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Sample records for arrhythmogenic right ventricular dysplasia

  1. Arrhythmogenic right ventricular dysplasia: A case report

    Directory of Open Access Journals (Sweden)

    Tessa Negrín Valdés

    2015-10-01

    Full Text Available Arrhythmogenic right ventricular dysplasia is a heart muscle disease that predominantly affects the right ventricle, bringing about the replacement of normal myocardium with fatty or fibrofatty tissue and causing sudden death in young individuals. Ventricular tachycardia is an important clinical manifestation, although there are reports of right or global heart failure. The diagnosis is confirmed by echocardiography and magnetic resonance imaging. The case of a 65-year-old former smoker, with hypertension and ischemic heart disease, a history of effort syncope symptoms and proven non-sustained ventricular tachycardia, with morphology of left bundle branch block, is reported. Relevant diagnostic studies were performed, and echocardiographic elements which were compatible with arrhythmogenic right ventricular dysplasia were found. Therefore, an implantable cardioverter defibrillator was implanted, after which the patient has had a favorable outcome.

  2. New ECG Criteria in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    NARCIS (Netherlands)

    M.G.P.J. Cox; J.J. van der Smagt; A.A.M. Wilde; A.C.P. Wiesfeld; D.E. Atsma; M.R. Nelen; L.M. Rodriguez; P. Loh; M.J. Cramer; P.A. Doevendans; J.P. van Tintelen; J.M.T. de Bakker; R.N.W. Hauer

    2009-01-01

    Background-Desmosomal changes, electric uncoupling, and surviving myocardial bundles in fibrofatty tissue characterize arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Resultant activation delay is pivotal for reentry and thereby ventricular tachycardia (VT). Current task force cr

  3. Arrhythmogenic right ventricular cardiomyopathy/dysplasia

    Directory of Open Access Journals (Sweden)

    Basso Cristina

    2007-11-01

    Full Text Available Abstract Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D is a heart muscle disease clinically characterized by life-threatening ventricular arrhythmias. Its prevalence has been estimated to vary from 1:2,500 to 1:5,000. ARVC/D is a major cause of sudden death in the young and athletes. The pathology consists of a genetically determined dystrophy of the right ventricular myocardium with fibro-fatty replacement to such an extent that it leads to right ventricular aneurysms. The clinical picture may include: a subclinical phase without symptoms and with ventricular fibrillation being the first presentation; an electrical disorder with palpitations and syncope, due to tachyarrhythmias of right ventricular origin; right ventricular or biventricular pump failure, so severe as to require transplantation. The causative genes encode proteins of mechanical cell junctions (plakoglobin, plakophilin, desmoglein, desmocollin, desmoplakin and account for intercalated disk remodeling. Familiar occurrence with an autosomal dominant pattern of inheritance and variable penetrance has been proven. Recessive variants associated with palmoplantar keratoderma and woolly hair have been also reported. Clinical diagnosis may be achieved by demonstrating functional and structural alterations of the right ventricle, depolarization and repolarization abnormalities, arrhythmias with the left bundle branch block morphology and fibro-fatty replacement through endomyocardial biopsy. Two dimensional echo, angiography and magnetic resonance are the imaging tools for visualizing structural-functional abnormalities. Electroanatomic mapping is able to detect areas of low voltage corresponding to myocardial atrophy with fibro-fatty replacement. The main differential diagnoses are idiopathic right ventricular outflow tract tachycardia, myocarditis, dialted cardiomyopathy and sarcoidosis. Only palliative therapy is available and consists of antiarrhythmic drugs

  4. Arrhythmogenic Right Ventricular Dysplasia in Neuromuscular Disorders

    Science.gov (United States)

    Finsterer, Josef; Stöllberger, Claudia

    2016-01-01

    OBJECTIVES Arrhythmogenic right ventricular dysplasia (ARVD) is a rare, genetic disorder predominantly affecting the right ventricle. There is increasing evidence that in some cases, ARVD is due to mutations in genes, which have also been implicated in primary myopathies. This review gives an overview about myopathy-associated ARVD and how these patients can be managed. METHODS A literature review was done using appropriate search terms. RESULTS The myopathy, which is most frequently associated with ARVD, is the myofibrillar myopathy due to desmin mutations. Only in a single patient, ARVD was described in myotonic dystrophy type 1. However, there are a number of genes causing either myopathy or ARVD. These genes include lamin A/C, ZASP/cypher, transmembrane protein-43, titin, and the ryanodine receptor-2 gene. Diagnosis and treatment are identical for myopathy-associated ARVD and nonmyopathy-associated ARVD. CONCLUSIONS Patients with primary myopathy due to mutations in the desmin, dystrophia myotonica protein kinase, lamin A/C, ZASP/cypher, transmembrane protein-43, titin, or the ryanodine receptor-2 gene should be screened for ARVD. Patients carrying a pathogenic variant in any of these genes should undergo annual cardiological investigations for cardiac function and arrhythmias. PMID:27790050

  5. QT dispersion in patients with arrhythmogenic right ventricular dysplasia

    DEFF Research Database (Denmark)

    Benn, Marianne; Hansen, P S; Pedersen, A K

    1999-01-01

    , of electrical instability. The present study was conducted to assess the occurrence of QT dispersion and its modulation during treatment with sotalol. Methods Twenty-five patients with the diagnosis of arrhythmogenic right ventricular dysplasia were studied retrospectively. Fourteen patients were considered low...

  6. Arrhythmogenic right ventricular dysplasia masquerading as an abdominal episode.

    Science.gov (United States)

    Kaya, Mehmet Gungor; Yalcin, Ridvan; Ozin, Bulent; Altunkan, Sekip; Cengel, Atiye

    2007-01-01

    A 19-year-old woman presented with abdominal pain. Aside from epigastric tenderness, the patient's physical examination was unremarkable. She developed ventricular tachycardia with left bundle branch block morphology shortly after admission. Echocardiography revealed a thin, enlarged, and hypokinetic right ventricle. Electron beam computed tomography demonstrated hypodense areas in the right ventricular free wall suggestive of fatty infiltration, which suggested arrhythmogenic right ventricular dysplasia. The diagnosis was confirmed with the use of cardiac magnetic resonance imaging. The patient received an implantable cardioverter-defibrillator. This case illustrates a noncardiac presentation of a rare yet treatable cardiac condition.

  7. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: an updated imaging approach.

    Science.gov (United States)

    Zimmerman, Stefan L

    2015-02-01

    Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a rare inherited cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium and risk of sudden death from ventricular tachyarrhythmias. Cardiac magnetic resonance (MR) imaging plays an important role in the diagnostic evaluation of patients and family members suspected of having ARVC/D. This article discusses the epidemiology and pathophysiology of ARVC/D, reviews typical MR imaging findings and diagnostic criteria, and summarizes potential pitfalls in the MR imaging evaluation of patients suspected of having ARVC/D.

  8. A Genetic Variants Database for Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    NARCIS (Netherlands)

    van der Zwaag, Paul A.; Jongbloed, Jan D. H.; van den Berg, Maarten P.; van der Smagt, Jasper J.; Jongbloed, Roselie; Bikker, Hennie; Hofstra, Robert M. W.; van Tintelen, J. Peter

    2009-01-01

    Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a hereditary cardiomyopathy characterized by fibrofatty replacement of cardiomyocytes, ventricular tachyarrhythmias and sudden death. ARVD/C is mainly caused by mutations in genes encoding desmosomal proteins. However, the pathoge

  9. Right ventricular cardiomyopathy meeting the arrhythmogenic right ventricular dysplasia revised criteria? Do not forget sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Vasaturo, Sabina; Ploeg, David E.; Zeppenfeld, Katja; Veselic-Charvat, Maud [Leiden University Medical Center, Leiden (Netherlands); Buitrago, Guadalupe [Dept. of Radiology, Hospital General Universitario Gregorio Maranon, Madrid (Spain)

    2015-06-15

    A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the {sup r}evised ARVD task force criteria{sup .} An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.

  10. Left Ventricular Involvement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Assessed by Echocardiography Predicts Adverse Clinical Outcome

    NARCIS (Netherlands)

    Mast, Thomas P.; Teske, Arco J.; van der Heijden, Jeroen F.; Groeneweg, Judith A.; Te Riele, Anneline S J M; Velthuis, Birgitta K.; Hauer, RNW; Doevendans, Pieter A.; Cramer, Maarten J.

    2015-01-01

    Background Among studies describing the phenotype of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), significant discrepancy exists regarding the extent and impact of left ventricular (LV) involvement. The capability of conventional and new quantitative echocardiographic techniqu

  11. Electroanatomic Correlates of Depolarization Abnormalities in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    NARCIS (Netherlands)

    Tanawuttiwat, Tanyanan; Te Riele, Anneline S J M; Philips, Binu; James, Cynthia A; Murray, Brittney; Tichnell, Crystal; Sawant, Abhishek C; Calkins, Hugh; Tandri, Harikrishna

    2016-01-01

    BACKGROUND: Epsilon waves and other depolarization abnormalities in the right precordial leads are thought to represent delayed activation of the right ventricular outflow tract in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However, no study has directly correlated cardiac e

  12. Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia.

    LENUS (Irish Health Repository)

    Corrado, Domenico

    2010-09-21

    The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy\\/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue.

  13. Impact of genotype on clinical course in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated mutation carriers

    NARCIS (Netherlands)

    Bhonsale, Aditya; Groeneweg, Judith A.; James, Cynthia A.; Dooijes, Dennis; Tichnell, Crystal; Jongbloed, Jan D. H.; Murray, Brittney; Riele, Anneline S. J. M. te; van den Berg, Maarten P.; Bikker, Hennie; Atsma, Douwe E.; de Groot, Natasja M.; Houweling, Arjan C.; van der Heijden, Jeroen F.; Russell, Stuart D.; Doevendans, Pieter A.; van Veen, Toon A.; Tandri, Harikrishna; Wilde, Arthur A.; Judge, Daniel P.; van Tintelen, J. Peter; Calkins, Hugh; Hauer, Richard N.

    2015-01-01

    AIMS: We sought to determine the influence of genotype on clinical course and arrhythmic outcome among arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated mutation carriers. METHODS AND RESULTS: Pathogenic mutations in desmosomal and non-desmosomal genes were identified in

  14. Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnostic task force criteria: impact of new task force criteria

    NARCIS (Netherlands)

    M.G.P.J. Cox; J.J. van der Smagt; M. Noorman; A.C. Wiesfeld; P.G.A. Volders; I.M. van Langen; D.E. Atsma; D. Dooijes; A.C. Houweling; P. Loh; L. Jordaens; Y. Arens; M.J. Cramer; P.A. Doevendans; J.P. van Tintelen; A.A.M. Wilde; R.N.W. Hauer

    2010-01-01

    BACKGROUND: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) Diagnostic Task Force Criteria (TFC) proposed in 1994 are highly specific but lack sensitivity. A new international task force modified criteria to improve diagnostic yield. A comparison of diagnosis by 1994 TFC versus ne

  15. Approach to family screening in arrhythmogenic right ventricular dysplasia/cardiomyopathy

    NARCIS (Netherlands)

    Te Riele, Anneline S J M; James, Cynthia A; Groeneweg, Judith A; Sawant, Abhishek C; Kammers, Kai; Murray, Brittney; Tichnell, Crystal; van der Heijden, Jeroen F; Judge, Daniel P; Dooijes, Dennis; van Tintelen, J Peter; Hauer, Richard N W; Calkins, Hugh; Tandri, Harikrishna

    2015-01-01

    AIMS: A combination of variable expression, age-related penetrance, and unpredictable arrhythmic events complicates management of relatives of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) patients. We aimed to (i) determine predictors of ARVD/C diagnosis and (ii) optimize arrhy

  16. Genetics Home Reference: arrhythmogenic right ventricular cardiomyopathy

    Science.gov (United States)

    ... with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression. Circulation. 2007 Apr ... healthcare professional . About Genetics Home Reference Site Map Customer Support Selection Criteria for Links USA.gov Copyright ...

  17. Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia:Proposed Modification of the Task Force Criteria

    Institute of Scientific and Technical Information of China (English)

    童鸿

    2011-01-01

    @@ The original 1994 International Task Force criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC / D) were based on structural,histological,ECG,arrhythmic,and familial features of the disease (Table 1).Abnormalities were subdivided into major and minor categories according to the specificity of their association with ARVC / D.Right precordial T-wave inversion,though well recognized in ARVC / D,was considered a minor criterion because of its presence in other conditions,including anterior ischemia and right ventricular (RV) hypertrophy.Arrhythmias of RV origin,another cardinal feature of ARVC / D,was designated a minor criterion because of its occurrence in other diseases,particularly idiopathic RV outflow tract tachycardia.

  18. Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy in the Pediatric Population Clinical Characterization and Comparison with Adult-Onset Disease

    NARCIS (Netherlands)

    Te Riele, Anneline S J M; James, Cynthia A.; Sawant, Abhishek C.; Bhonsale, Aditya; Groeneweg, Judith A.; Mast, Thomas P.; Murray, Brittney; Tichnell, Crystal; Dooijes, Dennis; Van Tintelen, J. Peter; Judge, Daniel P.; van der Heijden, Jeroen F.; Crosson, Jane; Hauer, Richard N W; Calkins, Hugh; Tandri, Harikrishna

    2015-01-01

    Objectives The aims of this study were to determine the clinical characteristics and outcomes of pediatric-onset arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and to compare these with those of adult-onset ARVD/C. Background Improved early detection and increased awareness of AR

  19. High interobserver variability in the assessment of epsilon waves : Implications for diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia

    NARCIS (Netherlands)

    Platonov, Pyotr G; Calkins, Hugh; Hauer, Richard N; Corrado, Domenico; Svendsen, Jesper H; Wichter, Thomas; Biernacka, Elżbieta Katarzyna; Saguner, Ardan M; Te Riele, Anneline S J M; Zareba, Wojciech

    2016-01-01

    BACKGROUND: Revision of the Task Force diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) has increased their sensitivity for the diagnosis of early and familial forms of the disease. The epsilon wave is a major diagnostic criterion in the context of ARVC/D, w

  20. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression.

    LENUS (Irish Health Repository)

    Sen-Chowdhry, Srijita

    2007-04-03

    According to clinical-pathological correlation studies, the natural history of arrhythmogenic right ventricular dysplasia\\/cardiomyopathy is purported to progress from localized to global right ventricular dysfunction, followed by left ventricular (LV) involvement and biventricular pump failure. The inevitable focus on sudden death victims and transplant recipients may, however, have created a skewed perspective of a genetic disease. We hypothesized that unbiased representation of the spectrum of disease expression in arrhythmogenic right ventricular dysplasia\\/cardiomyopathy would require in vivo assessment of families in a genetically heterogeneous population.

  1. The Different Substrate Characteristics of Arrhythmogenic Triggers in Idiopathic Right Ventricular Outflow Tract Tachycardia and Arrhythmogenic Right Ventricular Dysplasia: New Insight from Noncontact Mapping.

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    Van Buu Dan Do

    Full Text Available The aim of this study was to investigate the different substrate characteristics of repetitive premature ventricular complexed (PVC trigger sites by the non-contact mapping (NCM.Thirty-five consecutive patients, including 14 with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC and 21 with idiopathic right ventricular outflow tract tachycardia (RVOT VT, were enrolled for electrophysiological study and catheter ablation guided by the NCM. Substrate and electrogram (Eg characteristics of the earliest activation (EA and breakout (BO sites of PVCs were investigated, and these were confirmed by successful PVC elimination.Overall 35 dominant focal PVCs were identified. PVCs arose from the focal origins with preferential conduction, breakout, and spread to the whole right ventricle. The conduction time and distance from EA to BO site were both longer in the ARVC than the RVOT group. The conduction velocity was similar between the 2 groups. The negative deflection of local unipolar Eg at the EA site (EA slope3,5,10ms values was steeper in the RVOT, compared to ARVC patients. The PVCs of ARVC occurred in the diseased substrate in the ARVC patients. More radiofrequency applications were required to eliminate the triggers in ARVC patients.The substrate characteristics of PVC trigger may help to differentiate between idiopathic RVOT VT and ARVC. The slowing and slurred QS unipolar electrograms and longer distance from EA to BO in RVOT endocardium suggest that the triggers of ARVC may originate from mid- or sub-epicardial myocardium. More extensive ablation to the trigger site was required in order to create deeper lesions for a successful outcome.

  2. Clinical study of 39 Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    MA Ke-juan; LI Ning; WANG Hong-tao; CHU Jian-min; FANG Pi-hua; YAO Yan; MA Jian; HUA Wei; ZIIANG Shu; WANG Fang-zheng; Li Zhang; PU Jie-lin

    2009-01-01

    Background There are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). The purpose of this study was to describe the clinical characteristics of ARVD/C patients from China, particularly to define the features of electrocardiograph and treatment outcomes.Methods Thirty-nine patients hospitalized in Fu Wai Cardiovascular Hospital from 1998 to 2006 were included. The data were obtained from the medical archive and the follow-up records.Results Of these patients 33 were male and 6 female (age at the first presentation was (34.9±9.8) years). The most common symptoms were palpitation (62%) and syncope (44%). Right precordial QRSd ≥ 110 ms was detected in 69% of the patients, epsilon wave in 59%, and a ratio of QRSd in V1+V2+V3/V4+V5+V6 ≥ 1.2 in 82%. The most frequent features of electrocardiogram in patients without right bundle-branch block were T-wave inversions and S-wave upstroke in V1-V3 ≥55 ms (96% and 90% of 28 patients, respectively). Radiofrequency catheter ablation (RFCA) for ventricular tachycardia (VT) was successful in 15 (68%) of 22 patients. The recurrence rate of VT was 46% (7/15) during the follow-up of (16.7± 11.2) months. Seven patients had cardioverter/defibrillator (ICD) implanted plus drug therapy and 17 patients took antiarrhythmic drugs alone. During the follow-up of (35.6±19.0) months, all patients with ICD implanted received at least one appropriate ICD shock. One patient died of ventricular fibrillation suddenly and one patient underwent heart transplantation for progressive biventricular heart failure during the drug therapy alone.Conclusions This study demonstrated the clinical and ECG features of the 39 ARVD/C Chinese patients. ICD provided life-saving protection by effectively terminating malignant arrhythmias, and the high recurrence of VT was the major problem of RFCA therapy.

  3. Value of the Signal-Averaged Electrocardiogram in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

    Science.gov (United States)

    Kamath, Ganesh S.; Zareba, Wojciech; Delaney, Jessica; Koneru, Jayanthi N.; McKenna, William; Gear, Kathleen; Polonsky, Slava; Sherrill, Duane; Bluemke, David; Marcus, Frank; Steinberg, Jonathan S.

    2011-01-01

    Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited disease causing structural and functional abnormalities of the right ventricle (RV). The presence of late potentials as assessed by the signal averaged electrocardiogram (SAECG) is a minor Task Force criterion. Objective The purpose of this study was to examine the diagnostic and clinical value of the SAECG in a large population of genotyped ARVC/D probands. Methods We compared the SAECGs of 87 ARVC/D probands (age 37 ± 13 years, 47 males) diagnosed as affected or borderline by Task Force criteria without using the SAECG criterion with 103 control subjects. The association of SAECG abnormalities was also correlated with clinical presentation; surface ECG; VT inducibility at electrophysiologic testing; ICD therapy for VT; and RV abnormalities as assessed by cardiac magnetic resonance imaging (cMRI). Results When compared with controls, all 3 components of the SAECG were highly associated with the diagnosis of ARVC/D (p<0.001). These include the filtered QRS duration (fQRSD) (97.8 ± 8.7 msec vs. 119.6 ± 23.8 msec), low amplitude signal (LAS) (24.4 ± 9.2 msec vs. 46.2 ± 23.7 msec) and root mean square amplitude of the last 40 msec of late potentials (RMS-40) (50.4 ± 26.9 µV vs. 27.9 ± 36.3 µV). The sensitivity of using SAECG for diagnosis of ARVC/D was increased from 47% using the established 2 of 3 criteria (i.e. late potentials) to 69% by using a modified criterion of any 1 of the 3 criteria, while maintaining a high specificity of 95%. Abnormal SAECG as defined by this modified criteria was associated with a dilated RV volume and decreased RV ejection fraction detected by cMRI (p<0.05). SAECG abnormalities did not vary with clinical presentation or reliably predict spontaneous or inducible VT, and had limited correlation with ECG findings. Conclusion Using 1 of 3 SAECG criteria contributed to increased sensitivity and specificity for the diagnosis of ARVC/D. This

  4. Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members

    NARCIS (Netherlands)

    Groeneweg, Judith A.; Bhonsale, Aditya; James, Cynthia A.; Te Riele, Anneline S.; Dooijes, Dennis; Tichnell, Crystal; Murray, Brittney; Wiesfeld, Ans C P; Sawant, Abhishek C.; Kassamali, Bina; Atsma, Douwe E.; Volders, Paul G.; De Groot, Natasja M.; De Boer, Karin; Zimmerman, Stefan L.; Kamel, Ihab R.; van der Heijden, JF; Russell, Stuart D.; Cramer, MJ; Tedford, Ryan J.; Doevendans, Pieter A.; van Veen, AAB; Tandri, Harikrishna; Wilde, Arthur A.; Judge, Daniel P.; Van Tintelen, J. Peter; Hauer, Richard N. W.; Calkins, Hugh

    2015-01-01

    Background Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a progressive cardiomyopathy. We aimed to define long-term outcome in a transatlantic cohort of 1001 individuals. Methods and Results Clinical and genetic characteristics and follow-up data of ARVD/C index-patients (n=4

  5. Maturation-Based Model of Arrhythmogenic Right Ventricular Dysplasia Using Patient-Specific Induced Pluripotent Stem Cells.

    Science.gov (United States)

    Wen, Jian-Yan; Wei, Chuan-Yu; Shah, Khooshbu; Wong, Johnson; Wang, Cheng; Chen, Huei-Sheng Vincent

    2015-01-01

    Cellular reprogramming of somatic cells to patient-specific induced pluripotent stem cells (iPSCs) enables in-vitro modeling of human cardiac disorders for pathogenic and therapeutic investigations. However, using iPSC-derived cardiomyocytes (iPSC-CMs) to model an adult-onset heart disease remains challenging because of the uncertainty regarding the ability of relatively immature iPSC-CMs to fully recapitulate adult disease phenotypes. Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by pathological fibrofatty infiltration and cardiomyocyte (CM) loss predominantly in the right ventricle (RV), leading to heart failure and lethal arrhythmias. Over 50% of affected individuals have desmosome gene mutations, most commonly inPKP2encoding plakophilin-2. Using Yamanaka's pluripotent factors, we generated iPSC lines from ARVD patients withPKP2mutations. We first developed a method to induce metabolic maturation of iPSC-CMs and showed that induction of adult-like metabolic energetics from an embryonic/glycolytic state is essential to model an adult-onset cardiac disease using patient-specific iPSCs. Furthermore, we showed that coactivation of normal peroxisome proliferator-activated receptor (PPAR)-α and abnormal PPARγ pathways in ARVD iPSC-CMs resulted in exaggerated CM lipogenesis, CM apoptosis, Na(+)channel downregulation and defective intracellular calcium handling, recapitulating the pathological signatures of ARVD. Using this model, we revealed novel pathogenic insights that metabolic derangement in an adult-like metabolic milieu underlies ARVD pathologies, enabling us to propose novel disease-modifying therapeutic strategies.

  6. Displasia arritmogénica del ventrículo derecho y enfermedad del nódulo sinusal Arrhythmogenic right ventricular dysplasia and sick sinus syndrome

    Directory of Open Access Journals (Sweden)

    Domingo C. Balderramo

    2004-10-01

    Full Text Available La displasia arritmogénica del ventrículo derecho (DAVD compromete principalmente al ventrículo derecho y debe ser considerada en pacientes jóvenes que presentan síncope, taquicardia ventricular o paro cardíaco y en adultos con insuficiencia cardíaca congestiva. Las alteraciones eléctricas auriculares debidas a DAVD han sido poco descriptas. Informamos el caso de un varón de 60 años con DAVD que durante la evolución presentó enfermedad del nódulo sinusal (tiempo de recuperación del nódulo sinusal de 6113 mseg. Las arritmias auriculares se podrían explicar por el reemplazo gradual de los miocitos auriculares por tejido adiposo.Arrhythmogenic right ventricular dysplasia (ARVD involves primarily the right ventricle, and should be considered in young patients presenting with syncope, ventricular tachycardia, cardiac arrest or in adults patients with congestive heart failure. Atrial electrical abnormalities due to ARVD have been rarely described. We report a case of ARVD in a 60-year-old man who developed sick sinus syndrome during evolution (sinus node recovery time of 6113 mseg. Atrial arrhythmias may be explained by gradual replacement of right atrium myocytes by adipose tissue.

  7. Genetic bases of arrhythmogenic right ventricular cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Alessandra Rampazzo

    2010-05-01

    Full Text Available Arrhythmogenic right ventricular cardiomyopathy (ARVC is a heart muscle disease in which the pathological substrate is a fibro-fatty replacement of the right ventricular myocardium. The major clinical features are different types of arrhythmias with a left branch block pattern. ARVC shows autosomal dominant inheritance with incomplete penetrance. Recessive forms were also described, although in association with skin disorders. Ten genetic loci have been discovered so far and mutations were reported in five different genes. ARVD1 was associated with regulatory mutations of transforming growth factor beta-3 (TGFβ3, whereas ARVD2, characterized by effort-induced polymorphic arrhythmias, was associated with mutations in cardiac ryanodine receptor-2 (RYR2. All other mutations identified to date have been detected in genes encoding desmosomal proteins: plakoglobin (JUP which causes Naxos disease (a recessive form of ARVC associated with palmoplantar keratosis and woolly hair; desmoplakin (DSP which causes the autosomal dominant ARVD8 and plakophilin-2 (PKP2 involved in ARVD9. Desmosomes are important cell-to-cell adhesion junctions predominantly found in epidermis and heart; they are believed to couple cytoskeletal elements to plasma membrane in cell-to-cell or cell-to-substrate adhesions.

  8. Dynamic electrocardiographic changes in patients with arrhythmogenic right ventricular cardiomyopathy.

    LENUS (Irish Health Repository)

    Quarta, Giovanni

    2010-04-01

    Electrocardiographic (ECG) abnormalities of depolarisation and repolarisation contribute to the diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC). The development of diagnostic ECG features were investigated in a genotyped cohort with ARVC to provide more sensitive markers of early disease.

  9. A family with a complex clinical presentation characterized by arrhythmogenic right ventricular dysplasia/cardiomyopathy and features of branchio-oculo-facial syndrome.

    Science.gov (United States)

    Murray, Brittney; Wagle, Rohan; Amat-Alarcon, Nuria; Wilkens, Alisha; Stephens, Paul; Zackai, Elaine H; Goldmuntz, Elizabeth; Calkins, Hugh; Deardorff, Matthew A; Judge, Daniel P

    2013-02-01

    Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial form of cardiomyopathy typically caused by mutations in genes that encode an element of the cardiac desmosome. Branchio-oculo-facial syndrome (BOFS) is a craniofacial disorder caused by TFAP2A mutations. In a family segregating ARVD/C, some members also had features of BOFS. Genetic testing for ARVD/C identified a mutation in PKP2, encoding plakophilin-2, a component of the cardiac desmosome. Evaluation of dysmorphology by chromosome microarray (CMA) identified a 4.4 Mb deletion at chromosome 6p24 that included both TFAP2A and DSP, encoding desmoplakin, an additional component of the cardiac desmosome implicated in ARVD/C. A family member with both the 6p24 deletion and PKP2 mutation had more severe cardiac dysfunction. These findings suggest that this contiguous gene deletion contributes to both ARVD/C and BOFS, and that DSP haploinsufficiency may contribute to cardiomyopathy. This family provides a clinical example that underscores the need for careful evaluation in clinical scenarios where genetic heterogeneity is known to exist. Finally, it suggests that individuals with unexplained cardiomyopathy and dysmorphic facial features may benefit from CMA analysis.

  10. Late presentation of arrhythmogenic right ventricular cardiomyopathy: a case report

    Directory of Open Access Journals (Sweden)

    Papaioannou Georgios I

    2009-08-01

    Full Text Available Abstract Introduction Arrhythmogenic right ventricular cardiomyopathy is an inherited myocardial disease affecting predominantly young people and manifests as sustained ventricular tachycardia with left bundle branch block morphology, sudden death or isolated right or biventricular heart failure. However, its first manifestation as sustained ventricular tachycardia in older patients without preceding symptoms of heart failure is infrequent. To our knowledge, our patient is among the oldest reported in the literature presenting with ventricular tachycardia because of arrhythmogenic right ventricular cardiomyopathy without preceding symptoms of heart failure. Case presentation We present an unusual case of a very late presentation of a right ventricular cardiomyopathy in a 72-year-old white Caucasian man. The patient was admitted with symptoms of weakness, dizziness and chest discomfort for several hours. His electrocardiogram showed a wide-complex tachycardia with left bundle branch block morphology and left axis deviation. Because of continuing hemodynamic instability, the patient was cardioverted to sinus rhythm with a single 300 J shock. His post-cardioversion electrocardiogram, cardiac echocardiogram, coronary angiogram, magnetic resonance imaging and electrophysiological study confirmed the diagnosis of arrhythmogenic right ventricular cardiomyopathy. The patient was treated with an implantable cardioverter defibrillator and discharged on sotalol. Conclusion This case report demonstrates that arrhythmogenic right ventricular cardiomyopathy may have a very late presentation and this diagnosis should be considered as a potential cause of sustained ventricular tachycardia of right ventricular origin among the elderly and should be treated accordingly.

  11. Pregnancy course and outcomes in women with arrhythmogenic right ventricular cardiomyopathy

    NARCIS (Netherlands)

    Hodes, Anke R; Tichnell, Crystal; Te Riele, Anneline S J M; Murray, Brittney; Groeneweg, Judith A; Sawant, Abhishek C; Russell, Stuart D; van Spaendonck-Zwarts, Karin Y; van den Berg, Maarten P; Wilde, Arthur A; Tandri, Harikrishna; Judge, Daniel P; Hauer, Richard N W; Calkins, Hugh; van Tintelen, J Peter; James, Cynthia A

    2016-01-01

    OBJECTIVES: To characterise pregnancy course and outcomes in women with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). METHODS: From a combined Johns Hopkins/Dutch ARVD/C registry, we identified 26 women affected with ARVD/C (by 2010 Task Force Criteria) during 39 singleton preg

  12. Bradyarrhythmias: first presentation of arrhythmogenic right ventricular cardiomyopathy?

    Science.gov (United States)

    Burghouwt, Danielle E; Kammeraad, Janneke Ae; Knops, Paul; du Plessis, Frederik A; de Groot, Natasja Ms

    2015-04-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder characterized by progressive replacement of myocardial cells by fibro-fatty tissue giving rise to ventricular tachyarrhythmias. In this case report, we describe a pediatric patient with sinoatrial arrests and second degree atrioventricular conduction block several years before ARVC became apparent. These findings suggest that bradyarrhythmias can also be the first expression of ARVC.

  13. Right Ventricular Imaging and Computer Simulation for Electromechanical Substrate Characterization in Arrhythmogenic Right Ventricular Cardiomyopathy

    NARCIS (Netherlands)

    Mast, TP; Teske, Arco J; Walmsley, John; van der Heijden, Jeroen F; van Es, René; Prinzen, Frits W; Delhaas, Tammo; van Veen, Toon A; Loh, KP; Doevendans, Pieter A; Cramer, Maarten J; Lumens, Joost

    2016-01-01

    BACKGROUND: Previous studies suggested that electrical abnormalities precede overt structural disease in arrhythmogenic right ventricular cardiomyopathy (ARVC). Abnormal RV deformation has been reported in early ARVC without structural abnormalities. The pathophysiological mechanisms underlying thes

  14. ATIPICIDADES EN UN CASO CON DISPLASIA ARRITMOGÉNICA DEL VENTRÍCULO DERECHO O ENFERMEDAD DE UHL / Report of an atypical case with arrhythmogenic right ventricular dysplasia or Uhl´s anomaly

    Directory of Open Access Journals (Sweden)

    Aníbal González Trujillo

    2012-10-01

    Full Text Available ResumenLa displasia arritmogénica del ventrículo derecho es una miocardiopatía caracterizada por arritmias ventriculares malignas y anomalías estructurales progresivas, que afectan primariamente al ventrículo derecho. Se presenta por una sustitución progresiva parcial o masiva del miocardio por tejido adiposo o fibroadiposo. La enfermedad de Uhl puede ser una manifestación extrema y generalizada de la displasia arritmogénica del ventrículo derecho, trastorno congénito muy poco frecuente con ausencia de miocardio ventricular derecho, por lo que sus paredes son delgadas como el papel. Se comenta el caso de un paciente masculino de 56 años que presentó pérdida de conocimiento y se le realizó el diagnóstico clínico y ecocardiográfico. Se discuten las características clínicas, el diagnóstico y la conducta a seguir ante esta cardiopatía potencialmente letal en pacientes que sufren síncope, taquicardia ventricular o parada cardíaca. / AbstractArrhythmogenic right ventricular dysplasia is a cardiomyopathy characterized by malignant ventricular arrhythmias and progressive structural abnormalities, affecting primarily the right ventricle. It appears due to a partial or massive progressive replacement of the myocardium by fibroadipose or adipose tissue. Uhl's disease may be an extreme and widespread manifestation of arrhythmogenic right ventricular dysplasia, a rare congenital disorder with absence of right ventricular myocardium, so that its walls are paper thin. The case of a 56 year old male patient who had loss of consciousness and underwent clinical and echocardiographic diagnosis is presented. The clinical features, diagnosis and action to take against this potentially fatal heart disease in patients with syncope, ventricular tachycardia or cardiac arrest are discussed.

  15. Cardiac sarcoidosis mimicking right ventricular dysplasia.

    Science.gov (United States)

    Shiraishi, Jun; Tatsumi, Tetsuya; Shimoo, Kazutoshi; Katsume, Asako; Mani, Hiroki; Kobara, Miyuki; Shirayama, Takeshi; Azuma, Akihiro; Nakagawa, Masao

    2003-02-01

    A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.

  16. Missense variants in plakophilin-2 in arrhythmogenic right ventricular cardiomyopathy patients--disease-causing or innocent bystanders?

    DEFF Research Database (Denmark)

    Christensen, Alex Hørby; Benn, Marianne; Tybjærg-Hansen, Anne;

    2010-01-01

    Objectives: Mutations in genes encoding desmosomal proteins have been linked to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). We hypothesized that a Scandinavian ARVC/D population would have a different spectrum of plakophilin-2 (PKP2) mutations and that some of the reported...

  17. Arrhythmogenic right ventricular cardiomyopathy--time for Nordic cooperation!

    Science.gov (United States)

    Gjesdal, Knut

    2008-12-01

    This editorial discusses arrhythmogenic right ventricular cardiomyopathy (ARVC) with respect to diagnosis and organisation of patient care. Two papers in the current issue are commented upon. Aneq and coworkers present a long-term echocardiographic study on ARVC patients. Baseline changes were seldom diagnostic, but over years, changes in right ventricular structure and function occurred; the most consistent being increasing diameter of the right ventricular outflow tract. Haapalaita and coworkers used body surface ECG, comparing right and left ventricular types of ECG. The duration of electrical systole (QT-end) and the dispersion of the action potentials (QT peak-end) was longer in the right-sided compared to the left-sided leads in ARVC, at variance from in healthy controls, and the shortening effect of autonomic manoeuvres that activate sympathetic tone, was much more marked on the right side, compatible with the tendency of arrhythmias to occur under stress. An initiative to create a Nordic registry on ARVC has come from Denmark. This is highly welcomed; our individual institutions are too small to gain the experience needed for optimal patient care.

  18. Advanced quantitative echocardiography in arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Hastrup Svendsen, Jesper; Sogaard, Peter

    2007-01-01

    BACKGROUND: Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a regional disease of the RV myocardium with variable degrees of left ventricular involvement. Three-dimensional echocardiography and Doppler tissue imaging (DTI) are new echocardiographic modalities for the evaluation...... of global and regional function, but the diagnostic potential remains to be assessed. METHODS: Twenty patients with previously established ARVC were evaluated by 3-dimensional echocardiography and DTI, and compared with 32 age- and sex-matched control subjects. RESULTS: Using 3-dimensional echocardiography...... showed decreased regional systolic strain, but with wide variation in the measurements. CONCLUSION: Three-dimensional echocardiography identifies decreased RV ejection fraction in ARVC. Assessment of regional contractility by DTI is limited by wide variation. Echocardiographic evaluation...

  19. Case study of right ventricle arrhythmogenic dysplasia within one family

    Directory of Open Access Journals (Sweden)

    Bockeria L. A.

    2012-03-01

    Elder child had manifestations of end-stage heart failure which required heart transplantation associated with using the assisted circulation system. The second child manifested heart rhythm and conduction disturbances along with reduction of right ventricular myocardial contractility. He underwent subsequent implantation of cardioverter-defibrillator. The youngest child had no clinical signs of the disease.

  20. Usefulness of immunostaining for plakoglobin as a diagnostic marker of arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Munkholm, Julie; Christensen, Alex H; Svendsen, Jesper H;

    2012-01-01

    The clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is often challenging due to phenotypic variation, reduced/age-related penetrance, and lack of a diagnostic test. A single report has suggested quantitative myocardial immunoanalysis for the desmosomal protein...

  1. Arrhythmogenic right ventricular cardiomyopathy a still underrecognized clinic entity.

    Science.gov (United States)

    Thiene, G; Basso, C; Danieli, G; Rampazzo, A; Corrado, D; Nava, A

    1997-04-01

    Arrhythmogenic right ventricular cardiomyopathy is a new morbid entity that was discovered thanks to the study of sudden death in the young. This heart muscle disease is characterized by myocardial atrophy, mostly of the right ventricle, with massive fibro-fatty infiltration, accounting for ventricular electrical instability at risk of severe arrhythmias and even cardiac arrest. The disease was found to be the major cause of sudden death in young people and athletes in the Veneto Region, Italy. A familial occurrence with autosomal dominant transmission was then discovered, and the prevalence was estimated to be higher than 1 in 5000. The disease is genetically heterogeneous: Linkage analysis, carried out in a large family with recurrence of sudden deaths, led to map the gene to chromosome 14q23-q24. Linkage analysis in a second family allowed mapping of another gene to chromosome 1q42-q43. Clinical diagnosis can be achieved through electrocardiography, echocardiography, angiocardiography, magnetic resonance imaging, and endomyocardial biopsy. Diagnostic criteria have been put forward by a committee of the International Society and Federation of Cardiology. The disease was recently included among the cardiomyopathies in the revised World Health Organization (WHO) classification. Study of the natural history allowed us to distinguish (a) a covert phase in apparently normal subjects who have a risk of abrupt electrical instability and sudden death, (b) an overt arrhythmic phase with palpitations and impending cardiac arrest, (c) congestive heart failure with pump depression, sometimes so severe as to require heart transplantation. Both the etiology and pathogenesis of the disease are unknown. In particular, the mechanisms leading to progressive loss of myocardium and fibro-fatty replacement are still speculative. Apoptosis in the right ventricle occurring not only in infancy, as in the normal heart, but also in childhood and adulthood might account for the progressive

  2. Clinical and familial study of arrhythmogenic right ventricular cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    单其俊; 曹克将; 黄元铸; 廖铭扬; 陈明龙; 李闻奇; 邹建刚; 朱必顺; 马文珠

    2001-01-01

    Objective To explore the characteristics of arrhythmogenic rightventricular cardiomyopathy (ARVC). Methods Seven patients with arrhythmogenic right ventricular cardiomyopathy and 34 members of three families were studied. All patients and family members underwent history collection, clinical examination, electrocardiogram (ECG), two-dimensional echocardiography (2-DE) and a signal averaging electrocardiogram. Programmed ventricular stimulation was performed in five patients. Results All patients and family members had normal morphologic characteristics and normal function of the left ventricular by 2-DE. Fourteen persons had abnormal findings indicating ARVC. Five had enlargement of the right ventricular with diffused hypocontractility, eight had thin and systolic bulging in the focal anterior wall with hypokinesia and one had bulging of the inferior wall. Twenty-five persons (seven patients and 18 family members) had abnormal findings in ECG. Positive ventricular late potential was recorded in 13 persons (six patients). Two to three monomorphic ventricular tachycardia (VT) with left bundle branch block (LBBB) configurations were induced in five patients. Ventricular fibrillation was induced in two patients during the electrophysiologic study (EPS). Five patients had very high pacing threshold and/or ineffective pacing in one or many regions of the right ventricle. Two members of one family died suddenly. One member was a dwarf with ARVC. Spontaneous VT with a left bundle branch block (LBBB) configuration was recorded in five patients, polymorphic VT with extremely short coupling interval in one, and premature ventricular complexes with LBBB configuration in 12 (six patients). Conclusion Our familial study strongly suggests that ARVC may be a hereditary disease and it is helpful in the diagnosis and detection of ARVC. The most common manifestations were abnormal structure and function of the right ventricle and abnormal ECG of repolarization and ventricular

  3. Arrhythmogenic right ventricular cardiomyopathy: considerations from in silico experiments

    Directory of Open Access Journals (Sweden)

    Ronald eWilders

    2012-05-01

    Full Text Available Objective: Arrhythmogenic right ventricular cardiomyopathy (ARVC is associated with remodelling of gap junctions and also, although less well-defined, down-regulation of the fast sodium current. The gap junction remodelling and down-regulation of sodium current have been proposed as contributors to arrhythmo¬genesis in ARVC by slowing conduction. The objective of the present study was to assess the amount of conduction slowing due to the observed gap junction remodelling and down-regulation of sodium current.Methods: The effects of (changes in gap junctional conductance, cell dimensions and sodium current on both longitudinal and transversal con¬duction velocity were tested by simulating action potential propagation in linear strands of human ventricular cells that were either arranged end-to-end or side-by-side.Results: A 50% reduction in gap junction content, as commonly observed in ARVC, gives rise to an 11% decrease in longitudinal conduction velocity and a 29% decrease in transverse conduction velocity. A down-regulation of the sodium current through a 50% decrease in peak current density as well as a −15 mV shift in steady-state inactivation, as observed in an experimental model of ARVC, decreases conduction velocity in either direction by 32%. In combination, the gap junction remodelling and down-regulation of sodium current result in a 40% decrease in longitudinal conduction velocity and a 52% decrease in transverse conduction velocity.Conclusions: The gap junction remodelling and down-regulation of sodium current do result in conduction slowing, but heterogeneity of gap junction remodelling, in combination with down-regulation of sodium current, rather than gap junction remodelling per se may be a critical factor in arrhythmogenesis in ARVC.

  4. 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.)

  5. Arrhythmogenic cardiomyopathy : diagnosis, genetic background, and risk management

    NARCIS (Netherlands)

    Groeneweg, J. A.; van der Heijden, J. F.; Dooijes, D.; van Veen, T. A. B.; van Tintelen, J. P.; Hauer, R. N.

    2014-01-01

    Arrhythmogenic cardiomyopathy (AC), also known as arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), is a hereditary disease characterised by ventricular arrhythmias, right ventricular and/or left ventricular dysfunction, and fibrofatty replacement of cardiomyocytes. Patients with A

  6. Right ventricular strain by MR quantitatively identifies regional dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy

    NARCIS (Netherlands)

    Vigneault, Davis M; te Riele, Anneline S J M; James, Cynthia A; Zimmerman, Stefan L; Selwaness, Mariana; Murray, Brittney; Tichnell, Crystal; Tee, Michael; Noble, J Alison; Calkins, Hugh; Tandri, Harikrishna; Bluemke, David A

    2016-01-01

    BACKGROUND: Analysis of regional wall motion of the right ventricle (RV) is primarily qualitative with large interobserver variation in clinical practice. Thus, the purpose of this study was to use feature tracking to analyze regional wall motion abnormalities in patients with arrhythmogenic right v

  7. Abnormal atrial activation is common in patients with arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Platonov, Pyotr G; Christensen, Alex H; Holmqvist, Fredrik;

    2011-01-01

    INTRODUCTION: Structural right atrial abnormalities have been described in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, little is known about electrocardiographic signs of atrial involvement in ARVC because no systematic studies have been conducted. METHODS: P......%, whereas 15 patients (37%) had atypical P-wave positive in all 3 leads (P right ventricular abnormality. CONCLUSIONS: Patients with ARVC commonly demonstrate deteriorated...... atrial activation expressed either as prolonged P-wave duration or abnormal P-wave morphology. The P-wave abnormalities were not secondary to right ventricular dilatation. These findings show that atrial involvement is common in ARVC and may represent yet another manifestation of the disease...

  8. Mutation analysis and evaluation of the cardiac localization of TMEM43 in arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Christensen, A H; Andersen, C B; Tybjærg-Hansen, A;

    2011-01-01

    Christensen AH, Andersen CB, Tybjærg-Hansen A, Haunso S, Svendsen JH. Mutation analysis and evaluation of the cardiac localization of TMEM43 in arrhythmogenic right ventricular cardiomyopathy. A single report has associated mutations in TMEM43 (LUMA) with a distinctive form of arrhythmogenic right...... with anti-TMEM43, anti-plakoglobin, anti-plakophilin-2, anti-connexin-43, and anti-emerin antibodies was performed on myocardium from TMEM43-positive patients (n = 3) and healthy controls (n = 3). The genetic screening identified heterozygous variants in two families: one reported mutation (c.1073C> T...

  9. Transcatheter closure of atrial septal defect associated with arrhythmogenic right ventricular cardiomyopathy: a case report and literature review.

    Science.gov (United States)

    Wang, Haiyan; Ding, Hongyu; Lei, Lei; Zhang, Xiaohong; Gong, Yuling; Hou, Yinglong

    2015-03-01

    Arrhythmogenic right ventricular cardiomyopathy is characterised by progressive, fibrofatty replacement of myocardium, and ventricular arrhythmias, and its prognosis is usually poor. Arrhythmogenic right ventricular cardiomyopathy associated with atrial septal defect is very rare, and this combination may make the diagnosis, treatment, and prognosis difficult. We present a case of a patient with this association who underwent interventional treatment with a septal defect occluder. Transcatheter closure of atrial septal defect in a patient with arrhythmogenic right ventricular cardiomyopathy is hitherto unreported. During a 3-year follow-up he remained relatively stable. We also review the cases reported in the medical literature describing this uncommon association between arrhythmogenic right ventricular cardiomyopathy and atrial septal defect or patent foramen ovale.

  10. [Right ventricular dysplasia and sudden death in young people].

    Science.gov (United States)

    Maresi, E; Albano, N; Procaccianti, P; Campesi, G

    1990-06-01

    In this study two autoptic cases of right ventricular dysplasia, observed in young and asymptomatic subjects who died suddenly are reported. In these patients, the "primary" myocardial atrophy involved the right ventricle, the right atrium and the conduction system: the sinoatrial node and the internodal pathways (in both cases) and the hisian bifurcation (only in the second case). The primary ventricular dysplasia was always associated with "secondary" transmural hyperplasia of the sub-epicardial fat tissue except for the anterior wall of the pulmonary infundibulum. According to the findings observed, we think that: 1) in all cases of right ventricular dysplasia a careful examination of the conduction system must be performed due to the fact that the lethal arrhythmias can be not only "hyperkinetic" but also "hypokinetic" arrhythmias; 2) right ventricular dysplasia is a dysplastic-congenital disease and transmural fatty hyperplasia depends both on "primary" myocardial atrophy and on the presence of sub-epicardial fat tissue; the latter is related to the age of the patient.

  11. Outflow tract segmental arrhythmogenic right ventricular cardiomyopathy in an English Bulldog.

    Science.gov (United States)

    Santilli, Roberto A; Bontempi, Luigi V; Perego, Manuela; Fornai, Lara; Basso, Cristina

    2009-06-01

    A 5-year-old English Bulldog was presented for acute onset of syncope and fatigue caused by sustained ventricular tachycardia with left bundle block morphology and inferior axis. This arrhythmia had the electrocardiographic features of a ventricular tachycardia arising from the right ventricular outflow tract (RVOT), as described in an experimental canine model and in people. Since a RVOT aneurysm was identified by echocardiography, a segmental form of arrhythmogenic right ventricular cardiomyopathy (ARVC) was suspected. Gross examination of the heart confirmed the bulging of the RVOT and histological examination of the ventricular myocardium revealed segmental involvement of the RVOT with transmural fibro-fatty degeneration. To the authors' knowledge, this is the first reported case of AVRC in an English Bulldog and the first example of segmental AVRC described in the dog.

  12. Sudden death due to arrhythmogenic right ventricular cardiomyopathy: Two case reports

    Institute of Scientific and Technical Information of China (English)

    CHEN Xinshan; ZHANG Yigu; RAO Guangxun; HUANG Guangzhao

    2007-01-01

    Arrhythmogenic right ventricular cardiomyopathy(ARVC)is a kind of primary myocardial disease characterized by the regional or global replacement of right ventricular myocardium by fatty and fibrolipomatous tissues.The ARVC,usually presenting with difrerent clinical manifestations and pathological changes,were mainly seen in young men and is one of the main causes of sudden death in the young.Here two autopsied cases of Chinese men aged 30 and 23 years old who appeared healthy but died suddenly while at work arc reported respectively.One of the victims had extensive and severe pathological changes in his heart involving the left ventricular wall as well as the ventricular septum and the right atrium.Not only was there a global fatty and fibrolipomatous tissue replacement of the right ventricular myocardia,but also mild sarcoplasmic coagulation in the myocardium and focal lymphocytic infiltration in the myocardial interstitium of the right ventricular wall.In addition,slight atherosclerosis of the coronary artery and intimal thickening of the sino-atrial node were observed.It is believed that there are no marked differences in the pathological changes of ARVC between Chinese patients and patients from western countries.The etiology and pathogenesis of ARVC could not be explained by a single cause or factor and they are probably related to various congenital and acquired causes or factors.

  13. Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy

    DEFF Research Database (Denmark)

    Ruwald, Anne-Christine; Marcus, Frank; Estes, N A Mark;

    2015-01-01

    AIMS: It has been proposed that competitive sport increases the risk of ventricular tachyarrhythmias (VTA) and death in patients with arrhythmogenic right-ventricular cardiomyopathy (ARVC). However, it is unknown whether this only applies to competitive sport or if recreational sports activity al...

  14. Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges

    Institute of Scientific and Technical Information of China (English)

    Bruno; Pinamonti; Francesca; Brun; Luisa; Mestroni; Gianfranco; Sinagra

    2014-01-01

    Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement. Diagnosis of ARVC remains a clinical challenge mainly at its early stages and in patients with minimal echocardiographic right ventricular(RV) abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow ventricular tachycardia, Brugada syndrome, and myocarditis, due to arrhythmic expressivity and biventricular involvement. The identification of ARVC can be often challenging, because of the heterogeneous clinical presentation, highly variable intra- and inter-family expressivity and incomplete penetrance. This genotypephenotype “plasticity” is largely unexplained. A familial history of ARVC is present in 30% to 50% of cases, and the disease is considered a genetic cardiomyopathy, usually inherited in an autosomal dominant pattern with variable penetrance and expressivity; in addition, autosomal recessive forms have been reported(Naxos disease and Carvajal syndrome). Diagnosis of ARVC relays on a scoring system, with major or minorcriteria on the Revised Task Force Criteria. Implantable cardioverter defibrillators(ICDs) are increasingly utilized in patients with ARVC who have survived sudden death(SD)(secondary prevention). However, there are few data available to help identifying ARVC patients in whom the prophylactic implantation of an ICD is truly warranted. Prevention of SD is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are the mainstay of treatment of ARVC.

  15. Fast rate (≥ 250 beats/min) right ventricular burst stimulation is useful for ventricular tachycardia induction in arrhythmogenic right ventricular cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Ling-Min WU; Jing-Ru BAO; Yan YAO; Bing-Bo HOU; Li-Hui ZHENG; Shu ZHANG

    2016-01-01

    Background One of the major challenges in arrhythmogenic right ventricular cardiomyopathy (ARVC) ablation is ventricular tachy-cardia (VT) non-inducibility. The study aimed to assess whether fast rate (≥ 250 beats/min) right ventricular burst stimulation was useful for VT induction in patients with ARVC.Methods Ninety-one consecutive ARVC patients with clinical sustained VT that underwent electro-physiological study were enrolled. The stimulation protocol was implemented at both right ventricular apex and outflow tract as follows: Step A, up to double extra-stimuli; Step B, incremental stimulation with low rate (< 250 beats/min); Step C, burst stimulation with fast rate (≥ 250 beats/min); Step D, repeated all steps above with intravenous infusion of isoproterenol.Results A total of 76 patients had inducible VT (83.5%), among which 49 were induced by Step C, 15 were induced by Step B, 8 and 4 by Step A and D, respectively. Clinical VTs were induced in 60 patients (65.9%). Only two spontaneously ceased ventricular fibrillations were induced by Step C. Multivariate analysis showed that a narrower baseline QRS duration under sinus rhythm was independently associated with VT non-inducibility (OR: 1.1; 95% CI: 1.0–1.1;P = 0.019).ConclusionFast rate (≥ 250 beats/min) right ventricular burst stimulation provides a useful supplemental method for VT induction in ARVC patients.

  16. Nationwide (Denmark) Study of Symptoms Preceding Sudden Death due to Arrhythmogenic Right Ventricular Cardiomyopathy

    DEFF Research Database (Denmark)

    Sadjadieh, Golnaz; Jabbari, Reza; Risgaard, Bjarke

    2014-01-01

    In this study, we investigated medical history and symptoms before death in all subjects aged 1 to 35 years who died a sudden cardiac death (SCD) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in Denmark in the years 2000 to 2006. All deaths (n=6,629) in subjects aged 1 to 35 years...... in Denmark in the period 2000 to 2006 were included. A total of 16 cases of SCD due to ARVC were identified based on histopathologic examination. Information on medical history was retrieved from The National Patient Registry, general practitioners, and hospitals. Symptoms before death were compared with 2...... control groups in the same age group and time interval: one consisting of subjects who died in traffic accidents (n=74) and the other consisting of patients who died a SCD due to coronary artery disease (CAD; n=34). In the case group, 8 of the 16 patients with ARVC experienced antecedent cardiac symptoms...

  17. Right ventricular strain by MR quantitatively identifies regional dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy

    NARCIS (Netherlands)

    D.M. Vigneault (Davis M.); A.S. Te Riele (Anneline S.); C.A. James (Cynthia A.); S.L. Zimmerman (Stefan L.); M. Selwaness (Mariana); B. Murray (Brittney); C. Tichnell (Crystal); M. Tee (Michael); J.A. Noble (J. Alison); H. Calkins (Hugh); H. Tandri (Harikrishna); D.A. Bluemke (David)

    2016-01-01

    textabstractBackground Analysis of regional wall motion of the right ventricle (RV) is primarily qualitative with large interobserver variation in clinical practice. Thus, the purpose of this study was to use feature tracking to analyze regional wall motion abnormalities in patients with arrhythmoge

  18. Identification of a PKP2 gene deletion in a family with arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Li Mura, Ilena Egle Astrid; Bauce, Barbara; Nava, Andrea; Fanciulli, Manuela; Vazza, Giovanni; Mazzotti, Elisa; Rigato, Ilaria; De Bortoli, Marzia; Beffagna, Giorgia; Lorenzon, Alessandra; Calore, Martina; Dazzo, Emanuela; Nobile, Carlo; Mostacciuolo, Maria Luisa; Corrado, Domenico; Basso, Cristina; Daliento, Luciano; Thiene, Gaetano; Rampazzo, Alessandra

    2013-11-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary heart muscle disease characterized by progressive myocardial loss, with fibro-fatty replacement, and high frequency of ventricular arrhythmias that can lead to sudden cardiac death. ARVC is a genetically determined disorder, usually caused by point mutations in components of the cardiac desmosome. Conventional mutation screening of ARVC genes fails to detect causative mutations in about 50% of index cases, suggesting a further genetic heterogeneity. We performed a genome-wide linkage study and a copy number variations (CNVs) analysis, using high-density SNP arrays, in an ARVC family showing no mutations in any of the desmosomal genes. The CNVs analysis identified a heterozygous deletion of about 122 kb on chromosome 12p11.21, including the entire plakophilin-2 gene and shared by all affected family members. It was not listed on any of available public CNVs databases and was confirmed by quantitative real-time PCR. This is the first SNP array-based genome-wide study leading to the identification of a CNV segregating with the disease phenotype in an ARVC family. This result underscores the importance of performing additional analysis for possible genomic deletions/duplications in ARVC patients without point mutations in known disease genes.

  19. De novo desmin-mutation N116S is associated with arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Klauke, Baerbel; Kossmann, Sabine; Gaertner, Anna; Brand, Kristina; Stork, Ines; Brodehl, Andreas; Dieding, Mareike; Walhorn, Volker; Anselmetti, Dario; Gerdes, Désirée; Bohms, Birte; Schulz, Uwe; Zu Knyphausen, Edzard; Vorgerd, Matthias; Gummert, Jan; Milting, Hendrik

    2010-12-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease, frequently accompanied by sudden cardiac death and terminal heart failure. Genotyping of ARVC patients might be used for palliative treatment of the affected family. We genotyped a cohort of 22 ARVC patients referred to molecular genetic screening in our heart center for mutations in the desmosomal candidate genes JUP, DSG2, DSC2, DSP and PKP2 known to be associated with ARVC. In 43% of the cohort, we found disease-associated sequence variants. In addition, we screened for desmin mutations and found a novel desmin-mutation p.N116S in a patient with ARVC and terminal heart failure, which is located in segment 1A of the desmin rod domain. The mutation leads to the aggresome formation in cardiac and skeletal muscle without signs of an overt clinical myopathy. Cardiac aggresomes appear to be prominent, especially in the right ventricle of the heart. Viscosimetry and atomic force microscopy of the desmin wild-type and N116S mutant isolated from recombinant Escherichia coli revealed severe impairment of the filament formation, which was supported by transfections in SW13 cells. Thus, the gene coding for desmin appears to be a novel ARVC gene, which should be included in molecular genetic screening of ARVC patients.

  20. The value of magnetic resonance imaging for the diagnosis of arrhythmogenic right ventricular cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Maksimovic, Ruzica [Kerckhoff Heart Center, Department of Cardiology, Bad Nauheim (Germany); Clinical Center of Serbia, Department of Cardiology, Institute for Cardiovascular Diseases, Belgrade (Czechoslovakia); Ekinci, Okan; Reiner, Christian; Hamm, Christian W.; Pitschner, Heinz-F.; Dill, Thorsten [Kerckhoff Heart Center, Department of Cardiology, Bad Nauheim (Germany); Bachmann, Georg F. [Kerckhoff Heart Center, Department of Radiology, Bad Nauheim (Germany); Seferovic, Petar M.; Ristic, Arsen D. [Clinical Center of Serbia, Department of Cardiology, Institute for Cardiovascular Diseases, Belgrade (Czechoslovakia)

    2006-03-15

    This study evaluated the diagnostic significance of a magnetic resonance imaging (MRI) based scoring model for identification of arrhythmogenic right ventricular cardiomyopathy (ARVC) in patients with MRI evidence of RV abnormalities. Fifty-three patients with RV myocardial abnormalities on MRI were divided into a group with ARVC 1 (n=17) and a group with other RV arrhythmias (n=37). Decision tree learning (DTL) and linear classification (based on a modified ARVC scoring model of major and minor criteria) were used to identify and assess MRI criterion information value, and to induce ARVC diagnostic rules. All major ARVC criteria were more frequent in the ARVC group. Among minor criteria regional RV hypokinesia, mild segmental RV dilatation, and prominent trabeculae were more frequent in the ARVC group while mild global RV dilatation was more frequent in the non-ARVC group. RV aneurysm achieved highest importance in ARVC diagnosis (predictive accuracy 76.8%). Better diagnostic accuracy (sensitivity 93.3%, specificity 89.5%) was achieved when the MRI score for the major and minor criteria reached threshold value of four: two major criteria, or one major and two minor, or four minor criteria. Combinations between major and minor criteria contributed to a statistically valid model for ARVC diagnosis. (orig.)

  1. Genetic Fate Mapping Identifies Second Heart Field Progenitor Cells As a Source of Adipocytes in Arrhythmogenic Right Ventricular Cardiomyopathy

    Science.gov (United States)

    Lombardi, Raffaella; Dong, Jinjiang; Rodriguez, Gabriela; Bell, Achim; Leung, Tack Ki; Schwartz, Robert J.; Willerson, James T.; Brugada, Ramon; Marian, Ali J.

    2009-01-01

    The phenotypic hallmark of arrhythmogenic right ventricular cardiomyopathy, a genetic disease of desmosomal proteins, is fibroadipocytic replacement of the right ventricle. Cellular origin of excess adipocytes, the responsible mechanism(s) and the basis for predominant involvement of the right ventricle are unknown. We generated 3 sets of lineage tracer mice regulated by cardiac lineage promoters α-myosin heavy chain (αMyHC), Nkx2.5, or Mef2C. We conditionally expressed the reporter enhanced yellow fluorescent protein while concomitantly deleting the desmosomal protein desmoplakin in cardiac myocyte lineages using the Cre-LoxP technique. Lineage tracer mice showed excess fibroadiposis and increased numbers of adipocytes in the hearts. Few adipocytes in the hearts of αMyHC-regulated lineage tracer mice, but the majority of adipocytes in the hearts of Nkx2.5- and Mef2C-regulated lineage tracer mice, expressed enhanced yellow fluorescent protein. In addition, rare cells coexpressed adipogenic transcription factors and the second heart field markers Isl1 and Mef2C in the lineage tracer mouse hearts and in human myocardium from patients with arrhythmogenic right ventricular cardiomyopathy. To delineate the responsible mechanism, we generated transgenic mice expressing desmosomal protein plakoglobin in myocyte lineages. Transgene plakoglobin translocated to nucleus, detected by immunoblotting and immunofluorescence staining and coimmunoprecipitated with Tcf7l2, a canonical Wnt signaling transcription factor. Expression levels of canonical Wnt/Tcf7l2 targets bone morphogenetic protein 7 and Wnt5b, which promote adipogenesis, were increased and expression level of connective tissue growth factor, an inhibitor of adipogenesis, was decreased. We conclude adipocytes in arrhythmogenic right ventricular cardiomyopathy originate from the second heart field cardiac progenitors, which switch to an adipogenic fate because of suppressed canonical Wnt signaling by nuclear

  2. Vigorous physical activity impairs myocardial function in patients with arrhythmogenic right ventricular cardiomyopathy and in mutation positive family members

    DEFF Research Database (Denmark)

    Saberniak, Jørg; Hasselberg, Nina E; Borgquist, Rasmus;

    2014-01-01

    patients and 45 mutation-positive family members. Athletes were defined as subjects with ≥4 h vigorous exercise/week [≥1440 metabolic equivalents (METs × minutes/week)] during a minimum of 6 years. Athlete definition was fulfilled in 37/110 (34%) subjects. We assessed right ventricular (RV) and left......AIMS: Exercise increases risk of ventricular arrhythmia in subjects with arrhythmogenic right ventricular cardiomyopathy (ARVC). We aimed to investigate the impact of exercise on myocardial function in ARVC subjects. METHODS AND RESULTS: We included 110 subjects (age 42 ± 17 years), 65 ARVC...... family members (47 ± 3% vs. 52 ± 6%, P 

  3. In vitro functional analyses of arrhythmogenic right ventricular cardiomyopathy-associated desmoglein-2-missense variations.

    Directory of Open Access Journals (Sweden)

    Anna Gaertner

    Full Text Available BACKGROUND: Although numerous sequence variants in desmoglein-2 (DSG2 have been associated with arrhythmogenic right ventricular cardiomyopathy (ARVC, the functional impact of new sequence variations is difficult to estimate. METHODOLOGY/PRINCIPAL FINDINGS: To test the functional consequences of DSG2-variants, we established an expression system for the extracellular domain and the full-length DSG2 using the human cell line HT1080. We established new tools to investigate ARVC-associated DSG2 variations and compared wild-type proteins and proteins with one of the five selected variations (DSG2-p.R46Q, -p.D154E, -p.D187G, -p.K294E, -p.V392I with respect to prodomain cleavage, adhesion properties and cellular localisation. CONCLUSIONS/SIGNIFICANCE: The ARVC-associated DSG2-p.R46Q variation was predicted to be probably damaging by bioinformatics tools and to concern a conserved proprotein convertase cleavage site. In this study an impaired prodomain cleavage and an influence on the DSG2-properties could be demonstrated for the R46Q-variant leading to the classification of the variant as a potential gain-of-function mutant. In contrast, the variants DSG2-p.K294E and -p.V392I, which have an arguable impact on ARVC pathogenesis and are predicted to be benign, did not show functional differences to the wild-type protein in our study. Notably, the variants DSG2-p.D154E and -p.D187G, which were predicted to be damaging by bioinformatics tools, had no detectable effects on the DSG2 protein properties in our study.

  4. In Vitro Functional Analyses of Arrhythmogenic Right Ventricular Cardiomyopathy-Associated Desmoglein-2-Missense Variations

    Science.gov (United States)

    Gaertner, Anna; Klauke, Baerbel; Stork, Ines; Niehaus, Karsten; Niemann, Gesa; Gummert, Jan; Milting, Hendrik

    2012-01-01

    Background Although numerous sequence variants in desmoglein-2 (DSG2) have been associated with arrhythmogenic right ventricular cardiomyopathy (ARVC), the functional impact of new sequence variations is difficult to estimate. Methodology/Principal Findings To test the functional consequences of DSG2-variants, we established an expression system for the extracellular domain and the full-length DSG2 using the human cell line HT1080. We established new tools to investigate ARVC-associated DSG2 variations and compared wild-type proteins and proteins with one of the five selected variations (DSG2-p.R46Q, -p.D154E, -p.D187G, -p.K294E, -p.V392I) with respect to prodomain cleavage, adhesion properties and cellular localisation. Conclusions/Significance The ARVC-associated DSG2-p.R46Q variation was predicted to be probably damaging by bioinformatics tools and to concern a conserved proprotein convertase cleavage site. In this study an impaired prodomain cleavage and an influence on the DSG2-properties could be demonstrated for the R46Q-variant leading to the classification of the variant as a potential gain-of-function mutant. In contrast, the variants DSG2-p.K294E and -p.V392I, which have an arguable impact on ARVC pathogenesis and are predicted to be benign, did not show functional differences to the wild-type protein in our study. Notably, the variants DSG2-p.D154E and -p.D187G, which were predicted to be damaging by bioinformatics tools, had no detectable effects on the DSG2 protein properties in our study. PMID:23071725

  5. Genome-wide association identifies a deletion in the 3’ untranslated region of Striatin in a canine model of arrhythmogenic right ventricular cardiomyopathy

    Science.gov (United States)

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a familial cardiac disease characterized by rapid ventricular tachycardia and sudden cardiac death. It is most frequently inherited as an autosomal dominant trait with incomplete and age-related penetrance and variable clinical expression. Th...

  6. Is cardiac MRI an effective test for arrhythmogenic right ventricular cardiomyopathy diagnosis?

    Institute of Scientific and Technical Information of China (English)

    Santhi; Chellamuthu; Alyson; M; Smith; Steven; M; Thomas; Catherine; Hill; Peter; W; G; Brown; Abdallah; Al-Mohammad

    2014-01-01

    AIM:To evaluate the referrals with suspected arrhythmogenic right ventricular cardiomyopathy(ARVC)and compare cardiac MR(cMR)findings against clinical diagnosis.METHODS:A retrospective analysis of 114(age range16 to 83,males 55%and females 45%)patients referred for cMR with a suspected diagnosis of ARVC between May 2006 and February 2010 was performed after obtaining institutional approval for service evaluation.Reasons for referral including clinical symptoms and family history of sudden death,electrocardiogram and echo abnormalities,cMR findings,final clinical diagnosis and information about clinical management were obtained.The results of cMR were classified as major,minor,non-specific or negative depending on both functional and tissue characterisation and the cMR results were compared against the final clinical diagnosis.RESULTS:The most common reasons for referral included arrhythmias(30%)and a family history of sudden death(20%).Of the total cohort of 114 patients:4 patients(4%)had major cMR findings for ARVC,13patients(11%)had minor cMR findings,2 patients had non-specific cMR findings relating to the right ventricle and 95 patients had a negative cMR.Of the 4 patients who had major cMR findings,3(75%)had a positive clinical diagnosis.In contrast,of the 13 patients who had minor cMR findings,only 2(15%)had a positive clinical diagnosis.Out of the 95 negative patients,clinical details were available for 81 patients and none of them had ARVC.Excluding the 14 patients with no clinical data and final diagnosis,the sensitivity of the test was 100%,specificity 87%,positive predictive value29%and the negative predictive value 100%.CONCLUSION:CMR is a useful tool for ARVC evaluation because of the high negative predictive value as the outcome has a significant impact on the clinical decision-making.

  7. Linkage analysis of five Chinese families with arrhythmogenic right ventricular cardiomyopathy using microsatellite genetic markers

    Institute of Scientific and Technical Information of China (English)

    黄峻; 杨春梅; 马立隽; 单其俊; 许迪; 华子春; 曹克将

    2003-01-01

    Objective To explore the linkage relationship between specific genetic markers and arrhythmogenic right ventricular cardiomyopathy (ARVC) in Chinese pedigrees.Methods The microsatellite genetic markers D2S152, D14S252, and D10S1664 were studied for their linkages to ARVC in five Chinese ARVC pedigrees and a normal population of 121 Chinese individuals. Genomic DNA of the pedigrees and normal population was amplified using PCR techniques. Denaturing polyacrylamide sequencing gel (4%) electrophoresis was used to detect microsatellite repeat polymorphisms. Gels were silver-stained. A classical linkage analysis program was used assuming models of autosomal dominance and recession. Results The logarithm of the odds (LOD) scores of D2S152 with ARVC in LW, WD, DS, LC and TY pedigrees were 2.174, -0.589, -∞, - (indicating that linkage is not supported in this mode), and -∞ respectively in autosomal dominant model (recombination fraction=0.000 respectively)and were -∞, -∞, -∞, -∞, and 0.182 respectively in the autosomal recessive model. The LOD scores of D14S252 with ARVC in LW, WD, DS, LC and TY pedigrees were -, -, -∞, -, and 0 respectively in autosomal dominant model, and were -∞, -0.812, -∞, -∞, and 0.087 respectively in autosomal recessive model. The LOD scores of D2S152 with ARVC in LW, WD, DS, LC and TY pedigrees were -, -0.539, -, and 0.602 respectively in autosomal dominant model and were -, -∞, -∞, -∞, and -∞ respectively in autosomal recessive model. Conclusions The LOD score for D2S152 in the LW pedigree was 2.174, indicating that the chance of linkage is about 150∶ 1. This suggests that there is a possible ARVC-related gene near this marker. There were no clear linkage relationships between ARVC and D10S1664 and D14S252 in this family, and no linkages between ARVC and any of the three genetic markers in the other four families. These results also suggest that there is genetic heterogeneity in LW and in the other pedigrees.

  8. Evaluation of right and left ventricular function using speckle tracking echocardiography in patients with arrhythmogenic right ventricular cardiomyopathy and their first degree relatives

    Directory of Open Access Journals (Sweden)

    Aneq Meriam

    2012-09-01

    Full Text Available Abstract Introduction and aim The identification of right ventricular abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC in early stages is still difficult. The aim of this study was to investigate if longitudinal strain based on speckle tracking can detect subtle right (RV or left ventricular (LV dysfunction as an early sign of ARVC. Methods and results Seventeen male patients, fulfilling Task force criteria for ARVC, 49 (32–70 years old, nineteen male first degree relatives 29 (19–73 y.o. and twenty-two healthy male volunteers 36 (24–66 y.o participated in the study. Twelve-lead and signal-averaged electrocardiograms were recorded. All subjects underwent echocardiography. LV and RV diameters, peak systolic velocity from tissue Doppler and longitudinal strain based on speckle tracking were measured from the basal and mid segments in both ventricles. RV longitudinal strain measurement was successful in first degree relatives and controls (95 resp. 86% but less feasible in patients (59%. Results were not systematically different between first degree relatives and controls. Using discriminant analysis, we then developed an index based on echocardiographic parameters. All normal controls had an index  Conclusion Longitudinal strain of LV and RV segments was significantly lower in patients than in relatives and controls. An index was developed incorporating dimensional and functional echocardiographic parameters. In combination with genetic testing this index might help to detect early phenotype expression in mutation carriers.

  9. Patient's Guide to Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

    Science.gov (United States)

    ... Search Donate Circulation My alerts Sign In Join Facebook Twitter Home About this Journal Editorial Board General Statistics Circulation Doodle → Blip the Doodle Information for Advertisers Author Reprints Commercial Reprints Customer Service and Ordering ...

  10. Stop-gain mutations in PKP2 are associated with a later age of onset of arrhythmogenic right ventricular cardiomyopathy.

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    Mireia Alcalde

    Full Text Available BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC is a cardiac disease characterized by the presence of fibrofatty replacement of the right ventricular myocardium, which may cause ventricular arrhythmias and sudden cardiac death. Pathogenic mutations in several genes encoding mainly desmosomal proteins have been reported. Our aim is to perform genotype-phenotype correlations to establish the diagnostic value of genetics and to assess the role of mutation type in age-related penetrance in ARVC. METHODS AND RESULTS: Thirty unrelated Spanish patients underwent a complete clinical evaluation. They all were screened for PKP2, DSG2, DSC2, DSP, JUP and TMEM43 genes. A total of 70 relatives of four families were also studied. The 30 patients fulfilled definite disease diagnostic criteria. Genetic analysis revealed a pathogenic mutation in 19 patients (13 in PKP2, 3 in DSG2, 2 in DSP, and 1 in DSC2. Nine of these mutations created a truncated protein due to the generation of a stop codon. Familial assessment revealed 28 genetic carriers among family members. Stop-gain mutations were associated to a later age of onset of ARVC, without differences in the severity of the pathology. CONCLUSIONS: Familial genetic analysis helps to identify the cause responsible for the pathology. In discrepancy with previous studies, the presence of a truncating protein does not confer a worse severity. This information could suggest that truncating proteins may be compensated by the normal allele and that missense mutations may act as poison peptides.

