation of left ventricular ejection fraction (LVEF) with the washout rates was demonstrated (r=-0.819, p<0.05). A correlation was also observed between the variations in LVEF before and after β blocker therapy with the washout rates (r=-0.969, p<0.01), MIBG-myocardial SPECT suggested possibility of the evaluation of severity and prognosis in the patients with DCM. (author)

  17. 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).

  18. 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.

  19. 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.

  20. 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.)

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.

  3. 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.

  4. 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.

  5. Correlation between 67-gallium imaging and endo myocardial biopsy in children with severe dilated cardiomyopathy

    International Nuclear Information System (INIS)

    A comparative study between 67 Ga imaging and endo myocardial biopsy (EB) in children with severe dilated cardiomyopathy (DC) is performed. The results in a group of patients with active myocarditis submitted to immunosuppressive therapy are evaluated and repeated after six months. In 32 patients (72.7%) the EB revealed presence of inflammatory process; 21 (65.6%) of these had a positive 67 Ga uptake and 11 (34.4%) negative. Twelve patients with no evidence of inflammatory process in the EB, nine (75%) presented negative 67 Ga uptake. However, when the intensity of myocardial inflammatory was analysed (mild, moderate and severe) and correlated with 67 Ga imaging, was observed that the majority of patients with negative 67 Ga uptake (11 patients) had inflammatory infiltration (nine patients). In this way the 67 Ga uptake demonstrated a good correlation in the diagnosis of moderate and severe inflammatory process in children with DC. This is important because the use of immunosuppressive drugs is indicated only in these group. (author)

  6. Abnormal myocardial fatty acid metabolism in dilated cardiomyopathy detected by iodine-123 phenylpentadecanoic acid and tomographic imaging

    International Nuclear Information System (INIS)

    The radioidinated synthetic fatty acid iodine-123 phenylpentadecanoic acid (IPPA) has proven useful in the identification of regional abnormalities of cardiac metabolism in patients with myocardial ischemia. The present study was performed to test the hypothesis that the myocardial distribution and turnover of fatty acids, assessed noninvasively with IPPA, are altered in patients with cardiomyopathy. Nine normal volunteers and 19 patients with dilated cardiomyopathy of various etiologies underwent cardiac imaging with single-photon emission computed tomography (SPECT) after intravenous injection of IPPA. Apical short-axis and basal short-axis sections were reconstructed and quantitatively analyzed for relative IPPA activity distribution and washout. Patients with congestive cardiomyopathy demonstrated significantly greater heterogeneity of IPPA uptake than normal subjects (maximal percent variation of activity 27 +/- 11 vs 18 +/- 4, p less than 0.01). They also demonstrated a more rapid percent washout rate than control subjects (24 +/- 8 vs 17 +/- 6 for the apical short-axis section, p less than 0.05; 26 +/- 7 vs 18 +/- 5 for the basal short-axis section, p less than 0.01). These abnormalities of fatty acid distribution and turnover were independent of the etiology of the cardiomyopathy. The degree of heterogeneity of IPPA uptake was significantly related to the patients' New York Heart Association functional class (r = 0.64, p less than 0.01). Thus, compared with normal myocardium, the myocardium of patients with congestive cardiomyopathy demonstrates a more heterogeneous distribution of fatty acid uptake, which parallels the clinical severity of the disease. Furthermore, patients with congestive cardiomyopathy demonstrate a more rapid myocardial clearance of the labeled fatty acid, as assessed with SPECT imaging

  7. 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.)

  8. 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.

  9. Variability of M-mode versus two-dimensional echocardiography measurements in children with dilated cardiomyopathy.

    Science.gov (United States)

    Lee, Caroline K; Margossian, Renee; Sleeper, Lynn A; Canter, Charles E; Chen, Shan; Tani, Lloyd Y; Shirali, Girish; Szwast, Anita; Tierney, Elif Seda Selamet; Campbell, M Jay; Golding, Fraser; Wang, Yanli; Altmann, Karen; Colan, Steven D

    2014-04-01

    M-mode and 2-dimensional (2D) echocardiographic imaging are routinely used to quantify left-ventricular (LV) size and function in pediatric patients with dilated cardiomyopathy (DCM). The reproducibility of and correlation between these techniques are unknown. This analysis sought to compare interreader, intrareader, and interacquisition reproducibility of M-mode versus 2D measurements in pediatric DCM patients. The Ventricular Volume Variability study of the Pediatric Heart Network is a multicenter, prospective, observational study assessing the course of chronic DCM in children. Two sonographers performed baseline image acquisitions locally, and two readers performed measurements at the echocardiographic core laboratory. One reader repeated measurements 1 month later. These data were used to assess reproducibility and agreement between M-mode and 2D measurements. One hundred sixty-nine subjects were enrolled. M-mode had similar or greater reproducibility in both intrareader and interreader settings for LV dimensions, shortening fraction (SF), and most wall thicknesses. In contrast, 2D reproducibility was similar or better for nearly all variables in the interacquisition setting but not for SF. Interacquisition variability was approximately twice the intrareader variability. LV dimensions by either modality consistently had high reproducibility and had the highest agreement between modalities. In pediatric DCM patients, variability of linear echocardiographic assessment could be minimized by relying on a single reader and using a consistent method (M-mode or 2D) for serial measurements, preferably M-mode when SF is the primary variable of interest. Except for LV dimensions, M-mode and 2D values should not be used interchangeably due to poor agreement. PMID:24265000

  10. 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.

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

    Directory of Open Access Journals (Sweden)

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

    Directory of Open Access Journals (Sweden)

    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.

  13. 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

  14. The potential role of myocardial serotonin receptor 2B expression in canine dilated cardiomyopathy.

    Science.gov (United States)

    Fonfara, Sonja; Hetzel, Udo; Oyama, Mark A; Kipar, Anja

    2014-03-01

    Serotonin signalling in the heart is mediated by receptor subtype 2B (5-HTR2B). A contribution of serotonin to valvular disease has been reported, but myocardial expression of 5-HTR2B and its role in canine dilated cardiomyopathy (DCM) is not known. The aim of the present study was to investigate myocardial 5-HTR2B mRNA expression in dogs with DCM and to correlate results with expression of markers for inflammation and remodelling. Myocardial samples from eight healthy dogs, four dogs with DCM, five with cardiac diseases other than DCM and six with systemic non-cardiac diseases were investigated for 5-HTR2B mRNA expression using quantitative PCR (qPCR). The results were compared to mRNA expression of selected cytokines, matrix metalloproteinases (MMP) and tissue inhibitors of matrix metalloproteinase (TIMP). Laser microdissection with subsequent qPCR and immunohistochemistry were employed to identify the cells expressing 5-HTR2B. The myocardium of control dogs showed constitutive 5-HTR2B mRNA expression. In dogs with DCM, 5-HTR2B mRNA values were significantly greater than in all other groups, with highest levels of expression in the left ventricle and right atrium. Myocytes were identified as the source of 5-HTR2B mRNA and protein. A significant positive correlation of 5-HTR2B mRNA with expression of several cytokines, MMPs and TIMPs was observed. The findings suggest that serotonin might play a role in normal cardiac structure and function and could contribute to myocardial remodelling and functional impairment in dogs with DCM. PMID:24440442

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

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.

  17. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Genotype phenotype correlations of cardiac beta-myosin heavy chain mutations in Indian patients with hypertrophic and dilated cardiomyopathy

    DEFF Research Database (Denmark)

    Rai, Taranjit Singh; Ahmad, Shamim; Bahl, Ajay; Ahuja, Monica; Ahluwalia, Tarun Veer Singh; Singh, Balvinder; Talwar, K K; Khullar, Madhu

    The aim of the current study was to determine the frequency of mutations in the beta-myosin heavy chain gene (MYH7) in a cohort of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) and their families, and to investigate correlations between genotype and phenotype. About 130...... consecutive patients diagnosed with HCM or DCM (69 with HCM and 61 with DCM) attending the cardiology clinic of Post Graduate Institute of Medical Education and Research were screened for mutations in the MYH7 gene. The control group for genetic studies consisted of 100 healthy subjects. We report 14...... mutations in 6 probands (5 probands in HCM and 1 proband in DCM) and their family members. Out of these 6 mutations, 3 are new and are being reported for the first time. One known mutation (p.Gly716Arg) was found to be "de novo" which resulted in severe asymmetric septal hypertrophy (31 mm) and resulted in...

  20. Comparison of Benefits from Cardiac Resynchronization Therapy between Patients with Ischemic Cardiomyopathy and Patients with Idiopathic Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Talia Alenabi

    2009-06-01

    Full Text Available Background: Cardiac resynchronization therapy (CRT is an effective treatment for patients with moderate to severe heart failure. However, 20-30% of patients remain non-responders to CRT. We sought to identify which patients benefit the most from CRT in regard to the etiology of heart failure. Methods: Eighty-three consecutive patients (62 men who had a biventricular pacemaker inserted at Tehran Heart Center between May 2004 and March 2007 were evaluated retrospectively. The inclusion criteria were comprised of New York Heart Association (NYHA class III or IV, left ventricular ejection fraction120ms. After 6 months, response was defined as being alive, no hospitalization for cardiac decompensation, and an improvement in NYHA class>1 grade. Results: After 6 months, 60 patients out of the 83 patients were responders. Amongst the 83 patients, 48 had ischemic cardiomyopathy and 35 had non-ischemic cardiomyopathy. A cross-tabulation of response versus etiology showed no significant difference between ischemic versus non-ischemic cardiomyopathy with regard to response to CRT (P=0.322. Conclusion: According to our study, there was no difference in response to CRT between ischemic versus non-ischemic cardiomyopathy at six months’ follow-up.

  1. Evaluation of left ventricular volumes and ejection fraction by gated SPECT and cardiac MRI in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    The goal of this study was to evaluate the accuracy of gated single photon emission computed tomography (SPECT) in the assessment of left ventricular (LV) end-diastolic/end-systolic volumes (EDV, ESV) and ejection fraction (LVEF) in patients with dilated cardiomyopathy, using cardiac magnetic resonance imaging (MRI) as the reference method. Furthermore, software-specific characteristics of Quantitative Gated SPECT (QGS), Emory Cardiac Toolbox (ECTB) and 4D-MSPECT were analysed. Thirty-six patients with dilated cardiomyopathy who underwent gated 99mTc-methoxyisobutylisonitrile SPECT and cardiac MRI were included. LV EDV, ESV and LVEF values of gated SPECT were calculated using QGS, ECTB and 4D-MSPECT. The correlation between the results of gated SPECT and cardiac MRI was excellent for EDV [R = 0.872 (QGS), R = 0.879 (ECTB), R = 0.869 (4D-MSPECT)], ESV [R = 0.908 (QGS), R = 0.897 (ECTB), R = 0.880 (4D-MSPECT)] and LVEF [R = 0.794 (QGS), R = 0.763 (ECTB), R = 0.710 (4D-MSPECT)]. EDV and ESV assessed by QGS did not differ significantly from those assessed by cardiac MRI (all p = NS), whereas EDV and ESV were overestimated by ECTB and 4D-MSPECT compared with cardiac MRI (all p < 0.05). LVEF was overestimated by QGS, ECTB and 4D-MSPECT compared with cardiac MRI (all p < 0.05). The correlation between gated SPECT and cardiac MRI is excellent for LV volume and LVEF values calculated by QGS, ECTB and 4D-MSPECT in patients with dilated cardiomyopathy. However, algorithm-varying over- or underestimation of LV volumes and LVEF should be accounted for in the clinical context. (orig.)

  2. Anesthetic management of an elderly patient with kyphoscoliosis and dilated cardiomyopathy posted for abdominal hysterectomy and salpingo-oophorectomy

    Directory of Open Access Journals (Sweden)

    Suvidha Sood

    2015-01-01

    Full Text Available A 76-year-old kyphoscoliotic female patient presented with severe pain and sudden acute abdominal distension for 1-week and was diagnosed to have right-sided massive twisted ovarian cyst. The patient was a known case of hypertension, dilated cardiomyopathy with low 20% cardiac ejection fraction. Though very few incidences of multiple co-morbid conditions existing together in a single elderly patient have been reported in the past, it is important to titrate the dosage, type of anesthetic agents and their routes of administration in high risk patients.

  3. Heterozygous Lmna(delK32) mice develop dilated cardiomyopathy through a combined pathomechanism of haploinsufficiency and peptide toxicity

    DEFF Research Database (Denmark)

    Cattin, M. E.; Bertrand, A. T.; Schlossarek, S.;

    2013-01-01

    Dilated cardiomyopathy (DCM) associates left ventricular (LV) dilatation and systolic dysfunction and is a major cause of heart failure and cardiac transplantation. LMNA gene encodes lamins A/C, proteins of the nuclear envelope. LMNA mutations cause DCM with conduction and/or rhythm defects. The...... pathomechanisms linking mutations to DCM remain to be elucidated. We investigated the phenotype and associated pathomechanisms of heterozygous Lmna(K32/) (Het) knock-in mice, which carry a human mutation. Het mice developed a cardiac-specific phenotype. Two phases, with two different pathomechanisms, could be...... observed that lead to the development of cardiac dysfunction, DCM and death between 35 and 70 weeks of age. In young Het hearts, there was a clear reduction in lamin A/C level, mainly due to the degradation of toxic K32-lamin. As a side effect, lamin A/C haploinsufficiency probably triggers the cardiac...

  4. 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...

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

    Science.gov (United States)

    Stark, Klaus; Esslinger, Ulrike B; Reinhard, Wibke; Petrov, George; Winkler, Thomas; Komajda, Michel; Isnard, Richard; Charron, Philippe; Villard, Eric; Cambien, François; Tiret, Laurence; Aumont, Marie-Claude; Dubourg, Olivier; Trochu, Jean-Noël; Fauchier, Laurent; Degroote, Pascal; Richter, Anette; Maisch, Bernhard; Wichter, Thomas; Zollbrecht, Christa; Grassl, Martina; Schunkert, Heribert; Linsel-Nitschke, Patrick; Erdmann, Jeanette; Baumert, Jens; Illig, Thomas; Klopp, Norman; Wichmann, H-Erich; Meisinger, Christa; Koenig, Wolfgang; Lichtner, Peter; Meitinger, Thomas; Schillert, Arne; König, Inke R; Hetzer, Roland; Heid, Iris M; Regitz-Zagrosek, Vera; Hengstenberg, Christian

    2010-10-01

    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 a large SNP

  6. Genetic Association Study Identifies HSPB7 as a Risk Gene for Idiopathic Dilated Cardiomyopathy

    Science.gov (United States)

    Stark, Klaus; Esslinger, Ulrike B.; Reinhard, Wibke; Petrov, George; Winkler, Thomas; Komajda, Michel; Isnard, Richard; Charron, Philippe; Villard, Eric; Cambien, François; Tiret, Laurence; Aumont, Marie-Claude; Dubourg, Olivier; Trochu, Jean-Noël; Fauchier, Laurent; DeGroote, Pascal; Richter, Anette; Maisch, Bernhard; Wichter, Thomas; Zollbrecht, Christa; Grassl, Martina; Schunkert, Heribert; Linsel-Nitschke, Patrick; Erdmann, Jeanette; Baumert, Jens; Illig, Thomas; Klopp, Norman; Wichmann, H.-Erich; Meisinger, Christa; Koenig, Wolfgang; Lichtner, Peter; Meitinger, Thomas; Schillert, Arne; König, Inke R.; Hetzer, Roland; Heid, Iris M.; Regitz-Zagrosek, Vera; Hengstenberg, Christian

    2010-01-01

    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

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

    Directory of Open Access Journals (Sweden)

    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

  8. 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

  9. Usefulness of I-123 MIBG myocardial SPECT in patients with dilated cardiomyopathy for predicting cardiac events

    International Nuclear Information System (INIS)

    We evaluated cardiac sympathetic nerve activity in patients with dilated cardiomyopathy (DCMP) using I-123 metaiodobenzylguanidine (MIBG) myocardial SPECT, and tried to find variables to predict disease progression (DP). MIBG SPECT was performed in 17 patients (M:F = 11:6, age 63.9±11.1 years) with DCMP (idiopathic = 14, ischemic = 2 and other = 1). At 15 min and 4 hs after IV injection of I-123 MIBG (5 mCi), planar and SPECT images were acquired. Using planar images, heart to mediastinum (H/M) ratios, and washout rate (WR) 100 x [ (H-M)15min (H-M)4hr]/(H-M) 15min were calculated with ROIs drawn for heart contour and mediastinum (7x7 pixels). Using SPECT images, total uptake score (TUS) was defined as sum of 17 myocardial segments using 3-point scale. Using M-mode echocardiography, LV ejection fraction (LVEF) was calculated as follows: (LVDd2-LVDs2)/LVDd2. The decline of EF more than 5%, or hospitalization due to dyspnea aggravation was defined as DP. All the patients were observed for 339±106 days. During follow up periods, no mortality cases were observed but 2 cases suffered from EF decline, 2 hospitalization and 1 both EF decline and hospitalization. However Cox proportion hazard regression analysis did not identify significant parameter for DP among H/M ratio, WR, TUS, and deltaTUS. Patients could be divided into 2 groups based on WR, the known prognostic marker. The high WR patients (more than 50%, n=9) had significantly lower H/M ratio (1.55±0.27 versus 2.01±0.32 p<0.01) and significantly lower TUS4hr (12.1±13.9 versus 32.7±11.4, p<0.01) than low WR patients (less than 50%, n=8). WR and TUS4hr showed negative correlation (rho=-0.705, p<0.01). MIBG SPECT was applied to DCMP patients for evaluation of DP. Longer follow up period of more than 1 year seems to be essential for prognosis evaluation. In addition to WR, TUS obtained at 4 hour MIBG SPECT may be used as a useful marker of DCMP

  10. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  12. Measurement of glucose metabolism in patients with dilated cardiomyopathy using positron emission tomography with 18F-FDG: Initial Experience

    International Nuclear Information System (INIS)

    Introduction: Fluorine18 deoxyglucose (18F-FDG) has been used in numerous studies to determine the cardiac rate of glucose metabolism in normal and pathological conditions. It is known that during heart failure the metabolic pattern is altered. Patlack's graphical analysis allows the assessment of heart muscle glucose consumption in patients with non-ischaemic heart failure and normal subjects. Methods: Standardized measurement of glucose metabolism was performed in four patients with dilated cardiomyopathy and three healthy subjects. All subjects received an oral load of carbohydrates (75gr) previous to scanning. Dynamic images of the thorax were acquired. Myocardial uptake was estimated from time-activity curves in the atrium and left ventricle using Patlack's graphical analysis. Results: All subjects studied were male. 18F-FDG uptake rate for the group with dilated cardiomyopathy was 1.31±0.2, versus 1.26±0.37 ml/100gr/min in the control group. Conclusion: Measurement of cardiac glucose metabolism by 18F-FDG PET is feasible in a clinical service, allowing impact evaluation of physiologic and metabolic changes in the myocardium in different pathologic scenarios in addition to therapy assessment

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

    Directory of Open Access Journals (Sweden)

    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.