  11. "Awakening to" a new meaning of being at-risk for arrhythmogenic right ventricular cardiomyopathy: a grounded theory study.

    Science.gov (United States)

    Manuel, April; Brunger, Fern

    2015-04-01

    Efforts of social scientists to understand how individuals living in a family at risk for a genetically linked condition make health care decisions, having brought to the forefront the contextual nature of risk perception. Using a grounded theory approach, this study examines the experiences of 29 individuals living in families at risk for arrhythmogenic right ventricular cardiomyopathy (ARVC). Attention is paid to how individuals (re)construct the meaning of being at risk in relation to the developing science of gene discovery. Findings highlight that individuals living in a family at risk for ARVC juxtapose existing scientific knowledge against experiential knowledge as they "awaken to" the fact that they or a family member are at risk. This process is pragmatic and fluid and contingent upon whether and how symptoms are aligned with the constructed image of the at-risk relative.

  12. Arrhythmogenic substrate in hearts of rats with monocrotaline-induced pulmonary hypertension and right ventricular hypertrophy

    OpenAIRE

    Benoist, David; Stones, Rachel; Drinkhill, Mark; Bernus, Olivier; White, Ed

    2011-01-01

    Mechanisms associated with right ventricular (RV) hypertension and arrhythmias are less understood than those in the left ventricle (LV). The aim of our study was to investigate whether and by what mechanisms a proarrhythmic substrate exists in a rat model of RV hypertension and hypertrophy. Rats were injected with monocrotaline (MCT; 60 mg/kg) to induce pulmonary artery hypertension or with saline (CON). Myocardial levels of mRNA for genes expressing ion channels were measured by real-time R...

  13. New Features of Electrocardiogram in a Case Report of Arrhythmogenic Right Ventricular Cardiomyopathy: A Care-Compliant Article.

    Science.gov (United States)

    Pei, Haifeng; Yu, Qiujun; Su, Xiaohua; Wang, Zhen; Zhao, Heng; Yang, Dachun; Yang, Yongjian; Li, De

    2016-04-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a crucial health problem. With sudden death often being the first presentation, early diagnosis for ARVC is essential. Up to date, electrocardiogram (ECG) is a widely used diagnostic method without invasive harms. To diagnose and treat ARVC as well as possible, we should clearly elucidate its pathophysiological alterations. A 66-year-old farmer presented to the Emergency Department with continuous palpitation, chest tightness, profuse sweating, and nausea with no obvious predisposing causes. An ECG indicated ventricular tachycardia (VT). The patient experienced a sudden drop in blood pressure and acute confusion. After an immediate electrical conversion, his consciousness was gradually restored, and symptoms relieved. The patient was then transferred to the Department of Cardiology to receive ECG, echocardiography, coronary angiogram, biochemical assays, endocardiac tracing, and radiofrequency ablation. In the end, he was diagnosed with ARVC, evidenced by bilateral ventricle dilation and epsilon waves in leads V1-V3. Appropriate therapies were provided for this patient including pharmacological intervention and radiofrequency ablation. Although the diagnosis of ARVC is not difficult, this patient's ECG manifested several interesting features and should be further investigated: T wave inversions were found extensively in the anterior and inferior leads, revealing the involvement of bilateral ventricles; VTs with different morphologies and cycle lengths were found, and some VTs manifested the feature of irregularly irregular rhythm, reminding us to carefully differentiate some special VTs from atrial fibrillation (AF); and epsilon waves gradually appeared in leads V1-V3 and avR since the onset of ARVC. Most importantly, the epsilon waves behind QRS complex appeared in both sinus rhythm and ventricular premature beats/VT originating from cardiac apex, whereas the epsilon waves prior to QRS complex occurred in

  14. Mutation analysis of the phospholamban gene in 315 South Africans with dilated, hypertrophic, peripartum and arrhythmogenic right ventricular cardiomyopathies.

    Science.gov (United States)

    Fish, Maryam; Shaboodien, Gasnat; Kraus, Sarah; Sliwa, Karen; Seidman, Christine E; Burke, Michael A; Crotti, Lia; Schwartz, Peter J; Mayosi, Bongani M

    2016-02-26

    Cardiomyopathy is an important cause of heart failure in Sub-Saharan Africa, accounting for up to 30% of adult heart failure hospitalisations. This high prevalence poses a challenge in societies without access to resources and interventions essential for disease management. Over 80 genes have been implicated as a cause of cardiomyopathy. Mutations in the phospholamban (PLN) gene are associated with dilated cardiomyopathy (DCM) and severe heart failure. In Africa, the prevalence of PLN mutations in cardiomyopathy patients is unknown. Our aim was to screen 315 patients with arrhythmogenic right ventricular cardiomyopathy (n = 111), DCM (n = 95), hypertrophic cardiomyopathy (n = 40) and peripartum cardiomyopathy (n = 69) for disease-causing PLN mutations by high resolution melt analysis and DNA sequencing. We detected the previously reported PLN c.25C > T (p.R9C) mutation in a South African family with severe autosomal dominant DCM. Haplotype analysis revealed that this mutation occurred against a different haplotype background to that of the original North American family and was therefore unlikely to have been inherited from a common ancestor. No other mutations in PLN were detected (mutation prevalence = 0.2%). We conclude that PLN is a rare cause of cardiomyopathy in African patients. The PLN p.R9C mutation is not well-tolerated, emphasising the importance of this gene in cardiac function.

  15. A mutation in the Z-line Cypher/ZASP protein is associated with arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Lopez-Ayala, J M; Ortiz-Genga, M; Gomez-Milanes, I; Lopez-Cuenca, D; Ruiz-Espejo, F; Sanchez-Munoz, J J; Oliva-Sandoval, M J; Monserrat, L; Gimeno, J R

    2015-08-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an important cause of malignant arrhythmia and sudden death particularly in young people. Although it is considered a desmosomal disease, mutations in non-desmosomal genes have also been identified. We report on a family where a mutation in LDB3 is associated with this condition. The index case and first and second degree relatives underwent a complete clinical evaluation: physical examination, electrocardiography (ECG), signal-averaged ECG, 2D echocardiogram, cardiac magnetic resonance and 24-h monitoring. After ruling out mutations in the five desmosomal genes, genetic testing by means of Next Generation Sequencing was carried out on the proband. A heterozygous missense mutation in LDB3 c.1051A>G was identified. This result was confirmed by subsequent Sanger DNA sequencing. Another six carriers were identified amongst her relatives. Three subjects fulfilled the criteria for a definitive diagnosis of ARVC and one reached a borderline diagnosis. In conclusion, this is the first family with ARVC where a mutation in LDB3 is associated with ARVC. Next generation sequencing arises as a particular useful tool to point to new causative genes in ARVC.

  16. Functional effects of the TMEM43 Ser358Leu mutation in the pathogenesis of arrhythmogenic right ventricular cardiomyopathy

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    Rajkumar Revathi

    2012-03-01

    Full Text Available Abstract Background The Ser358Leu mutation in TMEM43, encoding an inner nuclear membrane protein, has been implicated in arrhythmogenic right ventricular cardiomyopathy (ARVC. The pathogenetic mechanisms of this mutation are poorly understood. Methods To determine the frequency of TMEM43 mutations as a cause of ARVC, we screened 11 ARVC families for mutations in TMEM43 and five desmosomal genes previously implicated in the disease. Functional studies were performed in COS-7 cells transfected with wildtype, mutant, and 1:2 wildtype:mutant TMEM43 to determine the effect of the Ser358Leu mutation on the stability and cellular localization of TMEM43 and other nuclear envelope and desmosomal proteins, assessed by solubility assays and immunofluorescence imaging. mRNA expression was assessed of genes potentially affected by dysfunction of the nuclear lamina. Results Three novel mutations in previously documented desmosomal genes, but no mutations in TMEM43, were identified. COS-7 cells transfected with mutant TMEM43 exhibited no change in desmosomal stability. Stability and nuclear membrane localization of mutant TMEM43 and of lamin B and emerin were normal. Mutant TMEM43 did not alter the expression of genes located on chromosome 13, previously implicated in nuclear envelope protein mutations leading to skeletal muscular dystrophies. Conclusions Mutant TMEM43 exhibits normal cellular localization and does not disrupt integrity and localization of other nuclear envelope and desmosomal proteins. The pathogenetic role of TMEM43 mutations in ARVC remains uncertain.

  17. Arrhythmogenic Right Ventricular Cardiomyopathy - 4 Swedish families with an associated PKP2 c.2146-1G>C variant

    Science.gov (United States)

    Svensson, Anneli; Åström-Aneq, Meriam; Widlund, Kjerstin Ferm; Fluur, Christina; Green, Anna; Rehnberg, Malin; Gunnarsson, Cecilia

    2016-01-01

    In this study, the genotype-phenotype correlations in four unrelated families with a PKP2 c.2146-1G>C gene variant were studied. Our primary aim was to determine the carriers that fulfilled the arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnostic criteria of 2010. Our secondary aim was to investigate whether any specific clinical characteristics can be attributed to this particular gene variant. Index patients were assessed using next generation ARVC panel sequencing technique and their family members were assessed by Sanger sequencing targeted at the PKP2 c.2146-1G>C variant. The gene variant carriers were offered a clinical follow-up, with evaluation based on the patient’s history and a standard set of non-invasive testing. The PKP2 c.2146-1G>C gene variant was found in 23 of 41 patients who underwent the examination. Twelve of the 19 family members showed “possible ARVC”. One with “borderline ARVC” and the rest with “definite ARVC” demonstrated re-polarization disturbances, but arrhythmia was uncommon. A lethal event occurred in a 14-year-old boy. In the present study, no definitive genotype-phenotype correlations were found, where the majority of the family members carrying the PKP2 c.2146-1G>C gene variant were diagnosed with “possible ARVC”. These individuals should be offered a long-term follow-up since they are frequently symptomless but still at risk for insidious sudden cardiac death due to ventricular arrhythmia. PMID:27335691

  18. Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping & paced surface ECG analysis.

    Directory of Open Access Journals (Sweden)

    Malcolm C Finlay

    Full Text Available AIMS: The concealed phase of arrhythmogenic right ventricular cardiomyopathy (ARVC may initially manifest electrophysiologically. No studies have examined dynamic conduction/repolarization kinetics to distinguish benign right ventricular outflow tract ectopy (RVOT ectopy from ARVC's early phase. We investigated dynamic endocardial electrophysiological changes that differentiate early ARVC disease expression from RVOT ectopy. METHODS: 22 ARVC (12 definite based upon family history and mutation carrier status, 10 probable patients without right ventricular structural anomalies underwent high-density non-contact mapping of the right ventricle. These were compared to data from 14 RVOT ectopy and 12 patients with supraventricular tachycardias and normal hearts. Endocardial & surface ECG conduction and repolarization parameters were assessed during a standard S1-S2 restitution protocol. RESULTS: Definite ARVC without RV structural disease could not be clearly distinguished from RVOT ectopy during sinus rhythm or during steady state pacing. Delay in Activation Times at coupling intervals just above the ventricular effective refractory period (VERP increased in definite ARVC (43 ± 20 ms more than RVOT ectopy patients (36 ± 14 ms, p = 0.03 or Normals (25 ± 16 ms, p = 0.008 and a progressive separation of the repolarisation time curves between groups existed. Repolarization time increases in the RVOT were also greatest in ARVC (definite ARVC: 18 ± 20 ms; RVOT ectopy: 5 ± 14, Normal: 1 ± 18, p<0.05. Surface ECG correlates of these intracardiac measurements demonstrated an increase of greater than 48 ms in stimulus to surface ECG J-point pre-ERP versus steady state, with an 88% specificity and 68% sensitivity in distinguishing definite ARVC from the other groups. This technique could not distinguish patients with genetic predisposition to ARVC only (probable ARVC from controls. CONCLUSIONS: Significant changes in dynamic conduction and repolarization

  19. Genotype-phenotype relationship in patients with arrhythmogenic right ventricular cardiomyopathy caused by desmosomal gene mutations: A systematic review and meta-analysis

    Science.gov (United States)

    Xu, Zhenyan; Zhu, Wengen; Wang, Cen; Huang, Lin; Zhou, Qiongqiong; Hu, Jinzhu; Cheng, Xiaoshu; Hong, Kui

    2017-01-01

    The relationship between clinical phenotypes and desmosomal gene mutations in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is poorly characterized. Therefore, we performed a meta-analysis to explore the genotype-phenotype relationship in patients with ARVC. Any studies reporting this genotype-phenotype relationship were included. In total, 11 studies involving 1,113 patients were included. The presence of desmosomal gene mutations was associated with a younger onset age of ARVC (32.7 ± 15.2 versus 43.2 ± 13.3 years; P = 0.001), a higher incidence of T wave inversion in V1–3 leads (78.5% versus 51.6%; P = 0.0002) or a family history of ARVC (39.5% versus 27.1%; P = 0.03). There was no difference in the proportion of males between desmosomal-positive and desmosomal-negative patients (68.3% versus 68.9%; P = 0.60). The presence of desmosomal gene mutations was not associated with global or regional structural and functional alterations (63.5% versus 60.5%; P = 0.37), epsilon wave (29.4% versus 26.2%; P = 0.51) or ventricular tachycardia of left bundle-branch morphology (62.6% versus 57.2%; P = 0.30). Overall, patients with desmosomal gene mutations are characterized by an earlier onset age, a higher incidence of T wave inversion in V1–3 leads and a strong family history of ARVC. PMID:28120905

  20. Myofibrillar myopathy with arrhythmogenic right ventricular cardiomyopathy 7: corroboration and narrowing of the critical region on 10q22.3.

    Science.gov (United States)

    Kuhl, Angelika; Melberg, Atle; Meinl, Edgar; Nürnberg, Gudrun; Nürnberg, Peter; Kehrer-Sawatzki, Hildegard; Jenne, Dieter E

    2008-03-01

    Several years ago, autosomal dominant myofibrillar myopathy (MFM) in combination with arrhythmogenic right ventricular cardiomyopathy (ARVC7) was tentatively mapped to a 10.6-Mbp (million base pairs) region on chromosome 10q22.3 between D10S605 (78.9 Mbp) and D10S215 (89.5 Mbp) in a Swedish family assuming that ARVC7 was allelic with cardiomyopathy, dilated 1C (CMD1C). To date, neither the genetic defect in ARVC7 nor CMD1C has been reported. In a comprehensive follow-up study we re-examined and confirmed the previous linkage data for ARVC7 using a high-density single nucleotide polymorphism marker panel from Affymetrix (Human Mapping 10K Array). No other regions with significant evidence for linkage were discovered. The critical interval was narrowed down to 4.27 Mbp between D10S1645 and D10S1786. This reduced the total number of candidate genes to 18 of which 17 (RAI17, PPIF, C10ORF56, SFTPA1, SFTPA2, SFTPA1B, SFTPA2B, SFTPD, C10ORF57, PLAC9, ANXA11, MAT1A, DYDC1, DYDC2, C10ORF58, TSPAN14 and SH2D4B) are shared with the CMD1C region. No disease-causing mutation was found in their coding regions. Moreover, metavinculin (VCL) and ZASP/cypher (LDB3) proximal and distal to this linked region were excluded by sequence analysis. To search for submicroscopic and intragenic deletions by PCR, we generated hybrid cell lines carrying only the affected or normal chromosome 10 homolog. All sequence tagged sites and exons were present on both homologs. We speculate that regulatory mutations in 1 of the 18 genes from 10q22.3 are responsible for a heterogenous spectrum of clinically distinct myodegenerative disorders, affecting both skeletal and cardiac muscles to variable degrees.

  1. Proteomic analysis reveals significant elevation of heat shock protein 70 in patients with chronic heart failure due to arrhythmogenic right ventricular cardiomyopathy.

    Science.gov (United States)

    Wei, Ying-Jie; Huang, Yin-Xia; Shen, Ya; Cui, Chuan-Jue; Zhang, Xiao-Ling; Zhang, Hao; Hu, Sheng-Shou

    2009-12-01

    As proteins are the ultimate biological determinants of phenotype of disease, we screened altered proteins associated with heart failure due to arrhythmogenic right ventricular cardiomyopathy (ARVC) to identify biomarkers potential for rapid diagnosis of heart failure. By 2-dimensional gel electrophoresis and mass spectrometry, we identified five commonly altered proteins with more than 1.5 fold changes in eight ARVC failing hearts using eight non-failing hearts as reference. Noticeably, one of the altered proteins, heat shock protein 70 (HSP70), was increased by 1.64 fold in ARVC failing hearts compared with non-failing hearts. The increase of cardiac HSP70 was further validated by Western blot, immunochemistry, and enzyme-linked immunosorbent assay (ELISA) in failing hearts due to not only ARVC, but also dilated (DCM, n = 18) and ischemic cardiomyopathy (ICM, n = 8). Serum HSP70 was also observed to be significantly increased in heart failure patients derived from the three forms of cardiomyopathies. In addition, we observed hypoxia/serum depletion stimulation induced significantly elevation of intracellular and extracellular HSP70 in cultured neonatal rat cardiomyocytes. For the first time to our knowledge, we revealed and clearly demonstrated significant up-regulation of cardiac and serum HSP70 in ARVC heart failure patients. Our results indicate that elevated HSP70 is the common feature of heart failure due to ARVC, DCM, and ICM, which suggests that HSP70 may be used as a biomarker for the presence of heart failure due to cardiomyopathies of different etiologies and may hold diagnostic/prognostic potential in clinical practice.

  2. 致心律失常性右室心肌病21例临床分析%Clinical study on arrhythmogenic right ventricular vardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王倩英

    2016-01-01

    目的 探讨致心律失常性右室心肌病(ARVC)的临床表现、心电图、影像学检查及治疗情况.方法 回顾性分析2007年至2014年收治的21例致心律失常性右室心肌病患者的资料,均符合2010年国际专家组《欧洲心脏杂志》再次修订的ARVC诊断标准,总结其临床特征及治疗情况.结果 21例患者主要表现为心悸、头晕、胸闷、气促者11例,心悸、反复晕厥者7例,窦房结功能障碍l例,体检发现右心增大2例,均出现室性心律失常,右胸导QRS平均间期大于左胸导,12例患者V1 ~3导出现Epsilon波,超声心动图及MRI示右心室增大,室壁局灶性变薄,心肌致密化不全,右室心尖部、流出道、前壁脂肪浸润,收缩功能减弱.8例导管射频消融患者即刻成功5例,2例置入ICD后产生有效放电干预,其余患者长期应用药物治疗.结论 ARVC临床表现无特异性,早期诊断困难,应提高疾病认识,综合分析家族史、临床表现、心电图及影像学检查等,选择合适治疗方法,提高诊疗效果.%Objective To investigate the clinical manifestation, electrocardiogram, imageological examination and treatment conditions of arrhythmogenic right ventricular vardiomyopathy(ARVC).Methods Retrospectively analyzed the clinical data of 21 patients with ARVC who were treated in the Fourth People' s Hospital of Shangqiu from February 2007 to March 2014.All of them conformed to ARVC diagnostic criteria which amended again by international expert group in "European Heart Journal" in 2010, and also summarized their clinical features and treatment conditions.Results Among the 21 cases, 11 patients had major performances with palpitation, dizzy giddy, chest congestion and anhelation, 7 patients with palpitation and recurrent syncope, 1 patient with sinus node dysfunction, and 2 patients with right heart enlargement though medical examination, all of them had the performance of ventricular arrhythmias.The QRS average period

  3. [Assessment of the right ventricular anatomy and function by advanced echocardiography: pathological and physiological insights].

    Science.gov (United States)

    Lakatos, Bálint; Kovács, Attila; Tokodi, Márton; Doronina, Alexandra; Merkely, Béla

    2016-07-01

    Accurate assessment of right ventricular geometry and function is of high clinical importance. However, several limitations have to be taken into consideration if using conventional echocardiographic parameters. Advanced echocardiographic techniques, such as speckle-tracking analysis or 3D echocardiography are reliable and simple tools providing a cost-effective and non-invasive alternative of current modalities used to characterize the right ventricle. There is a growing interest in the diagnostic and prognostic value of these methods regarding pathological (right ventricular infarction, pulmonary hypertension, arrhythmogenic right ventricular dysplasia, follow-up of heart transplantation) and even physiological (athlete's heart) alterations of the right ventricle. Orv. Hetil., 2016, 157(29), 1139-1146.

  4. Prevalence of Epsilan wave in patients with arrhythmogenic right ventricular cardiomyopathy%致心律失常性右室心肌病患者Epsilon波的检出率

    Institute of Scientific and Technical Information of China (English)

    王静; 居维竹; 陈凯; 曹克将; 杨兵; 陈明龙; 单其俊; 邹建刚; 陈椿; 张凤祥; 侯晓峰; 陈红武

    2009-01-01

    目的 探讨不同体表心电图记录方法对国人致心律失常性有室心肌病(arrhythmogenic right ventricular eardiomyopathy,ARVC)患者Epsilon波检出率的影响.方法 共人选ARVC患者32例,男性24例,女性8例,年龄18~70(42.3±13.3)岁.于安静仰卧位记录窦性心律情况下的标准12导联同步心电图(standard twelve leads electrocardiography,S-ECG)、右胸导联心电图(right precordial leads ECG,R-ECG)及Fontaine双极胸导联心电图(Fontaine bipolar precordial leads ECG,F-ECG)o Epsilon波判断标准为位于QRS波之后、sT段起始部的低波幅棘波、振荡波或平缓电位.结果 该组病例S-ECG、R-ECG及F-ECG对Epsilon波的检出率分别为37.5%、37.5%和50.0%,三种心电图记录方法之间Epsilon波检出率的比较差异无统计学意义(均为P>0.05).其中1例Epsilon波仅见于S-ECG,3例仅见于R-ECG,5例仅见于F-ECG.S-ECG联合R-ECG的检出率为50.0%,S-ECG联合F-ECG的检出率为56.3%,三种记录方法的联合检出率为65.6%,S-ECG联合F-ECG的枪出率与S-ECG相比明显提高(P<0.05),联合三种心电图记录方法的检出率与S-ECG相比有显著提高(P<0.01).结论 联合使用S-ECG、R-ECG及F-ECG三种心电图记录方法,可以显著提高ARVC患者Epsilon波的检出率,且三种记录方法之间可以相互补充.%Objective To investigate the prevalence of Epsilon wave in patients with arrhythmogenic right ventrieular cardiomyopathy (ARVC). Methods The Epsilon wave was detected in 32 patients [24 men, mean age (42.3±13.3) years] with ARVC using three different electrocardiography (ECG) recording methods: standard twelve leads ECG (S-ECG), right precordial leads ECG (R-ECG) and Fontaine bipolar precordiai leads ECG (F-ECG). The Epsilon wave was defined as wiggle, small spike wave and smooth potential between the end of the QRS complex and the beginning of the ST segment. Results Epsilon wave was detected in 37.5%, 37.5% and 50.0% patients with ARVC by S-ECG, R

  5. Science and practice of arrhythmogenic cardiomyopathy: A paradigm shift

    Directory of Open Access Journals (Sweden)

    Mohamed ElMaghawry

    2012-03-01

    Full Text Available The clinical, genetic, and molecular paradigm of arrhythmogenic right ventricular cardiomyopathy (ARVC has markedly progressed through the last three decades, shifting from the classical ARVC as a progressive condition characterized by fibrofatty replacement of the right ventricle,2,3,4 into a wider spectrum of arrhythmogenic cardiomyopathy (AC,5 which covers ARVC with its various clinical phases (occult, electric, right heart failure and late stage biventricular heart failure, biventricular arrhythmic cardiomyopathy, left dominant arrhythmic cardiomyopathy, Naxos and Carvajal syndromes. Epidemiologically, the disease was first associated with the Mediterranean basin (mainly Italy and France, however further studies have reported AC in many races and ethnic backgrounds6,7,8. Moreover, with regard to the pathoitiology of the disease, dysplasia was originally assumed as the disease mechanism. Other mechanisms were later postulated, such as inflammation and transdifferentiation. However, more recent animal models have established that dystrophy, either by myocyte necrosis or apoptosis, is the founding pathological process of AC.

  6. Right ventricular sarcoidosis: is it time for updated diagnostic criteria?

    Science.gov (United States)

    Vakil, Kairav; Minami, Elina; Fishbein, Daniel P

    2014-04-01

    A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient's case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.

  7. High-resolution electrocardiography in the diagnosis of arrhythmogenic right ventricular cardiomyopathy in Boxer dogs Eletrocardiografia de alta resolução no diagnóstico da cardiomiopatia arritmogênica do ventrículo direito em cães da raça Boxer

    Directory of Open Access Journals (Sweden)

    Evandro Zacché Pereira

    2013-06-01

    Full Text Available To evaluate the reliability of high-resolution electrocardiography in the diagnosis of arrhythmogenic right ventricular cardiomyopathy in Boxers, 20 dogs with no structural cardiac alterations at echocardiographic examination were grouped on the basis of frequency of ventricular arrhythmias, evaluated by 24-hour ambulatory ECG, and undergoing a high-resolution electrocardiography. High frequency QRS duration, duration of terminal QRS complex less than 40µV (LAS40 and root mean square voltage of the terminal 40 milliseconds of the QRS complex (RMS40 were measured. Differences in high-resolution ECG variables were not observed between groups. Therefore, the results of this investigation suggest that high-resolution electrocardiography is not a useful method for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in Boxers without detectable myocardial alterations or systolic dysfunction.Com o objetivo de avaliar a importância da eletrocardiografia de alta resolução no diagnóstico da cardiomiopatia arritmogênica do ventrículo direito do Boxer, 20 cães sem evidências de doença cardíaca estrutural à avaliação ecodopplercardiográfica foram agrupados de acordo com a frequência de arritmias ventriculares, avaliadas pela eletrocardiografia ambulatorial de 24 horas, e submetidos ao exame eletrocardiográfico de alta resolução. Duração do complexo QRS filtrado, duração dos sinais de baixa amplitude (menor que 40µV dos últimos 40 milissegundos do complexo QRS e raiz quadrada média da voltagem ao quadrado dos últimos 40 milissegundos do complexo QRS (RMS40 foram as variáveis avaliadas. Não foram observadas diferenças significativas entre os grupos em relação às variáveis estudadas. Sendo assim, os resultados do presente estudo sugerem que a eletrocardiografia de alta resolução não é uma ferramenta útil no auxílio diagnóstico da cardiomiopatia arritmogênica do ventrículo direito dos cães da raça Boxer

  8. Postpartal right ventricular thrombosis.

    Science.gov (United States)

    Velicki, Lazar; Milosavljević, Aleksandar; Majin, Marijan; Vujin, Bojan; Kovacević, Pavle

    2008-11-01

    The discovery of an intracardial mass in patients presents a serious diagnostic dilemma. The differential diagnosis of this condition may seem abundant, but myxomas and intracardial thrombosis are the most frequent diagnoses. A connection between pregnancy and the presence of thrombosis has been documented frequently. Normal pregnancy leads to changes of the coagulative and fibrinolytic status toward a hypercoagulable condition which has its own physiological justification (the risk of blood loss decreases during labor). The case of a patient suffering from postpartal right ventricular thrombosis, which was successfully resolved by surgery as described in this contribution, demonstrates the value of a multidisciplinary approach.

  9. Predictive value of electrical restitution in hypokalemia-induced ventricular arrhythmogenicity

    DEFF Research Database (Denmark)

    Osadchii, Oleg E; Larsen, Anders Peter; Olesen, Soren Peter

    2009-01-01

    the predictive value of electrical restitution in hypokalemia-induced arrhythmogenicity. We recorded monophasic APs and measured effective refractory periods (ERP) at distinct ventricular epicardial and endocardial sites and monitored volume-conducted ECG at baseline and after hypokalemic perfusion (2.5 mM K......The ventricular action potential (AP) shortens exponentially upon a progressive reduction of the preceding diastolic interval. Steep electrical restitution slopes have been shown to promote wavebreaks, thus contributing to electrical instability. The present study was designed to assess......(+) for 30 min) in isolated guinea pig heart preparations. The restitution of AP duration measured at 90% repolarization (APD(90)) was assessed after premature extrastimulus application at variable coupling stimulation intervals, and ERP restitution was assessed by measuring refractoriness over a wide range...

  10. Right ventricular metastasis of leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    Stagmo Martin

    2009-05-01

    Full Text Available Abstract Metastatic presentation of leiomyosarcoma in the heart is very rare. We present transthoracic echocardiography and combined PET/CT images of a case with a large right ventricular metastasis of leiomyosarcoma. The patient was placed on cytostatic drugs for palliative purposes, but passed away one month later because of an untreatable ventricular tackycardia.

  11. Arrhythmogenic cardiomyopathy in a patient with Noonan syndrome.

    Science.gov (United States)

    Altamirano, Eugenia; Drut, Ricardo

    2010-01-01

    Arrhythmogenic ventricular cardiomyopathy (AVC) presents with fat replacement of the myocardium, most commonly of the right ventricle, and ventricular arrhythmias. We report an 11-year-old boy with Noonan syndrome, ventricular arrhythmias, and an ultrasound depicting hypertrophy of the ventricular septum with subaortic stenosis. A surgical resection of the left side of the ventricular septum revealed a thick fibroelastotic endocardium covering a broad band of mature adipose tissue focally containing myocardial cells, fibrosis and chronic inflammatory infiltrates. The two layers covered a band of hypertrophic myocardiocytes with mild interstitial fibrosis. Arrhythmogenic ventricular cardiomyopathy has not been previously reported in the Noonan syndrome.

  12. Catheter ablation of ventricular tachycardia with arrhythmogenic right ventricular cardiomyopathy under the guidance of 3-dimentional mapping%三维标测指导致心律失常性右心室心肌病室性心动过速的射频消融

    Institute of Scientific and Technical Information of China (English)

    陈明龙; 吴延庆; 胡建新; 曹克将; 杨兵; 邹建刚; 陈红武; 居维竹; 徐东杰; 陈椿; 侯小锋; 单其俊

    2009-01-01

    目的 介绍致心律失常性右心室心肌病(ARVC)室性心动过速(室速)的三维标测方法 及其消融策略.方法 21例ARVC室速患者,因1~4种抗心律失常药物治疗无效,临床上呈反复发作、无休止发作或植入型心律转复除颤器(ICD)植入后频繁放电治疗,接受导管消融治疗.其中,男性19例,女性2例,平均年龄(32+12)岁.9例患者接受电解剖(Carto)标测,12例患者接受非接触标测(EnSite-Array).在首先明确病变基质的基础上,通过激动标测、拖带标测及起搏标测,分析心动过速的起源、可能的传导径路及其出口以及它们与病变基质的关系.通常于心动过速的出口处及其周边行局灶消融,术中病变基质周边的延迟激动电位应一并消融.结果 21例患者,2例呈无休止发作,1例患者表现为频繁室性早搏及加速性室性自主心律,余18例患者消融中共诱发出34种心动过速.所有心动过速均呈左束支阻滞形,平均心动过速周长为(289±68)ms.16例患者(28种室速)消融治疗即刻成功,3例患者(7种室速)部分成功,2例患者(2种室速)消融失败,即刻消融成功率76.2%.所有患者消融术后继续服用抗心律失常药物.平均随访6~30(14±7)个月,成功患者中2例复发,其中1例再次消融成功;未达即刻成功的5例患者,经抗心律失常药物治疗后,均无室性心律失常事件发生,其中包括1例消融后植入ICD者.结论 三维标测系统可首先明确ARVC患者的病变基质,在此基础上结合激动标测和心内各种电刺激技术,可直观显示心动过速的起源、缓慢传导区出口及折返环路,以此制定消融策略可成功治疗ARVC室速.心动过速起源于心肌深部或ARVC病变进展,是消融失败和复发的常见原因.%Objective To evaluate the application of 3-dimensional mapping and the ablation strate-gies in the treatment of ventricular tachycardia (VT) with arrhythmogenic right ventricular cardiomyopathy

  13. Procainamide and lidocaine produce dissimilar changes in ventricular repolarization and arrhythmogenicity in guinea-pig.

    Science.gov (United States)

    Osadchii, Oleg E

    2014-08-01

    Procainamide is class Ia Na(+) channel blocker that may prolong ventricular repolarization secondary to inhibition of IK r , the rapid component of the delayed rectifier K(+) current. In contrast to selective IN a blockers such as lidocaine, procainamide was shown to produce arrhythmogenic effects in the clinical setting. This study examined whether pro-arrhythmic responses to procainamide may be accounted for by drug-induced repolarization abnormalities including impaired electrical restitution kinetics, spatial gradients in action potential duration (APD), and activation-to-repolarization coupling. In perfused guinea-pig hearts, procainamide was found to prolong the QT interval on ECG and left ventricular (LV) epicardial monophasic APD, increased the maximum slope of electrical restitution, enhanced transepicardial APD variability, and eliminated the inverse correlation between the local APD and activation time values determined at distinct epicardial recording sites prior to drug infusion. In contrast, lidocaine had no effect on electrical restitution, the degree of transepicardial repolarization heterogeneities, and activation-to-repolarization coupling. Spontaneous episodes of monomorphic ventricular tachycardia were observed in 57% of procainamide-treated heart preparations. No arrhythmia was induced by lidocaine. In summary, this study suggests that abnormal changes in repolarization may contribute to pro-arrhythmic effects of procainamide.

  14. Left ventricular apical diseases.

    Science.gov (United States)

    Cisneros, Silvia; Duarte, Ricardo; Fernandez-Perez, Gabriel C; Castellon, Daniel; Calatayud, Julia; Lecumberri, Iñigo; Larrazabal, Eneritz; Ruiz, Berta Irene

    2011-08-01

    There are many disorders that may involve the left ventricular (LV) apex; however, they are sometimes difficult to differentiate. In this setting cardiac imaging methods can provide the clue to obtaining the diagnosis. The purpose of this review is to illustrate the spectrum of diseases that most frequently affect the apex of the LV including Tako-Tsubo cardiomyopathy, LV aneurysms and pseudoaneurysms, apical diverticula, apical ventricular remodelling, apical hypertrophic cardiomyopathy, LV non-compaction, arrhythmogenic right ventricular dysplasia with LV involvement and LV false tendons, with an emphasis on the diagnostic criteria and imaging features. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s13244-011-0091-6) contains supplementary material, which is available to authorized users.

  15. Arrhythmia in Acute Right Ventricular Infarction

    Directory of Open Access Journals (Sweden)

    Azin Alizadeh Asl

    2007-09-01

    Full Text Available Acute inferior myocardial infarction (MI frequently involves the right ventricle (RV.1-3 We assessed the prognostic impact of RV myocardial involvement in patients with inferior MI. One hundred seventy patients were admitted to the cardiac care unit of Madani Heart Hospital (Tabriz-Iran with the diagnosis of inferior MI with (group1 or without (group2 the simultaneous involvement of RV during the study period (from 2005 to 2006. Patients presenting within 12h of symptom onset were eligible for inclusion. Patients with simultaneous anterior wall MI or renal impairment (creatinine > 2 mg/dl, as well as those undergoing primary percutaneous translational coronary angioplasty, were excluded. Eighty eight percent of the patients with RVMI and 75% of those with isolated inferior MI had some type of arrhythmia. Atrioventricular (AV block occurred in 42% of the infarctions with RV involvement and only in 29% of the control group. Intra-ventricular conduction disturbance (IVCD was also more frequent in RVMI (29.4% vs. 13.1%, p=0.021, especially right bundle branch block (RBBB (20% vs. 7.4%, P=0.003. There was, however, no meaningful difference in the incidence of left bundle branch block (LBBB between the two groups (3.5% vs. 2.35%, P=0.95. Ventricular fibrillation (VF was observed in 5.2% and 1.2% and ventricular tachycardia in 26% and 12.2% of the patients in groups 1 and 2, respectively. In 27% of patients with RVMI, it was necessary to implant a pacemaker as compared to 10% of those in the control group. Mortality was higher in the patients with inferior infarction extended to the RV (15.3% vs. 3.5%, P= 0.0001. Thus, the differences between the findings in the two groups in terms of the occurrence of post-MI arrhythmias and conduction disorders were quite significant, but there was no meaningful difference with respect to the incidence of LBBB between the two groups. Additionally, patients with inferior MI who also had RV myocardial involvement were

  16. 植入型心律转复除颤器在致心律失常性右心室心肌病患者中的应用%Implantable cardioverter defibrillator therapy in patients with arrhythmogenic right ventricular cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    孙奇; 陈柯萍; 陈若菡; 戴研; 华伟; 张澍

    2013-01-01

    Objective To evaluate the role of implantable cardioverter defibrillator(ICD) therapy in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).Methods From Mar.2004 to Mar.2012,ARVC patients received ICD were enrolled in this study,and the clinical characteristics were collected.In the follow-up,we assessed the incidence,feature and ICD therapy in these patients.Results Eighteen patients were enrolled [12 men,6 women,mean age (46.4 ± 13.8) years].During the (46.6 ± 30.5) months followup period,ICD recorded 111 ventricular tachycardia (VT)/ventricular fibrillation (VF) episodes (1 VF,110 VT) in 6 patients.Among these,3 patients experienced 7 electrical storms(ES).Compare to patients without ICD therapy,C-reactive protein(CRP) level was higher in ICD therapy group.Among VT/VF episodes,12 episodes were terminated by shocks,99 episodes were terminated by anti-tachycardia pacing(ATP).Conclusions VT episodes were very common in ARVC patients,most of them could terminated by ATP.Repeated episodes of malignant arrhythmias in ARVC patients with ES were common.CRP level might be a significant predictor of appropriate ICD therapy in ARVC patients.%目的 总结阜外心血管病医院应用植入型心律转复除颤器(ICD)治疗致心律失常性右心室心肌病(ARVC)的经验.方法 入选我院2004年3月至2012年3月确诊并植入ICD的ARVC患者,收集临床资料,进行常规电话及门诊随诊.结果 共18例患者[男12例,女6例,平均年龄(46.4±13.8)岁]入选,平均随访(46.6±30.5)个月,有6例患者出现了111次室性心动过速(VT)/心室颤动(VF)事件,其中仅1次VF事件,其余均为VT事件,其中3例患者术后共出现了7次电风暴.ICD治疗组中的C反应蛋白(CRP)明显高于ICD未治疗组.111次VT/VF事件中,12次经电除颤终止心动过速,其余均通过抗心动过速起搏(ATP)终止了心动过速.结论 ARVC患者ICD植入后的VT/VF事件以VT为主,多数可经ATP终止;出现电风暴的ARVC患者

  17. Tubular hypoplasia of the aorta and right atrioventricular valve dysplasia in a Bulldog.

    Science.gov (United States)

    Robinson, Nicholas A; Armíen, Aníbal G

    2010-07-01

    A Bulldog puppy that died at 1 day of age was presented for postmortem evaluation. Macroscopically, there was marked hypoplasia of the ascending, transverse, and proximal segments of the descending thoracic aorta and almost complete secondary thrombosis of the left ventricle causing a functional stenosis of the left atrioventricular valve. Separately, there was right atrioventricular valve dysplasia with secondary dilation of the right atrium. Microscopically, the left ventricular outflow tract was occluded by chondroid metaplasia, fibrosing recanalization of a left-ventricular thrombus, and isolated Purkinje fiber degeneration and necrosis.

  18. Myocardial performance is reduced immediately prior to ventricular ectopy

    DEFF Research Database (Denmark)

    Bloch Thomsen, Poul Erik; Hansen, Thomas Fritz; Jons, Christian;

    2012-01-01

    of impulse conduction would also lead to changes in the contractile performance of sinus beats preceding ventricular ectopy using Tissue Doppler echocardiography. Methods: Twenty-three consecutive patients with VPBs were examined in the apical 4-chamber view with a frame rate of 150 Hz (GE VIVID VII). Eleven......Background: We recently demonstrated local voltage potentials indicating conduction impairment and block in the sinus beats preceding ventricular premature beats (VPBs) originating in the ventricular outflow tracts. Objective: The purpose of this study was to test the hypothesis that impairment...... patients had no structural heart disease, 5 had dilated cardiomyopathy, 4 had ischemic heart disease, 2 had arrhythmogenic right ventricular dysplasia, and 1 had aortic stenosis. The ectopy originated in the ventricular outflow tracts in 15 patients and in the left ventricle 8. Eleven of the patients...

  19. Rupture of Right Ventricular Free Wall Following Ventricular Septal Rupture in Takotsubo Cardiomyopathy with Right Ventricular Involvement.

    Science.gov (United States)

    Sung, June Min; Hong, Sung Jin; Chung, In Hyun; Lee, Hye Young; Lee, Jae Hoon; Kim, Hyun Jung; Byun, Young Sup; Kim, Byung Ok; Rhee, Kun Joo

    2017-01-01

    Most patients diagnosed with takotsubo cardiomyopathies are expected to almost completely recover, and their prognosis is excellent. However, complications can occur in the acute phase. We present a case of a woman with takotsubo cardiomyopathy with right ventricular involvement who developed a rupture of the right ventricular free wall following ventricular septal rupture, as a consequence of an acute increase in right ventricular afterload by left-to-right shunt. Our case report illustrates that takotsubo cardiomyopathy can be life threatening in the acute phase. Ventricular septal rupture in biventricular takotsubo cardiomyopathy may be a harbinger of cardiac tamponade by right ventricular rupture.

  20. Right ventricular mass estimation by angioechocardiography.

    Science.gov (United States)

    Arcilla, R A; Mathew, R; Sodt, P; Lester, L; Cahill, N; Thilenius, O G

    1976-01-01

    A combined angiocardiographic-echocardiographic method for estimating right ventricular wall mass is described. Biplane cineangiocardiograms are analyzed for ventricular volume in end-diastole, and wall thickness is determined from echocardiograms obtained with a high frequency transducer and strip chart recorder, The intracavitary and the external surface volumes of the ventricle are derived, and the difference multiplied by 1.050, the specific gravity of myocardium. Excellent correlation was observed between right ventricular wall mass and body surface area in normal children (r = 0.93). The mean right ventricular mass was 44.5 g/M2 as compared to 78.1 g/M2 for the left ventricle, corresponding mass/EDV values were 0.48 g/cm3 and 1.26 g/cm3, respectively. In isolated right ventricular pressure overload, the increase in right ventricular mass is chiefly due to the increase in wall thickness; in volume overload, it is due mostly to the increase in chamber volume,

  1. Chamber-specific effects of hypokalaemia on ventricular arrhythmogenicity in isolated, perfused guinea-pig heart

    DEFF Research Database (Denmark)

    Osadchii, Oleg E; Bentzen, Bo Hjorth; Olesen, Søren-Peter

    2009-01-01

    of hypokalaemic perfusion (2.5 mm K(+) for 30 min) were assessed in isolated guinea-pig heart preparations using simultaneous recordings of volume-conducted electrocardiogram and monophasic action potentials from six ventricular epicardial sites. Effective refractory periods, ventricular fibrillation thresholds...... in increased RV-to-LV transepicardial dispersion of refractoriness and action potential duration. Furthermore, hypokalaemic perfusion was associated with reduced ventricular fibrillation threshold and increased inducibility of tachyarrhythmias by programmed electrical stimulation and tachypacing as determined...... by extrastimulus application at earlier time points during LV repolarization. Increased prematurity of extrastimulus-evoked propagating responses was associated with exaggerated local inhomogeneities in intraventricular conduction and action potential duration in hypokalaemic LV, thus creating a favourable stage...

  2. Sudden Infant Death Syndrome and Left Ventricular Hypertrabeculation-Hidden Arrhythmogenic Entity?

    Directory of Open Access Journals (Sweden)

    G. Saayman

    2010-09-01

    Full Text Available Left ventricular noncompaction/hypertrabeculation is a condition which is characterized by a highly trabeculated, “spongy” myocardium. It can present at any age with heart failure, arrhythmia and/or thromboembolic events. A wide variety of mutations have been found to be a cause of hypertrabeculation and it is possible that there is a continuum of hypertrophic cardiomyopathy, dilated cardiomyopathy and hypertrabeculation/noncompaction. We present a case of left ventricular hypertrabeculation which presented as sudden infant death syndrome and we propose that this entity may be a hidden cause of arrhythmic death in some infants presenting as sudden infant death syndrome.

  3. Deadly proposal: a case of catecholaminergic polymorphic ventricular tachycardia.

    Science.gov (United States)

    Heiner, Jason D; Bullard-Berent, Jeffrey H; Inbar, Shmuel

    2011-11-01

    Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare adrenergically mediated arrhythmogenic disorder classically induced by exercise or emotional stress and found in structurally normal hearts. It is an important cause of cardiac syncope and sudden death in childhood. Catecholaminergic polymorphic ventricular tachycardia is a genetic cardiac channelopathy with known mutations involving genes affecting intracellular calcium regulation. We present a case of a 14-year-old boy who had cardiopulmonary arrest after an emotionally induced episode of CPVT while attempting to invite a girl to the school dance. Review of his presenting cardiac rhythm, induction of concerning ventricular arrhythmias during an exercise stress test, and genetic testing confirmed the diagnosis of CPVT. He recovered fully and was treated with β-blocker therapy and placement of an implantable cardioverter-defibrillator. In this report, we discuss this rare but important entity, including its molecular foundation, clinical presentation, basics of diagnosis, therapeutic options, and implications of genetic testing for family members. We also compare CPVT to other notable cardiomyopathic and channelopathic causes of sudden death in youth including hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, long QT syndrome, short QT syndrome, and Brugada syndrome.

  4. Positional Right Ventricular Obstruction in Pectus Excavatum.

    Science.gov (United States)

    Underwood, Katherine; Vorsanger, Matthew; Saric, Muhamed; Skolnick, Adam H

    2017-01-25

    Pectus excavatum is one of the most common congenital chest wall deformities. The degree of sternal depression, which may result in compression of the right heart by the chest wall, is variable. While typically asymptomatic, there are various symptoms that can result from severe pectus excavatum. We report on a patient with severe pectus excavatum leading to dynamic obstruction of the right ventricular outflow tract in the seated position.

  5. Evaluation of right ventricular volumes measured by magnetic resonance imaging

    DEFF Research Database (Denmark)

    Møgelvang, J; Stubgaard, M; Thomsen, C;

    1988-01-01

    Right ventricular volumes were determined in 12 patients with different levels of right and left ventricular function by magnetic resonance imaging (MRI) using an ECG gated multisection technique in planes perpendicular to the diastolic position of the interventricular septum. Right ventricular...

  6. EXPERIMENTAL STUDY OF RIGHT VENTRICULAR ASSIST IN ACUTE RIGHT VENTRICULAR FAILING

    Institute of Scientific and Technical Information of China (English)

    刘明辉; 苏鸿熙; 李功宋; 王加利; 董超; 童健

    1996-01-01

    The efficacy of right atrial-pulmonary artery (RA-PA)bypass during acute right ventricular failure(RVF)produced by pulmonary artery constriction in dogs was examined in this study Control group (n=7)was supported with conventional volume loading and inotropic therapy.In the experimental animals (n=8),RA-PA bypass was initiated 5 min.after the onset of severe RVF.Three control animals died from refractory ventricular fibrillation within one hour of RVF.No animals in the experimental group died within two hours of RA-PA bypass,but the histological study of the lungs in these animals demonstrated peribronchial,preivascular and intraalveolar hemorrhage.Lighr microscopic and electron microscopic examination of the myocardial specimens of the right ventricular free wall displayed the myocardial structures and ultrastructues were maintained effectively with RA-PA bypass wile irreversible myocardial injuries occurred in the control animasls after two hours of RVF with conventional therapy.During the 2hours of RA-PA bypass,the hemodynamic indices were also maintained better when compared to the control animals.It may be concluded,a roller pump right ventricular assist device effectively unloads the acute faijing right ventricle,maintains systemic cardisc output,and significatly reverses the myocardial ischemia during right venrricular failure,but RA-PA bypass may induce pulmonary hypertension due to increased pulmonary vascular resistance secondary to pulmonary edeme and interstitial hemorrhage.

  7. Right ventricular hydatid cyst ruptured to pericardium

    Directory of Open Access Journals (Sweden)

    Feridoun Sabzi

    2015-01-01

    Full Text Available Cardiac hydatidosis is rare presentation of body hydatidosis. Incidence of cardiac involvements range from 5% to 5% of patients with hydatid disease. Most common site of hydatid cyst in heart is interventricular septum and left ventricular free wall. Right ventricular free wall involvement by cyst that ruptured to pericardial cavity is very rare presentation of hydatid cyst. Cardiac involvement may have serious consequences such as rupture to blood steam or pericardial cavity. Both the disease and its surgical treatment carry a high complication rate, including rupture leading to cardiac tamponade, anaphylaxis and also death. In the present report, a 43-year-old man with constrictive pericarditis secondary to a pericardial hydatid cyst is described.

  8. MECHANISMS OF RIGHT VENTRICULAR FAILURE AND BIVENTRICULAR MECHANICAL CIRCULATORY SUPPORT

    Directory of Open Access Journals (Sweden)

    G. P. Itkin

    2013-01-01

    Full Text Available The paper presents a review of the problems of mechanical circulatory support with left ventricular bypass in total cardiac failure. Dysfunction of right ventricular defines the high mortality on left ventricular device. One of the effective methods for solving this problem is the use of right ventricular assisted devices. There are considered of the basic physiological mechanisms of interaction between the right and left ventricles of the heart, affecting on the function of the right heart. Shows the need to assess right ventricular function before deciding on mechanical circulatory support. Provides examples of the estimation the predictors of the right ventricular failure. The basic methods and devices of biventricular circulatory support were con- sidered. 

  9. Recurrent missense mutations in TMEM43 (ARVD5) due to founder effects cause arrhythmogenic cardiomyopathies in the UK and Canada

    KAUST Repository

    Haywood, Annika

    2012-11-15

    AimsAutosomal dominant arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) (in the group of arrhythmogenic cardiomyopathies) is a common cause of sudden cardiac death in young adults. It is both clinically and genetically heterogeneous, with 12 loci (ARVC/D1-12) and eight genes identified, the majority of which encode structural proteins of cardiac desmosomes. The most recent gene identified, TMEM43, causes disease due to a missense mutation in a non-desmosomal gene (p.S358L) in 15 extended families from Newfoundland, Canada. To determine whether mutations in TMEM43 cause ARVC/D and arrhythmogenic cardiomyopathy in other populations, we fully re-sequenced TMEM43 on 143 ARVC/D probands (families) from the UK and 55 probands (from 55 families) from Newfoundland.Methods and resultsBidirectional sequencing of TMEM43 including intron-exon boundaries revealed 33 variants, the majority located in non-coding regions of TMEM43. For the purpose of validation, families of probands with rare, potentially deleterious coding variants were subjected to clinical and molecular follow-up. Three missense variants of uncertain significance (p.R28W, p.E142K, p.R312W) were located in highly conserved regions of the TMEM43 protein. One variant (p.R312W) also co-segregated with relatives showing clinical signs of disease. Genotyping and expansion of the disease-associated haplotype in subjects with the p.R312W variant from Newfoundland, Canada, and the UK suggest common ancestry.ConclusionAlthough the p.R312W variant was found in controls (3/378), identification of an ancestral disease p R312W haplotype suggests that the p.R312W variant is a pathogenic founder mutation. © 2012 The Author.

  10. Right ventricular function before and after percutaneous balloon mitral valvuloplasty.

    Science.gov (United States)

    Burger, W; Brinkies, C; Illert, S; Teupe, C; Kneissl, G D; Schräder, R

    1997-01-01

    Aim of this study was to evaluate right ventricular performance in patients with mitral stenosis and its modification by balloon valvuloplasty. Right ventricular volumes of 24 patients with postrheumatic mitral stenosis were determined by thermodilution 1 or 2 days before and 1 or 2 days after valvuloplasty. Right ventricular ejection fraction at rest was 43 (36-47)% (median and interquartile range). Right ventricular end-diastolic volume was 100 (86-119) ml/m2. Supine bicycle exercise (50 Watt) reduced right ventricular ejection fraction to 30 (29-37)% (P volume to 124 (112-141) ml/m2 (P improvement of right ventricular ejection fraction correlated inversely with the value of this parameter before valvuloplasty (r = -0.88, P volume (r = 0.57, P < 0.01). The right ventricular function curve, disturbed before commissurotomy, was reestablished by the procedure. In conclusion, at the here investigated stage of mitral stenosis right ventricular function is reversibly impaired. This is predominantly caused by the hemodynamic consequences of the valvular defect and not by an impairment of right ventricular myocardial function.

  11. Dual Endothelin Receptor Blockade Abrogates Right Ventricular Remodeling and Biventricular Fibrosis in Isolated Elevated Right Ventricular Afterload.

    Directory of Open Access Journals (Sweden)

    Eva Amalie Nielsen

    Full Text Available Pulmonary arterial hypertension is usually fatal due to right ventricular failure and is frequently associated with co-existing left ventricular dysfunction. Endothelin-1 is a powerful pro-fibrotic mediator and vasoconstrictor that is elevated in pulmonary arterial hypertension. Endothelin receptor blockers are commonly used as pulmonary vasodilators, however their effect on biventricular injury, remodeling and function, despite elevated isolated right ventricular afterload is unknown.Elevated right ventricular afterload was induced by progressive pulmonary artery banding. Seven rabbits underwent pulmonary artery banding without macitentan; 13 received pulmonary artery banding + macitentan; and 5 did not undergo inflation of the pulmonary artery band (sham-operated controls.Right and left ventricular collagen content was increased with pulmonary artery banding compared to sham-operated controls and ameliorated by macitentan. Right ventricular fibrosis signaling (connective tissue growth factor and endothelin-1 protein levels; extra-cellular matrix remodeling (matrix-metalloproteinases 2 and 9, apoptosis and apoptosis-related peptides (caspases 3 and 8 were increased with pulmonary artery banding compared with sham-operated controls and decreased with macitentan.Isolated right ventricular afterload causes biventricular fibrosis, right ventricular apoptosis and extra cellular matrix remodeling, mediated by up-regulation of endothelin-1 and connective tissue growth factor signaling. These pathological changes are ameliorated by dual endothelin receptor blockade despite persistent elevated right ventricular afterload.

  12. RADIOFREQUENCY ABLATION OF IDIOPATHIC RIGHT VENTRICULAR TACHYCARDIA

    Institute of Scientific and Technical Information of China (English)

    华伟; JituVohra

    1998-01-01

    This paper presents our experieaee with radioreqencey ablation (RFA) for idiopathic ventricular tschycardia (VT) arising from right ventricle in 12 patients(pts). The age range d patients was 21~50, with a mean of 38. 5 years. Ten out of 12 were females, 1 patient had eandia failure due to almost incessant VT while the rest had normal left ventricular function.Twelve pts had VT arising from the fight ventricle; of those, 9 were from the outflow truct, 2 from the RV apex, and l from the RV inflow. In all tats the diagnostic study and therapeutic RFA were combined in a single procedure, pacemapping and local aetlvition time were used to guide the site of RFA in Ors with VT arising froth the tight ventricle.RFA was successful in 11 of the 12 pts ( 91%). Ntmaher of RF applications were 1~27, mean 9. 6; fluoroscopy time were 4~75, mean 26. 9 minutes. RFA for idiopathic RV has a high success rate. This mode of treament should be considered as a nonphartaaeologieal curative treatment for symptomatic pts.

  13. Effect of right ventricular pacing on ventricular rhythm during atrial fibrillation

    NARCIS (Netherlands)

    Wittkampf, F.H.M.; Jongste, M.J.L. de; Lie, H.I.; Meijler, F.L.

    1988-01-01

    In 13 patients with atrial fibrillation, the effect of right ventricular pacing at various rates on spontaneous RR intervals was studied. Five hundred consecutive RR intervals were recorded and measured before and during varying right ventricular pacing rates. As anticipated, all RR intervals longer

  14. Right ventricular exclusion for hepatocellular carcinoma metastatic to the heart

    Directory of Open Access Journals (Sweden)

    Fan Shou-Zen

    2010-10-01

    Full Text Available Abstract We used for the first time a right ventricular exclusion procedure for the treatment of hepatocellular carcinoma metastatic to the right ventricle. Our case report shows that this surgical option can be effective as rescue therapy for right ventricular outflow tract obstruction secondary to myocardial metastasis in critically ill patients. Most notably, this technique can prevent inadvertent dislodgement of tumor cells.

  15. Right Ventricular Adaptation in Congenital Heart Diseases

    Directory of Open Access Journals (Sweden)

    Beatrijs Bartelds

    2014-05-01

    Full Text Available In the last four decades, enormous progress has been made in the treatment of congenital heart diseases (CHD; most patients now survive into adulthood, albeit with residual lesions. As a consequence, the focus has shifted from initial treatment to long-term morbidity and mortality. An important predictor for long-term outcome is right ventricular (RV dysfunction, but knowledge on the mechanisms of RV adaptation and dysfunction is still scarce. This review will summarize the main features of RV adaptation to CHD, focusing on recent knowledge obtained in experimental models of the most prevalent abnormal loading conditions, i.e., pressure load and volume load. Models of increased pressure load for the RV have shown a similar pattern of responses, i.e., increased contractility, RV dilatation and hypertrophy. Evidence is accumulating that RV failure in response to increased pressure load is marked by progressive diastolic dysfunction. The mechanisms of this progressive dysfunction are insufficiently known. The RV response to pressure load shares similarities with that of the LV, but also has specific features, e.g., capillary rarefaction, oxidative stress and inflammation. The contribution of these pathways to the development of failure needs further exploration. The RV adaptation to increased volume load is an understudied area, but becomes increasingly important in the growing groups of survivors of CHD, especially with tetralogy of Fallot. Recently developed animal models may add to the investigation of the mechanisms of RV adaptation and failure, leading to the development of new RV-specific therapies.

  16. Effects of right atrial and ventricular DDD pacing on cardiac function and ventricular contraction synchrony

    Institute of Scientific and Technical Information of China (English)

    支力大; 华伟; 张澍; 史蓉芳; 王方正; 陈新

    2004-01-01

    Background Right ventricular apical pacing has been reported to reduce cardiac performance. But there are few reports on the effects of dual chamber (DDD) pacing on cardiac function compared to sinus rhythm. In this study, we evaluated the effects of right atrial and ventricular DDD pacing on cardiac function and ventricular contraction synchrony using equilibrium radionuclide angiography.Methods Ten patients implanted with a right atrial and ventricular DDD pacemaker underwent equilibrium radionuclide angiography. The scintigraphic data were obtained during sinus rhythm and pacing rhythm. Cardiac function parameters were obtained semimanually. Phase analysis was used to study the ventricular activation sequence and ventricular synchrony.Results The left ventricular 1/3 ejection fraction decreased significantly during pacing compared with that during sinus rhythm[(23.4 ±6.1)% vs(27.7 ±4.5)%, P =0.01]. Regional ejection fraction also decreased during pacing, although the difference was not statistically significant. Phase analysis showed that the right ventricle was activated earlier than the left ventricle during pacing, and that the phase shift was significantly greater during pacing than that during sinus rhythm[64.13°±16.80° vs 52.88°± 9.26°, P =0.007]. The activation of both ventricles occurred simultaneously during sinus rhythm, with the activation sequence from proximal septum or base of left ventricle to apex. The earliest activation during pacing occurred at the right ventricular apex, and subsequently spread to the base and left ventricle.Conclusion Right atrial and ventricular DDD pacing impairs left ventricular systolic function and ventricular synchrony.

  17. Acute right ventricular dysfunction: real-time management with echocardiography.

    Science.gov (United States)

    Krishnan, Sundar; Schmidt, Gregory A

    2015-03-01

    In critically ill patients, the right ventricle is susceptible to dysfunction due to increased afterload, decreased contractility, or alterations in preload. With the increased use of point-of-care ultrasonography and a decline in the use of pulmonary artery catheters, echocardiography can be the ideal tool for evaluation and to guide hemodynamic and respiratory therapy. We review the epidemiology of right ventricular failure in critically ill patients; echocardiographic parameters for evaluating the right ventricle; and the impact of mechanical ventilation, fluid therapy, and vasoactive infusions on the right ventricle. Finally, we summarize the principles of management in the context of right ventricular dysfunction and provide recommendations for echocardiography-guided management.

  18. Right ventricular noncompacted cardiomyopathy-time for a new classification?

    Institute of Scientific and Technical Information of China (English)

    Marija Zdravkovic; Mirjana Krotin

    2009-01-01

    @@ To the Editor: We read with a great interest the recent article by Zhang et al,~1 in which they reported a rare case of isolated right ventricular noncompaction with normal left ventricular systolic function in a 23-year-old female.

  19. Right ventricular failure after implantation of a continuous-flow left ventricular assist device

    DEFF Research Database (Denmark)

    Cordtz, Johan Joakim; Nilsson, Jens C; Hansen, Peter B;

    2014-01-01

    Right ventricular failure (RVF) is a significant complication after implantation of a left ventricular assist device. We aimed to identify haemodynamic changes in the early postoperative phase that predicted subsequent development of RVF in a cohort of HeartMate II (HMII) implanted patients....

  20. Delayed recovery of right ventricular systolic function after repair of long-standing tricuspid regurgitation associated with severe right ventricular failure.

    Science.gov (United States)

    Kim, Jong Hun; Kim, Kyung Hwa; Choi, Jong Bum; Kuh, Ja Hong

    2016-03-01

    After tricuspid valve surgery for long-standing tricuspid regurgitation associated with right ventricular failure, reverse remodelling of the enlarged right ventricle, including recovery of right ventricular systolic function, is unpredictable. We present the case of a 31-year old man with early reduction of dilated right ventricular dimensions and delayed recovery of impaired right ventricular systolic function after valve repair for traumatic tricuspid regurgitation lasting 16 years.

  1. Acute left ventricular dysfunction secondary to right ventricular septal pacing in a woman with initial preserved contractility: a case report

    Directory of Open Access Journals (Sweden)

    Gribaa Rim

    2011-10-01

    Full Text Available Abstract Introduction Right ventricular apical pacing-related heart failure is reported in some patients after long-term pacing. The exact mechanism is not yet clear but may be related to left ventricular dyssynchrony induced by right ventricular apical pacing. Right ventricular septal pacing is thought to deteriorate left ventricular function less frequently because of a more normal left ventricular activation pattern. Case presentation We report the case of a 55-year-old Tunisian woman with preserved ventricular function, implanted with a dual-chamber pacemaker for complete atrioventricular block. Right ventricular septal pacing induced a major ventricular dyssynchrony, severe left ventricular ejection fraction deterioration and symptoms of congestive heart failure. Upgrading to a biventricular device was associated with a decrease in the symptoms and the ventricular dyssynchrony, and an increase of left ventricular ejection fraction. Conclusion Right ventricular septal pacing can induce reversible left ventricular dysfunction and heart failure secondary to left ventricular dyssynchrony. This complication remains an unpredictable complication of right ventricular septal pacing.

  2. Continuous right ventricular volumetry by fast-response thermodilution during right ventricular ischemia: head-to-head comparison with conductance catheter measurements.

    NARCIS (Netherlands)

    Hein, M.; Roehl, A.B.; Baumert, J.H.; Rossaint, R.; Steendijk, P.

    2009-01-01

    OBJECTIVE: To evaluate the accuracy of right ventricular ejection fraction and right ventricular end-diastolic volume obtained by volumetric pulmonary artery catheter, using the conductance catheter as reference method. DESIGN: Prospective, comparative study. SETTING: Research laboratory of a univer

  3. Quantitative Assessment of Right Ventricular Systolic Function by the Analysis of Right Ventricular Contrast Time-intensity Curve

    Institute of Scientific and Technical Information of China (English)

    王林; 邓又斌; 李天亮; 杨好意

    2004-01-01

    To study reliability and reliable indices of quantitative assessment of right ventricular systolic function by time-intensity curve (TIC)with right ventricular contrast, 5 % sonicated human albumin was injected intravenously at a does of 0. 08 ml/kg into 10 dogs at baseline status and cardiac insufficiency. Apical four-chamber view was observed for washinand washout of contrast agent from right ventricle. The parameters of TIC were obtained by curve fitting. The differences of parameters were analyzed in different states of cardiac functions. Among the parameters derived from TIC, the time constant (k) was decreased significantly with decline of cardiac function(P<0.001). But half-time of decent of peak intensity (HT) and mean-transit-time (MTT) of washout were increased significantly (P<0. 001). The k was strongly related to cardiac output of right ventricle (CO) and ejection fraction (EF) of left ventricle and fractional shortening (FS)of left ventricle. Right ventricular systolic function could be assessed reliably by the parameters derived from TIC with right ventricular contrast echocardiography. The k, HT and MTT are reliable indices for quantitative assessment of right ventricular systolic function.

  4. Management of patients with Arrhythmogenic Right Ventricular Cardiomyopathy in the Nordic countries

    DEFF Research Database (Denmark)

    Haugaa, Kristina H; Bundgaard, Henning; Edvardsen, Thor;

    2015-01-01

    are sparse. This paper aims to give a practical overview of management strategies, risk stratification, and selection of appropriate therapies for patients with ARVC and their family members. DESIGN: This paper summarizes follow-up and treatment strategies in ARVC patients in the Nordic countries. The author......-up are required in patients with definite ARVC and in genetic-mutation-positive family members. Furthermore, ARVC patients with and without implantable cardioverter defibrillators (ICDs) require different follow-up strategies. CONCLUSION: Careful follow-up is required in patients with ARVC diagnosis to evaluate...

  5. Screening of Three Novel Candidate Genes in Arrhythmogenic Right Ventricular Cardiomyopathy

    DEFF Research Database (Denmark)

    Christensen, Alex Hørby; Benn, Marianne; Tybjærg-Hansen, Anne

    2011-01-01

    ), and PERP (PERP)-all important structural proteins located at the intercalated disc-were involved in the pathogenesis of ARVC. We screened 65 unrelated patients (55 fulfilling 1994 Task Force criteria and ten borderline cases) for mutations in CTNNB1, CTNNA3, and PERP by direct sequencing and Light...

  6. Primary prophylaxis of sudden death in hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and dilated cardiomyopathy.

    Science.gov (United States)

    Klein, George J; Krahn, Andrew D; Skanes, Allan C; Yee, Raymond; Gula, Lorne J

    2005-09-01

    We present an evidence-based overview of primary prevention of sudden cardiac death. Several recent studies have provided important data regarding pharmacologic and device-based therapy for patients with conditions that confer high risk for sudden death. A rational approach to these therapies, with emphasis on implanted cardiovertor defibrillators, is discussed.

  7. Ventricular repolarization time, location of pacing stimulus and current pulse amplitude conspire to determine arrhythmogenicity in mice

    DEFF Research Database (Denmark)

    Speerschneider, T; Grubb, Søren Jahn; Olesen, S P;

    2016-01-01

    AIM: In this study, we investigate the impact of altered action potential durations (APD) on ventricular repolarization time and proarrhythmia in mice with and without genetic deletion of the K(+) -channel-interacting protein 2 (KChIP2(-/-) and WT respectively). Moreover, we examine...

  8. Left and right ventricular diastolic function in hemodialysis patients

    Directory of Open Access Journals (Sweden)

    Rudhani Ibrahim

    2010-01-01

    Full Text Available The aim of this prospective study was the assessment of left ventricular and right ventricular diastolic function in patients on hemodialysis (HD and the correlation of this func-tion with the duration of HD. The study included 42 patients (22 females and 20 males with chro-nic renal failure (CRF, treated with HD, and 40 healthy subjects (24 females and 16 males with no history of cardiovascular disease and with normal renal function, who constituted the control group. The groups were matched for age and sex. All study patients and control subjects under-went detailed history taking and physical examination. They also underwent electrocardiogram, echocardiography and biochemical and hematological blood analyses. Significant differences were noted between the two groups in the two-dimensional and M-mode echocardiography findings concerning aortic root dimension, transverse diameter of the left atrium, thickness of the inter-ventricular septum, thickness of the left ventricular posterior wall, left ventricular diastolic diameter, left ventricular systolic diameter, shortening fraction, ejection fraction as well as findings from the pulse Doppler study, including E wave, A wave, E/A ratio, deceleration time of E wave (DT-E, acceleration time of E wave (AT-E, tricuspid E and A waves (E tr and A tr and E tr /A tr , ratio. There were significant changes in HD patients without arterial hypertension as well in the control group subjects. Our study suggests that the left ventricular and left atrial dimensions as well as the left ventricular wall thickness are augmented in patients with CRF treated with HD compared with the control group. Additionally, the left and right ventricular diastolic function is also reduced in these patients. These differences were also noted in patients with CRF without arterial hypertension. Left ventricular diastolic dysfunction had no correlation with the duration of HD.

  9. Use of right ventricular support with a centrifugal pump in post-valve surgery right ventricular failure: a case series.

    Science.gov (United States)

    Moulodi, Abdol Rasoul; Sheibat Zadeh, Gholam Reza; Sabzi, Feridoun

    2014-01-12

    The optimal treatment method for right ventricular failure after valve surgery complicated by a low cardiac output has not been determined, although several case reports have been published on patients with ventricular failure and arrhythmia who were bridged to cardiac transplantation using biventricular or left ventricular assist devices. This case series illustrates successful circulatory support of 4 patients with prolonged low cardiac outputs and right ventricular failure and arrhythmias after valvular heart surgery with or without severe pulmonary hypertension. In-hospital death occurred in one patient and 3 patients were discharged from the hospital with good general condition. At two years' follow-up, 2 patients were in functional class one but another patient underwent laparotomy for multiple splenic abscesses and died from multiple organ failure.

  10. Left and right ventricular diastolic function in hemodialysis patients.

    Science.gov (United States)

    Rudhani, Ibrahim Destan; Bajraktari, Gani; Kryziu, Emrush; Zylfiu, Bejtush; Sadiku, Shemsedin; Elezi, Ymer; Rexhepaj, Nehat; Vitia, Arber; Emini, Merita; Abazi, Murat; Berbatovci-Ukimeraj, M; Kryeziu, Kaltrina; Hsanagjekaj, Venera; Korca, Hajrije; Ukimeri, Aferdita

    2010-11-01

    The aim of this prospective study was the assessment of left ventricular and right ventricular diastolic function in patients on hemodialysis (HD) and the correlation of this function with the duration of HD. The study included 42 patients (22 females and 20 males) with chronic renal failure (CRF), treated with HD, and 40 healthy subjects (24 females and 16 males) with no history of cardiovascular disease and with normal renal function, who constituted the control group. The groups were matched for age and sex. All study patients and control subjects underwent detailed history taking and physical examination. They also underwent electrocardiogram, echocardiography and biochemical and hematological blood analyses. Significant differences were noted between the two groups in the two-dimensional and M-mode echocardiography findings concerning aortic root dimension, transverse diameter of the left atrium, thickness of the interventricular septum, thickness of the left ventricular posterior wall, left ventricular diastolic diameter, left ventricular systolic diameter, shortening fraction, ejection fraction as well as findings from the pulse Doppler study, including E wave, A wave, E/A ratio, deceleration time of E wave (DT-E), acceleration time of E wave (AT-E), tricuspid E and A waves (E tr and A tr ) and E tr /A tr , ratio. There were significant changes in HD patients without arterial hypertension as well in the control group subjects. Our study suggests that the left ventricular and left atrial dimensions as well as the left ventricular wall thickness are augmented in patients with CRF treated with HD compared with the control group. Additionally, the left and right ventricular diastolic function is also reduced in these patients. These differences were also noted in patients with CRF without arterial hypertension. Left ventricular diastolic dysfunction had no correlation with the duration of HD.

  11. Effects of Long-term Right Ventricular Apical Pacing on Left Ventricular Remodeling and Cardiac Function

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    Objective: To investigate the impacts of long-term right ventricular apical pacing on the ventricular remodeling and cardiac functions of patients with high-grade and third-degree atrioventricular blockage with normal heart structures and cardiac functions. In addition, we provide many evidences for choosing an optimal electrode implantation site.Methods: Study participants included patients who were admitted for pacemaker replacements and revisited for examinations of implanted pacemakers at outpatient. Pa...

  12. The feasibility and efficacy of right ventricular assistance without thoracotomy.

    Science.gov (United States)

    Yano, M; Matsuo, K; Hatane, T; Araki, K; Onitsuka, T; Shibata, K; Koga, Y

    1993-01-01

    Mechanical right ventricular assistance has never been percutaneously instituted. We invented a new method for administering right ventricular assistance without thoracotomy (RAWT) and evaluated its effects in acute experiments using dogs. To institute RAWT, an outflow cannula was inserted into the pulmonary artery from the femoral vein, and an inflow cannula was inserted into the right atrium from the right jugular vein. A Swan-Ganz catheter inserted into the outflow cannula led it to the pulmonary artery under blood pressure monitoring only. A Sarns' centrifugal pump was used as a blood pump. We applied this system to biventricular failed hearts supported by a left ventricular assist device. We could increase cardiac output from 53.3 +/- 31.8 to 77.8 +/- 31.7 ml/min/kg, and mean aortic pressure from 43.5 +/- 12.3 to 57.5 +/- 11.2 mm Hg with RAWT. The right ventricular unloading effect of RAWT was not disturbed by regurgitation through the pulmonary or tricuspid valve.

  13. Distinct loading conditions reveal various patterns of right ventricular adaptation

    NARCIS (Netherlands)

    Borgdorff, Marinus A. J.; Bartelds, Beatrijs; Dickinson, Michael G.; Steendijk, Paul; de Vroomen, Maartje; Berger, Rolf M. F.

    2013-01-01

    Right ventricular (RV) failure due to chronically abnormal loading is a main determinant of outcome in pulmonary hypertension (PH) and congenital heart disease. However, distinct types of RV loading have been associated with different outcomes. To determine whether the adaptive RV response depends o

  14. Imaging diagnosis of right ventricular infarction and tricuspid regurgitation by radionuclide first pass method

    Energy Technology Data Exchange (ETDEWEB)

    Koito, Hitoshi; Iwasaka, Toshiji; Yoshioka, Hiroshi (Kansai Medical School, Moriguchi, Osaka (Japan))

    1983-07-01

    Radionuclide first pass findings of right ventricular infarction and tricuspid regurgitation are reported. Findings of right ventricular infarction are reduced wall motion and regional ejection fraction in the right ventricular inferior wall. Tricuspid regurgitation is suggested when the dilatation of right atrium and right ventricle are seen with regurgitant RI images through tricuspid valve. We can get regurgitant fraction from time-activity curve. Dynamic images are also useful for assessing quality of right ventricular infarction and tricuspid regurgitation. We conclude that radionuclide first pass method is useful to diagnose right ventricular infarction and to quantify tricuspid regurgitation.

  15. Electrical heterogeneity of canine right ventricular transient outward potassium currents

    Institute of Scientific and Technical Information of China (English)

    杨新春; 周鹏; 李翠兰

    2004-01-01

    Background Some studies have confirmed that the right ventricular walls of most rodents, such as canines and humans, have evident transient outward potassium current (lto1) heterogeneity, and this heterogeneity is closely related to J point elevation, J wave formation, and some ventricular tachycardias such as ventricular fibrillations caused by Brugada syndrome. This study is designed to investigate transmural electrical heterogeneity of the canine right ventricle during repolarization (phase 1) from the viewpoint of 4-aminopyridine sensitive and calcium-independent lto1.Methods Adult canine single right ventricular epicardial (Epi) cells, mid-myocardial (M) cells, and endocardial (Endo) cells were enzymatically dissociated. Whole cell voltage-clamp recordings were made to compare the lto1 values of the three cell types.Results At 37℃ and using 0.2 Hz and + 70 mV depolarizing test potentials, the average peak lto1 values of Epi cells and M cells averaged (4070±1720) pA and (3540±1840) pA, respectively. The activated and inactivated Epi and M cells kinetic processes were in accordance with the Boltzmann distribution. Compared with lto1 in Epi cells and M cells, the average peak lto1 in Endo cells was very low, averaged (470±130) pA.Conclusions These results suggest that there are evident differences and potent gradients in lto1 between the three cardiac cell types, especially between Epi and Endo cells. These differences are among the prominent manifestations of right ventricular electrical heterogeneity, and may form an important ionic basis and prerequisite for some malignant arrhythmias in the right ventricle, including those arising from Brugada syndrome and other diseases.

  16. Right ventricular volume and mass determined by cine magnetic resonance imaging in HIV patients with possible right ventricular dysfunction

    DEFF Research Database (Denmark)

    Kjaer, Andreas; Lebech, Anne-Mette; Gerstoft, Jan;

    2006-01-01

    Impaired right ventricular (RV) function has been reported to occur in patients with HIV when studied by echocardiography. However, for accurate evaluation of RV function and morphology, first-pass radionuclide ventriculography (RNV) and cine magnetic resonance imaging (cine-MRI) are methods of c...

  17. Ventricular septal rupture and right ventricular intramyocardial dissection secondary to acute inferior myocardial infarction.

    Science.gov (United States)

    Bittencourt, Márcio Sommer; Seltmann, Martin; Muschiol, Gerd; Achenbach, Stephan

    2010-01-01

    A 61-year-old female patient presented with sub acute myocardial infarction with an occluded right coronary artery on invasive evaluation and a ventricular septal rupture on echocardiogram. Cardiac computed tomography (CT) was performed to better define the septal anatomy. As the anatomy on cardiac CT was considered unfavorable for percutaneous intervention, the patient underwent successful surgical repair.

  18. Right Ventricular Function and Left Ventricular Assist Device Placement: Clinical Considerations and Outcomes

    Science.gov (United States)

    Lainez, Romeo; Parrino, Gene; Bates, Michael

    2010-01-01

    The HeartMate II is an axial-flow left ventricular assist device that is approved for the treatment of advanced heart failure as a bridge to transplant or destination therapy. Despite the success of this device, right ventricular failure remains a persistent problem in most studies. Right ventricular dysfunction is usually defined as the need for right heart mechanical support or the persistent requirement for inotropes to support right heart function beyond 14 days. Over 21 months, 45 patients with end-stage heart disease underwent placement of the HeartMate II at our institution. This continuous cohort of patients underwent a retrospective review to evaluate the incidence of right heart failure. The perioperative survival was 91% with no incidents of mechanical support for the right ventricle and no requirements for inotropes beyond 14 days. This survival was consistent to beyond 1 year at the time of the study, and 18% of patients underwent heart transplant with 100% survival. PMID:21603391

  19. Right Ventricular Hemodynamics in Patients with Pulmonary Hypertension

    Science.gov (United States)

    Browning, James; Fenster, Brett; Hertzberg, Jean; Schroeder, Joyce

    2012-11-01

    Recent advances in cardiac magnetic resonance imaging (CMR) have allowed for characterization of blood flow in the right ventricle (RV), including calculation of vorticity and circulation, and qualitative visual assessment of coherent flow patterns. In this study, we investigate qualitative and quantitative differences in right ventricular hemodynamics between subjects with pulmonary hypertension (PH) and normal controls. Fifteen (15) PH subjects and 10 age-matched controls underwent same day 3D time resolved CMR and echocardiography. Echocardiography was used to determine right ventricular diastolic function as well as pulmonary artery systolic pressure (PASP). Velocity vectors, vorticity vectors, and streamlines in the RV were visualized in Paraview and total RV Early (E) and Atrial (A) wave diastolic vorticity was quantified. Visualizations of blood flow in the RV are presented for PH and normal subjects. The hypothesis that PH subjects exhibit different RV vorticity levels than normals during diastole is tested and the relationship between RV vorticity and PASP is explored. The mechanics of RV vortex formation are discussed within the context of pulmonary arterial pressure and right ventricular diastolic function coincident with PH.

  20. Right ventricular volume and mass determined by cine magnetic resonance imaging in HIV patients with possible right ventricular dysfunction

    DEFF Research Database (Denmark)

    Kjaer, Andreas; Lebech, Anne-Mette; Gerstoft, Jan

    2006-01-01

    Impaired right ventricular (RV) function has been reported to occur in patients with HIV when studied by echocardiography. However, for accurate evaluation of RV function and morphology, first-pass radionuclide ventriculography (RNV) and cine magnetic resonance imaging (cine-MRI) are methods...... ventricular ejection fraction (RVEF). To do so, we screened patients with RNV and performed an additional cine-MRI in those with reduced RVEF determined by RNV. Ninety patients with HIV were included. To evaluate the MRI measures exactly we included 18 age- and gender-matched healthy volunteers to establish...

  1. Hemodynamic Performance of a Novel Right Ventricular Assist Device (PERKAT)

    Science.gov (United States)

    Schulze, P. Christian; Ferrari, Markus W.

    2017-01-01

    Acute right ventricular failure (RVF) is an increasing clinical problem and a life-threatening condition. Right ventricular assist devices represent a reasonable treatment option for patients with refractory RVF. We here present a novel percutaneously implantable device for right ventricular support. The PERKAT device is based on a nitinol stent cage, which is covered with valve-carrying foils. A flexible outlet trunk with a pigtail tip is connected to the distal part. The device is driven by an intra-aortic balloon pump (IABP) drive unit, which inflates/deflates a standard IABP-balloon placed within the stent cage. In-vitro evaluation was done in a liquid bath containing water or blood analog. The PERKAT device was tested in different afterload settings using two different IABP-balloons and varying inflation/deflation rates. We detected flow rates ranging from 1.97 to 3.93 L/min depending on the afterload setting, inflation/deflation rate, balloon size, and the medium used. Flow rates between water and blood analog were nearly comparable, and in the higher inflation/deflation rate settings slightly higher with water. Based on this promising in vitro data, the innovative percutaneously implantable PERKAT device has a potential to become a therapeutic option for patients with RVF refractory to medical treatment. PMID:27831998

  2. A rare case of isolated non-compaction right ventricular myocardium

    Institute of Scientific and Technical Information of China (English)

    ZHANG Xiao-juan; ZHI Guang; HOU Hai-jun; ZHOU Xiao

    2009-01-01

    Isolated right ventricular noncompaction (IRNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The clinical syndrome includes systolic and diastolic dysfunction; some cases may have ventricular arrhythmias. We report a case of a female with the diagnosis of right ventricular non-compaction myocardium (RVNC) with normal left ventricular systolic function. To the best of our knowledge, there have been no reports of isolated ventricular non-compaction involving only the right ventricular before 2008, and there have only been described in very few cases of newborns and adult patients.

  3. Total Artificial Heart Implantation after Excision of Right Ventricular Angiosarcoma.

    Science.gov (United States)

    Bruckner, Brian A; Abu Saleh, Walid K; Al Jabbari, Odeaa; Copeland, Jack G; Estep, Jerry D; Loebe, Matthias; Reardon, Michael J

    2016-06-01

    Primary cardiac sarcomas, although rare, are aggressive and lethal, requiring thorough surgical resection and adjuvant chemotherapy for the best possible outcome. We report the case of a 32-year-old woman who underwent total artificial heart implantation for right-sided heart failure caused by right ventricular angiosarcoma. For the first several weeks in intensive care, the patient recovered uneventfully. However, a postoperative liver biopsy indicated hepatocellular injury consistent with preoperative chemotherapy. She developed continuing liver failure, from which she died despite good cardiac function.

  4. Chest radiographs fail to detect right ventricular enlargement and right atrial enlargement in patients with a pure restrictive ventilatory impairment.

    Science.gov (United States)

    Shivkumar, K; Ravi, K; Henry, J W; Eichenhorn, M S; Stein, P D

    1994-08-01

    The validity of measurements of the cardiac silhouette on chest radiographs for the evaluation of right ventricular enlargement and right atrial enlargement in patients with a pure restrictive ventilatory impairment was investigated in 19 patients. The forced vital capacity (FVC) percent predicted in these patients was 59 +/- 12 percent (mean +/- SD) (range, 29 to 79 percent). Right ventricular enlargement, by two-dimensional echocardiography, was defined as a right ventricular area > 20.4 cm2 and right atrial enlargement was defined as a right atrial area > 15.3 cm2. Chest radiographic measurements in the posteroanterior (PA) projection included distance from the midline to the farthest point of the right border of the cardiac silhouette, transverse cardiac diameter, and cardiothoracic ratio. Measurements in the lateral projection included the lateral horizontal transverse diameter, ventral portion of the lateral broad diameter, and obliteration of the retrosternal space. Neither the right ventricular area nor the right atrial area correlated with any of these radiographic measurements. There were no differences in these chest radiographic measurements among patients with normal right ventricular and right atrial dimensions, patients with right ventricular enlargement, and patients with right atrial enlargement. We conclude, therefore, that PA and lateral chest radiographs do not reliably detect right ventricular enlargement or right atrial enlargement in patients with a pure restrictive ventilatory impairment.

  5. Adverse ventricular-ventricular interactions in right ventricular pressure load: Insights from pediatric pulmonary hypertension versus pulmonary stenosis.

    Science.gov (United States)

    Driessen, Mieke M P; Hui, Wei; Bijnens, Bart H; Dragulescu, Andreea; Mertens, Luc; Meijboom, Folkert J; Friedberg, Mark K

    2016-06-01

    Right ventricular (RV) pressure overload has a vastly different clinical course in children with idiopathic pulmonary arterial hypertension (iPAH) than in children with pulmonary stenosis (PS). While RV function is well recognized as a key prognostic factor in iPAH, adverse ventricular-ventricular interactions and LV dysfunction are less well characterized and the pathophysiology is incompletely understood. We compared ventricular-ventricular interactions as hypothesized drivers of biventricular dysfunction in pediatric iPAH versus PS Eighteen iPAH, 16 PS patients and 18 age- and size-matched controls were retrospectively studied. Cardiac cycle events were measured by M-mode and Doppler echocardiography. Measurements were compared between groups using ANOVA with post hoc Dunnet's or ANCOVA including RV systolic pressure (RVSP; iPAH 96.8 ± 25.4 mmHg vs. PS 75.4 ± 18.9 mmHg; P = 0.011) as a covariate. RV-free wall thickening was prolonged in iPAH versus PS, extending beyond pulmonary valve closure (638 ± 76 msec vs. 562 ± 76 msec vs. 473 ± 59 msec controls). LV and RV isovolumetric relaxation were prolonged in iPAH (P < 0.001; LV 102.8 ± 24.1 msec vs. 63.1 ± 13.7 msec; RV 95 [61-165] vs. 28 [0-43]), associated with adverse septal kinetics; characterized by rightward displacement in early systole and leftward displacement in late RV systole (i.e., early LV diastole). Early LV diastolic filling was decreased in iPAH (73 ± 15.9 vs. PS 87.4 ± 14.4 vs. controls 95.8 ± 12.5 cm/sec; P = 0.004). Prolonged RVFW thickening, prolonged RVFW isovolumetric times, and profound septal dyskinesia are associated with interventricular mechanical discoordination and decreased early LV filling in pediatric iPAH much more than PS These adverse mechanics affect systolic and diastolic biventricular efficiency in iPAH and may form the basis for worse clinical outcomes. We used clinically derived data to study the pathophysiology of ventricular-ventricular

  6. Large Right Ventricular Clot in Pulmonary Atresia With Intact Ventricular Septum: In Defense of Biventricular Approach.

    Science.gov (United States)

    Dutta, Nilanjan; Ghosh, Rajarshi; Awasthy, Neeraj; Iyer, Parvathi U; Girotra, Sumir; Iyer, Krishna S

    2016-09-01

    Thrombus formation within the right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PAIVS) is not a very common occurrence and can be catastrophic. We present the case of a seven-month-old child with PAIVS and RV clot who successfully underwent biventricular repair. We discuss the interesting case and the rationale for management by means of biventricular repair over single ventricle repair when feasible in such a setting.

  7. Right ventricular outflow and apical pacing comparably worsen the echocardioghraphic normal left ventricle

    NARCIS (Netherlands)

    T.J.F. ten Cate (Tim); M.G. Scheffer (Michael); G.R. Sutherland (George); J.F. Verzijlbergen (Fred); N.M. van Hemel (Norbert)

    2008-01-01

    textabstractAims: A depressed left ventricular function (LVF) is sometimes observed during right ventricular apical (RVA) pacing, but any prediction of this adverse effect cannot be done. Right ventricular outflow tract (RVOT) pacing is thought to deteriorate LVF less frequently because of a more no

  8. The road to right ventricular septal pacing: techniques and tools.

    Science.gov (United States)

    Mond, Harry G

    2010-07-01

    Prolonged right ventricular (RV) apical pacing is associated with progressive left ventricular dysfunction due to dysynchronous ventricular activation and contraction. RV septal pacing allows a narrower QRS compared to RV apical pacing, which might reflect a more physiological and synchronous ventricular activation. Previous clinical studies, which did not consistently achieve RV septal pacing, were not confirmatory and need to be repeated. This review summarizes the anatomy of the RV septum, the radiographic appearances of pacing leads in the RV, the electrocardiograph correlates of RV septal lead positioning, and the techniques and tools required for implantation of an active-fixation lead onto the RV septum. Using the described techniques and tools, conventional active-fixation leads can now be reliably secured to either the RV outflow tract septum or mid-RV septum with very low complication rates and good long-term performance. Even though physiologic and hemodynamic studies on true RV septal pacing have not been completed, the detrimental effects of long-term RV apical pacing are significant enough to suggest that it is now time to leave the RV apex and secure all RV leads onto the septum.

  9. Assessment of right ventricular systolic function by tissue Doppler echocardiography

    DEFF Research Database (Denmark)

    Kjærgaard, Jesper

    2012-01-01

    myocardial velocities, but no changes in SR, strain or isovolumic acceleration could be observed [II and III]. Tissue Doppler echocardiography of the RV free wall in non-massive pulmonary embolism quantifies degree of RV dysfunction, and supports the existence of the McConnell sign of mid-ventricular RV...... structure including significant dilatation, but is well tolerated with only mild reduction in measures of global RV systolic function as estimated by 2D echocardiography in an experimental animal model. Regional RV myocardial function is also only mildly reduced. Also no differences in global or regional RV......This thesis summarizes a series of studies performed in order to assess the clinical usefulness of a novel echocardiographic technology that allows non-invasive assessment of regional right ventricular myocardial velocities and deformation: tissue Doppler echocardiography. While the technology...

  10. Right Ventricular Involvement in either Anterior or Inferior Myocardial Infarction

    Directory of Open Access Journals (Sweden)

    Firoozeh Abtahi

    2016-06-01

    Full Text Available Background: Unlike left ventricular function, less attention has been paid to Right Ventricular (RV function after Myocardial Infarction (MI. Objectives: The current study aimed to compare RV function in patients with inferior and anterior MI. Patients and Methods: During the study period, 60 patients consecutively presented to the Emergency Department with chest pain were divided into two groups based on their electrocardiographic findings. Accordingly, 25 patients had inferior MI (IMI group and 35 ones had anterior MI (AMI group. Echocardiography was performed 48 hours after starting the standard therapy. Conventional echocardiographic parameters and Tissue Doppler Imaging (TDI measurements were acquired from the standard views. Student t-test and the chi-square test were respectively used for comparisons of the normally distributed continuous and categorical variables in the two groups. Besides, P < 0.05 was considered to be statistically significant.

  11. A Novel Mathematical/Numerical Formula for Assessing Right Ventricular Torsion Using Echocardiographic Imaging

    CERN Document Server

    Ranjbar, Saeed

    2015-01-01

    Recently, the ventricular torsional parameters have received special attention because of their significant role in the ventricular systolic and diastolic function. Right ventricular (RV) rotational deformation is a sensitive index for RV performance but difficult to measure. Having assumed RV as a conic shape, the present study serves a novel mathematical formula of right ventricular rotation that uses velocity vector imaging (VVI) for quantifying RV.

  12. Right ventricular noncompaction in a neonate with complex congenital heart disease.

    Science.gov (United States)

    Alehan, Dursun; Dogan, Omer Faruk

    2005-08-01

    Ventricular noncompaction is a rare unclassified cardiomyopathy occurring because of arrest of the normal intrauterine compaction of the loose luminal component of the ventricular myocardium. There is limited data regarding its diagnosis and outcome in children. It is recognised, however, that right ventricular involvement is extremely rare. We report a case in which only the right ventricular myocardium was noncompacted, a situation which led to heart failure soon after birth.

  13. Impact of the right ventricular lead position on clinical outcome and on the incidence of ventricular tachyarrhythmias in patients with CRT-D

    DEFF Research Database (Denmark)

    Kutyifa, Valentina; Bloch Thomsen, Poul Erik; Huang, David T.;

    2013-01-01

    Data on the impact of right ventricular (RV) lead location on clinical outcome and ventricular tachyarrhythmias in cardiac resynchronization therapy with defibrillator (CRT-D) patients are limited.......Data on the impact of right ventricular (RV) lead location on clinical outcome and ventricular tachyarrhythmias in cardiac resynchronization therapy with defibrillator (CRT-D) patients are limited....

  14. [Long-term effect of polystan monocusp ventricular outflow patch after right ventricular outflow tract reconstruction].

    Science.gov (United States)

    Sugita, T; Matsumoto, M; Ogino, H; Nishizawa, J; Matsuyama, K; Yoshimura, S; Yoshioka, T; Tokuda, Y; Matsumura, M; Suda, K; Ueda, Y

    2000-09-01

    Forty-eight patients who underwent right ventricular outflow tract reconstruction with Monocusp Ventricular Outflow Patch (MVOP) fifty-five times and survived surgery, were reviewed in this study. Mean age at surgery was 6.4 years-old and mean follow-up interval was 75.2 months. There was no late death, however reoperation was performed 7 times. Freedom from reoperation rate was 97.2% and 80.7% after 5 and 10 years after surgery, respectively. The main cause for reoperation were right ventricular outflow obstruction RVOTO (5 cases). All of the RVOTO occurred at the distal end of the anastomosis. However, there was no RVOTO in patients who underwent RVOTR with MVOP during the past ten years. So, we considered the cause of RVOTO a technical problem. Pulmonary regurgitation was one to two degree early after surgery, and had worsened by almost two or three degrees more than 5 years after surgery. Moreover, five of six patients who underwent cardiac catheterization more than 10 years after surgery had three degrees of pulmonary regurgitation as well as a large CTR. In conclusion, according to long-term results, especially more than 10 years post operatively, pulmonary regurgitation was the most important problem.

  15. [Right ventricular outflow tract reconstruction with monocusp patch].

    Science.gov (United States)

    Ikeda, T; Yokota, Y; Okamoto, F; Nakayama, S; Matsuno, S; Ohtani, S; Nakanishi, K; Nishimori, H

    1990-02-01

    Two kinds of monocusp patch were used for reconstruction of right ventricular outflow tract in patients with a variety of congenital heart defects. A bovine pericardial patch with monocusp (MVOP) was used in 11 patients (group M), and a separate transannular outflow patch (STOP) with turned-down equine pericardial monocusp was used in 10 patients (group S). Mean age at operation was 9 years 5 months in group M, and 7 years 9 months in group S. There was one operative death in group M. Survivors have completed a mean follow-up of 19 months. There were no late deaths. In early postoperative period, mean peak systolic pressure gradient between right ventricle and main pulmonary artery was 21 +/- 11 mmHg in group M and 21 +/- 11 mmHg in group S, which were not statistically significant. During follow-up period, no significant increase of peak systolic pressure gradient was noted in both groups. In late postoperative period, pulmonary regurgitation was moderate in 7 and severe in 1 in group M, while it was non-significant in 1, mild in 6 and moderate in 2 in group S. The difference between two groups was significant (p < 0.005). In conclusion, both types of patches equally relieved right ventricular outflow obstruction, but STOP prevented postoperative pulmonary regurgitation more effectively.

  16. [Right-sided mechanical circulatory support in acute right ventricular failure in the dog].

    Science.gov (United States)

    Gabauer, I; Okolicány, J; Styk, J; Pancza, D; Holec, V

    1991-05-01

    The technique of surgically induced acute progressive right ventricular failure in experimental animals is described. It sumultates the hemodynamic situation of right ventricular failure in some patients after termination of extracorporeal circulation applied for carrying out procedures on the left ventricle. The described technique consists of rightsided longitudinal ventriculotomy, destruction of the tricuspid valve, and ligation of the right coronary artery. Nine control dogs died within two hours after induction of failure due to low stroke volume caused by low pulmonary and left atrial pressure. The use of rightsided support appliance draining blood from the right atrium in systole and pumping it into the trunk of the pulmonary artery in diastole by means of a membrane pump resulted in further 12 dogs in the restoration of left ventricular diastolic pressure, significant increase of aortal pressure (p less than 0.003) and stroke volume (p less than 0.003) and in a decrease of right atrial pressure (p less than 0.003). The study demonstrated that by using the described mechanical support the circulation can be adequately assisted so that the failing right side of the heart can get restored. (Tab.1,Fig.5,Ref.25.).

  17. Right ventricular outflow tract reconstructive model in adult sheep.

    Science.gov (United States)

    Rakow, Nancy; Barka, Noah; Nelson, Dale; Allen, Nan; Gringaard, Robyn; Falkner, Phillip; Wahlberg, Phil; Lemmon, Jack; Phillips, Lynette; Billstrom, Tina; Hill, Alex; Shecterle, Linda M; St Cyr, J A

    2007-01-01

    Patients born with congenital right ventricular outflow tract lesions are faced with invasive procedures to establish hemodynamic and physiological stability. Commonly, multiple subsequent surgical procedures are required due to deterioration of a previous repair. These procedures carry additive risks of mortality and morbidity. Less aggressive procedures with accompanying lower risk is ideal. Success in percutaneously placing a transcatheter valve has previously been reported; however, continued safety and efficacy of any technique needs continual assessment. We developed a model for preclinical evaluation of a percutaneous placement of a pulmonic transcatheter valve in adult sheep, including preoperative, surgical, and postoperative techniques for long-term evaluation. Adult sheep were assessed and determined to be acceptable for study enrollment. Perioperative antibiotics and analgesics were given prior to a left thoracotomy. A Medtronic, Hancock 1 valve conduit was inserted for reconstruction of the right ventricular outflow tract. The Hancock 1 valve conduit alone represented the control group and the test animals comprised the addition of a Melodytrade mark transcatheter pulmonary valve (TPV), within the Hancock 1 valve conduit. Fifteen adult sheep survived the surgical implant procedure with no perioperative mortality. There were four early postoperative deaths, three due to infection and one due to heart failure, secondary to intraoperative heart block. The remaining 11 animals remained healthy, gained weight, and survived to termination at 5 months. An initial definite-sized valve conduit was implanted, followed by inserting a single size TPV, which allowed a more accurate physiological assessment of any chosen valve. Our developed adult sheep model for percutaneous TPV implantation for right ventricular outflow tract lesions was successful for long-term assessment by utilizing our preoperative, surgical, and postoperative techniques.

  18. Successful management of late right ventricular perforation after pacemaker implantation

    Directory of Open Access Journals (Sweden)

    Amir K Bigdeli

    2010-01-01

    Full Text Available Amir K Bigdeli1, Andres Beiras-Fernandez1, Ingo Kaczmarek1, Christian Kowalski2, Michael Schmoeckel1, Bruno Reichart11Department of Cardiac Surgery, 2Department of Anesthesiology, Ludwig-Maximilians University, Munich, GermanyAbstract: Complications of pacemaker implantation include myocardial perforation, venous thrombosis, vegetations of the tricuspid valve or pacing lead, and tricuspid regurgitation. We report a patient presenting with a case of delayed ventricular lead perforation through the right ventricle. The lead was uneventfully extracted under transesophageal echocardiographic observation in the operating room with cardiac surgery backup.Keywords: pacemaker, lead perforation, complication

  19. The effect of open lung ventilation on right ventricular and left ventricular function in lung-lavaged pigs

    NARCIS (Netherlands)

    D.R. Miranda; L. Klompe; F. Cademartiri (Filippo); J.J. Haitsma (Jack); A. Palumbo (Alessandro); B.F. Lachmann (Burkhard); A.J.J.C. Bogers (Ad); D.A.M.P.J. Gommers (Diederik); J.J.M. Takkenberg (Hanneke)

    2006-01-01

    textabstractINTRODUCTION: Ventilation according to the open lung concept (OLC) consists of recruitment maneuvers, followed by low tidal volume and high positive end-expiratory pressure, aiming at minimizing atelectasis. The minimization of atelectasis reduces the right ventricular

  20. Assessment of right ventricular function by myocardial performance index in diabetic patients

    Directory of Open Access Journals (Sweden)

    Ayman Ahmed Abdelaziz

    2011-09-01

    Conclusion: Myocardial performance index is a useful noninvasive tool for the detection of early right ventricular systolic and diastolic dysfunction in diabetic patients, regardless of coexisting hypertension.

  1. Hemodynamic improvement by right ventricular septal pacing in elderly patients with chronic atrial fibrillation and slow ventricular response

    Institute of Scientific and Technical Information of China (English)

    Wei HUA; Shidong GUO; Shu ZHANG; Fangzheng WANG; Lida ZHI; Hongxia NIU; Xin CHEN

    2005-01-01

    Background and objectives Right ventricular apical (RVA) pacing has been reported impairing left ventricular (LV)performance. Alternative pacing sites in right ventricle (RV) has been explored to obtain better cardiac function. Our study was designed to compare the hemodynamic effects of right ventricular septal (RVS) pacing with RVA pacing. Methods Ten elderly patients with chronic atrial fibrillation (AF) and long RR interval or slow ventricular response (VR) received VVI pacing. The hemodynamic difference between RVS and RVA pacing were examined by transthoracic echocardiography (TTE). Results Pacing leads were implanted successfully at the RVA and then RVS in all patients without complication. The left ventricular (LV) parameters,measured during RVA pacing including left ventricular ejection fraction (LVEF), FS, stroke volume (SV) and peak E wave velocity (EV) were decreased significantly compared to baseline data, while during RVS pacing, they were significantly better than those during RVA pacing. However, after 3-6 weeks there was no statistical significant difference between pre- and post- RVS pacing.Conclusions The LV hemodynamic parameters during RVA pacing were significantly worse than baseline data. The short term LV hemodynamic parameters of RVS pacing were significantly better than those of RVA pacing; RVS pacing could improve the hemodynamic effect through maintaining normal ventricular activation sequence and biventricular contraction synchrony in patients with chronic AF and slow ventricular response.

  2. Right ventricular pacing: the best site is yet to be defined

    Institute of Scientific and Technical Information of China (English)

    Li ZHOU; Brian Olshansky

    2005-01-01

    @@ The right ventricular (RV) apex is the traditional site to provide stable and reliable chronic ventricular pacing. Interest in alternate site pacing has grown since RV apical pacing has been associated with increased mortality and morbidity compared to normal atrio-ventricular conduction. 1-4 Alternate pacing sites include the RV septum and outflow tract.

  3. Right ventricular dyssynchrony in patients with pulmonary hypertension is associated with disease severity and functional class

    Directory of Open Access Journals (Sweden)

    Murali Srinivas

    2005-08-01

    Full Text Available Abstract Background Abnormalities in right ventricular function are known to occur in patients with pulmonary arterial hypertension. Objective Test the hypothesis that chronic elevation in pulmonary artery systolic pressure delays mechanical activation of the right ventricle, termed dyssynchrony, and is associated with both symptoms and right ventricular dysfunction. Methods Fifty-two patients (mean age 46 ± 15 years, 24 patients with chronic pulmonary hypertension were prospectively evaluated using several echocardiographic parameters to assess right ventricular size and function. In addition, tissue Doppler imaging was also obtained to assess longitudinal strain of the right ventricular wall, interventricular septum, and lateral wall of the left ventricle and examined with regards to right ventricular size and function as well as clinical variables. Results In this study, patients with chronic pulmonary hypertension had statistically different right ventricular fractional area change (35 ± 13 percent, right ventricular end-systolic area (21 ± 10 cm2, right ventricular Myocardial Performance Index (0.72 ± 0.34, and Eccentricity Index (1.34 ± 0.37 than individuals without pulmonary hypertension (51 ± 5 percent, 9 ± 2 cm2, 0.27 ± 0.09, and 0.97 ± 0.06, p Conclusion Lower peak longitudinal right ventricular wall strain and significantly delayed time-to-peak strain values, consistent with right ventricular dyssynchrony, were found in a small heterogeneous group of patients with chronic pulmonary hypertension when compared to individuals without pulmonary hypertension. Furthermore, right ventricular dyssynchrony was associated with disease severity and compromised functional class.

  4. Right ventricular Hemodynamic Alteration after Pulmonary Valve Replacement in Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Hamid Bigdelian

    2015-03-01

    Full Text Available Introduction:  In patients who underwent surgery to repair Tetralogy of Fallot, right ventricular dilation from pulmonary regurgitation may be result in right ventricular failure, arrhythmias and cardiac arrest. Hence, pulmonary valve replacement may be necessary to reduce right ventricular volume overload. The aim of present study was to assess the effects of pulmonary valve replacement on right ventricular function after repair of Tetralogy of Fallot.   Materials and  Method:  This retrospective study was carried out between July 2011 and October 2013 on 21 consecutive patients in Chamran Heart Center (Esfahan. The study included 13 male (61.9% and 8 female (38.1%. Cardiac magnetic resonance was performed before, 6 and 12 months after pulmonary valve replacement in all patients (Babak Imaging Center, Tehran with the 1.5 Tesla system. The main reason for surgery at Tetralogy of Fallot repaired time was Tetralogy of Fallot + Pulmonary insufficiency (17 cases and Tetralogy of Fallot + Pulmonary atresia (4 cases. Right ventricular function was assessed before and after pulmonary valve replacement with Two-dimensional echocardiography and ttest was used to evaluate follow-up data.   Results:  Right ventricular end-diastolic volume, right ventricular end- systolic volume significantly decreased (P value ˂ 0.05.Right ventricular ejection fraction had a significant increase (P value ˂ 0.05. Right ventricular mass substantially shrank after pulmonary valve replacement. Moreover, pulmonary regurgitation noticeably decreased in patients. The other hemodynamic parameter such as left ventricular ejection fraction improved but was not significant (P value= 0.79. Conclusion:  Pulmonary valve replacement can successfully restores the impaired hemodynamic function of right ventricle which is caused by direct consequence of volume unloading in patient. Pulmonary valve surgery in children with Tetralogy of Fallot who have moderate to severe pulmonary

  5. Limitations and pitfalls in measurements of right ventricular stroke volume in an animal model of right heart failure.

    Science.gov (United States)

    Vildbrad, Mads Dam; Andersen, Asger; Andersen, Thomas Krarup; Axelgaard, Sofie; Holmboe, Sarah; Andersen, Stine; Ringgaard, Steffen; Nielsen-Kudsk, Jens Erik

    2015-05-01

    Right heart failure occurs in various heart and pulmonary vascular diseases and may be fatal. We aimed to identify limitations in non-invasive measurements of right ventricular stroke volume in an animal model of right ventricular failure. Data from previous studies randomising rats to pulmonary trunk banding (PTB, n = 33) causing pressure-overload right ventricular failure or sham operation (n = 16) was evaluated retrospectively. We measured right ventricular stroke volume by high frequency echocardiography and magnetic resonance imaging (MRI). We found correlation between right ventricular stroke volume measured by echocardiography and MRI in the sham animals (r = 0.677, p = 0.004) but not in the PTB group. Echocardiography overestimated the stroke volume compared to MRI in both groups. Intra- and inter-observer variation did not explain the difference. Technical, physiological and anatomical issues in the pulmonary artery might explain why echocardiography over-estimates stroke volume. Flow acceleration close to the pulmonary artery banding can cause uncertainties in the PTB model and might explain the lack of correlation. In conclusion, we found a correlation in right ventricular stroke volume measured by echocardiography versus MRI in the sham group but not the PTB group. Echocardiography overestimated right ventricular stroke volume compared to MRI.

  6. Primary angioplasty for infarction due to isolated right ventricular artery occlusion

    Institute of Scientific and Technical Information of China (English)

    Anwar; A; Chahal; Min-Young; Kim; Alexander; N; Borg; Yahya; Al-Najjar

    2014-01-01

    We report an unusual case of an isolated right ventricular infarction with haemodynamic compromise caused by spontaneous isolated proximal occlusion of the right ventricular branch of the right coronary artery(RCA), successfully treated by balloon angioplasty. A 58-yearold gentleman presented with epigastric pain radiating into both arms. Electrocardiograph with right ventricular leads confirmed ST elevation in V4 R and a diagnosis of isolated right ventricular infarction was made. Urgent primary percutaneous intervention was performed which revealed occlusion of the right ventricular branch of the RCA. During the procedure, the patient’s blood pressure dropped to 80/40 mm Hg, and echocardiography showed impaired right ventricular systolic function. Despite aggressive fluid resuscitation, the patient remained hypotensive, continued to have chest pain and persistent electrocardiograph changes, and hence balloon angioplasty was performed on the proximal right ventricular branch which restored flow to the vessel and revealed a severe ostial stenosis. This was treated with further balloon angioplasty which restored TIMI 3 flow with resolution of patient’s symptoms. Repeat echocardiography showed complete resolution of theST-elevation in leads V4 R and V5 R and partial resolution in V1. Subsequent dobutamine-stress echocardiography at 4 wk showed good left and right ventricular contractions. The patient was discharged after a 3-d inpatient stay without any complications.

  7. Two cases of giant Epsilon wave in patients with arrhythmogenic right ventricular dysplasia%致心律失常右心室心肌病心电图特征波新认识——附罕见粗大Epsilon波二例

    Institute of Scientific and Technical Information of China (English)

    陈静; 吴清华; 程晓曙; 洪葵; 俞建华; 邬涛; 杨人强; 李菊香; 黄慧萍; 胡金柱; 胡建新; 吴延庆

    2011-01-01

    @@ 致心律失常性右心室心肌病(ARVD)是主要累及右心室的非缺血性心肌病.90%的ARVD患者存在心电图异常,其中最常见的心电图改变为V1~V3导联T波倒置,大约25%~35%的患者存在特征性的Epsilon波.

  8. Quantitative assessment of regional right ventricular function with color kinesis.

    Science.gov (United States)

    Vignon, P; Weinert, L; Mor-Avi, V; Spencer, K T; Bednarz, J; Lang, R M

    1999-06-01

    We used color kinesis, a recent echocardiographic technique that provides regional information on the magnitude and timing of endocardial wall motion, to quantitatively assess regional right ventricular (RV) systolic and diastolic properties in 76 subjects who were divided into five groups, as follows: normal (n = 20), heart failure (n = 15), pressure/volume overload (n = 14), pressure overload (n = 12), and RV hypertrophy (n = 15). Quantitative segmental analysis of color kinesis images was used to obtain regional fractional area change (RFAC), which was displayed in the form of stacked histograms to determine patterns of endocardial wall motion. Time curves of integrated RFAC were used to objectively identify asynchrony of diastolic endocardial motion. When compared with normal subjects, patients with pressure overload or heart failure exhibited significantly decreased endocardial motion along the RV free wall. In the presence of mixed pressure/volume overload, the markedly increased ventricular septal motion compensated for decreased RV free wall motion. Diastolic endocardial wall motion was delayed in 17 of 72 segments (24%) in patients with RV pressure overload, and in 31 of 90 segments (34%) in patients with RV hypertrophy. Asynchrony of diastolic endocardial wall motion was greater in the latter group than in normal subjects (16% versus 10%: p kinesis images allows quantitative assessment of regional RV systolic and diastolic properties.

  9. Initial In Vivo Evaluation of the DexAide Right Ventricular Assist Device

    OpenAIRE

    Ootaki, Yoshio; Kamohara, Keiji; Akiyama, Masatoshi; Zahr, Firas; Kopcak, Michael W.; Dessoffy, Raymond AA; Massiello, Alex; Horvath, David; Chen, Ji-Feng; Benefit, Stephen; Golding, Leonard A. R.; Fukamachi, Kiyotaka

    2005-01-01

    Objectives: Despite the increasing use of left ventricular assist devices for patients with end-stage congestive heart failure, no implantable, centrifugal right ventricular assist devices (RVADs) are available for those patients with significant right ventricular failure. The DexAide RVAD was developed to provide an implantable RVAD option to surgeons. The aim of this study was to evaluate pump performance in an acute in vivo model.

  10. Independent effects of both right and left ventricular function on plasma brain natriuretic peptide

    DEFF Research Database (Denmark)

    Vogelsang, Thomas Wiis; Jensen, Ruben J; Monrad, Astrid L;

    2007-01-01

    BACKGROUND: Brain natriuretic peptide (BNP) is increased in heart failure; however, the relative contribution of the right and left ventricles is largely unknown. AIM: To investigate if right ventricular function has an independent influence on plasma BNP concentration. METHODS: Right (RVEF), left......, which is a strong prognostic marker in heart failure, independently depends on both left and right ventricular systolic function. This might, at least in part, explain why BNP holds stronger prognostic value than LVEF alone....

  11. Model for end-stage liver disease predicts right ventricular failure in patients with left ventricular assist devices.

    Science.gov (United States)

    Yost, Gardner L; Coyle, Laura; Bhat, Geetha; Tatooles, Antone J

    2016-03-01

    High rates of right ventricular failure continue to affect postoperative outcomes in patients implanted with left ventricular assist devices (LVADs). Development of right ventricular failure and implantation with right ventricular assist devices is known to be associated with significantly increased mortality. The model for end-stage liver disease (MELD) score is an effective means of evaluating liver dysfunction. We investigated the prognostic utility of postoperative MELD on post-LVAD implantation outcomes. MELD scores, demographic data, and outcomes including length of stay, survival, and postoperative right ventricular failure were collected for 256 patients implanted with continuous flow LVADs. Regression and Kaplan-Meier analyses were used to investigate the relationship between MELD and all outcomes. Increased MELD score was found to be an independent predictor of both right heart failure and necessity for RVAD implantation (OR 1.097, CI 1.040-1.158, p = 0.001; OR 1.121, CI 1.015, p = 0.024, respectively). Patients with RV failure and who underwent RVAD implantation had reduced postoperative survival compared to patients with RV dysfunction (no RV failure = 651.4 ± 609.8 days, RV failure = 392.6 ± 444.8 days, RVAD = 89.3 ± 72.8 days; p right heart failure and the necessity for RVAD implantation. Those patients with RV failure and RVADs experience significantly increased postoperative mortality compared to those without RV dysfunction.

  12. Right ventricular dysfunction after cardiac surgery - diagnostic options

    DEFF Research Database (Denmark)

    Grønlykke, Lars; Ravn, Hanne Berg; Gustafsson, Finn;

    2017-01-01

    Right ventricular (RV) failure after cardiac surgery is associated with an ominous prognosis. The etiology of RV failure is multifaceted and the ability to recognize RV failure early is paramount in order to initiate timely treatment. The present review focuses on different diagnostic modalities......, reproducibility and comparability of the next generation of diagnostic modalities we propose to use simple, but obtainable echocardiographic measurements and ultimately the insertion of a pulmonary artery catheter (PAC) in order to diagnose RV failure after cardiac surgery....... for RV function and discusses the normal versus abnormal findings in RV monitoring after cardiac surgery and the limitations of the applicable diagnostic modalities. There are specific challenges in RV assessment after cardiac surgery due to a loss of longitudinal contraction and a concomitant gain...

  13. Non-invasive diagnosis and follow-up of right ventricular overload

    NARCIS (Netherlands)

    Henkens, Ivo Reinier

    2008-01-01

    Right ventricular overload covers a spectrum ranging from volume overload to pressure overload, and often is a combination of these, compromising cardiac function. Part I focuses on right ventricular volume overload in adults with Fallot’s tetralogy corrected in early childhood. We determined whic

  14. Left and right ventricular contributions to the formation of the interventricular septum in the mouse heart

    NARCIS (Netherlands)

    D. Franco; S.M. Meilhac; V.M. Christoffels; A. Kispert; M. Buckingham; R.G. Kelly

    2006-01-01

    Mammalian heart development involves complex morphogenetic events which lead to the formation of fully separated left and right atrial and ventricular chambers from a tubular heart. Separation of left and right ventricular chambers is dependent on a single structure, the interventricular septum (IVS

  15. Right ventricular function in patients with mitral valve disease; Evaluation by radionuclide blood pool scan

    Energy Technology Data Exchange (ETDEWEB)

    Hiraki, Yoshio; Shimizu, Mitsuharu; Noriyasu, Toshiaki; Nakagawa, Tomio; Aono, Kaname; Yanagi, Hidekiyo; Seno, Yoshimasa; Teramoto, Shigeru; Nagaya, Isao (Okayama Univ. (Japan). School of Medicine)

    1989-10-01

    Right ventricular function was studied in 13 patients with mitral valve stenosis (MS), 10 patients with mitral valve regurgitation (MR) and 10 patients after mitral valve replacement (MVR) with radionuclide blood pool scan. In MS, right ventricular end-diastolic and end-systolic volumes were larger than MVR. In MR, right ventricular ejection fraction (RVEF) was smaller and right ventricular end-systolic volume was larger than MVR. In both MS and MR, there was no significant linear correlation between RVEF and mean pulmonary arterial pressure (mPAP) at rest, but during exercise RVEF of patients with elevated mPAP decreased more than that of patients with normal mPAP. RVEF in patients with MS and MR was significantly decreased during exercise, while that in patients after MVR showed no significant change. Radionuclide blood pool scan seems to be useful for the evaluation of right ventricular function in mitral valve disease. (author).

  16. Isolated Left Ventricular Apical Hypoplasia with Right Ventricular Outflow Tract Obstruction: A Rare Combination.

    Science.gov (United States)

    Zhao, Yonghui; Zhang, Jiaying; Zhang, Jing

    2015-09-01

    Isolated left ventricular (LV) apical hypoplasia is a unusual and recently recognized congenital cardiac anomaly. A 19-year-old man was found to have an abnormal ECG and cardiac murmur identified during a routine health check since joining work. His ECG revealed normal sinus rhythm, right-axis deviation, poor R wave progression, and T wave abnormalities. On physical examination, a 2/6~3/6 systolic murmur was heard at the second intercostal space along the left sternal border. Subsequent echocardiography and cardiac magnetic resonance imaging confirmed the LV apical hypoplasia. Of note, we first found that LV apical hypoplasia was accompanied by RV outflow tract obstruction due to exaggerated rightward bulging of the basal-anterior septum during systole. A close follow-up was performed for the development of heart failure, pulmonary hypertension, and potentially tachyarrhythmia.

  17. Echocardiographic evaluation of right ventricular function in congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    Yiu-fai Cheung

    2014-01-01

    Objective This review aims to provide an overview of conventional and novel indices used in clinical and research arenas for evaluation of right ventricular (RV) function in congenital heart diseases with a dual-chambered circulation.Data sources Articles cited in this review were selected using PubMed search of publications in English with no date limits.The search terms included "echocardiography","right ventricle","RV function","cardiac function",and "congenital heart disease".Key references were also searched for additional publications.Study selection Articles related to description of echocardiographic techniques in the evaluation of subpulmonary or systemic RV function and their applications in congenital cardiac malformations were retrieved and reviewed.Results Three approaches have been used to evaluate subpulmonary and systemic RV function:(1) assessment of changes in RV size in the cardiac cycle,(2) determination of Doppler-derived velocities and systolic and diastolic time intervals,and (3) quantification of myocardial velocities and deformation.Conclusions Conventional and novel echocardiographic techniques enable the evaluation of subpulmonary and systemic RV function.Novel echocardiographic techniques have further allowed quantification of RV volumes and direct interrogation of myocardial deformation.These new techniques show promise in a more comprehensive evaluation beyond "eye-bailing" of RV function in the growing population of adolescent and adult congenital heart patients.

  18. Right ventricular cardiomyopathies: a multidisciplinary approach to diagnosis.

    Science.gov (United States)

    Limongelli, Giuseppe; Rea, Alessandra; Masarone, Daniele; Francalanci, M Paola; Anastasakis, Aris; Calabro', Raffaele; Giovanna, Russo Maria; Bossone, Eduardo; Elliott, Perry Mark; Pacileo, Giuseppe

    2015-01-01

    The physiological importance of the right ventricle (RV) has been underestimated over the past years. Finally in the early 1950s through the 1970s, cardiac surgeons recognized the importance of RV function. Since then, the importance of RV function has been recognized in many acquired cardiac heart disease. RV can be mainly or together with left ventricle (LV) affected by inherited or acquired cardiomyopathy. In fact, RV morphological and functional remodeling occurs more common during cardiomyopathies than in ischemic cardiomyopathies and more closely parallels LV dysfunction. Moreover, there are some cardiomyopathy subtypes showing a predominant or exclusive involvement of the RV, and they are probably less known by cardiologists. The clinical approach to right ventricular cardiomyopathies is often challenging. Imaging is the first step to raise the suspicion and to guide the diagnostic process. In the differential diagnosis, cardiologists should consider athlete's heart, congenital heart diseases, multisystemic disorders, and inherited arrhythmias. However, a multiparametric and multidisciplinary approach, involving cardiologists, experts in imaging, geneticists, and pathologists with a specific expertise in these heart muscle disorders is required.

  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. Subharmonic microbubble emissions for noninvasively tracking right ventricular pressures.

    Science.gov (United States)

    Dave, Jaydev K; Halldorsdottir, Valgerdur G; Eisenbrey, John R; Raichlen, Joel S; Liu, Ji-Bin; McDonald, Maureen E; Dickie, Kris; Wang, Shumin; Leung, Corina; Forsberg, Flemming

    2012-07-01

    Right heart catheterization is often required to monitor intra-cardiac pressures in a number of disease states. Ultrasound contrast agents can produce pressure modulated subharmonic emissions that may be used to estimate right ventricular (RV) pressures. A technique based on subharmonic acoustic emissions from ultrasound contrast agents to track RV pressures noninvasively has been developed and its clinical potential evaluated. The subharmonic signals were obtained from the aorta, RV, and right atrium (RA) of five anesthetized closed-chest mongrel dogs using a SonixRP ultrasound scanner and PA4-2 phased array. Simultaneous pressure measurements were obtained using a 5-French solid state micromanometer tipped catheter. Initially, aortic subharmonic signals and systemic blood pressures were used to obtain a calibration factor in units of millimeters of mercury per decibel. This factor was combined with RA pressures (that can be obtained noninvasively) and the acoustic data from the RV to obtain RV pressure values. The individual calibration factors ranged from -2.0 to -4.0 mmHg/dB. The subharmonic signals tracked transient changes in the RV pressures within an error of 0.6 mmHg. Relative to the catheter pressures, the mean errors in estimating RV peak systolic and minimum diastolic pressures, and RV relaxation [isovolumic negative derivative of change in pressure over time (-dP/dt)] by use of the subharmonic signals, were -2.3 mmHg, -0.8 mmHg, and 2.9 mmHg/s, respectively. Overall, acoustic estimates of RV peak systolic and minimum diastolic pressures and RV relaxation were within 3.4 mmHg, 1.8 mmHg, and 5.9 mmHg/s, respectively, of the measured pressures. This pilot study demonstrates that subharmonic emissions from ultrasound contrast agents have the potential to noninvasively track in vivo RV pressures with errors below 3.5 mmHg.

  1. Electrically contractile polymers augment right ventricular output in the heart.

    Science.gov (United States)

    Ruhparwar, Arjang; Piontek, Patricia; Ungerer, Matthias; Ghodsizad, Ali; Partovi, Sasan; Foroughi, Javad; Szabo, Gabor; Farag, Mina; Karck, Matthias; Spinks, Geoffrey M; Kim, Seon Jeong

    2014-12-01

    Research into the development of artificial heart muscle has been limited to assembly of stem cell-derived cardiomyocytes seeded around a matrix, while nonbiological approaches to tissue engineering have rarely been explored. The aim of the study was to apply electrically contractile polymer-based actuators as cardiomyoplasty for positive inotropic support of the right ventricle. Complex trilayer polypyrrole (PPy) bending polymers for high-speed applications were generated. Bending motion occurred directly as a result of electrochemically driven charging and discharging of the PPy layers. In a rat model (n = 5), strips of polymers (3 × 20 mm) were attached and wrapped around the right ventricle (RV). RV pressure was continuously monitored invasively by direct RV cannulation. Electrical activation occurred simultaneously with either diastole (in order to evaluate the polymer's stand-alone contraction capacity; group 1) or systole (group 2). In group 1, the pressure generation capacity of the polymers was measured by determining the area under the pressure curve (area under curve, AUC). In group 2, the RV pressure AUC was measured in complexes directly preceding those with polymer contraction and compared to RV pressure complexes with simultaneous polymer contraction. In group 1, the AUC generated by polymer contraction was 2768 ± 875 U. In group 2, concomitant polymer contraction significantly increased AUC compared with complexes without polymer support (5987 ± 1334 U vs. 4318 ± 691 U, P ≤ 0.01). Electrically contractile polymers are able to significantly augment right ventricular contraction. This approach may open new perspectives for myocardial tissue engineering, possibly in combination with fetal or embryonic stem cell-derived cardiomyocytes.

  2. Massive right-sided cardiac thrombosis in Chagas' heart disease without left ventricular dysfunction.

    Science.gov (United States)

    Bestetti, Reinaldo B; Corbucci, Hélio A R; Cardinalli-Neto, Augusto

    2011-02-01

    A 63-year-old woman with the diagnosis of mega-oesophagus secondary to chronic Chagas' disease and no past cardiac history was referred for cardiac evaluation. The resting ECG showed right bundle-branch block, whereas a 2-D echocardiogram revealed marked right ventricular dilatation with hypokinesia, right atrial dilatation, normal pulmonary artery pressure, and normal left ventricular ejection fraction. A large, irregularly shaped mass, arising from the right atrium and protruding into the right ventricle through the tricuspid valve, with several different bizarre forms inside the right atrium during systole and/or diastole was seen on 2-D echocardiogram. Therefore, massive right-sided thrombosis can be detected in Chagas' disease patients with no overt right- and left-sided ventricular failure.

  3. Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Sherwin S. [Children' s Mercy Hospital and Clinics, Department of Radiology, Kansas City, MO (United States); Whitehead, Kevin K.; Kim, Timothy S.; Fu, Gregory L.; Fogel, Mark A.; Harris, Matthew A. [Children' s Hospital of Philadelphia, Department of Cardiology, Philadelphia, PA (United States); Keller, Marc S. [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States)

    2015-09-15

    There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m{sup 2} vs. 118 ± 30 mL/m{sup 2}), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation. (orig.)

  4. QRS fragmentation: its role in sherlocking the arrhythmogenic heart

    Directory of Open Access Journals (Sweden)

    Hafeez Ul Hassan Virk

    2016-07-01

    Full Text Available The electrocardiogram (ECG is a commonly available basic diagnostic modality in in-patient, out-patient, and emergency departments. In patients with coronary artery disease (CAD, the presence of a fragmented QRS (f-QRS, which is an extra R wave (R′, notching of the single R wave, notching of the S wave in at least two contiguous leads on the 12-lead ECG, is associated with a myocardial scar from previous myocardial injury. Furthermore, the presence of f-QRS has been shown to be associated with adverse outcomes in CAD and non-CAD patients. In the present paper, we will solely focus on the usefulness and utilization of f-QRS in predicting ventricular tachyarrhythmia in many heart diseases, that is, ischemic cardiomyopathy, non-ischemic cardiomyopathy, hypertrophic obstructive cardiomyopathy, Brugada syndrome, and arrhythmogenic right ventricular cardiomyopathy. In the majority of such cases, ventricular tachyarrhythmia results in sudden cardiac death. Diagnosing them beforehand can lead to prevention and/or early treatment of these arrhythmias to prevent potential morbidity and mortality.

  5. Percutaneous mechanical assist for severe cardiogenic shock due to acute right ventricular failure.

    Science.gov (United States)

    Kipp, Ryan; Raval, Amish N

    2015-05-01

    Acute right ventricular failure can lead to severe cardiogenic shock and death. Recovery may be achieved with early supportive measures. In many patients, intravenous fluid and inotropic resuscitation is inadequate to improve cardiac output. In these cases, percutaneous mechanical assist may provide a non-surgical bridge to recovery. Herein, we describe a case series of patients with severe, refractory cardiogenic shock due to acute right ventricular failure who received a continuous flow percutaneous ventricular device primarily utilizing the right internal jugular vein for out flow cannula placement.

  6. Screening for, and management of, possible arrhythmogenic syndromes (channelopathies/ion channel diseases)

    DEFF Research Database (Denmark)

    Svendsen, Jesper Hastrup; Geelen, Peter

    2010-01-01

    This survey assesses the current management strategies for individuals with electrocardiographic features, suggesting an arrhythmogenic syndrome [including long QT syndrome (LQTS), Brugada syndrome (BS), catecholaminergic polymorphic ventricular tachycardia (CPVT) or short QT syndrome] or family...

  7. Serial Doppler echocardiographic assessment of left and right ventricular performance after a first myocardial infarction

    DEFF Research Database (Denmark)

    Møller, J E; Søndergaard, E; Poulsen, S H;

    2001-01-01

    We sought to investigate the relation between left ventricular (LV) and right ventricular (RV) function assessed with the Doppler-derived myocardial performance index (MPI), to assess serial changes, and to investigate the prognostic value of biventricular assessment of cardiac function after a f...

  8. Right atrial pressure affects the interaction between lung mechanics and right ventricular function in spontaneously breathing COPD patients.

    Directory of Open Access Journals (Sweden)

    Bart Boerrigter

    Full Text Available INTRODUCTION: It is generally known that positive pressure ventilation is associated with impaired venous return and decreased right ventricular output, in particular in patients with a low right atrial pressure and relative hypovolaemia. Altered lung mechanics have been suggested to impair right ventricular output in COPD, but this relation has never been firmly established in spontaneously breathing patients at rest or during exercise, nor has it been determined whether these cardiopulmonary interactions are influenced by right atrial pressure. METHODS: Twenty-one patients with COPD underwent simultaneous measurements of intrathoracic, right atrial and pulmonary artery pressures during spontaneous breathing at rest and during exercise. Intrathoracic pressure and right atrial pressure were used to calculate right atrial filling pressure. Dynamic changes in pulmonary artery pulse pressure during expiration were examined to evaluate changes in right ventricular output. RESULTS: Pulmonary artery pulse pressure decreased up to 40% during expiration reflecting a decrease in stroke volume. The decline in pulse pressure was most prominent in patients with a low right atrial filling pressure. During exercise, a similar decline in pulmonary artery pressure was observed. This could be explained by similar increases in intrathoracic pressure and right atrial pressure during exercise, resulting in an unchanged right atrial filling pressure. CONCLUSIONS: We show that in spontaneously breathing COPD patients the pulmonary artery pulse pressure decreases during expiration and that the magnitude of the decline in pulmonary artery pulse pressure is not just a function of intrathoracic pressure, but also depends on right atrial pressure.

  9. [Ventricular tachyarrhythmias in patients with cardiomyopathy

    DEFF Research Database (Denmark)

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

    2008-01-01

    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......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......), 57 (45%) patients had arrhythmogenic right ventricular cardiomyopathy (ARVC) and 13 (10%) had hypertrophic cardiomyopathy (HCM). The average age was 44 years for HCM, 41 years for ARVC and 58 years for DCM. The majority of the patients were male. ICD treatment was used in 95% of the patients...

  10. Prostacyclin therapy increases right ventricular capillarisation in a model for flow-associated pulmonary hypertension

    NARCIS (Netherlands)

    van Albada, Mirjam E.; Berger, Rolf M. F.; Niggebrugge, Marnix; van Veghel, Richard; Cromme-Dijkhuis, Adri H.; Schoemaker, Regien G.

    2006-01-01

    Pulmonary hypertension, and consequently right ventricular failure, complicates several congenital heart defects. Although intervention in the prostacyclin-thromboxane ratio is known to improve outcome, the underlying mechanism is not clear. Therefore, effects of acetyl salicylic acid and iloprost a

  11. Acute inferior myocardial infarction with right ventricular infarction is more prone to develop cardiogenic shock.

    Science.gov (United States)

    Bari, M A; Roy, A K; Islam, M Z; Aditya, G; Bhuiyan, A S

    2015-01-01

    Cardiogenic shock is rare in isolated acute inferior myocardial infarction but there is relationship of cardiogenic shock with inferior myocardial infarction if associated with right ventricular infarction. A prospective study was carried out to see the association of cardiogenic shock with inferior myocardial infarction if associated with right ventricular infarction. This study was conducted from January 2011 to November 2011. A total of 100 cases were selected as study population which was taken from the Department of Cardiology, Mymensingh Medical College Hospital, Mymensingh, Bangladesh. Among them 50 were in Group A and 50 were in Group B. Group A was the patients of acute myocardial infarction with right ventricular infarction. Group B was the patients of acute myocardial infarction without right ventricular infarction. It revealed that 9(18%) in Group A and 3(6%) in Group B developed cardiogenic shock which is statistically significant (pcardiogenic shock.

  12. Isolated right ventricular cardiomyopathy with autoimmune hypothyroidism: a rare association in an adolescent

    Science.gov (United States)

    Yelve, Kavita; Panandikar, Gajanan Ashok; Pazare, Amar; Bajpai, Smrati

    2015-01-01

    A 13-year-old girl presented with progressive dyspnoea and palpitation, diagnosed on echocardiography as primary right ventricular cardiomyopathy with atrial fibrillation. Her thyroid profile was positive for antithyroid microsomal antibody, and antithyroid peroxidase antibodies were suggestive of autoimmune hypothyroidism. She was managed with furosemide, digoxin, acenocoumarol and thyroxine following which she showed significant improvement. This is a rare case of isolated right ventricular cardiomyopathy and its association with autoimmune hypothyroidism presenting at the age of 13. PMID:25795745

  13. Large right ventricular fibroma in a 6-month-old infant.

    Science.gov (United States)

    Horovitz, Alice; van Geldorp, Irene E; Roubertie, François; Thambo, Jean-Benoit

    2012-12-01

    This report describes the case of a 6-month-old girl with a large cardiac fibroma in the right ventricle. Ventricular tachycardia associated with the fibroma was successfully treated with amiodarone. At the age of 3 years, surgical resection was indicated because of right ventricular outflow tract obstruction caused by progression of the tumor. The fibroma was successfully resected, and further follow-up evaluation was uneventful.

  14. Isolated right ventricular infarct presenting as ventricular fibrillation arrest and confirmed by delayed-enhancement cardiac MRI.

    Science.gov (United States)

    Cavalcante, João L; Al-Mallah, Mouaz; Hudson, Michael

    2010-10-01

    Malignant ventricular arrhythmias resulting from isolated right ventricular myocardial infarction (RVMI) without left ventricular myocardial ischaemia or infarction occur rarely. We present a case of a 61 year-old male with acute onset of chest pain and ventricular fibrillation cardiac arrest requiring prompt defibrillation. Subsequent 15-lead EKG, showed ST-segment elevation in the anterior and right precordial leads without ST-segment elevation in the inferior leads. Angiography documented occlusion of a large RV marginal branch. Delayed enhancement cardiac magnetic resonance imaging (DE-CMR) with gadolinium performed two days post-infarct showed isolated RVMI. Patient remained symptom free and haemodynamically stable throughout his hospital stay. The clinical presentation of isolated RV infarct can be misleading and diagnosis difficult. EKG findings can resemble acute anterior wall myocardial infarction, while its course can be accompanied by life-threatening ventricular arrhythmias. This case uniquely combines this rare clinical sequence with DE-CMR images using gadolinium to confirm isolated RVMI. A brief review of RVMI presentation and associated EKG patterns is also discussed.

  15. Effects of CPAP on right ventricular myocardial performance index in obstructive sleep apnea patients without hypertension

    Directory of Open Access Journals (Sweden)

    Gür Sükrü

    2006-02-01

    Full Text Available Abstract Objectives Obstructive sleep apnoea (OSA might cause right ventricular dysfunction and pulmonary hypertension. We aimed to determine the effects of nasal continuous positive airway pressure (CPAP therapy on right ventricular myocardial performance index (MPI in OSA patients without hypertension. Methods 49 subjects without hypertension, diabetes mellitus, any cardiac and pulmonary disease had overnight polysomnography and echocardiography. In 18 moderate-severe OSA (apnea-hypopnea index ≥ 15 patients, right ventricular free wall diameter (RVFWD was measured by M-mode, and right ventricular MPI was calculated as (isovolumic contraction time+ isovolumic relaxation time / pulmonary ejection time using Doppler at baseline and after 6 months CPAP therapy. Results Mean age was 46.5 ± 4.9 year. Patients had high body mass index (BMI: 30.6 ± 4,0 kg/m2, but there was no change in either BMI or blood pressures after 6 months. Right ventricular end-diastolic and end-systolic diameters were in normal limits at baseline, and did not change after CPAP usage. Baseline RVFWD (7.1 ± 2.1 mm significantly decreased after CPAP therapy (6.2 ± 1.7 mm, p Conclusion CPAP therapy significantly decreases RVFWD and improves right ventricular diastolic and global functions (MPI in OSA patients without hypertension.

  16. Right ventricular wall abscess in structurally normal heart after leg osteomyelitis: First case.

    Science.gov (United States)

    Ahmad, Tanveer; Pasarad, Ashwini Kumar; Kishore, Kolkebaile Sadanand; Maheshwarappa, Nandakumar Neralakere

    2016-09-01

    A 3-year-old girl presented with fever and acute dyspnea for 4 days. She had suffered an injury to the left lower leg 3 weeks earlier, with abscess formation. Magnetic resonance imaging showed osteomyelitis of the lower tibia. Echocardiography showed a mass in the right ventricular wall. She underwent concomitant heart surgery for removal of the right ventricular mass and limb arthrotomy. We believe this is a first reported case in which a ventricular wall abscess developed in a structurally normal heart following leg osteomyelitis.

  17. Ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia.

    Science.gov (United States)

    Malik, Rabiya; Zilberman, Mark V; Tang, Liwen; Miller, Susan; Pandian, Natesa G

    2015-03-01

    Ectopia cordis, defined as partial or complete displacement of the heart outside of the thoracic cavity, is a rare congenital malformation. If not surgically corrected during the early years of life, ectopia cordis can prove to be a fatal abnormality. However, due to the presence of multiple intracardiac and extracardiac malformations, a corrective surgery might not always be successful. The pathology of ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia is discussed, highlighting the complexities involved in such a rare disorder.

  18. Sildenafil attenuates pulmonary inflammation and fibrin deposition, mortality and right ventricular hypertrophy in neonatal hyperoxic lung injury

    Directory of Open Access Journals (Sweden)

    Boersma Hester

    2009-04-01

    Full Text Available Abstract Background Phosphodiesterase-5 inhibition with sildenafil has been used to treat severe pulmonary hypertension and bronchopulmonary dysplasia (BPD, a chronic lung disease in very preterm infants who were mechanically ventilated for respiratory distress syndrome. Methods Sildenafil treatment was investigated in 2 models of experimental BPD: a lethal neonatal model, in which rat pups were continuously exposed to hyperoxia and treated daily with sildenafil (50–150 mg/kg body weight/day; injected subcutaneously and a neonatal lung injury-recovery model in which rat pups were exposed to hyperoxia for 9 days, followed by 9 days of recovery in room air and started sildenafil treatment on day 6 of hyperoxia exposure. Parameters investigated include survival, histopathology, fibrin deposition, alveolar vascular leakage, right ventricular hypertrophy, and differential mRNA expression in lung and heart tissue. Results Prophylactic treatment with an optimal dose of sildenafil (2 × 50 mg/kg/day significantly increased lung cGMP levels, prolonged median survival, reduced fibrin deposition, total protein content in bronchoalveolar lavage fluid, inflammation and septum thickness. Treatment with sildenafil partially corrected the differential mRNA expression of amphiregulin, plasminogen activator inhibitor-1, fibroblast growth factor receptor-4 and vascular endothelial growth factor receptor-2 in the lung and of brain and c-type natriuretic peptides and the natriuretic peptide receptors NPR-A, -B, and -C in the right ventricle. In the lethal and injury-recovery model we demonstrated improved alveolarization and angiogenesis by attenuating mean linear intercept and arteriolar wall thickness and increasing pulmonary blood vessel density, and right ventricular hypertrophy (RVH. Conclusion Sildenafil treatment, started simultaneously with exposure to hyperoxia after birth, prolongs survival, increases pulmonary cGMP levels, reduces the pulmonary

  19. Reflex Seizures Triggered by Exposure to Characters With Numerical Value: A Case With Right Temporal Cortical Dysplasia.

    Science.gov (United States)

    Erdener, Şefik Evren; Tezer, F Irsel; Oğuz, Kader K; Kamışlı, Özden; Ergün, Eser Lay; Söylemezoğlu, Figen; Saygi, Serap

    2016-07-01

    Reflex seizures can be triggered by a variety of stimuli. We present a case with drug-resistant complex partial seizures originating in right temporal lobe triggered extensively by visual, auditory, and mental exposure to multidigit numbers. The patient was investigated in video-EEG monitoring unit and seizures were triggered by numerical stimuli. Scalp EEG findings suggested a right temporal focus but ictal semiological findings suspicious for an extratemporal area necessitated the invasive EEG study. A right anterior temporal seizure focus was established with invasive monitoring and cortical stimulation studies. Magnetic resonance imaging showed a cortical dysplasia in right anterior temporal lobe and ictal single-photon emission computed tomography confirmed the epileptogenic focus, leading to a right temporal lobectomy and amygdalohippocampectomy and a pathological diagnosis of focal cortical dysplasia type Ia. The patient is seizure-free at the end of the second postoperative year despite repeated exposures to numbers. To our knowledge, this is the first report of seizures triggered by numbers. It is also of particular importance as the reflex seizures are associated with a cortical lesion and it may suggest involvement of right anterior temporal lobe in numerical processing.

  20. [Ventricular tachyarrhythmias. A retrospective analysis of etiology, demography and treatment

    DEFF Research Database (Denmark)

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

    2008-01-01

    to ventricular tachyarrhythmias. MATERIALS AND METHODS: We conducted a retrospective review of 993 patients discharged from Rigshospitalet over 6 years and 5 months with the diagnostic codes ventricular tachycardia, ventricular fibrillation or premature ventricular contractions. RESULTS: The population had...... an average age of 59 years (ranging 15-95 years) with a majority of males (76%). Among the patients with known etiology ischemic heart disease (60%), dilated cardiomyopathy (6%) and arrhythmogenic right ventricular cardiomyopathy (6%) were the most frequent. A substantial number of the patients (15%) had...... unknown etiology; 492 (50%) of the patients overall had an ICD implanted, the majority of whom had been categorized as having ventricular tachycardia (92%); 168 patients had previous cardiac arrest, 127 of whom did not have a potential reversible cause. Of this group 75 (59%) had an ICD implanted...

  1. Right Ventricular Enlargement within Months of Arteriovenous Fistula Creation in 2 Hemodialysis Patients.

    Science.gov (United States)

    Ragupathi, Loheetha; Johnson, Drew; Marhefka, Gregary D

    2016-08-01

    Surgically created arteriovenous fistulae (AVF) for hemodialysis can contribute to hemodynamic changes. We describe the cases of 2 male patients in whom new right ventricular enlargement developed after an AVF was created for hemodialysis. Patient 1 sustained high-output heart failure solely attributable to the AVF. After AVF banding and subsequent ligation, his heart failure and right ventricular enlargement resolved. In Patient 2, the AVF contributed to new-onset right ventricular enlargement, heart failure, and ascites. His severe pulmonary hypertension was caused by diastolic heart failure, diabetes mellitus, and obstructive sleep apnea. His right ventricular enlargement and heart failure symptoms did not improve after AVF ligation. We think that our report is the first to specifically correlate the echocardiographic finding of right ventricular enlargement with AVF sequelae. Clinicians who treat end-stage renal disease patients should be aware of this potential sequela of AVF creation, particularly in the upper arm. We recommend obtaining preoperative echocardiograms in all patients who will undergo upper-arm AVF creation, so that comparisons can be made postoperatively. Alternative consideration should be given to creating the AVF in the radial artery, because of less shunting and therefore less potential for right-sided heart failure and pulmonary hypertension. A multidisciplinary approach is optimal when selecting patients for AVF banding or ligation.

  2. Transcatheter stenting of the right ventricular outflow tract augments pulmonary arterial growth in symptomatic infants with right ventricular outflow tract obstruction and hypercyanotic spells.

    Science.gov (United States)

    McGovern, Eimear; Morgan, Conall T; Oslizlok, Paul; Kenny, Damien; Walsh, Kevin P; McMahon, Colin J

    2016-10-01

    We retrospectively reviewed all the children with right ventricular outflow tract obstruction, hypoplastic pulmonary annulus, and pulmonary arteries who underwent stenting of the right ventricular outflow tract for hypercyanotic spells at our institution between January, 2008 and December, 2013; nine patients who underwent cardiac catheterisation at a median age of 39 days (range 12-60 days) and weight of 3.6 kg (range 2.6-4.3 kg) were identified. The median number of stents placed was one stent (range 1-4). The median oxygen saturation increased from 60% to 96%. The median right pulmonary artery size increased from 3.3 to 5.5 mm (-2.68 to -0.92 Z-score), and the median left pulmonary artery size increased from 3.4 to 5.5 mm (-1.93 to 0 Z-scores). Among all, one patient developed transient pulmonary haemorrhage, and one patient had pericardial tamponade requiring drainage. Complete repair of tetralogy of Fallot +/- atrioventricular septal defect or double-outlet right ventricle was achieved in all nine patients. Transcatheter stent alleviation of the right ventricular outflow tract obstruction resolves hypercyanotic spells and allows reasonable growth of the pulmonary arteries to facilitate successful surgical repair. This represents a viable alternative to placement of a systemic-to-pulmonary artery shunt, particularly in small neonates.

  3. Iloprost improves ventricular function in the hypertrophic and functionally impaired right heart by direct stimulation

    DEFF Research Database (Denmark)

    Holmboe, Sarah; Andersen, Asger; Vildbrad, Mads D;

    2013-01-01

    and dysfunctional right ventricle (RV). Rats were randomized to monocrotaline injection (60 mg/kg; [Formula: see text]), pulmonary trunk banding (PTB; [Formula: see text]), or a sham operation ([Formula: see text]). RV function was evaluated with magnetic resonance imaging, echocardiography, and invasive pressure...... that iloprost has inotropic properties directly improving ventricular function in the hypertrophic and dysfunctional right heart of the rat....

  4. Kidney Dysplasia

    Science.gov (United States)

    ... Disease Ectopic Kidney Medullary Sponge Kidney Kidney Dysplasia Kidney Dysplasia What is kidney dysplasia? Kidney dysplasia is a condition in which ... Kidney dysplasia in one kidney What are the kidneys and what do they do? The kidneys are ...

  5. Ventricular assist device

    Science.gov (United States)

    VAD; RVAD; LVAD; BVAD; Right ventricular assist device; Left ventricular assist device; Biventricular assist device; Heart pump; Left ventricular assist system; LVAS; Implantable ventricular assist device

  6. Late Chronic Tamponade after Intraoperative Right Ventricular Rupture Repair with Mediastinal Fat

    OpenAIRE

    Gualis, Javier; Castaño, Mario; Rodríguez, Miguel Angel; García, Cristina

    2015-01-01

    Advanced age and female sex are known risk factors for ventricular wall rupture during open-heart procedures. We present the case of an 83-year-old female patient with an intraoperative traumatic right ventricular free wall rupture during an aortic valve replacement procedure. Pledgetted interrupted sutures reinforced with large pieces of mediastinal fat were used for rupture repair. After 6 months, the patient was readmitted with the diagnosis of a retrosternal mediastinal mass and clinical ...