  14. Effects of Omega-3 Polyunsaturated Fatty Acids on Heart Function and Oxidative Stress Biomarkers in Pediatric Patients with Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Omidreza Firuzi

    2013-06-01

    Full Text Available Objectives: Dilated cardiomyopathy is the most prevalent type of cardiomyopathy in children, which results in congestive heart failure and causes significant morbidity and mortality. This study, aims to investigate the effect of supplementation with omega-3 polyunsaturated fatty acids (n-3 PUFA on heart function and oxidative stress biomarkers in these patients. Methods: The present research was a case-control study on pediatric patients with dilated cardiomyopathy, who received n-3 PUFA and anti-failure therapy for 6 months (group 1, n = 6, or anti-failure therapy alone for 6 months (group 2, n = 6, as well as age matched normal individuals (group 3, n = 6, and evaluated the cardiac function and biomarkers of oxidative stress. Results: Echocardiographic parameters, such as left ventricular ejection fraction, shortening fraction, tissue Doppler Ea and Aa waves of lateral annulus of tricuspid valve, and Ea and S wave of septum, were significantly improved in group 1 after n-3 PUFA compared to pre- treatment status, while they were not changed after treatment in group 2. Antioxidant enzymes, including catalase and glutathione peroxidase activities in erythrocytes were slightly decreased, while plasma 8-iso-prostaglandin F2α concentrations were somewhat increased in group 1 compared to groups 2 and 3, however these changes were not statistically significant. Total antioxidant capacity of plasma was similar in all 3 groups. Conclusions: The results indicate that some echocardiographic parameters were significantly improved in patients receiving omega-3 fish oil. However, omega-3 had no significant effect on oxidative stress biomarkers.

  15. No mutation but high mRNA expression of Coxsackie-Adenovirus Receptor was observed in both dilated and ischemic cardiomyopathy.

    Science.gov (United States)

    Tatrai, Eniko; Bedi, Katalin; Kovalszky, Ilona; Hartyanszky, Istvan; Laszik, Andras; Acsady, Gyorgy; Sotonyi, Peter; Hubay, Marta

    2011-10-10

    The most common causes of acute myocarditis and the possible consequence of dilated cardiomyopathy are virus infections. The receptor of the two most common viruses connected to these myocardial diseases was identified as Coxsackie-Adenovirus Receptor. The purpose of this study was to assess Coxsackie-Adenovirus Receptor mRNA expression in the myocardium and search for mutations in the Coxsackie-Adenovirus Receptor gene to compare dilated, inflammatory and ischemic cardiomyopathy with control group. All the myocardial samples were obtained from 35 explanted hearts during heart transplantation, than DNA and RNA were isolated from the muscle samples. cDNA was generated from RNA using reverse transcription, and real-time PCR was performed with relative quantification by β-actin gene as endogenous control. Using DNA extracted from the myocardial samples, we sequenced all the seven exons of the Coxsackie-Adenovirus Receptor gene. Coxsackie-Adenovirus Receptor mRNA expression was higher in both ischemic and dilated cardiomyopathy groups than in inflammatory cardiomyopathy and healthy control groups. Sequencing of CAR gene showed no sign of mutation. Therefore, the sequences result of CAR exons did not show any mutation or polymorphism, that explains a determinant role of CAR in dilated or ischemic CM. Our results suggest that high mRNA expression of Coxsackie-Adenovirus Receptor may support its role in regeneration of the damaged myocardium rather than having any role in viral mediated heart disease. PMID:21641134

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

    Directory of Open Access Journals (Sweden)

    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.

  17. Early combined treatment with sildenafil and adipose-derived mesenchymal stem cells preserves heart function in rat dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Fu Morgan

    2010-09-01

    Full Text Available Abstract Background We investigated whether early combined autologous adipose-derived mesenchymal stem cell (ADMSC and sildenafil therapy offers an additive benefit in preserving heart function in rat dilated cardiomyopathy (DCM. Methods Adult Lewis rats (n = 8 per group were divided into group 1 (normal control, group 2 (saline-treated DCM rats, group 3 [2.0 × 106 ADMSC implanted into left ventricular (LV myocardium of DCM rats], group 4 (DCM rats with sildenafil 30 mg/kg/day, orally, and group 5 (DCM rats with combined ADMSC-sildenafil. Treatment was started 1 week after DCM induction and the rats were sacrificed on day 90. Results The results showed that mitochondrial protein expressions of connexin43 and cytochrome-C were lowest in group 2, and lower in groups 3 and 4 than in group 5 (p Conclusion Early combined ADMSC/sildenafil is superior to either treatment alone in preserving LV function.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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

  20. Clinical validation of the gated blood pool SPECT QBS processing software for evaluating cardiac function in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Full text: The study was carried out to evaluate the commercially available automated GBPS processing software for LVEF and RVEF measurement in patients with cardiomyopathy. Patients (total=41; 24 males; mean age-44 ± 14 years) referred to our department for assessment of cardiac function in patients having dilated cardiomyopathy were prospectively included in the study. In patients with DCM, First pass radionuclide ventriculography (FP-RNV) and planar multi-gated radionuclide angiography (MUGA) were performed at rest after in-vivo labeling of RBCs. LVEF was also calculated by Echo in these patients. LVEF and RVEF were calculated by MUGA and FP-RNV respectively. Immediately after obtaining the planar views, GBPS was performed with GE Infinia Hawkeye4 camera, detectors in L-mode configuration, with step and shoot mode acquisition - 60 steps over 180 deg, 20 s per step, 16 frames per cardiac cycle, 64 x 64 matrix. GBPS was processed using the fully automated QBS algorithm and LVEF, RVEF were calculated. Spearman's coefficient (r) of correlation was calculated for the different sets of values. The level of statistical significance was set at < 5%. Bland-Altman plots were inspected to visually assess the association between measurements from different methods. Results: Head on comparison of the mean values for patients: PLANAR QBPS ECHO LVEF (%) 31 ± 11 VS 34 ± 12; RVEF (%) 46 ± 14 VS 43 ± 17. In patients with DCM, LVEF values calculated from GBPS showed very good correlation with MUGA (r=0.91) and echocardiography (r=0.83; all p<0.0001). Bland-Altman plot showed overestimation for GBPS-LVEF values compared to MUGA. RVEF values calculated by FPRNV and QBS-SPECT also showed good correlation (r=0.87; p<0.0001). Bland-Altman plot for RVEF values showed a tendency for an overestimation of higher RVEF values with GBPS. Patients with DCM also tend to suffer from right ventricular cardiomyopathy. Isolated right sided cardiomyopathy is also a known entity. Since RVEF is

  1. 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.

  2. Primary Systemic Carnitine Deficiency Presenting as Recurrent Reye-Like Syndrome and Dilated Cardiomyopathy

    OpenAIRE

    Jia-Woei Hou

    2002-01-01

    Carnitine deficiency syndrome is a rare and potentially fatal but treatable metabolic disorder.I present a 6-year-old girl with primary systemic carnitine deficiency (SCD) provedby very low plasma carnitine level. Her major clinical features included neonatal metabolicacidosis, epilepsy, recurrent infections, acute encephalopathy, and dilated cardiomyopathywith heart failure before 4 years of age. Other features such as hepatomegaly, hypoglycemia,or hyperammonemia were noted around 5 years of...

  3. 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...

  4. 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.

  5. 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.

  6. An uncommon clinical presentation of relapsing dilated cardiomyopathy with identification of sequence variations in MYNPC3, KCNH2 and mitochondrial tRNA cysteine

    OpenAIRE

    Guillen Sacoto, Maria J.; Chapman, Kimberly A; Deneen Heath; Mary Beth Seprish; Zand, Dina J.

    2015-01-01

    We describe a young girl with dilated cardiomyopathy, long QT syndrome, and possible energy deficiency. Two major sequence changes were identified by whole exome sequencing (WES) and mitochondrial DNA analysis that were interpreted as potentially causative. Changes were identified in the KCNH2 gene and mitochondrial tRNA for cysteine. A variation was also seen in MYPBC3. Since the launch of WES as a clinically available technology in 2010, there has been concern regarding the identification o...

  7. 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

  8. A novel LMNA mutation (R189W in familial dilated cardiomyopathy: evidence for a 'hot spot' region at exon 3: a case report

    Directory of Open Access Journals (Sweden)

    Biagini Andrea

    2010-03-01

    Full Text Available Abstract We describe a case of a patient with idiopathic dilated cardiomyopathy and cardiac conduction abnormalities who presented a strong family history of sudden cardiac death. Genetic screening of lamin A/C gene revealed in proband the presence of a novel missense mutation (R189W, near the most prevalent lamin A/C mutation (R190W, suggesting a "hot spot" region at exon 3.

  9. Myocardial enhancement on magnetic resonance imaging with gadolinium-diethylenetriamine pentaacetic acid and improvement of left ventricular function in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    This study evaluated the significance of myocardial gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) enhancement on magnetic resonance imaging for the improvement of left ventricular function in patients with dilated cardiomyopathy. Twenty-seven patients with dilated cardiomyopathy (mean age 59±11 years) were studied. The magnitude of myocardial Gd-DTPA enhancement was quantitatively assessed using signal intensity ratio and compared to changes in left ventricular function and adverse cardiac events during a relatively long follow-up period. Regional high signal intensity ratio, defined as ≥mean+2SD in seven normal subjects, was found in 14 patients: in three or more regions out of five myocardial regions analyzed in six patients (extensive enhancement) and in only one or two regions in eight patients (limited enhancement). The remaining 13 patients had no high signal ratio in any of the five regions analyzed (no enhancement). During the follow-up period of 3.9±1.9 years, four patients died of cardiac causes. The incidence of cardiac death was 33.3% in patients with extensive enhancement, 12.5% in those with limited enhancement and 7.7% in those without enhancement, but there was no statistical difference. Mild improvement in fractional shortening was observed in patients without enhancement during the follow-up (19±4%→27±10%, p=0.03). Evaluation of myocardial Gd-DTPA enhancement on magnetic resonance imaging may provide useful prognostic information for patients with dilated cardiomyopathy. (author)

  10. The relationship between the improvement of cardiac function and the myocardial uptake of I-123 metaiodobenzylguanidine in patients with dilated cardiomyopathy treated by beta-blocker

    International Nuclear Information System (INIS)

    Chronic β-blocker therapy improves hemodynamics and cardiac function in patients with idiopathic dilated cardiomyopathy. However, the change in myocardial uptake of I-123 metaiodobenzylguanidine (123I-MIBG) before and after treatment has not been determined. Myocardial imaging with 123I-MIBG was performed before and 2 or 3 months after β-blocker (bisoprolol) therapy in 11 patients with dilated cardiomyopathy. The following parameters were compared before and after the treatment : 1) New York Heart Association functional class, 2) X-ray cardiothoracic ratio, 3) heart rate and blood pressure, 4) echocardiographic data (left ventricular end-diastolic and end-systolic diameters, and left ventricular ejection fraction), 5) plasma concentrations of epinephrine, norepinephrine and human atrial natriuretic peptide (HANP), and 6) exercise tolerance time by treadmill. The heart-to-mediastinum ratio of 123I-MIBG activities obtained 3 hours after intravenous injection (late H/M) and washout rate improved significantly after β-blocker therapy. Cardiothoracic ratio, heart rate, echocardiographic parameters, HANP and exercise tolerance also improved significantly. Late H/M had no significant relationship with any of the clinical parameters, but washout rate was significantly related to left ventricular ejection fraction. These findings suggest that washout rate may be useful to assess the effect of short-term β-blocker therapy in dilated cardiomyopathy patients. (author)

  11. Systems analysis reveals down-regulation of a network of pro-survival miRNAs drives the apoptotic response in dilated cardiomyopathy

    Science.gov (United States)

    Isserlin, Ruth; Merico, Daniele; Wang, Dingyan; Vuckovic, Dajana; Bousette, Nicolas; Gramolini, Anthony O.; Bader, Gary D.; Emili, Andrew

    2016-01-01

    Apoptosis is a hallmark of multiple etiologies of heart failure, including dilated cardiomyopathy. Since microRNAs are master regulators of cardiac development and key effectors of intracellular signaling, they represent novel candidates for understanding the mechanisms driving the increased dysfunction and loss of cardiomyocytes during cardiovascular disease progression. To determine the role of cardiac miRNAs in the apoptotic response, we used microarray technology to monitor miRNA levels in a validated murine phospholambam mutant model of dilated cardiomyopathy. 24 miRNAs were found to be differentially expressed, most of which have not been previously linked to dilated cardiomyopathy. We showed that individual silencing of 7 out of 8 significantly down-regulated miRNAs (mir-1, −29c, −30c, −30d, −149, −486, −499) led to a strong apoptotic phenotype in cell culture, suggesting they repress pro-apoptotic factors. To identify putative miRNA targets most likely relevant to cell death, we computationally integrated transcriptomic, proteomic and functional annotation data. We showed the dependency of prioritized target abundance on miRNA expression using RNA interference and quantitative mass spectrometry. We concluded that down regulation of key pro-survival miRNAs causes up-regulation of apoptotic signaling effectors that contribute to cardiac cell loss, potentially leading to system decompensation and heart failure. PMID:25361207

  12. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  14. 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)

  15. Effect of Atorvastatin vs. Rosuvastatin on cardiac sympathetic nerve activity in non-diabetic patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Effects of statin therapy on cardiac sympathetic nerve activity in patients with chronic heart failure (CHF) have not previously been evaluated. To compare the effects of lipophilic atorvastatin and hydrophilic rosuvastatin on cardiac sympathetic nerve activity in CHF patients with dilated cardiomyopathy (DCM), 63 stable outpatients with DCM, who were already receiving standard therapy for CHF, were randomized to atorvastatin (n=32) or rosuvastatin (n=31). We evaluated cardiac sympathetic nerve activity by cardiac 123I-metaiodobenzylguanidine (MIBG) scintigraphy, hemodynamic parameters and neurohumoral factors before and after 6 months of treatment. There were no differences in the baseline characteristics of the 2 groups. In the rosuvastatin group, there were no changes in MIBG parameters, left ventricular ejection fraction or plasma levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) after 6 months of treatment. In contrast, the atorvastatin group showed a significant increase in the delayed heart/mediastinum count ratio (2.18±0.4 vs. 2.36±0.4, P<0.0001), and the washout rate was significantly decreased (34.8±5.7 vs. 32.6±6.3%, P=0.0001) after 6 months of treatment compared with the baseline values. The plasma NT-proBNP level was also significantly decreased (729±858 vs. 558±747 pg/ml, P=0.0139). Lipophilic atorvastatin but not hydrophilic rosuvastatin improves cardiac sympathetic nerve activity in CHF patients with DCM. (author)

  16. 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 , ...

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. Heart Mitochondrial Proteome Study Elucidates Changes in Cardiac Energy Metabolism and Antioxidant PRDX3 in Human Dilated Cardiomyopathy

    Science.gov (United States)

    Roselló-Lletí, Esther; Tarazón, Estefanía; Barderas, María G.; Ortega, Ana; Otero, Manuel; Molina-Navarro, Maria Micaela; Lago, Francisca; González-Juanatey, Jose Ramón; Salvador, Antonio; Portolés, Manuel; Rivera, Miguel

    2014-01-01

    Background Dilated cardiomyopathy (DCM) is a public health problem with no available curative treatment, and mitochondrial dysfunction plays a critical role in its development. The present study is the first to analyze the mitochondrial proteome in cardiac tissue of patients with DCM to identify potential molecular targets for its therapeutic intervention. Methods and Results 16 left ventricular (LV) samples obtained from explanted human hearts with DCM (n = 8) and control donors (n = 8) were extracted to perform a proteomic approach to investigate the variations in mitochondrial protein expression. The proteome of the samples was analyzed by quantitative differential electrophoresis and Mass Spectrometry. These changes were validated by classical techniques and by novel and precise selected reaction monitoring analysis and RNA sequencing approach increasing the total heart samples up to 25. We found significant alterations in energy metabolism, especially in molecules involved in substrate utilization (ODPA, ETFD, DLDH), energy production (ATPA), other metabolic pathways (AL4A1) and protein synthesis (EFTU), obtaining considerable and specific relationships between the alterations detected in these processes. Importantly, we observed that the antioxidant PRDX3 overexpression is associated with impaired ventricular function. PRDX3 is significantly related to LV end systolic and diastolic diameter (r = 0.73, p value<0.01; r = 0.71, p value<0.01), fractional shortening, and ejection fraction (r = −0.61, p value<0.05; and r = −0.62, p value<0.05, respectively). Conclusion This work could be a pivotal study to gain more knowledge on the cellular mechanisms related to the pathophysiology of this disease and may lead to the development of etiology-specific heart failure therapies. We suggest new molecular targets for therapeutic interventions, something that up to now has been lacking. PMID:25397948

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

    Directory of Open Access Journals (Sweden)

    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.

  2. Heart mitochondrial proteome study elucidates changes in cardiac energy metabolism and antioxidant PRDX3 in human dilated cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Esther Roselló-Lletí

    Full Text Available Dilated cardiomyopathy (DCM is a public health problem with no available curative treatment, and mitochondrial dysfunction plays a critical role in its development. The present study is the first to analyze the mitochondrial proteome in cardiac tissue of patients with DCM to identify potential molecular targets for its therapeutic intervention.16 left ventricular (LV samples obtained from explanted human hearts with DCM (n = 8 and control donors (n = 8 were extracted to perform a proteomic approach to investigate the variations in mitochondrial protein expression. The proteome of the samples was analyzed by quantitative differential electrophoresis and Mass Spectrometry. These changes were validated by classical techniques and by novel and precise selected reaction monitoring analysis and RNA sequencing approach increasing the total heart samples up to 25. We found significant alterations in energy metabolism, especially in molecules involved in substrate utilization (ODPA, ETFD, DLDH, energy production (ATPA, other metabolic pathways (AL4A1 and protein synthesis (EFTU, obtaining considerable and specific relationships between the alterations detected in these processes. Importantly, we observed that the antioxidant PRDX3 overexpression is associated with impaired ventricular function. PRDX3 is significantly related to LV end systolic and diastolic diameter (r = 0.73, p value<0.01; r = 0.71, p value<0.01, fractional shortening, and ejection fraction (r = -0.61, p value<0.05; and r = -0.62, p value<0.05, respectively.This work could be a pivotal study to gain more knowledge on the cellular mechanisms related to the pathophysiology of this disease and may lead to the development of etiology-specific heart failure therapies. We suggest new molecular targets for therapeutic interventions, something that up to now has been lacking.