  7. The effect of septoplasty on pulmonary artery pressure and right ventricular function in nasal septum deviation.

    Science.gov (United States)

    Ozkececi, Gulay; Akci, Onder; Bucak, Abdulkadir; Ulu, Sahin; Yalım, Zafer; Aycicek, Abdullah; Onrat, Ersel; Avsar, Alaettin

    2016-11-01

    Nasal septum deviation (NSD) can cause obstruction of the upper airway, which may lead to increased pulmonary artery pressure (PAP) and right ventricle dysfunction. The aim of the present study was to evaluate the effect of septoplasty on right ventricular function and mean PAP of patients with marked NSD. 25 patients with marked NSD (mean age = 31.8 ± 12.3 years) and 27 healthy volunteers (mean age = 34.5 ± 10.8 years) were enrolled. Echocardiography was performed for all subjects and right ventricular function and mean PAP were evaluated before and 3 months after septoplasty. Tricuspid annular plane systolic excursion (TAPSE) and tricuspid annulus early diastolic myocardial velocity (E') were significantly lower in patients with NSD than control subjects, while right ventricle myocardial performance index (RVMPI) and mean PAP were significantly higher (respectively, p = 0.006, 0.037, 0.049, 0.046). When preoperative and postoperative findings were compared, the mean PAP decreased whereas TAPSE increased significantly (respectively, p = 0.007, 0.03). The results of the present study demonstrated that mean PAP increased and right ventricular function worsened in patients with NSD. However, mean PAP decreased and right ventricular function tended to recover after septoplasty.

  8. Cone Reconstruction of Atypical Ebstein Anomaly Associated with Right Ventricular Apical Hypoplasia.

    Science.gov (United States)

    Reddin, Gemma; Poterucha, Joseph T; Dearani, Joseph A; Warnes, Carole A; Cetta, Frank

    2016-02-01

    Cone reconstruction for tricuspid valve repair has revolutionized the surgical treatment of Ebstein anomaly. We present the case of a 58-year-old woman with atypical Ebstein anomaly and right ventricular apical hypoplasia who was spared from palliative shunt physiology by our use of cone reconstruction. Compared with other techniques, cone reconstruction of the tricuspid valve more closely replicates normal valvular anatomy and function. This surgical procedure can be applied to many anatomic variations of Ebstein anomaly, as in our patient's apparently unique instance of atypical Ebstein anomaly with right ventricular apical hypoplasia.

  9. ANP, BNP and D-dimer predict right ventricular dysfunction in patients with acute pulmonary embolism

    DEFF Research Database (Denmark)

    Mortensen, Jann; Jensen, Claus V; Von Der Recke, Peter;

    2010-01-01

    The aim of this study was to predict right ventricular dysfunction (RVD) using plasma concentration of D-dimer, pro-atrial natriuretic peptide (pro-ANP), brain natriuretic peptide (BNP), endothelin-1 (ET-1) and cardiac troponin I (TNI) in patients with pulmonary embolism (PE).......The aim of this study was to predict right ventricular dysfunction (RVD) using plasma concentration of D-dimer, pro-atrial natriuretic peptide (pro-ANP), brain natriuretic peptide (BNP), endothelin-1 (ET-1) and cardiac troponin I (TNI) in patients with pulmonary embolism (PE)....

  10. Wildervanck's syndrome with severe inner ear dysplasia and agenesis of the right internal carotid artery.

    Science.gov (United States)

    Hernando, Mónica; Urbasos, María; Amarillo, Viviana Elizabeth; Herrera, María Teresa; García-Peces, Victoria; Plaza, Guillermo

    2014-04-01

    We describe a case with Wildervanck syndrome (cervico-oculo-acoustic syndrome) comprising Klippel-Feil anomaly, retractio bulbi (Duane syndrome), and congenital sensorineural deafness. An 18-month male baby had a severe inner ear dysplasia, and MRI also showed a complex vascular carotid malformation associated.

  11. Safety of American Heart Association-recommended minimum exercise for desmosomal mutation carriers

    NARCIS (Netherlands)

    Sawant, Abhishek C; Te Riele, Anneline S J M; Tichnell, Crystal; Murray, Brittney; Bhonsale, Aditya; Tandri, Harikrishna; Judge, Daniel P; Calkins, Hugh; James, Cynthia A

    2016-01-01

    BACKGROUND: Endurance exercise is associated with adverse outcomes in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Exercise recommendations for family members remain undetermined. OBJECTIVE: The purposes of this study were to determine if (1) endurance exercise (

  12. First successful repair of an aortico-to-right ventricular tunnel (ARVT) in d-transposition of the great arteries with aortic valve atresia and ventricular septal defect.

    Science.gov (United States)

    Wagner, Robert; Vollroth, Marcel; Daehnert, Ingo; Kostelka, Martin

    2015-04-01

    The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

  13. [RIGHT VENTRICULAR DIASTOLIC FUNCTION AND PERIPHERAL HEMODYNAMICS IN PATIENTS WITH CHRONIC COR PULMONALE RECEIVING VARIOUS THERAPY REGIMENS].

    Science.gov (United States)

    2010-01-01

    Right ventricular diastolic function, pulmonary hemodynamics, and peripheral endothelial vasoregulatory function were studied in patients with chronic cor pulmonale during complex treatment over time. The study confirmed the vasodilatory effect of ozone therapy and amlodipine during standard therapy, which appeared as lower blood pressure and better right ventricular diastolic function.

  14. Combined left and right ventricular volume determination by radionuclide angiocardiography using double bolus and equilibrium technique

    DEFF Research Database (Denmark)

    Stokholm, K H; Stubgaard, M; Møgelvang, J;

    1990-01-01

    by indicator dilution. The radionuclide technique comprised four steps: (1) a first-pass study of right ventricle; (2) a bolus study of left ventricle; (3) an equilibrium study of left ventricle; (4) determination of the distribution volume of red blood cells. Absolute volumes of left ventricle were determined......Eighteen patients with ischaemic heart disease were studied. Left and right ventricular volumes including cardiac output (forward flow) were determined by radionuclide angiocardiography using a double bolus and equilibrium technique. As reference, cardiac output was simultaneously measured...... from steps 2 + 3 + 4. Absolute volumes of right ventricle were calculated from stroke volume and right ventricular ejection fraction (EF) which in turn was determined from step 1 by creating composite systolic and composite diastolic images. There was an acceptable agreement between stroke volume...

  15. Left ventricular to right atrial shunt resulting from infective endocarditis.

    Science.gov (United States)

    Ellis, C J; Gray, K E; Ainscow, D A

    1975-02-01

    A unique case is described in which infective endocarditis of the aortic valve led to intractable heart failure and necropsy examination showed that the patient had developed a communication between the left ventricle and right atrium.

  16. Right ventricular septal pacing: Safety and efficacy in a long term follow up

    Institute of Scientific and Technical Information of China (English)

    Eraldo; Occhetta; Gianluca; Quirino; Lara; Baduena; Rosaria; Nappo; Chiara; Cavallino; Emanuela; Facchini; Paolo; Pistelli; Andrea; Magnani; Miriam; Bortnik; Gabriella; Francalacci; Gabriele; Dell’Era; Laura; Plebani; Paolo; Marino

    2015-01-01

    AIM: To evaluate the safety and efficacy of the permanent high interventricular septal pacing in a long term follow up, as alternative to right ventricular apical pacing. METHODS: We retrospectively evaluated:(1) 244 patients(74 ± 8 years; 169 men, 75 women) implanted with a single(132 pts) or dual chamber(112 pts) pacemaker(PM) with ventricular screw-in lead placed at the right ventricular high septal parahisian site(SEPTAL pacing);(2) 22 patients with permanent pacemaker and low percentage of pacing(< 20%)(NO pacing);(3) 33 patients with high percentage(> 80%) right ventricular apical pacing(RVA). All patients had a narrow spontaneous QRS(101 ± 14 ms). We evaluated New York Heart Association(NYHA) class, quality of life(Qo L), 6 min walking test(6MWT) and left ventricular function(end-diastolic volume, LV-EDV; end-systolic volume, LVESV; ejection fraction, LV-EF) with 2D-echocardiography. RESULTS: Pacing parameters were stable duringfollow up(21 mo/patient). In SEPTAL pacing group we observed an improvement in NYHA class, Qo L score and 6MWT. While LV-EDV didn’t significantly increase(104 ± 40 m L vs 100 ± 37 m L; P = 0.35), LV-ESV slightly increased(55 ± 31 m L vs 49 ± 27 m L; P = 0.05) and LV-EF slightly decreased(49% ± 11% vs 53% ± 11%; P = 0.001) but never falling < 45%. In the RVA pacing control group we observed a worsening of NYHA class and an important reduction of LV-EF(from 56% ± 6% to 43% ± 9%, P < 0.0001).CONCLUSION: Right ventricular permanent high septal pacing is safe and effective in a long term follow up evaluation; it could be a good alternative to the conventional RVA pacing in order to avoid its deleterious effects.

  17. Aorto-right ventricular fistula: a complication of aortic valve replacement.

    Science.gov (United States)

    Najib, Mohammad Q; Ng, Daniel; Vinales, Karyne L; Chaliki, Hari P

    2012-01-01

    The occurrence of aorto-right ventricular (aorto-RV) fistula after prosthetic aortic valve replacement is rare. Transthoracic echocardiography (TTE) with color-flow Doppler, transesophageal echocardiography (TEE), or both may be required for diagnosis. A 42-year-old woman sought care for palpitations and dyspnea due to atrial flutter 2 weeks after prosthetic aortic valve replacement and graft replacement of the ascending aorta. TTE and TEE revealed left-to-right shunt due to aorto-RV fistula.

  18. Right ventricular visualization by Tl-201 myocardial scintigraphy in chronic obstructive pulmonary disease

    Energy Technology Data Exchange (ETDEWEB)

    Fujii, Tadashige; Tanaka, Masao; Takeda, Masashi; Matsuzawa, Yukinori; Handa, Kenjiro; Yoshimura, Kazuhiko (Shinshu Univ., Matsumoto, Nagano (Japan). Faculty of Medicine)

    1993-04-01

    Tl-201 myocardial scintigraphy was performed in 130 patients with chronic obstructive pulmonary disease (COPD) to evaluate right ventricular hypertrophy, and the clinical significance of this method was studied. Tl-201 uptake ratios of the right ventricle, which represents the ratio of total counts of the right ventricle to counts of the administered dose of Tl-201, was higher in COPD, especially in pulmonary emphysema and B type COPD by Burrows classification than in controls. The grade of visualization of the right ventricle by visual assessment (RVV) was marked (+++) in only a few cases and moderate (++) in many cases (more than 80%) in all diseases except bronchial asthma. The incidence of right ventricular hypertrophy by electrocardiogram, right-sided heart failure and marked dyspnea (Hugh-Jones 4[center dot]5) were very low in cases with RVV grade ++ and very high in cases with +++. The grade of RVV was related to the severity of pulmonary perfusion impairment, although in diffuse panbronchiolitis the RVV was relatively slight compared with the impairment of perfusion. May parameters of pulmonary function such as %VC, FEV1.0%, RV/TLC, V25, %DLCO, Raw, [Delta]N[sub 2] and PaO[sub 2] showed abnormal values in patients with RVV grade of (++) or (+++) in all diseases except bronchial asthma. In COPD, Tl-201 myocardial scintigraphy seems to be useful for assessment of right ventricular overloading, and for follow-up observation and differentiation between cor pulmonale and right ventricular hypertrophy secondary to cardiac diseases by observing Tl-201 uptake of the lung and left ventricle. (author).

  19. A cornerstone of heart failure treatment is not effective in experimental right ventricular failure

    NARCIS (Netherlands)

    Borgdorff, Marinus A.; Bartelds, Beatrijs; Dickinson, Michael G.; Steendijk, Paul; Berger, Rolf M. F.

    2013-01-01

    Background: Right ventricular (RV) failure due to increased pressure load causes significant morbidity and mortality in patients with congenital heart diseases and pulmonary arterial hypertension. It is unknown whether renin-angiotensin-aldosterone-system (RAAS) inhibition (the cornerstone of left v

  20. Right ventricular lipomatous mass and biventricular multifocal fat in a young woman: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Bo Rahm; Park, Jae Hyeong; Ahn, Kye Taek; Kim, Song Soo; Jeong, Jin Ok; Choi, Si Wan; Jin, Seon Ah; Lee, Jae Hwan [Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon (Korea, Republic of)

    2015-04-15

    Cardiac lipoma is a rare neoplasm of encapsulated mature adult adipose tissue. It is usually asymptomatic, but it may be related to hemodynamic obstruction depending on its location. We report a typical case of right ventricular lipomatous mass and multifocal fat infiltration of both ventricles, which were detected incidentally in a young woman.

  1. Changes in Fetal Left and Right Ventricular Strain Mechanics during Normal Pregnancy.

    NARCIS (Netherlands)

    Kapusta, L.; Mainzer, G.; Weiner, Z.; Deutsch, L.; Khoury, A.; Haddad, S.; Lorber, A.

    2013-01-01

    BACKGROUND: The aim of this study was to detect normal changes in fetal two-dimensional speckle-tracking echocardiography-derived values for global and regional longitudinal left and right ventricular strain, strain rate, and time to peak (T2P) global strain during pregnancy. METHODS: Forty-four hea

  2. Right ventricular dysfunction in patients with end-stage renal disease on regular hemodialysis

    Directory of Open Access Journals (Sweden)

    Mohamed Momtaz

    2013-01-01

    Conclusion Subclinical RV dysfunction - as estimated by RV function indices; tricuspid plane systolic excursion, right ventricle fractional area change, and LTDISº - is increased among HD patients. A high prevalence of pulmonary hypertension was found among HD patients and this was not associated significantly with RV or left ventricular dysfunction in these patients.

  3. Extra-anatomic right ventricle to pulmonary artery conduit: the apical-aortic equivalent for complex right ventricular outflow tract reconstruction.

    Science.gov (United States)

    Mori, Makoto; Siddharthan, Ragavan; Kogon, Brian

    2015-03-01

    We describe the case of a 22-year-old with tetralogy of Fallot, who underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery conduit placement. She has undergone numerous subsequent conduit changes, each complicated by early stenosis and failure. Unfortunately, serial conduit changes can become increasingly challenging. While extra-anatomic conduits have been described for complex left ventricular outflow tract obstruction, they have not been described for right-sided obstruction. Herein, we present a patient who underwent successful placement of an extra-anatomic valved right ventricle to pulmonary artery conduit in the setting of complex right ventricular outflow tract obstruction.

  4. Wnt5a attenuates hypoxia-induced pulmonary arteriolar remodeling and right ventricular hypertrophy in mice.

    Science.gov (United States)

    Jin, Yuling; Wang, Wang; Chai, Sanbao; Liu, Jie; Yang, Ting; Wang, Jun

    2015-12-01

    Hypoxic pulmonary hypertension (HPH), which is characterized by pulmonary arteriolar remodeling and right ventricular hypertrophy, is still a life-threatening disease with the current treatment strategies. The underlying molecular mechanisms of HPH remain unclear. Our previously published study showed that Wnt5a, one of the ligands in the Wnt family, was critically involved in the inhibition of hypoxia-induced pulmonary arterial smooth muscle cell proliferation by downregulation of β-catenin/cyclin D1 in vitro. In this study, we investigated the possible functions and mechanisms of Wnt5a in HPH in vivo. Recombinant mouse Wnt5a (rmWnt5a) or phosphate buffered saline (PBS) was administered to male C57/BL6 mice weekly from the first day to the end of the two or four weeks after exposed to hypoxia (10% O2). Hypoxia-induced pulmonary hypertension was associated with a marked increase in β-catenin/cyclin D1 expression in lungs. Right ventricular systolic pressure and right ventricular hypertrophy index were reduced in animals treated with rmWnt5a compared with PBS. Histology showed less pulmonary vascular remodeling and right ventricular hypertrophy in the group treated with rmWnt5a than with PBS. Treatment with rmWnt5a resulted in a concomitant reduction in β-catenin/cyclin D1 levels in lungs. These data demonstrate that Wnt5a exerts its beneficial effects on HPH by regulating pulmonary vascular remodeling and right ventricular hypertrophy in a manner that is associated with reduction in β-catenin/cyclin D1 signaling. A therapy targeting the β-catenin/cyclin D1 signaling pathway might be a potential strategy for HPH treatment.

  5. [Right ventricular involvement in hypertrophic cardiomyopathy. A case report and brief review of the literature].

    Science.gov (United States)

    Comella, Alessandro; Magnacca, Massimo; Gistri, Roberto; Lombardi, Massimo; Neglia, Danilo; Poddighe, Rosa; Pesola, Antonio

    2004-02-01

    A clinical case of non-obstructive hypertrophic cardiomyopathy with involvement of the right ventricle is reported. The patient was a 42-year-old male with symptoms suggesting an effort angina of recent onset. The diagnosis was established by echocardiography, which showed asymmetric hypertrophy of the interventricular septum (20 mm), hypertrophy of the right ventricular free wall, and severe hypertrophy of the septal papillary muscle of the tricuspid valve. The patient underwent a complete diagnostic evaluation, including exercise stress test, Holter monitoring, magnetic resonance, myocardial tomoscintigraphy and complete hemodynamic assessment. Medical treatment with atenolol 50 mg day was started; at 1-year follow-up the patient's clinical conditions are good, with decrease of anginal episodes. The literature review elicits the paucity of information about this condition, despite a frequent involvement of both ventricles in hypertrophic obstructive cardiomyopathy. The case reported shows two atypical aspects: a) the involvement of the right ventricle in non-obstructive hypertrophic cardiomyopathy is anecdotal; b) this pattern of hypertrophy (right ventricular free wall/septal papillary muscle) has never been previously reported. Right ventricular involvement in patients with hypertrophic cardiomyopathy must be carefully investigated, because it may be more frequent than conventionally deemed.

  6. Interventricular cardiac resynchronization with right ventricular apical pacing in a patient with Ebstein's defect.

    Science.gov (United States)

    Maffe, Stefano; Perucca, Antonello; Paffoni, Paola; Signorotti, Fabiana; Pardo, Nicolò Franchetti; Dellavesa, Pierfranco; Parravicini, Umberto; Zenone, Franco; Paino, Anna Maria; Bielli, Massimo; Carola, Federico; Zanetta, Marco

    2008-06-01

    We present the case of a patient with Ebstein's defect surgically corrected, and a complete right bundle branch block (RBBB) documented on echocardiogram. After an episode of near syncope due to a high-grade atrioventricular (AV) block, the patient was assisted with a bicameral DDDR pacemaker implanted with traditional right ventricular apical pacing. After the DDDR, and after stimulation with an AV delay of 180 ms, a narrow QRS complex was observed. Meanwhile, the typical left bundle branch block morphology of the right ventricular pacing and the native RBBB morphology were missing. The QRS complex narrowing persisted, even with physical activity and also with the heart rate progression. An echocardiographic study confirmed an improvement of the cardiac resynchronization parameters with this programmed stimulation.

  7. Late Chronic Tamponade after Intraoperative Right Ventricular Rupture Repair with Mediastinal Fat.

    Science.gov (United States)

    Gualis, Javier; Castaño, Mario; Rodríguez, Miguel Angel; García, Cristina

    2015-12-01

    Advanced age and female sex are known risk factors for ventricular wall rupture during open-heart procedures. We present the case of an 83-year-old female patient with an intraoperative traumatic right ventricular free wall rupture during an aortic valve replacement procedure. Pledgetted interrupted sutures reinforced with large pieces of mediastinal fat were used for rupture repair. After 6 months, the patient was readmitted with the diagnosis of a retrosternal mediastinal mass and clinical signs of cardiac tamponade that required reoperation.

  8. Evaluation of global and regional left ventricular systolic function in patients with frequent isolated premature ventricular complexes from the right ventricular outflow tract

    Institute of Scientific and Technical Information of China (English)

    YAO Jing; XU Jing; YONG Yong-hong; CAO Ke-jiang; CHEN Shao-liang; XU Di

    2012-01-01

    Background Frequent premature ventricular complexes from the right ventricular outflow tract (RVOT-PVCs) are associated with left ventricular dysfunction.This study adopted two-dimensional speckle tracking imaging to evaluate global and regional left ventricular myocardial function in patients with frequent RVOT-PVCs.Methods This study included 30 patients with frequent RVOT-PVCs and 30 healthy subjects.Aortic systolic velocity-time integral (AoVTI) and myocardium strain in circumferential (CS),radial (RS) and longitudinal (LS) directions were evaluated by conventional echocardiography and speckle tracking imaging.All values of patients with RVOT-PVCs were recorded during sinus (PVC-S) and PVC beats (PVC-V).Results Significant differences were demonstrated in global CS,RS and LS between the control subjects and the PVC-V (CS:(17.46±2.48)% vs.(11.52±3.28)%,RS:(48.26±10.20)% vs.(20.92±9.78)%,LS:(19.89±2.62)% vs.(11.79±3.66)%,P <0.01),and in segmental RS and LS of nearly all the left ventricular segments.Statistical differences in segmental CS between the PVC-V and the control subjects were only observed in anterior,anteroseptal and septal segments (only seen in anteroseptal and septal segments at apex).Furthermore,V/S AoVTI (AoVTI during the PVC beat divided by AoVTI during the sinus beat,then multiplied by 100%) correlated with coupling interval (r=0.67,P <0.001) and global strain (CS:r=0.48,P=0.007; RS:r=0.65,P <0.001; LS:r=0.65,P <0.001).Conclusions Frequent RVOT-PVCs can induce global and regional left ventricular systolic dysfunction.The reduction of hemodynamic parameters relates to the coupling interval and the global systolic function.

  9. Latest insights in therapeutic options for systemic right ventricular failure: a comparison with left ventricular failure

    NARCIS (Netherlands)

    M.M. Winter; B.J. Bouma; M. Groenink; T.C. Konings; J.G.P. Tijssen; D.J. van Veldhuisen; B.J.M. Mulder

    2009-01-01

    The number of adult patients with a systemic right ventricle (RV) is steadily increasing. Survival is relatively good in these patients, but deterioration of the systemic RV seems inevitable. Although therapeutic options for patients with LV failure are well established, their role in patients with

  10. Development of left ventricular longitudinal speckle tracking echocardiography in very low birth weight infants with and without bronchopulmonary dysplasia during the neonatal period.

    Directory of Open Access Journals (Sweden)

    Christoph Czernik

    Full Text Available OBJECTIVES: In preterm infants, postnatal myocardial adaptation may be complicated by bronchopulmonary dysplasia (BPD. We aimed to describe the development of left ventricular function by serial 2D, Doppler, and speckle tracking echocardiography (2D-STE in infants with and without BPD during the neonatal period and compare these to anthropometric and conventional hemodynamic parameters. STUDY DESIGN: Prospective echocardiography on day of life (DOL 1, 7, 14, and 28 in 119 preterm infants 10% were seen for the apical segment. While anthropometric parameters show rapid development during the first 4 weeks of life, the speckle tracking parameters did not differ statistically significantly during the neonatal period. Infants with and without BPD differed significantly (p<0.001 in the development of anthropometric parameters, conventional hemodynamic parameters except for heart rate, and 2D-STE parameters: global longitudinal systolic strain rate (GLSSR and longitudinal systolic strain for the mid left wall (LSSR. The largest differences were seen at DOL 1 and 7 in GLSSR (p<0.001 and in LSSR (p<0.01. CONCLUSIONS: Reproducible 2D-STE measurements are possible in preterm infants <1500 g. Cardiac deformation reveals early (DOL 1 and 7 ventricular changes (GLSSR and LSSR in very low birth weight infants who develop BPD.

  11. High Altitude Pulmonary Edema Without Appropriate Action Progresses to Right Ventricular Strain: A Case Study.

    Science.gov (United States)

    Mills, Logan; Harper, Chris; Rozwadowski, Sophie; Imray, Chris

    2016-09-01

    Mills, Logan, Chris Harper, Sophie Rozwadowski, and Chris Imray. High altitude pulmonary edema without appropriate action progresses to right ventricular strain: A case study. High Alt Med Biol. 17:228-232, 2016.-A 24-year-old male developed high altitude pulmonary edema (HAPE) after three ascents to 4061 m over 3 days, sleeping each night at 2735 m. He complained of exertional dyspnea, dry cough, chest pain, fever, nausea, vertigo, and a severe frontal headache. Inappropriate continuation of ascent despite symptoms led to functional impairment and forced a return to the valley, but dyspnea persisted in addition to new orthopnea. Hospital admission showed hypoxemia, resting tachycardia, and systemic hypertension. ECG revealed right ventricular strain and a chest X-ray revealed right lower zone infiltrates. This case demonstrates that HAPE can develop in previously unaffected individuals given certain precipitating factors, and that in the presence of HAPE, prolonged exposure to altitude with exercise (or exertion) does not confer acclimatization with protective adaptations and that rest and descent are the appropriate actions. The case additionally demonstrates well-characterized right ventricular involvement.

  12. Inflammatory Pseudotumor Originating from the Right Ventricular Outflow Tract

    Directory of Open Access Journals (Sweden)

    Mohita Singh

    2016-01-01

    Full Text Available Introduction. Inflammatory pseudotumor is an uncommon entity, and its cardiac origin is exceedingly rare. Case History. A previously healthy 27-year-old man was found to have a systolic murmur during preemployment screening evaluation. A transthoracic echocardiogram revealed a 4 × 2.5 cm mass originating from the right ventricle (RV outflow tract extending into the aortic root. A computed tomography guided biopsy confirmed an IgG4-related inflammatory pseudotumor. Patient was started on oral prednisone with subsequent reduction in mass size. Conclusion. Cardiac inflammatory pseudotumors are markedly rare tumors that should be considered in the differential of intracardiac tumors which otherwise includes cardiac fibromas, myxomas, and sarcomas.

  13. Right ventricular dysfunction in patients with Brugada-like electrocardiography: a two dimensional strain imaging study

    Directory of Open Access Journals (Sweden)

    Murata Kazuya

    2011-11-01

    Full Text Available Abstract Background Sodium channel blockers augment ST-segment elevation in the right precordial leads in patients undergoing Brugada-type electrocardiography (ECG. However, their effect on echocardiographic features is not known. We address this by assessing global and regional ventricular function using conventional Doppler and two- dimensional (2D speckle tracking techniques. Methods Thirty-one patients with Brugada-type ECG were studied. A pure sodium channel blocker, pilsicainide, was used to provoke an ECG response. The percentage longitudinal systolic myocardial strain at the base of both the right ventricular (RV free wall and the interventricular septum wall was measured using 2D speckle tracking. Left ventricular (LV and RV myocardial performance (TEI indices were also measured. Results The pilsicainide challenge provoked a positive ECG response in 13 patients (inducible group. In the inducible group, longitudinal strain was significantly reduced only at the RV (-27.3 ± 5.4% vs -22.1 ± 3.6%, P P P Conclusions Temporal and spatial analysis using the TEI index and 2D strain imaging revealed the deterioration of global ventricular function associated with conduction disturbance and RV regional function in patients with Brugada-type ECG and coved type ST elevation due to administration of a sodium channel blocker.

  14. Is cardiac resynchronization therapy for right ventricular failure in pulmonary arterial hypertension of benefit?

    Science.gov (United States)

    Rasmussen, Jason T; Thenappan, Thenappan; Benditt, David G; Weir, E Kenneth; Pritzker, Marc R

    2014-12-01

    Pulmonary arterial hypertension is a manifestation of a group of disorders leading to pulmonary vascular remodeling and increased pulmonary pressures. The right ventricular (RV) response to chronic pressure overload consists of myocardial remodeling, which is in many ways similar to that seen in left ventricular (LV) failure. Maladaptive myocardial remodeling often leads to intraventricular and interventricular dyssychrony, an observation that has led to cardiac resynchronization therapy (CRT) for LV failure. CRT has proven to be an effective treatment strategy in subsets of patients with LV failure resulting in improvement in LV function, heart failure symptoms, and survival. Current therapy for pulmonary arterial hypertension is based on decreasing pulmonary vascular resistance, and there is currently no effective therapy targeting the right ventricle or maladaptive ventricular remodeling in these patients. This review focuses on the RV response to chronic pressure overload, its effect on electromechanical coupling and synchrony, and how lessons learned from left ventricular cardiac resynchronization might be applied as therapy for RV dysfunction in the context of pulmonary arterial hypertension.

  15. The influence of right ventricular apical pacing on left atrial volume in patients with normal left ventricular function

    Directory of Open Access Journals (Sweden)

    AR Moaref1

    2008-03-01

    Full Text Available Background: Right ventricular apical (RVA pacing has been reported to induce several deleterious effects particularly in the presence of structural heart disease but can also involve patients with normal left ventricular (LV function. Left atrial (LA enlargement is one of these effects, but the majority of studies have measured LA dimension rather than volume.Objective: The present prospective study was designed to assess the effect of RVA pacing on LA volume in patients with normal LV function.Patients and Methods: The study comprised 41 consecutive patients with LV ejection fraction ≥ 45% and LV end diastolic dimension ≤ 56 mm who underwent single-or dual- chamber pacemaker implantation in RVA and followed for LA volume measurement and pacemaker analysis at least during the ensuing 4.2 months. Results: In all, 21 patients were excluded from the study due to five spontaneous wide QRS complex (≥120msec, one recent acute coronary syndrome,one significant valvular heart disease, three pacing frequency <90%, eight death or losing follow up in three cases. In remaining 20 patients, LA volume ragned from 21 to 54 mm3 with mean of 37.3±9.7 mm3 prior to pacemaker implantation that increased to 31 to 103 mm3 (54.3±17.0 during follow-up (P<0.001.Conclusion: RVA pacing might lead to an increase in LA volume even in patients with normal LV function.

  16. Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet.

    Science.gov (United States)

    Noly, Pierre-Emmanuel; Guihaire, Julien; Coblence, Matthieu; Dorfmüller, Peter; Fadel, Elie; Mercier, Olaf

    2015-11-04

    An original piglet model of Chronic Thromboembolic Pulmonary Hypertension (CTEPH) associated with chronic Right Ventricular (RV) dysfunction is described. Pulmonary Hypertension (PH) was induced in 3-week-old piglets by a progressive obstruction of the pulmonary vascular bed. A ligation of the left Pulmonary Artery (PA) was performed first through a mini-thoracotomy. Second, weekly embolizations of the right lower pulmonary lobe were done under fluoroscopic guidance with n-butyl-2-cyanoacrylate during 5 weeks. Mean Pulmonary Arterial Pressure (mPAP) measured by ritght heart catheterism, increased progressively, as well as Right Atrial pressure and Pulmonary Vascular Resistances (PVR) after 5 weeks compared to sham animals. Right Ventricular (RV) structural and functional remodeling were assessed by transthoracic echocardiography (RV diameters, RV wall thickness, RV systolic function). RV elastance and RV-pulmonary coupling were assessed by Pressure-Volume Loops (PVL) analysis with conductance method. Histologic study of the lung and the right ventricle were also performed. Molecular analyses on RV fresh tissues could be performed through repeated transcutaneous endomyocardial biopsies. Pulmonary microvascular disease in obstructed and unobstructed territories was studied from lung biopsies using molecular analyses and pathology. Furthermore, the reliability and the reproducibility was associated with a range of PH severity in animals. Most aspects of the human CTEPH disease were reproduced in this model, which allows new perspectives for the understanding of the underlying mechanisms (mitochondria, inflammation) and new therapeutic approaches (targeted, cellular or gene therapies) of the overloaded right ventricle but also pulmonary microvascular disease.

  17. Asymptomatic fistula from a giant aneurysmatic left anterior descending artery to the right ventricular outflow tract.

    Science.gov (United States)

    Mustelier, Juan Valiente; Rego, Julio Oscar Cabrera; Aquiles, Eddy W Olivares; Llerena, Luis Roberto

    2010-12-01

    Coronary artery fistulas are unusual congenital or acquired coronary artery abnormalities in which blood is shunted into a cardiac chamber, great vessel or other structure, bypassing the myocardial capillary network (Jung et al. in Cardiovasc Ultrasound 5:10, 2007). We present a young adult patient with an asymptomatic fistula from a giant aneurysmatic left anterior descending artery to the right ventricular outflow tract, first diagnosed by echocardiography examination and further confirmed by 128-slice computed tomography coronary angiography.

  18. OBSTRUCTION OF RIGHT VENTRICULAR OUTFLOW TRACT CAUSED BY INTRACAVITARY METASTATIC DISEASE OF PANCREATIC TUMOR

    Institute of Scientific and Technical Information of China (English)

    Meng-tao Li; Wen-ling Zhu

    2005-01-01

    @@ THE pericardium (90%) is the most common loca tion of cardiac involvement by secondary tumor,followed by myocardium and endocardium (10%).1 Cases of right ventricular outflow tract obstruction caused by intracavitary metastatic tumor growth were rarely reported, al though clinical presentation were unique. We herein reported such a case with literature review in order to improve the understanding of malignant cardiac metastasis.

  19. Right ventricular effects of intracoronary delivery of mesenchymal stem cells (MSC) in an animal model of pressure overload heart failure.

    Science.gov (United States)

    Molina, Ezequiel J; Palma, Jon; Gupta, Dipin; Gaughan, John P; Houser, Steven; Macha, Mahender

    2009-12-01

    In a rat model of left ventricular pressure overload hypertrophy with biventricular failure, we studied the effects of intracoronary delivery of mesenchymal stem cells (MCS) upon right ventricular hemodynamic performance, profiles of local inflammation and apoptosis, and determinants of extracellular matrix remodeling. Sprague-Dawley rats underwent aortic banding and were followed by echocardiography. After a decrease in left ventricular fractional shortening of 25% from the baseline (relative 50% reduction), animals were randomized to an intracoronary injection of MSC (n=28) or PBS (n=20). Right ventricular hemodynamic assessment and measurement of local inflammatory markers, proapoptotic factors, and determinants of extracellular matrix remodeling were performed on post-transplantation day 7, 14, 21 or 28. MSC injection improved right ventricular systolic function in the MSC group compared to the control group (mean+/-SD, max dP/dt 772+/-272 mm Hg/s vs. 392+/-132 at 28 days, PRight ventricular levels of IL-1, IL-6, TNF-alpha, bax, bak and p38 were significantly decreased in the MSC treated animals. Expression of MMP-3, MMP-6, MMP-9, TIMP-1 and TIMP-3 declined in the MSC group compared with controls after 28 days. In this model of left ventricular pressure overload hypertrophy and biventricular failure, intracoronary delivery of MSC was associated with an improvement in the right ventricular hemodynamic performance, profiles of local inflammation and apoptosis, and determinants of extracellular matrix remodeling.

  20. Advanced echocardiography and cardiac magnetic resonance in congenital heart disease : insights in right ventricular mechanics and clinical implications

    NARCIS (Netherlands)

    Hulst, Anna Elisabeth van der

    2011-01-01

    The thesis provides new insights into advanced echocardiographic and magnetic resonance imaging techniques for comprehensive mechanical assessment of the right ventricle in healthy children and in pediatric patients with right ventricular dysfunction. It is shown that the right ventricle does not co

  1. [Right ventricular perforation and cardiac tamponade caused by a central venous catheter].

    Science.gov (United States)

    Fukuda, H; Kasuda, H; Shimizu, R

    1993-02-01

    A 5 year old girl with ASD was scheduled for open heart surgery. A central venous catheter was placed via the right infraclavicular vein after induction of anesthesia. Thirty minutes after insertion of the catheter, a decrease in arterial pressure and pulse pressure, an increase in heart rate and central venous pressure were observed. Cardiac tamponade was revealed by rapid opening of the chest. Gushing blood out of a hole in the right ventricular free wall was confirmed by pericardiotomy. The hemodynamics were stabilized by blood transfusion and surgical closure of the hole on the ventricle. This perforation was thought to be caused by careless insertion of a relatively stiff central venous catheter.

  2. Successful extraction of right ventricular lead remnants using the FlexCath® steerable sheath.

    Science.gov (United States)

    Tanawuttiwat, Tanyanan; Cheng, Alan; Rickard, John; Chow, Grant V; Sciortino, Christopher M; Brinker, Jeffrey

    2016-01-01

    In patients undergoing laser lead extraction, incomplete or failed lead removal occur in over 3 % of leads. Because the current available tools have limitations in reaching the right ventricle (RV), the procedure becomes challenging when the lead breaks and its fragments remain lodged in the RV. We describe two cases in which the FlexCath® steerable sheath, normally used in cryoballoon catheter ablation for atrial fibrillation, was useful in directing a bioptome to right ventricular lead fragments and thus allowing for complete lead extraction.

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

    Science.gov (United States)

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

    2014-03-01

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

  4. Angiographic evidence of absent ductus arteriosus in severe right ventricular outflow obstruction.

    Science.gov (United States)

    Lacina, S J; Hamilton, W T; Thilenius, O G; Bharati, S; Lev, M; Arcilla, R A

    1983-01-01

    The angiocardiograms of 5 newborn infants with autopsy and/or surgically-proven congenital absence of the ductus arteriosus (ADA) and right ventricular outflow obstruction (Group A), and of 14 neonates with pulmonary atresia complex and patent ductus arteriosus (Group B) were reviewed. Aortic size was similar in both groups; however, the diameters of the right and left pulmonary arteries were much smaller in Group A than in Group B (right pulmonary artery: 2.6 vs 4.5 mm, P less than 0.005; left pulmonary artery: 2.5 vs 4.3 mm, P less than 0.005). Extensive bronchial collaterals were observed in Group A but not in Group B. Tricuspid aortic valve stenosis was present in 2 patients in Group A but in none in Group B. The diagnosis of ADA may be made in newborn infants with severe right ventricular outflow obstruction if the angiocardiograms reveal hypoplasia of the pulmonary arteries, extensive bronchial collaterals, and nonvisualization of the ductus arteriosus. Other suggestive features include aortic valve stenosis and/or right aortic arch with aberrant left subclavian artery.

  5. Concomitant Right Ventricular Outflow Tract Cryoablation during Pulmonary Valve Replacement in a Patient with Tetralogy of Fallot

    Science.gov (United States)

    Shin, Hong Ju; Song, Seunghwan; Shin, Yu Rim; Park, Han Ki; Park, Young Hwan

    2017-01-01

    A 38-year-old female patient with a history of tetralogy of Fallot repair at 10 years of age underwent pulmonary valve replacement with a mechanical prosthesis, tricuspid annuloplasty, and right ventricular outflow tract cryoablation due to pulmonary regurgitation, tricuspid regurgitation, and multiple premature ventricular contractions with sustained ventricular tachycardia. After surgery, she had an uneventful postoperative course with arrhythmia monitoring. She was discharged without incident, and a follow-up Holter examination showed a decrease in the number of ventricular ectopic beats from 702 to 41. PMID:28180102

  6. Usefulness of right ventricular fractional area change to predict death, heart failure, and stroke following myocardial infarction (from the VALIANT ECHO Study)

    DEFF Research Database (Denmark)

    Anavekar, Nagesh S; Skali, Hicham; Bourgoun, Mikhail;

    2008-01-01

    Severe right ventricular dysfunction independent of left ventricular ejection fraction increased the risk of heart failure (HF) and death after myocardial infarction (MI). The association between right ventricular function and other clinical outcomes after MI was less clear. Two-dimensional echoc...

  7. Predictors of right ventricular function as measured by tricuspid annular plane systolic excursion in heart failure

    Directory of Open Access Journals (Sweden)

    Køber Lars V

    2009-11-01

    Full Text Available Abstract Introduction Tricuspid Annular Plane Systolic Excursion (TAPSE has independent prognostic value in heart failure patients but may be influenced by left ventricular (LV ejection fraction. The present study assessed the association of TAPSE and clinical factors, global and regional LV function in 634 patients admitted for symptomatic heart failure. Methods & Results TAPSE were correlated with global and regional measures of longitudinal LV function, segmental wall motion scores and measures of diastolic LV function as measured from transthoracic echocardiography. LV ejection fraction, wall motion index scores, atrio-ventricular annular plane systolic excursion of the mitral annulus were significantly related to TAPSE. Septal and posterior mitral annular plane systolic excursion (β = 0.56, p 2 = 0.28, p interaction = NS. Conclusion TAPSE is reduced with left ventricular dysfunction in heart failure patients, in particular with reduced septal longitudinal motion. TAPSE is decreased in patients with heart failure of ischemic etiology. However, the absolute reduction in TAPSE is small and seems to be of minor importance in the clinical utilization of TAPSE whether applied as a measure of right ventricular systolic function or as a prognostic factor.

  8. Acute right ventricular myocardial injury and sudden cardiac arrest in a patient with persistent spontaneous coronary vasospasm

    Institute of Scientific and Technical Information of China (English)

    Hung Ming-Yow; Li Ju-Chi; Hao Wen-Rui; Wu Cheng-Hsueh; Hung Ming-Jui

    2011-01-01

    Coronary vasospasm is a rare diagnosis resulting in sudden arrhythmic cardiac arrest. We report a case of a healthy,non-smoking elderly woman resuscitated from arrhythmic cardiac arrest. She had persistent spontaneous coronaxy vasospasm, leading to right ventricular myocardial injury and failure, and shock. She responded quickly to intravenous normal saline bolus infusion, but had irreversible neurological sequelae. Additionally, she had atrial fibrillation preceding ischemic ventricular fibrillation, a rare finding in coronary vasospasm-related cardiac arrest. We suggest immediate coronary angiography of patients in sudden arrhythmic cardiac arrest with acute right ventricular failure for a prompt,accurate diagnosis and appropriate management of the coronary vasospasm.

  9. Determination of the Effects of Digoxin on the Right Ventricular Function in Patients Undergoing Pneumonectomy

    Directory of Open Access Journals (Sweden)

    Alireza Sharifian Attar

    2014-02-01

    Full Text Available Introduction: Pneumonectomy is the standard treatment of lung cancer, even though patients should undergo several evaluations before surgery; deterioration of cardiopulmonary function after pulmonary resection is inevitable. We have evaluated the effects of digoxin on the improvement of right ventricular function and prevention of probable complications after lung resection surgery. Materials and Methods: All patients who were candidate for pneumonectomy or extensive lobectomy in Ghaem hospital from 2010 to 2012 were enrolled into this study and were divided into two groups randomly. The first group (group D received digoxin during surgery and in the second group (group C normal saline was administered as placebo. Echocardiographic evaluation of the patients was accomplished the day before and the day after surgery. Results: Among 20 patients in each group, male to female ratio was almost 2:1 and mean age was 63.8 (ranged 46-83 years. The most common cause of pneumonectomy was lung cancer. Comparison of the preoperative demographic variables, blood biochemistry, pulmonary function tests, echocardiographic and blood gas indexes showed no statistically significant differences between two groups. ,But postoperative evaluations showed a significant improvement in left ventricular ejection fraction in group D. Right ventricular systolic and diastolic diameters and pulmonary artery pressure were decreased significantly  as well. Conclusion: According to our results, we suggest a single dose of digoxin during lung resection surgery to improve cardiac performance after pneumonectomy.

  10. Thallium-201 myocardial imaging in children with heart disease. Quantitative assessment of right ventricular pressure

    Energy Technology Data Exchange (ETDEWEB)

    Mitomori, Toshihiro; Ono, Yasuo; Kamiya, Tetsuro; Nishimura, Tsunehiko; Kozuka, Takahiro (National Cardiovascular Center, Suita, Osaka (Japan)); Kohata, Tohru; Tanimoto, Takeshi

    1985-02-01

    201-TlCl myocardial imaging studies were performed to evaluate systolic pressure of right ventricle on 107 patients including 89 patients with congenital cardiac disease, patients with primary pulmonary hypertension and 13 patients with history of MCLS with the age range of 2 months to 17 years. The congenital cardiac disease group included 30 patients with tetralogy of Fallot, 8 patients with pulmonary stenosis (included 1 patient with double chambered right ventricle), 20 patients with ventricular septal defect, 10 patients with patent ductus arteriosus, 9 patients with atrial septal defect and 12 patients with complete transposition of great arteries. None of the patients with history of MCLS had coronary involvement. 50 ..mu..Ci/kg of 201-TlCl was infused intravenously and after 15 minutes the images were obtained from 5 directions (anterior, LAO 30, LAO 45, LAO 60 and lateral). The angle was determined to demonstrate the intraventricular septum and ventricular free walls cleary separated. The image of end-diastolic phase was obtained with ECG synchronized gated method from that direction. The ROI (region of interest) was defined as a slice line by drawing two lines perpendicular to the septum and the counts of the left and right ventricular free wall (Cl and Cr) were analyzed to evaluate the pressure of right ventricle. The pressure of ventricles were obtained by cardiac catheterization performed at the same time with myocardial imaging. The ratio of Cl and Cr (Cr/Cl) had good correlation with the ratio of peak systolic pressure of left ventricle and right ventricle (regression equation Y=0.84X + 0.08, r=0.87). In each congenital heart disease group, very good correlation was also demonstrated (Y=1.46X - 0.30, r=0.96 in PS; Y=0.70X0.20, r=0.91 in VSD; Y=0.86X + 0.15, r=0.78 in ASD and Y=0.72X + 0.24, r=0.80 in PDA). Thus 201-TlCl myocardial imaging was useful method to evaluate right ventricular pressure noninvasively in congenital cardiac disease.

  11. Cardiac MRI assessment of right ventricular function: impact of right bundle branch block on the evaluation of cardiac performance parameters

    Energy Technology Data Exchange (ETDEWEB)

    Marterer, Robert; Tschauner, Sebastian; Sorantin, Erich [Medical University of Graz, Division of Pediatric Radiology, Department of Radiology, Graz (Austria); Zeng, Hongchun [First Affiliated Hospital of Xinjiang Medical University, Department of Ultrasonography, Urumqi (China); Koestenberger, Martin [Medical University of Graz, Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, Graz (Austria)

    2015-12-15

    Right ventricular (RV) function represents a prognostic marker in patients with corrected congenital heart disease. In up to 80 % of these patients, right branch bundle block (RBBB) exists and leads to asynchronous ventricular contraction. The aim of this retrospective study was to evaluate the change of RV performance parameters considering delayed RV end-systolic contraction. RV volumes of 33 patients were assessed twice: 1) not taking account of RBBB (group I), and 2) considering RBBB (group II). According to the RV ejection fraction (EF) for both groups, RV function was classified in different categories (>50 % = normal, 40-50 % = mildly-, 30-40 % = moderately-, <30 % = severely-reduced). The mean time difference between maximal systolic contraction of the septum and RV free wall was 90.7 ms ± 42.6. Consequently, RV end-systolic volume was significantly decreased in group II (p < 0.001). Accordingly, RV stroke volume and RV EF were significantly higher in group II (p < 0.001). There was also a significant change in the assessment of RV function (p < 0.02). RBBB induced delayed RV contraction can be detected at CMR. Ignoring the RV physiology in RBBB patients leads to a statistically significant underscoring of RV performance parameters. (orig.)

  12. Phosphorylation of PTEN increase in pathological right ventricular hypertrophy in rats with chronic hypoxia induced pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    Nie Xin; Shi Yiwei; Yu Wenyan; Xu Jianying; Hu Xiaoyun; Du Yongcheng

    2014-01-01

    Background Phosphatase and tensin homologue on chromosome ten (PTEN) acts as a convergent nodal signalling point for cardiomyocyte hypertrophy,growth and survival.However,the role of PTEN in cardiac conditions such as right ventricular hypertrophy caused by chronic hypoxic pulmonary,hypertension remains unclear.This study preliminarily discussed the role of PTEN in the cardiac response to increased pulmonary vascular resistance using the hypoxia-induced PH rats.Methods Male Sprague Dawley rats were exposed to 10% oxygen for 1,3,7,14 or 21 days to induce hypertension and right ventricular hypertrophy.Right ventricular systolic pressure was measured via catheterization.Hypertrophy index was calculated as the ratio of right ventricular mass to left ventricle plus septum mass.Tissue morphology and fibrosis were measured using hematoxylin,eosin and picrosirius red staining.The expression and phosphorylation levels of PTEN in ventricles were determined by real time PCR and Western blotting.Results Hypoxic exposure of rats resulted in pathological hypertrophy,interstitial fibrosis and remodelling of the right ventricle.The phosphorylation of PTEN increased significantly in the hypertrophic right ventricle compared to the normoxic control group.There were no changes in protein expression in either ventricle.Conclusion Hypoxia induced pulmonary hypertension developed pathological right ventricular hypertrophy and remodelling probablv related to an increased phosohorvlation of PTEN.

  13. Ventricular function following radiation damage of the right ventricle. [Dogs, gamma radiation

    Energy Technology Data Exchange (ETDEWEB)

    Stone, H.L.; Bishop, V.S.; Guyton, A.C.

    The right ventricles of four dogs were exposed to 20,000 R /sup 60/Co irradiation. The animals were carried through complete stages of: (a) control studies for several weeks, (b) irradiation, and (c) postirradiation studies until death from typical heart failure 23 to 33 days later. Several different types of cardiac function curves were measured at intervals during the control and postirradiation periods. No evident deterioration of the ventricles could be discerned from the function curves during the first 14 days following irradiation. However, during the ensuing 9 to 19 days the ventricles deteriorated rapidly, as evidenced by deteriorating ventricular function curves, terminating in death. Ventricular function, as estimated by the method used in the present study, declined to an average of 43% below that of normal ventricles prior to death of the animal.

  14. Epileptic seizure in a patient with an implantable cardioverter-defibrillator: Quo vadis right ventricular lead?

    Science.gov (United States)

    Wedekind, Horst; Rozhnev, Andrey; Kleine-Katthöfer, Peter; Kranig, Wolfgang

    2016-03-01

    The case of a 77-year-old man admitted for suspected epileptic seizure is reported. Patient history showed implantation of a single-chamber implantable cardioverter-defibrillator (ICD) after cardiac arrest in 2007 with replacement in 2012 due to battery depletion; the patient reported no previous syncope, unconsciousness or seizures. Interrogation records of the ICD showed five ventricular tachyarrhythmia episodes that corresponded to the "seizure". Further examination revealed incorrect position of the RV-lead. Diagnosis was a provoked epileptic seizure due to undersensing of ventricular tachycardia because of improper ICD lead implantation in the coronary sinus. Treatment consisted of implantation of a new device with an additional ICD lead into the right ventricle.

  15. Late Gadolinium Enhancement of the right ventricular myocardium: Is it really different from the left ?

    Directory of Open Access Journals (Sweden)

    Macgowan Christopher K

    2008-05-01

    Full Text Available Abstract It has been suggested that, in late gadolinium enhancement, the signal of right ventricular myocardium is nulled at a shorter inversion time than the left. While we initially made the same observation, we believe that the difference is not real, but results from artifacts. We present 7 cases as well as computer simulations to describe the nature of these artifacts and explain how they can create the impression of different inversion times for the right and left ventricle. At inversion times that are shorter than ideal for the myocardium a black rim can be seen at the border of the myocardium with blood on the inside and with fat on the outside. This is most likely a partial volume effect. The thin myocardium of the right ventricle is sandwiched between these black rims and, at a low spatial resolution, is no longer visible. In this case, the adjacent black rims may then be misinterpreted as myocardium. While black rims also occur on the left side, the myocardium is thicker and remains discernable as a separate layer. As a consequence, the optimal inversion time for the right ventricle only appears different from that for the left. In fact, in the presence of hypertrophy of the right ventricle or during systolic wall thickening we did not find a difference in inversion times between the left and right ventricle. We conclude that sufficient spatial resolution is important for adequate late gadolinium enhancement of the right ventricle.

  16. Acute effects of levosimendan in experimental models of right ventricular hypertrophy and failure

    DEFF Research Database (Denmark)

    Vildbrad, Mads D; Andersen, Asger; Holmboe, Sarah;

    2014-01-01

    Pulmonary arterial hypertension (PAH) is a fatal disease, and the ultimate cause of death is right ventricular (RV) failure. In this study, we investigated the acute hemodynamic effects of levosimendan in two rat models of RV hypertrophy and failure. Wistar rats were randomized to receive sham....... PTB and MCT injection caused hypertrophy, dilatation, and failure of the RV compared with sham surgery. Levosimendan increased RV end systolic pressure (sham surgery: 16.0% ± 3.8% [P = 0.0038]; MCT: 9.9% ± 3.1% [P = 0.018]; PTB: 24.5% ± 3.3% [P = 0.0001]; mean ± SEM) compared with placebo...

  17. Assessment of right ventricular ejection fraction during intravenous /sup 133/Xe pulmonary ventilation study

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, S.M.; Spencer, R.P.; Herrera, N.E.

    1985-01-01

    Intravenous /sup 133/Xe in saline was used to estimate right-ventricular ejection fraction (RVEF). In three healthy volunteers, the first pass RVEF with /sup 133/Xe was followed by RVEF estimation by intravenous /sup 99m/Tc-pertechnetate. There was agreement between the /sup 133/Xe and 99mTc determined values. Subjects rebreathed the liberated /sup 133/Xe gas, allowing an estimation of regional pulmonary ventilation. Intravenous /sup 133/Xe in saline may have potential use in measuring RVEF, as well as in evaluating pulmonary function in cor pulmonale.

  18. Quantitative evaluation of right ventricular overload in cor pulmonale using sup 201 Tl myocardial SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Kato, Hiroshi; Misawa, Toshihiro; Kutsumi, Yasunori (Fukui Medical School, Matsuoka (Japan)) (and others)

    1991-01-01

    To determine quantitatively the discriminant and characteristics of cor pulmonale, {sup 201}Tl myocardial perfusion SPECT was performed in 16 patients with chronic obstructive pulmonary disease (COPD) and 7 with restrictive pulmonary disease (RPD). One section of the short-axis SPECT image in which the right ventricle was most clearly visualized was selected. Tl-score was defined as the ratio of the sum of counts in the region of interest (ROI) at the anterior, mid, and posterior regions of the right ventricular free wall to the sum of counts in ROI at the posterior, lateral, and anterior walls of the left ventricle, and the anterior and posterior regions of the interventricular septum. In the group of COPD patients, Tl-score was positively correlated with mean pulmonary arterial pressure (mPAP), total pulmonary vascular resistance (TPR), and arterial carbon dioxide tension (PaCO{sub 2}), while it was inversely correlated with arterial oxygen tension (PaO{sub 2}). However, there was no significant correlation between Tl-score and mPAP, TPR, PaCO{sub 2}, and PaO{sub 2} in the group of RPD patients. In assessing pulmonary hypertension as defined by mPAP over 20 mmHg, a Tl-score greater than 0.25 was useful with a sensitivity of 69% and a specificity of 90%. The occurrence of cor pulmonale is a major factor in determining the prognosis of COPD patients. It was concluded that {sup 201}Tl myocardial SPECT is useful for evaluating right ventricular overload quantitatively, as well as for assessing core pulmonale, especially in COPD patients, since the ratio of Tl counts in the right and left ventricles was significantly correlated with right cardiopulmonary hemodynamic parameters. (N.K.).

  19. One case of tachycardia-induced cardiomyopathy caused by right ventricular outflow tract-ventricular tachycardia%右室流出道室性心动过速诱发心动过速性心肌病1例

    Institute of Scientific and Technical Information of China (English)

    王广强; 杨洋; 张博; 孙健

    2013-01-01

    A 36-year-old woman has suffered from paroxysmal palpitations, chest tightness and shortness of breath for more than 10 years. ECG showed broad QRS, ventricular tachycardia and atrioventricular separation. The ECG pointed to an origin in the right ventricular outflow tract. Echocardiography showed that the left ventricular end-diastolic diameter was 53 mm and the ejection fraction was 40%. B type natriuretic peptide was 7 920 pg/ ml. A single radiofrequency application at the site of ectopy was immediately effective. Holter monitoring was normal after ablation. Final diagnosis was right ventricular outflow tract-ventricular tachycardia, tachycardia-induced cardiomyopathy.

  20. 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.

  1. Recovery of right and left ventricular function after acute pulmonary embolism

    Energy Technology Data Exchange (ETDEWEB)

    Klok, F.A., E-mail: f.a.klok@lumc.nl [Section of Vascular Medicine, Department of General Internal Medicine-Endocrinology, Leiden University Medical Center, Leiden (Netherlands); Romeih, S. [Department of Cardiology, Leiden University Medical Center, Leiden (Netherlands); Kroft, L.J.M.; Westenberg, J.J.M. [Department of Radiology, Leiden University Medical Center, Leiden (Netherlands); Huisman, M.V. [Section of Vascular Medicine, Department of General Internal Medicine-Endocrinology, Leiden University Medical Center, Leiden (Netherlands); Roos, A. de [Department of Radiology, Leiden University Medical Center, Leiden (Netherlands)

    2011-12-15

    Aim: To evaluate recovery of cardiac function after acute pulmonary embolism (PE). Materials and methods: Routine breath-held computed tomography (CT)-pulmonary angiography was performed in patients with suspected PE to confirm or exclude the diagnosis of PE at initial presentation. Electrocardiogram (ECG)-triggered cardiac CT was performed to assess biventricular function. After 6 months, cardiac magnetic resonance imaging (MRI) was performed. In total, 15 consecutive patients with PE and 10 without were studied. A significant change in ventricular volume was defined as a >15% change in end-diastolic or -systolic volumes (EDV, ESV), and significant ventricular function improvement as a >5% increase in ejection fraction (EF) as based on reported cut-off values. Results: Right and left ventricular (RV and LV) EDV and ESV changed non-significantly (<1.3%) in the patients without PE, indicating good comparability of those values measured by CT and MRI. PE patients with baseline normal RV function (RVEF {>=}47%) revealed a >5% improvement in the RVEF (+5.4 {+-} 3.1%) due to a decrease in the RVESV. Patients with baseline abnormal RV function showed a >5% improvement in the RVEF (+14 {+-} 15%) due to decreases in both the RVESV and RVEDV. Furthermore, the LVEDV increased in this latter patient group. Conclusions: The present study demonstrated an improvement in RV function in the majority of patients with PE, independent of baseline RV function. The degree of RV and LV recovery was dependent on the severity of baseline RV dysfunction.

  2. Altered right ventricular contractile pattern after cardiac surgery: monitoring of septal function is essential.

    Science.gov (United States)

    Nguyen, Tin; Cao, Long; Movahed, Assad

    2014-10-01

    Assessment of right ventricular (RV) function is important in the management of various forms of cardiovascular disease. Accurately assessing RV volume and systolic function is a challenge in day-to-day clinical practice due to its complex geometry. Tricuspid annular plane systolic excursion (TAPSE) and systolic excursion velocity (S') have been reviewed to further assess their suitability and objectivity in evaluating RV function. Multiple studies have validated their diagnostic and prognostic values in numerous pathologic conditions. Diminished longitudinal contraction after cardiothoracic surgery is a well-known phenomenon, but it is not well validated. Despite significant reduction in RV performance along the long-axis assessed by TAPSE and S' after cardiac surgery, RV ejection fractions did not change as well as the left ventricular parameters and exercise capacity. RV contractile patterns were markedly altered with decreased longitudinal shortening and increased transverse shortening, which are likely resulted from the septal damage during cardiac surgery. The septum is essential for RV performance due to its oblique fiber orientation. This allows ventricular twisting, which is a vital mechanism against increased pulmonary vascular resistance. The septum function along with TAPSE and S' should be adequately assessed during cardiac surgery, and evidence of septal dysfunction should lead to reevaluation of myocardial protection methods.

  3. Comparison of Venous Return Characteristics with Right Ventricular Mechanics During Cephalic Fluid Shift

    Science.gov (United States)

    Elliott, Morgan; Martin, David

    2015-01-01

    For my summer internship project, I organized a pilot study to analyze the effects of a cephalic fluid shift on venous return and right ventricular mechanics to increase right ventricular and venous knowledge. To accomplish this pilot study, I wrote a testing protocol, obtained Institutional Review Board (IRB) approval, completed subject payment forms, lead testing sessions, and analyzed the data. This experiment used -20deg head down tilt (20 HDT) as the ground based simulation for the fluid shift that occurs during spaceflight and compared it to data obtained from the seated and supine positions. Using echocardiography, data was collected for the right ventricle, hepatic vein, internal jugular vein, external jugular vein, and inferior vena cava. Additionally, non-invasive venous pressure measurements, similar to those soon to be done in-orbit, were collected. It was determined that the venous return from below the heard is increased during 20 HDT, which was supported by increased hepatic vein velocities, increased right ventricular inflow, and increased right ventricular strain at 20 HDT relative to seated values. Jugular veins in the neck undergo an increase in pressure and area, but no significant increase in flow, relative to seated values when a subject is tilted 20 HDT. Contrary to the initial expectations based on this jugular flow, there was no significant increase in central venous pressure, as evidenced by no change in Doppler indices for right arterial pressure or inferior vena cava diameter. It is suspected that these differences in pressure are due to the hydrostatic pressure indifference point shifting during tilt; there is a potential for a similar phenomenon with microgravity. This data will hopefully lead to a more in-depth understanding of the response of the body to microgravity and how those relate to the previously mentioned cardiovascular risk of fluid shift that is associated with spaceflight. These results were presented in greater detail

  4. Premature ventricular complex-induced chronic cough and cough syncope.

    Science.gov (United States)

    Stec, S; Dabrowska, M; Zaborska, B; Bielicki, P; Maskey-Warzechowska, M; Tarnowski, W; Chazan, R; Kulakowski, P

    2007-08-01

    The present case study reports a case of chronic cough and cough syncope associated with frequent premature ventricular complexes (PVCs). Careful analysis of cough-related symptoms and ECG monitoring led to the suspicion of PVC-induced cough. A coincidence between PVCs and episodes of cough was also documented by a portable multichannel recorder. Moreover, Doppler echocardiography revealed a PVC-induced transient increase in the pulmonary artery blood flow. After exclusion of other possible aetiologies, complete relief of chronic cough and cough syncope was achieved by radiofrequency ablation of the arrhythmogenic focus located in the right ventricular outflow tract. Premature ventricular complexes should be considered as a cause of chronic cough and cough syncope and an interdisciplinary cooperation can lead to successful diagnosis and treatment of this condition.

  5. Carney complex with right ventricular myxoma following second excision of left atrial myxoma.

    Science.gov (United States)

    Tamura, Yamato; Seki, Toshio

    2014-01-01

    We report a case of Carney complex with massive right ventricular myxoma after two-time excision of a left atrial myxoma. The patient was a 45-year-old woman with pyrexia. She temporarily lost consciousness during examination, and echocardiography and computed tomography (CT) showed a massive tumor in the right ventricle. Loss of consciousness was determined to be caused by intracardiac obstruction of blood flow due to the tumor, and corrective surgery was performed. Pathological findings indicated myxoma with no malignancy. Myxomas are benign, but there is frequent recurrence of tumors associated with Carney complex. Because her myxomas were accompanied by unusual skin pigmentation, she was diagnosed with Carney complex. Carney complex has a high rate of myxoma recurrence, and often runs in families. In all cases, it is necessary to observe the patient's course closely.

  6. Sporadic multicentric right atrial and right ventricular myxoma presenting as acute pulmonary thromboembolism

    Directory of Open Access Journals (Sweden)

    Satyajit Singh

    2016-01-01

    Full Text Available Multicentric cardiac myxoma is a rare syndrome; usually it is familial. We report a rare case of sporadic right atrium (RA and right ventricle (RV myxoma in a 26-year-old female presenting to our hospital for the evaluation of sudden onset of dyspnea and left precordial pain attributed to the embolization of degenerating tumor fragments to the pulmonary artery (PA. The exact incidence of sporadic multicentric RA and RV myxoma presenting as acute pulmonary embolism is unknown as multicentric RA and RV myxoma are very rare. Myxomas presenting as pulmonary embolism is <10%. Majority of cardiac myxomas present as exertional dyspnea, chest pain, positional syncope, fever, weight loss and other constitutional symptoms. Any young patient presenting with acute onset dyspnea with multiple cardiac masses may have tumor embolization to the PA diagnosis with transthoracic echocardiography and high-resolution computed tomography of thorax, fast-tracks patient transfer for urgent cardiac surgery to prevent further embolization.

  7. Assessment of Right Ventricular Free Wall Longitudinal Myocardial Deformation Using Speckle Tracking Imaging in Normal Subjects

    Institute of Scientific and Technical Information of China (English)

    Chun TONG; Chunlei LI; Jialin SONG; Hongyun LIU; Youbin DENG

    2008-01-01

    To assess right ventricular free wall longitudinal myocardium deformation and examine the changes with normal age by speckle tracking imaging (STI), myocardial systolic peak strain (ε), systolic peak strain rate (SRs), early diastolic peak strain rate (Sre), late diastolic peak strain rate (Sra), the ratio of Sre/Sra were measured in the basal, middle and apical segments of right ventricular free wall in 75 healthy volunteers (age range: 21-71 y) by STI from the apical 4-chamber view. RV longitudinal strain and strain rate were highest in the basal segment of the free wall. Older subjects had lower early diastolic strain rate (Sre) than younger subjects, but they had higher late diastolic strain rate (Sra). A negative correlation between age and the ratio of Sre/Sra was found in all RV free wall segments (r=-0.466 - -0.614, P<0.01). It is concluded that RV diastolic strain rate changes with age and STI can be used for the study of RV myocardial deformation.

  8. Pre-chemotherapy values for left and right ventricular volumes and ejection fraction by gated tomographic radionuclide angiography using a cadmium-zinc-telluride detector gamma camera

    DEFF Research Database (Denmark)

    Haarmark, Christian; Haase, Christine; Jensen, Maria Maj

    2016-01-01

    BACKGROUND: Estimation of left ventricular ejection fraction (LVEF) using equilibrium radionuclide angiography is an established method for assessment of left ventricular function. The purpose of this study was to establish normative data on left and right ventricular volumes and ejection fractio...

  9. Beneficial aspects of real time flow measurements for the management of acute right ventricular heart failure following continuous flow ventricular assist device implantation

    Directory of Open Access Journals (Sweden)

    Spiliopoulos Sotirios

    2012-11-01

    Full Text Available Abstract Background Optimal management of acute right heart failure following the implantation of a left ventricular assist device requires a reliable estimation of left ventricular preload and contractility. This is possible by real-time pump blood flow measurements. Clinical case We performed implantation of a continuous flow left ventricular assist device in a 66 years old female patient with an end-stage heart failure on the grounds of a dilated cardiomyopathy. Real-time pump blood flow was directly measured by an ultrasonic flow probe placed around the outflow graft. Diagnosis The progressive decline of real time flow and the loss of pulsatility were associated with an increase of central venous pressure, inotropic therapy and progressive renal failure suggesting the presence of an acute right heart failure. Diagnosis was validated by echocardiography and thermodilution measurements. Treatment Temporary mechanical circulatory support of the right ventricle was successfully performed. Real time flow measurement proved to be a useful tool for the diagnosis and ultimately for the management of right heart failure including the weaning from extracorporeal membrane oxygenation.

  10. Pulmonary endarterectomy normalizes interventricular dyssynchrony and right ventricular systolic wall stress

    Directory of Open Access Journals (Sweden)

    Mauritz Gert-Jan

    2012-01-01

    Full Text Available Abstract Background Interventricular mechanical dyssynchrony is a characteristic of pulmonary hypertension. We studied the role of right ventricular (RV wall stress in the recovery of interventricular dyssynchrony, after pulmonary endarterectomy (PEA in chronic thromboembolic pulmonary hypertension (CTEPH. Methods In 13 consecutive patients with CTEPH, before and 6 months after pulmonary endarterectomy, cardiovascular magnetic resonance myocardial tagging was applied. For the left ventricular (LV and RV free walls, the time to peak (Tpeak of circumferential shortening (strain was calculated. Pulmonary Artery Pressure (PAP was measured by right heart catheterization within 48 hours of PEA. Then the RV free wall systolic wall stress was calculated by the Laplace law. Results After PEA, the left to right free wall delay (L-R delay in Tpeak strain decreased from 97 ± 49 ms to -4 ± 51 ms (P P = 0.18. The RV wall stress decreased significantly from 15.2 ± 6.4 kPa to 5.7 ± 3.4 kPa (P P = 0.78. The reduction of L-R delay in Tpeak was more strongly associated with the reduction in RV wall stress (r = 0.69,P = 0.007 than with the reduction in systolic PAP (r = 0.53, P = 0.07. The reduction of L-R delay in Tpeak was not associated with estimates of the reduction in RV radius (r = 0.37,P = 0.21 or increase in RV systolic wall thickness (r = 0.19,P = 0.53. Conclusion After PEA for CTEPH, the RV and LV peak strains are resynchronized. The reduction in systolic RV wall stress plays a key role in this resynchronization.

  11. Congenital heart defects in oculodentodigital dysplasia: Report of two cases.

    Science.gov (United States)

    Izumi, Kosuke; Lippa, Andrew M; Wilkens, Alisha; Feret, Holly A; McDonald-McGinn, Donna M; Zackai, Elaine H

    2013-12-01

    Oculodentodigital dysplasia is caused by mutations in the GJA1 gene. Oculodentodigital dysplasia presents with a spectrum of clinical features including craniofacial, ocular, dental, and limb anomalies. Although recent findings implicate the major role of GJA1 during cardiac organogenesis, congenital heart defects are infrequently reported in oculodentodigital dysplasia. Here we report on two patients with GJA1 mutations presenting with cardiac malformations and type III syndactyly. Patient 1 presented with pulmonary atresia, an intact septum, right ventricular hypoplasia and tricuspid stenosis. The infant had a small nose, thin columella and bilateral 4-5 syndactyly of the fingers. A de novo c.226C>T (p.Arg76Cys) mutation was identified. Patient 2 presented at 6 months with a ventricular septal defect. The child had hypoplastic alae nasi with a thin columella and bilateral 4-5 syndactyly of the digits. A de novo missense mutation, c.145C>G (p.Gln49Glu) was found. Our two patients underscore the importance of cardiac evaluations as part of the initial workup for patients with findings of oculodentodigital dysplasia. Conversely, those patients with type III syndactyly and congenital heart defect should be screened for GJA1 mutations.

  12. Contemporary management of acute right ventricular failure: a statement from the Heart Failure Association and the Working Group on Pulmonary Circulation and Right Ventricular Function of the European Society of Cardiology.

    Science.gov (United States)

    Harjola, Veli-Pekka; Mebazaa, Alexandre; Čelutkienė, Jelena; Bettex, Dominique; Bueno, Hector; Chioncel, Ovidiu; Crespo-Leiro, Maria G; Falk, Volkmar; Filippatos, Gerasimos; Gibbs, Simon; Leite-Moreira, Adelino; Lassus, Johan; Masip, Josep; Mueller, Christian; Mullens, Wilfried; Naeije, Robert; Nordegraaf, Anton Vonk; Parissis, John; Riley, Jillian P; Ristic, Arsen; Rosano, Giuseppe; Rudiger, Alain; Ruschitzka, Frank; Seferovic, Petar; Sztrymf, Benjamin; Vieillard-Baron, Antoine; Yilmaz, Mehmet Birhan; Konstantinides, Stavros

    2016-03-01

    Acute right ventricular (RV) failure is a complex clinical syndrome that results from many causes. Research efforts have disproportionately focused on the failing left ventricle, but recently the need has been recognized to achieve a more comprehensive understanding of RV anatomy, physiology, and pathophysiology, and of management approaches. Right ventricular mechanics and function are altered in the setting of either pressure overload or volume overload. Failure may also result from a primary reduction of myocardial contractility owing to ischaemia, cardiomyopathy, or arrhythmia. Dysfunction leads to impaired RV filling and increased right atrial pressures. As dysfunction progresses to overt RV failure, the RV chamber becomes more spherical and tricuspid regurgitation is aggravated, a cascade leading to increasing venous congestion. Ventricular interdependence results in impaired left ventricular filling, a decrease in left ventricular stroke volume, and ultimately low cardiac output and cardiogenic shock. Identification and treatment of the underlying cause of RV failure, such as acute pulmonary embolism, acute respiratory distress syndrome, acute decompensation of chronic pulmonary hypertension, RV infarction, or arrhythmia, is the primary management strategy. Judicious fluid management, use of inotropes and vasopressors, assist devices, and a strategy focusing on RV protection for mechanical ventilation if required all play a role in the clinical care of these patients. Future research should aim to address the remaining areas of uncertainty which result from the complexity of RV haemodynamics and lack of conclusive evidence regarding RV-specific treatment approaches.

  13. Effect of pericardiocentesis on right and left ventricular function and volumes in pericardial effusion.

    Science.gov (United States)

    Manyari, D E; Kostuk, W J; Purves, P

    1983-07-01

    To assess the effects of pericardial effusion on ventricular performance and volumes, electrocardiographically gated blood pool cardiac scintigraphy was performed immediately before and after 14 pericardiocenteses in 10 patients, 7 men and 3 women, aged 28 to 73 years (mean 50). Cardiac tamponade was present in 5 patients. After removal of 140 to 1,100 ml of pericardial fluid (527 +/- 305 ml [mean +/- standard deviation]), left ventricular (LV) ejection fraction increased from 63 +/- 5 to 64 +/- 4% (p greater than 0.05) and right ventricular (RV) ejection fraction decreased from 47 +/- 4 to 46 +/- 2% (p greater than 0.05). LV end-diastolic and end-systolic volumes increased (p less than 0.01) by 28 and 33%, and RV volumes by 40 and 43%, respectively. There were 8 patients with normal LV function (ejection fraction greater than 60%) and 6 patients with subnormal LV function. Changes in ejection fraction were nonsignificant in the 4 subgroups. LV end-diastolic volume changes were more marked (p less than 0.01) in patients with cardiac tamponade (+ 56%) than in those without tamponade (+ 17%), and in those with normal LV function (+ 36%) than in those with subnormal LV function (+ 21%). RV end-diastolic volume increased more markedly (p less than 0.05) in patients with tamponade (+ 72%) than in those without tamponade (+ 23%), but were similar in patients with normal (+ 38%) and abnormal (+ 43%) LV function. After pericardiocentesis, RV volume increased more markedly than did LV volume. Thus, hemodynamic and clinical improvement after pericardiocentesis may be related only to an increase in stroke volume. RV and LV ejection fraction, a measure of myocardial contractility, was not affected significantly by the presence of pericardial effusion, even in those patients who had cardiac tamponade.