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

    Science.gov (United States)

    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

  4. A genome-wide association study identifies two loci associated with heart failure due to dilated cardiomyopathy

    Science.gov (United States)

    Villard, Eric; Perret, Claire; Gary, Françoise; Proust, Carole; Dilanian, Gilles; Hengstenberg, Christian; Ruppert, Volker; Arbustini, Eloisa; Wichter, Thomas; Germain, Marine; Dubourg, Olivier; Tavazzi, Luigi; Aumont, Marie-Claude; DeGroote, Pascal; Fauchier, Laurent; Trochu, Jean-Noël; Gibelin, Pierre; Aupetit, Jean-François; Stark, Klaus; Erdmann, Jeanette; Hetzer, Roland; Roberts, Angharad M.; Barton, Paul J.R.; Regitz-Zagrosek, Vera; Aslam, Uzma; Duboscq-Bidot, Laëtitia; Meyborg, Matthias; Maisch, Bernhard; Madeira, Hugo; Waldenström, Anders; Galve, Enrique; Cleland, John G.; Dorent, Richard; Roizes, Gerard; Zeller, Tanja; Blankenberg, Stefan; Goodall, Alison H.; Cook, Stuart; Tregouet, David A.; Tiret, Laurence; Isnard, Richard; Komajda, Michel; Charron, Philippe; Cambien, François

    2011-01-01

    Aims Dilated cardiomyopathy (DCM) is a major cause of heart failure with a high familial recurrence risk. So far, the genetics of DCM remains largely unresolved. We conducted the first genome-wide association study (GWAS) to identify loci contributing to sporadic DCM. Methods and results One thousand one hundred and seventy-nine DCM patients and 1108 controls contributed to the discovery phase. Pools of DNA stratified on disease status, population, age, and gender were constituted and used for testing association of DCM with 517 382 single nucleotide polymorphisms (SNPs). Three DCM-associated SNPs were confirmed by individual genotyping (P < 5.0 10−7), and two of them, rs10927875 and rs2234962, were replicated in independent samples (1165 DCM patients and 1302 controls), with P-values of 0.002 and 0.009, respectively. rs10927875 maps to a region on chromosome 1p36.13 which encompasses several genes among which HSPB7 has been formerly suggested to be implicated in DCM. The second identified locus involves rs2234962, a non-synonymous SNP (c.T757C, p. C151R) located within the sequence of BAG3 on chromosome 10q26. To assess whether coding mutations of BAG3 might cause monogenic forms of the disease, we sequenced BAG3 exons in 168 independent index cases diagnosed with familial DCM and identified four truncating and two missense mutations. Each mutation was heterozygous, present in all genotyped relatives affected by the disease and absent in a control group of 347 healthy individuals, strongly suggesting that these mutations are causing the disease. Conclusion This GWAS identified two loci involved in sporadic DCM, one of them probably implicates BAG3. Our results show that rare mutations in BAG3 contribute to monogenic forms of the disease, while common variant(s) in the same gene are implicated in sporadic DCM. PMID:21459883

  5. 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

  6. Successful delivery by a cesarean section in a parturient with severe dilated cardiomyopathy, an implantable cardioverter defibrillator, and a repaired tetralogy of fallot

    Directory of Open Access Journals (Sweden)

    Rafid Fayadh Al-Aqeedi

    2011-01-01

    Full Text Available Repaired congenital heart disease has become more prevalent in women of childbearing age. We report an unusual case of a 24-year-old multigravida with a repaired tetralogy of Fallot, severe dilated cardiomyopathy, and implantable cardioverter defibrillator placement who was managed successfully by a cesarean section three times. This case underscores the impact of such events on maternal and fetal safety and the importance of a multidisciplinary approach in the management of pregnant patients with complex congenital and medical problems.

  7. Therapeutic effects of coenzyme Q10 on dilated cardiomyopathy. Assessment by 123I-BMIPP myocardial single photon emission computed tomography (SPECT). A multicenter trial in Osaka University Medical School Group

    International Nuclear Information System (INIS)

    To evaluate therapeutic effects of Coenzyme Q10 (CoQ10), 15 patients with dilated cardiomyopathy were investigated by 123I-BMIPP myocardial single photon emission computed tomography (SPECT). The BMIPP defect score was determined semiquantitatively by using representative short and long axial SPECT images. Mean BMIPP defect score with CoQ10 treatment was significantly low, 7.7±6.1 compared to 12.7±7.4 without CoQ10 treatment. On the other hand, in 8 patients of dilated cardiomyopathy, % fractional shortening using echocardiography was not different before and after CoQ10 treatment. In conclusion, 123I-BMIPP myocardial SPECT was proved to be sensitive to evaluate the therapeutic effects of CoQ10, which improve myocardial mitochondrial function, in the cases of dilated cardiomyopathy. (author)

  8. 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’.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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

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

    Directory of Open Access Journals (Sweden)

    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

  12. 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

  13. 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

  14. Association of myocardial inotropic reserve and adrenergic nerve alterations in idiopathic dilated cardiomyopathy. A dobutamine stress echocardiographic and 123-I-MIBG scintigraphic study

    International Nuclear Information System (INIS)

    Aim: Evaluation of contractile reserve is important in congestive hear failure. The aim of this study was to examine the relationship between the myocardial response to dobutamine by stress echocardiography and the sympathetic nerve alterations by 123-I-Metaiodobenzylguanidine (MIBG)scintigraphy and how both contribute to predict exercise capacity in patients with idiopathic dilated cardiomyopathy. Materials-Methods: We studied 20 patients with idiopathic dilated cardiomyopathy (IDC) and ejection fraction < 45% (M/F 13/7, age 56±11 years) while 15 healthy individuals served as controls. Echocardiography and myocardial scintigraphic study with 123-I- MIBG, provided quantitative assessment of left ventricular (LV) wall motion and heart to mediastinum uptake(H/M) ratio and washout. All patients underwent a cardiopulmonary exercise test with a modified Naughton protocol and gas exchange data were analyzed. According to LV response to dobutamine, patients were divided into two groups: those in whom contractility improved in ≥ five segments (Group I: 11 patients) and those in whom contractility improved in < 5 segments (Group II : 9 patients). Results: MIBG uptake was significantly lower in patients than in controls (p < 0.001). MIBG uptake and washout was higher in Group I compared to Group II (P<0,01 and p<0,05). Late MIBG H/M was correlated with resting ejection fraction (r=0,70), wall motion score index (WMSI) (r=-0,50), end systolic wall stress (r=-0,61), washout (r=-0,57), and oxygen consumption at peak exercise (r=0,64) and at anaerobic threshold (r=0,67). LV ejection fraction increased in both groups at Dobutamine, with a higher increase in Group I (p=0,008). WMSI changes at dobutamine correlated significantly with resting ejection fraction (r=0,46) early (r=0,53) and late (r=0,54) MIBG. Multivariate analysis revealed that only the late MIBG uptake was independently associated with the improvement in WMSI. Conclusions: The present data indicate that in

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

  18. [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

  19. [Peripartum cardiomyopathy].

    Science.gov (United States)

    Fennira, S; Demiraj, A; Khouaja, A; Boujnah, M R

    2006-10-01

    Peripartum cardiomyopathy is a rare and under recognized form of dilated cardiomyopathy, defined as a heart failure in the last month of pregnancy or in the first five months post-partum with absence of determinable cause for cardiac failure and absence of demonstrable heart disease. The incidence of peripartum cardiomyopathy ranges from 1 in 1300 to 1 in 15,000 pregnancy. Advanced maternal age, multiparity, twin births, preeclampsia and black race are known risk factors. The etiology of peripartum cardiomyopathy remains unknown but viral, autoimmune or idiopathic myocarditis are highly suggested. The clinical presentation on patients with peripartum cardiomyopathy is similar to that of patients with systolic heart failure. The treatment is based on drugs for sympyomatic control. Studies in graeter populations are need to determine the role of immunosupressive treatment. About half patients of peripartum cardiomyopathy recover. The left ventricular ejection fraction and the left ventricular end-diastolic diameter are statistically significant prognostic factors. The risk of developing peripartum cardiomyopathy in subsequent pregnancies remains high. The place of dobutamine stress test in counseling the patients who desire pregnancy must be more studied. PMID:17078264

  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

    Directory of Open Access Journals (Sweden)

    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

    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

  3. 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

  4. Myocardial {beta}-adrenoceptor downregulation in idiopathic dilated cardiomyopathy measured in vivo with PET using the new radioligand (S)-[{sup 11}C]CGP12388

    Energy Technology Data Exchange (ETDEWEB)

    Jong, Richard M. de; Blanksma, Paul K.; Veldhuisen, Dirk J. van [Groningen University Hospital, Deparment of Cardiology, Groningen (Netherlands); Willemsen, Antoon T.M.; Slart, Riemer H.J.A.; Waarde, Aren van; Vaalburg, Willem; Elsinga, Philip H. [Groningen University Hospital, PET-Center, Groningen (Netherlands); Cornel, Jan Hein [Medical Center, Alkmaar (Netherlands)

    2005-04-01

    The {beta}-adrenoceptor ({beta}-AR) plays an important role in heart failure. Recently, the new tracer (S)-[{sup 11}C]CGP12388 has been developed. It displays excellent properties for investigation of the cardiac {beta}-ARs in vivo with positron emission tomography (PET). Furthermore, the simple production method allows its use in a routine clinical setting. The aim of this study was to investigate whether decreased myocardial {beta}-AR density in patients with idiopathic dilated cardiomyopathy (IDC) can be estimated using (S)-[{sup 11}C]CGP12388 PET. Myocardial {beta}-AR density was investigated in six patients with IDC and six age-matched healthy controls, using (S)-[{sup 11}C]CGP12388 PET. {beta}-AR densities of 5.4{+-}1.3 pmol/g (mean {+-} SD) were observed in patients; these values were significantly lower than those observed in healthy controls (8.4{+-}1.5 pmol/g, p<0.005). This study indicates that PET with (S)-[{sup 11}C]CGP12388 is applicable for the measurement of myocardial {beta}-AR density in patients. A highly significant reduction in {beta}-AR density was found in patients with IDC compared with healthy controls. (orig.)

  5. 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)

  6. 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.

  7. 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

  8. 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.

  9. 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.

  10. 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

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

    International Nuclear Information System (INIS)

    The present study was undertaken to assess whether repeated measurement of cardiac 123I-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)

  12. Use of iodine-123 metaiodobenzylguanidine myocardial imaging to predict the effectiveness of {beta}-blocker therapy in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Fukuoka, Shuji [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Hayashida, Kohei [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Hirose, Yoshiaki [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Shimotsu, Yoriko [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Ishida, Yoshio [Department of Radiology, National Cardiovascular Center, Osaka (Japan); Kakuchi, Hiroyuki [Department of Internal Medicine, National Cardiovascular Center, Osaka (Japan); Eto, Tanenao [First Department of Internal Medicine, Miyazaki Medical College, Miyazaki (Japan)

    1997-05-01

    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 {sup 123}I-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 {sup 123}I-MIBG washout rate (r-WR) in the SPET image, and the global {sup 123}I-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 <5% increase in LVEF (group II, n=6). In normal subjects, the r-WR values in each of the anterior, lateral, septal and inferior segments were significantly lower than those in groups I and II. These values were 18%{+-}9%, 18%{+-}15%, 20%{+-}12% and 21%{+-}15%, respectively. This study demonstrated that with regional assessment {sup 123}I-MIBG SPET imaging can be used to predict the functional improvement of LVEF at 1 month of {beta}-blocker therapy in patients with DCM. (orig./VHE). With 4 figs., 1 tab.

  13. 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.

  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 <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

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

    International Nuclear Information System (INIS)

    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

  16. 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

  17. 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.)

  18. 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 .

  19. The absence of dystrophin brain isoform expression in healthy human heart ventricles explains the pathogenesis of 5' X-linked dilated cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Neri Marcella

    2012-03-01

    Full Text Available Abstract Background In X-linked dilated cardiomyopathy due to dystrophin mutations which abolish the expression of the M isoform (5'-XLDC, the skeletal muscle is spared through the up-regulation of the Brain (B isoform, a compensatory mechanism that does not appear to occur in the heart of affected individuals. Methods We quantitatively studied the expression topography of both B and M isoforms in various human heart regions through in-situ RNA hybridization, Reverse-Transcriptase and Real-Time PCR experiments. We also investigated the methylation profile of the B promoter region in the heart and quantified the B isoform up regulation in the skeletal muscle of two 5'-XLDC patients. Results Unlike the M isoform, consistently detectable in all the heart regions, the B isoform was selectively expressed in atrial cardiomyocytes, but absent in ventricles and in conduction system structures. Although the level of B isoform messenger in the skeletal muscle of 5'-XLDC patients was lower that of the M messenger present in control muscle, it seems sufficient to avoid an overt muscle pathology. This result is consistent with the protein level in XLDC patients muscles we previously quantified. Methylation studies revealed that the B promoter shows an overall low level of methylation at the CG dinucleotides in both atria and ventricles, suggesting a methylation-independent regulation of the B promoter activity. Conclusions The ventricular dilatation seen in 5'-XLDC patients appears to be functionally related to loss of the M isoform, the only isoform transcribed in human ventricles; in contrast, the B isoform is well expressed in heart but confined to the atria. Since the B isoform can functionally replace the M isoform in the skeletal muscle, its expression in the heart could potentially exert the same rescue function. Methylation status does not seem to play a role in the differential B promoter activity in atria and ventricles, which may be governed by

  20. 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)

  1. 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.

  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 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.

  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. Evaluation of therapy for dilated cardiomyopathy with heart failure by iodine-123 metaiodobenzyl-guanidine imaging. Comparison with heart rate variability power spectral analysis

    International Nuclear Information System (INIS)

    The relationship between the myocardial uptake of iodine-123 metaiodobenzylguanidine (123I-MIBG) and heart rate variability parameters has not been determined. This study determined the relationship between the change in myocardial uptake of 123I-MIBG and improvement in left ventricular function after treatment, to determine the usefulness of 123I-MIBG imaging to assess the effect of therapy on heart failure due to dilated cardiomyopathy (DCM). 123I-MIBG imaging and power spectral analysis of heart rate variability were performed before and after treatment in 17 patients with heart failure due to DCM. The following parameters were compared before and after treatment: New York Heart Association (NYHA) functional class, radiographic cardiothoracic ratio (CTR), blood pressure, echocardiographic data (left ventricular end-systolic (LVDs) and end-diastolic (LVDd) diameters, left ventricular ejection fraction (LVEF)), plasma concentrations of norepinephrine and epinephrine, heart rate variability power spectral analysis data (mean low frequency (MLF) and high frequency power (MHF)) and the myocardium to mediastinum activity ratio (MYO/M) obtained in early and late images, and washout rate calculated by anterior planar imaging of 123I-MIBG. The NYHA functional class, LVEF, LVDs, CTR, MLF and MHF improved after treatment. Early MYO/M and late MYO/M improved after treatment. The rate of increase in late MYO/M was positively correlated with the rate of improvement of LVEF after treatment. Furthermore, the late MYO/M was negatively correlated with MLF. Washout rate revealed no correlation with hemodynamic parameters. These findings suggest that late MYO/M is more useful than washout rate to assess the effect of treatment on heart failure due to DCM. Furthermore, the 123I-MIBG imaging and heart rate variability parameters are useful to assess the autonomic tone in DCM with heart failure. (author)

  6. Relationship between evaluation by quantitative fatty acid myocardial scintigraphy and response to {beta}-blockade therapy in patients with dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Tatsuo; Hoshida, Shiro; Nishino, Masami; Aoi, Toshiyuki; Egami, Yasuyuki; Takeda, Toshihiro; Kawabata, Masayoshi; Tanouchi, Jun; Yamada, Yoshio; Kamada, Takenobu [Div. of Cardiology, Osaka Rosai Hospital (Japan)

    2001-12-01

    Predicting the effect of {beta}-blockade therapy on the clinical outcome of patients with dilated cardiomyopathy (DCM) is difficult prior to the initiation of therapy. Myocardial fatty acid metabolism has been shown to be impaired in patients with DCM. We examined whether the extent of myocardial injury, as assessed by iodine-123 15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid (BMIPP) myocardial scintigraphy, is related to the response of patients with DCM to {beta}-blockade therapy. Thirty-seven patients with DCM were examined using BMIPP myocardial scintigraphy before and after 6 months of treatment with metoprolol. Myocardial BMIPP uptake (%BM uptake) was estimated quantitatively as a percentage of the total injected count ratio. The left ventricular end-diastolic and end-systolic dimensions (LVDd, LVDs) and ejection fraction (LVEF) were also evaluated. The patients were divided into two groups according to their functional improvement (>10% elevation of LVEF) after 6 months of metoprolol therapy. Twenty-eight patients responded to the therapy, while nine did not. Prior to the therapy, no significant differences in LVDd, LVDs or LVEF were observed between the responders and non-responders. However, the %BM uptake was significantly lower in the non-responders than in the responders (1.0%{+-}0.2% vs 2.1%{+-}0.5%, P<0.001). The %BM uptake could be used to distinguish the responders from the non-responders with a sensitivity of 0.93 and a specificity of 1.00 at a threshold value of 1.4. After the metoprolol therapy, the %BM uptake improved significantly in the responders (2.5%{+-}0.5%, P<0.01) but did not change in the non-responders. These results indicate that myocardial BMIPP uptake could predict the response of DCM patients to {beta}-blockade therapy. (orig.)

  7. Relationship between evaluation by quantitative fatty acid myocardial scintigraphy and response to β-blockade therapy in patients with dilated cardiomyopathy

    International Nuclear Information System (INIS)

    Predicting the effect of β-blockade therapy on the clinical outcome of patients with dilated cardiomyopathy (DCM) is difficult prior to the initiation of therapy. Myocardial fatty acid metabolism has been shown to be impaired in patients with DCM. We examined whether the extent of myocardial injury, as assessed by iodine-123 15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid (BMIPP) myocardial scintigraphy, is related to the response of patients with DCM to β-blockade therapy. Thirty-seven patients with DCM were examined using BMIPP myocardial scintigraphy before and after 6 months of treatment with metoprolol. Myocardial BMIPP uptake (%BM uptake) was estimated quantitatively as a percentage of the total injected count ratio. The left ventricular end-diastolic and end-systolic dimensions (LVDd, LVDs) and ejection fraction (LVEF) were also evaluated. The patients were divided into two groups according to their functional improvement (>10% elevation of LVEF) after 6 months of metoprolol therapy. Twenty-eight patients responded to the therapy, while nine did not. Prior to the therapy, no significant differences in LVDd, LVDs or LVEF were observed between the responders and non-responders. However, the %BM uptake was significantly lower in the non-responders than in the responders (1.0%±0.2% vs 2.1%±0.5%, P<0.001). The %BM uptake could be used to distinguish the responders from the non-responders with a sensitivity of 0.93 and a specificity of 1.00 at a threshold value of 1.4. After the metoprolol therapy, the %BM uptake improved significantly in the responders (2.5%±0.5%, P<0.01) but did not change in the non-responders. These results indicate that myocardial BMIPP uptake could predict the response of DCM patients to β-blockade therapy. (orig.)

  8. 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.

  9. 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.

  10. A predictive model for canine dilated cardiomyopathy—a meta-analysis of Doberman Pinscher data

    Directory of Open Access Journals (Sweden)

    Siobhan Simpson

    2015-03-01

    Full Text Available Dilated cardiomyopathy is a prevalent and often fatal disease in humans and dogs. Indeed dilated cardiomyopathy is the third most common form of cardiac disease in humans, reported to affect approximately 36 individuals per 100,000 individuals. In dogs, dilated cardiomyopathy is the second most common cardiac disease and is most prevalent in the Irish Wolfhound, Doberman Pinscher and Newfoundland breeds. Dilated cardiomyopathy is characterised by ventricular chamber enlargement and systolic dysfunction which often leads to congestive heart failure. Although multiple human loci have been implicated in the pathogenesis of dilated cardiomyopathy, the identified variants are typically associated with rare monogenic forms of dilated cardiomyopathy. The potential for multigenic interactions contributing to human dilated cardiomyopathy remains poorly understood. Consistent with this, several known human dilated cardiomyopathy loci have been excluded as common causes of canine dilated cardiomyopathy, although canine dilated cardiomyopathy resembles the human disease functionally. This suggests additional genetic factors contribute to the dilated cardiomyopathy phenotype.This study represents a meta-analysis of available canine dilated cardiomyopathy genetic datasets with the goal of determining potential multigenic interactions relating the sex chromosome genotype (XX vs. XY with known dilated cardiomyopathy associated loci on chromosome 5 and the PDK4 gene in the incidence and progression of dilated cardiomyopathy. The results show an interaction between known canine dilated cardiomyopathy loci and an unknown X-linked locus. Our study is the first to test a multigenic contribution to dilated cardiomyopathy and suggest a genetic basis for the known sex-disparity in dilated cardiomyopathy outcomes.

  11. 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

  12. 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...

  13. 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.

  14. The effect of β-blocker on hamster model BIO 53.58 with dilated cardiomyopathy determined using 123I-MIBG myocardial scintigraphy

    International Nuclear Information System (INIS)

    123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy is currently used to evaluate cardiac sympathetic nerve function, but MIBG also has the capacity to evaluate dilated cardiomyopathy (DCM) severity and therapeutic effectiveness. In this study, we administered β-blockers to a DCM hamster model and evaluated the effect of therapy using MIBG. We also pathologically compared the effects of myocardial fibrosis suppression. BIO 53.58 hamsters were divided into the following five groups based on β-blocker administration: vehicle (COT), 2 mg/kg/day carvedilol (CLT), 20 mg/kg/day (CHT) carvedilol, 4 mg/kg/day (MLT) metoprolol, 40 mg/kg/day (MHT) metoprolol. F1B hamsters were administered a vehicle (COF). Plasma catecholamine, noradrenaline (p-NADR), adrenaline (p-ADR), and dopamine (p-DOPA) were assayed, and MIBG was performed. The count ratio of the heart to the mediastinum (H/M) and left ventricle myocardial washout ratio (WR) were calculated. We then performed an autopsy and calculated the percent change in fibrotic area from myocardial sections. H/M of the initial image in the COT group was significantly lower at 2.4±0.2 than the 2.9±0.7 in the COF group (p<0.05). The CLT and CHT groups had higher H/M values compared to the COT group (3.1±0.6, 3.0±0.6 versus 2.4±0.2: p<0.05). Significant correlations were evident between the H/M of the delayed image and p-NADR and p-DOPA (p<0.05, p<0.01, respectively) as well as between WR and p-NADR and p-DOPA (p<0.05). Percent change in fibrotic area was significantly lower in the β-blocker groups than in the COT group (p<0.05). Significant negative correlations were seen between the H/M of the delayed image and the percent change in fibrosis area. The delayed image H/M and WR acutely reflected cardiac disorder and sympathetic nerve function disorder in BIO 53.58 hamsters. In the carvedilol-administered groups, there was improvement compared to the initial H/M image, indicating the efficacy of the β-blocker in DCM

  15. 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

  16. 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

  17. 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

  18. Myocardial scintigraphy using iodine-123 15-(p-Iodophenyl)-3-R, S-methylpentadecanoic acid predicts the response to beta-blocker therapy in patients with dilated cardiomyopathy but does not reflect therapeutic effect

    International Nuclear Information System (INIS)

    Myocardial fatty acid metabolism is disturbed in patients with idiopathic dilated cardiomyopathy. Myocardial scintigraphy using iodine-123 15-(p-iodophenyl)-3-R, S-methylpentadecanoic acid (BMIPP) was used to assess the response to β-blocker therapy in 19 patients with dilated cardiomyopathy. BMIPP myocardial scintigraphy was performed before and 6 months after initiating β-blocker therapy with metoprolol. Cardiac BMIPP uptake was assessed as the total defect score (TDS) and heart-to-mediastinum activity (H/M) ratio. Patients were classified retrospectively as responders with an improvement of at least one functional class (New York Heart Association) or an increase in ejection fraction of ≥0.10 at 6 months, or as nonresponders meeting neither criterion. Responders had a significantly better pretreatment TDS (p<0.005) and H/M ratio (p<0.0001) than nonresponders. TDS exhibited no significant changes over 6 months in either group (responders: 13.2±3.7 vs 12.5±3.3; nonresponders: 20.8±6.5 vs 20.5±3.0). Responders showed no significant changes in H/M ratio (2.47±0.28 vs 2.43±0.42); paradoxically, nonresponders showed a significant increase from 1.82±0.11 to 2.10±0.19 (p<0.05), suggesting that β-blocker therapy protected the myocardial fatty acid metabolism even in the absence of clinical improvement. BMIPP myocardial scintigraphy provides a prediction of response to β-blocker treatment, but does not reflect the therapeutic effect in responders at 6 months. (author)

  19. Myocardial scintigraphy using iodine-123 15-(p-Iodophenyl)-3-R, S-methylpentadecanoic acid predicts the response to beta-blocker therapy in patients with dilated cardiomyopathy but does not reflect therapeutic effect

    Energy Technology Data Exchange (ETDEWEB)

    Yoshinaga, Keiichiro; Tahara, Minoru; Torii, Hiroyuki; Akimoto, Masaki [Kagoshima City Medical Association Hopital (Japan); Kihara, Koichi; Tei, Chuwa

    2000-05-01

    Myocardial fatty acid metabolism is disturbed in patients with idiopathic dilated cardiomyopathy. Myocardial scintigraphy using iodine-123 15-(p-iodophenyl)-3-R, S-methylpentadecanoic acid (BMIPP) was used to assess the response to {beta}-blocker therapy in 19 patients with dilated cardiomyopathy. BMIPP myocardial scintigraphy was performed before and 6 months after initiating {beta}-blocker therapy with metoprolol. Cardiac BMIPP uptake was assessed as the total defect score (TDS) and heart-to-mediastinum activity (H/M) ratio. Patients were classified retrospectively as responders with an improvement of at least one functional class (New York Heart Association) or an increase in ejection fraction of {>=}0.10 at 6 months, or as nonresponders meeting neither criterion. Responders had a significantly better pretreatment TDS (p<0.005) and H/M ratio (p<0.0001) than nonresponders. TDS exhibited no significant changes over 6 months in either group (responders: 13.2{+-}3.7 vs 12.5{+-}3.3; nonresponders: 20.8{+-}6.5 vs 20.5{+-}3.0). Responders showed no significant changes in H/M ratio (2.47{+-}0.28 vs 2.43{+-}0.42); paradoxically, nonresponders showed a significant increase from 1.82{+-}0.11 to 2.10{+-}0.19 (p<0.05), suggesting that {beta}-blocker therapy protected the myocardial fatty acid metabolism even in the absence of clinical improvement. BMIPP myocardial scintigraphy provides a prediction of response to {beta}-blocker treatment, but does not reflect the therapeutic effect in responders at 6 months. (author)

  20. Abstention from alcohol in dilated cardiomyopathy: Complete regression of the clinical disease but persistence of myocardial perfusion defects on exercise thallium-201 tomography

    International Nuclear Information System (INIS)

    This case report describes a 43-year-old man with dilated cadiomyopathy reversed by abstention from alcohol over 1 year but with persistence of previous myocardial perfusion defects on exercise thallium-201 tomography. This suggests that despite the near normalization of left ventricular function, a permanent myocardial disease seems to persist. (orig.)

  1. 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 ...

  2. 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

  3. 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。

  4. 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.

  5. 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.

  6. 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

  7. 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

  8. 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

  9. 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.

  10. Pediatric Cardiomyopathies

    Science.gov (United States)

    ... Pressure High Blood Pressure Tools & Resources Stroke More Pediatric Cardiomyopathies Updated:Oct 22,2015 Patient education material ... oxygen or high blood pressure. According to the Pediatric Cardiomyopathy Registry, one in every 100,000 children ...

  11. 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.

  12. O impacto da desnutrição na cardiomiopatia dilatada idiopática na infância The impact of malnutrition on idiopathic dilated cardiomyopathy in children

    Directory of Open Access Journals (Sweden)

    Vitor M. P. Azevedo

    2004-06-01

    Full Text Available OBJETIVO: Determinar a importância da desnutrição como marcadora e preditora do óbito na cardiomiopatia dilatada idiopática na infância. MÉTODOS: Este é um estudo retrospectivo envolvendo 165 pacientes (setembro de 1979 a março de 2003. As variáveis analisadas foram sexo, idade e história de infecção viral nos últimos 3 meses, classe funcional da New York Heart Association (NYHA, percentil e desvio padrão do peso (índice z e avaliação do estado nutricional. Foram realizadas 744 pesagens nos primeiros 72 meses e 93 no primeiro mês de evolução. Análise estatística: qui-quadrado, teste t de Student e análise de variância. Foram utilizados o valor alfa de 0,05 e o valor beta de 0,80. RESULTADOS: A idade no diagnóstico foi de 2,1±3,2 anos, com maior incidência nos menores de 2 anos (75,8%; IC95 = 68,5-82,1% (p OBJECTIVE: To analyze the prognostic value of malnutrition in children with idiopathic dilated cardiomyopathy. METHODS: This is a retrospective study of 165 patients with idiopathic dilated cardiomyopathy, diagnosed from September 1979 to March 2003. It analyzed the following variables: gender, age, previous viral illness in the preceding 3 months, functional class according to the New York Heart Association (NYHA, evaluation of nutritional status (normal vs malnutrition, percentile and standard deviation (z index of weight. Weight was measured 744 times during the first 72 months, 93 during the first month. Statistical analysis was performed by chi-squared, Student t test and analysis of variance for repeated measures (ANOVA. Ninety-five percent confidence intervals (CI95 and odds ratios (OR were calculated. An alpha value of 0.05 and beta of 0.80 were used. RESULTS: Mean age at presentation was 2.2±3.2 years with higher incidence in those younger than 2 years (75.8%-CI95 = 68.5% to 82.1% (p < 0.0001. NYHA classes III and IV were observed in 81.2% (CI95 = 74.4% to 86.9% (p < 0.0001 and all 40 deaths were this group

  13. 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.)

  14. In vitro evaluation of mitochondrial dysfunction and treatment with adeno-associated virus vector in fibroblasts from Doberman Pinschers with dilated cardiomyopathy and a pyruvate dehydrogenase kinase 4 mutation.

    Science.gov (United States)

    Sosa, Ivan; Estrada, Amara H; Winter, Brandy D; Erger, Kirsten E; Conlon, Thomas J

    2016-02-01

    OBJECTIVE To compare mitochondrial oxygen consumption rate (OCR) of fibroblasts from Doberman Pinschers with and without dilated cardiomyopathy (DCM) and mutation of the gene for pyruvate dehydrogenase kinase isozyme 4 (PDK4) and to evaluate in vitro whether treatment with adeno-associated virus (AAV) vector (ie, gene therapy) would alter metabolic efficiency. ANIMALS 10 Doberman Pinschers screened for DCM and PDK4 mutation. PROCEDURES Fibroblasts were harvested from skin biopsy specimens obtained from Doberman Pinschers, and dogs were classified as without DCM or PDK4 mutation (n = 3) or with occult DCM and heterozygous (4) or homozygous (3) for PDK4 mutation. Fibroblasts were or were not treated with tyrosine mutant AAV type 2 vector containing PDK4 at multiplicities of infection of 1,000. Mitochondrial OCR was measured to evaluate mitochondrial metabolism. The OCR was compared among dog groups and between untreated and treated fibroblasts within groups. RESULTS Mean ± SD basal OCR of fibroblasts from heterozygous (74 ± 8 pmol of O2/min) and homozygous (58 ± 12 pmol of O2/min) dogs was significantly lower than that for dogs without PDK4 mutation (115 ± 9 pmol of O2/min). After AAV transduction, OCR did not increase significantly in any group (mutation-free group, 121 ± 26 pmol of O2/min; heterozygous group, 88 ± 6 pmol of O2/min; homozygous group, 59 ± 3 pmol of O2/min). CONCLUSIONS AND CLINICAL RELEVANCE Mitochondrial function was altered in skin fibroblasts of Doberman Pinschers with DCM and PDK4 mutation. Change in mitochondrial function after in vitro gene therapy at the multiplicities of infection used in this study was not significant. (Am J Vet Res 2016;77:156-161). PMID:27027709

  15. 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

  16. 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.

  17. 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

  18. An autopsied case of cardiomyopathy demonstrated specific findings by thallium-201 myocardial scintigraphy

    International Nuclear Information System (INIS)

    A few cases showing dilatation and decreased contraction of the left ventricle at the terminal stage of hypertrophic cardiomyopathy (dilated-HCM) have been reported. Recently, we experienced a case of dilated-HCM and compared thallium-201 myocardial scintigraphic findings with histological findings. Thallium-201 myocardial scintigraphic findings resembled that of dilated cardiomyopathy (dilated biventricle and large perfusion defect). But, histological findings revealed large fibrosis and disarray of myocardial cells. These observation from thallium-201 myocardial scintigraphy was useful for the assessment of myocardial fibrosis of hypertrophic cardiomyopathy. (author)

  19. 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

  20. 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)

  1. Importance of transesophageal echocardiography in peripartum cardiomyopathy undergoing lower section cesarean section under regional anesthesia

    OpenAIRE

    Kapoor, Poonam Malhotra; Goyal, Sameer; Irpachi, Kalpana; Smita, Barya

    2014-01-01

    Peripartum cardiomyopathy is a relatively rare but life threatening disease. The etiology and pathogenesis of peripartum cardiomyopathy is generally centered upon viral and autoimmune mechanism. This case report describes the anesthetic management of a patient with term pregnancy suffering from dilated peripartum cardiomyopathy planned for cesarean section, successfully managed with epidural anesthesia after precipitate labour.

  2. Indium-111 antimyosin monoclonal antibody uptake in patients with cardiomyopathy and myocarditis

    International Nuclear Information System (INIS)

    Prognostic significance of myocardial uptake of indium-111 antimyosin antibody was evaluated in 17 patients with idiopathic cardiomyopathy; 10 patients with dilated cardiomyopathy and 7 patients with hypertrophic cardiomyopathy. Seven of 10 patients with dilated cardiomyopathy showed positive images. Three of these 7 patients with strongly positive scans died after scintigraphic examination. Six of 7 patients with hypertrophic cardiomyopathy showed positive images. Three of the patients with dilated left ventricle had prominent positive scans and higher heart to lung ratio. The heart to lung ratio of antimyosin uptake in total patients was correlated with left ventricular end-diastolic dimension and ejection fraction measured by echocardiography. In patients with myocarditis, all three patients showed positive scintigrams within 4 weeks after the onset of the disease and 1 of 6 patients was positive thereafter, who had dilated ventricle and decreased cardiac function. Thus, indium-111 antimyosin antibody imaging may be useful to evaluate prognosis of patients with cardiomyopathy and myocarditis. (author)

  3. 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. ...

  4. Insights into restrictive cardiomyopathy from clinical and animal studies

    OpenAIRE

    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...

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

    International Nuclear Information System (INIS)

    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. (author)

  6. 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.

  7. 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 ...

  8. 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

  9. 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

  10. 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

  11. 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...

  12. The diagnostic value of cardiac blood pool imaging in cardiomyopathy

    International Nuclear Information System (INIS)

    Twenty-two normal controls and thirty-three patients with dilated and hypertrophic cardiomyopathy were examined by multigated cardiac imaging and phase analysis. The imaging traits of hypertrophic ones displayed thicken septal wall. The differences of synchronization of ventricular systolic function between hypertrophic ones and normal were statistically significant (P < 0.01). The imaging features of dilated cardiomyopathy showed that ventricular wall motion was seriously hypokinitic or akinitic and left ventricule enlarged abnormally. The function and synchronization of ventricular systole in dilated ones were worse than these in the hypertrophic (P < 0.01). These data suggest that the cardiac blood pool images can diagnose and differentiate the hypertrophic from dilated cardiomyopathy

  13. Paschke Dilations

    OpenAIRE

    Westerbaan, Abraham; Westerbaan, Bas

    2016-01-01

    In 1973 Paschke defined a factorization for completely positive maps between C*-algebras. In this paper we show that for normal maps between von Neumann algebras, this factorization has a universal property, and coincides with Stinespring's dilation for normal maps into B(H ).

  14. 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

  15. [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...