  14. Mitogen-activated protein kinase signal pathways play an important role in right ventricular hypertrophy of tetralogy of Fallot

    Institute of Scientific and Technical Information of China (English)

    ZHANG Hong-shan; WU Qing-yu; XU Ming; ZHOU Yu-xiang; SHUI Chao-xiang

    2012-01-01

    Background Tetralogy of Fallot (TOF) is the most common malformation of children with an incidence of approximately 10% of congenital heart disease patients.There can be a wide spectrum to the severity of the anatomic defects,which include ventricular septal defect,aortic override,right ventricular outflow tract obstruction,and right ventricular hypertrophy.We examined the relationship between right ventricular hypertrophy in patients with TOF and the gene expression of factors in the mitogen-activated protein kinase (MAPK) signal pathway.Methods To gain insight into the characteristic gene(s) involved in molecular mechanisms of right ventricular hypertrophy in TOF,differential mRNA and micro RNA expression profiles were assessed using expression-based micro array technology on right ventricular biopsies from young TOF patients who underwent primary correction and on normal heart tissue.We then analyzed the gene expression of the MAPK signal pathway using reverse transcription-polymerase chain reaction (RT-PCR) in normals and TOF patients.Results Using the micro RNA chip V3.0 and human whole genome oligonucleotide microarray V1.0 to detect the gene expression,we found 1068 genes showing altered expression of at least two-fold in TOF patients compared to the normal hearts,and 47 micro RNAs that showed a significant difference of at least two-fold in TOF patients.We then analyzed these mRNAs and micro RNAs by target gene predicting software Microcosm Targets version 5.0,and determined those mRNA highly relevant to the right ventricular hypertrophy by RT-PCR method.There were obvious differences in the gene expression of factors in the MAPK signal pathway when using RT-PCR,which was consistent to the results of the cDNA microarray.Conclusion The upregulation of genes in the MAPK signal pathway may be the key events that contribute to right ventricular hypertroohv and stunted angiogenesis in patients with TOF.

  15. Deformation of the left and right ventricular longitudinal myocardium in fetuses with umbilical cord around neck

    Institute of Scientific and Technical Information of China (English)

    ZUO Dong-mei; WANG Chao-hong; WANG Yue-heng

    2012-01-01

    Background Umbilical cord around neck,a common obstetric complication,affects fetal hemodynamics.Does it influence fetal cardiac functions? The purpose of this study was to investigate the left and right ventricular systolic and diastolic functions of fetuses with umbilical cord around neck in the third trimester by applying velocity vector imaging (VVI).Methods Thirty-five cases of fetuses with umbilical cord around neck whose gestational ages from 35 to 40 weeks were selected,including 20 cases of umbilical artery ratio of the highest systolic velocity (S) to the lowest diastolic velocity (D) (S/D) <3.0 and 15 cases of umbilical artery S/D ≥3.0,while 20 cases of normal fetuses of 35-40 gestational weeks were selected as the control group.The changes in longitudinal velocity,strain,and strain rate of fetal left and right ventricle in systole and diastole in two groups,and the changes in fetal cardiac function under the situation of umbilical cord around neck were analyzed.Results Longitudinal strain and strain rate overall of fetal left and right ventricle in systole and diastole were less in fetuses with umbilical artery S/D ≥3.0 and umbilical cord around neck than those in fetuses with umbilical artery S/D <3.0and those in control group (P <0.05); there was no significant difference (P >0.05) in longitudinal strain and strain rate overall of fetal left and right ventricle in systole and diastole between fetuses with umbilical artery S/D <3.0 and those in control group.Conclusions Left and right ventricular systolic and diastolic dysfunction was detected in fetuses with umbilical cord around neck and umbilical artery S/D ≥3.0.WI could sensitively respond to cardiac function changes in fetuses with umbilical cord around neck,which provides another valuable method in the evaluation of fetal cardiac function.

  16. Reduced Right Ventricular Function Predicts Long-Term Cardiac Re-Hospitalization after Cardiac Surgery.

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    Leela K Lella

    Full Text Available The significance of right ventricular ejection fraction (RVEF, independent of left ventricular ejection fraction (LVEF, following isolated coronary artery bypass grafting (CABG and valve procedures remains unknown. The aim of this study is to examine the significance of abnormal RVEF by cardiac magnetic resonance (CMR, independent of LVEF in predicting outcomes of patients undergoing isolated CABG and valve surgery.From 2007 to 2009, 109 consecutive patients (mean age, 66 years; 38% female were referred for pre-operative CMR. Abnormal RVEF and LVEF were considered 30 days outcomes included, cardiac re-hospitalization, worsening congestive heart failure and mortality. Mean clinical follow up was 14 months.Forty-eight patients had reduced RVEF (mean 25% and 61 patients had normal RVEF (mean 50% (p<0.001. Fifty-four patients had reduced LVEF (mean 30% and 55 patients had normal LVEF (mean 59% (p<0.001. Patients with reduced RVEF had a higher incidence of long-term cardiac re-hospitalization vs. patients with normal RVEF (31% vs.13%, p<0.05. Abnormal RVEF was a predictor for long-term cardiac re-hospitalization (HR 3.01 [CI 1.5-7.9], p<0.03. Reduced LVEF did not influence long-term cardiac re-hospitalization.Abnormal RVEF is a stronger predictor for long-term cardiac re-hospitalization than abnormal LVEF in patients undergoing isolated CABG and valve procedures.

  17. Improved Right Ventricular Performance with Increased Tricuspid Annular Excursion in Athlete’s Heart

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    Himanshu eGupta

    2015-04-01

    Full Text Available Background: Marathon runners (MTH and patients with mitral regurgitation (MR exhibit left ventricular (LV overload, and LV geometric changes in these groups have been reported. In this study, right ventricular (RV adaptation to chronic volume overload was evaluated in MTH and MR and normal controls together with interventricular septal remodeling and tricuspid annulus (TA motion. Methods: A total of 60 age-matched subjects (including 19 MTH, 17 isolated chronic compensated MR patients and 24 normal subjects underwent conventional cine and tagged cardiac magnetic resonance imaging. Myocardial strain and curvature were computed on the interventricular septum and RV free wall. A dual-propagation technique was applied to construct RV volume-time curves for a single cardiac cycle. Similarly, the tricuspid annulus was tracked throughout the cardiac cycle to create displacement over time curve.Results: Septal curvature was significantly lower in MTH and MR compared to controls. No significant differences in RV free wall strain or RV ejection fraction were noted amongst the 3 groups. However, longitudinal TA excursion was significantly higher in MTH compared to controls (p=0.0061. The peak late diastolic TA velocity in MR was significantly faster than MTH (p=0.0031 and controls (p=0.020. Conclusions: Increased TA kinetics allows for improved RV performance in MTH. Septal remodeling was observed in both MR and MTH, therefore a direct relationship of septal remodeling to TA kinetics in athlete’s heart could not be elucidated in this study.

  18. Mitochondrial integrity in a neonatal bovine model of right ventricular dysfunction.

    Science.gov (United States)

    Bruns, Danielle R; Brown, R Dale; Stenmark, Kurt R; Buttrick, Peter M; Walker, Lori A

    2015-01-15

    Right ventricular (RV) function is a key determinant of survival in patients with both RV and left ventricular (LV) failure, yet the mechanisms of RV failure are poorly understood. Recent studies suggest cardiac metabolism is altered in RV failure in pulmonary hypertension (PH). Accordingly, we assessed mitochondrial content, dynamics, and function in hearts from neonatal calves exposed to hypobaric hypoxia (HH). This model develops severe PH with concomitant RV hypertrophy, dilation, and dysfunction. After 2 wk of HH, pieces of RV and LV were obtained along with samples from age-matched controls. Comparison with control assesses the effect of hypoxia, whereas comparison between the LV and RV in HH assesses the additional impact of RV overload. Mitochondrial DNA was unchanged in HH, as was mitochondrial content as assessed by electron microscopy. Immunoblotting for electron transport chain subunits revealed a small increase in mitochondrial content in HH in both ventricles. Mitochondrial dynamics were largely unchanged. Activity of individual respiratory chain complexes was reduced (complex I) or unchanged (complex V) in HH. Key enzymes in the glycolysis pathway were upregulated in both HH ventricles, alongside upregulation of hypoxia-inducible factor-1α protein. Importantly, none of the changes in expression or activity were different between ventricles, suggesting the changes are in response to HH and not RV overload. Upregulation of glycolytic modulators without chamber-specific mitochondrial dysfunction suggests that mitochondrial capacity and activity are maintained at the onset of PH, and the early RV dysfunction in this model results from mechanisms independent of the mitochondria.

  19. Fatal right ventricular failure and pulmonary hypertension after protamine administration during cardiac transplantation

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    Bibek S Pannu

    2016-01-01

    Full Text Available Protamine sulfate is the only Food and Drug administration approved medication for reversal of intraoperative heparin-induced anticoagulation during cardiac and vascular surgeries. One of the rare side effects of protamine sulfate is an idiosyncratic reaction resulting in acute pulmonary hypertension (APH and right ventricular (RV failure occurring after protamine administration. These reactions are rare but catastrophic with high mortality. A 36-year-old female with severe congestive heart failure was undergoing cardiac transplant surgery. After successful implantation of the donor heart, the patient was weaned off cardiopulmonary bypass. Protamine was then administered to reverse the heparin anticoagulation. She immediately developed APH and RV failure immediately after protamine infusion. The patient required immediate administration of inotropic agents, nitric oxide (NO, and subsequently required a number of mechanical support devices including an RV assist device (RVAD and ultimately full veno-arterial extracorporeal membrane oxygenation (VA-ECMO. Despite heroic efforts, the patient developed refractory multi-organ failure in the Intensive Care Unit and died after family requested discontinuation of resuscitative efforts. This case probably represents the first reported occurrence of fatal protamine-induced APH and ventricular failure in the setting of cardiac transplantation surgery. A number of interventions including inhaled NO, systemic vasopressors, RVAD, and ultimately VA-ECMO failed to reverse the situation, and the patient died of multi-organ failure.

  20. Acute Effects of Hemodialysis on Left and Right Ventricular Function: A Doppler Tissue Imaging Study

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    Tansel Erol

    2012-08-01

    Full Text Available Purpose: Doppler tissue imaging (DTI allows noninvasive assessment of both left ventricular (LV and right ventricular (RV function. The aim of this study was to evaluate the effect of hemodialysis (HD on LV and RV function using DTI. Method: Our study group included 30 patients on chronic HD program (mean age 45 15 years. Myocardial (Sm, Em, Am and annular velocities (Ea, Aa were measured in several cardiac territories before and after HD. Results: After HD, Ea significantly reduced from 10.8 3.4 cm/s to 9.6 2.4 cm/s (p = 0.029. Patients exhibited a lower Em following HD in all measured territories. Em/Am ratio was also reduced for each LV wall investigated after HD in all measured territories. At the RV segments, Sm, Em, and Am decreased significantly in all measured territories. Em of the anterior wall was positively related to ultrafiltration volume (r = 0.25, p = 0.006, whereas the decrease of Sm of RV basal segment correlated with a decrease of diastolic blood pressure (r = 0.23, p < 0.01. Conclusion: Our data indicate that a single HD session is associated with acute changes of systolic and diastolic parameters of LV and RV. [Cukurova Med J 2012; 37(4.000: 215-222

  1. Right ventricular relative wall thickness as a predictor of outcomes and of right ventricular reverse remodeling for patients with pulmonary hypertension.

    Science.gov (United States)

    Sano, Hiroyuki; Tanaka, Hidekazu; Motoji, Yoshiki; Fukuda, Yuko; Mochizuki, Yasuhide; Hatani, Yutaka; Matsuzoe, Hiroki; Hatazawa, Keiko; Shimoura, Hiroyuki; Ooka, Junichi; Ryo-Koriyama, Keiko; Nakayama, Kazuhiko; Matsumoto, Kensuke; Emoto, Noriaki; Hirata, Ken-Ichi

    2017-03-01

    Mid-term right ventricular (RV) reverse remodeling after treatment in patients with pulmonary hypertension (PH) is associated with long-term outcome as well as baseline RV remodeling. However, baseline factors influencing mid-term RV reverse remodeling after treatment and its prognostic capability remain unclear. We studied 54 PH patients. Mid-term RV remodeling was assessed in terms of the RV area, which was traced planimetrically at the end-systole (RVESA). RV reverse remodeling was defined as a relative decrease in the RVESA of at least 15% at 10.2 ± 9.4 months after treatment. Long-term follow-up was 5 years. Adverse events occurred in ten patients (19%) and mid-term RV reverse remodeling after treatment was observed in 37 (69%). Patients with mid-term RV reverse remodeling had more favorable long-term outcomes than those without (log-rank: p = 0.01). Multivariate logistic regression analysis showed that RV relative wall thickness (RV-RWT), as calculated as RV free-wall thickness/RV basal linear dimension at end-diastole, was an independent predictor of mid-term RV reverse remodeling (OR 1.334; 95% CI, 1.039-1.713; p = 0.03). Moreover, patients with RV-RWT ≥0.21 showed better long-term outcomes than did those without (log-rank p = 0.03), while those with RV-RWT ≥0.21 and mid-term RV reverse remodeling had the best long-term outcomes. Patients with RV-RWT remodeling, on the other hand, had worse long-term outcomes than other sub-groups. In conclusions, RV-RWT could predict mid-term RV reverse remodeling after treatment in PH patients, and was associated with long-term outcomes. Our finding may have clinical implications for better management of PH patients.

  2. Relation of epicardial fat thickness to right ventricular cavity size in obese subjects.

    Science.gov (United States)

    Iacobellis, Gianluca

    2009-12-01

    Epicardial fat is the visceral fat depot of the heart and is commonly increased in obese subjects. Obesity is also associated with right ventricular (RV) enlargement. Nevertheless, whether epicardial fat might be independently associated with RV morphology is unknown. I evaluated the correlation between echocardiographic epicardial fat and RV cavity size in subjects with a wide range of adiposity. Echocardiographic epicardial fat thickness and RV end-diastolic diameter (RVEDD) were assessed in 50 obese and 50 normal weight subjects. Patients with clinical conditions that could affect the RVEDD were excluded. Obese subjects had a significantly greater epicardial fat thickness and RVEDD than normal weight subjects (10 +/- 4 vs 5 +/- 2 mm and 22 +/- 10 vs 15 +/- 5, respectively; p subjects. Multiple regression analysis showed that epicardial fat thickness was the best independent correlate of RVEDD (r(2) = 0.42, p subjects. In conclusion, increased RV epicardial fat accumulation is associated with enlarged RV cavity size.

  3. Automated Assessment of Right Ventricular Volumes and Function Using Three-Dimensional Transesophageal Echocardiography.

    Science.gov (United States)

    Nillesen, Maartje M; van Dijk, Arie P J; Duijnhouwer, Anthonie L; Thijssen, Johan M; de Korte, Chris L

    2016-02-01

    Assessment of right ventricular (RV) function is known to be of diagnostic value in patients with RV dysfunction. Because of its complex anatomic shape, automated determination of the RV volume is difficult and strong reliance on geometric assumptions is not desired. A method for automated RV assessment was developed using three-dimensional (3-D) echocardiography without relying on a priori knowledge of the cardiac anatomy. A 3-D adaptive filtering technique that optimizes the discrimination between blood and myocardium was applied to facilitate endocardial border detection. Filtered image data were incorporated in a segmentation model to automatically detect the endocardial RV border. End-systolic and end-diastolic RV volumes, as well as ejection fraction, were computed from the automatically segmented endocardial surfaces and compared against reference volumes manually delineated by two expert cardiologists. The results reported good performance in terms of correlation and agreement with the results from the reference volumes.

  4. Evaluation of Right Ventricular Function by Tissue Doppler Echocardiography in Asthmatic Children

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    Mehdi Ghaderian

    2016-11-01

    Full Text Available BackgroundAsthma is the most chronic inflammatory disorder of the airways in children and asthmatic patients can experience cardiac dysfunction, pulmonary hypertension and finally cor pulmonale later in life. We aimed to investigate Right Ventricular (RV functions in asthmatic children by conventional and tissue Doppler echocardiography (TDE.Materials and Methods Pulmonary function tests, conventional and TDE examinations were performed on 42 asthmatic and 42 age- and gender matched healthy controls subjects (n=42.Results Compared with healthy children the RV wall was statistically thicker among asthmatic patients (P= 0.01. Conventional echocardiography had not significant difference between cases and controls, but TDE had significant difference between these two groups. Peak E’ velocity, A’ velocity, E’/A’ ratio and S’ in lateral and medial sites of tricuspid annulus valve, were significantly differ from control group in our patients (P

  5. Prenatal diagnosis of Pallister-Killian syndrome associated with pulmonary stenosis and right ventricular dilatation.

    Science.gov (United States)

    Park, In Yang; Shin, Jong Chul; Kwon, Ji Young; Koo, Bo Kyung; Kim, Myungshin; Lim, Jihyang; Kim, Yonggoo; Han, Kyungja

    2009-08-01

    Pallister-Killian syndrome (PKS) is a rare disorder characterized cytogenetically by tetrasomy 12p for isochromosome of the short arm of chromosome 12. PKS is diagnosed by prenatal genetic analysis through chorionic villous sampling, genetic amniocentesis, and cordocentesis, or by chromosomal analysis of skin fibroblasts, but is not usually detected by chromosomal analysis of peripheral blood cells. Herein, we report a case of a gravida at 23 weeks gestation with pulmonary stenosis and right ventricular dilation of the heart which were detected by sonography. Fluorescence in situ hybridization and a multicolor banding technique were performed to verify the diagnosis as 47,XX, +mar.ish i(12)(p10)(TEL++)[16]/46,XX[4], and an autopsy confirmed the cardiac anomalies detected on antenatal sonography.

  6. Right ventricular failure due to chronic pressure load : What have we learned in animal models since the NIH working group statement?

    NARCIS (Netherlands)

    Borgdorff, Marinus A J; Dickinson, Michael G; Berger, Rolf M F; Bartelds, Beatrijs

    2015-01-01

    Right ventricular (RV) failure determines outcome in patients with pulmonary hypertension, congenital heart diseases and in left ventricular failure. In 2006, the Working Group on Cellular and Molecular Mechanisms of Right Heart Failure of the NIH advocated the development of preclinical models to s

  7. Quantitative assessment of pulmonary regurgitation in patients with and without right ventricular tract obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Spiewak, Mateusz, E-mail: mspiewak@ikard.pl [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Biernacka, Elzbieta K., E-mail: kbiernacka@ikard.pl [Department of Congenital Heart Diseases, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Malek, Lukasz A., E-mail: lmalek@ikard.pl [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Department of Interventional Cardiology and Angiology, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Misko, Jolanta, E-mail: jmisko@wp.pl [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Department of Radiology, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Kowalski, Miroslaw, E-mail: mkowalski@ikard.pl [Department of Congenital Heart Diseases, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Milosz, Barbara, E-mail: barbara-milosz@o2.pl [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Department of Radiology, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Petryka, Joanna, E-mail: joannapetryka@hotmail.com [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Zabicka, Magdalena, E-mail: mzabicka@onet.eu [Cardiovascular Magnetic Resonance Unit, Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Dept. of Radiology, Inst. of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland); Ruzyllo, Witold, E-mail: wruzyllo@ikard.pl [Inst. of Cardiology, ul. Alpejska 42, 04-628 Warsaw (Poland)

    2011-11-15

    Background: There are concerns whether there is a difference in clinical utility of pulmonary regurgitation (PR) fraction (PRF) and PR volume (PRV) in subgroups of patients with isolated PR and individuals with combined PR and right ventricular outflow tract obstruction (RVOTO). The aim of the study was to compare PRF and PRV in patients with or without RVOTO. Methods and results: 82 consecutive patients after repair of tetralogy of Fallot (TOF) who underwent cardiovascular magnetic resonance and echocardiography were studied. There was no difference in PRF between patients with moderate and severe right ventricular (RV) dilatation (32 {+-} 13% vs. 37 {+-} 12%; p = 0.18). Significant difference in PRV was observed between these groups (23 {+-} 10 ml/m{sup 2} vs. 31 {+-} 12 ml/m{sup 2}, respectively; p = 0.02). PRV had better ability than PRF in identification of severe RV dilatation, both in group with RVOTO [area under the curve (AUC) 0.82 vs. 0.72, p = 0.005] and in patients without RVOTO (AUC 0.83 vs. 0.77, p = 0.04). A strong correlation was seen between PRF and PRV both in patients with and without RVOTO [r = 0.93, p < 0.0001 and r = 0.92, p < 0.0001, respectively]. In both subgroups high variability of PRF was found in subjects with similar degree of PRV. Conclusions: PRV shows better ability than PRF in evaluating influence of PR on RV in patients after TOF repair, both in population with and without concomitant RVOTO.

  8. The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy

    Science.gov (United States)

    Guo, Xiying; Fan, Chaomei; Tian, Lei; Zhang, Xiuling; Zhao, Xing; Wang, Fengqi; Zhu, Hongguang; Lin, Aiqing; Wu, Xia; Li, Yishi

    2017-01-01

    Introduction Severe right ventricular hypertrophy (SRVH) is a rare phenotype in hypertrophic cardiomyopathy (HCM) for which limited information is available. This study was undertaken to investigate the clinical, prognostic and genetic characteristics of HCM patients with SRVH. Methods HCM with SRVH was defined as HCM with a maximum right ventricular wall thickness ≥10 mm. Whole-genome sequencing (WGS) was performed in HCM patients with SRVH. Multivariate Cox proportional hazards regression models were used to identify risk factors for cardiac death and events in HCM with SRVH. Patients with apical hypertrophic cardiomyopathy (ApHCM) were selected as a comparison group. The clinical features and outcomes of 34 HCM patients with SRVH and 273 ApHCM patients were compared. Results Compared with the ApHCM group, the HCM with SRVH group included younger patients and a higher proportion of female patients and also displayed higher cardiovascular morbidity and mortality. The multivariate Cox proportional hazards regression models identified 2 independent predictors of cardiovascular death in HCM patients with SRVH, a New York Heart Association class ≥III (hazard ratio [HR] = 8.7, 95% confidence interval (CI): 1.43-52.87, p = 0.019) and an age at the time of HCM diagnosis ≤18 (HR = 5.5, 95% CI: 1.24-28.36, p = 0.026). Among the 11 HCM patients with SRVH who underwent WGS, 10 (90.9%) were identified as carriers of at least one specific sarcomere gene mutation. MYH7 and TTN mutations were the most common sarcomere mutations noted in this study. Two or more HCM-related gene mutations were observed in 9 (82%) patients, and mutations in either other cardiomyopathy-related genes or ion-channel disease-related genes were found in 8 (73%) patients. Conclusions HCM patients with SRVH were characterized by poor clinical outcomes and the presentation of multiple gene mutations. PMID:28323875

  9. Left and right ventricular diastolic dysfunction and diastolic heart failure: does one lead to the other?

    Institute of Scientific and Technical Information of China (English)

    Faramarz Tehrani; Anita Phan; Ernst R. Schwarz

    2009-01-01

    Background and Objective Diastolic dysfunction of the left ventricle is a mechanical abnormality diagnosed primarily by echocardiogram, and can be distinguished into three separate degrees based on the severity of reduction in passive compliance and active myocardial relaxation. Methods A literature search was performed for basic science studies, clinical studies and major practice guidelines on the subject of diastolic dysfunction and diastolic heart failure. Important findings were analyzed and correlated with regard to clinical relevance. Results Left ventricular diastolic dysfunction appears to compromise exercise tolerance and is believed to contribute to the pathophysiology in patients with diastolic heart failure. In the clinical setting, however, oftentimes no clear distinction is made between echocardiographically diagnosed diastolic dysfunction and diastolic heart failure, and adequate treatment recommendations are sparse and aimed to prevent worsening and progression of clinical symptoms. To date, there is a lack of high powered trials assessing the possible progression rate from echocardiographically diagnosed diastolic dysfunction to the clinical diagnosis of diastolic heart failure. Furthermore, there are no solid indices to assess the degree of severity of diastolic dysfunction or its progression. Pure right ventricular diastolic dysfunction appears to be even less understood and under-recognized, although it may play a role in the development of both right and left heart failure. Currently there are few but interesting data on the possible interaction between ventricles with diastolic dysfunction and the overall affect on the development of heart failure. Conclusions The timeline and progression of diastolic dysfunction to diastolic heart failure have not been well established and warrant further investigation.

  10. Outcome in Heart Failure with Preserved Ejection Fraction: The Role of Myocardial Structure and Right Ventricular Performance.

    Directory of Open Access Journals (Sweden)

    Georg Goliasch

    Full Text Available Heart failure with preserved ejection fraction (HFpEF is recognized as a major cause of cardiovascular morbidity and mortality. Thus, a profound understanding of the pathophysiologic changes in HFpEF is needed to identify risk factors and potential treatment targets in this specific patient population. Therefore, we aimed to comprehensively assess the impact of left- and right-ventricular function and hemodynamics on long-term mortality and morbidity in order to improve risk prediction in patients with HFpEF.We prospectively included 142 consecutive patients with HFpEF into our observational, non-interventional registry. Echocardiography, cardiac magnetic resonance imaging and invasive hemodynamic assessments including myocardial biopsy were performed at baseline. We detected significant correlations between left ventricular extracellular matrix and left ventricular end-diastolic diameter (r = -0.64;p = 0.03 and stroke volume (r = -0.53;p = 0.04. Hospitalization for heart failure and/or cardiac death was observed over a median follow up of 10 months. The strongest risk factors were reduced right ventricular function (adj. HR 6.62;95%CI 3.12- 14.02;p<0.001, systolic pulmonary arterial pressure (adj. HR per 1-SD 1.55;95%CI 1.15- 2.09;p = 0.004 and the pulmonary artery wedge pressure (adj. HR per 1-SD 1.51;95%CI 1.09-2.08; p = 0.012. The area under the ROC curve for right ventricular function was 0.63, for systolic pulmonary arterial pressure 0.75, and for pulmonary artery wedge pressure 0.68.The current study emphasizes the importance of right ventricular function and pulmonary pressures on outcome in patients with HFpEF providing pathophysiological insights into the hemodynamic changes in HFpEF.

  11. Prognostic value of right ventricular function in patients after acute myocardial infarction treated with primary percutaneous coronary intervention

    NARCIS (Netherlands)

    M.L. Antoni (Louisa); R.W.C. Scherptong (Roderick); J.Z. Atary (Jael); H. Boersma (Eric); E.R. Holman (Eduard); E.E. van der Wall (Ernst); M.J. Schalij (Martin Jan); J.J. Bax (Jeroen)

    2010-01-01

    textabstractBackground-Data on the association between right ventricular (RV) function and adverse events after acute myocardial infarction (AMI) are scarce. The purpose of the current study was to evaluate the relation between RV function and adverse events in patients treated with primary percutan

  12. Impacts of acute severe pulmonary regurgitation on right ventricular geometry and contractility assessed by tissue-Doppler echocardiography

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Iversen, Kasper K; Vejlstrup, Niels G;

    2010-01-01

    Little is known of the impact of acute right ventricular (RV) volume overload on RV function. We assessed the impact of acute severe pulmonary regurgitation (PR) on global and regional RV function by applying novel quantitative echocardiographic markers of myocardial performance in an animal model....

  13. Impacts of acute severe pulmonary regurgitation on right ventricular geometry and contractility assessed by tissue-Doppler echocardiography

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; K. Iversen, Kasper; G Vejlstrup, Niels;

    2010-01-01

    AIMS: Little is known of the impact of acute right ventricular (RV) volume overload on RV function. We assessed the impact of acute severe pulmonary regurgitation (PR) on global and regional RV function by applying novel quantitative echocardiographic markers of myocardial performance in an animal...

  14. Use of a circular mapping and ablation catheter for ablation of atypical right ventricular outflow tract arrhythmia.

    Science.gov (United States)

    Katritsis, Demosthenes G; Giazitzoglou, Eleftherios; Paxinos, George

    2010-02-01

    A new technique for ablation of persistent ectopic activity with atypical electrocardiographic characteristics at the vicinity of the right ventricular outflow tract is described. A new circular mapping and ablation catheter initially designed for pulmonary vein ablation was used. Abolition of ectopic activity was achieved with minimal fluoroscopy and ablation times.

  15. Anatomical Closure of Left-to-Right Shunts in Premature Infants with Bronchopulmonary Dysplasia and Pulmonary Hypertension: A Cautionary Tale

    Directory of Open Access Journals (Sweden)

    Narendra R. Dereddy

    2015-10-01

    Full Text Available Closure of a systemic to pulmonary shunt in premature infants with bronchopulmonary dysplasia may be beneficial, but in the presence of pulmonary hypertension is controversial. Here, we discuss two premature infants with pulmonary hypertension who developed acute pulmonary hypertensive crisis after closure of these shunts and hence advise caution.

  16. The importance of trabecular hypertrophy in right ventricular adaptation to chronic pressure overload.

    Science.gov (United States)

    van de Veerdonk, Mariëlle C; Dusoswa, Sophie A; Marcus, J Tim; Bogaard, Harm-Jan; Spruijt, Onno; Kind, Taco; Westerhof, Nico; Vonk-Noordegraaf, Anton

    2014-02-01

    To assess the contribution of right ventricular (RV) trabeculae and papillary muscles (TPM) to RV mass and volumes in controls and patients with pulmonary arterial hypertension (PAH). Furthermore, to evaluate whether TPM shows a similar response as the RV free wall (RVFW) to changes in pulmonary artery pressure (PAP) during follow-up. 50 patients underwent cardiac magnetic resonance (CMR) and right heart catheterization at baseline and after one-year follow-up. Furthermore 20 controls underwent CMR. RV masses were assessed with and without TPM. TPM constituted a larger proportion of total RV mass and RV end-diastolic volume (RVEDV) in PAH than in controls (Mass: 35 ± 7 vs. 25 ± 5 %; p TPM mass was related to the RVFW mass in patients (baseline: R = 0.65; p TPM from the assessment resulted in altered RV mass, volumes and function than when included (all p TPM mass (β = 0.44; p = 0.004) but not the changes in RVFW mass (p = 0.095) were independently related to changes in PAP during follow-up. RV TPM showed a larger contribution to total RV mass in PAH (~35 %) compared to controls (~25 %). Inclusion of TPM in the analyses significantly influenced the magnitude of the RV volumes and mass. Furthermore, TPM mass was stronger related to changes in PAP than RVFW mass. Our results implicate that TPM are important contributors to RV adaptation during pressure overload and cannot be neglected from the RV assessment.

  17. Ventricular fibrillation during right coronary arteriography with ioxaglate, iohexol and iopamidol in dogs.

    Science.gov (United States)

    Morris, T W

    1988-03-01

    Radiograph contrast media (CM) are known to produce myocardial disturbances during cardiac angiography. The most severe electrical disturbance is ventricular fibrillation (VF). Previous studies using prolonged right coronary exposures have demonstrated a higher incidence of VF with dilute low sodium CM than with dilute CM containing more physiologic levels of sodium. In this study the incidence of VF was examined for more conventional concentrations of iopamidol, iohexol and ioxaglate and for sodium supplemented iohexol. The incidence of VF was determined during 25-second injections of contrast media into the canine right coronary artery at a rate of 0.4 mL/sec. Injections of iohexol and iopamidol at concentrations of 160, 240 and 320 mgI/mL produced significantly more VF (P less than .005, Fisher Exact Test) than meglumine/sodium ioxaglate or iohexol supplemented with 20 mM sodium chloride. The time required to produce a 50% incidence of VF with iohexol and iopamidol was significantly related to sodium concentration (r = .92, P less than .01).

  18. Rapamycin attenuates hypoxia-induced pulmonary vascular remodeling and right ventricular hypertrophy in mice

    Directory of Open Access Journals (Sweden)

    Tillmanns Harald H

    2007-02-01

    Full Text Available Abstract Background Chronic hypoxia induces pulmonary arterial hypertension (PAH. Smooth muscle cell (SMC proliferation and hypertrophy are important contributors to the remodeling that occurs in chronic hypoxic pulmonary vasculature. We hypothesized that rapamycin (RAPA, a potent cell cycle inhibitor, prevents pulmonary hypertension in chronic hypoxic mice. Methods Mice were held either at normoxia (N; 21% O2 or at hypobaric hypoxia (H; 0.5 atm; ~10% O2. RAPA-treated animals (3 mg/kg*d, i.p. were compared to animals injected with vehicle alone. Proliferative activity within the pulmonary arteries was quantified by staining for Ki67 (positive nuclei/vessel and media area was quantified by computer-aided planimetry after immune-labeling for α-smooth muscle actin (pixel/vessel. The ratio of right ventricle to left ventricle plus septum (RV/[LV+S] was used to determine right ventricular hypertrophy. Results Proliferative activity increased by 34% at day 4 in mice held under H (median: 0.38 compared to N (median: 0.28, p = 0.028 which was completely blocked by RAPA (median HO+RAPA: 0.23, p = 0.003. H-induced proliferation had leveled off within 3 weeks. At this time point media area had, however, increased by 53% from 91 (N to 139 (H, p Conclusion Therapy with rapamycin may represent a new strategy for the treatment of pulmonary hypertension.

  19. Simultaneous ramp right heart catheterization and echocardiography in a ReliantHeart left ventricular assist device

    Science.gov (United States)

    Banerjee, Dipanjan; Dutt, Debleena; Duclos, Sebastien; Sallam, Karim; Wheeler, Matthew; Ha, Richard

    2017-01-01

    Many clinicians caring for patients with continuous flow left ventricular assist devices (CF-LVAD) use ramp right heart catheterization (RHC) studies to optimize pump speed and also to troubleshoot CF-LVAD malfunction. An investigational device, the ReliantHeart Heart Assist 5 (Houston, TX), provides the added benefit of an ultrasonic flow probe on the outflow graft that directly measures flow through the CF-LVAD. We performed a simultaneous ramp RHC and echocardiogram on a patient who received the above CF-LVAD to optimize pump parameters and investigate elevated flow through the CF-LVAD as measured by the flow probe. We found that the patient’s hemodynamics were optimized at their baseline pump speed, and that the measured cardiac output via the Fick principle was lower than that measured by the flow probe. Right heart catheterization may be useful to investigate discrepancies between flow measured by a CF-LVAD and a patient’s clinical presentation, particularly in investigational devices where little clinical experience exists. More data is needed to elucidate the correlation between the flow measured by an ultrasonic probe and cardiac output as measured by RHC. PMID:28163837

  20. Determination of right ventricular ejection fraction in children with cystic fibrosis, using krypton-81m

    Energy Technology Data Exchange (ETDEWEB)

    Piepsz, A.; Ham, H.R.; Millet, E.; Dab, I.

    1984-01-01

    The diagnosis of cor pulmonale and incipient heart failure remains difficult to assess in cystic fibrosis (CF) on the basis of the clinical as well as the biological parameters. The measurement of the right ventricular ejection fraction has been facilitated these last years by the introduction of the radionuclide methods. Methodological difficulties are however encountered when Tc-99m RBC are used, and are mainly related to heart chambers superposition (equilibrium method) or the low count density (first pass method). Few papers have been published on RVEF in cystic fibrosis and the results are somewhat contradictory. The authors have recently introduced a new method for the determination of RVEF, using equilibrium study during continuous injection of Kr-81m in glucose solution. This method offers several advantages related to an increased accuracy and a favorable dosimetry. In 25 patients aged 2 to 23 years with CF, one or more RVEF studies were performed. The severity of the disease was evaluated on the basis of the clinical Schwachman score, the lung function tests, the ventilation scan and the pa02. RVEF tended to decrease with the progression of the lung disease, although, owing to the spread of the results, no RVEF could be predicted on the basis of the other parameters. The decrease of RVEF in patients with advanced lung disease was moderate and terminal lung disease was sometimes associated with normal right heart contractility.

  1. Effects of diltiazem on transmembrane potential and current of right ventricular papillary muscle of ferrets.

    Science.gov (United States)

    Kanaya, S; Katzung, B G

    1984-01-01

    We studied the effects of diltiazem on electrical properties of isolated ferret right ventricular papillary muscles. By using standard microelectrode recording techniques and current clamp and voltage clamp protocols (single sucrose gap method), we measured action potential variables, depolarization-induced automaticity, slow (or second) inward current (Isi) and time-dependent and isochronal (1 sec) outward current. Resting potential was unaffected at all concentrations studied (from 2 nM-11 microM). At concentrations below 2 microM and at slow rates of stimulation (0.5 Hz), a small reduction of overshoot and prolongation of the action potential duration at 80% of full repolarization were observed. At concentrations of 2.2 microM or greater, marked use-dependent reductions of overshoot and plateau duration were observed that reversed with rest. Depolarization-induced automaticity was selectively suppressed at less negative diastolic potentials. In voltage clamp studies, peak Isi was markedly diminished over the concentration range studied (50% inhibitory concentration, 0.5 microM), but the current-voltage relation for Isi was not shifted on the voltage axis. The diminution in Isi was strongly use-dependent and voltage-dependent. Diltiazem (1.1 microM) had small effects on outward currents. Steady-state (isochronal) outward current and the time-dependent outward current were both reduced by 10 to 20% over the entire voltage range. Diltiazem is a potent inhibitor of the slow inward current in ventricular muscle. Its interaction with slow channel receptors appears to be strongly modulated by the state of the channels.

  2. Angiotensin-(1-7) treatment mitigates right ventricular fibrosis as a distinctive feature of diabetic cardiomyopathy.

    Science.gov (United States)

    Hao, Pan-Pan; Yang, Jian-Min; Zhang, Ming-Xiang; Zhang, Kai; Chen, Yu-Guo; Zhang, Cheng; Zhang, Yun

    2015-05-01

    In diabetic patients, left ventricular (LV) remodeling is highly prevalent; however, little is known about the impact of diabetes on right ventricular (RV) structure and function. We recently found that overexpression of angiotensin (ANG)-converting enzyme 2 (ACE2), which metabolizes ANG-II to ANG-(1-7) and ANG-I to ANG-(1-9), may improve LV remodeling in diabetic cardiomyopathy (DCM). Here, we aimed to assess whether LV remodeling and dysfunction are paralleled by RV alterations and the effects of ANG-(1-7) on RV remodeling in DCM. After 12 wk of diabetes induced by a single intraperitoneal injection of streptozotocin, rats were treated with saline, ANG-(1-7), perindopril, ANG-(1-7) plus perindopril, ANG-(1-7) plus Mas receptor antagonist A779, or ANG-(1-7) plus ANG-II type 2 receptor antagonist PD123319 for 4 wk. RV remodeling in diabetic rats was indicated by fibrosis of the RV free wall in the absence of hypertrophy and apoptosis. Treatment with ANG-(1-7) prevented diabetes-induced RV fibrosis and dysfunction. ANG-(1-7) (800 ng·kg(-1)·min(-1)) was superior to perindopril in improving RV fibrosis. The major mechanisms involved a complex interaction of ANG-II type 2 and Mas receptors for subsequent downregulation of ACE expression and activity and ANG-II type 1 receptor expression, as well as upregulation of ACE2 expression and activity and the expression of ANG-II type 2 receptor and sarco(endo)plasmic reticulum Ca(2+)-ATPase. Thus RV fibrosis and dysfunction plays a central role in DCM, and ANG-(1-7) mitigates diabetes-induced RV alterations.

  3. Evaluation of right ventricle by speckle tracking and conventional echocardiography in rats with right ventricular heart failure.

    Science.gov (United States)

    Kimura, Koichi; Daimon, Masao; Morita, Hiroyuki; Kawata, Takayuki; Nakao, Tomoko; Okano, Tomoko; Lee, Seitetsu L; Takenaka, Katsu; Nagai, Ryozo; Yatomi, Yutaka; Komuro, Issei

    2015-05-13

    Speckle tracking echocardiography (STE) has been reported to be a promising technique for evaluating right ventricular (RV) function in the clinical setting. On the other hand, the usefulness of STE for RV evaluation in small animal models has not been clarified, although the rat model is among the most commonly used animal models to develop novel effective treatments against pulmonary hypertension and RV heart failure (HF).We validated the use of STE and conventional echocardiographic variables for evaluating RV functions in a rat model by comparing the echocardiographic values of RVHF rats (n = 12) induced by monocrotaline injection with those of control rats (n = 12).Most conventional echocardiographic variables demonstrated that RVHF rats have significant RV dysfunction. The area under the curve (AUC) values to distinguish RV dysfunction in RVHF rats from normal RV function in control rats using fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), RV myocardial performance index (MPI), peak tissue Doppler tricuspid annular velocities at systole (Sa), and at early diastole (Ea) were 0.71, 0.98, 0.79, 0.92, and 0.91, respectively. However, using STE analysis for RV evaluation, limited reproducibility was observed (variability 19-37 %, ICC 0.74-0.88) and the only circumferential strain showed significantly lower absolute values (P = 0.039, AUC = 0.76).To evaluate RV function in rat models, circumferential strain may be useful, however, the reproducibility and diagnostic utility were limited. Conventional echocardiographic variables such as TAPSE, tissue Doppler Sa, and Ea have superior diagnostic utility.

  4. Contrast Circulation Time to Assess Right Ventricular Dysfunction in Pulmonary Embolism: A Retrospective Pilot Study

    Science.gov (United States)

    Platon, Alexandra; Poletti, Pierre-Alexandre; Perrier, Arnaud; Bendjelid, Karim

    2016-01-01

    Objective To optimize enhancement of pulmonary arteries and facilitate diagnosis of pulmonary embolism (PE), modern computed tomography angiography (CTA) contains a contrast bolus tracking system. We explored the diagnostic accuracy of the time-intensity curves given by this automated system to identify right ventricular dysfunction (RVD) in acute PE. Methods 114 CTAs with a diagnosis of PE were reviewed. RVD was defined as right-to-left ventricular diameter ratio of 1 or greater. Four parameters on time-intensity curves were identified. Parameters between CTAs with and those without RVD were compared with the Wilcoxon rank-sum test. The ability of the four parameters to discriminate patients with RVD was explored by compiling the area under the operating curves (AUC). Results The time needed by the contrast media to reach the pulmonary artery [8 seconds (IQR: 7–9) versus 7 seconds (IQR: 6–8), p<0.01], the time needed to reach 40 Hounsfield units (HU) [11 seconds (IQR: 8.5–14) versus 9.5 seconds (IQR: 8–10.5), p<0.01], and the contrast intensity reached after 10 seconds [19 HU (IQR: 4–67) versus 53 HU (IQR: 32–80), p<0.05] were all statistically different between CTA with and CTA without RVD. Those three parameters changed gradually across severity categories of RVD (p<0.05 for trend). Their AUC to identify RVD ranged from 0.63 to 0.66. The slope of contrast intensity over time was not informative: [31 HU/s (IQR: 20–57) in CTA with, compared to 36 HU/s (IQR: 22.5–53) in CTA without RVD, p = 0.60]. Conclusion Several parameters of the time-intensity curve obtained by the bolus tracking system are associated with RVD assessed on CTA images. Of those, the time needed to reach a predefined threshold seems to be the easiest to obtain in any CTA without additional processing time or contrast injection. However, the performance of those parameters is globally low. PMID:27551831

  5. Assessment of the right and left ventricular function in patients with cor pulmonale using high-definition CT

    Institute of Scientific and Technical Information of China (English)

    GAO Yan; QIN Jian; DU Xiang-ying; YANG Jing; LI Kun-cheng

    2013-01-01

    Background Cor pulmonale is often associated with changes of structure and function of the right ventricle (RV) and thus further affects functional changes of the left heart.Our study aimed to assess the left ventricular (LV) and RV function in patients with cor pulmonale using high-definition CT (HDCT).Methods We prospectively studied 18 cor pulmonale patients determined by the pulmonary function test,clinical examination,chest radiograph,electrocardiogram,and echocardiogram.The subject group was compared to a control group consisting of 18 subjects.The RV and LV functions and RV myocardial mass (MM) were obtained by HDCT in the two groups.The results were compared between the two groups using the independent sample t test.Echocardiographic examination for cardiac function analysis was performed on the same day.Results The RV end-diastolic volume (EDV),RV end-systolic volume (ESV) and RV myocardial mass were significantly larger in the 18 cor pulmonale patients than in the control group (P<0.05).The right ventricular ejection fraction (RVEF) was significantly lower in the 18 cor pulmonale patients than in controls (P<0.01).The left ventricular EDV (LVEDV) and LVEF were significantly lower in cor pulmonale patients than in controls (P<0.01).There were strong correlations between MDCT and echocardiography,rRVEF=0.839 and rLVEF=0.916,respectively.Conclusions HDCT can accurately quantify RV and LV function.The right ventricular function is impaired in patients with cor pulmonale,while at the same time the left ventricular function is also impaired.

  6. Current status of percutaneous right ventricular assist devices: First-in-man use of a novel dual lumen cannula.

    Science.gov (United States)

    Aggarwal, Vikas; Einhorn, Bryce N; Cohen, Howard A

    2016-09-01

    Ventricular assist devices have become an accepted therapeutic solution for patients with severe left ventricular dysfunction when pharmacology fails to maintain sufficient cardiac output. Despite various technologies that have allowed left ventricular assist devices to become more reliable and versatile in the past decade, comparatively little attention has been applied to right heart assistance which is still in the early stage of its development. The extracorporeal devices developed thus far have been associated with mobility issues and complications common to ventricular assist devices in general, such as infection, bleeding, and thromboembolism. Designed to obviate the problems previously experienced by other right ventricle (RV)-focused devices, the Protek Duo (CardiacAssist, Pittsburgh, PA) is a novel, fully percutaneous, dual lumen cannula for RV support used in conjunction with the paracorporeal TandemHeart(®) (CardiacAssist, Pittsburgh, PA) pump. We describe our initial experience with the Protek Duo cannula in two different clinical scenarios. In addition, we summarize the current percutaneous mechanical support technology for RV assistance and propose modification of current technology to facilitate its application. © 2016 Wiley Periodicals, Inc.

  7. 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.

  8. QUANTIFICATION OF RIGHT VENTRICULAR FUNCTION IN ATRIAL SEPTAL DEFECT USING ULTRASOUND-BASED STRAIN RATE IMAGING

    Institute of Scientific and Technical Information of China (English)

    MENG Xiang-chun; SUN Kun; ZHANG Yu-qi; HUANG Mei-rong; GAO Wei; ZHANG Zhi-fang; SHEN Rong; CHEN Shu-bao

    2005-01-01

    Objective To study the validation of ultrasound-based strain rate imaging in the quantitative assessment of right ventricular (RV) function in atrial septal defect (ASD). Methods Tissue Doppler images (TDI) of RV longitudinal and short axes were recorded from the apical 4-chamber view and the subcostal short-axis view in 18 normal controls, 28 children with ASD and 14 children after Amplazter closure of ASD respectively. Peak systolic velocities (V), peak systolic strain rates (SR), peak systolic strains (S) at the basal segment, middle segment of RV lateral wall and the basal septum from the longitudinal axis, the middle segment of RV free wall from the short axis were quantitatively measured using QLAB TM tissue velocity quantification software system respectively. Peak dp/dt from the RV isovolumic contraction determined during the right cardiac catheterization in 28 ASD patients was used as the gold standard of RV contractility. Peak systolic indices were compared against max dp/dt by linear correlation. Results Peak systolic indices at the basal and middle segments of RV lateral wall from the longitudinal axis increased significantly in 28 ASD patients.Peak systolic indices at the basal septum also increased in patient group, but not significantly. Significant decreases in peak systolic indices at the basal and middle segments of RV lateral wall were observed after the Amplatzer closure in 14 ASD patients. There was no significant difference at the middle segment of RV free wall from the short axis between patient group and normal control. A strong correlation was found between max dp/dt and peak systolic indices at the basal and middle segments of RV lateral wall (P<0.05). Conclusion Ultrasound-based strain rate imaging can assess quantitatively RV function in CHD. Peak systolic strains determined at the basal and middle segments of RV lateral wall are strong noninvasive indices of RV contractility.

  9. Progressive development of pulmonary hypertension leading to right ventricular hypertrophy assessed by echocardiography in rats.

    Science.gov (United States)

    Kato, Yosuke; Iwase, Mitsunori; Kanazawa, Hiroaki; Kawata, Natsuki; Yoshimori, Yukie; Hashimoto, Katsunori; Yokoi, Toyoharu; Noda, Akiko; Takagi, Kenzo; Koike, Yasuo; Nishizawa, Takao; Nishimura, Masahiko; Yokota, Mitsuhiro

    2003-07-01

    The present study aimed to evaluate the development of pulmonary hypertension by serial echocardiography, including measurements of pulmonary artery (PA) flow velocities, and correlate echocardiographic indices with pathological findings in rats administered monocrotaline (MCT). MCT (60 mg/kg body weight) or physiologic saline was administered to a total of 9 male Wistar rats at the age of 4 weeks (MCT group: n = 4, control group: n = 5, respectively). Echocardiography was performed serially until the age of 8 weeks. The ratio of right ventricular (RV) outflow tract dimensions to aortic dimensions increased progressively in the MCT group and became significantly greater than that of the control group after the age of 6 weeks. Peak PA velocity (Peak V) in the MCT group was significantly less than that of the control group at the ages of 7 and 8 weeks. The ratio of acceleration time to ejection time (AT/ET) in PA flow waveforms declined progressively and was significantly less than that of the control group after the age of 6 weeks. The ratio of RV weight to body weight (RVW/BW) in the MCT group was significantly greater than that of the control group. Both AT/ET ratio and Peak V were significantly inversely correlated with RVW/BW ratio. Furthermore, these echocardiographic findings were also significantly inversely correlated with the mean cross-sectional RV myocyte area. In conclusion, the progressive development of pulmonary hypertension leading to RV hypertrophy can be evaluated appropriately by echocardiography including PA flow Doppler indices in rats.

  10. Effect of blood donation-mediated volume reduction on regional right ventricular deformation in healthy subjects.

    Science.gov (United States)

    Açar, Göksel; Alizade, Elnur; Avci, Anıl; Cakir, Hakan; Efe, Suleyman Cagan; Kalkan, Mehmet Emin; Tabakci, Mehmet Mustafa; Toprak, Cuneyt; Tanboğa, Ibrahim Halil; Esen, Ali Metin

    2014-03-01

    Strain (S) and strain rate (SR) are known to be altered in diseases associated with right ventricular (RV) pressure/volume overload and RV myocardial dysfunction; however determinants of S/SR are incompletely understood. The aim of this study was to examine the effect of blood donation-mediated volume reduction on regional RV deformation in healthy young adults. Study population was composed of 61 consecutive healthy subjects who were volunteers for blood donation. All underwent standard echocardiography and two-dimensional S and SR imaging by speckle tracking before and after 450 mL blood donation. We found no change in RV lateral wall SR in all three segments. However, the S in the apical and mid segments of the RV lateral wall immediately decreased after blood donation [-26.2 ± 3.3 vs. -23.2 ± 3.3 % (p subjects caused a regional difference in RV longitudinal deformation with the lower mid and apical S that was related to parameters of volume load severity. However, RV systolic SR was found to be resistant to the effects of volume depletion.

  11. Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Walsh, Rowan; Salem, Yishay [Mount Sinai School of Medicine, Division of Pediatric Cardiology, New York, NY (United States); Shah, Amee; Lai, Wyman W. [Morgan Stanley Children' s Hospital of New York Presbyterian, New York, NY (United States); Nielsen, James C. [Mount Sinai School of Medicine, Division of Pediatric Cardiology, New York, NY (United States); Mount Sinai Children' s Heart Center, Box 1201, New York, NY (United States)

    2011-08-15

    The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical. This investigation examines the repeatability of CMRI for RV size and function. Forty consecutive people with congenital heart disease involving the RV were retrospectively identified. Contouring of RV volumes was performed by two expert CMRI clinicians. The coefficient of variability and repeatability coefficients were calculated. Repeatability coefficients were multiplied by the mean value for each group studied to define a threshold beyond which measurement error was unlikely to be responsible. The variability for indexed RV end-diastolic volume = 3.2% and 3.3% for intra- and interobserver comparisons, respectively. The repeatability coefficients were 13.2% and 14.9% for intra- and interobserver comparisons, which yielded threshold values of 15.1 ml/m{sup 2} and 20.2 ml/m{sup 2}, respectively. For RV ejection fraction (EF), the repeatability coefficients for intra- and interobserver comparisons were 5.0% and 6.0%, which resulted in threshold values of 2.6 EF% and 3.0 EF%. The threshold values generated can be used during serial assessment of RV size and function. (orig.)

  12. Assessment of right ventricular systolic function by echocardiography after surgical repair of congenital heart defects.

    Science.gov (United States)

    Khraiche, Diala; Ben Moussa, Nidhal

    2016-02-01

    Postoperative impairment of right ventricular (RV) systolic function can appear after surgical repair of complex congenital heart defects, such as tetralogy of Fallot; it is caused by chronic volume and/or pressure overload due to pulmonary regurgitation and/or stenosis. RV dysfunction is strongly associated with prognosis in these patients. Cardiac magnetic resonance imaging is the gold standard for quantification of RV volumes and ejection fraction in patients with congenital heart diseases; however, it is costly and is not widely available. Echocardiography is the imaging modality that is most available and most frequently used to assess RV systolic function. However, RV ejection fraction cannot be measured accurately by standard two-dimensional echocardiography because of its pyramidal shape. Surrogate parameters of RV systolic function are mostly used in routine practice. New techniques of two-dimensional strain and three-dimensional quantification of RV volumes and ejection fraction have been developed in recent years. The aim of this article is to show the pertinence of each variable of RV systolic function measured by echocardiography in patients with repaired congenital heart disease and residual chronic RV overload.

  13. Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure

    DEFF Research Database (Denmark)

    Gillespie, Matthew J; McElhinney, Doff B; Kreutzer, Jacqueline

    2015-01-01

    BACKGROUND: Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure. METHODS: Retrospective review of databa...... early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR....... of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials. RESULTS: Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure....... Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p

  14. Ectodermal dysplasia

    Science.gov (United States)

    ... the womb. Alternative Names Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome Images Skin layers References Grange DK. Ectodermal dysplasias. Rimoin D, Korf B, eds. In: Emery and Rimoin's Principles and Practice of Medical Genetics . 6th ed. Philadelphia, PA: Elsevier; 2013:chap ...

  15. Right ventricular electrical and mechanical synchronization by properly timed septal pacing in a patient with right bundle branch block and first degree AV block--a case report.

    Science.gov (United States)

    Siliste, Calin; Suran, Maria-Claudia-Berenice; Margulescu, Andrei-Dumitru; Vinereanu, Dragos

    2015-03-01

    We present a case of near-normalization of the QRS by septal pacing in a patient with dual-chamber pacemaker and underlying complete right bundle branch block and first degree atrioventricular block. The right ventricular mechanical synchronization suggested by the ECG was validated as such by strain echo. To the best of our knowledge, this is the first time it has been shown that the narrowing of the QRS corresponds to mechanical synchronization in a case of this seldom-recognized phenomenon.

  16. [Long-term results of Rygg's monocusp ventricular outflow patch for the reconstruction of right ventricular outflow tract in tetralogy of Fallot].

    Science.gov (United States)

    Morikawa, M; Abe, T; Takagi, N; Ito, T; Hachiro, Y; Sato, S; Komatsu, K; Kikuchi, S

    2001-07-01

    Thirty surviving patients after corrective surgery for tetralogy of Fallot with right ventricular outflow tract reconstruction (RVOTR) using monocusp ventricular outflow patch (MVOP) were reviewed retrospectively to determine the long-term results. The age at operation ranged from 2 to 55 years with a mean of 19 years, and follow-up extended to 18.2 years (cumulative: 345.4 patient-year). There were 4 late deaths (1.2% per patient-year), and the cumulative survival rate was 85.3% at 18 years after the corrective surgery. Eight patients (2.3% per patient-year) required intracardiac reoperations mostly resulted from problems after RVOTR with MVOP, such as recurrent stenosis of right ventricular outflow tract (3 cases) or pulmonary valvular incompetence (4 cases). In addition, one patient underwent balloon angioplasty for the recurrent stenosis located in the distal end of MVOP. Freedom from surgical or catheter reintervention for the MVOP-related complication was 60.6% at 18 years after the corrective surgery. MVOP caused compression of the pulmonary artery at the distal end of the anastomosis and reoperation in a younger patients quite early after the corrective surgery. Like other transannular patches, tissue failure and degeneration of MVOP were inevitable, and resulted in severe pulmonary valvular incompetence that required the valve replacement in 4 patients (1.3% per patient-year). Freedom from pulmonary valve replacement was 71.2% at 18 years after the corrective surgery. As long-term results, our experiences emphasize the need for an innovative transannular patch that possesses significantly better long-term durability.

  17. Evaluation of right ventricular volume and function by 2D and 3D echocardiography compared to MRI

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Petersen, Claus Leth; Kjaer, Andreas;

    2005-01-01

    AIMS: Radionuclide techniques, and recently MRI, have been used for clinical evaluation of right ventricular (RV) volumes function (RVEF) and volumes; but with the introduction of 3D echocardiography, new echocardiographic possibilities for RV evaluation independent of geometrical assumptions have...... emerged. This study compared classic and new echocardiographic and radionuclide estimates, including gated blood pool single-photon emission computed tomography (SPECT) of RV size and function to RV volumes, and ejection fraction (RVEF) measured by magnetic resonance imaging (MRI). METHODS AND RESULTS...

  18. Horizontal right axillary minithoracotomy: aesthetic and effective option for atrial and ventricular septal defect repair in infants and toddlers

    OpenAIRE

    Luciana da Fonseca da Silva; José Pedro da Silva; Turquetto,Aida L R; Sonia Meiken Franchi; Cascudo,Cybelle M; Rodrigo Moreira Castro; Walter José Gomes; Christian Schreiber

    2014-01-01

    Introduction:Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD) and atrial septal defect (ASD) repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as as...

  19. Clinical impact of left ventricular eccentricity index using cardiac MRI in assessment of right ventricular hemodynamics and myocardial fibrosis in congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Yamasaki, Yuzo; Kamitani, Takeshi; Yamanouchi, Torahiko; Honda, Hiroshi [Kyushu University, Departments of Clinical Radiology, Graduate School of Medical Sciences, Fukuoka (Japan); Nagao, Michinobu; Kawanami, Satoshi [Kyushu University, Molecular Imaging and Diagnosis, Graduate School of Medical Sciences, Fukuoka (Japan); Yamamura, Kenichiro [Kyushu University, Pediatrics, Graduate School of Medical Sciences, Fukuoka (Japan); Sakamoto, Ichiro [Kyushu University, Cardiovascular Medicine, Graduate School of Medical Sciences, Fukuoka (Japan); Yabuuchi, Hidetake [Kyushu University, Health SciencesGraduate School of Medical Sciences, Fukuoka (Japan)

    2016-10-15

    To investigate the utility of eccentricity index (EI) using cardiac cine MRI for the assessment of right ventricular (RV) hemodynamics in congenital heart disease (CHD). Fifty-five patients with CHD (32 women; mean age, 40.7 ± 20.9 years) underwent both cardiac MRI and right heart catheterization. EI was defined as the ratio of the distance between the anterior-posterior wall and the septal-lateral wall measured in the short-axis of mid-ventricular cine MRI. Correlations between EIs and RV hemodynamic parameters were analyzed. EIs were compared between patients with and without late gadolinium enhancement (LGE). A strong correlation between mean pulmonary artery pressure (PAP) and systolic EI (r = 0.81, p < 0.0001) and a moderate negative correlation between diastolic EI and RV ejection fraction (EF) (r = -0.62, p < 0.0001) were observed. Receiver operating characteristic analysis revealed optimal EI thresholds for detecting patients with mean PAP ≥40 mmHg with C-statistics of 0.90 and patients with RVEF <40 % with C-statistics of 0.78. Systolic EIs were significantly greater for patients with LGE (1.45 ± 0.05) than for those without LGE (1.15 ± 0.07; p < 0.001). EI offers a simple, comprehensive index that can predict pulmonary hypertension and RV dysfunction in CHD. (orig.)

  20. Different predictors of right and left ventricular metabolism in healthy middle-aged men

    Directory of Open Access Journals (Sweden)

    Marja eHeiskanen

    2015-12-01

    Full Text Available Dysfunction of the right ventricle (RV plays a crucial role in the outcome of various cardiovascular diseases. Previous studies on RV metabolism are sparse although evidence implies it may differ from left ventricular (LV metabolism. Therefore, the aims of this study were 1 to determine predictors of RV glucose uptake (GU and free fatty acid uptake (FFAU and 2 to compare them to predictors of LV metabolism in healthy middle-aged men. Altogether 28 healthy, sedentary, middle-aged (40-55 years men were studied. Insulin-stimulated GU and fasting FFAU were measured by positron emission tomography and RV and LV structural and functional parameters by cardiac magnetic resonance. Several parameters related to whole-body health were also measured. Predictors of RV and LV metabolism were determined by pairwise correlation analysis, lasso regression models, and variable clustering using heatmap. RVGU was most strongly predicted by age and moderately by RV ejection fraction (EF. The strongest determinants of RVFFAU were exercise capacity (peak oxygen uptake, resting heart rate, LVEF, and whole-body insulin-stimulated glucose uptake rate. When considering LV metabolism, age and RVEF were associated also with LVGU. In addition, LVGU was strongly, and negatively, influenced by whole-body insulin-stimulated glucose uptake rate. LVFFAU was predicted only by LVEF. This study shows that while RV and LV metabolism have shared characteristics, they also have unique properties. Age of the subject should be taken into account when measuring myocardial glucose utilization. Ejection fraction is related to myocardial metabolism, and even so that RVEF may be more closely related to GU of both ventricles and LVEF to FFAU of both ventricles, a finding supporting the ventricular interdependence. However, only RV fatty acid utilization associates with exercise capacity so that better physical fitness in a relatively sedentary population is related with decreased RV fat

  1. Right ventricular function quantification in Takotsubo cardiomyopathy using two-dimensional strain echocardiography.

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    Felix Heggemann

    Full Text Available AIMS: This study sought to characterize global and regional right ventricular (RV myocardial function in patients with Takotsubo cardiomyopathy (TC using 2D strain imaging. METHODS: We compared various parameters of RV and left ventricular (LV systolic function between 2 groups of consecutive patients with TC at initial presentation and upon follow-up. Group 1 had RV involvement and group 2 did not have RV involvement. RESULTS: At initial presentation, RV peak systolic longitudinal strain (RVPSS and RV fractional area change (RVFAC were significantly lower in group 1 (-13.2±8.6% vs. -21.8±5.4%, p = 0.001; 30.7±9.3% vs. 43.5±6.3%, p = 0.001 and improved significantly upon follow-up. Tricuspid annular plane systolic excursion (TAPSE did not differ significantly at initial presentation between both groups (14.8±4.1 mm vs. 17.9±3.5 mm, p = 0.050. Differences in regional systolic RV strain were only observed in the mid and apical segments. LV ejection fraction (LVEF and LV global strain were significantly lower in group 1 (36±8% vs. 46±10%, p = 0.006 and -5.5±4.8% vs. -10.2±6.2%, p = 0.040 at initial presentation. None of the parameters were significantly different between the 2 groups upon follow-up. A RVPSS cut-off value of >-19.1% had a sensitivity of 85% and a specificity of 71% to discriminate between the 2 groups. CONCLUSION: In TC, RVFAC, RVPSS, LVEF and LV global strain differed significantly between patients with and without RV dysfunction, whereas TAPSE did not. 2 D strain imaging was feasible for the assessment of RV dysfunction in TC and could discriminate between patients with and without RV involvement in a clinically meaningful way.

  2. Reverse right ventricular structural and extracellular matrix remodeling by estrogen in severe pulmonary hypertension.

    Science.gov (United States)

    Nadadur, Rangarajan D; Umar, Soban; Wong, Gabriel; Eghbali, Mansour; Iorga, Andrea; Matori, Humann; Partow-Navid, Rod; Eghbali, Mansoureh

    2012-07-01

    Chronic pulmonary hypertension (PH) leads to right-ventricular failure (RVF) characterized by RV remodeling. Ventricular remodeling is emerging as an important process during heart failure and recovery. Remodeling in RVF induced by PH is not fully understood. Recently we discovered that estrogen (E2) therapy can rescue severe preexisting PH. Here, we focused on whether E2 (42.5 μg·kg(-1)·day(-1), 10 days) can reverse adverse RV structural and extracellular matrix (ECM) remodeling induced by PH using monocrotaline (MCT, 60 mg/kg). RV fibrosis was evident in RVF males. Intact females developed less severe RV remodeling compared with males and ovariectomized (OVX) females. Novel ECM-degrading disintegrin-metalloproteinases ADAM15 and ADAM17 transcripts were elevated ∼2-fold in all RVF animals. E2 therapy reversed RV remodeling in all groups. In vitro, E2 directly inhibited ANG II-induced expression of fibrosis markers as well as the metalloproteinases in cultured cardiac fibroblasts. Estrogen receptor-β agonist diarylpropionitrile (DPN) but not estrogen receptor-α agonist 4,4',4″-(4-propyl-[1H]-pyrazole-1,3,5-triyl)trisphenol (PPT) was as effective as E2 in inhibiting expression of these genes. Expression of ECM-interacting cardiac fetal-gene osteopontin (OPN) also increased ∼9-fold in RVF males. Intact females were partially protected from OPN upregulation (∼2-fold) but OVX females were not. E2 reversed OPN upregulation in all groups. Upregulation of OPN was also reversed in vitro by E2. Plasma OPN was elevated in RVF (∼1.5-fold) and decreased to control levels in the E2 group. RVF resulted in elevated Akt phosphorylation, but not ERK, in the RV, and E2 therapy restored Akt phosphorylation. In conclusion, E2 therapy reverses adverse RV remodeling associated with PH by reversing fibrosis and upregulation of novel ECM enzymes ADAM15, ADAM17, and OPN. These effects are likely mediated through estrogen receptor-β.

  3. Impact of untreated obstructive sleep apnea on left and right ventricular myocardial function and effects of CPAP therapy.

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    Christoph Hammerstingl

    Full Text Available BACKGROUND: Obstructive sleep apnea (OSA has deteriorating effect on LV function, whereas its impact on RV function is controversial. We aimed to determine the effect of OSA and continuous positive airway pressure (CPAP treatment on left and right ventricular (LV, RV function using transthoracic echocardiography (TTE and 2 dimensional speckle tracking (2D ST analysis of RV deformation capability. METHODS AND RESULTS: 82 patients with OSA and need for CPAP therapy were prospectively enrolled and underwent TTE at study inclusion and after 6 months of follow up (FU. Multivariate regression analysis revealed an independent association between baseline apical right ventricular longitudinal strain (RV-Sl, BMI and the severity of OSA (apical RV-Sl: P = 0.0002, BMI: P = 0.02. After CPAP therapy, LV functional parameters (LVEF: P30:54.1 ± 12.4%, 68.2 ± 13.6%[P30: -6.3 ± 5.7%, -17.9 ± 11.2% [P<0.0001]. CONCLUSIONS: OSA seems to have deteriorating effect on LV and RV function. We found a beneficial effect of CPAP on LV and RV functional parameters predominately in patients with severe OSA. 2D speckle tracking might be of value to determine early changes in global and regional right ventricular function.

  4. Computed tomography angiography with pulmonary artery thrombus burden and right-to-left ventricular diameter ratio after pulmonary embolism.

    Science.gov (United States)

    Ouriel, Kenneth; Ouriel, Richard L; Lim, Yeun J; Piazza, Gregory; Goldhaber, Samuel Z

    2017-02-01

    Purpose Computed tomography angiography is used for quantifying the significance of pulmonary embolism, but its reliability has not been well defined. Methods The study cohort comprised 10 patients randomly selected from a 150-patient prospective trial of ultrasound-facilitated fibrinolysis for acute pulmonary embolism. Four reviewers independently evaluated the right-to-left ventricular diameter ratios using the standard multiplanar reformatted technique and a simplified (axial) method, and thrombus burden with the standard modified Miller score and a new, refined Miller scoring system. Results The intraclass correlation coefficient for intra-observer variability was .949 and .970 for the multiplanar reformatted and axial methods for estimating right-to-left ventricular ratios, respectively. Inter-observer agreement was high and similar for the two methods, with intraclass correlation coefficient of .969 and .976. The modified Miller score had good intra-observer agreement (intraclass correlation coefficient .820) and was similar to the refined Miller method (intraclass correlation coefficient .883) for estimating thrombus burden. Inter-observer agreement was also comparable between the techniques, with intraclass correlation coefficient of .829 and .914 for the modified Miller and refined Miller methods. Conclusions The reliability of computed tomography angiography for pulmonary embolism was excellent for the axial and multiplanar reformatted methods for quantifying the right-to-left ventricular ratio and for the modified Miller and refined Miller scores for quantifying of pulmonary artery thrombus burden.

  5. Relation Between Obesity, Metabolic Syndrome, Successful Long-Term Weight Reduction, and Right Ventricular Function.

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    Zeller, Judith; Strack, Christina; Fenk, Sabine; Mohr, Margareta; Loew, Thomas; Schmitz, Gerd; Maier, Lars; Fischer, Marcus; Baessler, Andrea

    2016-07-27

    This study sought to examine the relationships between right ventricular (RV) function and geometry, morbid obesity with and without the metabolic syndrome, and the effect of long-term weight loss. Obese (n = 153, BMI 41.2 ± 8.7 kg/m(2)) and healthy non-obese control subjects (n = 38, BMI 25.5 ± 3.3 kg/m(2)) of similar age and gender distribution were prospectively studied during the course of a 1-year weight reduction program with echocardiography at baseline and after one year of follow up. Function and geometry of the right heart were evaluated by tricuspid annular plane systolic excursion (TAPSE), tricuspid annular systolic velocity (TDI S'), RV myocardial performance index (TEI), RV end-diastolic (RVEDD) and end-systolic diameter (RVESD), area of the right atrium (RAA), and systolic pulmonary artery pressure (PAP). Whereas parameters of systolic and diastolic LV function were significantly worse in the obese subjects than those in the non-obese subjects (EF 66 ± 6 versus 69 ± 6%, P = 0.004; E/E' 7.4 ± 2.5 versus 6.3 ± 2.6, P = 0.010), parameters of RV function (TAPSE 25.6 ± 4.5 versus 25.1 ± 3.5 mm, P = 0.528; TDI S' 13.5 ± 2.9 versus 13.8 ± 2.9 mm/second, P = 0.553; TEI 0.25 ± 0.13 versus 0.28 ± 0.09, P = 0.283) as well as geometry measurements were comparable between the obese and non-obese participants and also in obese subjects with full blown metabolic syndrome. Additionally, successful weight reduction did not alter the RV parameters. Nevertheless, in the few obese subjects with RV dysfunction (n = 7), metabolic syndrome parameters were more pronounced than in obese with normal RV function.Morbid obesity with and without the metabolic syndrome is accompanied by an impaired LV systolic and diastolic function. In contrast, RV function appears to be less affected by obesity independent of the presence of the metabolic syndrome.

  6. Right ventricular rupture and tamponade caused by malposition of the Avalon cannula for venovenous extracorporeal membrane oxygenation

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    Hirose Hitoshi

    2012-04-01

    Full Text Available Abstract Placement of the Avalon Elite bicaval dual lumen cannula for venovenous extracorporeal membrane oxygenation (VV-ECMO via the internal jugular vein requires precise positioning of the cannula tip in the inferior vena cava with echocardiography or fluoroscopy guidance. Correct guidewire placement is clearly the key first step in assuring proper advancement of the cannula. We report a case of unexpected wire migration into the right ventricle at the time of final cannula advancement, resulting in right ventricular rupture and tamponade. Transesophageal echocardiography is an important monitoring modality for appropriate placement of the VV-ECMO guidewire and Avalon cannula, and in particular, for early identification of potential complications.

  7. Effects of chronic severe pulmonary regurgitation and percutaneous valve repair on right ventricular geometry and contractility assessed by tissue Doppler echocardiography

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Iversen, Kasper K; Vejlstrup, Niels G;

    2010-01-01

    Pulmonary regurgitation (PR) following repair of right ventricular (RV) outflow obstruction is related to slowly progressive RV dilatation and heart failure and will eventually require surgical intervention, but optimal timing of pulmonary valve replacement is challenging. Tissue Doppler based...