  16. 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

  17. 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

  18. 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.

  19. 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

  20. 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...

  1. Magnetic Resonance Imaging of cardiomyopathy

    International Nuclear Information System (INIS)

    MRI has been proposed as a noninvasive method for evaluating patients with a variety of cardiomyopathies. Arrhythmogenic right ventricular dysplasia (ARVD) is defined as a primary disorder of the right ventricle characterized by partial or total replacement of muscle by adipose or fibrous tissue. Its diagnosis currently rests on techniques that accurately identify specific anatomic and functional abnormalities of the right ventricle. The aim of our study was to analyze the MRI features in 41 patients (age 19-64 years; 26 patients with ARVD, 7 patients with hypertrophic cardiomyopathy (HCM) and 8 patients with dilated cardiomyopathy (DCM)). They were investigated by spin-echo (SE) and gradient-echo (GE) Cine using a 0.5T unit Toshiba 50A with cardiac software. SE and Cine MRI allowed assessment of localization (hence type of HC) and extent of changes in visualized wall and ventricular dysfunction. In all patients with ARVD MRI SE-T1 W allowed localization of the abnormally bright signal intensity from the RV-free wall, apex and subtricuspid region, characteristic for the subcutenous fat. In 8 patients the structural changes were also present in left ventricular myocardium. MRI can be a useful method in supplying additional information to that obtained in 2D Echo by identifying the site, extent and type of cardiomyopathy. MRI is a very useful non-invasive method in diagnosis of ARVD. (author)

  2. 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.

  3. 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.

  4. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

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

    2014-01-01

    Cirrhosis is known to cause alterations in the systemic haemodynamic system. Cirrhotic cardiomyopathy designates a cardiac dysfunction that includes impaired cardiac contractility with systolic and diastolic dysfunction, as well as electromechanical abnormalities in the absence of other known...... causes of cardiac disease. This condition is primarily revealed by inducing physical or pharmacological stress, but echocardiography is excellent at revealing diastolic dysfunction and might also be used to detect systolic dysfunction at rest. Furthermore, measurement of circulating levels of cardiac...... 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....

  5. 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...

  6. Primary antiphospholipid syndrome, hypertrophic non-obstructive cardiomyopathy and hypotelorism.

    Science.gov (United States)

    Kellermair, Joerg; Kammler, Juergen; Laubichler, Peter; Steinwender, Clemens

    2016-01-01

    Antiphospholipid syndrome (APS) is an autoimmune disorder associated with arterial/venous thrombosis. Cardiac manifestations of APS include valve stenosis/insufficiency, coronary artery disease and myocardial dysfunction presenting as dilated cardiomyopathy. In the following report, we present the case of a man with primary APS, hypertrophic non-obstructive cardiomyopathy and hypotelorism-a combination that has not yet been reported in the literature. PMID:27048398

  7. 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.

  8. 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 ( ...

  9. MRI of restrictive cardiomyopathy

    International Nuclear Information System (INIS)

    Objective: To evaluate the diagnostic value of MRI in combination of delayed gadolinium-enhanced MR imaging for the identification of restrictive cardiomyopathy (RCM). Methods: One hundred sixteen patients with RCM underwent ECG, thoracic radiography, echocardiography and MRI. The final diagnosis was made on comprehensive evaluation in consideration of patient history, clinical symptoms and imaging appearances. Fifty-five normal subjects were used as the controls. All patients were divided into two groups according to contrast-enhanced MRI patterns: RCM with delayed enhancement (RCM with DE, n=35) and RCM without delayed enhancement (RCM without DE, n=81). Bi-atrial and bi-ventricular size, ventricular septal and left free wall thickness were measured. A paired t-test was used for statistic analysis. Results: Bi-atrial size, right ventricular diastolic diameter (RVDD), ventricular septal and left free wall thickness were significantly larger in RCM patients than in normal subjects (P0.05). Visual observation showed mild mitral regurgitation (50 cases), moderate mitral regurgitation (24 cases ), mild tricuspid regurgitation (32 cases) and severe tricuspid regurgitation (46 cases). Thirty-five RCMs with DE presented diffuse (15 cases) or segmental (20 cases) enhancement. Twelve RCMs with diffuse delayed enhancement showed powdery, enhancement, and 3 showed petaline enhancement. Three cases with powdery enhancement were histologically proven as myocardial amyloidosis. Ventricular septum was the most vulnerable segment in patients with segmental enhancement. Six cases presented subendocardial enhancement that corresponded to apical obliteration, of which one case was confirmed as hypereosinophilia by bone marrow biopsy and the other 14 cases didn't present any regular enhancement. In 81 RCMs without DE, marked bi-atrial dilation, near-normal ventricular chambers and near-normal ventricular thickness were presented. Conclusion: MRI is an excellent imaging modality for

  10. 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

  11. 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 ...

  12. Cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Søren; Henriksen, Jens H

    2010-01-01

    Increased cardiac output was first described in patients with cirrhosis more than fifty years ago. Later, various observations have indicated the presence of a latent cardiac dysfunction, which includes a combination of reduced cardiac contractility with systolic and diastolic dysfunction and......, nitric oxide overproduction, and cannabinoid receptor activation. Systolic incompetence in patients can be revealed by pharmacological or physical strain and during stressful procedures, such as transjugular intrahepatic portosystemic shunt insertion and liver transplantation. Systolic dysfunction has...... cirrhotic patients and it may be normalised by beta-blockers. No specific therapy for cirrhotic cardiomyopathy can be recommended, but treatment should be supportive and directed against the cardiac dysfunction. Future research should better describe the prevalence, impact on morbidity and survival, and...

  13. Decreased Levels of BAG3 in a Family with a Rare Variant and in Idiopathic Dilated Cardiomyopathy†

    OpenAIRE

    Feldman, Arthur M.; Begay, Rene L.; Knezevic, Tijana; Myers, Valerie D.; Slavov, Dobromir B.; Zhu, Weizhong; Gowan, Katherine; Graw, Sharon L.; Jones, Kenneth L.; Tilley, Douglas G.; Coleman, Ryan C.; Walinsky, Paul; Cheung, Joseph Y.; Mestroni, Luisa; Khalili, Kamel

    2014-01-01

    The most common cause of dilated cardiomyopathy and heart failure (HF) is ischemic heart disease; however, in a third of all patients the cause remains undefined and patients are diagnosed as having idiopathic dilated cardiomyopathy (IDC). Recent studies suggest that many patients with IDC have a family history of HF and rare genetic variants in over 35 genes have been shown to be causative of disease. We employed whole-exome sequencing to identify the causative variant in a large family with...

  14. Metabolic imaging of patients with cardiomyopathy

    International Nuclear Information System (INIS)

    The cardiomyopathies comprise a diverse group of illnesses that can be characterized functionally by several techniques. However, the delineation of derangements of regional perfusion and metabolism have been accomplished only relatively recently with positron emission tomography (PET). Regional myocardial accumulation and clearance of 11C-palmitate, the primary myocardial substrate under most conditions, demonstrate marked spatial heterogeneity when studied under fasting conditions or with glucose loading. PET with 11C-palmitate permits the noninvasive differentiation of patients with nonischemic from ischemic dilated cardiomyopathy, since patients with ischemic cardiomyopathy demonstrate large zones of intensely depressed accumulation of 11C-palmitate, probably reflecting prior infarction. Patients with hypertrophic cardiomyopathy and Duchenne's muscular dystrophy demonstrate relatively unique patterns of myocardial abnormalities of perfusion and metabolism. The availability of new tracers and techniques for the evaluation of myocardial metabolism (11C-acetate), perfusion (H2(15)O), and autonomic tone (11-C-hydroxyephedrine) should facilitate further understanding of the pathogenesis of the cardiomyopathies

  15. 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.

  16. 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. )

  17. 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 ...

  18. Athletes’ Stress Cardiomyopathy

    OpenAIRE

    E. A. Gavrilova

    2012-01-01

    The article is focused on the examination of 1352 male athletes, engaged in different sports, actively training and participating in competitions. Stress cardiomyopathy was exposed in 14.8 % of cases. The article considers differential diagnostics of stress cardiomyopathy.

  19. Indium-111 antimyosin monoclonal antibody Fab imaging in patients with cardiomyopathy

    International Nuclear Information System (INIS)

    Six patients with cardiomyopathy were imaged following intravenous injection of an indium-111 labeled monoclonal antibody directed against the heavy chain of cardiac myosin. Two patients had hypertrophic non-obstructive cardiomyopathy (HNCM), two patients had dilated cardiomyopathy (DCM), and two patients had specific heart muscle disease. One of 2 patients with HNCM and one of 2 patients with DCM had a positive antimyosin scan. The 2 patients with specific heart muscle disease manifested persistent blood pool activity of the antibody, thereby precluding interpretation of the images. The present report demonstrates that antimyosin antibody imaging may provide evidence of myocardial injury, or necrosis in some patients with cardiomyopathy. (author)

  20. A Systematic Review of Phenotypic Features Associated With Cardiac Troponin I Mutations in Hereditary Cardiomyopathies

    DEFF Research Database (Denmark)

    Mogensen, Jens; Hey, Thomas; Lambrecht, Sascha

    2015-01-01

    BACKGROUND: Genetic investigations have established that mutations in proteins of the contractile unit of the myocardium, known as the sarcomere, may be associated with hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), and dilated cardiomyopathy (DCM). It has become clinical...... in relation to phenotypic features reported to be associated with mutations in cardiac troponin I (cTnI; TNNI3), which is a recognized sarcomeric disease gene in all 3 cardiomyopathies. RESULTS: The results of this review did not identify specific genotype-phenotype relations in HCM or DCM, and c...

  1. 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

  2. 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)

  3. 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)

  4. 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.

  5. 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

  6. 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

  7. 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

  8. 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.

  9. 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.

  10. 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.

  11. 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.

  12. EBCT in diagnosis on primary cardiomyopathy

    International Nuclear Information System (INIS)

    Objective: To evaluate the diagnostic value of electron beam CT (EBCT) in primary cardiomyopathy (PCM). Methods: EBCT including coronary scanning and heart movie study was performed in 15 patients. The diagnosis of PCM was established by clinical and imaging findings or/and pathologic examination. Results: BECT findings related to the classification of PCM drawn up by WHO/ISFC were as follows: (1) dilated PCM (8 cases): dilated left ventricle (LV) was found in 7 of 8 cases and dilated right ventricle (RV) in the remaining one. Hypokinetic contraction of the LV wall with (250 ± 101) ml LVEDV and (18.9 ±6.6)% LVEF presented in the patients with dilated LV. (2) Hypertrophic PCM (5 cases): Hypertrophic ventricular septum (16.5 ± 2.3) mm was demonstrated in all cases, hypertrophy of the LV wall or the apex in 3 of 5 cases, and stenosis of the LV outflow tract in 2 cases. (3) Restrictive PCM (2 cases): Thickening of endocardium with lower density than cardiac muscle, reduced RV, obliteration of apex, hypokinetic contraction of the RV wall, and dilatation of right atrium (RA) with parietal thrombus formation of RA was identified in one patient. Both the LV and the RV were involved, with enlargement of atrium. Conclusion: The findings of PCM on EBCT seem to reflect pathologic and functional changes of PCM, and may be useful for diagnosis and classification of PCM

  13. 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)

  14. Comparison of myocardial thallium and β-methyl iodophenyl pentadecanoic acid (BMIPP) distribution in cardiomyopathy hamster

    International Nuclear Information System (INIS)

    The usefulness of fatty acid imaging in the detection of cardiomyopathy was evaluated by comparing thallium and BMIPP myocardial distribution in Bio 14.6 Syrian Hamster (25 week ages). Autoradiography was performed in 3 using 3.7 MBq (100 μCi) of 125I-BMIPP and 37 MBq (1 mCi) of 201TlCl. In vivo pin-hole imaging was performed in 3 using 37 MBq (1 mCi) of 123I-BMIPP and 37 MBq (1 mCi) of 201TlCl. In all cardiomyopathy hamsters, decreased uptake of BMIPP compared to that of thallium was demonstrated. These findings suggest dilated cardiomyopathy is associated with severe focal alternation in the substrate used for the performance of myocardial work. In conclusion, myocardial imaging using BMIPP may be useful for early detection of myocardial degeneration compared to thallium in patients with dilated cardiomyopathy. (author)

  15. 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

  16. 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...

  17. 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

  18. [Dilated cardiomyopathy and visceral anomalies in myotonic dystrophy].

    Science.gov (United States)

    Pentimone, F; Del Corso, L; Vannini, A; Mori, L; Moruzzo, D

    1990-05-01

    In dystrophia myotonica clinical evidence of cardiac involvement usually appears several years after the onset of neuromuscular symptoms. In more than 90% of cases there is damage to the specialized cardiac tissues and in about 7% of cases there are alterations to the myocardium. We report a case characterized by early and spread deterioration of the pump function developing into refractory congestive heart failure. The contemporary involvement of the smooth muscle of gallbladder and colon confirms the hypothesis that dystrophia myotonica is a pan-muscle disease. PMID:2234457

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

    OpenAIRE

    Helena Furtado Martino; Paulo Sérgio Oliveira; Edmilson Assunção; Rita Villela; Miriam Gaze; Patrícia C. dos Santos Costa; Fernando César Castro Souza; Luiz Henrique Weitzel; Ana Paula R. Velloso; Amarino Oliveira Júnior; Antônio C. Campos de Carvalho

    2006-01-01

    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 di...

  20. 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.

  1. 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 ...

  2. Review of cardiomyopathy imaging

    International Nuclear Information System (INIS)

    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

  3. Rapidly Progressing Chagas Cardiomyopathy.

    Science.gov (United States)

    Hollowed, John; McCullough, Matthew; Sanchez, Daniel; Traina, Mahmoud; Hernandez, Salvador; Murillo, Efrain

    2016-04-01

    Chagas disease, caused by the parasiteTrypanosoma cruzi, can cause a potentially life-threatening cardiomyopathy in approximately 10-40% of afflicted individuals. The decline in cardiac function characteristically progresses over the course of many years. We report a case of Chagas disease in which the patient experienced an atypical rapid deterioration to severe cardiomyopathy over the course of 16 months. This case argues the need for increased routine surveillance for patients with confirmedT. cruziinfection, who are determined to be at high-risk for worsening cardiomyopathy. PMID:26856912

  4. 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.

  5. 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。

  6. Mutation Glu82Lys in lamin A/C gene is associated with cardiomyopathy and conduction defect

    International Nuclear Information System (INIS)

    Dilated cardiomyopathy is a form of heart muscle disease characterized by impaired systolic function and ventricular dilation. The mutations in lamin A/C gene have been linked to dilated cardiomyopathy. We screened genetic mutations in a large Chinese family of 50 members including members with dilated cardiomyopathy and found a Glu82Lys substitution mutation in the rod domain of the lamin A/C protein in eight family members, three of them have been diagnosed as dilated cardiomyopathy, one presented with heart dilation. The pathogenic mechanism of lamin A/C gene defect is poorly understood. Glu82Lys mutated lamin A/C and wild type protein was transfected into HEK293 cells. The mutated protein was not properly localized at the inner nuclear membrane and the emerin protein, which interacts with lamin A/C, was also aberrantly distributed. The nuclear membrane structure was disrupted and heterochromatin was aggregated aberrantly in the nucleus of the HEK293 cells stably transfected with mutated lamin A/C gene as determined by transmission electron microscopy

  7. 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.)

  8. 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

  9. Influence of respiration in the very low frequency modulation of QRS slopes and heart rate variability in cardiomyopathy patients

    OpenAIRE

    Hernando, David; Alcaine, A; Pueyo, Esther; Laguna, Pablo; Arcentales, Andrés; Giraldo Giraldo, Beatriz; Voss, Andreas; Bayés-Genís, Antoni; Bailón, Raquel

    2013-01-01

    This work investigates the very low frequency (VLF) modulation of QRS slopes and heart rate variability (HRV). Electrocardiogram (ECG) and respiratory flow signal were acquired from patients with dilated cardiomyopathy and ischemic cardiomyopathy. HRV as well as the upward QRS slope (IUS) and downward QRS slope (IDS) were extracted from the ECG. The relation between HRV and QRS slopes in the VLF band was measured using ordinary coherence in 5-minute segments. Partial coherence was then use...

  10. Takotsubo cardiomyopathy following subarachnoid hemorrhage

    International Nuclear Information System (INIS)

    Takotsubo cardiomyopathy corresponds to a syndrome characterized by a transient myocardial dysfunction affecting the left ventricular apex that classically occurs after major physical or emotional stress (also called 'broken heart syndrome' or 'stress-induced cardiomyopathy'). The author describes the case of a patient with takotsubo cardiomyopathy induced by subarachnoid hemorrhage. (author)

  11. Exercise Rehabilitation in Pediatric Cardiomyopathy

    OpenAIRE

    Somarriba, Gabriel; Extein, Jason; Miller, Tracie L.

    2008-01-01

    Children with cardiomyopathy carry significant risk of morbidity and mortality. New research and technology have brought about significant advancements to the diagnosis and clinical management of children with cardiomyopathy. However, currently heart transplantation remains the standard of care for children with symptomatic and progressive cardiomyopathy. Cardiovascular rehabilitation programs have yielded success in improving cardiac function, overall physical activity, and quality of life i...

  12. 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.

  13. 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.

  14. Assessment of cardiac function by radionuclide angiocardiography in children with cardiomyopathy

    International Nuclear Information System (INIS)

    Radionuclide angiography was performed in four children, each having dilated cardiomyopathy (DCM), hypertrophic nonobstructive cardiomyopathy (HCM), hypertrophic obstructive cardiomyopathy (HOCM), or restrictive cardiomyopathy (RCM), whose ages ranged from 5 to 8 years. The peak to peak time of flow from the right to left ventricle, which was corrected by heart rate, was prolonged in all the patients. Left ventricular ejection fraction was low in the DCM patient, and high in the HOCM patient. In the DCM patient, both peak ejection rate and peak filling rate of the left ventricle were low. Two patients with either DCM or HOCM had the prolongation of percent duration to peak filling rate, as opposed to the RCM patient having short percent duration. In the RCM patient, right ventricular ejection fraction was low, and time-volume curve showed rapid filling during early diastole. Radionuclide angiography is recommended as a noninvasive method of evaluating cardiac functional characteristics for various types of myocardiopathy. (Namekawa, K.)

  15. Commutative dilation theory

    Czech Academy of Sciences Publication Activity Database

    Ambrozie, Calin-Grigore; Müller, Vladimír

    Basel: Springer, 2015 - (Alpay, D.), s. 1093-1124 ISBN 978-3-0348-0666-4 Institutional support: RVO:67985840 Keywords : commuting multioperator * dilation * von Neumann inequality Subject RIV: BA - General Mathematics http://link.springer.com/referenceworkentry/10.1007%2F978-3-0348-0667-1_58

  16. 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

  17. 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 ...

  18. 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& ...

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

    Science.gov (United States)

    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; Regitz-Zagrosek, Vera; Hacker, Timothy A.; Saupe, Kurt W.; Dec, G. William; Ellinor, Patrick T.; MacRae, Calum A.; Spallek, Bastian; Fischer, Robert; Perrot, Andreas; Özcelik, Cemil; Saar, Kathrin; Hubner, Norbert; Gotthardt, Michael

    2013-01-01

    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 rats resembles the human pathology. Deep sequencing of the human and rat cardiac transcriptome revealed an RBM20 dependent regulation of alternative splicing. Additionally to titin we identified a set of 30 genes with conserved regulation between human and rat. This network is enriched for genes previously linked to cardiomyopathy, ion-homeostasis, and sarcomere biology. Our studies emphasize the importance of posttranscriptional regulation in cardiac function and provide mechanistic insights into the pathogenesis of human heart failure. PMID:22466703

  20. Mutations in NEBL encoding the cardiac Z-disk protein nebulette are associated with various cardiomyopathies

    Science.gov (United States)

    Tomasov, Pavol; Villard, Eric; Faludi, Reka; Melacini, Paola; Lossie, Janine; Lohmann, Nadine; Richard, Pascale; De Bortoli, Marzia; Angelini, Annalisa; Varga-Szemes, Akos; Sperling, Silke R.; Simor, Tamás; Veselka, Josef; Özcelik, Cemil; Charron, Philippe

    2016-01-01

    Introduction Transgenic mice overexpressing mutated NEBL, encoding the cardiac-specific Z-disk protein nebulette, develop severe cardiac phenotypes. Since cardiomyopathies are commonly familial and because mutations in a single gene may result in variable phenotypes, we tested the hypothesis that NEBL mutations are associated with cardiomyopathy. Material and methods We analyzed 389 patients, including cohorts of patients with dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), and left ventricular non-compaction cardiomyopathy (LVNC). The 28 coding exons of the NEBL gene were sequenced. Further bioinformatic analysis was used to distinguish variants. Results In total, we identified six very rare heterozygous missense mutations in NEBL in 7 different patients (frequency 1.8%) in highly conserved codons. The mutations were not detectable in 320 Caucasian sex-matched unrelated individuals without cardiomyopathy and 192 Caucasian sex-matched blood donors without heart disease. Known cardiomyopathy genes were excluded in these patients. The mutations p.H171R and p.I652L were found in 2 HCM patients. Further, p.Q581R and p.S747L were detected in 2 DCM patients, while the mutation p.A175T was identified independently in two unrelated patients with DCM. One LVNC patient carried the mutation p.P916L. All HCM and DCM related mutations were located in the nebulin-like repeats, domains responsible for actin binding. Interestingly, the mutation associated with LVNC was located in the C-terminal serine-rich linker region. Conclusions Our data suggest that NEBL mutations may cause various cardiomyopathies. We herein describe the first NEBL mutations in HCM and LVNC. Our findings underline the notion that the cardiomyopathies are true allelic diseases.

  1. Assessment of myocardial damage in cardiomyopathy using 111In-antimyosin Fab myocardial scintigraphy

    International Nuclear Information System (INIS)

    111In-antimyosin Fab (AM) myocardial scintigraphy was carried out in (A) 10 patients with idiopathic dilated cardiomyopathy, (B) 7 with dilated phase of hypertrophic cardiomyopathy and (C) 8 with normal (control) individuals. Imaging was taken 48 hours after intravenous injection of 74 MBq of AM. Myocardial uptake of AM was evaluated qualitatively and quantitatively. Positive uptake was observed in 9/10 (90%), 7/7 (100%) and 0/8 (0%) in groups A, B and C, respectively. AM index (heart/lung ratio) in groups A and B were 2.04±0.24 and 2.46±0.49, Values significantly higher than that obtained in the control patient without cardiomyopathy (1.51±0.13)(p<0.01). Positive monoclonal antimyosin antibody studies were highly prevalent in dilated cardiomyopathic and dilated phase of hypertrophic cardiomyopathic patients, even in the presence of negative right ventricular biopsy. It is suggested that this method was useful for the noninvasive assessment of active myocardial damage in these patients. (author)

  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. Humoral immunity in cardiomyopathy.

    OpenAIRE

    Lowry, P J; Thompson, R. A.; Littler, W. A.

    1983-01-01

    Sera from patients with heart disease were examined by single sandwich indirect immunofluorescence for the presence of antibodies to human heart muscle. Endocardial biopsy specimens were also examined by direct immunofluorescence for deposition of antibodies in vivo. No consistent or specific abnormality was found in the biopsy specimens or sera of patients with congestive cardiomyopathy. The presence of anti-heart antibodies in cardiac disease appears to reflect damage to cardiac muscle what...

  4. 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...

  5. 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

  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. Percutaneous dilatational tracheostomy

    DEFF Research Database (Denmark)

    Johnsen, R.

    2015-01-01

    Introduction Since the introduction and development of percutaneous dilatational tracheostomy (PDT), this procedure is accepted and incorporated in ICUs worldwide. In spite of obvious benefits for the patients, who obtain more comfort and mobility and less use of sedatives, the procedure also...... implies the risk of several complications, some of which may be lethal. Severe complications include hemorrhage, displacement and pneumothorax. Different methods of PDT are described in the literature, each with disadvantages and benefits. The aim of this study was to analyze complications due to PDTs...

  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. 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.

  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. 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.

  12. 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.)

  13. 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....

  14. 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

  15. Myocardial glucose metabolism in patients with hypertrophic cardiomyopathy. Assessment by F-18-FDG PET study

    International Nuclear Information System (INIS)

    In an investigation of myocardial metabolic abnormalities in hypertrophic myocardium, the myocardial glucose metabolism was evaluated with F-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) in 32 patients with hypertrophic cardiomyopathy, and the results were compared with those in 9 patients with hypertensive heart disease. F-18-FDG PET study was performed in the fasting and glucose-loading states. The myocardial regional %dose uptake was calculated quantitatively. The average regional %dose uptake in the fasting state in the patients with asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy was significantly higher than that in the patients with hypertensive heart disease (0.75±0.34%, 0.65±0.25%, and 0.43±0.22%/100 g myocardium, respectively). In contrast, the average %dose uptake in the glucose-loading state in the patients with asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy was not significantly different from that in patients with hypertensive heart disease (1.17±0.49%, 0.80±0.44% and 0.99±0.45%, respectively). The patients with apical hypertrophy had also low %dose uptake in the fasting state (0.38±0.21%) as in the hypertensive heart disease patients, so that the characteristics of asymmetric septal hypertrophy and dilated-phase hypertrophic cardiomyopathy are considered to be high FDG uptake throughout the myocardium in the fasting state. Patients with apical hypertrophy are considered to belong to other disease categories metabolically. F-18-FDG PET study is useful in the evaluation of the pathophysiologic diagnosis of patients with hypertrophic cardiomyopathy. (author)

  16. Roentgenoendovascular dilatation of brachycephalic arteries

    International Nuclear Information System (INIS)

    The authors reported the technique, methods of and indications for roentgenoendovascular dilation in stenotic and occlusive lesions of the brachycephalic branches of the aorta. A total of 102 vascular dilations were perfomed in 76 patients resulting in a good angiographic and clinical effect. In 2 patients the first world prosthetics was performed using an original coiled nitinol prosthesis

  17. 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.

  18. Myocardial imaging with radioiodinated beta-methyl-branched fatty acid in cardiomyopathy

    International Nuclear Information System (INIS)

    The purpose of our experimental and clinical studies was to examine whether myocardial distribution of beta-methyl iodophenyl pentadecanoic acid (BMIPP) is different from that of thallium in cardiomyopathic hamsters and how single photon emission computed tomography (SPECT) delineate the different between thallium and BMIPP distributions in patients with cardiomyopathy. Quantitative dual tracer autoradiography demonstrated an uncoupling of myocardial thallium and [I-125]BMIPP distributions as well as a regional heterogeneity of [I-125]BMIPP distribution in cardiomyopathic hamsters. In patients with septal or apical hypertrophy and normal contractility, SPECT showed reduced [I-123]BMIPP uptake in the thickened myocardium with normal or high thallium uptake. In patients with hypertrophy and systolic dysfunction as well as those with dilated cardiomyopathy, SPECT with thallium and [I-123]BMIPP showed similar heterogeneous distributions. In conclusion, SPECT with [I-123]BMIPP may provide unique features different from thallium imaging and may delineate regional abnormalities of myocardial fatty acid metabolism in cardiomyopathy. (author)

  19. 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

  20. Responsiveness of cardiodynamics to exercise loading in normal subjects and in patients with idiopathic cardiomyopathy

    International Nuclear Information System (INIS)

    In the present study, we examined the responses of the left ventricular systolic function and diastolic function to exertion by cardiac blood pool scintigraphy using 99mTc in reference to changes with aging and changes in the cases of idiopathic cardiomyopathy. In order to study the functional response with aging to exertion, 38 normal subjects were divided by age. Subsequently, 28 hypertrophic cardiomyopathy cases (Group H), 13 dilated cardiomyopathy cases (Group D) and 14 normal cases (Group N) were studied. Ejection Fraction (EF) and Peak Ejection Rate (PER) were used as the indicators for the systolic function. Peak Filling Rate (PER), 1/3 Filling Rate (1/3FR) and Time to Peak Filling (TPF) were used as the indicators for the diastolic function. When comparison was made among the normal subjects by age, the systolic function and diastolic function at rest, varied as they were, showed no significant change with aging. The %delta EF and %delta PER tended to decrease linearly with aging (Y=-4E-X+28, p<0.0001; Y=-X+57, p<0.0001). As to the %delta PFR, %delta 1/3FR and %delta TPF, however, correlation with aging was not found. In comparing hypertrophic cardiomyopathy and dilated cardiomyopathy, Group D showed significantly lower values before exertion and after maximum exertion than the other two groups. A difference in the response of the systolic function and diastolic function to exertion was noted in both the comparative study on aging of the normal subjects and the study of cases of cardiomyopathy. The presence of the compensatory mechanism due to elevation of the left atrial pressure and an increase in the left atrial contractile power may be mentioned as a reservoir of blood and also as a booster pump. Particularly, the effect of the latter compensates for the decline in the left ventricular compliance, which may lead to the maintenance of the diastolic function. (K.H.)

  1. Cine MRI of hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Cine MRI was performed using 1.5T or 0.5T MR units in eleven patients with hypertrophic cardiomyopathy. Septal and posterior wall thickness measured by cine MRI correlated well with those obtained by ultrasonographic cardiogram. In hypertrophic obstructive cardiomyopathy, cine MRI demonstrated the site and nature of obstructive change in left ventricle. Cine MRI also showed flow void due to mitral regurgitation successfully. We considered cine MRI is useful means to evaluate the anatomical and functional findings in hypertrophic cardiomyopathy. (author)

  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. Efficacy of Gd-DTPA-enhanced MRI in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    The cabability of magnetic resonance (MR) imaging to detect tissue characterization or myocardial degeneration process of the hypertrophied myocardium was evaluated in 15 patients with hypertrophic cardiomyopathy. T1-weighted MR images were obtained with a 1.5 T MR unit by using ECG-gated spin-echo techniques. MR images were visually reviewed before and after enhancement of Gd-DTPA. Four patients had an increase in signal intensity mainly in the endocardium of the left ventricular septum on non-enhanced MR images, 3 of whom had widespread high intensity in addition to two-thirds of the wall. Gd-DTPA enhanced-MR images showed high intensity over the whole septum in 5 patients and also in the antero-lateral endocardium in 4 patients. Decreased intensity on non-enhanced MR images, as shown in 4 patients, became clear on enhanced-MR images. According to findings on enhanced-MR images, signal intensity was defined as normal (N), septum (S), and diffuse (D). Patients in Group D tended to be younger and have more frequently family history. Regarding both interventricular septum thickness and left ventricular posterior wall thickness, there was no significant difference among the three groups. Both left ventricular diastolic diameter and left ventricular systolic diameter were significantly larger in Group D than the other two groups. Left ventricular ejection fraction was significantly lower in both Group S and Group D. Widespread abnormal intensity on Gd-DTPA enhanced MR images was associated with findings similar to dilated cardiomyopathy, such as dilated left ventricular lumen and decreased ejection fraction. Gd-DTPA enhanced MR imaging seemed to be useful for visualizing myocardial degeneration in hypertrophic cardiomyopathy.(N.K.)

  4. Dilated uropathies in children

    International Nuclear Information System (INIS)

    These uropathies are frequent in children and are often diagnosed by ante-natal ultrasound examination. The dilatation, hydronephrosis or uretero-hydro-nephrosis may be due to a large pattern of malformations, either anatomical dysplasia, vesico-ureteric reflux or obstruction of the pelvi-ureteric junction, of the vesical-ureteric junction or due to a chronic urethral obstruction. The investigations must determine the exact urinary tract abnormalities, the renal function and the uro-dynamic change. They are guided by the ultrasound findings and cystography. In obstructive malformation, the MAG3 renogram with furosemide test is the best way to precise the uro-dynamic status, but it requires a careful technique in children and its interpretation is sometimes equivocal. DMSA scan is very useful to give precise separate kidney function and to follow the maturational change with age. All the results must be carefully analysed ; the final therapeutic decision and specially surgery is dependent of the type of uropathy and its natural history. In many cases, surgical treatment is only indicated after a longer follow-up and repeated evaluations. (authors). 11 refs., 2 figs

  5. 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 ...

  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. 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.)

  8. 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.

  9. 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...

  10. New insights into cirrhotic cardiomyopathy

    DEFF Research Database (Denmark)

    Møller, Søren; Hove, Jens D; Dixen, Ulrik;

    2013-01-01

    beta-receptor function seem involved in the autonomic and cardiac dysfunction. Cirrhotic cardiomyopathy can be revealed by tissue Doppler imaging but is best demasked by physical or pharmacological stress. Liver transplantation may revert cardiac dysfunction but surgery and shunt insertion may also......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 reduced...

  11. Microvascular dysfunction in nonfailing arrhythmogenic right ventricular cardiomyopathy

    International Nuclear Information System (INIS)

    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 H215 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 H215 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 (MBFrest 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-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. Role of cardiac FDG PET-CT in inflammatory cardiomyopathy

    International Nuclear Information System (INIS)

    Full text: Role of cardiac FDG PET-CT in the diagnosis and monitoring treatment response of inflammatory cardiomyopathy. Materials and Methods: This is a retrospective analysis of 72 patients (over a period of 3 years) referred for cardiac PET-CT with dilated cardiomyopathy (DCM) or idiopathic ventricular tachycardia (VT), to rule out inflammatory cardiomyopathy. FDG cardiac PET was done with patients following very high fat low carbohydrate protein preferred diet (VHFLCPPD) protocol one day prior to scan. Myocardial perfusion scan with 99mTc-tetrofosmin or 13N-NH3 was done prior to FDG PET. All patients underwent breathhold CECT, chest PET-CT and cardiac PET-CT 45 min post FDG injection. Scans were reported jointly by a radiologist and nuclear medicine physician. Clinical/pathological follow-up was obtained where possible. Results: 50(69.4%) patients had positive myocardial FDG uptake with matched perfusion defect on NH3/Tc scan in the absence of CAD. 30(42%) patients had associated metabolically active lymphadenopathy and organ lesions. 4 patients had metabolically active lymphnodes with no myocardial inflammation. 18 patients had no abnormality detected on the scan. Patients with positive lymphnodes underwent biopsy and proven to have granulomatous disease either sarcoidosis and with TB or TB alone. 15 patients had post treatment scan which showed complete metabolic response in 7, partial response in 6 and stable disease in 2. Conclusion: Evaluating patients with DCM or idiopathic VT secondary to inflammatory myocarditis is challenging. Cardiac PET scan with VHFLCPPD protocol is the most sensitive modality available for assessment of disease activity in inflammatory cardiomyopathy and monitoring the treatment response especially in patients with ICD

  13. 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.)

  14. 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

  15. 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.

  16. 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...

  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 ...... febrile neutropenia. Testing for the A1555G mutation is recommended in patients frequently treated with aminoglycosides....

  19. 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.

  20. 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

  1. 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

  2. Dilatation effect of ''quantum clocks''

    International Nuclear Information System (INIS)

    The relativistic dilatation effect of the life-time of unstable microparticles combined with quantum symmetry of their description results in the ''quantum-dilatation'' dilemma. It is due to the classical character of the relativity theory which here reveals itself in the classical world-line of the clock necessary in order to deduce the dilatation effect from the Lorentz transformation. It is shown how to solve this dilemma, basing on the relation continuum C4. Two types of measurements of time intervals, the direct and indirect one, are analyzed. The former type corresponds to the external space-time continuum, where any direct measurement takes place, and the latter, to the internal relation continuum C4, where the internal structures of isolated micro-systems are sunk. (author)

  3. Fluid dynamics of dilatant fluid

    DEFF Research Database (Denmark)

    Nakanishi, Hiizu; Nagahiro, Shin-ichiro; Mitarai, Namiko

    2012-01-01

    of the state variable, we demonstrate that the model can describe basic features of the dilatant fluid such as the stress-shear rate curve that represents discontinuous severe shear thickening, hysteresis upon changing shear rate, and instantaneous hardening upon external impact. An analysis of the......A dense mixture of granules and liquid often shows a severe shear thickening and is called a dilatant fluid. We construct a fluid dynamics model for the dilatant fluid by introducing a phenomenological state variable for a local state of dispersed particles. With simple assumptions for an equation...... model reveals that the shear thickening fluid shows an instability in a shear flow for some regime and exhibits the shear thickening oscillation (i.e., the oscillatory shear flow alternating between the thickened and the relaxed states). The results of numerical simulations are presented for one- and...

  4. 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.

  5. 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.

  6. 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...

  7. 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...

  8. 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...

  9. 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.

  10. 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.

  11. What Is a Comprehensive Dilated Eye Exam?

    Science.gov (United States)

    ... su oculista What is a comprehensive dilated eye exam? Listen You may think your eyes are healthy, ... eye care professional for a comprehensive dilated eye exam is the only way to really be sure. ...