  8. Efeitos hemodinâmicos da sobrecarga ventricular direita aguda experimental Efectos hemodinámicos de la sobrecarga ventricular derecha aguda experimental Hemodynamic effects of experimental acute right ventricular overload

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    Flávio Brito Filho

    2011-04-01

    el estudio, siendo divididos en 4 grupos: uno control, no sometido a la oclusión vascular pulmonar, y tres de sobrecarga ventricular derecha sometidos a la oclusión de las siguientes arterias pulmonares: SVD1 (arteria pulmonar izquierda; SVD2 (arteria pulmonar izquierda y del lóbulo inferior derecho y SVD3 (arteria pulmonar izquierda, del lóbulo inferior derecho y del lóbulo mediastinal, obstruyendo la vasculatura pulmonar en 42, 76 y 82,0% respectivamente. Variables de hemodinámica fueron medidas cada 15 minutos durante una hora del estudio. En el análisis estadístico, fueron utilizados ajustes de modelos lineares mixtos con estructura de variancias y covariancias. RESULTADOS:En las comparaciones intergrupales, hubo aumento significativo de la frecuencia cardíaca (p = 0,004, presión arterial pulmonar media (p = 0,001 y presión capilar pulmonar (p BACKGROUND: Acute right ventricular overload is associated with high morbidity and mortality clinical situations such as: extensive lung resection, pulmonary thromboembolism, lung transplantation and high altitude pulmonary edema. Some points of its pathophysiology remain unclear. OBJECTIVE: To assess the hemodynamic effects of experimental acute right ventricular overload in pigs. METHODS: Right ventricular overload was induced through the occlusion of the pulmonary arteries using ligationss. Twenty pigs were used in the study, divided into 04 groups: one control group not subject to pulmonary vascular occlusion, and three right ventricular overload groups subject to occlusion of the following pulmonary arteries: SVD1 (left pulmonary artery; SVD2 (left pulmonary artery and right lower lobe and SVD3 (left pulmonary artery, right lower lobe and mediastinal lobe, obstructing the pulmonary vasculature in 42, 76 and 82.0% respectively. Hemodynamic variables were measured every 15 minutes during one hour of study. The statistical analysis employed mixed linear models with variance and covariance structures. RESULTS

  9. Right ventricular failure due to chronic pressure load: What have we learned in animal models since the NIH working group statement?

    OpenAIRE

    Marinus A. J. Borgdorff; Dickinson, Michael G.; Berger, Rolf M. F.; Bartelds, Beatrijs

    2015-01-01

    Right ventricular (RV) failure determines outcome in patients with pulmonary hypertension, congenital heart diseases and in left ventricular failure. In 2006, the Working Group on Cellular and Molecular Mechanisms of Right Heart Failure of the NIH advocated the development of preclinical models to study the pathophysiology and pathobiology of RV failure. In this review, we summarize the progress of research into the pathobiology of RV failure and potential therapeutic interventions. The pictu...

  10. Use of myocardial tomo-scintigraphy by {sup 123}I - MIBG in right ventricle arrhythmia-gen dysplasia; Interet de la tomoscintigraphie myocardique a la {sup 123}I - MIBG dans la dysplasie arrythmogene du ventricule droit

    Energy Technology Data Exchange (ETDEWEB)

    Agostini, D.; Manrique, A.; Darlas, Y. [Service de Medecine Nucleaire, CHU Cote de Nacre, Caen (France); Loiselet, P.; Scanu, P.; Grollier, G.; Potier, J.C. [Service de Cardiologie, CHU Cote de Nacre, Caen (France); Bouvard, G. [Service de Medecine Nucleaire, CHU Cote de Nacre, Caen (France)

    1997-12-31

    The dysfunction of myocardial sympathetic system was implied in occurrence of ventricular arrhythmias in patients with a right ventricle arrhythmia-gen dysplasia (RVAD). The goal of this study is to evaluate the myocardial pre-synaptic adrenergic regional function by using the cardiac tomo-scintigraphy with {sup 123}I - meta-iodo-benzyl-guanidine (MIBG). Fourteen patients (12 M, 2 F, age: 46{+-} 13) in whom the disease`s diagnosis was done on the basis of the parameters of European Task Force (electric, angiographic, histologic), were studied. Six healthy subjects (32 {+-} 12 years) were at the same time studied as control group. Each patient benefited by an at-rest tomo-scintigraphy by {sup 201}Tl to eliminate any hypo-perfusion which could hinder the interpretation of MIBG fixation. A 48 h delay was necessary between the two isotopic examinations. After blocking the thyroid by Lugol fort, an at-rest tomo-scintigraphy by {sup 123}I - MIBG was effected 4 h after the injection IV of 259 MBq of tracer following a classical acquisition of a myocardial tomography with a tracer-adopted collimator (Elscint camera). Global and regional evaluations of the cardiac adrenergic neuronal function were effected by using the cardio-mediastinal ratio (CMR) and the circumferential profile, respectively, for the localization, extension and amplitude of regional adrenergic defects. The CMR is within the normal limits (236 {+-} 39% vs 234 {+-} 14% in healthy subjects). The adrenergic defects are present in 11/14 patients (a reduction of 50% of capture of MIBG as compared with the control group, < average - 2 ET) in the anterior and lateral regions of left ventricle. In conclusion, the tomo-scintigraphy by {sup 123}I - MIBG allows the detecting of presence of a sympathetic dys-innervation of left ventricle in patients afflicted with RVAD

  11. Analysis of right ventricular kinesis by means of transesophageal echocardiography: present problems and perspectives.

    Science.gov (United States)

    Kozàkovà, M; Palombo, C; Benanti, C; L'Abbate, A; Distante, A

    1994-03-01

    The evaluation of right ventricular (RV) kinesis by two-dimensional echocardiography represents a difficult task. Transthoracic echocardiography can visualize the RV in several projections, but the image quality and the variability of imaging views usually do not allow quantitative analysis. We investigated the potential of transesophageal echocardiography (TEE) for evaluating RV global function and regional kinesis, in 32 controls and in 16 patients with inferior myocardial infarction (MI) and asynergy involving the inferior wall of both ventricles. Good-quality images of at least one horizontal section of the RV were obtained in 73% of subjects by conventional, 90 degrees sector and in 100% of subjects by wide-angle, "panoramic" sector. Images of the RV in short-axis view at medium level were acquired and evaluated in 93% of cases, but at basal and apical levels only in 67% and 39%, respectively. The low percentage of successful detection and evaluation of the RV at apical level can be explained by prominent motion and trabeculation of the apex. Global systolic area changes (SAC) in controls attained similar values at apical and medium levels (60% and 59%, respectively), but were significantly lower (48%, P less than 0.05) at basal level. In patients with previous inferior MI and inferoposterior asynergy, global SAC were significantly (P less than 0.01) lower at medium and basal levels (32% and 27%, respectively) compared with controls. Regional kinesis of RV was assessed as segmental SAC in 12 different segments, by fixed and float system of center of cavity.(ABSTRACT TRUNCATED AT 250 WORDS)

  12. Impact of endoscopic lung volume reduction on right ventricular myocardial function.

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    Carmen Pizarro

    Full Text Available Endoscopic lung volume reduction (ELVR provides a minimally invasive therapy for patients with severe lung emphysema. As its impact on right ventricular (RtV function is undefined, we examined the extent of RtV functional changes following ELVR, as assessed by use of speckle tracking-based RtV deformation analysis.We enrolled 32 patients with severe emphysematous COPD scheduled for bronchoscopic LVR using endobronchial valves (Zephyr, PulmonX, Inc., comprising 16 matched clinical responders and 16 non-responders. Echocardiography was conducted one day prior to ELVR and at an eight-week postprocedural interval.Patients were predominantly of late middle-age (65.8 ± 8.7 yrs, male (62.5% and presented advanced COPD emphysema (means FEV1 and RV: 32.6% and 239.1% of predicted, respectively. After ELVR, RtV apical longitudinal strain improved significantly in the total study cohort (-7.96 ± 7.02% vs. -13.35 ± 11.48%, p = 0.04, whereas there were no significant changes in other parameters of RtV function such as RtV global longitudinal strain, TAPSE or pulmonary arterial systolic pressure. In responding patients, 6MWT-improvement correlated with a decrease in NT-proBNP (Pearson´s r: -0.53, p = 0.03. However, clinical non-responders did not exhibit any RtV functional improvement.ELVR beneficially impacts RtV functional parameters. Speckle tracking-based RtV apical longitudinal strain analysis allows early determination of RtV contractile gain and identification of clinical responsiveness.

  13. The reservoir-wave approach to characterize pulmonary vascular-right ventricular interactions in humans.

    Science.gov (United States)

    Ghimire, Anukul; Andersen, Mads J; Burrowes, Lindsay M; Bouwmeester, J Christopher; Grant, Andrew D; Belenkie, Israel; Fine, Nowell M; Borlaug, Barry A; Tyberg, John V

    2016-12-01

    Using the reservoir-wave approach (RWA) we previously characterized pulmonary vasculature mechanics in a normal canine model. We found reflected backward-traveling waves that decrease pressure and increase flow in the proximal pulmonary artery (PA). These waves decrease right ventricular (RV) afterload and facilitate RV ejection. With pathological alterations to the pulmonary vasculature, these waves may change and impact RV performance. Our objective in this study was to characterize PA wave reflection and the alterations in RV performance in cardiac patients, using the RWA. PA pressure, Doppler-flow velocity, and pulmonary arterial wedge pressure were measured in 11 patients with exertional dyspnea. The RWA was employed to analyze PA pressure and flow; wave intensity analysis characterized PA waves. Wave-related pressure was partitioned into two components: pressures due to forward-traveling and to backward-traveling waves. RV performance was assessed by examining the work done in raising reservoir pressure and that associated with the wave components of systolic PA pressure. Wave-related work, the mostly nonrecoverable energy expended by the RV to eject blood, tended to vary directly with mean PA pressure. Where PA pressures were lower, there were pressure-decreasing/flow-increasing backward waves that aided RV ejection. Where PA pressures were higher, there were pressure-increasing/flow-decreasing backward waves that impeded RV ejection. Pressure-increasing/flow-decreasing backward waves were responsible for systolic notches in the Doppler flow velocity profiles in patients with the highest PA pressure. Pulmonary hypertension is characterized by reflected waves that impede RV ejection and an increase in wave-related work. The RWA may facilitate the development of therapeutic strategies.

  14. Evaluation of Right Ventricular Volume and Systolic Function by Real-time Three-dimensional Echocardiography

    Institute of Scientific and Technical Information of China (English)

    WANG Jing; WANG Xinfang; XIE Mingxing; YANG Ya; LV Qing; YANG Ying; WANG Liangyu

    2005-01-01

    The optimal plane for measurement of the right ventricular (RV) volumes by real-time three-dimensional echocardiography (RT3DE) was determined and the feasibility and accuracy of RT3DE in studying RV systolic function was assessed. RV "Full volume" images were acquired by RT3DE in 22 healthy subjects. RV end-diastolic volumes (RVEDV) and end-systolic volumes (RVESV) were outlined using apical biplane, 4-plane, 8-plane, 16-plane offline separately. RVSV and RVEF were calculated. Meanwhile tricuspid annual systolic excursion (TASE) was measured by M-mode echo. LVSV was outlined by 2-D echo according to the biplane Simpsons rule. The results showed: (1) There was a good correlation between RVSV measured from series planes and LVSV from 2-D echo (r=0.73; r=0.69; r=0.63; r=0.66, P<0.25-0. 0025); (2) There were significant differences between RVEDV in biplane and those in 4-, 8-, 16-plane (P<0. 001). There was also difference between RV volume in 4-plane and that in 8-plane (P<0.05), but there was no significant difference between RV volume in 8-plane and that in 16-plane (P>0.05); (3) Inter-observers and intro-observers variability analysis showed that there were close agreements and relations for RV volumes (r=0. 986, P<0. 001; r=0.93, P<0. 001); (4) There was a significantly positive correlation of TASE to RVSV and RVEF from RT3DE (r=0.83; r=0.90). So RV volume measures with RT3DE are rapid, accurate and reproducible. In view of RVs complex shape,apical 8-plane method is better in clinical use. It may allow early detection of RV systolic function.

  15. Right ventricular arrhythmogenesis in failing human heart: the role of conduction and repolarization remodeling

    Science.gov (United States)

    Lou, Qing; Janks, Deborah L.; Holzem, Katherine M.; Lang, Di; Onal, Birce; Ambrosi, Christina M.; Fedorov, Vadim V.; Wang, I-Wen

    2012-01-01

    Increased dispersion of repolarization has been suggested to underlie increased arrhythmogenesis in human heart failure (HF). However, no detailed repolarization mapping data were available to support the presence of increased dispersion of repolarization in failing human heart. In the present study, we aimed to determine the existence of enhanced repolarization dispersion in the right ventricular (RV) endocardium from failing human heart and examine its association with arrhythmia inducibility. RV free wall preparations were dissected from five failing and five nonfailing human hearts, cannulated and coronary perfused. RV endocardium was optically mapped from an ∼6.3 × 6.3 cm2 field of view. Action potential duration (APD), dispersion of APD, and conduction velocity (CV) were quantified for basic cycle lengths (BCL) ranging from 2,000 ms to the functional refractory period. We found that RV APD was significantly prolonged within the failing group compared with the nonfailing group (560 ± 44 vs. 448 ± 39 ms, at BCL = 2,000 ms, P < 0.05). Dispersion of APD was increased in three failing hearts (161 ± 5 vs. 86 ± 19 ms, at BCL = 2,000 ms). APD alternans were induced by rapid pacing in these same three failing hearts. CV was significantly reduced in the failing group compared with the nonfailing group (81 ± 11 vs. 98 ± 8 cm/s, at BCL = 2,000 ms). Arrhythmias could be induced in two failing hearts exhibiting an abnormally steep CV restitution and increased dispersion of repolarization due to APD alternans. Dispersion of repolarization is enhanced across the RV endocardium in the failing human heart. This dispersion, together with APD alternans and abnormal CV restitution, could be responsible for the arrhythmia susceptibility in human HF. PMID:23042951

  16. Patients' and physicians' needs, experiences and preferences in the treatment of right ventricular outflow tract dysfunction

    Directory of Open Access Journals (Sweden)

    Luciana Scalone

    2012-06-01

    Full Text Available

    Background: patients with congenital heart defects, developing right ventricular outflow tract (rVoT dysfunction, can face repeated open chest interventions over their lifetime. repeating surgery increases difficulties and procedural risks, and exposes patients to burdensome and long recovery times that may induce them to postpone the treatment, with possible severe and irreversible consequences for their health. The percutaneous procedure was introduced to delay the need for open chest surgery. uncertainties still exist regarding the lifelong consequences that may result from adopting different treatment strategies. current decisions on treatment depend on patients’ clinical needs, but also on physicians’ experience and opinion, patients’ preferences, and procedural costs. The objective is to identify which treatment characteristics influence decisions on how to treat patients with rVoT dysfunction.

    Methods: a literature review was conducted, followed by a discussion with a panel of experts. Ten treatment characteristics, potentially relevant for treatment, were identified and rated in a survey, according to the importance assigned to each characteristic by specialist physicians, patients and/or their caregivers.

    Results: while some characteristics appear to be more important (risk of severe complications associated with intervention delays or less important (scar to both physicians and patients/caregivers, other characteristics are rated differently in importance depending on subjects consulted, e.g., risk of complications during the months post intervention was among the most important characteristics for patients/caregivers, but the fifth most important characteristic for physicians.

    Conclusions: to optimize benefits and efficiency of the treatment strategies, perceptions and opinions from the different subjects involved, together with patients

  17. Lead reduces tension development and the myosin ATPase activity of the rat right ventricular myocardium

    Directory of Open Access Journals (Sweden)

    D.V. Vassallo

    2008-09-01

    Full Text Available Lead (Pb2+ poisoning causes hypertension, but little is known regarding its acute effects on cardiac contractility. To evaluate these effects, force was measured in right ventricular strips that were contracting isometrically in 45 male Wistar rats (250-300 g before and after the addition of increasing concentrations of lead acetate (3, 7, 10, 30, 70, 100, and 300 µM to the bath. Changes in rate of stimulation (0.1-1.5 Hz, relative potentiation after pauses of 15, 30, and 60 s, effect of Ca2+ concentration (0.62, 1.25, and 2.5 mM, and the effect of isoproterenol (20 ng/mL were determined before and after the addition of 100 µM Pb2+. Effects on contractile proteins were evaluated after caffeine treatment using tetanic stimulation (10 Hz and measuring the activity of the myosin ATPase. Pb2+ produced concentration-dependent force reduction, significant at concentrations greater than 30 µM. The force developed in response to increasing rates of stimulation became smaller at 0.5 and 0.8 Hz. Relative potentiation increased after 100 µM Pb2+ treatment. Extracellular Ca2+ increment and isoproterenol administration increased force development but after 100 µM Pb2+ treatment the force was significantly reduced suggesting an effect of the metal on the sarcolemmal Ca2+ influx. Concentration of 100 µM Pb2+ also reduced the peak and plateau force of tetanic contractions and reduced the activity of the myosin ATPase. Results showed that acute Pb2+ administration, although not affecting the sarcoplasmic reticulum activity, produces a concentration-dependent negative inotropic effect and reduces myosin ATPase activity. Results suggest that acute lead administration reduced myocardial contractility by reducing sarcolemmal calcium influx and the myosin ATPase activity. These results also suggest that lead exposure is hazardous and has toxicological consequences affecting cardiac muscle.

  18. Right Ventricular Dysfunction in Patients Experiencing Cardiotoxicity during Breast Cancer Therapy

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    Anna Calleja

    2015-01-01

    Full Text Available Background. Right ventricular (RV dysfunction during cancer therapy related cardiotoxicity and its prognostic implications have not been examined. Aim. We sought to determine the incidence and prognostic value of RV dysfunction at time of LV defined cardiotoxicity. Methods. We retrospectively identified 30 HER2+ female patients with breast cancer treated with trastuzumab (± anthracycline who developed cardiotoxicity and had a diagnostic quality transthoracic echocardiography. LV ejection fraction (LVEF, RV fractional area change (RV FAC, and peak systolic longitudinal strain (for both LV and RV were measured on echocardiograms at the time of cardiotoxicity and during follow-up. Thirty age balanced precancer therapy and HER2+ breast cancer patients were used as controls. Results. In the 30 patients with cardiotoxicity (mean ± SD age 54 ± 12 years RV FAC was significantly lower (42 ± 7 versus 47 ± 6%, P=0.01 compared to controls. RV dysfunction defined by global longitudinal strain (GLS < −20.3% was seen in 40% (n=12. During follow-up in 16 out of 30 patients (23 ± 15 months, there was persistent LV dysfunction (EF < 55% in 69% (n=11. Concomitant RV dysfunction at the time of LV cardiotoxicity was associated with reduced recovery of LVEF during follow-up although this was not statistically significant. Conclusion. RV dysfunction at the time of LV cardiotoxicity is frequent in patients with breast cancer receiving trastuzumab therapy. Despite appropriate management, LV dysfunction persisted in the majority at follow-up. The prognostic value of RV dysfunction at the time of cardiotoxicity warrants further investigation.

  19. Intralipid prevents and rescues fatal pulmonary arterial hypertension and right ventricular failure in rats.

    Science.gov (United States)

    Umar, Soban; Nadadur, Rangarajan D; Li, Jingyuan; Maltese, Federica; Partownavid, Parisa; van der Laarse, Arnoud; Eghbali, Mansoureh

    2011-09-01

    Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling leading to right ventricular (RV) hypertrophy and failure. Intralipid (ILP), a source of parenteral nutrition for patients, contains γ-linolenic acid and soy-derived phytoestrogens that are protective for lungs and heart. We, therefore, investigated the therapeutic potential of ILP in preventing and rescuing monocrotaline-induced PAH and RV dysfunction. PAH was induced in male rats with monocrotaline (60 mg/kg). Rats then received daily ILP (1 mL of 20% ILP per day IP) from day 1 to day 30 for prevention protocol or from day 21 to day 30 for rescue protocol. Other monocrotaline-injected rats were left untreated to develop severe PAH by day 21 or RV failure by approximately day 30. Saline or ILP-treated rats served as controls. Significant increase in RV pressure and decrease in RV ejection fraction in the RV failure group resulted in high mortality. Therapy with ILP resulted in 100% survival and prevented PAH-induced RV failure by preserving RV pressure and RV ejection fraction and preventing RV hypertrophy and lung remodeling. In preexisting severe PAH, ILP attenuated most lung and RV abnormalities. The beneficial effects of ILP in PAH seem to result from the interplay of various factors, among which preservation and/or stimulation of angiogenesis, suppression and/or reversal of inflammation, fibrosis and hypertrophy, in both lung and RV, appear to be major contributors. In conclusion, ILP not only prevents the development of PAH and RV failure but also rescues preexisting severe PAH.

  20. Right Ventricular Myocardial Tissue Velocities, Myocardial Performance Index, and Tricuspid Annular Plane Systolic Excursion in Totally Corrected Tetralogy of Fallot Patients

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    Asadolah Tanasan

    2015-10-01

    Full Text Available Background: Longer survival after the total repair of the Tetralogy of Fallot increases the importance of late complications such as right ventricular dysfunction. This is a prospective study of the right ventricular function in totally corrected Tetralogy of Fallot patients versus healthy children.Methods: Thirty-two healthy children were prospectively compared with 30 totally corrected Tetralogy of Fallot patients. Right ventricular myocardial tissue velocities, right ventricular myocardial performance index, and tricuspid annular plane systolic excursion were investigated as well as the presence and severity of pulmonary regurgitation.Results: The two groups were age-and sex-matched. Mean systolic peak velocity (Sa and tricuspid annular plane systolic excursion were significantly decreased, while myocardial performance index and early to late diastolic velocity (Ea/Aa were significantly increased in the Tetralogy of Fallot patients. Early diastolic velocity (Ea showed no significant difference between the two groups. Sa correlated significantly with tricuspid annular plane systolic excursion in both the normal children and totally corrected Tetralogy of Fallot patients. Myocardial performance index was significantly higher in the patients with moderate to severe pulmonary regurgitation than in those with mild regurgitation. However, there was no significant correlation between this index and right ventricular myocardial tissue velocities.Conclusion: In this study, systolic right ventricular function indices (Sa and tricuspid annular plane systolic excursion were impaired in the totally corrected Tetralogy of Fallot patients. Myocardial performance index was affected by the severity of pulmonary regurgitation.

  1. A meta-analysis for the echocardiographic assessment of right ventricular structure and function in ARVC: a Study by the Research and Audit Committee of the British Society of Echocardiography

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    Mohammad Qasem

    2016-10-01

    Full Text Available Introduction: Arrhythmogenic right ventricular cardiomyopathy ARVC is an inherited pathology that can increase the risk of sudden death. Current task force criteria for echocardiographic diagnosis do not include new, regional assessment tools which may be relevant in a phenotypically diverse disease. We adopted a systematic review and meta-analysis approach to highlight echocardiographic indices that differentiated ARVC patients and healthy controls. Methods: Data was extracted and analysed from prospective trials that employed a case–control design meeting strict inclusion and exclusion as well as a priori quality criteria. Structural indices included proximal RV outflow tract (RVOT1 and RV diastolic area (RVDarea. Functional indices included RV fractional area change (RVFAC, tricuspid annular systolic excursion (TAPSE, peak systolic and early diastolic myocardial velocities (S′ and E′, respectively and myocardial strain. Results: Patients with ARVC had larger RVOT1 (mean ± s.d.; 34 vs 28 mm, P < 0.001 and RVDarea (23 vs 18 cm2, P < 0.001 compared with healthy controls. ARVC patients also had lower RVFAC (38 vs 46%, P < 0.001, TAPSE (17 vs 23 mm, P < 0.001, S′ (9 vs 12 cm/s, P < 0.001, E′ (9 vs 13 cm/s, P < 0.001 and myocardial strain (−17 vs −30%, P < 0.001. Conclusion: The data from this meta-analysis support current task force criteria for the diagnosis of ARVC. In addition, other RV measures that reflect the complex geometry and function in ARVC clearly differentiated between ARVC and healthy controls and may provide additional diagnostic and management value. We recommend that future working groups consider this data when proposing new/revised criteria for the echocardiographic diagnosis of ARVC.

  2. A meta-analysis for the echocardiographic assessment of right ventricular structure and function in ARVC: a Study by the Research and Audit Committee of the British Society of Echocardiography

    Science.gov (United States)

    Qasem, Mohammad; Utomi, Victor; George, Keith; Somauroo, John; Zaidi, Abbas; Forsythe, Lynsey; Bhattacharrya, Sanjeev; Lloyd, Guy; Rana, Bushra; Ring, Liam; Robinson, Shaun; Senior, Roxy; Sheikh, Nabeel; Sitali, Mushemi; Sandoval, Julie; Steeds, Richard; Stout, Martin; Willis, James

    2016-01-01

    Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited pathology that can increase the risk of sudden death. Current task force criteria for echocardiographic diagnosis do not include new, regional assessment tools which may be relevant in a phenotypically diverse disease. We adopted a systematic review and meta-analysis approach to highlight echocardiographic indices that differentiated ARVC patients and healthy controls. Methods Data was extracted and analysed from prospective trials that employed a case–control design meeting strict inclusion and exclusion as well as a priori quality criteria. Structural indices included proximal RV outflow tract (RVOT1) and RV diastolic area (RVDarea). Functional indices included RV fractional area change (RVFAC), tricuspid annular systolic excursion (TAPSE), peak systolic and early diastolic myocardial velocities (S′ and E′, respectively) and myocardial strain. Results Patients with ARVC had larger RVOT1 (mean ± s.d.; 34 vs 28 mm, P < 0.001) and RVDarea (23 vs 18 cm2, P < 0.001) compared with healthy controls. ARVC patients also had lower RVFAC (38 vs 46%, P < 0.001), TAPSE (17 vs 23 mm, P < 0.001), S′ (9 vs 12 cm/s, P < 0.001), E′ (9 vs 13 cm/s, P < 0.001) and myocardial strain (−17 vs −30%, P < 0.001). Conclusion The data from this meta-analysis support current task force criteria for the diagnosis of ARVC. In addition, other RV measures that reflect the complex geometry and function in ARVC clearly differentiated between ARVC and healthy controls and may provide additional diagnostic and management value. We recommend that future working groups consider this data when proposing new/revised criteria for the echocardiographic diagnosis of ARVC. PMID:27686556

  3. Right ventricular recovery after cervical extra corporeal membrane oxygenation in a four-month-old male leading to left Berlin Heart Excor assistance.

    Science.gov (United States)

    Henaine, Roland; Moutaouekkil, Mehdi; Di-Filippo, Sylvie; Bastien, Olivier

    2010-12-01

    A 4-month-old male with cardiogenic shock was implanted with cervical extracorporeal membrane oxygenation (ECMO). He had a biventricular heart failure associated to multiple organ failure (MOF). Right ventricular (RV) function and MOF recovered during five days ECMO leading to left ventricular assistance device (LVAD) implantation. This case shows the advantages of the double bridge strategy providing intervention time for complications refractory to VAD implantation and evaluation time of right and left ventricular function for potential mono- or bi-VAD implantation. Furthermore, there are no reports describing this strategy in young children leading to RV function recovery and its advantage.

  4. Comparison of mortality rates and progression of left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy and dilated versus nondilated right ventricular cavities.

    Science.gov (United States)

    Sun, J P; James, K B; Yang, X S; Solankhi, N; Shah, M S; Arheart, K L; Thomas, J D; Stewart, W J

    1997-12-15

    This study assesses the influence of right ventricular (RV) dilation on the progression of left ventricular (LV) dysfunction and survival in patients with idiopathic dilated cardiomyopathy (IDC). Using transthoracic echocardiography, we studied 100 patients with IDC aged 20 to 80 years (mean 55 +/- 14); 67% were men. In the apical 4-chamber view, diastolic LV and RV chamber area measurements classified patients into 2 groups: group RV enlargement+ (RV area/LV area > 0.5) included 54 patients; group RV enlargement- (no RV enlargement) had RV area/LV area < or = 0.5. Echocardiographic studies were repeated in all patients after a mean of 33 +/- 16 months. At the time of the initial study, the 2 groups did not differ in age, gender, incidence of atrial fibrillation and diabetes, left ventricular mass, and LV ejection fraction, but the RV enlargement+ group had more severe tricuspid regurgitation and less LV enlargement. After 47 +/- 22 months (range 12 to 96), patients in group RV enlargement+ had lower LV ejection fraction (29% vs 34%, p = 0.006) than patients with initial RV enlargement-. At clinical follow-up, mortality was higher (43%) in patients with initial RV enlargement+ than the RV enlargement- patients (15%), p = 0.002. For survivors, the mitral deceleration time averaged 157 +/- 36 ms; for nonsurvivors or patients who required transplant, the mitral deceleration time averaged 97 +/- 12 ms (p < 0.0001). With use of a multivariate Cox model adjusting for LV ejection fraction, LV size, and age, the relative risk ratio of mortality from initial RV enlargement+ was 4.4 (95% confidence limits 1.7 to 11.1) (p = 0.002). Thus, patients with significant RV dilation had nearly triple the mortality over 4 years and more rapidly deteriorating LV function than patients with less initial RV dilation. In IDC, RV enlargement is a strong marker for adverse prognosis that may represent a different morphologic subset.

  5. Taquicardia ventricular del tracto de salida del ventrículo derecho durante el embarazo Right ventricular outflow tachycardia during pregnancy

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    Ariel K. Saad

    2012-06-01

    Full Text Available Durante el embarazo aumentan el metabolismo basal, el consumo de O2, la frecuencia cardíaca, el volumen sistólico, el volumen minuto y la volemia y disminuyen la tensión arterial y la resistencia periférica. Diferentes estudios han demostrado que durante este período la posibilidad de que ocurra una arritmia cardíaca o se produzca la exacerbación de una arritmia preexistente es mayor. No obstante, en su enorme mayoría carecen de importancia pronóstica tanto para la madre como para el feto. La taquicardia ventricular del tracto de salida del ventrículo derecho es una arritmia poco frecuente y su aparición se ha correlacionado con el aumento del tono adrenérgico. Se presentan los casos de dos pacientes que mostraron en el curso de la gestación reiterados episodios de taquicardia ventricular del tracto de salida del ventrículo derecho. Se analiza la asociación del embarazo con la ocurrencia de trastornos del ritmo cardíaco.During pregnancy, there is an increase in metabolism, oxygen consumption, heart rate, stroke volume, cardiac output, blood volume and a decrease in blood pressure and peripheral resistance. Studies have shown that during this period the occurrence of cardiac arrhythmias is not uncommon. Fortunately, malignant arrhythmias are rare. Herein we report two young patients who presented with symptomatic right ventricular outflow tachycardia during pregnancy that required antiarrhythmic therapy. Possible pathophysiologic mechanisms are discussed.

  6. Implication of Right Ventricular Dysfunction on Long-term Outcome in Patients with Ischemic Cardiomyopathy Undergoing Coronary Artery Bypass Grafting with or without Surgical Ventricular Reconstruction

    Science.gov (United States)

    Kukulski, Tomasz; She, Lilin; Racine, Normand; Gradinac, Sinisa; Panza, Julio A.; Velazquez, Eric J.; Chan, Kwan; Petrie, Mark C.; Lee, Kerry L.; Pellikka, Patricia A.; Romanov, Alexander; Biernat, Jolanta; Rouleau, Jean L.; Batlle, Carmen; Rogowski, Jan; Ferrazzi, Paolo; Zembala, Marian; Oh, Jae K.

    2014-01-01

    Background Whether right ventricular (RV) dysfunction affects clinical outcome after CABG with or without SVR is still unknown. Thus, the aim of the study was to assess the impact of RV dysfunction on clinical outcome in patients with ischemic cardiomyopathy undergoing coronary artery bypass grafting (CABG) with or without surgical ventricular reconstruction (SVR). Methods and Results Of 1,000 STICH patients with coronary artery disease (CAD), left ventricular (LV) ejection fraction (EF) ≤35% and anterior dysfunction randomized to undergo CABG or CABG + SVR, baseline RV function could be assessed by echocardiography in 866 patients. Patients were followed for a median of 48 months. All-cause mortality or cardiovascular hospitalization was the primary endpoint, and all-cause mortality alone was a secondary endpoint. RV dysfunction was mild in 102 (12%) patients and moderate or severe in 78 (9%) patients. Moderate to severe RV dysfunction was associated with larger LV, lower EF, more severe mitral regurgitation, higher filling pressure, and higher pulmonary artery systolic pressure (all p<0.0001) compared to normal or mildly reduced RV function. A significant interaction between RV dysfunction and treatment allocation was observed. Patients with moderate or severe RV dysfunction who received CABG + SVR had significantly worse outcomes compared to patients who received CABG alone on both the primary (HR=1.86; CI=1.06–3.26; p=0.028) and the secondary endpoint (HR=3.37; CI=1.36–8.37; p=0.005). After adjusting for all other prognostic clinical factors, the interaction remained significant with respect to all-cause mortality (p=0.022). Conclusion Adding SVR to CABG may worsen long-term survival in ischemic cardiomyopathy patients with moderate to severe RV dysfunction, which reflects advanced LV remodeling. PMID:25451487

  7. [Right ventricular outflow tract reconstruction using monocusp valved outflow patch for pulmonary atresia with ventricular septal defect: influence of the presence of major aorto-pulmonary collateral arteries].

    Science.gov (United States)

    Hisamochi, K; Ishino, K; Kawada, M; Ohshima, Y; Aoki, A; Arai, S; Sano, S

    2001-07-01

    We have preferably utilized monocusp valved outflow patch (MVOP) for right ventricular outflow tract (RVOT) reconstruction in pulmonary atresia with ventricular septal defect (PA + VSD). The purpose of this study was to evaluate the influence of the presence of major aorto-pulmonary collateral arteries (MAPCAs) on probability of MVOP reconstruction and development of RVOT restenosis in midterm. 49 patients underwent complete repair (either MVOP reconstruction or Rastelli procedure) of PA + VSD in our service. These patients were divided into 2 groups: group 1; 21 patients with MAPCAs, group 2; 28 patients without MAPCAs. There was one operative death (group 1). The probably of MVOP reconstruction was similar between group 1 and group 2 (71 vs 79%, p = 0.57, chi 2 test). Follow-up was completed for 48 survivors with the period ranged 3-108 months (mean 47 months). In group 1, one patient died suddenly at home 10 months after surgery. For 47 long-term patients, the ratio of freedom from RVOT restenosis was 72% (95% CI: 52-92%, Kaplan-Meier method) at 5 year. There was no difference between 2 groups (group 1; 73%, 95% CI: 45-100%, group 2; 74%, 95% CI: 48-99%, respectively, p = 0.85 by Log-Rank test). The presence of MAPCAs in PA + VSD was not a risk factor for either the probably of MVOP reconstruction or development of RVOT restenosis in midterm.

  8. Reverse left ventricular remodeling is more likely in non ischemic cardiomyopathy patients upgraded to biventricular stimulation after chronic right ventricular pacing

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    Morales Maria-Aurora

    2011-12-01

    Full Text Available Abstract Background Chronic right ventricular (RV apical pacing may lead to left ventricular (LV dyssynchrony and LV dysfunction. In heart failure due to RV pacing, upgrading to biventricular stimulation (CRT can improve NYHA Class and LV function. A proportion of patients do not respond to upgrading. Aim was to assess whether etiology of LV dysfunction accounts for responses to CRT in RV-paced patients. Methods Sixty-two patients treated by CRT, under RV pacing from 50.2 ± 5.4 months, were studied. Cause of LV dysfunction was non-ischemic (NIC in 28 and ischemic cardiomyopathy (IC in 34 patients. Clinical and conventional echocardiographic parameters were available within 1 month before RV pacing, within 1 month before CRT and at 12 ± 2 months of follow-up (FU. Results Decreased LVEF (from 37.0 ± 8.8 to 25.6 ± 6.1%, p 10% decrease in LVESD was observed in 24 patients: 5 with IC, 19 with NIC (p 10% decrease in LVESD remained highly significant (p Conclusions CRT improves functional class even after long-lasting pacing. Reverse remodeling is evident in a small population, more likely with NIC.

  9. Impact of right-ventricular apical pacing on the optimal left-ventricular lead positions measured by phase analysis of SPECT myocardial perfusion imaging

    Energy Technology Data Exchange (ETDEWEB)

    Hung, Guang-Uei [Chang Bing Show Chwan Memorial Hospital, Changhua (China); China Medical University, Department of Biomedical Imaging and Radiological Science, Taichung (China); Huang, Jin-Long [Taichung Veterans General Hospital, Cardiovascular Center, Taichung (China); School of Medicine, National Yang-Ming University, Institute of Clinical Medicine, and Cardiovascular Research Institute, Department of Medicine, Taipei (China); Chung-Shan Medical University, Department of Medicine, School of Medicine, Taichung (China); Lin, Wan-Yu; Tsai, Shih-Chung [Taichung Veterans General Hospital, Department of Nuclear Medicine, Taichung (China); Wang, Kuo-Yang [Taichung Veterans General Hospital, Cardiovascular Center, Taichung (China); Chung-Shan Medical University, Department of Medicine, School of Medicine, Taichung (China); Chen, Shih-Ann [School of Medicine, National Yang-Ming University, Institute of Clinical Medicine, and Cardiovascular Research Institute, Department of Medicine, Taipei (China); Taipei Veterans General Hospital, Division of Cardiology, Department of Medicine, Taipei (China); Lloyd, Michael S.; Chen, Ji [Emory University, Department of Radiology and Imaging Sciences, Atlanta, GA (United States)

    2014-06-15

    The use of SPECT phase analysis to optimize left-ventricular (LV) lead positions for cardiac resynchronization therapy (CRT) was performed at baseline, but CRT works as simultaneous right ventricular (RV) and LV pacing. The aim of this study was to assess the impact of RV apical (RVA) pacing on optimal LV lead positions measured by SPECT phase analysis. This study prospectively enrolled 46 patients. Two SPECT myocardial perfusion scans were acquired under sinus rhythm with complete left bundle branch block and RVA pacing, respectively, following a single injection of {sup 99m}Tc-sestamibi. LV dyssynchrony parameters and optimal LV lead positions were measured by the phase analysis technique and then compared between the two scans. The LV dyssynchrony parameters were significantly larger with RVA pacing than with sinus rhythm (p ∝0.01). In 39 of the 46 patients, the optimal LV lead positions were the same between RVA pacing and sinus rhythm (kappa = 0.861). In 6 of the remaining 7 patients, the optimal LV lead positions were along the same radial direction, but RVA pacing shifted the optimal LV lead positions toward the base. The optimal LV lead positions measured by SPECT phase analysis were consistent, no matter whether the SPECT images were acquired under sinus rhythm or RVA pacing. In some patients, RVA pacing shifted the optimal LV lead positions toward the base. This study supports the use of baseline SPECT myocardial perfusion imaging to optimize LV lead positions to increase CRT efficacy. (orig.)

  10. A comparative study of right ventricular outflow tract pacing and right ventricular apical pacing in children%儿童右室流出道起搏与右室心尖起搏的对比研究

    Institute of Scientific and Technical Information of China (English)

    陈晶; 曾国洪; 曾少颖; 王树水

    2014-01-01

    目的:回顾性对比研究儿童行VVI起搏治疗的右室流出道起搏与右室心尖部起搏对近期心功能、QRS波时限及起搏参数的影响。方法:回顾2006年7月至2013年11月,在我科行VVI起搏治疗的38例患儿病例资料,根据术中记录螺旋电极植入的部位分为右室流出道组(n=22)和右室心尖部组(n=16)。记录术前和术后的心脏彩超心功能指标及QRS波时限,术中与术后起搏参数指标进行比较。结果:右室流出道组与右室心尖组在术前及术后心功能指标、术中及术后的起搏阈值差值、导线阻抗差值、R波幅度差值差异无统计学意义。右室流出道起搏组与右室心尖起搏组术后QRS波时限增宽,差异有统计学意义。术前与术后的QRS波时限差值[(134.95±12.86)ms vs(147.44±22.35)ms,t=1.35,P=0.01],差异有统计学意义。结论:儿童右室流出道起搏安全可行,两者起搏术后QRS波时限增宽,但右室心尖起搏组QRS波时限增宽更明显。%Objective To compare the impacts of right ventricular outflow tract pacing and right ventricular apical pacing on short-term cardiac function, QRS duration and pacing parameters in children undergoing VVI pacemaker therapy. Methods The clinical data of 38 children undergoing VVI pacemaker treatment in our department from July 2006 to November 2013 were retrospectively reviewed and analyzed to make grouping: 22 with right ventricular outflow tract pacing based on the operational records were assigned in one group and 16 with right ventricular apical pacing as the other group. The two groups were compared in terms of cardiac function indexes , QRS complex width and intra- and post- operative parameters of the implanted pacemakers. Results There were no significant differences between the two groups in pre-and post-operative cardiac function indexes , intra- and post-operative difference in pacing thresholds

  11. The accuracy and optimal slice thickness of multislice helical computed tomography for right and left ventricular volume measurement

    Institute of Scientific and Technical Information of China (English)

    崔炜; 近藤武; 安野泰史; 郭玉印; 佐藤贵久; 皿井正義; 篠崎仁史; 柿澤聡士; 杉浦厚司; 大岛慶太; 片田和廣; 菱田仁

    2004-01-01

    Background Multislice helical computed tomography (MSCT) has been used to depict coronary anatomy noninvasively, and proved useful for evaluating ventricular function. The aim of our study was to assess the accuracy of ventricular volume as measured by MSCT. Methods Fourteen human left ventricular (LV) and 15 right ventricular (RV) casts were scanned by MSCT. A series of LV and RV short-axis images were reconstructed later with slice thickness of 2.0 mm, 3.5 mm, 5.0 mm, 7.0 mm, and 10.0 mm. Ventricular volume was calculated by the multislice tomographic Simpson's method. True LV and RV cast volumes were determined by water displacement. Results Both calculated LV and RV volumes correlated highly with the corresponding true volumes (all r>0.95, P<0.01). But with slice thickness from 2.0 mm to 10.0 mm, MSCT scanning overestimated the corresponding true volume by (3.21±5.95) ml to (12.58±8.56) ml for LV and (10.22±8.45) ml to (23.91±12.24) ml for RV (all P<0.01). There was a very high correlation between the overestimation and the selected slice thickness for both LV and RV volume measurements (r=0.998 and 0.996, P<0.01, respectively). However, when slice thickness was reduced to 5.0 mm, the overestimation for both LV and RV volume measurements became nonsignificant for slice thickness from 2.0 mm to 5.0 mm. Conclusions Both LV and RV volumes can be accurately estimated by MSCT. Thinner slice has more accurate calculated volume. However, 5.0 mm slice thickness is thin enough for an accurate measurement of LV or RV volume.

  12. The pathophysiologic aspects and clinical implications of electrocardiographic parameters of ventricular conduction delay in repaired tetralogy of Fallot.

    Science.gov (United States)

    Udink ten Cate, Floris E A; Sreeram, Narayanswami; Brockmeier, Konrad

    2014-01-01

    The 12-lead surface electrocardiogram is a valuable and feasible clinical tool in the management of patients following tetralogy of Fallot (TOF) repair. The importance of QRS duration in TOF patients has long been acknowledged. A prolonged QRS complex has been associated with increased risk for subsequent life-threatening ventricular arrhythmia and sudden cardiac death. Our current ability to risk-stratify TOF patients for malignant arrhythmogenic events primarily on the basis of QRS duration is rather limited. Nevertheless, increasing evidence suggests that QRS morphology and duration may be useful as surrogate markers of infundibular and regional right ventricular myocardial disease. The aim of this review is to provide a critical appraisal of the clinical implications of established and new electrocardiographic markers of ventricular conduction delay in TOF patients following surgical correction with a particular focus on QRS duration, lengthening, and fragmentation. In addition, the pathophysiological background of these parameters is addressed.

  13. Right ventricular volumes and function in thalassemia major patients in the absence of myocardial iron overload

    Directory of Open Access Journals (Sweden)

    Porter John B

    2010-04-01

    Full Text Available Abstract Aim We aimed to define reference ranges for right ventricular (RV volumes, ejection fraction (EF in thalassemia major patients (TM without myocardial iron overload. Methods and results RV volumes, EF and mass were measured in 80 TM patients who had no myocardial iron overload (myocardial T2* > 20 ms by cardiovascular magnetic resonance. All patients were receiving deferoxamine chelation and none had evidence of pulmonary hypertension or other cardiovascular comorbidity. Forty age and sex matched healthy non-anemic volunteers acted as controls. The mean RV EF was higher in TM patients than controls (males 66.2 ± 4.1% vs 61.6 ± 6%, p = 0.0009; females 66.3 ± 5.1% vs 62.6 ± 6.4%, p = 0.017, which yielded a raised lower threshold of normality for RV EF in TM patients (males 58.0% vs 50.0% and females 56.4% vs 50.1%. RV end-diastolic volume index was higher in male TM patients (mean 98.1 ± 17.3 mL vs 88.4 ± 11.2 mL/m2, p = 0.027, with a higher upper limit (132 vs 110 mL/m2 but this difference was of borderline significance for females (mean 86.5 ± 13.6 mL vs 80.3 ± 12.8 mL/m2, p = 0.09, with upper limit of 113 vs 105 mL/m2. The cardiac index was raised in TM patients (males 4.8 ± 1.0 L/min vs 3.4 ± 0.7 L/min, p Conclusion The normal ranges for functional RV parameters in TM patients with no evidence of myocardial iron overload differ from healthy non-anemic controls. The new reference RV ranges are important for determining the functional effects of myocardial iron overload in TM patients.

  14. Implantation of Lumenless Pacing Leads at the Inter-atrial Septum and Right Ventricular Outflow Tract with Deflectable Catheter-sheath

    Institute of Scientific and Technical Information of China (English)

    Rong BAI; Ruth KAM; Chi Keong CHING; Li Fern HSU; Wee Siong TEO

    2008-01-01

    Current permanent fight ventricular and fight atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asyno chronous electrical activation pattern, which results in asynchronous ventricular contraction and re-laxation. When pacing from right atrial appendage, the conduction time between two atria will be prolonged, which results in heterogeneity for both depolarization and repolarization. Six patients with Class Ⅰ indication for permanent pacing were implanted with either single chamber or dual chamber pacemaker. The SelectSecure 3830 4-French (Fr) lumenless lead and the SelectSite C304 8.5-Fr steerable catheter-sheath (Medtronic Inc., USA) were used. Pre-selected pacing sites included inter-atrial septum and right ventricular outflow tract, which were defined by ECG and fluoroscopic criteria. All the implanting procedures were successful without complication. Testing results (mean atrial pacing threshold: 0.87 V; mean P wave amplitude: 2.28 mV; mean ventricular pacing threshold:0.53V; mean R wave amplitude: 8.75 mV) were satisfactory. It is concluded that implantation of a 4-Fr lumenless pacing lead by using a streerable catheter-sheath to achieve inter-atrial septum or right ventricular outflow tract pacing is safe and feasible.

  15. Evaluation of right ventricular function by NuSMUGA software : gated blood-pool SPECT vs. first-pass radionuclide angiography

    NARCIS (Netherlands)

    Slart, RHJA; Poot, L; Piers, DA; van Veldhuisen, DJ; Jager, PL

    2003-01-01

    Background: In comparison with planar imaging gated blood-pool single photon emission computed tomography (GBPS) has the advantage of separating left and right ventricle. The purpose of this investigation was to evaluate the right ventricular ejection fraction (RVEF) calculations by GBPS software ('

  16. Screening system for drug-induced arrhythmogenic risk combining a patch clamp and heart simulator

    Science.gov (United States)

    Okada, Jun-ichi; Yoshinaga, Takashi; Kurokawa, Junko; Washio, Takumi; Furukawa, Tetsushi; Sawada, Kohei; Sugiura, Seiryo; Hisada, Toshiaki

    2015-01-01

    To save time and cost for drug discovery, a paradigm shift in cardiotoxicity testing is required. We introduce a novel screening system for drug-induced arrhythmogenic risk that combines in vitro pharmacological assays and a multiscale heart simulator. For 12 drugs reported to have varying cardiotoxicity risks, dose-inhibition curves were determined for six ion channels using automated patch clamp systems. By manipulating the channel models implemented in a heart simulator consisting of more than 20 million myocyte models, we simulated a standard electrocardiogram (ECG) under various doses of drugs. When the drug concentrations were increased from therapeutic levels, each drug induced a concentration-dependent characteristic type of ventricular arrhythmia, whereas no arrhythmias were observed at any dose with drugs known to be safe. We have shown that our system combining in vitro and in silico technologies can predict drug-induced arrhythmogenic risk reliably and efficiently. PMID:26601174

  17. The detailed assessment of left and right ventricular functions by tissue Doppler imaging in patients with familial Mediterranean fever.

    Science.gov (United States)

    Tavil, Yusuf; Ureten, Kemal; Oztürk, Mehmet Akif; Sen, Nihat; Kaya, Mehmet Güngör; Cemri, Mustafa; Cengel, Atiye

    2008-02-01

    In the contrary to other rheumatologic disorders, there have been limited numbers of studies investigating the cardiac involvement in patients with familial Mediterranean fever (FMF), although the disease may carry a potential for cardiovascular disorders because of sustained inflammation during its course. In the present study, we used high usefulness tissue Doppler echocardiography for detailed analysis of cardiac changes in FMF patients. The study population included 30 patients with FMF (11 men, 19 women; mean age, 35 +/- 7 years, mean disease duration, 15.4 +/- 7.6 years) and 30 healthy subjects as controls (12 men, 18 women; mean age, 33 +/- 7 years). The diagnosis of FMF was established according to the Tell-Hashomer criteria. Left and right ventricular functions were measured using echocardiography comprising standard two-dimensional, M-mode, and conventional Doppler as well as tissue Doppler imaging. The conventional echocardiographic paratemeters were similar apart from left ventricular relaxation time was longer (107 +/- 25 vs 85 +/- 10 ms, p < 0.001, respectively) in patients with FMF. According to the tissue Doppler measurements, while systolic velocities of both ventricles were not different, diastolic filling velocities of left ventricle including E'(m) (12.6 +/- 3.4 vs 14.7 +/- 3.3 cm/s, p = 0.04), A'(m) (10.1 +/- 2.6 vs 8.6 +/- 2.0 cm/s, p = 0.015), and E'(m)/ A'(m) (1.24 +/- 0.4 vs 1.71 +/- 0.5 cm/s, p = 0.012) values were statistically different between the groups. Left ventricular myocardial performance indices and right ventricular diastolic functions were found similar between two groups. In addition, there were no significant correlations between the disease duration, clinical features, and echocardiographic parameters. In conclusion, we have demonstrated that although systolic functions were comparable in the patients and controls, left ventricular diastolic function indices were impaired in FMF patients by using tissue Doppler analysis.

  18. Ectodermal dysplasia

    Directory of Open Access Journals (Sweden)

    Sonia Saggoo

    2009-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.

  19. Effect of eight weeks of endurance exercise training on right and left ventricular volume and mass in untrained obese subjects: a longitudinal MRI study.

    Science.gov (United States)

    Vogelsang, T W; Hanel, B; Kristoffersen, U S; Petersen, C L; Mehlsen, J; Holmquist, N; Larsson, B; Kjaer, A

    2008-06-01

    The aim of the present investigation was to examine how 8 weeks of intense endurance training influenced right and left ventricular volumes and mass in obese untrained subjects. Ten overweight subjects (19-47 years; body mass index of 34+/-5 kg/m(2)) underwent intensive endurance training (rowing) three times 30 min/week for 8 weeks at a relative intensity of 72+/-8% of their maximal heart rate response (mean+/-SD). Before and after 8 weeks of endurance training, the left and the right end-diastolic volume (EDV), end-systolic volume (ESV), ejection fraction (EF), stroke volume (SV) and ventricular mass (VM) were measured by Magnetic resonance imaging (MRI). Submaximal heart rate decreased from 126+/-5 to 113+/-3 b.p.m. (10%; Pright side of the heart showed significant changes in SV, EDV and VM with increase of 4%, 4% and 12%, respectively (Pright ventricular SV, due to an increase in left ventricular EDV and right ventricular EDV. Furthermore, left VM and right VM increased. We conclude that using MRI and a longitudinal design it was possible to demonstrate similar and balanced changes in the right and left ventricle in response to training.

  20. Non-ECG-gated CT pulmonary angiography and the prediction of right ventricular dysfunction in patients suspected of pulmonary embolism

    DEFF Research Database (Denmark)

    Gutte, Henrik; Mortensen, Jann; Mørk, Mette Louise;

    2016-01-01

    PURPOSE: Right ventricular dysfunction (RVD) is an important prognostic factor of 30-day mortality in patients with acute pulmonary embolism (PE). The aim of our study was to evaluate whether non-electrocardiogram (ECG)-gated cardiovascular parameters attained during computed tomography pulmonary...... angiography (CTPA) could predict RVD in patients suspected of PE using ECG-gated cardiac CT angiography as reference. METHODS: Consecutive patients suspected of PE were referred to a ventilation/perfusion single-photon emission tomography (V/Q-SPECT) as first-line imaging procedure. Patients had a V....../Q-SPECT/CT, a CTPA and an ECG-gated cardiac CT angiography performed the same day. RESULTS: A total of 71 patients were available for analysis. Seventeen patients (24%) had RVD. The non-ECG-gated dimensions of left and right ventricle and the major vessels were correlated with ECG-gated cardiac dimensions. The size...

  1. Isolated Pulmonary Infective Endocarditis with Septic Pulmonary Embolism Complicating a Right Ventricular Outflow Tract Obstruction: Scarce and Devious Presentation

    Directory of Open Access Journals (Sweden)

    Abdelrahmen Abdelbar

    2013-01-01

    Full Text Available We present a case of a fifty-three-year-old male who presented with severe sepsis. He had been treated as a pneumonia patient for five months before the admission. Investigations revealed isolated pulmonary valve endocarditis and septic pulmonary embolism in addition to undiagnosed right ventricular outflow tract (RVOT obstruction. The patient underwent surgery for the relief of RVOT obstruction by substantial muscle resection of the RVOT, pulmonary artery embolectomy, pulmonary valve replacement, and reconstruction of RVOT and main pulmonary artery with two separate bovine pericardial patches. He was discharged from our hospital after 6 weeks of intravenous antibiotics. He recovered well on follow-up 16 weeks after discharge. A high-suspicion index is needed to diagnose right-side heart endocarditis. Blood cultures and transesophageal echocardiogram are the key diagnostic tools.

  2. Effect of eight weeks of endurance exercise training on right and left ventricular volume and mass in untrained obese subjects: a longitudinal MRI study

    DEFF Research Database (Denmark)

    Vogelsang, T W; Hanel, B; Kristoffersen, U S

    2008-01-01

    The aim of the present investigation was to examine how 8 weeks of intense endurance training influenced right and left ventricular volumes and mass in obese untrained subjects. Ten overweight subjects (19-47 years; body mass index of 34+/-5 kg/m(2)) underwent intensive endurance training (rowing......) and ventricular mass (VM) were measured by Magnetic resonance imaging (MRI). Submaximal heart rate decreased from 126+/-5 to 113+/-3 b.p.m. (10%; P...

  3. Early right ventricular fibrosis and reduction in biventricular cardiac reserve in the dystrophin-deficient mdx heart.

    Science.gov (United States)

    Meyers, Tatyana A; Townsend, DeWayne

    2015-02-15

    Duchenne muscular dystrophy (DMD) is a progressive disease of striated muscle deterioration. Respiratory and cardiac muscle dysfunction are particularly clinically relevant because they result in the leading causes of death in DMD patients. Despite the clinical and physiological significance of these systems, little has been done to understand the cardiorespiratory interaction in DMD. We show here that prior to the onset of global cardiac dysfunction, dystrophin-deficient mdx mice have increased cardiac fibrosis with the right ventricle being particularly affected. Using a novel biventricular cardiac catheterization technique coupled with cardiac stress testing, we demonstrate that both the right and left ventricles have significant reductions in both systolic and diastolic function in response to dobutamine. Unstimulated cardiac function is relatively normal except for a significant reduction in the ventricular pressure transient duration compared with controls. These biventricular analyses also reveal the absence of a dobutamine-induced increase in isovolumic relaxation in the right ventricle of control hearts. Simultaneous assessment of biventricular pressure demonstrates a dobutamine-dependent enhancement of coupling between the ventricles in control mice, which is absent in mdx mice. Furthermore, studies probing the passive-extension properties of the left ventricle demonstrate that the mdx heart is significantly more compliant compared with age-matched C57BL/10 hearts, which have an age-dependent stiffening that is completely absent from dystrophic hearts. These new results indicate that right ventricular fibrosis is an early indicator of the development of dystrophic cardiomyopathy, suggesting a mechanism by which respiratory insufficiency may accelerate the development of heart failure in DMD.

  4. Right Ventricular Tissue Doppler Assessment in Space During Circulating Volume Modification using the Braslet-M Device

    Science.gov (United States)

    Hamilton, D. R.; Sargsyan, A. E.; Fincke, E. M.; Magnus, S. H.; Lonchakov, Y. V.; Alferova, I. V.; Dulchavsky, S. A.; Ebert, D.; Garcia, K.; Martin, D.; Matveev, V. P.; Voronkov, Y. I.; Melton, S. L.; Duncan, J. M.; Bogomolov, V. V.

    2009-01-01

    This joint U.S. - Russian work aims to establish a methodology for assessing cardiac function in microgravity in association with manipulation of central circulating volume. Russian Braslet-M occlusion cuffs were used to temporarily increase the volume of blood in the lower extremities, which effectively reduces the volume returning to the heart in the central circulation. A novel methodology was tested on the International Space Station (ISS) to assess the volume status of crewmembers by evaluating the responses to application and release of the Braslet-on-occlusion cuffs, as well as to modified Valsalva and Mueller maneuvers. Baseline echocardiographic tissue Doppler imaging (TDI) of the right ventricular free wall with no Braslet applied shows early diastolic E' (16 cm/sec), late diastolic A' (14 cm/sec), and systolic (12 cm/sec) velocities compatible with normal subjects on Earth. TDI of the RV free wall with Braslet applied shows that early diastolic E' decreased by 50% (8 cm/sec), late diastolic A' increased by 45%, and systolic S' remains unchanged. TDI of the RV free wall approximately 8 beats after the Braslet was released shows early diastolic E' (8 cm/sec), late diastolic A' (12 cm/sec), and systolic S' (13 cm/sec) velocities. During this portion of the release, early diastolic E' did not recover to baseline values but late diastolic A' and systolic S' recovered to pre-Braslet values. The pre-systolic cross-sectional area of the internal jugular vein with Braslet off was 1.07 cm(sup 2) and 1.13 cm(sup 2) 10 min after the Braslet was applied. The presystolic cross-sectional area of the common femoral vein with Braslet off was 0.50 cm(sup 2), and was 0.54 cm(sup 2) 10 min after the Braslet was applied. The right ventricular myocardial performance Tei index also was calculated for comparison with typical values found in healthy subjects on Earth. Baseline and Braslet-on values for Tei index were 0.25 and 0.22 respectively. Braslet Tei indices are within

  5. Predictors of right ventricular function as measured by tricuspid annular plane systolic excursion in heart failure

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Iversen, Kasper K; Akkan, Dilek

    2009-01-01

    motion index scores, atrio-ventricular annular plane systolic excursion of the mitral annulus were significantly related to TAPSE. Septal and posterior mitral annular plane systolic excursion (beta = 0.56, p ... in heart failure patients, in particular with reduced septal longitudinal motion. TAPSE is decreased in patients with heart failure of ischemic etiology. However, the absolute reduction in TAPSE is small and seems to be of minor importance in the clinical utilization of TAPSE whether applied as a measure...

  6. Thallium myocardial perfusion scans for the assessment of right ventricular hypertrophy in patients with cystic fibrosis. A comparison with other noninvasive techniques

    Energy Technology Data Exchange (ETDEWEB)

    Newth, C.J.; Corey, M.L.; Fowler, R.S.; Gilday, D.L.; Gross, D.; Mitchell, I.

    1981-01-01

    The incidence of right ventricular hypertrophy in 32 patients with cystic fibrosis was studied using thallium 201 (TI-201) myocardial perfusion scans, and compared with other noninvasive techniques including electrocardiography, vectorcardiography, and M-mode echocardiography. The patients (mean age, 17.3 yr; range, 7 to 33) had a wide range of clinical and pulmonary abnormalities (mean Shwachman-Kulczycki score, 66.6). In the total study group, TI-201 scans, like the vectorcardiograms and the M-mode echocardiograms, gave a surprisingly high proportion of positive predictions for right ventricular hypertrophy (RVH) (44%). The correlations with all other noninvasive methods were uniformly poor, so caution must be exercised in using this technique to predict early RVH in order to follow the natural history of cor pulmonale in cystic fibrosis. At the time of the study, 6 patients had clinical evidence of right ventricular failure, and in this disease setting must have had RVH. In 3 patients, RVH was confirmed at autopsy, and it was successfully predicted by TI-201 scans in 5 of the 6 patients. The false negative scan may have been due to regional myocardial ischemia secondary to severe right ventricular failure. In contrast, the vectorcardiogram, using Fowler's new criteria, made a successful prediction of RVH in all 6 patients, and the electro cardiogram in only 3. Although the M-mode echocardiogram was abnormal in all patients, it would have predicted RVH (with increased right ventricular anterior wall thickness) in only 1 patient. We concluded that TI-201 myocardial perfusion cans are good at confirming RVH in cases with established right ventricular failure, but have no advantage over vectorcardiographic assessments, which are logistically easier to perform and carry no radiation risks.

  7. Prognostic value of echocardiographic right/left ventricular end-diastolic diameter ratio in idiopathic pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    ZENG Wei-jie; SUN Yun-juan; XIONG Chang-ming; GU Qing; HE Jian-guo

    2011-01-01

    Background An echocardiographic right/left ventricular end-diastolic diameter ratio (RV/LV ratio) ≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. Right ventricular dilation is a common characteristic of both acute pulmonary embolism and idiopathic pulmonary arterial hypertension (IPAH).However, the prognostic value of the RV/LV ratio in patients with IPAH is unknown.Methods Ninety-five consecutive patients with newly diagnosed IPAH were included, 17 were re-evaluated by echocardiography after 3-12 months of targeted therapy. Follow-up data were obtained by telephone interviews and review of the patients' records.Results Higher RV/LV ratios were associated with greater functional impairment and with a higher mean right atrial pressure.The RV/LV ratio was positively correlated with pulmonary vascular resistance (r=0.549, P <0.001) and plasma N-terminal pro-brain natriuretic peptide level (r=0.575, P <0.001), but negatively correlated with cardiac output (r= -0.517, P <0.001) and mixed venous oxygen saturation (r= -0.599, P <0.001). During a follow-up period of (21 ± 15) months, 27 patients died.Sensitivity and specificity of an RV/LV ratio ≥0.84 for predicting death were 85.2% and 51.5%, respectively. The RV/LV ratio and body mass index were independent predictors of death by multivariate Cox analysis (P <0.01). A baseline RV/LV ratio ≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis (P <0.01).Conclusion The RV/LV ratio helps to assess the severity of IPAH and may serve as an independent predictor of prognosis in patients with IPAH.

  8. Early teatment with hepatocyte growth factor improves pulmonary artery and right ventricular remodeling in rats with pulmonary artery hypertension by modulating cytokines expression

    Institute of Scientific and Technical Information of China (English)

    王晓林

    2014-01-01

    Objective To investigate the effect of early treatment with hepatocyte growth factor(HGF)on the cytokine expression and pulmonary artery,right ventricular(RV)remodeling in the rat model of pulmonary artery hypertension(PAH).Methods The rat model of PAH was produced by injecting monocrotaline,and the model rats were randomly divided into empty adenovirus transfection group(MCT group,n=10)and HGF gene transfection group(HGF group,n=10).Another group of rats served as the Sham operation group(Sham group n=10).After 4 weeks of HGF gene transfection,the histological sections of the lungs and right ventricular(RV)

  9. The incremental effect of obstructive sleep apnea syndrome on right and left ventricular myocardial performance in newly diagnosed essential hypertensive subjects.

    Science.gov (United States)

    Gao, Jing; Hua, Qi; Li, Jing; Wang, Cai-Rong

    2009-03-01

    Obstructive sleep apnea syndrome (OSAS) may predispose patients to congestive heart failure, suggesting a deleterious effect of OSAS on myocardial contractility. We investigated whether essential hypertensive individuals with OSAS are characterized by decreased right and left ventricular myocardial performance. Our study population consisted of 45 consecutive patients with newly diagnosed untreated stage I-II essential hypertension suffering from OSAS (35 men, aged 49+/-8 years) and 48 hypertensives without OSAS, matched for age, sex, level of blood pressure, heart rate, body mass index and smoking status. All subjects underwent polysomnography and echocardiography. Right and left ventricular functions were evaluated using the myocardial performance index (MPI). Right and left ventricular functions were altered in hypertensives with OSAS. The mean right MPI was 0.26+/-0.11 in hypertensives without OSAS and 0.51+/-0.16 in hypertensives with OSAS (PRight and left MPI correlated positively and significantly with apnea-hypopnea index (rho=0.40, P=0.002).OSAS is associated with impaired right and left ventricular function. These phenomena were independent of hypertension.

  10. Prevalence, Diagnosis, Perioperative Monitoring and Treatment of Right Ventricular Dysfunction and/or Pulmonary Arterial Hypertension in Cardiac Surgical Patients in Germany-A Postal Survey.

    Science.gov (United States)

    Heringlake, Matthias; Schön, Julika; Pliet, Teresa; Haake, Nils; Reinecke, Alexander; Habicher, Marit; Sander, Michael; Markewitz, Andreas; Reuter, Daniel A; Groesdonk, Heinrich Volker; Trummer, Georg; Pilarzyk, Kevin; von der Brelie, Michael; Bein, Berthold; Schirmer, Uwe

    2016-02-24

    Background Sparse data are available on the prevalence of right ventricular dysfunction and/or pulmonary arterial hypertension in patients scheduled for cardiac surgery in Germany as well as on the intensity and modalities used for diagnosis, perioperative monitoring, and treatment of these comorbidities. Methods A postal survey including questions on the prevalence of preoperative right ventricular dysfunction and/or pulmonary arterial hypertension in patients undergoing cardiac surgery in 2009 was sent to 81 German heart centers. Total 47 of 81 (58%) heart centers returned the questionnaires. The centers reported data on 51,095 patients, and 49.8% of the procedures were isolated coronary artery bypass grafting. Results Data on the prevalence of preoperative pulmonary hypertension and/or right ventricular dysfunction were not available in 54% and 64.6% of centers. In the remaining hospitals, 19.5% of patients presented right heart dysfunction and 10% pulmonary arterial hypertension. Preoperative echocardiography was performed in only 45.3% of the coronary artery bypass grafting cases. Preoperative pharmacologic treatment of pulmonary hypertension or right ventricular dysfunction with oral sildenafil, inhaled prostanoids, or nitric oxide was initiated in 71% and 95.7% of the centers, respectively. Intra- and postoperative treatment was most frequently accomplished with phosphodiesterase-III inhibitors. Conclusion The prevalence of preoperative right heart dysfunction and pulmonary arterial hypertension in cardiac surgical patients in Germany seems to be substantial. However, in more than 50% of the patients, no preoperative data on right ventricular function and pulmonary arterial pressure are available. This may lead to underestimation of perioperative risk and inappropriate management of this high-risk population.

  11. Phenotyping of left and right ventricular function in mouse models of compensated hypertrophy and heart failure with cardiac MRI.

    Directory of Open Access Journals (Sweden)

    Bastiaan J van Nierop

    Full Text Available BACKGROUND: Left ventricular (LV and right ventricular (RV function have an important impact on symptom occurrence, disease progression and exercise tolerance in pressure overload-induced heart failure, but particularly RV functional changes are not well described in the relevant aortic banding mouse model. Therefore, we quantified time-dependent alterations in the ventricular morphology and function in two models of hypertrophy and heart failure and we studied the relationship between RV and LV function during the transition from hypertrophy to heart failure. METHODS: MRI was used to quantify RV and LV function and morphology in healthy (n = 4 and sham operated (n = 3 C57BL/6 mice, and animals with a mild (n = 5 and a severe aortic constriction (n = 10. RESULTS: Mice subjected to a mild constriction showed increased LV mass (P0.05. Animals with a severe constriction progressively developed LV hypertrophy (P<0.001, depressed LVEF (P<0.001, followed by a declining RVEF (P<0.001 and the development of pulmonary remodeling, as compared to controls during a 10-week follow-up. Myocardial strain, as a measure for local cardiac function, decreased in mice with a severe constriction compared to controls (P<0.05. CONCLUSIONS: Relevant changes in mouse RV and LV function following an aortic constriction could be quantified using MRI. The well-controlled models described here open opportunities to assess the added value of new MRI techniques for the diagnosis of heart failure and to study the impact of new therapeutic strategies on disease progression and symptom occurrence.

  12. Primary primitive neuroectodermal tumor: An unusual cause of right ventricular intracavitary obstruction in a child

    Directory of Open Access Journals (Sweden)

    Thachil Ajit

    2008-01-01

    Full Text Available A six-year-old boy presented with a brief history suggestive of right heart failure. Investigations revealed a mass filling almost the entire right ventricle. Palliative resection of the mass was done. The operative specimen revealed a primary primitive neuroectodermal tumor of the heart, the first of its kind reported in the pediatric age group .

  13. Pre-implant right ventricular function might be an important predictor of the response to cardiac resynchronization therapy

    Directory of Open Access Journals (Sweden)

    Ring Margareta

    2011-10-01

    Full Text Available Abstract Objective Cardiac resynchronization therapy is proven efficacious in patients with heart failure (HF. Presence of biventricular HF is associated with a worse prognosis than having only left ventricular (LV HF and pacing might deteriorate heart function. The aim of the study was to assess a possible significance of right ventricular (RV pre-implant systolic function to predict response to CRT. Design We studied 22 HF-patients aged 72 ± 11 years, QRS-duration 155 ± 20 ms and with an LV ejection fraction (EF of 26 ± 6% before and four weeks after receiving a CRT-device. Results There were no changes in LV diameters or end systolic volume (ESV during the study. However, end diastolic volume (EDV decreased from 226 ± 71 to 211 ± 64 ml (p = 0.02 and systolic maximal velocities (SMV increased from 2.2 ± 0.4 to 2.6 ± 0.9 cm/s (p = 0.04. Pre-implant RV-SMV (6.2 ± 2.6 cm/s predicted postoperative increase in LV contractility, p = 0.032. Conclusions Pre-implant decreased RV systolic function might be an important way to predict a poor response to CRT implicating that other treatments should be considered. Furthermore we found that 3D- echocardiography and Tissue Doppler Imaging were feasible to detect short-term changes in LV function.

  14. Ventricular septal defect (image)

    Science.gov (United States)

    Ventricular septal defect is a congenital defect of the heart, that occurs as an abnormal opening in ... wall that separates the right and left ventricles. Ventricular septal defect may also be associated with other ...

  15. Usefulness of myocardial performance index for assessing right ventricular function after percutaneous closure of atrial septal defect

    Institute of Scientific and Technical Information of China (English)

    Jingdong Ding; Genshan Ma; Yaoyao Huang; Xiaoli Zhang; Jian Zhu; Rong Yang; Fengxiang Lu

    2007-01-01

    Objective Assessment of right ventricular function in patients with atrial septal defect (ASD) is difficult. The Doppler myocardial performance index (MPI) may provide a method of assessing function in these patients. The purposes of this study were to evaluate the right ventricular function and its changes in patients with ASD after transcatheter closure of ASD. Methods MPI, defined as the sum of isovolumic relaxation time and isovolumic contraction time derived by ejection time, was measured from tricuspid inflow and right ventricular outflow; Doppler velocity profiles recorded during routine echocardiography. Twenty nine patients ( 13 men, 16 women;mean age 25.28 ± 12.69, range 6 to 57 years) were diagnosed to secundum ASD [the stretched diameters of ASD were from 9 To 36 (24.91 ±7.98) mm], and had a successfully placed Amplatzer septal occluder (ASO) (the sizes of ASO were from 11 to 40 mm ); there were 81 sex-matched, age-matched healthy people (control group 41men, 40 women; mean age 29.02 ± 14.22, range 4 to 45 years).MPI was measured again on 3 days and 1 month after closure of ASD. Change in the study group was assessed and compared to the control subjects with structurally normal hearts. A complete 2- dimensional and Doppler echocardiographic examination was performed in all study groups. Results 1) The isovolumic relaxation and isovolumic contraction times [respectively(77.59 ± 14.39)ms vs (60.93 ± 12.94)ms, P<0.0001; (28.28 ± 10.88)ms vs (23.64 ± 9.01)ms, P=0.027] were prolonged, and ejection time [(260.65 ± 21.86 )ms vs (271.85 ± 21.92)ms, P=0.033] was shortened in patients with ASD compared with that in control subjects, resulting in a marked increase in the MPI(0.40 ± 0.07 vs 0.31 ± 0.05, P<0.0001) from normal values; 2) by Pearson's correlations, the MPI had no correlation with heart rate and blood pressure in control subjects and patients with ASD, but it correlated positively with age in patients with ASD; 3) by Pearson

  16. Association of Right Ventricular Pressure and Volume Overload with Non-Ischemic Septal Fibrosis on Cardiac Magnetic Resonance.

    Directory of Open Access Journals (Sweden)

    Jiwon Kim

    Full Text Available Non-ischemic fibrosis (NIF on cardiac magnetic resonance (CMR has been linked to poor prognosis, but its association with adverse right ventricular (RV remodeling is unknown. This study examined a broad cohort of patients with RV dysfunction, so as to identify relationships between NIF and RV remodeling indices, including RV pressure load, volume and wall stress.The population comprised patients with RV dysfunction (EF 6-fold more common in the highest, vs. the lowest, common tertile of PASP and RV size (p<0.001.Among wall stress components, NIF was independently associated with RV chamber dilation and afterload, supporting the concept that NIF is linked to adverse RV chamber remodeling.

  17. Haemostasis with fibrin glue injection into the pericardial space for right ventricular perforation caused by an iatrogenic procedural complication.

    Science.gov (United States)

    Arai, Hirofumi; Miyamoto, Takamichi; Hara, Nobuhiro; Obayashi, Tohru

    2016-05-17

    An 89-year-old woman with severe aortic valve stenosis and bradycardia presented with circulatory shock due to cardiac tamponade. We performed pericardiocentesis, and then diagnosed right ventricular perforation by echocardiography with microcavitation contrast medium just before inserting a drainage tube. We then inserted the drainage tube in the appropriate position and withdrew blood-filled fluid. The patient was haemodynamically stabilised, but haemorrhage from the perforation site continued for a few days. We injected fibrin glue into the pericardial space through the drainage tube and achieved haemostasis. Thus, we avoided surgery to close the perforation in this high-risk patient. There was no recurrence of haemorrhage. She subsequently had elective aortic valve replacement at another hospital. No adhesions in the pericardial space were seen during surgery.