  12. Unital Dilations of Completely Positive Semigroups

    OpenAIRE

    Gaebler, David J.

    2013-01-01

    Dilations of completely positive semigroups to endomorphism semigroups have been studied by numerous authors. Most existing dilation theorems involve a non-unital embedding, corresponding to the embedding of $B(H)$ as a corner of $B(K)$ for Hilbert spaces $H \\subset K$. A 1986 paper of Jean-Luc Sauvageot shows how to achieve a unital dilation, but does not specify how to do so while also preserving continuity properties of the original semigroup. This thesis further develops Sauvageot's dilat...

  13. 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...

  14. 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.

  15. 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

    at the population level is unclear. In a nationwide cohort, we examined the risk of cardiomyopathy by family history of premature death (data, we constructed a cohort of 3.9 million persons born from 1950 to 2008. We......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 (... ascertained family history of premature (

  16. 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

  17. Balloon dilatations of esophageal strictures

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Jeong Jin; Juhng, Seon Kwan; Kim, Jae Kyu; Chung, Hyon De [Chonnam National University College of Medicine, Seoul (Korea, Republic of)

    1990-04-15

    Most benign esophageal strictures can be successfully dilated with conventional bougienage technique. But occasionally strictures are so tight, lengthy, or sometimes irregular that this technique fail, and surgical intervention is required. Since 1974 Gruentzig balloon catheter has succeed when used for strictures in the cardiac and peripheral vasculatures, the biliary and urinary tracts, the colon of neonates after inflammatory disease and also in the esophagus. Fluoroscopically guided balloon catheters were used to dilate 30 esophageal strictures in 30 patients over 3 years at Department of Diagnostic Radiology, Chonnam University, College of Medicine. The distribution of age was from 7 years to 71 days and the ratio of male to female was 15:15. The causes of benign stricture (23 cases) were post-operative strictures (13), chemical (4), achalasia (3), chronic inflammation (2), esophageal rupture (1) and those of malignant stricture (7 cases) were post-radiation stricture of primary esophageal cancer (6) and metastatic esophageal cancer (1). The success rate of procedure was 93% (28/30). The causes of failure were the failure of passage of stricture due to markedly dilated proximal segment of esophagus (1 case) and too long segment of stricture (1 case). Complication of procedure was the diverticular-formation of esophagus in 3 cases, but has no clinical significance in follow-up esophagography. In conclusion, fluoroscopically guided balloon dilation of esophageal stricture appears to be safe, effective treatment and may be have theoretical advantages over conventional bougienage and also should be considered before other methods of treatment are used.

  18. Balloon dilatations of esophageal strictures

    International Nuclear Information System (INIS)

    Most benign esophageal strictures can be successfully dilated with conventional bougienage technique. But occasionally strictures are so tight, lengthy, or sometimes irregular that this technique fail, and surgical intervention is required. Since 1974 Gruentzig balloon catheter has succeed when used for strictures in the cardiac and peripheral vasculatures, the biliary and urinary tracts, the colon of neonates after inflammatory disease and also in the esophagus. Fluoroscopically guided balloon catheters were used to dilate 30 esophageal strictures in 30 patients over 3 years at Department of Diagnostic Radiology, Chonnam University, College of Medicine. The distribution of age was from 7 years to 71 days and the ratio of male to female was 15:15. The causes of benign stricture (23 cases) were post-operative strictures (13), chemical (4), achalasia (3), chronic inflammation (2), esophageal rupture (1) and those of malignant stricture (7 cases) were post-radiation stricture of primary esophageal cancer (6) and metastatic esophageal cancer (1). The success rate of procedure was 93% (28/30). The causes of failure were the failure of passage of stricture due to markedly dilated proximal segment of esophagus (1 case) and too long segment of stricture (1 case). Complication of procedure was the diverticular-formation of esophagus in 3 cases, but has no clinical significance in follow-up esophagography. In conclusion, fluoroscopically guided balloon dilation of esophageal stricture appears to be safe, effective treatment and may be have theoretical advantages over conventional bougienage and also should be considered before other methods of treatment are used

  19. Idiopathic Cardiomyopathy Following Metal-on-Metal Hip Arthroplasty: The New Face of “Beer Drinker’s Cardiomyopathy”

    Directory of Open Access Journals (Sweden)

    Erik O’Connell,

    2013-01-01

    Full Text Available In 1966, the “beer drinker’s cardiomyopathy” was described in Quebec City, Canada among heavy drinkers following the addition of cobalt by breweries for foam stabilisation. Almost half of those affected died. A similar process has been proposed for cardiovascular periprosthetic metallosis in patients receiving cobalt-containing hip prostheses. This case describes a young man with congenital osteonecrosis treated with cobalt containing prosthetic arthroplasty. Postoperatively, he developed pericardial effusion, dilated cardiomyopathy, polycythemia and lactic acidosis. His serum cobalt level was 156 mcg/L ( normal 0.1 – 0.4 mcg/L. Metal-on-metal hip implants have been associated with arthroprosthetic cobaltisim, a presentation indistinguishable from “beer drinker’s cardiomyopathy.” Up to 35% of hip replacements utilize metal-on-metal bearing surfaces, making this a potentially wide-spread and relatively underappreciated etiology of postoperative idiopathic cardiomyopathy. This clinical vignette emphasizes the importance of considering cobalt cardio- toxicity, or “beer drinker’s cardiomyopathy,” as a differential diagnosis of idiopathic cardiomyopathy following total hip arthroplasty.

  20. 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.

  1. Mode of left ventricular diastolic filling in hypertrophic cardiomyopathy as studied by pulsed Doppler echocardiography and multigated blood pool scan

    International Nuclear Information System (INIS)

    The clinical significance of the mode of left ventricular (LV) diastolic filling in hypertrophic cardiomyopathy was studied by the LV inflow velocity patterns (LVIF) of pulsed Doppler echocardiography and LV early diastolic filling rates (V2) of radionuclide (RI) angiography. The relationship between the deceleration time (DT) obtained from LVIF and the V2 was evaluated in 34 patients with nonobstructive hypertrophic cardiomyopathy (HCM) and in nine with obstructive hypertrophic cardiomyopathy (HOCM), and the results were compared with those of 10 patients with dilated cardiomyopathy (DCM), of two with restrictive cardiomyopathy (RCM), and of 19 normal subjects. HCM was subdivided into the following groups according to V1-DT relationships: Group 1 with prolonged DT and decreased V1, Group 2 with normal or short DT and normal V1, and Group 3 with normal or short DT and decreased V1. There were significant negative correlations between V2 and DT in Groups 1 and 2 of HCM, normal and HOCM. However, there were significant positive correlations in Group 3 of HCM, DCM and RCM. Nearly all patients in Group 3 had decreased LV ejection fraction and % fractional shortening, distinct B-B' step formation of the mitral valve echogram, and huge A wave of the apexcardiogram. These findings suggested that the LV rapid filling interval gradually became shorter because the LV contraction is decreased as myocardial fibrosis develops in HCM, and that we must pay attention to the diagnosis of such abnormalities, similar to those of DCM or RCM. (author)

  2. 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

  3. Identification of the Syrian hamster cardiomyopathy gene.

    Science.gov (United States)

    Nigro, V; Okazaki, Y; Belsito, A; Piluso, G; Matsuda, Y; Politano, L; Nigro, G; Ventura, C; Abbondanza, C; Molinari, A M; Acampora, D; Nishimura, M; Hayashizaki, Y; Puca, G A

    1997-04-01

    The BIO14.6 hamster is a widely used model for autosomal recessive cardiomyopathy. These animals die prematurely from progressive myocardial necrosis and heart failure. The primary genetic defect leading to the cardiomyopathy is still unknown. Recently, a genetic linkage map localized the cardiomyopathy locus on hamster chromosome 9qa2.1-b1, excluding several candidate genes. We now demonstrate that the cardiomyopathy results from a mutation in the delta-sarcoglycan gene that maps to the disease locus. This mutation was completely coincident with the disease in backcross and F2 pedigrees. This constitutes the first animal model identified for human sarcoglycan disorders. PMID:9097966

  4. Myocardial ischemia in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Myocardial ischemia in hypertrophic cardiomyopathy is multifactorial and explains the occurrence of angina, in about 50% of patients. The pathophysiology of myocardial ischemia may be explained by the increase of the ventricular mass and relative paucity of the coronary microcirculation; the elevated ventricular filling pressures and myocardial stiffness causing a compression of the coronary microvessels; the impaired coronary vasodilator flow reserve caused by anatomic and functional abnormalities; and the systolic compression of epicardial vessel (myocardial bridges). Myocardial ischemia must be investigated by perfusion scintigraphic methods since its presence influences the prognosis and has relevant clinical implications for management of patients. Patients with hypertrophic cardiomyopathy and documented myocardial ischemia usually need to undergo invasive coronary angiography to exclude the presence of concomitant atherosclerotic coronary disease. (author)

  5. Primary Carnitine Deficiency and Cardiomyopathy

    OpenAIRE

    Fu, Lijun; Huang, Meirong; Chen, Shubao

    2013-01-01

    Carnitine is essential for the transfer of long-chain fatty acids from the cytosol into mitochondria for subsequent β-oxidation. A lack of carnitine results in impaired energy production from long-chain fatty acids, especially during periods of fasting or stress. Primary carnitine deficiency (PCD) is an autosomal recessive disorder of mitochondrial β-oxidation resulting from defective carnitine transport and is one of the rare treatable etiologies of metabolic cardiomyopathies. Patients affec...

  6. 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.

  7. 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...

  8. 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

  9. Recent views on hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Recent Views on Hypertrophic Cardiomyopathy provides a review of results obtained in the area of hypertrophic cardiomyopathy, as well as a perspective for the future treatment of this cardiac disorder. The debate on the existence of a true obstruction to left ventricular outflow is expertly presented, as are current concepts of pathophysiology. Long-term treatment with the calcium antagonist, verapamil is thoroughly reviewed and surgical treatment and its implications, as applied to 160 patients suffering from hypertrophic cardiomyopathy, is thoroughly discussed. In addition, presentations on echocardiography and radionuclide cardiology as non-invasive procedures provide up-to-date views. Results obtained from pressure-volume loops and stress-strain relationships in patients with HCM, both before and after verapamil and propranolol treatments, are of special interest. To provide a better insight of this peculiar disease, two hemodynamic concepts - cavity angulation and atrioventricle - are introduced, emphasizing the interaction between morphology and function, and stressing the importance of the integrity of left atrial function. (Auth.)

  10. 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

  11. [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

  12. 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.

  13. Dilation theory yesterday and today

    CERN Document Server

    Arveson, William

    2009-01-01

    Paul Halmos' work in dilation theory began with a question and its answer: Which operators on a Hilbert space can be extended to normal operators on a larger Hilbert space? The answer is interesting and subtle. The idea of representing operator-theoretic structures in terms of conceptually simpler structures acting on larger Hilbert spaces has become a central one in the development of operator theory and, more generally, noncommutative analysis. The work continues today. In this article we summarize some of these diverse results and their history.

  14. Feline hypertrophic cardiomyopathy: variations on a theme

    International Nuclear Information System (INIS)

    The history and the physical, radiographic, electrocardiographic, echocardiographic, haemo-dynamic and angiocardiographic findings from 18 cats with idiopathic hypertrophic cardiomyopathy are described. These data document a diverse spectrum of clinical manifestations of this disease in cats. The variety and complexity of feline hypertrophic cardiomyopathy complicate the diagnosis and challenge the idea that this disorder is a single disease entity

  15. 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

  16. 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.

  17. 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

  18. The pupil dilation response to visual detection

    Science.gov (United States)

    Privitera, Claudio M.; Renninger, Laura W.; Carney, Thom; Klein, Stanley; Aguilar, Mario

    2008-02-01

    The pupil dilation reflex is mediated by inhibition of the parasympathetic Edinger-Westphal oculomotor complex and sympathetic activity. It has long been documented that emotional and sensory events elicit a pupillary reflex dilation. Is the pupil response a reliable marker of a visual detection event? In two experiments where viewers were asked to report the presence of a visual target during rapid serial visual presentation (RSVP), pupil dilation was significantly associated with target detection. The amplitude of the dilation depended on the frequency of targets and the time of the detection. Larger dilations were associated with trials having fewer targets and with targets viewed earlier during the trial. We also found that dilation was strongly influenced by the visual task.

  19. 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.

  20. [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

  1. 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

  2. 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.

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

    OpenAIRE

    Gopi C Khilnani; Neetu Jain; Pavan Tiwari; Vijay Hadda; Lavleen Singh

    2015-01-01

    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. Investigation...

  4. 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; Hvidsten, Svend; Zebrowski, David C; Engel, Felix Benedikt; Sheikh, Søren Paludan

    2016-01-01

    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 be an...... intermediate response before the onset of complete heart regeneration. Myocardial damage was performed by AR in postnatal day 1 C57BL/6 mice, and myocardial function and scarring assessed at day 180 using F-18-fluorodeoxyglucose positron emission tomography (FDG-PET) and histology, respectively. AR mice...

  5. 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.

  6. 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...

  7. 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...

  8. 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.

  9. 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.

  10. Gene Expression Analyses during Spontaneous Reversal of Cardiomyopathy in Mice with Repressed Nuclear CUG-BP, Elav-Like Family (CELF) Activity in Heart Muscle

    OpenAIRE

    Dasgupta, Twishasri; Coram, Ryan J.; Stillwagon, Samantha J.; Ladd, Andrea N

    2015-01-01

    CUG-BP, Elav-like family (CELF) proteins regulate cell type- and developmental stage-specific alternative splicing in the heart. Repression of CELF-mediated splicing activity via expression of a nuclear dominant negative CELF protein in heart muscle was previously shown to induce dysregulation of alternative splicing, cardiac dysfunction, cardiac hypertrophy, and dilated cardiomyopathy in MHC-CELFΔ transgenic mice. A “mild” line of MHC-CELFΔ mice that expresses a lower level of the dominant n...

  11. 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.

  12. [Takotsubo cardiomyopathy: origin and variants].

    Science.gov (United States)

    Aronov, D M

    2008-01-01

    This literature review is devoted to the " tako-tsubo " cardiomyopathy - rare type of cardiomyopathy characterized by transient myocardial stunning. In acute phase the disease resembles myocardial infarction. However no involvement of coronary arteries is found at angiography. Echocardiography and ventriculography reveal a- or - hypokinesia of various parts of the left ventricle. Classic (initial) variant of the disease is associated with concomitant apical akinesia and hyperkinesis of basal segments. The heart acquires a distinctive configuration with ballooning apex which resembles device used to trap octopus. The author refers to described by him 11 cases of myocardial damage with infarct-like clinic without changes of coronary arteries in healthy men younger than 35 years (D.M.Aronow, 1968, 1974). These cases occurred during severe physical stress and had in their basis hypercatecholaminemia which led to reversible myocardial damage of the myocardium which corresponded to modern concept of myocardial stunning. During exercise tests these patients had 3 times greater increase of urinal epinephrine excretion compared with 61 patients of the same age with atherosclerotic heart disease. PMID:18991836

  13. 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.

  14. Comparison of wall thickening and perfusion analysis in cardiomyopathy by gated Tc-99m MIBI

    International Nuclear Information System (INIS)

    The objective of this study was to clarify the relationship between left ventriculr (LV) function, wall thickening (WT) and perfusion in cardiomyopathy. Twenty-six cases (15 males, 11 females: mean age, 58 y), consisting of 10 healthy control subjects, 10 patients with hypertrophic cardiomyopathy (HCM) and 6 patients with idiopathic dilated cardiomyopathy (DCM) was studied with gated Tc-99m MIBI SPECT. LV WT, LV perfusion and function (LV wall motion, LVEDV, LVESV and LVEF) by using gated SPECT quantification and CEqual software were analysed. In HCM and DCM group, mean WT was significantly decreased. (HCM: 15.2±6.7%, DCM: 4.9%, control: 34.1±4.9% (p<0.01). Adenosine stress test didn't show any reversible or fixed perfusion defects in all HCM patients, controls and five DCM patients except one patient (but, no coronary artery stenosis in coronary angiography). LVEF for HCM, DCM and control was 48.4±13.2%, 28.7±8.8%, 62.7±9.5%, respectively (p<0.05)). LVEDV and LVESV didn't show a significant differences between HCM and control group, not DCM group. In HCM, WT was decreased as like DCM even though LV myocardial perfusion and function was reserved

  15. Evaluation of regional myocardial perfusion in patients with cardiomyopathy using dipyridamole thallium-201 emission computed tomography

    International Nuclear Information System (INIS)

    Myocardial emission computed tomography with thallium-201 was performed immediately and at 3 hr after iv infusion of dipyridamole in 20 patients with nonobstructive hypertrophic cardiomyopathy (HNCM) and 20 patients with dilated cardiomyopathy (DCM). The results were compared with findings of two-dimensional echocardiography and pathological findings. In the HNCM group, early Tl-201 imaging did not show perfusion defects in 9 patients (Group I), but showed them in 11 patients (Group II). Redistribution (Rd) was seen on delayed Tl-201 images in all of the HNCM group. Although percent fractional shortening of the septal and posterior walls was significantly reduced in Group II when compared with Group I, there was no difference in echocardiographic findings between the Groups. Percent fibrosis was significantly higher in Group II than Group I. All patients of the DCM group showed Tl-201 perfusion defects on early images, 9 of whom had Rd on delayed images. Percent fractional shortening was significantly elevated in patients with Rd than those without Rd. Ventricular wall motion abnormality was slight in the area with Rd, as compared with that without Rd. Percent fibrosis of the septal wall was higher in the presence of Rd than in the absence of Rd. The results indicate reduced coronary flow reverse in some of the cardiomyopathy patients. The reduced coronary reverse may be attributable to the mechanism involved in the fibrosis and be related to pathophysiologic progression. (Namekawa, K.)

  16. Evaluation of Sympathetic Innervation in Cardiomyopathy with 123I-MIBG

    International Nuclear Information System (INIS)

    123I-Iodine-metaiodobenzylguanidine(MIBG) which is a norepinephrine analogue, can be used to evaluate the sympathetic innervation of the heart. In this study, cardiac imaging with 123I-MIBG was performed in patients with 9 dilated cardiomyopathy, 2 ischemic cardiomyopathy and 1 acute myocardial infarction to evaluate the sympathetic nervous function. 123I-MIBG imaging showed multifocal defects (8), diffuse defect (2), near non-visualization (2). The defects of MIBG scans were found to be larger and more severe on 4 hours image than 30 minutes. Heart to lung, heart to mediastinum ratios were decreased at 4 hours than those at 30 minutes. Measured LVEF values were not correlated with the severity of MIBG uptake. 99mTc-MIBI imaging was also performed in all patients to find the relationship with 123I-MIBG scan. 99mTc-MIBI scan showed multifocal defects in 9 patients, diffuse defects in 1 patient and no defect in 2 patients. The defects are similar in size, severity and extent, but more larger and severe on 123I-MIBG imaging. Therefore, cardiac 123I-MIBG imaging is a useful method to evaluate the sympathetic nervous function in cardiomyopathy.

  17. Left Ventricular Tonic Contraction as a Novel Biomarker of Cardiomyopathy in Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Su, Jennifer A; Ramos-Platt, Leigh; Menteer, JonDavid

    2016-04-01

    Dilated cardiomyopathy (DCM) inevitably afflicts patients with Duchenne muscular dystrophy (DMD) as a consequence of cell death induced by unguarded calcium influx into cardiomyocytes. This mechanism may also inhibit muscle relaxation in early stages of cardiomyopathy. ACE inhibition (ACEi) is known to delay the onset and slow the progression of DCM in DMD. The objective of this study is to use echocardiography to assess for preclinical cardiac changes consistent with intracellular calcium dysregulation before the onset of overt ventricular dysfunction, and to evaluate how prophylactic ACEi may alter these pre-cardiomyopathic changes in the pediatric DMD population. We examined 263 echocardiograms from 70 pediatric patients with DMD. We defined abnormal tonic contraction (TC) as left ventricular internal dimension in diastole (LVIDd) Z-score hypertrophy, but rather may be a clinical correlate of the "calcium hypothesis" of DMD pathophysiology. LV TC is thus a promising biomarker for early detection of cardiomyopathy in DMD. ACEi prophylaxis suppresses LV TC and delays the development of DCM in DMD. PMID:26714815

  18. Esophageal achalasia : results of balloon dilation

    International Nuclear Information System (INIS)

    To evaluate the clinical effectiveness of fluoroscopically guided balloon dilation in the treatment of esophageal achalasia. Under fluoroscopic guidance, 21 balloon dilation procedures were performed in 14 patients with achalasia. A balloon with a diameter of 20 mm was used for the initial attempt.If the patient tolerated this well, the procedure was repeated with a 10-20 mm balloon, placed alongside at the same session. If, however the patient complained of severe chest pain and/or a postprocedural esophagogram showed an improvement,the additional balloon was not used. For patients whose results were unsatisfactory, the dilation procedure was repeated at sessions three to seven days apart. Succesful dilation was achieved in 13 of 14 patients(92.9%), who needed a total of 20 sessions of balloon dilation, ranging from one to three sessions per patient(mean, 1.54 sessions). Esophageal rupture occured in one of 14 patients(7.1%) ; of the 13 patients who underwent a successful dilation procedure, 12(92.3%) were free of recurrent symptoms during the follow-up period of 1-56(mean, 18.5) months. The remaning patient(7.7%) had a recurrence seven months after dilation. Fluoroscopically guided balloon dilation seems to be safe and effective in the treatment of esophageal achalasia

  19. Esophageal achalasia : results of balloon dilation

    Energy Technology Data Exchange (ETDEWEB)

    Ki, Won Woo; Kang, Sung Gwon; Yoon, Kwon Ha; Kim, Nam Hyeon; Lee, Hyo Jeong; Yoon, Hyun Ki; Sung, Kyu Bo; Song, Ho Young [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-08-01

    To evaluate the clinical effectiveness of fluoroscopically guided balloon dilation in the treatment of esophageal achalasia. Under fluoroscopic guidance, 21 balloon dilation procedures were performed in 14 patients with achalasia. A balloon with a diameter of 20 mm was used for the initial attempt.If the patient tolerated this well, the procedure was repeated with a 10-20 mm balloon, placed alongside at the same session. If, however the patient complained of severe chest pain and/or a postprocedural esophagogram showed an improvement,the additional balloon was not used. For patients whose results were unsatisfactory, the dilation procedure was repeated at sessions three to seven days apart. Succesful dilation was achieved in 13 of 14 patients(92.9%), who needed a total of 20 sessions of balloon dilation, ranging from one to three sessions per patient(mean, 1.54 sessions). Esophageal rupture occured in one of 14 patients(7.1%) ; of the 13 patients who underwent a successful dilation procedure, 12(92.3%) were free of recurrent symptoms during the follow-up period of 1-56(mean, 18.5) months. The remaning patient(7.7%) had a recurrence seven months after dilation. Fluoroscopically guided balloon dilation seems to be safe and effective in the treatment of esophageal achalasia.

  20. Myocardial metabolic, hemodynamic, and electrocardiographic significance of reversible thallium-201 abnormalities in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Exercise-induced abnormalities during thallium-201 scintigraphy that normalize at rest frequently occur in patients with hypertrophic cardiomyopathy. However, it is not known whether these abnormalities are indicative of myocardial ischemia. Fifty patients with hypertrophic cardiomyopathy underwent exercise 201Tl scintigraphy and, during the same week, measurement of myocardial lactate metabolism and hemodynamics during pacing stress. Thirty-seven patients (74%) had one or more 201Tl abnormalities that completely normalized after 3 hours of rest; 26 had regional myocardial 201Tl defects, and 26 had apparent left ventricular cavity dilatation with exercise, with 15 having coexistence of these abnormal findings. Of the 37 patients with reversible 201Tl abnormalities, 27 (73%) had metabolic evidence of myocardial ischemia during rapid atrial pacing compared with four of 13 patients (31%) with normal 201Tl scans (p less than 0.01). Eleven patients had apparent cavity dilatation as their only 201Tl abnormality; their mean postpacing left ventricular end-diastolic pressure was significantly higher than that of the 13 patients with normal 201Tl studies (33 +/- 5 versus 21 +/- 10 mm Hg, p less than 0.001). There was no correlation between the angiographic presence of systolic septal or epicardial coronary arterial compression and the presence or distribution of 201Tl abnormalities. Patients with ischemic ST segment responses to exercise had an 80% prevalence rate of reversible 201Tl abnormalities and a 70% prevalence rate of pacing-induced ischemia. However, 69% of patients with nonischemic ST segment responses had reversible 201Tl abnormalities, and 55% had pacing-induced ischemia. Reversible 201Tl abnormalities during exercise stress are markers of myocardial ischemia in hypertrophic cardiomyopathy and most likely identify relatively underperfused myocardium

  1. Aptamer binding and neutralization of β1-adrenoceptor autoantibodies: basics and a vision of its future in cardiomyopathy treatment.

    Science.gov (United States)

    Haberland, Annekathrin; Wallukat, Gerd; Schimke, Ingolf

    2011-08-01

    Autoantibodies directed against the second extracellular receptor loop of the β(1) receptor (β(1)-ECII-AABs) that belong to the superfamily of G protein-coupled receptors have been frequently found in patients with idiopathic dilated cardiomyopathy, Chagas' cardiomyopathy, and peripartum cardiomyopathy and have been clearly evidenced to be related to disease pathogenesis. Consequently, specific proteins or peptides used as binders in immunoapheresis or as in vivo neutralizers of β(1)-ECII-AABs have been suggested for patient treatment. Aptamers, which are target specifically selected short single- or double-stranded RNA or DNA sequences, are a recently introduced new molecule class applicable to bind and neutralize diverse molecule species, including antibodies. This article reviews selection technologies and characteristics of aptamers with respect to a single-stranded DNA aptamer recently identified as having a very high affinity against β(1)-ECII-AABs. The potential of this aptamer for the elimination of β(1)-ECII-AABs and in vivo neutralization is critically analyzed in view of its potential for future use in cardiomyopathy treatment. PMID:22814426

  2. 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.)

  3. Recurrent Takotsubo Cardiomyopathy Related to Recurrent Thyrotoxicosis

    Science.gov (United States)

    Patel, Keval; Griffing, George T.; Hauptman, Paul J.

    2016-01-01

    Takotsubo cardiomyopathy, or transient left ventricular apical ballooning syndrome, is characterized by acute left ventricular dysfunction caused by transient wall-motion abnormalities of the left ventricular apex and mid ventricle in the absence of obstructive coronary artery disease. Recurrent episodes are rare but have been reported, and several cases of takotsubo cardiomyopathy have been described in the presence of hyperthyroidism. We report the case of a 55-year-old woman who had recurrent takotsubo cardiomyopathy, documented by repeat coronary angiography and evaluations of left ventricular function, in the presence of recurrent hyperthyroidism related to Graves disease. After both episodes, the patient's left ventricular function returned to normal when her thyroid function normalized. These findings suggest a possible role of thyroid-hormone excess in the pathophysiology of some patients who have takotsubo cardiomyopathy. PMID:27127432

  4. 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

  5. DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling.

    Science.gov (United States)

    Richter-Dennerlein, Ricarda; Korwitz, Anne; Haag, Mathias; Tatsuta, Takashi; Dargazanli, Sascha; Baker, Michael; Decker, Thorsten; Lamkemeyer, Tobias; Rugarli, Elena I; Langer, Thomas

    2014-07-01

    Prohibitins form large protein and lipid scaffolds in the inner membrane of mitochondria that are required for mitochondrial morphogenesis, neuronal survival, and normal lifespan. Here, we have defined the interactome of PHB2 in mitochondria and identified DNAJC19, mutated in dilated cardiomyopathy with ataxia, as binding partner of PHB complexes. We observed impaired cell growth, defective cristae morphogenesis, and similar transcriptional responses in the absence of either DNAJC19 or PHB2. The loss of PHB/DNAJC19 complexes affects cardiolipin acylation and leads to the accumulation of cardiolipin species with altered acyl chains. Similar defects occur in cells lacking the transacylase tafazzin, which is mutated in Barth syndrome. Our experiments suggest that PHB/DNAJC19 membrane domains regulate cardiolipin remodeling by tafazzin and explain similar clinical symptoms in two inherited cardiomyopathies by an impaired cardiolipin metabolism in mitochondrial membranes. PMID:24856930

  6. Acute gastritis-induced Takotsubo's cardiomyopathy

    OpenAIRE

    Villablanca, Pedro A.; Sukhal, Shashvat; Ansari, Asimul; Mohammed, Dergham

    2013-01-01

    Key Clinical Message A 50-year-old lady presented with epigastralgia, electrocardiogram (ECG) showed T-wave inversions and the echocardiogram low ejection fraction (EF) with apical ballooning. An esophagogastroduodenoscopy (EGD) revealed gastritis. She recovered with proton pump inhibitors treatment. This is the first case that describes gastritis-induced stress cardiomyopathy. Clinicians should be aware of Takotsubo's cardiomyopathy (TCM) as a possible complication of gastritis.

  7. RATIONAL PHARMACOTHERAPY IN TAKOTSUBO CARDIOMYOPATHY

    Directory of Open Access Journals (Sweden)

    S. Marchev

    2015-12-01

    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.

  8. 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

  9. 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...

  10. 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.

  11. Balloon catheter dilatation of esophageal strictures

    International Nuclear Information System (INIS)

    The authors performed 27 fluoroscopically guided balloon dilatation in 12 patients of esophageal stricture during recent 3 years. The causes of esophageal stricture were corrosive esophagitis (N=2) and congenital narrowing (N=1), including postoperative narrowing in achalasia (N=3), esophageal varix (N=3), lye stricture (N=2) and esophageal cancer (N=1). Successful dilatation of the stricture was achieved during the procedure in 10 patients(83%). Major complication such as esophageal rupture was not found. The authors conclude that fluoroscopically guided esophageal balloon dilatation is a safe and effective method for treatment of symptomatic esophageal strictures

  12. Balloon catheter dilatation of esophageal strictures

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jeung Sook; Yoon, Yup; Sung, Dong Yook; Choi, Woo Suk; Nam, Kyung Jin; Lim, Jae Hoon [Kyunghee University College of Medicine, Seoul (Korea, Republic of)

    1990-07-15

    The authors performed 27 fluoroscopically guided balloon dilatation in 12 patients of esophageal stricture during recent 3 years. The causes of esophageal stricture were corrosive esophagitis (N=2) and congenital narrowing (N=1), including postoperative narrowing in achalasia (N=3), esophageal varix (N=3), lye stricture (N=2) and esophageal cancer (N=1). Successful dilatation of the stricture was achieved during the procedure in 10 patients(83%). Major complication such as esophageal rupture was not found. The authors conclude that fluoroscopically guided esophageal balloon dilatation is a safe and effective method for treatment of symptomatic esophageal strictures.

  13. Balloon dilatation of alimentary tract strictures

    International Nuclear Information System (INIS)

    From a series of balloon dilatations of alimentary tract strictures 11 patients with different types of gastric and oesophagogastric anastomotic stenoses are reported. The dilatation of gastric outflow tract obstructions was highly effective in the treatment of retention. In 7 out of 8 cases with gastric outflow stenosis surgery could be entirely avoided and replaced by balloon dilatation. The importance of eliminating retention in the healing of gastric ulcer is discussed. Fistulation in oesophagogastric anastomoses due to stenosis of the outflow portion was successfully treated. The importance of achieving a free outflow tract in order to prevent insufficiency of the anastomosis and formation of a fistula is stressed. (orig.)

  14. 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.

  15. Diagnosis of arrhythmogenic right ventricular cardiomyopathy using MRI

    International Nuclear Information System (INIS)

    Objective: To evaluate MR findings of arrhythmogenic right ventricular cardiomyopathy (ARVC) with MR new techniques and to study MR scanning techniques. Methods: 15 patients of ARVC diagnosed or suspected by clinical and echocardiography were performed with MRI. Using GE signa 1.5 Tesla CV/i MR system, scanning sequences included: Black-blood techniques: Double-IRFSE and Triple-IRFSE sequences, white blood technique: Fastcine sequence. Scanning plane included short axis view, four-chamber view, and long axis view. Results: Ten patients were diagnosed as ARVC and the main MR features of ARVC included: fat signal intensity of right ventricular (RV) wall (3 cases), thinning of RV wall (9 cases), dilatation of the RV (6 cases), ventricular wall aneurysm formation (2 cases), slow blood flow signal within the RV (9 cases), declined ejection fraction of the RV (6 cases), enlargement of the right atrium (3 cases), and involvement of the papillary muscle of the RV, apex of the left ventricle and anterior ventricular septum (2 cases). Black-blood techniques could show the cardiac anatomy, morphologic structure, and tissue specificity, while white blood techniques mainly obtained information about cardiac function and myocardial wall motion. Short axis view and four-chamber view revealed lesions with satisfaction. Conclusion: MR findings of ARVC have some specific features. Multi-sequence and multi-plane imaging with new MR techniques can accurately diagnose ARVC

  16. 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

  17. A Novel Homoplasmic Mutation in mtDNA with a Single Evolutionary Origin as a Risk Factor for Cardiomyopathy

    OpenAIRE

    Shin, Wee Soo; Tanaka, Masashi; Suzuki, Jun-ichi; Hemmi, Chieko; Toyo-oka, Teruhiko

    2000-01-01

    To clarify the relationship between variation in mtDNA and the development of cardiomyopathy (CM), the complete sequences of mtDNAs of two brothers with dilated CM were compared with those of 181 patients who had CM and with those of 168 control subjects. Five patients with CM shared a novel homoplasmic point mutation (G12192A tRNAHis), and all of them demonstrated the evolutionarily related D-loop sequence. The results suggest that this novel mutation originated from the same ancestor and th...

  18. On Demand Urethral Dilatation Versus Intermittent Urethral Dilatation: Results and Complications in Women With Urethral Stricture

    OpenAIRE

    Heidari, Fatemeh; Abbaszadeh, Shahin; Ghadian, Alireza; Tehrani Kia, Farahnaz

    2014-01-01

    Background: The treatment of urethral stricture in female patients is through dilatation of the urethra by tubes of increasing diameter. There are two main methods: intermittent dilatation and on demand dilatation. Objectives: The main aim of this study was to compare the results of these two methods, and to determine the best one. Patients and Methods: In this clinical trial study, we reviewed the documents of women diagnosed with urethral stricture, who came to the Baqiyatallah Clinic from ...

  19. Image Denoising Based on Dilated Singularity Prior

    OpenAIRE

    Wufan Chen; Zhiwu Liao; Shaoxiang Hu

    2012-01-01

    In order to preserve singularities in denoising, we propose a new scheme by adding dilated singularity prior to noisy images. The singularities are detected by canny operator firstly and then dilated using mathematical morphology for finding pixels “near” singularities instead of “on” singularities. The denoising results for pixels near singularities are obtained by nonlocal means in spatial domain to preserve singularities while the denoising results for pixels in smooth regions are obtained...

  20. Experience with esophageal dilatations in children

    Directory of Open Access Journals (Sweden)

    G. Christopoulos-Geroulanos .

    2007-03-01

    Full Text Available SUMMARY Aim: Esophageal stenosis requiring treatment is a serious complication of a variety of otherwise benign conditions in children. Dilatation is the treatment of choice. However, the method and acceptable duration are largely a matter of personal preference. We present our experience with 81 children undergoing dilatations for benign strictures due to a variety of causes and discuss the resulting problems. Material: During the period 1987-2001, eighty-one children were treated for strictures of the esophagus. The causes were: correction of esophageal atresia (EATEF, gastroesophageal reflux (GER, stricture of the cerevical anastomosis following esophageal replacement (ER, burn due to ingestion of caustic agents (CB, tight fundoplication (TF, achalasia (EA, congenital esophageal stenosis (CES and stenosis following sclerotherapy of esophageal varices (EV. All dilatations were performed under general anesthesia. Balloon dilatations were performed under fluoroscopic control following endoscopic insertion of the guide-wire. Results: The results were excellent or good in 58 children (78.3%. Twenty-four children (25.9% had to be treated surgically either to stabilize the result of the dilatations or to correct an intractable stenosis. Complications occurred in 6 children (7.4%: Four suffered a rupture at the level of the stricture following bougienage. Transverse suture of the longitudinal tear resulted in cure of the stenosis in 3. In the fourth, a cervical esophagostomy and gastrostomy had to be placed. Finally one child had a subdiaphragmatic rupture at the esophago-gastric junction, also following bougienage, treated with drainage. Strictures following esophageal replacement required the most dilatations (mean 11.3 per patient. An increased number of procedures were also required in esophageal burns (mean 6.3. Conclusion: 1 Esophageal dilatation is an effective treatment for strictures. 2 Rupture is a serious complication best treated