  18. Right ventricular dysfunction following continuous flow left ventriccular assist device placement in 51 patients: predicators and outcomes

    Directory of Open Access Journals (Sweden)

    Neragi-Miandoab Siyamek

    2012-06-01

    Full Text Available Abstract Background Right ventricular (RV dysfunction following implantation of a left ventricular assist device (LVAD is a serious condition and is associated with increased mortality. Methods The aim of the study is to investigate the significance of pre-existing RV dysfunction, tricuspid valve (TV insufficiency, and the severity of septal deviation following LVAD implantation on RV dysfunction, as well as the outcome and short-term complications in 51 patients from June 2006 to August 2010. Student t test was used to compare the data and estimate the p value. Results Mean age was 55.1 ± 13, with a male to female ratio of 3.25. The 30-day mortality was 13.7% (7/51 patients, and the overall mortality was 23.5% (12/51 patients. Meanwhile, 21 patients (21/51; 41.2% have undergone orthotopic heart transplantation. The mean time of support was 314.5±235 days with a median of 240 days at the time of closing this study. Echocardiographic evaluation of RV function pre- and post-implantation of an LVAD demonstrated septal deviation towards the left ventricle in immediate postoperative phase, which correlated with acute RV dysfunction (p = 0.002. Preoperative RV dysfunction was a significant predictor of postoperative right heart dysfunction following implantation of an LVAD (p = 0.001. Conclusion Preoperative RV dysfunction is a predictor of RV failure in LVAD patients. The adjustment of septal deviation through gradual increase of the LVAD flow can prevent the acute RV dysfunction following LVAD placement.

  19. Proximal pulmonary arterial obstruction decreases the time constant of the pulmonary circulation and increases right ventricular afterload.

    Science.gov (United States)

    Pagnamenta, Alberto; Vanderpool, Rebecca; Brimioulle, Serge; Naeije, Robert

    2013-06-01

    The time constant of the pulmonary circulation, or product of pulmonary vascular resistance (PVR) and compliance (Ca), called the RC-time, has been reported to remain constant over a wide range of pressures, etiologies of pulmonary hypertension, and treatments. We wondered if increased wave reflection on proximal pulmonary vascular obstruction, like in operable chronic thromboembolic pulmonary hypertension, might also decrease the RC-time and thereby increase pulse pressure and right ventricular afterload. Pulmonary hypertension of variable severity was induced either by proximal obstruction (pulmonary arterial ensnarement) or distal obstruction (microembolism) eight anesthetized dogs. Pulmonary arterial pressures (Ppa) were measured with high-fidelity micromanometer-tipped catheters, and pulmonary flow with transonic technology. Pulmonary ensnarement increased mean Ppa, PVR, and characteristic impedance, decreased Ca and the RC-time (from 0.46 ± 0.07 to 0.30 ± 0.03 s), and increased the oscillatory component of hydraulic load (Wosc/Wtot) from 25 ± 2 to 29 ± 2%. Pulmonary microembolism increased mean Ppa and PVR, with no significant change in Ca and characteristic impedance, increased RC-time from 0.53 ± 0.09 to 0.74 ± 0.05 s, and decreased Wosc/Wtot from 26 ± 2 to 13 ± 2%. Pulse pressure increased more after pulmonary ensnarement than after microembolism. Concomitant measurements with fluid-filled catheters showed the same functional differences between the two types of pulmonary hypertension, with, however, an underestimation of Wosc. We conclude that pulmonary hypertension caused by proximal vs. distal obstruction is associated with a decreased RC-time and increased pulsatile component of right ventricular hydraulic load.

  20. Clinical characteristics, left and right ventricular ejection fraction, and long-term prognosis in patients with non-insulin-dependent diabetes surviving an acute myocardial infarction

    DEFF Research Database (Denmark)

    Melchior, T; Gadsbøll, N; Hildebrandt, P;

    1996-01-01

    (with diabetes: median 46% vs without diabetes: median 43%; p = 0.89). Median right ventricular ejection fraction (62%) was within normal limits in both groups and did not differ statistically. Survival data were obtained for all patients. The 5-year mortality was increased in patients with diabetes...

  1. Semi-quantitative assessment of right ventricular function in comparison to a 3D volumetric approach: A cardiovascular magnetic resonance study

    NARCIS (Netherlands)

    Nijveldt, Robin; Germans, Tjeerd; McCann, Gerald; Beek, Aernout; Rossum, van Albert

    2008-01-01

    Right ventricular (RV) volume measurements with cardiovascular magnetic resonance (CMR) is considered the gold standard, but acquisition and analysis remain timeconsuming. The aim of our study was therefore to investigate the accuracy and performance of a semi-quantitative assessment of RV function

  2. Arytmogen højre ventrikel kardiomyopati

    DEFF Research Database (Denmark)

    Benn, Marianne; Schiønning, J D; Baandrup, Ulrik

    1998-01-01

    Arrhythmogenic right ventricular dysplasia is a rare cardiomyopathy, but a frequent cause of ventricular tachyarrhythmia and sudden cardiac death among young otherwise healthy individuals. This article contains a review of the current knowledge on epidemiology, diagnosis, symptoms and signs as we...... as theories on etiology and pathogenesis, prognosis and treatment. The aim is to draw attention to the disease as a cause of syncope, ventricular tachycardia and sudden cardiac death....

  3. Cardiac CT Angiography in Congestive Heart Failure.

    Science.gov (United States)

    Levine, Avi; Hecht, Harvey S

    2015-06-01

    Cardiac CT angiography has become an important tool for the diagnosis and treatment of congestive heart failure. Differentiation of ischemic from nonischemic cardiomyopathy; evaluation of myocardial perfusion; characterization of hypertrophic cardiomyopathy, left ventricular noncompaction, and arrhythmogenic right ventricular dysplasia; and delineation of congenital heart defects and valvular abnormalities are the primary diagnostic applications. Therapeutic use includes visualization of the coronary venous anatomy for optimal implementation of cardiac resynchronization therapy and evaluation of left ventricular assist devices and transplant vasculopathy.

  4. Acquired Aorto-Right Ventricular Fistula following Transcatheter Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Muhammad Tariq Shakoor

    2015-01-01

    Full Text Available Transcatheter aortic valve replacement (TAVR techniques are rapidly evolving, and results of published trials suggest that TAVR is emerging as the standard of care in certain patient subsets and a viable alternative to surgery in others. As TAVR is a relatively new procedure and continues to gain its acceptance, rare procedural complications will continue to appear. Our case is about an 89-year-old male with extensive past medical history who presented with progressive exertional dyspnea and angina secondary to severe aortic stenosis. Patient got TAVR and his postoperative course was complicated by complete heart block, aorto-RV fistula, and ventricular septal defect (VSD formation as a complication of TAVR. To the best of our knowledge, this is the third reported case of aorto-RV fistula following TAVR as a procedural complication but the first one to show three complications all together in one patient.

  5. Evaluation of Longitudinal Right Ventricular Mechanical Dyssynchrony before and Early after Cardiac Resynchronization Therapy: A Strain Imaging Study

    Directory of Open Access Journals (Sweden)

    Mozhgan Parsaee

    2011-02-01

    Full Text Available Background: The right ventricular (RV dyssynchrony has not been studied extensively and the existing literature has established the effect of cardiac resynchronization therapy (CRT on the left ventricular (LV dyssynchrony, but there is a dearth of data on the effect of CRT on the forgotten ventricle. We sought to evaluate the presence of mechanical right ventricular dyssynchrony in patients with systolic heart failure, selected for CRT, and track the changes early afterward utilizing the longitudinal strain analysis.Methods: Thirty-six patients with severe left ventricular systolic dysfunction, candidated for CRT, were enrolled in this study. Mechanical dyssynchrony was assessed using tissue Doppler echocardiography. The time interval between the onset of the QRS to the peak systolic longitudinal strain at the RV free wall and the septum was obtained. The RV mechanical delay was calculated as the absolute value of the difference in the time-to-peak measurements between the RV and septum. The RV dyssynchrony was defined as the calculated delay in strain imaging, which was ± 2 SD above the mean value for the control subjects (20 cases. The RV function was evaluated using the RV fractional area change (RVFAC, tricuspid annulus plane systolic excursion (TAPSE, and peak systolic strain values of the RV free wall. Four to 7 days after CRT implantation, echocardiographic reevaluations were done.Results: The calculated cut-off value for the RV dyssynchrony was 41.5 msec, according to which the pre-CRT analysis specified two patient groups: Group 1 (16 cases with RV dyssynchrony and Group 2 (20 patients without RV dyssynchrony. Significant improvement in the RV dyssynchrony was noted in Group 1 after CRT (30 ± 28.9 msec vs. 68.8 ± 21 msec; p value < 0.01 vs. 14 ± 10 msec vs. 19 ± 16.5 msec; p value = 0.18 respectively. A significant correlation was found between the severity of the RV dyssynchrony and peak systolic strain in the RV free wall (r = -0

  6. 右心室不同位点起搏对QRS时限的影响%Effect of different right ventricular pacing sites on QRS complex duration

    Institute of Scientific and Technical Information of China (English)

    任晓庆; 唐闽; 方丕华; 王方正; 张澍

    2013-01-01

    Objective To investigate the effect of right ventricular different pacing site on QRS complex width,and provide a reference for lead location.Methods Two hundred and sixteen patients[mean age,(69± 13) years] scheduled for a permanent pacemaker implantation,which met the class Ⅰ or Ⅱ a pacemaker installed indications,were enrolled for the study.Pacing and electrocardiography recording were underwent at right ventricular apex,right ventricular inflow tract (RVIT),mid septum,high septum and right ventricular outflow tract (RVOT),respectively.Then the duration and morphology of QRS complex paced in different right ventricular locations were analysed and compared.Results QRS durations at all different right ventricular pacing sites were significantly lengthened compared with baseline electrocardiography (P<0.001).QRS duration with right ventricular apex pacing was most broad (168± 16)ms,QRS duration with RVIT pacing was (166± 15) ms,and QRS duration with RVOT pacing was (165± 15)ins.There was no significant difference in QRS duration with apex,RVIT and RVOT (P> 0.05).QRS duration at septum pacing was significantly narrower (P<0.001),QRS morphology and electrical axis was normal compared with other right ventricular pacing sites.QRS duration with mid-septum pacing was most narrow (139±19) ms,and shorter than that with high septum (153± 14) ms.Conclusions QRS duration at mid-septum pacing was most narrow,QRS morphology and electrical axis was normal compared with any other right ventricular pacing sites.Mid-septum may be the best choice for right ventricular pacing site.%目的 观察右心室不同起搏位点的QRS时限,为临床导线放置提供参考依据.方法 选择216例符合Ⅰ类或Ⅱa类起搏器植入适应证的患者,在放置心室导线过程中,分别在右心室心尖部、流入道、中位间隔部、高位间隔部和流出道等不同位点起搏右心室,记录起搏时的体表心电图,比较不同位点QRS

  7. Right and left ventricular cardiac function in a developed world population with human immunodeficiency virus studied with radionuclide ventriculography

    DEFF Research Database (Denmark)

    Lebech, Anne-Mette; Gerstoft, Jan; Hesse, Birger

    2004-01-01

    BACKGROUND: Cardiac dysfunction has been reported in a substantial part of patients infected with the human immunodeficiency virus (HIV). However, most studies are from a time before the introduction of highly active antiretroviral treatment (HAART), which has significantly reduced HIV-associated......BACKGROUND: Cardiac dysfunction has been reported in a substantial part of patients infected with the human immunodeficiency virus (HIV). However, most studies are from a time before the introduction of highly active antiretroviral treatment (HAART), which has significantly reduced HIV......-associated morbidity and mortality rates. Accordingly, the prevalence of HIV-associated cardiac dysfunction may also have changed. The aim of the study was to establish the prevalence of right- and left-sided cardiac dysfunction in a Danish HIV population, most of whom were undergoing HAART, with radionuclide...... ventricular ejection fraction and 6 (7%) had a reduced right ventricle ejection fraction (0.35-0.42) compared with reference values from the age- and sex-matched reference population. Patients with HIV and reduced cardiac function did not differ in the duration of HIV, CD4 count, CD4 nadir, or HIV RNA load...

  8. Right ventricular function assessed by tissue Doppler echocardiography in older subjects without evidence for structural cardiac disease.

    Science.gov (United States)

    Laszlo, Roman; Baumann, Tobias; Konz, Hanna; Dallmeier, Dhayana; Klenk, Jochen; Denkinger, Michael; Koenig, Wolfgang; Rothenbacher, Dietrich; Steinacker, Juergen Michael

    2016-05-31

    The aim of our study was to obtain right ventricular (RV) tissue Doppler imaging (TDI) data in older subjects (n = 95, mean age: 74.5 ± 4.6 years) without evidence of hemodynamically significant structural heart disease recruited from a large population-based cohort (ActiFE-Ulm study). Our data indicate that aging may be accompanied by decreasing RV diastolic function and at most little alterations of RV systolic function. Mean values of all parameters were still within the guideline-suggested reference range with most of them closer to the abnormality thresholds. On an individual basis, respective thresholds were also exceeded in some subjects (almost all parameters subjects, presumed pathological measures might still be compatible with physiological age-related alterations. Therefore, they always have to be interpreted across the clinical context and in relation to other parameters of morphology and function obtained by other ultrasound imaging techniques (M-mode, B-mode, etc.) in the context of echocardiographic evaluation of the right heart.

  9. Radial left ventricular dyssynchrony by speckle tracking in apical versus non apical right ventricular pacing- evidence of dyssynchrony on medium term follow up

    Directory of Open Access Journals (Sweden)

    Dinesh Choudhary

    2016-03-01

    Conclusion: Pacing in non apical location (RV mid septum or low RVOT is associated with less dyssynchrony by specific measures like 2D radial strain and correlates with better ventricular function in long term.

  10. Tissue Doppler assessment of right ventricular function in female patients with limited form of systemic sclerosis

    Directory of Open Access Journals (Sweden)

    Ayman Ahmed Abdelaziz

    2013-09-01

    Conclusions: Patients with limited form of SSc present with pulsed-tissue Doppler imaging indices indicative of right ventricle dysfunction, which had significant correlations with disease duration. Tissue Doppler is a valuable non-invasive tool for detecting RV myocardial involvement in patients with limited SSc.

  11. Quantitative right and left ventricular functional analysis during gated whole-chest MDCT: A feasibility study comparing automatic segmentation to semi-manual contouring

    Energy Technology Data Exchange (ETDEWEB)

    Coche, Emmanuel, E-mail: Emmanuel.coche@uclouvain.b [Department of Medical Imaging, Universite Catholique de Louvain, Cliniques Universitaires St-Luc (UCL), Avenue Hippocrate, 10, 1200 Brussels (Belgium); Walker, Matthew J. [Philips Healthcare, CT Clinical Science, Cleveland, OH (United States); Zech, Francis [Department of Internal Medicine, Universite Catholique de Louvain, Cliniques Universitaires St-Luc, Brussels (Belgium); Crombrugghe, Rodolphe de [Department of Medical Imaging, Universite Catholique de Louvain, Cliniques Universitaires St-Luc (UCL), Avenue Hippocrate, 10, 1200 Brussels (Belgium); Vlassenbroek, Alain [Philips Healthcare, Brussels (Belgium)

    2010-06-15

    Purpose: To evaluate the feasibility of an automatic, whole-heart segmentation algorithm for measuring global heart function from gated, whole-chest MDCT images. Material and methods: 15 patients with suspicion of PE underwent whole-chest contrast-enhanced MDCT with retrospective ECG synchronization. Two observers computed right and left ventricular functional indices using a semi-manual and an automatic whole-heart segmentation algorithm. The two techniques were compared using Bland-Altman analysis and paired Student's t-test. Measurement reproducibility was calculated using intraclass correlation coefficient. Results: Ventricular analysis with automatic segmentation was successful in 13/15 (86%) and in 15/15 (100%) patients for the right ventricle and left ventricle, respectively. Reproducibility of measurements for both ventricles was perfect (ICC: 1.00) and very good for automatic and semi-manual measurements, respectively. Ventricular volumes and functional indices except right ventricular ejection fraction obtained from the automatic method were significantly higher for the RV compared to the semi-manual methods. Conclusions: The automatic, whole-heart segmentation algorithm enabled highly reproducible global heart function to be rapidly obtained in patients undergoing gated whole-chest MDCT for assessment of acute chest pain with suspicion of pulmonary embolism.

  12. Computational modelling of left-ventricular diastolic mechanics: effect of fibre orientation and right-ventricle topology.

    Science.gov (United States)

    Palit, Arnab; Bhudia, Sunil K; Arvanitis, Theodoros N; Turley, Glen A; Williams, Mark A

    2015-02-26

    Majority of heart failure patients who suffer from diastolic dysfunction retain normal systolic pump action. The dysfunction remodels the myocardial fibre structure of left-ventricle (LV), changing its regular diastolic behaviour. Existing LV diastolic models ignored the effects of right-ventricular (RV) deformation, resulting in inaccurate strain analysis of LV wall during diastole. This paper, for the first time, proposes a numerical approach to investigate the effect of fibre-angle distribution and RV deformation on LV diastolic mechanics. A finite element modelling of LV passive inflation was carried out, using structure-based orthotropic constitutive law. Rule-based fibre architecture was assigned on a bi-ventricular (BV) geometry constructed from non-invasive imaging of human heart. The effect of RV deformation on LV diastolic mechanics was investigated by comparing the results predicted by BV and single LV model constructed from the same image data. Results indicated an important influence of RV deformation which led to additional LV passive inflation and increase of average fibre and sheet stress-strain in LV wall during diastole. Sensitivity of LV passive mechanics to the changes in the fibre distribution was also examined. The study revealed that LV diastolic volume increased when fibres were aligned more towards LV longitudinal axis. Changes in fibre angle distribution significantly altered fibre stress-strain distribution of LV wall. The simulation results strongly suggest that patient-specific fibre structure and RV deformation play very important roles in LV diastolic mechanics and should be accounted for in computational modelling for improved understanding of the LV mechanics under normal and pathological conditions.

  13. Extent of late gadolinium enhancement at right ventricular insertion points in patients with hypertrophic cardiomyopathy: relation with diastolic dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Zhu, Yinsu [Seoul National University Hospital, Department of Radiology, Seoul (Korea, Republic of); The First Affiliated Hospital of Nanjing Medical University, Department of Radiology, Nanjing, Jiangsu (China); Park, Eun-Ah; Lee, Whal; Chu, Ajung; Chung, Jin Wook; Park, Jae Hyung [Seoul National University Hospital, Department of Radiology, Seoul (Korea, Republic of); Kim, Hyung-Kwan [Seoul National University Hospital, Division of Cardiology, Department of Internal Medicine, Seoul (Korea, Republic of)

    2015-04-01

    Our aim was to examine the association between the extent of late gadolinium enhancement (LGE) at right ventricular insertion points (RVIP) and left ventricular (LV) functional parameters in patients with hypertrophic cardiomyopathy (HCM). Sixty-one HCM patients underwent echocardiography and cardiovascular magnetic resonance (CMR) within one week. Mitral annular velocities (E/E') were obtained from echocardiography; LV ejection fraction (EF), LV mass index, LV wall maximal thickness, and left atrial volume index (LAVI) were obtained from MR. LGE extent was quantified (proportion of total LV myocardial mass) according to location: % RVIP-LGE and % non-RVIP-LGE. Although LGE was commonly present in both apical (74 %) and non-apical HCMs (88 %) (p = 0.163), RVIP-LGE was more frequent (86 % vs. 47 %, p = 0.002) in non-apical HCMs in which E/E' was significantly higher (19.23 ± 8.40 vs. 13.13 ± 5.06, p = 0.009). In addition, RVIP-LGE extent was associated with LV diastolic dysfunction (r = 0.45, p < 0.001 for E/E'; r = 0.53, p < 0.001 for LAVI) and lower LVEF (r = -0.42, p = 0.001). There was no correlation between non-RVIP-LGE extent and other parameters. Multiple linear regression analysis revealed RVIP-LGE extent as an independent predictor of E/E' (β = 0.45, p < 0.001) and LAVI in HCM patients (β = 0.53, p < 0.001). The extent of LGE at RVIPs in HCM patients is associated with increased estimated LV filling pressure and chronic diastolic burden. (orig.)

  14. The Early Predictive Value of Right Ventricular Strain in Epirubicin-Induced Cardiotoxicity in Patients with Breast Cancer

    Science.gov (United States)

    Chang, Wei-Ting; Shih, Jhih-Yuan; Feng, Yin-Hsun; Chiang, Chun-Yen; Kuo, Yu Hsuan; Chen, Wei-Yu; Wu, Hong-Chang; Cheng, Juei-Tang; Wang, Jhi-Joung; Chen, Zhih-Cherng

    2016-01-01

    Background As cancer therapies have improved, patient life spans have been extended but quality of life has been threatened by chemotherapy induced cardiotoxicity. Most cardiac complications remain unobserved until specific symptoms develop. Speckle-tracking echocardiography is a sensitive imaging modality in detecting early occult myocardial dysfunction. Methods A total number of 35 patients newly diagnosed with breast cancer and preparing for epirubicin therapy were prospectively recruited. Echocardiography, including speckle-tracking echocardiography, was performed sequentially at baseline (T1), after the first cycle (T2) and after the third cycle (T3) of epirubicin. At each visit, the severity of dyspnea was evaluated by the assessment scale. Results Compared with the baseline, right ventricular longitudinal strain (RVLS_FW) at T2 significantly declined (-22.49 ± 4.97 vs. -18.48 ± 4.46, p = 0.001), which was also positively associated with the development of dyspnea (R2 = 0.8, p = 0.01). At T3, both the left ventricular global longitudinal strain and RVLS_FW were significantly impaired (-21.4 ± 4.12 vs. -16.94 ± 6.81%; -22.49 ± 4.97 vs. -16.86 ± 7.27%, p = 0.01; 0.001, respectively). Also, the accumulating dose of epirubicin positively correlated with the development of dyspnea (R2 = 0.38, p = 0.04) and the decline of RVLS_FW (R2 = 0.53, p = 0.02). Notably, compared with the other echocardiographic parameters only RVLS_FW at the early stage (T2) significantly correlated with the development of dyspnea (odds ratio: 1.84, 95% confidence interval: 1.22-2.78, p = 0.04). Conclusions RVLS_FW sensitively predicts dyspnea development in breast cancer patients receiving epirubicin therapy. However, larger scale studies are required to validate its role in long-term patient survival. PMID:27713603

  15. Paced QRS duration and myocardial scar amount: predictors of long-term outcome of right ventricular apical pacing.

    Science.gov (United States)

    Lee, Seung-Ah; Cha, Myung-Jin; Cho, Youngjin; Oh, Il-Young; Choi, Eue-Keun; Oh, Seil

    2016-07-01

    Long-term right ventricular apical pacing (RVAP) is reportedly associated with heart failure (HF) development. However, the predictors of pacing-induced HF (PHF) remained unclear. We retrospectively enrolled 234 patients without structural heart disease who underwent a permanent pacemaker implantation with RVAP between 1982 and 2004. RVAP-induced HF was defined as left ventricular ejection fraction decrease >5 % with HF symptom without other HF development etiology. The QRS duration of a paced beat (pQRSd) and myocardial scar score were analyzed from each patient's 12-lead ECG. During a mean 15.6 years (range 3.3-30.0 years), 48 patients (20.5 %) patients developed RVAP-induced HF. The PHF group patients had a longer pQRSd (192.4 ± 13.5 vs. 175.7 ± 14.7 ms in non-PHF patients, p scar score (5.2 ± 1.9 vs. 2.7 ± 1.9, respectively p scar score (HR 1.23, 95 % CI 1.03-1.49, p = 0.037), and a higher percentage of ventricular pacing (HR 1.31, 95 % CI 1.01-1.49, p = 0.010) were independent predictors of PHF. Based on the results of the receiver-operating characteristic (ROC) curve, the pQRSd cutoff was 185 ms (AUC 0.79, sensitivity 66.7 %, specificity 76.3 %) and myocardial scar score cutoff value was 4 (AUC 0.81, sensitivity 81.3 %, specificity 66.1 %). The pQRSd was positively correlated with scar score (r = 0.70, p scar score ≥4 might be independent long-term prognostic markers of PHF.

  16. Clinical and prognostic relevance of echocardiographic evaluation of right ventricular geometry in patients with idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Ghio, Stefano; Pazzano, Anna Sara; Klersy, Catherine; Scelsi, Laura; Raineri, Claudia; Camporotondo, Rita; D'Armini, Andrea; Visconti, Luigi Oltrona

    2011-02-15

    The aim of the present study was to assess the clinical and prognostic significance of right ventricular (RV) dilation and RV hypertrophy at echocardiography in patients with idiopathic pulmonary arterial hypertension. Echocardiography and right heart catheterization were performed in 72 consecutive patients with idiopathic pulmonary arterial hypertension admitted to our institution. The median follow-up period was 38 months. The patients were grouped according to the median value of RV wall thickness (6.6 mm) and the median value of the RV diameter (36.5 mm). On multivariate analysis, the mean pulmonary artery pressure (p = 0.018) was the only independent predictor of RV wall thickness, and age (p = 0.011) and moderate to severe tricuspid regurgitation (p = 0.027) were the independent predictors of RV diameter. During follow-up, 22 patients died. The death rate was greater in the patients with a RV diameter >36.5 mm than in patients with a RV diameter ≤36.5 mm: 15.9 (95% confidence interval 9.4 to 26.8) vs 6.6 (95% confidence interval 3.3 to 13.2) events per 100-person years (p = 0.0442). In contrast, the death rate was similar in patients with RV wall thickness above or below the median value. However, among the patients with a RV wall thickness >6.6 mm, a RV diameter >36 mm was not associated with a poorer prognosis (p = 0.6837). In conclusion, in patients with idiopathic pulmonary arterial hypertension, a larger RV diameter is a marker of a poor prognosis but a greater RV wall thickness reduces the risk of death associated with a dilated right ventricle.

  17. Right Ventricular Compression Mimicking Brugada-Like Electrocardiogram in a Patient with Recurrent Pectus Excavatum

    Science.gov (United States)

    Ahn, Jinhee; Shim, Jaemin; Lee, Sung Ho; Kim, Young-Hoon

    2017-01-01

    Pectus excavatum (PE), the most common skeletal anomaly of chest wall, sometimes requires a surgical correction but recurrent PE is not uncommon. PE usually has a benign course; however, this chest deformity may be associated with symptomatic tachyarrhythmias due to mechanical compression. We report a case of a patient with recurrent PE after surgical correction presenting with palpitation and electrocardiogram (ECG) showing ST-segment elevation on the right precordial leads, which could be mistaken for a Brugada syndrome (BrS).

  18. Role of hybrid operating room in surgery for the right atrial thrombus, pulmonary thrombi, and ventricular septal rupture after myocardial infarction

    Science.gov (United States)

    Singh, Ajmer; Mehta, Yatin; Parakh, Rajiv; Kohli, Vijay; Trehan, Naresh

    2016-01-01

    Free-floating right heart thrombi are uncommon and need emergency treatment in view of their tendency to dislodge and cause pulmonary embolism. We report a successful surgical management of a patient who had large mobile right atrial thrombus, bilateral pulmonary thrombi, coronary artery disease, and postmyocardial infarction ventricular septal rupture (VSR). The patient underwent coronary angiography, inferior vena cava filter placement, removal of thrombi from the right atrium and pulmonary arteries, repair of VSR, and coronary artery bypass graft surgery in a hybrid operating room. PMID:27716704

  19. The ratio of right ventricular volume to left ventricular volume reflects the impact of pulmonary regurgitation independently of the method of pulmonary regurgitation quantification

    Energy Technology Data Exchange (ETDEWEB)

    Śpiewak, Mateusz, E-mail: mspiewak@ikard.pl [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw (Poland); Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Małek, Łukasz A., E-mail: lmalek@ikard.pl [Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw (Poland); Petryka, Joanna, E-mail: joannapetryka@hotmail.com [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw (Poland); Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Mazurkiewicz, Łukasz, E-mail: lmazurkiewicz@ikard.pl [Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Cardiomyopathy, Institute of Cardiology, Warsaw (Poland); Miłosz, Barbara, E-mail: barbara-milosz@o2.pl [Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Radiology, Institute of Cardiology, Warsaw (Poland); Biernacka, Elżbieta K., E-mail: kbiernacka@ikard.pl [Department of Congenital Heart Diseases, Institute of Cardiology, Warsaw (Poland); Kowalski, Mirosław, E-mail: mkowalski@ikard.pl [Department of Congenital Heart Diseases, Institute of Cardiology, Warsaw (Poland); Hoffman, Piotr, E-mail: phoffman@ikard.pl [Department of Congenital Heart Diseases, Institute of Cardiology, Warsaw (Poland); Demkow, Marcin, E-mail: mdemkow@ikard.pl [Department of Coronary Artery Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw (Poland); Miśko, Jolanta, E-mail: jmisko@wp.pl [Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw (Poland); Department of Radiology, Institute of Cardiology, Warsaw (Poland); Rużyłło, Witold, E-mail: wruzyllo@ikard.pl [Institute of Cardiology, Warsaw (Poland)

    2012-10-15

    Background: Previous studies have advocated quantifying pulmonary regurgitation (PR) by using PR volume (PRV) instead of commonly used PR fraction (PRF). However, physicians are not familiar with the use of PRV in clinical practice. The ratio of right ventricle (RV) volume to left ventricle volume (RV/LV) may better reflect the impact of PR on the heart than RV end-diastolic volume (RVEDV) alone. We aimed to compare the impact of PRV and PRF on RV size expressed as either the RV/LV ratio or RVEDV (mL/m{sup 2}). Methods: Consecutive patients with repaired tetralogy of Fallot were included (n = 53). PRV, PRF and ventricular volumes were measured with the use of cardiac magnetic resonance. Results: RVEDV was more closely correlated with PRV when compared with PRF (r = 0.686, p < 0.0001, and r = 0.430, p = 0.0014, respectively). On the other hand, both PRV and PRF showed a good correlation with the RV/LV ratio (r = 0.691, p < 0.0001, and r = 0.685, p < 0.0001, respectively). Receiver operating characteristic analysis showed that both measures of PR had similar ability to predict severe RV dilatation when the RV/LV ratio-based criterion was used, namely the RV/LV ratio > 2.0 [area under the curve (AUC){sub PRV} = 0.770 vs AUC{sub PRF} = 0.777, p = 0.86]. Conversely, with the use of the RVEDV-based criterion (>170 mL/m{sup 2}), PRV proved to be superior over PRF (AUC{sub PRV} = 0.770 vs AUC{sub PRF} = 0.656, p = 0.0028]. Conclusions: PRV and PRF have similar significance as measures of PR when the RV/LV ratio is used instead of RVEDV. The RV/LV ratio is a universal marker of RV dilatation independent of the method of PR quantification applied (PRF vs PRV)

  20. Evaluation of right ventricular systolic function in patients with right ventricular myocardial infarction by ultrasound tissue tracking and strain rate imaging%组织追踪和应变率显像技术评价右室心肌梗死患者右室收缩功能

    Institute of Scientific and Technical Information of China (English)

    卢永昭; 谭深; 闫丽娟; 赵君智; 张景峰; 麦兴盛; 左鹏飞; 张耀仁

    2011-01-01

    Objective To explore the clinical application value of ultrasound tissue tracking( TTI) and strain rate imaging( SRI) in diagnosis of right ventricular myocardial infarction and evaluating right ventricular systolic function. Methods Twenty patients with acute right ventricular myocardial infarction and 24 healthy controls were enrolled in this study, Their right ventricular apical two chamber view and apical four chamher view were examined by TTI and SRI. The peak systolic displacement( PDS) of right ventricular anterior, inferior wall, lateral wall and the maximum systolic strain rate ( SSR) were detected. Results The PDS and SSR in patients with right ventricular myocardial infarction were lower than those in control group (P <0. 05 ) , and the diagnostic sensitivity of SSR was higher than PDS( P <0. 05 ) . Conclusion TTI and SRI can accurately diagnose right ventricular myocardial infarction,and can quantitatively evaluate the right ventricular systolic function.%目的 探讨超声组织追踪(TTI)和应变率显像(SRI)技术在诊断右室壁心肌梗死和评价右室局部收缩功能中的临床应用价值.方法 采用TTI和SRI技术对20例急性右室梗死患者和24例健康对照者的右室心尖两腔心切面和四腔心切面进行扫查,检测分析右室前壁、下壁、侧壁收缩期峰值位移值(PDS)、收缩期最大应变率(SSR).结果 右室心肌梗死患者的PDS和SSR均较对照组降低(P<0.05),而SSR较PDS显示结果更敏感(P<0.01).结论 TTI和SRI技术能够准确地诊断右室壁心肌梗死,并能对右室局部收缩功能进行定量评价.

  1. Echocardiographic assessment of global longitudinal right ventricular function in patients with an acute inferior ST elevation myocardial infarction and proximal right coronary artery occlusion.

    Science.gov (United States)

    Hutyra, Martin; Skála, Tomáš; Horák, David; Köcher, Martin; Tüdös, Zbyněk; Zapletalová, Jana; Přeček, Jan; Louis, Albert; Smékal, Aleš; Táborský, Miloš

    2015-03-01

    Right ventricular (RV) myocardial infarction (MI) is a frequent concomitant of an acute inferior MI. We set out to determine the diagnostic value of speckle tracking echocardiography in comparison with cardiac magnetic resonance (CMR) for RV stunning and scar prediction. 55 patients (66 ± 11 years) with an acute inferior ST elevation MI who underwent percutaneous coronary intervention (PCI) of an occlusion in the proximal right coronary artery were prospectively enrolled. An echocardiography was done on the day of presentation and on the 5th day thereafter. A CMR was subsequently performed 1 month after the MI. The CMR was used to differentiate between the group with RV scar (n = 26) and without RV scar (n = 29). RV peak systolic longitudinal strain (RV-LS) at presentation determined RV scar (-21.1 ± 5.1% vs. -9.9 ± 4.6%, p -15.8% had a sensitivity of 92% and a specificity of 83% in RV scar prediction (AUC 0.93). RV-LS was superior to TAPSE and TDI in determining the presence of RV scar. According to RV-LS values at presentation and on the 5th day, 3 subgroups were defined: G1-normal deformation (RV-LS -20%, 5th day RV-LS -20%). In G1, there was neither RV scar nor clinically relevant hypotension. In G2, 58% of patients developed RV scar and 36% had hypotension. In the G3, 83% developed RV scar and 55% had hypotension. The myocardial deformation analysis could provide an early prediction of RV scar. It allowed the patients to be divided into subgroups with normal RV function, stunning and persistent RV dysfunction.

  2. Ablação de taquicardia ventricular idiopática com morfologia de bloqueio de ramo esquerdo localizada no tronco da artéria pulmonar Ablation of idiopathic ventricular tachycardia with left bundle-branch block morphology located in the pulmonary trunk

    Directory of Open Access Journals (Sweden)

    Luiz Roberto Leite

    2005-02-01

    Full Text Available Paciente de 26 anos, sem cardiopatia estrutural, apresentando palpitações e pré-síncopes devido à taquicardia ventricular não sustentada, foi submetida a estudo eletrofisiológico para tentativa de ablação do foco arritmogênico, usando-se como local, os critérios de mapeamento. Sem obter êxito com o mapeamento da via de saída do ventrículo direito, posicionou-se o cateter dentro da artéria pulmonar com mapeamento de foco satisfatório, eliminando a taquicardia tão logo iniciada a radiofreqüência. Durante seguimento de 14 meses, a paciente permanece assintomática, sem arritmia ao Holter e não nessecitando de drogas antiarrítmicas.We report the case of a 26-year-old female patient with palpitations and presyncopes due to nonsustained ventricular tachycardia, who had no structural heart disease. The patient underwent electrophysiological study in an attempt to ablate the arrhythmogenic focus, whose location was determined by using mapping criteria. Because mapping of the right ventricular outflow tract was not successful, the catheter was placed inside the pulmonary artery with satisfactory mapping of the arrhythmogenic focus, and tachycardia was eliminated as soon as radiofrequency was initiated. The patient has remained asymptomatic for 14 months, with no treatment with antiarrhythmic drugs, and no arrhythmias on serial 24-hour Holter.

  3. Comparison of right ventricular contractile abnormalities in hypertrophic cardiomyopathy versus hypertensive heart disease using two dimensional strain imaging: a cross-sectional study.

    Science.gov (United States)

    Afonso, Luis; Briasoulis, Alex; Mahajan, Nitin; Kondur, Ashok; Siddiqui, Fayez; Siddiqui, Sabeeh; Alesh, Issa; Cardozo, Shaun; Kottam, Anupama

    2015-12-01

    Hypertrophic cardiomyopathy (HCM) affects the right ventricle (RV) because of the anatomically hypertrophied septum and plausibly by extension of the myopathic process to the RV. We sought to investigate RV strain in patients with left ventricular hypertrophy secondary to either HCM or hypertension (H-LVH). Our cross-sectional study included 32 patients with HCM, 21 patients with H-LVH, and 11 healthy subjects, who were evaluated with transthoracic echocardiography. Using a dedicated software package, bi-dimensional acquisitions were analyzed to measure segmental longitudinal strain in apical views. Right ventricular global longitudinal strain (GLS) was calculated by averaging septal and right free wall strains. The HCM and H-LVH groups were comparable for age and demographic characteristics. Right ventricular tricuspid annular plane systolic excursion was not significantly different between HCM and H-LVH subjects. Moreover, RV GLS, septal and lateral RV myocardial strain were significantly impaired in patients with HCM (all p 14.9% differentiated HCM and H-LVH with a 90% sensitivity and a 95% specificity (p < 0.001). RV strain parameters are impaired in patients with HCM. Assessment of two-dimensional RV strain parameters could help differentiate between HCM and H-LVH.

  4. 超声心动图在右心功能评价中的应用%Echocardiography in Evaluation of Right Ventricular Function

    Institute of Scientific and Technical Information of China (English)

    彭玲

    2012-01-01

    右心功能不全或右心衰竭是一种复杂的临床综合征,常导致患者预后不良,病死率高.为早期发现右心功能障碍,及时指导治疗和评价治疗效果,对右心功能的监测越来越引起临床医生的关注.超声心动图具有无创、准确和重复性好等优点而广泛应用于心功能的评价.我们就如何运用超声心动图来简便有效、准确敏感地评价右心功能做一综述,为其能进一步推广应用于临床打下良好的基础.%Right ventricular dysfunction or right heart failure is a complex clinical syndrome and often leads to a poor prognosis and high mortality. In order to detect right ventricular dysfunction at an early stage, provide a therapy guidance and evaluate treatment outcomes, right ventricular function evaluation has aroused more and more concern in clinical physicians. With the advantages of being non-invasive, accuracy and repetitiveness, echocardiography is used extensively in the assessment of heart function. In this review, we focus on how to use echocardiography to evaluate right ventricular function easily, efficiently, accurately and sensitively, and provide a good foundation for its further clinical application.

  5. The Correlation between Left and Right Ventricular Ejection Fractions in Patients with Ischemic Heart Disease, Documented by Cardiac Magnetic Resonance Imaging

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    Ali Eshraghi

    2016-03-01

    Full Text Available Introduction: The correlation between right and left ventricular ejection fractions (RVEF and LVEF, respectively has been studied in only a small number of patients with a marked decrease in RVEF and LVEF. The aim of the present study was to compare LVEF and RVEF in patients with ischemic heart disease. RVEF and LVEF were measured by Cardiovascular Magnetic Resonance (CMR imaging. Materials and Methods: This observational study was done in Ghaem general hospital in 2014.  LVEF and RVEF were measured in a series of 33 patients with ischemic heart disease, undergoing CMR for the evaluation of myocardial viability. The correlation between RVEF and LVEF in patients with ischemic heart disease was studied, using Pearson product-moment correlation coefficient analysis.   This study was done in Ghaem general hospital in 2014 with simple sapling. Results: Right ventricular end diastolic volume (186.33±58.90 and left ventricular end diastolic volume (121.72±61.64 were significantly correlated (r=0.223, P=0.005. Moreover, there was a significant correlation between right ventricular end systolic volume (88.18±40.90 and left ventricular end systolic volume (140.96±35.33 (r=0.329, P=0.000. The most significant association was observed between RVEF and LVEF (r=0.913, P=0.000. Conclusion: Based on the findings, RVEF and LVEF were significantly correlated in patients with ischemic heart disease, although this association was not always present in all cardiac patients. The cause of this discrepancy is still unknown.

  6. Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension.

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    Courand, Pierre-Yves; Pina Jomir, Géraldine; Khouatra, Chahéra; Scheiber, Christian; Turquier, Ségolène; Glérant, Jean-Charles; Mastroianni, Bénédicte; Gentil, Béatrice; Blanchet-Legens, Anne-Sophie; Dib, Alfred; Derumeaux, Geneviève; Humbert, Marc; Mornex, Jean-François; Cordier, Jean-François; Cottin, Vincent

    2015-01-01

    Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy. In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78). After a median follow-up of 4.1 years, 41 deaths occurred, including 35 from cardiovascular causes. Patients with a (median) baseline RVEF >25% had better survival than those with a RVEF arterial hypertension.

  7. Right Ventricular Doppler Echocardiographic Study of Indeterminate Form of Chagas Disease

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    Rogério Gomes Furtado

    2015-03-01

    Full Text Available Background: Patients with indeterminate form of Chagas disease/cardiac normality (ICD/CN exhibited normal electrocardiograms and chest X-rays; however, more sophisticated tests detected some degree of morphological and functional changes in the heart. Objective: To assess the prevalence of systolic and diastolic dysfunction of the right ventricle (RV in patients with ICD/CN. Methods: This was a case–control and prevalence study. Using Doppler two-dimensional echocardiography (2D, 92 patients were assessed and divided into two groups: group I (normal, n = 31 and group II (ICD/CN, n = 61. Results: The prevalence of RV systolic dysfunction in patients in groups I and II was as follows: fractional area change (0.0% versus 0.6%, mobility of the tricuspid annulus (0.0% versus 0.0%, and S-wave tissue Doppler (6.4% versus 26.0%, p = 0.016. The prevalence of global disorders such as the right myocardial performance index using tissue Doppler (16.1% versus 27.8%, p = 0.099 and pulsed Doppler (61.3% versus 68%, p = 0.141 and diastolic disorders such as abnormal relaxation (0.0% versus 6.0%, pseudonormal pattern (0.0% versus 0.0%, and restrictive pattern (0.0% versus 0.0% was not statistically different between groups. Conclusion: The prevalence of RV systolic dysfunction was estimated to be 26% (S wave velocity compared with other variables, suggesting incipient changes in RV systolic function in the ICD/CN group.

  8. Relationship between lipidslevelsand right ventricular volume overload in congestive heart failure

    Institute of Scientific and Technical Information of China (English)

    Ying CHEN; Yu TIAN; Gang LIU; Zhen-Guo JI; Kun-Shen LIU; Chao LIU; Xiao-Mei HE; Hong MENG; Qing-Zhen ZHAO; Yu-Zhi ZHEN; Li TIAN; Le WANG; Li-Shuang JI; Guo-Ping MA

    2014-01-01

    BackgroundThe relationship between lipids and coronary artery disease has been well established. However, this is not the case between lipids and heart failure. Ironically, high lipid levels are associated with better outcomes in heart failure, but the mechan-isms underlying the phenomenon are not fully understood. This study was performed to test the hypothesis that reduced intestinal lipid absorption due to venous congestion may lead to low lipid levels.MethodsWe collected data of clinical characteristics, echocardio-graph, and lipid profile in 442 unselected patients with congestive heart failure. Correlations between lipid levels[including total cho-lesterol(TCL), high-density lipoprotein cholesterol(HDL-C), low-density lipoprotein cholesterol(LDL-C), and triglycerides(TG)]and right ventricle end diastolic diameter (RVEDD), left ventricle end diastolic diameter (LVEDD), right atrium diameter (RA), left atrium diameter (LA), or left ventricle ejection fraction (LVEF) were analyzed using Pearson correlation and partial correlation. RVEDD, LVEDD, RA, and LA were indexed to the body surface area.ResultsThere was a significantly inverse correlation between TCL le-vels and RVEDD (r=-0.34,P<0.001) and RA (r=-0.36,P<0.001). Other lipids such as LDL-C, HDL-C, and TG had asimilar inverse correlation with RVEDD and RA. All these correlations remained unchanged after adjusting for age, gender, smoking status, physical activity levels, comorbidities, and medication use.ConclusionsLipid levels were inversely correlated to RVEDD in patients with congestive heart failure; however, because this was an observational study, further investigation is needed to verify our results as wellas identify a causal relationship, if any.

  9. Right ventricular ejection fraction during exercise in normal subjects and in coronary artery disease patients: assessment by multiple-gated equilibrium scintigraphy

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    Maddahi, J.; Berman, D.S.; Matsuoka, D.T.; Waxman, A.D.; Forrester, J.S.; Swan, H.J.C.

    1980-07-01

    The response of right ventricular ejection fraction (RVEF) during exercise and its relationship to the location and extent of coronary artery disease are not fully understood. We have recently developed and validated a new method for scintigraphic evaluation of RVEF using rapid multiple-gated equilibrium scintigraphy and multiple right ventricular regions of interest. The technique has been applied during upright bicycle exercise in 10 normal subjects and 20 patients with coronary artery disease. Resting RVEF was not significantly different between the groups (0.49 +- 0.04 vs 0.47 +- 0.09, respectively, mean +- SD). In all 10 normal subjects RVEF rose (0.49 +- 0.04 to 0.66 +- 0.08, p < 0.01) at peak exercise. At peak exercise in coronary artery disease patients, the group RVEF remained unchanged (0.47 +- 0.09 to 0.50 +- 0.11, p = NS), but the individual responses varied. In the coronary artery disease patients, the relationship between RVEF response to exercise and exercise left ventricular function, septal motion and right coronary artery stenosis were studied. Significant statistical association was found only between exercise RVEF and right coronary artery stenosis. RVEF rose during exercise in seven of seven patients without right coronary artery stenosis (0.42 +- 0.06 to 0.58 +- 0.08, p = 0.001) and was unchanged or fell in 12 of 13 patients with right coronary artery stenosis (0.50 +- 0.09 to 0.45 +- 0.10, p = NS). We conclude that (1) in normal subjects RVEF increases during upright exercise and (2) although RVEF at rest is not necessarily affected by coronary artery disease, failure of RVEF to increase during exercise, in the absence of chronic obstructive pulmonary disease or valvular heart disease, may be related to the presence of significant right coronary artery stenosis.

  10. Plasma CCN2/connective tissue growth factor is associated with right ventricular dysfunction in patients with neuroendocrine tumors

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    Aakhus Svend

    2010-01-01

    Full Text Available Abstract Background Carcinoid heart disease, a known complication of neuroendocrine tumors, is characterized by right heart fibrotic lesions. Carcinoid heart disease has traditionally been defined by the degree of valvular involvement. Right ventricular (RV dysfunction due to mural involvement may also be a manifestation. Connective tissue growth factor (CCN2 is elevated in many fibrotic disorders. Its role in carcinoid heart disease is unknown. We sought to investigate the relationship between plasma CCN2 and valvular and mural involvement in carcinoid heart disease. Methods Echocardiography was performed in 69 patients with neuroendocrine tumors. RV function was assessed using tissue Doppler analysis of myocardial systolic strain. Plasma CCN2 was analyzed using an enzyme-linked immunosorbent assay. Mann-Whitney U, Kruskal-Wallis, Chi-squared and Fisher's exact tests were used to compare groups where appropriate. Linear regression was used to evaluate correlation. Results Mean strain was -21% ± 5. Thirty-three patients had reduced RV function (strain > -20%, mean -16% ± 3. Of these, 8 had no or minimal tricuspid and/or pulmonary regurgitation (TR/PR. Thirty-six patients had normal or mildly reduced RV function (strain ≤ -20%, mean -25% ± 3. There was a significant inverse correlation between RV function and plasma CCN2 levels (r = 0.47, p Conclusions Elevated plasma CCN2 levels are associated with RV dysfunction and valvular regurgitation in NET patients. CCN2 may play a role in neuroendocrine tumor-related cardiac fibrosis and may serve as a marker of its earliest stages.

  11. Comparison of right ventricular functions according to infarct localization using advanced echocardiographic methods in myocardial infarction with ST elevation

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    Mehmet Ata Akıl

    2012-12-01

    Full Text Available Objectives: In this study, we aimed to compare the effectsof infarct localization in patients with ST ElevatedMyocardial Infarction (STEMI on the right ventricular(RV functions by using advanced echocardiographicmethods.Materials and methods: A total of 89 patients withSTEMI were included into the study and patients weredivided to three groups as anterior, isolated-inferior andinferior+RV MI groups. In addition to standard echocardiographicmesurements, RV tissue doppler, RV EjectionFraction (RVEF, Myocardial performance index (MPIand TAPSE measurements of all patients were performedbetween 24-72 hours after the event.Results: Compared to groups, RV functions in inferior MIwith RV involvement group were deteriorated. Tricuspidannular plane systolic excursion (TAPSE value for theinferior MI with RV involvement (19±1mm group werelower than those for Inferior MI group without RV involvement(23±1mm and anterior MI (23±1mm (p<0.05. TheRV MPI value for inferior MI group with RV involvement(0.76±0.14 were found to be higher than those for anterior(0.64±0.1 and inferior MI (0.56±0.1 group withoutRV involvement (p<0.05. Peak Sm (r = -0.35, p =0.01, TAPSE (r = -0.47, p<0.001 and RV EF (r = -0.46,p<0.001 showed a negative correlation with RV MPI value.Furthermore, RV tricuspid E/A rate (r = -0.19, p = 0.7and RV free wall tissue doppler Em/Am rate (r = -0.26, p =0.01 displayed a negative correlation with RV MPI value.Conclusions: Use of advanced methods addition to theconventional echocardiographic methods in STEMI patients,could produce more valuable information to evaluateRV functions and provide a positive impact on treatmentstrategies.Key words: Acute myocardial infarction, right ventricle,echocardiography, TAPSE, MPI

  12. Right Ventricular Adaptation Is Associated with the Glu298Asp Variant of the NOS3 Gene in Elite Athletes.

    Science.gov (United States)

    Szelid, Zsolt; Lux, Árpád; Kolossváry, Márton; Tóth, Attila; Vágó, Hajnalka; Lendvai, Zsuzsanna; Kiss, Loretta; Maurovich-Horvat, Pál; Bagyura, Zsolt; Merkely, Béla

    2015-01-01

    Nitric oxide (NO), an important endogenous pulmonary vasodilator is synthetized by the endothelial NO synthase (NOS3). Reduced NO bioavailability and thus the Glu298Asp polymorphism of NOS3 may enhance right ventricular (RV) afterload and hypertrophic remodeling and influence athletic performance. To test this hypothesis world class level athletes (water polo players, kayakers, canoeists, rowers, swimmers, n = 126) with a VO2 maximum greater than 50ml/kg/min were compared with non-athletic volunteers (n = 155). Cardiopulmonary exercise tests and cardiac magnetic resonance imaging (cMRI) were performed to determine structural or functional changes. Genotype distribution of the NOS3 Glu298Asp polymorphism was not affected by gender or physical performance. Cardiac MRI showed increased stroke volume with eccentric hypertrophy in all athletes regardless of their genotype. However, the Asp allelic variant carriers had increased RV mass index (32±6g versus 27±6g, pgene function and NO bioavailability in sport related cardiac adaptation.

  13. Ventricular performance in congential left-to-right shunt: temporal Fourier analysis of gated blood-pool data

    Energy Technology Data Exchange (ETDEWEB)

    Takeda, K.

    1983-09-01

    Using global time-activity curves, the phase and amplitude at fundamental frequency were calculated, and emptying patterns of the right and left ventricles (RV, LV) were evaluated by phase difference and RV/LV amplitude ratio. In 21 subjects with normal cardiac function, D(phase) was minimal (mean 2.2 +- 6.1/sup 0/), regardless of heart rate, and R(amp) was distributed from 0.31 to 0.92 (mean 0.57 +- 0.20). In 19 patients of ventricular septal defect (VSD), R(amp) remained within the normal range, whereas D(phase) became larger in proportion to the ratio of pulmonary-to-systemic blood flow, Qp/Qs (p < 0.001). Especially, cases with Qp/Qs over 2.0 showed a significant RV phase lag. By contrast, nine patients with patent ductus arteriosus (PDA), showed no RV phase lag, but - particularly in cases with Qp/Qs > 2.0 - R(amp) was smaller than normal (p < 0.001). Thus this method is valuable for pathophysiologic investigation of diseases with L-to-R shunt, and can help in the noninvasive differential diagnosis between VSD and PDA.

  14. Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload

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    Gotsis Efstathios D

    2011-07-01

    Full Text Available Abstract Background Thalassaemia major (TM patients need regular blood transfusions that lead to accumulation of iron and death from heart failure. Deferiprone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular (LV function but little is known of their relative effects on the right ventricle (RV, which is being increasingly recognised as an important prognostic factor in cardiomyopathy. Therefore data from a prospective randomised controlled trial (RCT comparing these chelators was retrospectively analysed to assess the RV responses to these drugs. Methods In the RCT, 61 TM patients were randomised to receive either deferiprone or deferoxamine monotherapy, and CMR scans for T2* and cardiac function were obtained. Data were re-analysed for RV volumes and function at baseline, and after 6 and 12 months of treatment. Results From baseline to 12 months, deferiprone reduced RV end systolic volume (ESV from 37.7 to 34.2 mL (p = 0.008, whilst RV ejection fraction (EF increased from 69.6 to 72.2% (p = 0.001. This was associated with a 27% increase in T2* (p Conclusion In this retrospective analysis of a prospective RCT, deferiprone monotherapy was superior to deferoxamine for improvement in RVEF and end-systolic volume. This improvement in the RV volumes and function may contribute to the improved cardiac outcomes seen with deferiprone.

  15. Right Ventricular Adaptation Is Associated with the Glu298Asp Variant of the NOS3 Gene in Elite Athletes.

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    Zsolt Szelid

    Full Text Available Nitric oxide (NO, an important endogenous pulmonary vasodilator is synthetized by the endothelial NO synthase (NOS3. Reduced NO bioavailability and thus the Glu298Asp polymorphism of NOS3 may enhance right ventricular (RV afterload and hypertrophic remodeling and influence athletic performance. To test this hypothesis world class level athletes (water polo players, kayakers, canoeists, rowers, swimmers, n = 126 with a VO2 maximum greater than 50ml/kg/min were compared with non-athletic volunteers (n = 155. Cardiopulmonary exercise tests and cardiac magnetic resonance imaging (cMRI were performed to determine structural or functional changes. Genotype distribution of the NOS3 Glu298Asp polymorphism was not affected by gender or physical performance. Cardiac MRI showed increased stroke volume with eccentric hypertrophy in all athletes regardless of their genotype. However, the Asp allelic variant carriers had increased RV mass index (32±6g versus 27±6g, p<0.01 and larger RV stroke volume index (71±10ml versus 64±10ml, p<0.01 than athletes with a Glu/Glu genotype. Genotype was not significantly associated with athletic performance. In the non-athletic group no genotype related differences were detected. The association between the NOS3 Glu298Asp polymorphism and RV structure and dimension in elite athletes emphasizes the importance of NOS3 gene function and NO bioavailability in sport related cardiac adaptation.

  16. Early right ventricular systolic dysfunction in patients with systemic sclerosis without pulmonary hypertension: a Doppler Tissue and Speckle Tracking echocardiography study

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    Baumann Gert

    2010-01-01

    Full Text Available Abstract Background Isovolumetric acceleration (IVA is a novel tissue Doppler parameter for the assessment of systolic function. The aim of this study was to evaluate IVA as an early parameter for the detection of right ventricular (RV systolic dysfunction in patients with systemic sclerosis (SSc without pulmonary hypertension. Methods 22 patients and 22 gender- and age-matched healthy subjects underwent standard echocardiography with tissue Doppler imaging (TDI and speckle tracking strain to assess RV function. Results Tricuspid annular plane systolic excursion (TAPSE (23.2 ± 4.1 mm vs. 26.5 ± 2.9 mm, p 2 vs. 4.1 ± 0.8 m/s2, p Conclusion IVA is a useful tool with high-predictive power to detect early right ventricular systolic impairment in patients with SSc and without pulmonary hypertension.

  17. Right-To-Left Ventricular Differences in the Expression of Mitochondrial Hexokinase and Phosphorylation of Akt

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    Petra Waskova-Arnostova

    2013-01-01

    Full Text Available Background/Aims: Hexokinase (HK is a key glycolytic enzyme which promotes the maintenance of glucose homeostasis in cardiomyocytes. HK1 isoform is predominantly bound to the outer mitochondrial membrane and highly supports oxidative phosphorylation by increasing the availability of ADP for complex V of the respiratory chain. HK2 isoform is under physiological conditions predominantly localized in the cytosol and upon stimulation of PI3K/ Akt pathway associates with mitochondria and thus can prevent apoptosis. The purpose of this study was to investigate expression and subcellular localization of both HK isoforms in left (LV and right (RV heart ventricles of adult male Wistar rats. Methods: Real-Time RT-PCR, Western blotting, and quantitative immunofluorescence microscopy were used. Results: Our results showed a significantly higher expression of both HK1 and HK2 at mRNA and protein levels in the RV compared to the LV. These findings were corroborated by immunofluorescence staining which revealed substantially higher fluorescence signals of both HKs in the RV than in the LV. The ratios of phospho-Ser473-Akt/non-phospho-Akt and phospho-Thr308-Akt/non-phospho-Akt were also markedly higher in the RV than in the LV. Conclusion: These results suggest that the RV has a higher activity of aerobic glycolytic metabolism and may be able to respond faster and more powerfully to stressful stimuli than the LV.

  18. Evaluation of right ventricular function in adults with congenital heart defects.

    Science.gov (United States)

    Bussadori, Claudio; Di Salvo, Giovanni; Pluchinotta, Francesca R; Piazza, Luciane; Gaio, Giampiero; Russo, Maria Giovanna; Carminati, Mario

    2015-01-01

    The right ventricle (RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease (CHD). Despite major progress being made, precise measurements of the RV are challenging because of its peculiar anatomical structure that is not adaptable to any planar geometrical assumption. This is particularly true in adult patients with CHD where the RV shape eludes any standardization, it may assume various morphologies, and its modality of contraction depends on previous surgical treatment and/or pathophysiological conditions. However, reliable and repeatable quantification of RV dimensions and function for these patients are essential to provide appropriate timing for intervention to optimize outcomes. In this population, echocardiographic evaluation should not be limited to an observational and subjective functional assessment of the RV but must provide quantitative values repeatable and clinically reliable to help the decision-making process. The aim of this review was to discuss the echocardiographic approach to the RV in adult patients with CHD in general and in specific cases of pressure or volume overload.

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

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    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

  20. Response of right ventricular size, function, and pressure to supine exercise: a comparison of patients with chronic obstructive lung disease and normal subjects

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    Slutsky, R.; Hooper, W.; Ackerman, W.; Moser, K.

    1982-12-01

    The response of right ventricular ejection fraction (RVEF) and right ventricular end-diastolic volume (RVEDV) to exercise was studied in 11 patients with severe (FEV/sub 25/sub(%)sub(-)/sub 75/sub(%)=0.32+-0.13, mean+-SD) chronic obstructive pulmonary disease (COPD). Using gated radionuclide cardiac blood pool imaging techniques, the response of the patients with COPD was compared with that of 15 control subjects. Arterial blood gases, pulmonary arterial pressures, wedge pressure, and right ventricular pressures also were monitored in patients with COPD. The resting RVEF was lower and the resting RVEDV was higher in patients with COPD than in normals (both, P<0.01). Two of the 11 COPD patients had a RVEF during rest that was below lower limits, while 10 of 11 patients had RV dilation. Right ventricular end-diastolic pressure, measured during rest in patients with COPD, was normal (6.1+-2.1 mm Hg) and cardiac index was within normal limits (3.55+-0.82 l/min/m/sup 2/). With exercise this cardiac index rose to 5.52+-1.7/min/m/sup 2/(P<0.01) due to the increase in heart rate (83+-18 to 125+-25 beats/min; P<0.01) while stroke volume did not significantly change. During exercise, normal subjects showed an increase in RVEF while RVEDV did not change; in patients with COPD, the RVEF fell and the RVEDV increased. In the patients with COPD, mild resting arterial hypoxemia and hypercapnia were both exaggerated during exercise; and mild resting pulmonary arterial hypertension (PAm=24.3+-7.65 mm Hg) also worsened with exercise (PAm=41+-19 mm Hg, P<0.01). Correlation between change in RVEF and PAm was -0.58, and between change in RVEDV and PAm was 0.63. We conclude that patients with severe COPD often have right ventricular dilation at rest and commonly respond to supine exercise with a fall in FV ejection fraction and further dilation of the right ventricle.

  1. Oculodentodigital dysplasia

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    Dharmil C Doshi

    2016-01-01

    Full Text Available Oculodentodigital dysplasia is a rare, autosomal dominant disorder with high penetrance and variable expressivity, caused by mutations in the connexin 43 or gap junction protein alpha-1 gene. It has been diagnosed in fewer than 300 people worldwide with an incidence of around 1 in 10 million. It affects many parts of the body, particularly eyes (oculo, teeth (dento, and fingers and/or toes (digital. The common clinical features include facial dysmorphism with thin nose, microphthalmia, syndactyly, tooth anomalies such as enamel hypoplasia, anodontia, microdontia, early tooth loss and conductive deafness. Other less common features are abnormalities of the skin and its appendages, such as brittle nails, sparse hair, and neurological abnormalities. To prevent this syndrome from being overlooked, awareness of possible symptoms is necessary. Early recognition can prevent blindness, dental problems and learning disabilities. Described here is the case of a 21-year-old male who presented to the ophthalmology outpatient department with a complaint of bilateral progressive loss of vision since childhood.

  2. Usefulness of color kinesis imaging for evaluation of regional right ventricular wall motion in patients with surgically repaired tetralogy of Fallot.

    Science.gov (United States)

    Hayabuchi, Y; Matsuoka, S; Kubo, M; Kuroda, Y

    1998-11-15

    We evaluated regional right ventricular wall motion during systole in patients with surgically repaired tetralogy of Fallot (TOF) using color kinesis imaging. Color kinesis images were obtained in a subcostal sagittal view from 19 patients with repaired TOF (TOF group), aged 3 to 5 years, and 20 age-matched normal subjects (control group). For regional wall motion analysis, the endocardial motion distance and the fractional area change (FAC) were calculated for 6 segments obtained from color kinesis images. The endocardial inward excursion distances and the FACs in the upper, middle, and lower posterior segments of the TOF group were significantly greater than those of the control group (p kinesis measurements was excellent (y = 1.14x - 1.30, r = 0.87 for the endocardial inward excursion, and y = 1.03x +/- 0.56, r = 0.91 for the FAC). In conclusion, color kinesis is a useful method for assessing regional right ventricular wall motion. Segmental analysis of color kinesis images provides accurate, automated, and quantitative diagnosis of regional right ventricular wall motion abnormalities in patients with surgically repaired TOF.

  3. The Frank-Starling mechanism involves deceleration of cross-bridge kinetics and is preserved in failing human right ventricular myocardium.

    Science.gov (United States)

    Milani-Nejad, Nima; Canan, Benjamin D; Elnakish, Mohammad T; Davis, Jonathan P; Chung, Jae-Hoon; Fedorov, Vadim V; Binkley, Philip F; Higgins, Robert S D; Kilic, Ahmet; Mohler, Peter J; Janssen, Paul M L

    2015-12-15

    Cross-bridge cycling rate is an important determinant of cardiac output, and its alteration can potentially contribute to reduced output in heart failure patients. Additionally, animal studies suggest that this rate can be regulated by muscle length. The purpose of this study was to investigate cross-bridge cycling rate and its regulation by muscle length under near-physiological conditions in intact right ventricular muscles of nonfailing and failing human hearts. We acquired freshly explanted nonfailing (n = 9) and failing (n = 10) human hearts. All experiments were performed on intact right ventricular cardiac trabeculae (n = 40) at physiological temperature and near the normal heart rate range. The failing myocardium showed the typical heart failure phenotype: a negative force-frequency relationship and β-adrenergic desensitization (P 0.05), indicating that this regulatory mechanism is preserved in heart failure. Length-dependent assessment of twitch kinetics mirrored these findings; normalized dF/dt slowed down with increasing length of the muscle and was virtually identical in diseased tissue. This study shows for the first time that muscle length regulates cross-bridge kinetics in human myocardium under near-physiological conditions and that those kinetics are preserved in the right ventricular tissues of heart failure patients.

  4. Turner's syndrome, fibromuscular dysplasia, and stroke.

    Science.gov (United States)

    Lancman, M; Mesropian, H; Serra, P; Granillo, R

    1991-02-01

    We report a 43-year-old woman who presented with a right frontoparietotemporal ischemic stroke. She had been diagnosed with Turner's syndrome during childhood and had a history of chronic estrogen therapy. Cerebral angiography showed lesions characteristics of fibromuscular dysplasia involving the right internal carotid and right vertebral arteries. We are not aware of any previous reports describing an association between fibromuscular dysplasia and Turner's syndrome. Although chronic estrogen therapy cannot be ruled out as a cause of this patient's stroke, we suggest a possible etiologic relation between these two entities.

  5. Right Ventricular Strain, Torsion, and Dyssynchrony in Healthy Subjects using 3D Spiral Cine DENSE Magnetic Resonance Imaging.

    Science.gov (United States)

    Suever, Jonathan; Wehner, Gregory; Jing, Linyuan; Powell, David; Hamlet, Sean; Grabau, Jonathan; Mojsejenko, Dimitri; Andres, Kristin; Haggerty, Christopher; Fornwalt, Brandon

    2016-12-29

    Mechanics of the left ventricle (LV) are important indicators of cardiac function. The role of right ventricular (RV) mechanics is largely unknown due to the technical limitations of imaging its thin wall and complex geometry and motion. By combining 3D Displacement Encoding with Stimulated Echoes (DENSE) with a post-processing pipeline that includes a local coordinate system, it is possible to quantify RV strain, torsion, and synchrony. In this study, we sought to characterize RV mechanics in 50 healthy individuals and compare these values to their LV counterparts. For each cardiac frame, 3D displacements were fit to continuous and differentiable radial basis functions, allowing for the computation of the 3D Cartesian Lagrangian strain tensor at any myocardial point. The geometry of the RV was extracted via a surface fit to manually delineated endocardial contours. Throughout the RV, a local coordinate system was used to transform from a Cartesian strain tensor to a polar strain tensor. It was then possible to compute peak RV torsion as well as peak longitudinal and circumferential strain. A comparable analysis was performed for the LV. Dyssynchrony was computed from the standard deviation of regional activation times. Global circumferential strain was comparable between the RV and LV (-18.0% for both) while longitudinal strain was greater in the RV (-18.1% vs. -15.7%). RV torsion was comparable to LV torsion (6.2 vs. 7.1 degrees, respectively). Regional activation times indicated that the RV contracted later but more synchronously than the LV. 3D spiral cine DENSE combined with a post-processing pipeline that includes a local coordinate system can resolve both the complex geometry and 3D motion of the RV.

  6. Nicorandil prevents right ventricular remodeling by inhibiting apoptosis and lowering pressure overload in rats with pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Xiang-Rong Zuo

    Full Text Available BACKGROUND: Most of the deaths among patients with severe pulmonary arterial hypertension (PAH are caused by progressive right ventricular (RV pathological remodeling, dysfunction, and failure. Nicorandil can inhibit the development of PAH by reducing pulmonary artery pressure and RV hypertrophy. However, whether nicorandil can inhibit apoptosis in RV cardiomyocytes and prevent RV remodeling has been unclear. METHODOLOGY/PRINCIPAL FINDINGS: RV remodeling was induced in rats by intraperitoneal injection of monocrotaline (MCT. RV systolic pressure (RVSP was measured at the end of each week after MCT injection. Blood samples were drawn for brain natriuretic peptide (BNP ELISA analysis. The hearts were excised for histopathological, ultrastructural, immunohistochemical, and Western blotting analyses. The MCT-injected rats exhibited greater mortality and less weight gain and showed significantly increased RVSP and RV hypertrophy during the second week. These worsened during the third week. MCT injection for three weeks caused pathological RV remodeling, characterized by hypertrophy, fibrosis, dysfunction, and RV mitochondrial impairment, as indicated by increased levels of apoptosis. Nicorandil improved survival, weight gain, and RV function, ameliorated RV pressure overload, and prevented maladaptive RV remodeling in PAH rats. Nicorandil also reduced the number of apoptotic cardiomyocytes, with a concomitant increase in Bcl-2/Bax ratio. 5-hydroxydecanoate (5-HD reversed these beneficial effects of nicorandil in MCT-injected rats. CONCLUSIONS/SIGNIFICANCE: Nicorandil inhibits PAH-induced RV remodeling in rats not only by reducing RV pressure overload but also by inhibiting apoptosis in cardiomyocytes through the activation of mitochondrial ATP-sensitive K(+ (mitoK(ATP channels. The use of a mitoK(ATP channel opener such as nicorandil for PAH-associated RV remodeling and dysfunction may represent a new therapeutic strategy for the amelioration of RV

  7. Morphology of tricuspid valve in pulmonary atresia with intact ventricular septum.

    Science.gov (United States)

    Choi, Y H; Seo, J W; Choi, J Y; Yun, Y S; Kim, S H; Lee, H J

    1998-01-01

    Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.

  8. Cardiac-specific genetic inhibition of nuclear factor-κB prevents right ventricular hypertrophy induced by monocrotaline.

    Science.gov (United States)

    Kumar, Sandeep; Wei, Chuanyu; Thomas, Candice M; Kim, Il-Kwon; Seqqat, Rachid; Kumar, Rajesh; Baker, Kenneth M; Jones, W Keith; Gupta, Sudhiranjan

    2012-04-15

    Uncontrolled pulmonary arterial hypertension (PAH) results in right ventricular (RV) hypertrophy (RVH), progressive RV failure, and low cardiac output leading to increased morbidity and mortality (McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA, McGoon MD, Park MH, Rosenson RS, Rubin LJ, Tapson VF, Varga J. J Am Coll Cardiol 53: 1573-1619, 2009). Although the exact figures of its prevalence are difficult to obtain because of the diversity of identifiable causes, it is estimated that the incidence of pulmonary hypertension is seven to nine cases per million persons in the general population and is most prevalent in the age group of 20-40, occurring more commonly in women than in men (ratio: 1.7 to 1; Rubin LJ. N Engl J Med 336: 111-117, 1997). PAH is characterized by dyspnea, chest pain, and syncope. Unfortunately, there is no cure for this disease and medical regimens are limited (Simon MA. Curr Opin Crit Care 16: 237-243, 2010). PAH leads to adverse remodeling that results in RVH, progressive right heart failure, low cardiac output, and ultimately death if left untreated (Humbert M, Morrell NW, Archer SL, Stenmark KR, MacLean MR, Lang IM, Christman BW, Weir EK, Eickelberg O, Voelkel NF, Rabinovitch M. J Am Coll Cardiol 43: 13S-24S, 2004; Humbert M, Sitbon O, Simonneau G. N Engl J Med 351: 1425-1436, 2004. LaRaia AV, Waxman AB. South Med J 100: 393-399, 2007). As there are no direct tools to assess the onset and progression of PAH and RVH, the disease is often detected in later stages marked by full-blown RVH, with the outcome predominantly determined by the level of increased afterload (D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT, et al. Ann Intern Med 115: 343-349, 1991; Sandoval J, Bauerle O, Palomar A, Gomez A, Martinez-Guerra ML, Beltran M, Guerrero ML. Validation of a prognostic equation Circulation 89: 1733-1744, 1994). Various studies have been

  9. 2-D Speckle tracking in the assessment of left and right ventricular functions in hemodialysis versus recently diagnosed uremic patients with preserved systolic function

    Directory of Open Access Journals (Sweden)

    Hatem Abdel Rahman Helmy Ali

    2016-12-01

    Full Text Available Several studies have demonstrated that uremic patients who have preserved left ventricular (LV systolic function could still have subtle systolic dysfunction. In our study, we assessed the right ventricular (RV and LV myocardial functions measured by conventional echocardiography and two-dimensional (2D longitudinal speckle-tracking in hemodialysis and non-dialysis recently diagnosed uremic patients. The study population consisted of 24 newly diagnosed uremic patients, 25 hemodialysis patients, and 20 healthy controls. The RV and LV longitudinal strains were significantly lower in patients than in controls (−9.6 vs. −15.3, P < 0.001 for RV and −11.3 vs. −14.8, p < 0.001 for LV. In the hemodialysis group, the RV longitudinal strain was significantly lower than in the non-dialysis group (p = 0.018. The RV longitudinal strain was correlated with hypertension and LV strain. The 2-D longitudinal speckle tracking can detect early ventricular (left and right systolic dysfunction in patients with uremia in the presence of normal systolic function by conventional methods.

  10. Right ventricular failure due to chronic pressure load: What have we learned in animal models since the NIH working group statement?

    Science.gov (United States)

    Borgdorff, Marinus A J; Dickinson, Michael G; Berger, Rolf M F; Bartelds, Beatrijs

    2015-07-01

    Right ventricular (RV) failure determines outcome in patients with pulmonary hypertension, congenital heart diseases and in left ventricular failure. In 2006, the Working Group on Cellular and Molecular Mechanisms of Right Heart Failure of the NIH advocated the development of preclinical models to study the pathophysiology and pathobiology of RV failure. In this review, we summarize the progress of research into the pathobiology of RV failure and potential therapeutic interventions. The picture emerging from this research is that RV adaptation to increased afterload is characterized by increased contractility, dilatation and hypertrophy. Clinical RV failure is associated with progressive diastolic deterioration and disturbed ventricular-arterial coupling in the presence of increased contractility. The pathobiology of the failing RV shows similarities with that of the LV and is marked by lack of adequate increase in capillary density leading to a hypoxic environment and oxidative stress and a metabolic switch from fatty acids to glucose utilization. However, RV failure also has characteristic features. So far, therapies aiming to specifically improve RV function have had limited success. The use of beta blockers and sildenafil may hold promise, but new therapies have to be developed. The use of recently developed animal models will aid in further understanding of the pathobiology of RV failure and development of new therapeutic strategies.

  11. Clinical characteristics of arrhythmogenic right ventricular cardiomyopathy accompanied with giant epsilon wave%巨大Epsilon波致心律失常性右室心肌病临床和心电图特点

    Institute of Scientific and Technical Information of China (English)

    谢启应; 杨天伦; 井然; 马静; 易军; 孙泽琳

    2016-01-01

    目的 总结心电图表现为巨大Epsilon波致心律失常右室心肌病(ARVC)患者的临床和心电图特点.方法 分析本院于2011年1月至2014年12月收治的12导联心电图示巨大Epsilon波ARVC患者的临床和心电图特点.通过查询Medline、CNKI、VIP和万方数据库等总结该心电图特征ARVC患者的特点.结果 5例患者中男性4例.首发症状均为心悸、头昏.发作时心电图为右室起源室性心动过速.心脏超声示右室扩大;右室壁变薄,可见小凹.心电图V1导联QRS呈“M”型;巨大Epsilon波振幅为(0.37±0.16)mV,振幅与S波比值为1.20±0.23;持续时间为(90±12.75)ms,持续时间与QRS波相比为0.60±0.06.文献报道5例患者均为男性.临床表现为心悸,晕厥发作.心电图V1导联QRS均呈rsR ',Fontain导联、心向量和晚电位证实该R '波为Epsilon波.V1导联Ep-silon波振幅为(0.52±0.35)mV,振幅与S波比值为1.18±0.74;持续时间(96.0±11.4)ms,持续时间与QRS波相比为0.65±0.08.结论 临床上巨大Epsilon波ARVC患者少见,右室激动延缓,心律失常发生率高.

  12. Fibromuscular dysplasia

    Directory of Open Access Journals (Sweden)

    Jeunemaitre Xavier

    2007-06-01

    Full Text Available Abstract Fibromuscular dysplasia (FMD, formerly called fibromuscular fibroplasia, is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervicocranial FMD is probably half that. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histological lesions. Renovascular hypertension is the most common manifestation of renal artery FMD. Multifocal stenoses with the 'string-of-beads' appearance are observed at angiography in more than 80% of cases, mostly in women aged between 30 and 50 years; they generally involve the middle and distal two-thirds of the main renal artery and in some case also renal artery branches. Cervicocranial FMD can be complicated by dissection with headache, Horner's syndrome or stroke, or can be associated with intracerebral aneurysms with a risk of subarachnoid or intracerebral hemorrhage. The etiology of FMD is unknown, although various hormonal and mechanical factors have been suggested. Subclinical lesions are found at arterial sites distant from the stenotic arteries, and this suggests that FMD is a systemic arterial disease. It appears to be familial in 10% of cases. Noninvasive diagnostic tests include, in increasing order of accuracy, ultrasonography, magnetic resonance angiography and computed tomography angiography. The gold standard for diagnosing FMD is catheter angiography, but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization during the same procedure. Differential diagnosis include

  13. Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy [v1; ref status: indexed, http://f1000r.es/17s

    Directory of Open Access Journals (Sweden)

    Ben Paylor

    2013-06-01

    Full Text Available Arrhythmogenic cardiomyopathy (AC is a disease of the heart involving myocardial dystrophy leading to fibrofatty scarring of the myocardium and is associated with an increased risk of both ventricular arrhythmias and sudden cardiac death. It often affects the right ventricle but may also involve the left. Although there has been significant progress in understanding the role of underlying desmosomal genetic defects in AC, there is still a lack of data regarding the cellular processes involved in its progression. The development of cardiac fibrofatty scarring is known to be a principal pathological process associated with ventricular arrhythmias, and it is vital that we elucidate the role of various cell populations involved in the disease if targeted therapeutics are to be developed. The known role of mesenchymal progenitor cells in the reparative process of both the heart and skeletal muscle has provided inspiration for the identification of the cellular basis of fibrofatty infiltration in AC. Here we hypothesize that reparative processes triggered by myocardial degeneration lead to the differentiation of tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors into adipocytes and fibroblasts, which compose the fibrofatty lesions characteristic of AC.

  14. Epicardial Ventricular Tachycardia Ablation: Clinical Practice and Recent Developments

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    Michalis Efremidis MD

    2011-08-01

    Full Text Available Mapping and radiofrequency (RF catheter ablation of ventricular tachycardia (VT is a demanding procedure, with variable success rates (1. The presence of deep subendocardial or epicardial re-entry circuits is regarded as one of the reasons of failure of endocardial ablation, and these circuits have been acknowledged in ischemic and non-ischemic dilated cardiomyopathy (CMP, other types of CMP and especially in arrhythmogenic right ventricular cardiomyopathy (ARVC.The significance of epicardial VT circuits was brought to light in Chagas’ disease, which characteristically results in epicardial involvement in approximately 70% of patients (2. A recent study found one third of VTs to be epicardial in origin among patients with nonischemic CMP, about double the incidence among those with ischemic heart disease(3. Mapping and ablation of these epicardial circuits is quite exigent. Although coronary veins can be used to perform epicardial mapping, the manipulation of the catheter is strictly limited to the anatomical distribution of these vessels. Thus, the subxiphoid percutaneous approach to the pericardial space is the only technique that allows extensive, unhampered mapping of the epicardial surface of both ventricles.

  15. Downregulation of Kv4.2 and Kv4.3 channel gene expression in right ventricular hypertrophy induced by monocrotaline in rat

    Institute of Scientific and Technical Information of China (English)

    Tian-tai ZHANG; Bing CUI; De-zai DAI

    2004-01-01

    AIM: To investigate the differences in gene expression of transient outward potassium ion channel between the free wall of right ventricle, free wall of left ventricle, and the septum in monocrotaline (MCT)-induced right ventricular hypertrophy of rat. METHODS: Twenty rats were randomly divided into two groups: a single injection of monocrotaline (MCT) 60 mg/kg (model) or saline (control). Four weeks later, hemodynamic parameters were measured and the gene expression of Ito channels were detected by semi-quantitative RT-PCR. RESUITS: After 28 d, the right ventricular systolic pressure and central venous pressure were remarkably elevated by 128 % and 533 % in the MCT-treated group, accompanied by an overt right ventricle (RV) remodeling. The difference of mRNA expression of Kv1.4 was not significant in free wall of RV, left ventricle (LV), and septum in MCT group compared with control group. In contrast, mRNA of Ky4.2 and Ky4.3 in the free wall of RV in MCT-induced rat was dramatically decreased by 45.2 % and 51.1% vs control, however, in free wall of LV and septum, no difference was found. In addition, mRNA expression level of Ky4.2 in control rat was significantly lower in septum than that in free wall of RV and LV. CONCLUSION: Expression of Kv1.4. Ky4.2, and Kv4.3 differs between regions in normal rat hearts. The down-regulation of Ky4 family gene expression of Ito contributed to the pathophysiological changes in ventricular hypertrophy and pulmonary hypertension induced by MCT.

  16. Assessment value of real-time three-dimensional echocardiography on right ventricular systolic function in patients with tetralogy of Fallot

    Institute of Scientific and Technical Information of China (English)

    Jin-Hua Su; Hai-Yan Kou; Li Liang

    2016-01-01

    Objective:To analyze the assessment value of real-time three-dimensional echocardiography on right ventricular systolic function in patients with tetralogy of Fallot (TOF).Methods:63 cases of children with tetralogy of Fallot undergoing surgical treatment in our hospital from September 2011 to September 2014 were chosen as observation group, 72 cases of normal children receiving health examination in our hospital during the same period were chosen as normal control group, all of them received two-dimensional and three-dimensional ultrasound examination, and right ventricular systolic function of TOF children was compared and observed.Results:1) TOF patients’ preoperative left pulmonary artery inner diameter was 5.38–9.65 mm, right pulmonary artery inner diameter was 5.34–9.15 mm, average pulmonary artery index (Nakata index) was (160.92±21.58) mm2/m2; 2) TOF patients’ preoperative RVEDV and RVESV values were greater than those of normal control group and RVEF value was less than that of normal control group (P<0.05); RVEDV and RVESV 6 months after surgery increased, RVEF decreased, and compared with before surgery, there was significant difference (P<0.05); 3) TOF patients’ preoperative three-dimensional ultrasound RVESV and RVEDV values were higher than results of two-dimensional ultrasound examination (P<0.05); 4) TOF patients’ preoperative RVEF had significant positive correlation with Nakata index, r=0.653 (P<0.05).Conclusions: Real-time three-dimensional echocardiography can accurately and objectively assess right ventricular systolic function in patients with Tetralogy of Fallot, and contributes to the assessment of treatment effect and long-term prognosis.

  17. Arrhythmogenic remodelling of activation and repolarization in the failing human heart.

    Science.gov (United States)

    Holzem, Katherine M; Efimov, Igor R

    2012-11-01

    Heart failure is a major cause of disability and death worldwide, and approximately half of heart failure-related deaths are sudden and presumably due to ventricular arrhythmias. Patients with heart failure have been shown to be at 6- to 9-fold increased risk of sudden cardiac death compared to the general population. (AHA. Heart Disease and Stroke Statistics-2003 Update. Heart and Stroke Facts. Dallas, TX: American Heart Association; 2002) Thus, electrophysiological remodelling associated with heart failure is a leading cause of disease mortality and has been a major investigational focus examined using many animal models of heart failure. While these studies have provided an important foundation for understanding the arrhythmogenic pathophysiology of heart failure, the need for corroborating studies conducted on human heart tissue has been increasingly recognized. Many human heart studies of conduction and repolarization remodelling have now been published and shed some light on important, potentially arrhythmogenic, changes in human heart failure. These studies are being conducted at multiple experimental scales from isolated cells to whole-tissue preparations and have provided insight into regulatory mechanisms such as decreased protein expression, alternative mRNA splicing of ion channel genes, and defective cellular trafficking. Further investigations of heart failure in the human myocardium will be essential for determining possible therapeutic targets to prevent arrhythmia in heart failure and for facilitating the translation of basic research findings to the clinical realm.

  18. Truncating plakophilin-2 mutations in arrhythmogenic cardiomyopathy are associated with protein haploinsufficiency in both myocardium and epidermis

    DEFF Research Database (Denmark)

    Rasmussen, Torsten B; Nissen, Peter H; Palmfeldt, Johan;

    2014-01-01

    BACKGROUND: Arrhythmogenic cardiomyopathy (AC) is a hereditary cardiac condition associated with ventricular arrhythmias, heart failure, and sudden death. The disease is most often caused by mutations in the desmosomal gene for plakophilin-2 (PKP2), which is expressed in both myocardial...... individuals. It was evident that PKP2 mutations introducing a premature termination codon in the reading frame were associated with PKP2 transcript and protein levels reduced to ≈50%, whereas a missense variant did not seem to affect the amount of PKP2 protein. CONCLUSIONS: The results of this study showed...

  19. Effects of milrinone and epinephrine or dopamine on biventricular function and hemodynamics in an animal model with right ventricular failure after pulmonary artery banding.

    Science.gov (United States)

    Hyldebrandt, Janus Adler; Sivén, Eleonora; Agger, Peter; Frederiksen, Christian Alcaraz; Heiberg, Johan; Wemmelund, Kristian Borup; Ravn, Hanne Berg

    2015-07-01

    Right ventricular (RV) failure due to chronic pressure overload is a main determinant of outcome in congenital heart disease. Medical management is challenging because not only contractility but also the interventricular relationship is important for increasing cardiac output. This study evaluated the effect of milrinone alone and in combination with epinephrine or dopamine on hemodynamics, ventricular performance, and the interventricular relationship. RV failure was induced in 21 Danish landrace pigs by pulmonary artery banding. After 10 wk, animals were reexamined using biventricular pressure-volume conductance catheters. The maximum pressure in the RV increased by 113% (P animals. LV volume was reduced in both the dopamine and epinephrine groups with increasing doses In the failing pressure overloaded RV, milrinone improved CI and increased contractility. Albeit additional dose-dependent effects of both epinephrine and dopamine on CI and contractility, neither of the interventions improved SVI due to reduced filling of the LV.

  20. Prevalence and determinants of anemia in adults with complex congenital heart disease and ventricular dysfunction (subaortic right ventricle and single ventricle physiology).

    Science.gov (United States)

    Collins, Nicholas; Piran, Sanaz; Harrison, Jeanine; Azevedo, Eduardo; Oechslin, Erwin; Silversides, Candice K

    2008-09-01

    Anemia is well recognized as a marker of poor prognosis in patients with acquired heart disease and heart failure. Adults with complex congenital heart disease and ventricular dysfunction (subaortic right ventricle or single-ventricle physiology) represent a different population, because they are typically much younger and have less co-morbidity compared with patients with acquired forms of heart disease. The purpose of this study was to evaluate the prevalence and determinants of anemia in this population. Baseline hemoglobin levels were recorded at the time of the initial clinic visit, and final hemoglobin levels were those recorded before death or transplantation or at study completion. Anemia was defined as hemoglobin complex congenital heart disease and ventricular dysfunction, in particular those with Fontan physiology.

  1. Relation of fragmented QRS complex to right ventricular fibrosis detected by late gadolinium enhancement cardiac magnetic resonance in adults with repaired tetralogy of fallot.

    Science.gov (United States)

    Park, Seung-Jung; On, Young Keun; Kim, June Soo; Park, Seung Woo; Yang, Ji-Hyuk; Jun, Tae-Gook; Kang, I-Seok; Lee, Heung Jae; Choe, Yeon Hyeon; Huh, June

    2012-01-01

    Fragmented QRS (fQRS) on 12-lead electrocardiography reflects conduction delay caused by myocardial fibrosis and dysfunction. Ventricular fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is reportedly correlated with worse clinical outcomes in adults with repaired tetralogy of Fallot (TOF). The aim of this study was to assess whether the presence of fQRS is associated with right ventricular (RV) fibrosis or dysfunction in this patient group. In 37 consecutive patients (median age 30 years, median age at repair 6.6 years), the number of leads showing fQRS, defined as the presence of >2 notches on the R/S wave in ≥2 contiguous leads, was counted. RV systolic function, dilatation, and LGE score were measured using LGE CMR. Ventricular LGE was observed mainly at the previous surgical sites: the RV outflow tract (33 of 37), ventricular septal defect patch region (15 of 37), and RV anterior wall (11 of 37). Fragmented QRS was found mostly in the right and mid precordial leads. The fQRS group (n = 20) demonstrated higher RV LGE scores (p <0.001) and lower RV ejection fractions (p = 0.02) and a trend toward larger RV end-diastolic and end-systolic volumes (p = 0.12 and p = 0.06, respectively) compared to the non-fQRS group (n = 17). The number of electrocardiographic leads showing fQRS was positively correlated with RV LGE score (r = 0.75, p <0.001). The presence of fQRS remained independently associated with the presence of supramedian RV LGE score, even after adjusting for relevant parameters. In conclusion, fQRS was closely associated with more extensive RV fibrosis and dysfunction in adults with repaired tetralogy of Fallot.

  2. Echocardiographic estimation of right ventricular function in patients with congenital heart disease%超声心动图对先天性心脏病右心室功能的评价

    Institute of Scientific and Technical Information of China (English)

    崔亚玲; 黄新胜; 黄奕高; 黄涛; 张曹进

    2012-01-01

    目的 以右心室造影为对照,使用超声心动图常规的四腔心切面和全新的右心室全显示切面定量评价右心室射血分数(right ventricular ejection fraction,RVEF),探讨右心室全显示切面在右心功能评价中的应用价值.方法 2011年4月至2011年11月通过临床和超声心动图检查选择伴有右心室形态或血流动力学改变的先天性心脏病患者22例(男13例,女9例,年龄16~67岁)为研究对象.除对入选患者行常规的超声心动图检查外,还使用四腔心切面和右心室全显示切面测量RVEF.在心导管实验室使用右心室造影测量RVEF.将四腔心和右心室全显示切面RVEF与右心室造影RVEF行随机区组设计方差分析及Pearson相关性分析,右心室全显示切面RVEF与其余右心功能指标行Pearson相关分析,并采用Bland -Altman法评价右心室全显示切面与右心室造影的一致性.结果 3种方法测得的RVEF分别为右心室全显示切面48.0%±11.3%、四腔心切面49.5%±13.1%、右心室造影48.7%±12.1%.3种方法测量结果比较,差异无统计学意义(F=0.327,P=0.723).右心室全显示切面RVEF与右心室造影RVEF呈高度相关(r=0.908,P<0.001),四腔心切面RVEF与右心室造影RVEF呈中度相关(r=0.659,P=0.001).右心室全显示切面RVEF与肺动脉收缩压及主肺动脉宽度负相关(P<0.05),与右心室每搏输出量正相关(P<0.05),与其他右心功能评价指标则无明显相关性.结论 与常规的四腔心测量方法相比,超声心动图右心室全显示切面测量的RVEF与右心室造影的相关性更好,可能是一种准确和可靠的评价右心室收缩功能的方法.%Objectives A novel method was developed for calculating right ventricular ejection fraction (RVEF) from total right ventricular view of two-dimensional echocardiography, and was compared with the conventional four chamber view and right anterior oblique view of right ventricular angiography. The

  3. Myopericarditis with predominantly right ventricular involvement with normal B-type natriuretic peptide and cardiac tamponade as the initial manifestation of systemic lupus erythematosus.

    Science.gov (United States)

    Manautou, Luis; Jerjes-Sanchez, Carlos; Meraz, Manuel; Perez-Garcia, Luis F; Diaz-Cid, Antonio; de la Peña-Almaguer, Erasmo; Avila, Cesar; Sanchez, Luis

    2014-08-01

    A previously healthy young man presented with a 12-hour history of sudden dyspnea and severe chest pain at rest. Initial findings of physical examination, electrocardiogram and chest radiography showed typical pericarditis and clinical instability. Echocardiogram revealed small pericardial effusion with right ventricle dilatation. The patient was admitted in the ICU; a new echocardiogram revealed moderate pericardial effusion and diagnosis of pericarditis complicated with acute cardiac tamponade was established. The patient transiently improved after pericardial window. In the following hours, the diagnosis of myocarditis with predominantly right ventricular involvement (MPRVI) with severe right heart failure was supported by clinical, chest radiography and echocardiogram data, despite normal B-type natriuretic peptide. On day 2, cardiac troponin I detection was observed. By day 3, B-type natriuretic peptide in the range of ventricular dysfunction was identified. Cardiovascular magnetic resonance findings supported the diagnosis of MPRVI. A systematic MEDLINE/PubMed from 1993 to 2013 does not identify any cases of MPRVI related to systemic lupus erythematosus. Simultaneous acute MPRVI with normal B-type natriuretic peptide and acute cardiac tamponade heralding the diagnosis of systemic lupus erythematosus, to the best of our knowledge, has not been previously described.

  4. Quantification of Coupled Stiffness and Fiber Orientation Remodeling in Hypertensive Rat Right-Ventricular Myocardium Using 3D Ultrasound Speckle Tracking with Biaxial Testing

    Science.gov (United States)

    Park, Dae Woo; Sebastiani, Andrea; Yap, Choon Hwai; Simon, Marc A.; Kim, Kang

    2016-01-01

    Mechanical and structural changes of right ventricular (RV) in response to pulmonary hypertension (PH) are inadequately understood. While current standard biaxial testing provides information on the mechanical behavior of RV tissues using surface markers, it is unable to fully assess structural and mechanical properties across the full tissue thickness. In this study, the mechanical and structural properties of normotensive and pulmonary hypertension right ventricular (PHRV) myocardium through its full thickness were examined using mechanical testing combined with 3D ultrasound speckle tracking (3D-UST). RV pressure overload was induced in Sprague–Dawley rats by pulmonary artery (PA) banding. The second Piola–Kirchhoff stress tensors and Green-Lagrangian strain tensors were computed in the RV myocardium using the biaxial testing combined with 3D-UST. A previously established non-linear curve-fitting algorithm was applied to fit experimental data to a Strain Energy Function (SEF) for computation of myofiber orientation. The fiber orientations obtained by the biaxial testing with 3D-UST compared well with the fiber orientations computed from the histology. In addition, the re-orientation of myofiber in the right ventricular free wall (RVFW) along longitudinal direction (apex-to-outflow-tract direction) was noticeable in response to PH. For normotensive RVFW samples, the average fiber orientation angles obtained by 3D-UST with biaxial test spiraled from 20° at the endo-cardium to -42° at the epi-cardium (Δ = 62°). For PHRV samples, the average fiber orientation angles obtained by 3D-UST with biaxial test had much less spiral across tissue thickness: 3° at endo-cardium to -7° at epi-cardium (Δ = 10°, P<0.005 compared to normotensive). PMID:27780271

  5. Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry

    Directory of Open Access Journals (Sweden)

    Swift Andrew J

    2012-06-01

    Full Text Available Abstract Background Cardiovascular Magnetic Resonance (CMR imaging is accurate and reproducible for the assessment of right ventricular (RV morphology and function. However, the diagnostic accuracy of CMR derived RV measurements for the detection of pulmonary hypertension (PH in the assessment of patients with suspected PH in the clinic setting is not well described. Methods We retrospectively studied 233 consecutive treatment naïve patients with suspected PH including 39 patients with no PH who underwent CMR and right heart catheterisation (RHC within 48hours. The diagnostic accuracy of multiple CMR measurements for the detection of mPAP ≥ 25 mmHg was assessed using Fisher’s exact test and receiver operating characteristic (ROC analysis. Results Ventricular mass index (VMI was the CMR measurement with the strongest correlation with mPAP (r = 0.78 and the highest diagnostic accuracy for the detection of PH (area under the ROC curve of 0.91 compared to an ROC of 0.88 for echocardiography calculated mPAP. Late gadolinium enhancement, VMI ≥ 0.4, retrograde flow ≥ 0.3 L/min/m2 and PA relative area change ≤ 15% predicted the presence of PH with a high degree of diagnostic certainty with a positive predictive value of 98%, 97%, 95% and 94% respectively. No single CMR parameter could confidently exclude the presence of PH. Conclusion CMR is a useful alternative to echocardiography in the evaluation of suspected PH. This study supports a role for the routine measurement of ventricular mass index, late gadolinium enhancement and the use of phase contrast imaging in addition to right heart functional indices in patients undergoing diagnostic CMR evaluation for suspected pulmonary hypertension.

  6. Single coronary artery with origin of right coronary artery from left circumflex in a patient with ventricular tachycardia: a case report

    Directory of Open Access Journals (Sweden)

    Sanjeev Sanghvi

    2016-03-01

    Full Text Available Only a few cases of a single coronary artery (CA have been described. Almost all cases reported so far also had associated atherosclerotic coronary artery or valvular heart disease. We present a 48 years old male with atypical chest pain with an episode of Ventricular Tachycardia (VT on treadmill test (TMT. Coronary angiography (CAG showed a single Left coronary artery (LCA with Right coronary artery (RCA arising from left circumflex artery (LCX without any atherosclerotic disease. [Int J Res Med Sci 2016; 4(3.000: 960-962

  7. Evaluation of Right Ventricular Global Longitudinal Function in Patients with Tetralogy of Fallot by Two-dimensional Ultrasound Speckle Tracking Imaging

    Institute of Scientific and Technical Information of China (English)

    李玉曼; 谢明星; 王新房; 吕清; 卢晓芳; 杨亚利; 马红; 方凌云; 张静; 李卫芹

    2010-01-01

    Quantification of right ventricular(RV)volume and function remains a challenge because of RV complex geometry by conventional echocardiography.The purpose of this study was to assess RV global longitudinal function in patients with tetralogy of Fallot(TOF)by 2-dimensional ultrasound speckle tracking imaging(STI).Thirty-eight patients with TOF were enrolled in this study and divided into child group(n=25)and adult group(n=13)according to age.Thirty-eight age-and sex-matched normal subjects were selected as c...

  8. Beating Heart Mitral Valve Replacement Surgery without Aortic Cross-Clamping via Right Thoracotomy in a Patient with Compromised Left Ventricular Functions

    Directory of Open Access Journals (Sweden)

    Ahmet Baris-Durukan

    2015-10-01

    Full Text Available Global myocardial ischemia and ischemia-reperfusion injury are potential adverse events related with cardioplegic arrest. Beating heart surgery has avoided such complications and adapted to valve surgery following successful results published on myocardial revascularization. Difficulty in weaning from cardiopulmonary bypass may be lessened by using on-pump beating heart surgery for mitral valve interventions. Here we describe a 64-year-old male patient with severe mitral regurgitation and dilated cardiomyopathy. Beating heart mitral valve replacement surgery was performed without aortic cross-clamping through a right thoracotomy approach. We believe that, particularly in patients with poor left ventricular functions, beating heart mitral valve surgery may be advantageous

  9. Mapeamento epicárdico da taquicardia ventricular sustentada em cardiopatias não isquêmicas Mapeo epicárdico de la taquicardia ventricular sostenida en cardiopatías no isquémicas Epicardial mapping of sustained ventricular tachycardia in nonischemic heart disease

    Directory of Open Access Journals (Sweden)

    Geórgia Guedes da Silva

    2011-02-01

    ón de la taquicardia ventricular (TV sostenida en pacientes con cardiopatía no isquémica. MÉTODOS: Veinte y seis pacientes con TV sostenida recurrente, siendo 22 (84,6% con cardiopatía chagásica crónica, dos (7,7% con cardiomiopatía dilatada idiopática y dos (7,7% portadores de displasia arritmogénica del ventrículo derecho, fueron sometidos a mapeo epicárdico con dos o tres microcatéteres, con 8 electrodos cada uno, simultáneamente al mapeo endocárdico convencional. Se utilizó catéter con punta de 4 mm para ablación con radiofrecuencia (RF realizada durante la TV inducida. RESULTADOS: De las 33 TVs inducidas, 25 fueron mapeadas y 20 tuvieron su origen definido. Once con origen epicárdica y 9 endocárdica. La estimulación ventricular programada no indujo TV sostenida en 11 (42,0% de los 26 pacientes después de la ablación. Eventos como recurrencia de la TV y muerte ocurrieron en 10,0% de los pacientes sometidos a la ablación con éxito, y en 59,0% de los fracasos, en control ambulatorio medio de 357 ± 208 días. CONCLUSIÓN: Circuitos subepicárdicos son frecuentes en pacientes con cardiopatía no isquémica. El mapeo epicárdico con múltiples catéteres realizado simultáneamente con el mapeo endocárdico contribuye a la identificación de estos circuitos en un mismo procedimiento.BACKGROUND: The complexity of reentrant circuits related to ventricular tachycardias decreases the success rate of radiofrequency ablation procedures. OBJECTIVE: To evaluate whether the epicardial mapping with multiple electrodes carried out simultaneously with the endocardial mapping helps in ablation procedures of sustained ventricular tachycardia (VT in patients with nonischemic heart disease. METHODS: Twenty-six patients with recurrent sustained VT, of which 22 (84.6% presenting chronic chagasic cardiomyopathy, 2 (7.7% with idiopathic dilated cardiomyopathy and 2 with right ventricular arrhythmogenic dysplasia (RVAD, were submitted to epicardial mapping with two or

  10. Pressure-overload hypertrophy of the developing heart reveals activation of divergent gene and protein pathways in the left and right ventricular myocardium.

    Science.gov (United States)

    Friehs, Ingeborg; Cowan, Douglas B; Choi, Yeong-Hoon; Black, Kendra M; Barnett, Reanne; Bhasin, Manoj K; Daly, Christian; Dillon, Simon J; Libermann, Towia A; McGowan, Francis X; del Nido, Pedro J; Levitsky, Sidney; McCully, James D

    2013-03-01

    Right ventricular (RV) and left ventricular (LV) myocardium differ in their pathophysiological response to pressure-overload hypertrophy. In this report we use microarray and proteomic analyses to identify pathways modulated by LV-aortic banding (AOB) and RV-pulmonary artery banding (PAB) in the immature heart. Newborn New Zealand White rabbits underwent banding of the descending thoracic aorta [LV-AOB; n = 6]. RV-PAB was achieved by banding the pulmonary artery (n = 6). Controls (n = 6 each) were sham-manipulated. After 4 (LV-AOB) and 6 (RV-PAB) wk recovery, the hearts were removed and matched RNA and proteins samples were isolated for microarray and proteomic analysis. Microarray and proteomic data demonstrate that in LV-AOB there is increased transcript expression levels for oxidative phosphorylation, mitochondria energy pathways, actin, ILK, hypoxia, calcium, and protein kinase-A signaling and increased protein expression levels of proteins for cellular macromolecular complex assembly and oxidative phosphorylation. In RV-PAB there is also an increased transcript expression levels for cardiac oxidative phosphorylation but increased protein expression levels for structural constituents of muscle, cardiac muscle tissue development, and calcium handling. These results identify divergent transcript and protein expression profiles in LV-AOB and RV-PAB and provide new insight into the biological basis of ventricular specific hypertrophy. The identification of these pathways should allow for the development of specific therapeutic interventions for targeted treatment and amelioration of LV-AOB and RV-PAB to ameliorate morbidity and mortality.

  11. Combined Right Ventricular Outflow Tract Epicardial and Endocardial Late Potential Ablation for Treatment of Brugada Storm: A Case Report and Review of the Literature.

    Science.gov (United States)

    Saha, Sandeep A; Krishnan, Kousik; Madias, Christopher; Trohman, Richard G

    2016-12-01

    A 34-year-old man with Brugada syndrome (BrS) presented with electrical storm, manifested as multiple appropriate shocks from his implantable cardioverter-defibrillator over a period of 7 hours. He had not tolerated prior treatment with quinidine, and had self-discontinued cilostazol citing persistent palpitations. After stabilization with intravenous isoproterenol, an electrophysiology study was performed but no spontaneous or induced ventricular ectopic beats were identified. A three-dimensional (3D) endocardial electro-anatomic map of the right ventricular outflow tract (RVOT), pulmonic valve, and pulmonary artery, as well as a 3D epicardial map of the RVOT, were created. Low voltage, complex, fractionated electrograms and late potentials were targeted for irrigated radiofrequency ablation both endocardially and epicardially. Post-procedure, he was maintained on cilostazol (referring clinician preference), and has had no further ventricular tachyarrhythmia episodes over the past forty-one months. We propose that this novel ablation strategy may be useful for acute management of selected patients with BrS.

  12. Ventricular arrhythmias in Chagas disease

    Directory of Open Access Journals (Sweden)

    Marco Paulo Tomaz Barbosa

    2015-02-01

    Full Text Available Sudden death is one of the most characteristic phenomena of Chagas disease, and approximately one-third of infected patients develop life-threatening heart disease, including malignant ventricular arrhythmias. Fibrotic lesions secondary to chronic cardiomyopathy produce arrhythmogenic substrates that lead to the appearance and maintenance of ventricular arrhythmias. The objective of this study is to discuss the main clinical and epidemiological aspects of ventricular arrhythmias in Chagas disease, the specific workups and treatments for these abnormalities, and the breakthroughs needed to determine a more effective approach to these arrhythmias. A literature review was performed via a search of the PubMed database from 1965 to May 31, 2014 for studies of patients with Chagas disease. Clinical management of patients with chronic Chagas disease begins with proper clinical stratification and the identification of individuals at a higher risk of sudden cardiac death. Once a patient develops malignant ventricular arrhythmia, the therapeutic approach aims to prevent the recurrence of arrhythmias and sudden cardiac death by the use of implantable cardioverter defibrillators, antiarrhythmic drugs, or both. In select cases, invasive ablation of the reentrant circuit causing tachycardia may be useful. Ventricular arrhythmias are important manifestations of Chagas cardiomyopathy. This review highlights the absence of high-quality evidence regarding the treatment of ventricular arrhythmias in Chagas disease. Recognizing high-risk patients who require specific therapies, especially invasive procedures such as the implantation of cardioverter defibrillators and ablative approaches, is a major challenge in clinical practice.

  13. A new look at bronchopulmonary dysplasia: postcapillary pathophysiology and cardiac dysfunction

    Science.gov (United States)

    Malikiwi, Andra; Paul, Eldho; Tan, Kenneth; Menahem, Samuel

    2016-01-01

    Abstract Pulmonary hypertension (PH) and right ventricular function are the focus of cardiovascular effects of bronchopulmonary dysplasia (BPD). We assessed cardiac indexes reflecting systemic afterload and pulmonary venous back pressure as pathophysiologic factors. Cardiac parameters were measured by conventional echocardiography in 20 preterm infants with severe BPD and compared with those of 10 preterm infants with no BPD and 20 healthy term infants. In infants with severe BPD, PH was noted in 5 (25%) by tricuspid regurgitation Doppler jet ≥2.8 m/s and in 15 (75%) by time to peak velocity/right ventricular ejection time valve stroke volume (4.7 ± 0.7 vs. 5.6 ± 0.6 vs. 5.9 ± 0.1; P = 0.002), and myocardial performance index (0.33 ± 0.05 vs. 0.28 ± 0.01 vs. 0.27 ± 0.05; P = 0.03). Left ventricular output was significantly lower in the BPD cohort (183 ± 45 vs. 189 ± 9 vs. 191 ± 32 mL/kg/min; P = 0.03). Altered systemic (left-sided) cardiac function was noted in infants with BPD, which may lead to pulmonary venous congestion contributing to a continued need for respiratory support. PMID:28090292

  14. Hipertrofia cardíaca esquerda e direita em necropsias de hipertensos Left and right ventricular hypertrophy at autopsy of hypertensive individuals

    Directory of Open Access Journals (Sweden)

    Mirella Pessoa Sant'Anna

    2012-02-01

    Full Text Available OBJETIVO: Medir a espessura ventricular direita e esquerda em falecidos com história de hipertensão arterial, submetidos a necropsias clínicas. MÉTODOS: Foram selecionados 90 casos do Serviço de Verificação de Óbitos de Recife -PE, de ambos os sexos, com história de hipertensão arterial essencial, com relação à espessura das paredes cardíacas, além da correlação com outros achados de necropsia e informes clínicos. RESULTADOS: Observouse associação significativa entre a presença de hipertrofia ventricular esquerda (HVE e direita (HVD, e de cardiopatia hipertensiva grave e HVD. Houve predomínio da HVD e HVE em homens, na faixa etária dos 60-79 anos, com maior prevalência nas etnias parda e negra, e naqueles com estado nutricional adequado ou com sobrepeso e em obesos. CONCLUSÃO: Observou-se que a presença de HVD relaciona-se com HVE, sugerindo que há fatores patogênicos semelhantes envolvidos no desenvolvimento da hipertrofia bilateral. A HVD parece associar-se à doença cardíaca mais grave, podendo, a partir de outros estudos, ser considerada novo fator prognóstico na avaliação dos pacientes hipertensos.OBJECTIVE: To measure the right and left ventricular thickness in deceased individuals with a history of hypertension submitted to clinical autopsies. METHODS: We selected 90 cases from the Death Verification Service of the city of Recife, state of Pernambuco, Brazil, of both sexes, with a history of essential arterial hypertension related to heart wall thickness, in addition to correlation with autopsy findings and other clinical reports. RESULTS: There was a significant association between the presence of left ventricular hypertrophy (LVH and right ventricular hypertrophy (RVH and between severe hypertensive cardiomyopathy and RVH. There was a predominance of RVH and LVH in men aged 60-79 years and a higher prevalence in the Brazilian mulatto and Black ethnic groups and in those with adequate nutritional status

  15. Genetics Home Reference: metatropic dysplasia

    Science.gov (United States)

    ... damage the spinal cord; either a sunken chest (pectus excavatum) or a protruding chest (pectus carinatum); and joint ... Disease InfoSearch: Metatrophic dysplasia MalaCards: metatropic dysplasia Nemours Children's Health System Orphanet: Metatropic dysplasia Patient Support and ...

  16. Cervical dysplasia - series (image)

    Science.gov (United States)

    ... to detect cervical cancer. Limited or early cervical cancer (carcinoma in situ, or cervical intraepithelial neoplasia, or dysplasia) requires treatment with ablation therapy, usually in the form of ...

  17. Serial right ventricular endomyocardial biopsy in rapid-onset severe heart failure due to giant cell myocarditis

    NARCIS (Netherlands)

    van Haelst, Paul L.; Brugemann, Johan; Diercks, Gilles F.; Suurmeijer, Albert; van Veldhuisen, Dirk J.

    2006-01-01

    Giant cell myocarditis (GCM) is a serious condition that warrants immediate diagnosis and treatment. It often presents as rapidly progressive heart failure and/or malignant ventricular arrhythmias. Here, we describe a 34-year-old patient with myasthenia gravis who presented with GCM 2 weeks after re

  18. A Rare Case of Pulmonary Atresia with Ventricular Septal Defect with a Right Sided Aortic Arch and a Calcified Pulmonary AVM Presenting in an Adult without Cyanosis

    Directory of Open Access Journals (Sweden)

    Devendra V. Kulkarni

    2014-01-01

    Full Text Available Pulmonary atresia with ventricular septal defect (PA-VSD with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly. Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early. We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

  19. Displasia arritmogénica del ventrículo derecho: reporte de un caso y revisión de la literatura

    Directory of Open Access Journals (Sweden)

    Gilberth Vásquez Esquivel

    2011-06-01

    Full Text Available La displasia arritmogénica del ventrículo derecho se caracteriza por atrofia y reemplazo fibroso y graso del miocardio, y arritmias ventriculares. Se reporta el caso de una mujer de 54 años que se presentó con choque circulatorio fatal, haciéndose el diagnostico patológico de displasia arritmogénica del ventrículo derecho. Se discuten las características clínicas, diagnóstico y manejo de esta cardiopatía potencialmente letal y aún poco comprendida.Arrhythmogenic Right Ventricular Dysplasia: A Case Report and Review of the Literature Arrhythmogenic right ventricular dysplasia is characterized by atrophy, myocardial fibrofatty replacement and ventricular arrhythmias. The case of a 54-year- old woman with fatal cardiogenic shock and a pathological diagnosis of arrhythmogenic right ventricular dysplasia is presented. The clinical characteristics, diagnosis and management of this potentially lethal, and still poorly understood, cardiopathy are discussed.

  20. Nitroprusside modulates pulmonary vein arrhythmogenic activity

    Directory of Open Access Journals (Sweden)

    Chen Yao-Chang

    2010-03-01

    Full Text Available Abstract Background Pulmonary veins (PVs are the most important sources of ectopic beats with the initiation of paroxysmal atrial fibrillation, or the foci of ectopic atrial tachycardia and focal atrial fibrillation. Elimination of nitric oxide (NO enhances cardiac triggered activity, and NO can decrease PV arrhythmogensis through mechano-electrical feedback. However, it is not clear whether NO may have direct electrophysiological effects on PV cardiomyocytes. This study is aimed to study the effects of nitroprusside (NO donor, on the ionic currents and arrhythmogenic activity of single cardiomyocytes from the PVs. Methods Single PV cardiomyocytes were isolated from the canine PVs. The action potential and ionic currents were investigated in isolated single canine PV cardiomyocytes before and after sodium nitroprusside (80 μM, using the whole-cell patch clamp technique. Results Nitroprusside decreased PV cardiomyocytes spontaneous beating rates from 1.7 ± 0.3 Hz to 0.5 ± 0.4 Hz in 9 cells (P Conclusion Nitroprusside regulates the electrical activity of PV cardiomyocytes, which suggests that NO may play a role in PV arrhythmogenesis.

  1. Segmentation of real-time three-dimensional ultrasound for quantification of ventricular function: a clinical study on right and left ventricles.

    Science.gov (United States)

    Angelini, Elsa D; Homma, Shunichi; Pearson, Gregory; Holmes, Jeffrey W; Laine, Andrew F

    2005-09-01

    Among screening modalities, echocardiography is the fastest, least expensive and least invasive method for imaging the heart. A new generation of three-dimensional (3-D) ultrasound (US) technology has been developed with real-time 3-D (RT3-D) matrix phased-array transducers. These transducers allow interactive 3-D visualization of cardiac anatomy and fast ventricular volume estimation without tomographic interpolation as required with earlier 3-D US acquisition systems. However, real-time acquisition speed is performed at the cost of decreasing spatial resolution, leading to echocardiographic data with poor definition of anatomical structures and high levels of speckle noise. The poor quality of the US signal has limited the acceptance of RT3-D US technology in clinical practice, despite the wealth of information acquired by this system, far greater than with any other existing echocardiography screening modality. We present, in this work, a clinical study for segmentation of right and left ventricular volumes using RT3-D US. A preprocessing of the volumetric data sets was performed using spatiotemporal brushlet denoising, as presented in previous articles Two deformable-model segmentation methods were implemented in 2-D using a parametric formulation and in 3-D using an implicit formulation with a level set implementation for extraction of endocardial surfaces on denoised RT3-D US data. A complete and rigorous validation of the segmentation methods was carried out for quantification of left and right ventricular volumes and ejection fraction, including comparison of measurements with cardiac magnetic resonance imaging as the reference. Results for volume and ejection fraction measurements report good performance of quantification of cardiac function on RT3-D data compared with magnetic resonance imaging with better performance of semiautomatic segmentation methods than with manual tracing on the US data.

  2. Postsystolic Shortening Is Associated with Altered Right Ventricular Function in Children after Tetralogy of Fallot Surgical Repair

    Science.gov (United States)

    2017-01-01

    and diastolic dysfunction in children after Tetralogy of Fallot surgical repair. Postsystolic shortening in the basal lateral segment is commonly seen in children after the Tetralogy of Fallot surgical repair, and is associated with altered right ventricular systolic and diastolic function. PMID:28046050

  3. Ventricular arrhythmias associated with long-term endurance sports: what is the evidence?

    Science.gov (United States)

    Heidbuchel, Hein; Prior, David L; La Gerche, André

    2012-11-01

    Athletic performance tests the limits of the human body and mind. Awe-inspiring achievements is what makes sports so fascinating. It is well appreciated however that top-level sports may sometimes overtax the body, and can lead to injuries, most notably of musculo-skeletal nature. This paper defends the thesis that the heart can also develop sports injuries at the ventricular level. We will elaborate on our hypothesis, originally put forward in 2003, that intense endurance activities put a particularly high strain on the right ventricle (RV), which over time, may lead to a proarrhythmic state resembling right (or less often) left ventricular cardiomyopathy. This can develop even in the absence of underlying demonstrable genetic abnormalities, probably just as a result of excessive RV wall stress during exercise. The syndrome of 'exercise-induced arrhythmogenic RV cardiomyopathy' may easily be overlooked. Sports cardiologists, like orthopaedic specialists, should be prepared to realise that excessive sports activity can lead to cardiac sports injuries in some, which will help to council on safe participation in all.

  4. [Dextrocardia with situ solitus, ventricular loop, double outlet hypoplastic right ventricle and L-malposition of the great arteries. Description and surgical treatment of a rare and complex cardiopathy].

    Science.gov (United States)

    Fraisse, A; Ghez, O; Ligi, I; Laporte-Giugliano, V; Chetaille, P; Bonnet, J L; Aubert, F; Metras, D

    2002-05-01

    Despite the cardiac surgery improvements allowing the correction of the majority of congenital heart diseases with ventricle-great vessels discontinuity, some abnormalities increase the risk of bi-ventricular reparation. We herein report the case of a patient presenting a rare form of double outlet right ventricle with a ventricular loop, with moderate right ventricle hypoplasia. L-malposition of great vessels and pulmonary artery stenosis, and for whom we opted for a palliative surgical treatment including a systemic-pulmonary anastomosis followed by a upper right bi-directional cavo-pulmonary derivation. The last surgery was followed by recurrent right pleural effusions disappearing after the embolization of the systemic-pulmonary anastomosis by catheterism as it probably obstructed the draining of the cavo-pulmonary anastomosis. The relevance of this clinical case reported is, firstly the description of this ventricle loop resulted from a marked ventricular malposition which is a rare heart disease, and secondly the discussion about the surgical treatment, especially about the choice between palliative and curative surgery. Only comparative studies on long term morbidity and mortality between the bi-ventricular reparation and mono-ventricular palliation will allow the selection of the most appropriate surgical treatment.

  5. Right ventricular dysfunction as an independent predictor of short- and long-term mortality in patients with heart failure

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Akkan, Dilek; Iversen, Kasper Karmark;

    2007-01-01

    by co-existing chronic obstructive pulmonary disease (COPD). AIMS: To examine the prognostic information from TAPSE adjusted for the potential confounding effects of co-existing cardiovascular and COPD in a large series of patients admitted for new onset or worsening HF. METHODS AND RESULTS: Eight...... in HF including left ventricular function. The co-existence of COPD is also associated with an adverse prognosis independent of the RV systolic function....

  6. One-year cardiac morphological and functional evolution following permanent pacemaker implantation in right ventricular septal position in chagasic patients

    Directory of Open Access Journals (Sweden)

    Otaviano da Silva Júnior

    2012-06-01

    Full Text Available INTRODUCTION: The septal position is an alternative site for cardiac pacing (CP that is potentially less harmful to cardiac function. METHODS: Patients with Chagas disease without heart failure submitted to permanent pacemaker (PP implantation at the Clinics Hospital of the Triângulo Mineiro Federal University (UFTM, were selected from February 2009 to February 2010. The parameters analyzed were ventricular remodeling, the degree of electromechanical dyssynchrony (DEM, exercise time and VO2 max during exercise testing (ET and functional class (NYHA. Echocardiography was performed 24 to 48h following implantation and after one year follow-up. The patients were submitted to ET one month postprocedure and at the end of one year. RESULTS: Thirty patients were included. Patient mean age was 59±13 years-old. Indication for PP implantation was complete atrioventricular (AV block in 22 (73.3% patients and 2nd degree AV block in the other eight (26.7%. All patients were in NYHA I and no changes occurred in the ET parameters. No variations were detected in echocardiographic remodeling measurements. Intraventricular dyssynchrony was observed in 46.6% of cases and interventricular dyssynchrony in 33.3% of patients after one year. CONCLUSIONS: The findings of this work suggest that there is not significant morphological and functional cardiac change following pacemaker implantation in septal position in chagasic patients with normal left ventricular function after one year follow-up. Thus, patients may remain asymptomatic, presenting maintenance of functional capacity and no left ventricular remodeling.

  7. 小鼠 PE右心室导管的制作及应用研究%Design and application research of PE right ventricular catheter for mice

    Institute of Scientific and Technical Information of China (English)

    徐刚; 高文祥; 陈德伟; 李晓栩; 刘福玉; 黄瑊; 高钰琪

    2014-01-01

    目的:建立一种制作简单、成本低廉、性能稳定的小鼠右心室压力检测方法。方法选取一段长度约为15 cm 的PE-50导管(外径:0.9 mm ,内径:0.5 mm),将一端制成适宜弧度,另一端插入7号注射器针头连接压力换能器。选取80只SPF级雄性C57BL/6小鼠,用自制PE右心室导管行右侧颈外静脉插管至右心室,记录成功例数和每次操作时间。另取40只SPF级雄性C57BL/6小鼠随机分为对照组和慢性低压低氧组,每组20只。慢性低压低氧组小鼠在模拟海拔5000 m低压舱内不间断喂养4周,对照组在舱外同时喂养。模型复制成功后,用自制PE右心室导管行右心室插管,记录右心室收缩压;分离左、右心室并称质量,计算Hermann-Willson指数并与右心室收缩压进行相关性分析。结果采用此自制PE右心室导管,右心室插管成功率为90%(72/80),从分离血管至检测出右心室波形所需时间每只3~5 min。慢性低压低氧组右心室收缩压(39.52±4.34)mm Hg和Hermann-Willson指数0.356±0.039均显著高于对照组(21.24±2.70)mm Hg、0.256±0.020,二者呈显著正相关(P<0.01)。结论采用本法检测右心室压力,制作简单,使用方便快捷,成功率高,性能稳定且成本低廉,可以推广应用。%Objective To establish a simple ,low cost and stable method to detect right ventricular pressure in mice .Methods A PE-50 duct length 15 cm(outside diameter :0 .9 mm ,inner diameter :0 .5 mm) was bent on one terminal and the other terminal was inserted into a 7# syringe needle to connect to a pressure transducer .This duct was intubated into right ventricle via right external jugular vein to detect right ventricular pressure in 80 SPF grade male C57BL/6 mice .Successful cases and operation time were re-corded .Besides ,40 SPF grade male C57BL/6 mice were randomized into the control group (n=20) and chronic

  8. Assessment of right ventricular outflow tract in children by two-dimensional echocardiography using a new subcostal view. Angiocardiographic and morphologic correlative study.

    Science.gov (United States)

    Isaaz, K; Cloez, J L; Danchin, N; Marçon, F; Worms, A M; Pernot, C

    1985-09-15

    Evaluation of the right ventricular (RV) outflow tract in congenital heart disease is extremely important for surgical management. Therefore, the value of 2-dimensional echocardiography (2-D echo) to assess the RV outflow tract was studied using a new approach: the subcostal elongated right oblique view. Twenty normal children and 49 children with congenital heart disease, aged 1 day to 11 years, were studied. Significant pulmonary infundibular obstruction was present in 22 patients with conotruncal malformations. To obtain the subcostal elongated right oblique view from the short-axis view at the aortic valve level, the transducer was slightly rotated clockwise with an anterior angulation of about 30 degrees so that the ascending aorta was seen in its long axis, providing an image similar to that obtained by a right ventriculogram in the elongated right anterior oblique view. The deviation of infundibular septum was appreciated by measurement of the angle alpha, defined by the long axis of the infundibular septum and the plane of aortic cusps. This view could be obtained in 64 patients (92%). In correlation with angiographic or anatomic data, the subcostal elongated right oblique view permitted recognition of several types of RV outflow tract: type I--normally formed RV outflow tract; type II--disorganized RV outflow tract with obstruction (alpha less than 90 degrees); type III and IV--disorganized RV outflow tract with obstruction (alpha greater than 90 degrees). This view could visualize the crista supraventricularis in type I, but also the anatomic components of RV outflow tract that may contribute to obstruction in the other types: infundibular septum, septoparietal trabeculations and trabecula septomarginalis.(ABSTRACT TRUNCATED AT 250 WORDS)

  9. 频发右心室流出道室性期前收缩对右心室流出道结构影响的研究%Effect of frequent idiopathic ventricular premature contractions from right ventricular outflow tract on structure of right ventricular outflow tract

    Institute of Scientific and Technical Information of China (English)

    孔强; 史力生; 张钰聪; 陈宇嘉; 张帆; 徐东

    2012-01-01

    目的 探讨右心室流出道(RVOT)起源频发室性期前收缩对RVOT结构的影响.方法 选取2009~2011年行射频消融治疗的频发RVOT起源室性期前收缩患者30例,分析其心电图特征、动态心电图、心脏彩色超声结果及术中精确定位,分析室性期前收缩对RVOT结构的影响.结果 射频消融术前RVOT直径为(31.76±3.33)mm,术后6个月为(30.93±2.68)mm(P<0.01);相关性分析显示:RVOT直径与室性期前收缩负荷呈正相关(r=0.484,P<0.05).RVOT间隔部来源室性期前收缩QRS时限为(157.69±18.33) ms,游离壁来源室性期前收缩QRS时限为(179.23±16.05)ms(P<0.01),QRS时限与来源部位相关(r=0.566,P<0.01).室性期前收缩QRS时限与RVOT直径无相关性(r=0.097,P>0.05).结论 RVOT来源室性期前收缩经射频消融治疗后,RVOT直径有减小的趋势,其与室性期前收缩负荷呈正相关,与室性期前收缩形态无相关性.%Objective To investigate the effect of frequent ventricular premature contractions(VPCs) from right ventricular outflow tract (RVOT) on the structure of right ventricular outflow tract. Methods 30 patients with frequent VPCs from RVOT who underwent radiofrequency ablation from 2009 to 2011 were enrolled. The site of origin of VPCs was identified by the characters of ECG according to the stepwise electrocardiographic algorithm and the accurate anatomical location was identified by 3D electroanatomical non-contact mapping. Parameters of haemodynamic were accessed by means of transthoracic echocardiography before and 6 months after intervention. The average daily amount of VPCs was evaluated by Holter and the ECG characters of VPCs were also analyzed. Results Before and 6 months after intervention, the diameter of RVOT was (31. 76 ±3. 33) mm and (30. 93 ±2. 68) mm( P 0. 05). Conclusion The diameter of RVOT decreased after radiofrequency ablation for the patients with VPCs from RVOT and it correlated positively with the average daily

  10. Cardiac MR imaging in arrhythmogenic heart diseases; Kardiale MRT in der Diagnostik arrhythmogener Herzerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Boehm, C.K.; Dinter, D.J.; Diehl, S.J.; Neff, K.W. [Universitaetsklinikum Mannheim, Institut fuer Klinische Radiologie, Mannheim (Germany); Papavassiliu, T.; Borggrefe, M. [Universitaetsklinikum Mannheim, Medizinische Klinik, Mannheim (Germany)

    2007-04-15

    Cardiac arrhythmias are assessed with a combination of history, clinical examination, electrocardiogram, Holter monitor, if necessary supplemented by invasive cardiac electrophysiology. In ischemic heart disease (IHD) coronary angiography is performed in addition. Echocardiography is usually the primary imaging modality. MRI is increasingly recognized as an important investigation allowing more accurate cardiac morphological and functional assessment. Approximately one-fifth of deaths in Western countries are due to sudden cardiac death, 80% of which are caused by arrhythmias. Typical causes range from diseases with high prevalence (IHD in men 30%) to myocarditis (prevalence 1-9%) and rare cardiomyopathies (prevalence HCM 0.2%, ARVC 0.02%, Brugada syndrome approx. 0.5%). The characteristic MRI features of arrhythmogenic diseases and the new aspects of characteristic distribution of late enhancement allow etiologic classification and differential diagnosis. MRI represents an important tool for detection of the underlying cause and for risk stratification in many diseases associated with arrhythmias. (orig.) [German] Herzrhythmusstoerungen werden durch die Zusammenschau von Anamnese, klinischer Untersuchung, Elektrokardiogramm, Langzeit-EKG sowie ggf. einer invasiven elektrophysiologischen Untersuchung beurteilt. Bei der koronaren Herzerkrankung (KHK) erfolgt zusaetzlich eine Koronarangiographie. Die Echokardiographie stellt das primaere bildgebende Verfahren dar. Die MRT des Herzens ermoeglicht eine genauere morphologische und funktionelle Darstellung des Herzens und gewinnt damit zunehmend an Bedeutung. Etwa jeder 5. Todesfall in westlichen Industriestaaten ist auf einen ploetzlichen Herztod zurueckzufuehren, davon sind ca. 80% durch Herzrhythmusstoerungen verursacht. Typische Ursachen reichen von Krankheiten mit hoher Praevalenz (KHK bei Maennern 30%) ueber Myokarditiden (Praevalenz 1-9%) bis zu selteneren Kardiomyopathien (Praevalenz HCM 0,2%, ARVC 0,02%, Brugada

  11. Role of temporary pacing at the right ventricular outflow tract in anesthetic management of a patient with asymptomatic sick sinus syndrome.

    Science.gov (United States)

    Nag, Kusha; Nagella, Amrutha Bindu; Kumar, V R Hemanth; Singh, Dewan Roshan; Ravishankar, M

    2015-01-01

    A 60-year-old woman posted for percutaneous nephrolithotomy with ureterolithotripsy was found to have a history of hypertension and ischemic heart disease from past 6 months on regular treatment. Pulse rate was irregularly irregular in a range of 56-60/min, unresponsive to atropine, with a sinus pause on the electrocardiogram. Although the patient was asymptomatic, anticipating unmasking of the sick sinus syndrome during general anesthesia in the prone position, a temporary pacemaker was implanted at right ventricular outflow tract (RVOT) septum before the scheduled surgery. A balanced anesthesia technique with endotracheal intubation was administered. There were several episodes of continuous pacing by the temporary pacemaker intraoperatively, which may be attributed to unmasking of the sinus node dysfunction due to general anesthesia. At the end of surgery, patient was extubated after adequate reversal from neuromuscular blockade. Postoperative period remained uneventful, and the pacemaker wires were removed on the 2(nd) postoperative day. With this case report, we highlight the importance of inserting a temporary pacemaker prior to anesthesia even in an asymptomatic patient if a sinus node dysfunction is suspected preoperatively and if intraoperative access to transvenous pacing is difficult such as in prone position. Pacing at RVOT septum minimizes ventricular dyssynchrony and improves hemodynamic parameters.

  12. The dynamic study of the pulmonary artery obstruction degree and the right ventricular function in massive pulmonary embolism on CT pulmonary angiography

    Institute of Scientific and Technical Information of China (English)

    Jianguo Wang; Yulin Guo; Xiaojuan Guo; Min Liu; Youmin Guo; Chen Wang; Yuanhua Yang; Zhenguo Zhai; Li Zhu; Hongxia Ma

    2008-01-01

    Objective: To analyze the value of CTPA in assessing the dissolve of embolus and the function of the fight ventricle dynamically.Methods:Twenty-three cases of massive pulmonary embolism were analyzed retrospectively. The pulmonary artery obstruction index and the fight ventricular function parameters were collected and analyzed on CTPA before thrombolytic therapy, 24 hours and 14 days after therapy, respectively. Results:The pulmonary artery obstruction index decreased gradually, and there was significant difference before therapy, 24 hours and 2 weeks after therapy. Twenty-four hours after therapy, the maximal short axes diameter and the maximal transverse area of fight ventricle(RVd, RVs) decreased significantly, the maximal short axes diameter and the maximal transverse area of left ventricle(LVd, LVs) increased significantly, and the RVd/LVd, RVs/LVs decreased apparently. The pulmonary artery symbolic pressure before and 24 hours after therapy were apparently different. There was no significant difference between azygos vein, the super vena cava, the main pulmonary artery and vein reflux before and after therapy. Conclusion:CTPA can evaluate the pulmonary artery obstruction degree and right ventricular function dynamically.

  13. What does the time constant of the pulmonary circulation tell us about the progression of right ventricular dysfunction in pulmonary arterial hypertension?

    Science.gov (United States)

    Bellofiore, A; Wang, Z; Chesler, N C

    2015-06-01

    Compliance (C) and resistance (R) maintain a unique, inverse relationship in the pulmonary circulation, resulting in a constant characteristic time [Formula: see text] that has been observed in healthy subjects as well as patients with pulmonary arterial hypertension (PAH). However, little is known about the dependence of right ventricular (RV) function on the coupled changes in R and C in the context of this inverse relationship. We hypothesized three simple dependencies of RV ejection fraction (RVEF) on R and C. The first model (linear-R) assumes a linear RVEF-R relation; the second (linear-C) assumes a linear RVEF-C relation; and the third one combines the former two in a mixed linear model. We found that the linear-R model and the mixed linear model are in good agreement with clinical evidence. A conclusive validation of these models will require more clinical data. Longitudinal data in particular are needed to identify the time course of ventricular-vascular impairment in PAH. Simple models like the ones we present here, once validated, will advance our understanding of the mechanisms of RV failure, which could improve strategies to manage RV dysfunction in PAH.

  14. Quantitative assessment of right ventricular function and pulmonary regurgitation in surgically repaired tetralogy of Fallot using 256-slice CT: comparison with 3-Tesla MRI

    Energy Technology Data Exchange (ETDEWEB)

    Yamasaki, Yuzo; Yonezawa, Masato; Matsuo, Yoshio; Kamitani, Takeshi; Higuchi, Ko; Honda, Hiroshi [Graduate School of Medical Sciences, Kyushu University, Department of Clinical Radiology, Higashi-ku, Fukuoka (Japan); Nagao, Michinobu; Kawanami, Satoshi [Graduate School of Medical Sciences, Kyushu University, Department of Molecular Imaging and Diagnosis, Higashi-ku, Fukuoka (Japan); Yamamura, Kenichiro [Graduate School of Medical Sciences, Kyushu University, Department of Pediatrics, Higashi-ku, Fukuoka (Japan); Sakamoto, Ichiro [Graduate School of Medical Sciences, Kyushu University, Department of Cardiovascular Medicine, Higashi-ku, Fukuoka (Japan); Shiokawa, Yuichi [Graduate School of Medical Sciences, Kyushu University, Department of Cardiovascular Surgery, Higashi-ku, Fukuoka (Japan); Yabuuchi, Hidetake [Graduate School of Medical Sciences, Kyushu University, Department of Health Sciences, Higashi-ku, Fukuoka (Japan)

    2014-12-15

    To compare 256-slice cardiac computed tomography (CCT) with cardiac magnetic resonance (CMR) imaging to assess right ventricular (RV) function and pulmonary regurgitant fraction (PRF) in patients with repaired tetralogy of Fallot (TOF). Thirty-three consecutive patients with repaired TOF underwent retrospective ECG-gated CCT and 3-Tesla CMR. RV and left ventricular (LV) end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) were measured using CCT and CMR. PRF-CCT (%) was defined as (RVSV - LVSV)/RVSV. PRF-CMR (%) was measured by the phase-contrast method. Repeated measurements were performed to determine intra- and interobserver variability. CCT measurements, including PRF, correlated highly with the CMR reference (r = 0.71-0.96). CCT overestimated RVEDV (mean difference, 17.1 ± 2.9 ml), RVESV (12.9 ± 2.1 ml) and RVSV (4.2 ± 2.0 ml), and underestimated RVEF (-2.6 ± 1.0 %) and PRF (-9.1 ± 2.0 %) compared with CMR. The limits of agreement between CCT and CMR were in a good range for all measurements. The variability in CCT measurements was lower than those in CMR. The estimated effective radiation dose was 7.6 ± 2.6 mSv. 256-slice CCT can assess RV function and PRF with relatively low dose radiation exposure in patients with repaired TOF, but overestimates RV volume and underestimates PRF. (orig.)

  15. Spondylo-costal dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.

    1984-02-01

    Fourteen patients with spondylo-costal dysplasia were analysed. 3 of them presented without obvious associated anomalies ''pure'' spondylo-costal dysplasias; 2 had several components consistent with Vater (Vacterl)-Association; 2 showed malformations which are often encountered in Vater (Vacterl)-Association; 4 presented with minor malformations; 3 had major associated malformations rarely seen in Vater (Vacterl)-Association. Thoracic spine and costal malsegmentation can be sporadically observed in other ''errors in septation complex'' (axial mesodermal dysplasia) including severe myelomeningocoele and diastematomyelia.

  16. Right ventricular systolic function in patients with COPD by echocardio-graphy%超声心动图在慢性阻塞性肺疾病患者右心收缩功能中的评估价值

    Institute of Scientific and Technical Information of China (English)

    尤雷英; 杨永红

    2015-01-01

    Objective:To investigate the value of right ventricular systolic function in patients with chronic obstructive pulmona-ry disease( COPD) by echocardiography. Methods:56 patients with COPD and 53 healthy individuals in our hospital from June 2012 to June 2014 were chosen,respectively divided into COPD group and the control group. Right ventricular systolic function and structures were analyzed between the two groups by the performance of echocardiography. Results:The RA-1,RA-2,RA-3 and right ventricular outflow tract(RVOT)inner diameter in the COPD group were higher than those in the control group(P<0. 05);The right ventricular wall thickness(RVWT)in the COPD group was wider than that in the control group(P<0. 05);The tricuspid annular planesystolic ex-cursion(TAPSE)in the COPD group was lower than that in the control group(P<0. 05);The right ventricular myocardial performance index( RV-MPI) and longitudinal strain of right ventricular lateral wall basal segment( RV-strain) in the COPD group were higher than those in the control group(P<0. 05). Conclusion:The echocardiography can assess the systolic function and structure of right heart quantitatively. Right heart size of COPD patients is greater, and systolic and diastolic function has decreased. Therefore, echocardio-graphy can be used for early diagnosis of COPD.%目的::探讨超声心动图在评估慢性阻塞性肺疾病( chronic obstructive pulmonary disease,COPD)患者右心收缩功能中的临床价值。方法:选取2012年6月至2014年6月因COPD在我院诊治的患者56例设为COPD组及同期在我院体检的健康者53例设为对照组,通过超声心动图的表现情况分析两组间右心收缩功能。结果: COPD组右心房上下径、右心房左右径、右心房面积及右心室流出道近端( right ventricular outflow tract,RVOT)内径均大于对照组,差异有统计学意义( P <0.05);COPD组右心室侧壁厚度(right ventricular wall thickness,RVWT)厚于对照

  17. Localization of precise origin of idiopathic ventricular tachycardia from the right ventricular outflow tract by a 12-lead ECG: a study of pace mapping using a multielectrode "basket" catheter.

    Science.gov (United States)

    Yoshida, Y; Hirai, M; Murakami, Y; Kondo, T; Inden, Y; Akahoshi, M; Tsuda, M; Okamoto, M; Yamada, T; Tsuboi, N; Hirayama, H; Ito, T; Toyama, J; Saito, H

    1999-12-01

    Radiofrequency catheter ablation guided by pace-mapping techniques has proven effective in eliminating idiopathic ventricular tachycardia (VT) originating from the right ventricular outflow tract (RVOT). A method for rapidly identifying the origin of VT from 12-lead electrocardiogram (ECG) findings would be helpful for the catheter ablation procedure. The purpose of this study is to precisely localize the origin of idiopathic VT from the RVOT by a 12-lead ECG from a study of multipoint pace mapping. In one patient with premature ventricular complex (PVC) and 3 with VT, a "basket" catheter was deployed in the RVOT for bipolar pacing from 56 sites in the endocardium of RVOT. Under fluoroscopy the pacing sites were classified into the septum and free wall. We investigated the QRS morphology in leads, I, II, and III; the depth of the QS wave in leads aVR and aVL; and the height of the initial r wave in leads V1 and V2. Pacing was captured in 30-47 of 56 sites (54%-84%). As the pacing sites changed from the anterior to posterior of the septum, the QS notch (-) type in lead I shifted through rs to R, while a shift from R type to rR' or Rr' was noted in leads II and III. As the pacing sites changed from the anterior to posterior of the free wall, lead I showed a shift from the QS notch (+) type to R, while a shift from rR' to Rr' (or rR' unchanged) was found in leads II and III. The depth of the QS wave in leads aVR and aVL showed a tendency for aVR to be deeper than aVL toward the posterolateral attachment of both the septum and free wall, whereas aVL tended to be deeper than aVR toward the anterior attachment. The initial r waves in V1 and V2 became greater as the pacing site was positioned at a higher or more posterior location. These findings may provide more precise and clinically useful diagnostic information on the site of the origin of idiopathic VT originating from the RVOT by a 12-lead ECG.

  18. Right ventricular outflow tract stenting in a low birth weight infant born with tetralogy of fallot and prostaglandin e1 dependency.

    Science.gov (United States)

    Bang, Sunhee; Ko, Hong Ki; Yu, Jeong Jin; Han, Myung-Ki; Kim, Young-Hwue; Ko, Jae-Kon; Park, In-Sook

    2011-12-01

    Surgical skill and strategy for the correction of tetralogy of Fallot (TOF) have improved and resulted in satisfactory outcomes. However, prematurity and low birth weight continue to remain risk factors for poor outcomes. We present a case of a 2,150 g neonate born with TOF, in whom palliation was achieved with right ventricular outflow tract (RVOT) stenting. Seventy-seven days after the procedure, stenosis of RVOT below the stent was identified. At that time his body weight was 4.9 kg and total corrective surgery was deemed feasible. Eight months following surgical repair, the patient remained well without medical intervention. RVOT stenting may be a viable interim procedure while waiting for a low birth weight neonate born with TOF and prostaglandin E1 dependency to reach optimal weight to undergo corrective surgery.

  19. Assessment of right ventricular myocardium motion in patient with DDD pacing using VVI%速度向量成像观察双腔起搏前后右心室运动状态

    Institute of Scientific and Technical Information of China (English)

    周春蕾; 许迪; 陆凤翔; 江立红; 郑真; 施斌斌

    2011-01-01

    Objective To assess right ventricular myocardium motion in patients with dual chamber pacing (DDD) using velocity vector imaging (VVI), and to explore the clinical application value of VVI in evaluating right ventricular motion. Methods 23 patients before and after DDD pacemaker implantation were enrolled in this study. The peak systolic displacement of right ventricular segments was measured with VVI.The difference between pre and post pacemaker implantation was analyzed. Results Right ventricular displacement before and after DDD pacing was significantly decreasing from basal, mid to apical segments ( P <0.01 ). Compared with the values at the baseline, the displacement of interventricular septum, free wall and right ventricle with pacing was decreased (P < 0.01 ). Conclusions Right ventricular displacement with pacing was significantly decreased compared with the values at the baseline. VVI can accurately assess right ventricular systolic displacement in patients with DDD pacing, and objectively reflect right ventricular myocardial motion and function.%目的 探讨速度向量成像(VVI)技术分析双腔(DDD)起搏前后右心室心肌运动位移的变化规律,初步探讨VVI技术在评价右心室运动方面的应用价值.方法 23例DDD起搏患者术前术后均采用VVI技术检测右心室室壁各节段心肌收缩期纵向运动位移,并比较术前术后之差异.结果 术前术后右心室各室壁不同节段心肌收缩峰值位移表现为从基底段到心尖段依次降低,其中基底段与心尖段的差异有统计学意义(P<0.01);与术前相比,术后后间隔、游离壁及右心室整体位移较术前显著降低,差异有统计学意义(P<0.01).结论 DDD起搏后右心室心肌运动位移较术前明显降低;VVI可精确测量DDD起搏前后右心室心肌位移的程度,从而客观反映右心室之运动状态,进而判断右心室之功能.

  20. Adaptive right ventricular performance in response to acutely increased afterload in a lamb model of congenital heart disease: evidence for enhanced Anrep effect.

    Science.gov (United States)

    Johnson, Rebecca C; Datar, Sanjeev A; Oishi, Peter E; Bennett, Stephen; Maki, Jun; Sun, Christine; Johengen, Michael; He, Youping; Raff, Gary W; Redington, Andrew N; Fineman, Jeffrey R

    2014-04-15

    Patients with pulmonary hypertension associated with congenital heart disease survive longer with preserved right ventricular (RV) function compared with those with primary pulmonary hypertension. The purpose of this study was to test the hypothesis that superior RV performance can be demonstrated, at baseline and when challenged with increased RV afterload, in lambs with chronic left-to-right cardiac shunts compared with control lambs. A shunt was placed between the pulmonary artery and the aorta in fetal lambs (shunt). RV pressure-volume loops were obtained 4 wk after delivery in shunt and control lambs, before and after increased afterload was applied using pulmonary artery banding (PAB). Baseline stroke volume (8.7 ± 1.8 vs. 15.8 ± 2.7 ml, P = 0.04) and cardiac index (73.0 ± 4.0 vs. 159.2 ± 25.1 ml·min(-1)·kg(-1), P = 0.02) were greater in shunts. After PAB, there was no difference in the change in cardiac index (relative to baseline) between groups; however, heart rate (HR) was greater in controls (168 ± 7.3 vs. 138 ± 6.6 beats/min, P = 0.01), and end-systolic elastance (Ees) was greater in shunts (2.63 vs. 1.31 × baseline, P = 0.02). Control lambs showed decreased mechanical efficiency (71% baseline) compared with shunts. With acute afterload challenge, both controls and shunts maintained cardiac output; however, this was via maladaptive responses in controls, while shunts maintained mechanical efficiency and increased contractility via a proposed enhanced Anrep effect-the second, slow inotropic response in the biphasic ventricular response to increased afterload, a novel finding in the RV. The mechanisms related to these physiological differences may have important therapeutic implications.

  1. Lamin A/C mutation affecting primarily the right side of the heart

    Directory of Open Access Journals (Sweden)

    Laura Ollila

    2013-04-01

    Full Text Available LMNA mutations are amongst the most important causes of familial dilated cardiomyopathy. The most important cause of arrhythmogenic right ventricular cardiomyopathy (ARVC is desmosomal pathology. The aim of the study was to elucidate the role of LMNA mutations among Finnish cardiomyopathy patients. We screened 135 unrelated cardiomyopathy patients for LMNA mutations. Because of unusual phenotype, two patients were screened for the known Finnish ARVC-related mutations of desmosomal genes, and their Plakophilin-2b gene was sequenced. Myocardial samples from two patients were examined by immunohistochemical plakoglobin staining and in one case by electron microscopy. We found a new LMNA mutation Phe237Ser in a family of five affected members with a cardiomyopathy affecting primarily the right side of the heart. The phenotype resembles ARVC but does not fulfill the Task Force Criteria. The main clinical manifestations of the mutation were severe tricuspid insufficiency, right ventricular enlargement and failure. Three of the affected patients died of the heart disease, and the two living patients received heart transplants at ages 44 and 47. Electron microscopy showed nuclear blebbing compatible with laminopathy. Immunohisto - chemical analysis did not suggest desmosomal pathology. No desmosomal mutations were found. The Phe237Ser LMNA mutation causes a phenotype different from traditional cardiolaminopathy. Our findings suggest that cardiomyopathy affecting primarily the right side of the heart is not always caused by desmosomal pathology. Our observations highlight the challenges in classifying cardiomyopathies, as there often is significant overlap between the traditional categories.

  2. Cervical deciduosis imitating dysplasia.

    Science.gov (United States)

    van Diepen, Diederik Anthony; Hellebrekers, Bart; van Haaften, Anne-Marie; Natté, Remco

    2015-09-22

    Ectopic cervical deciduosis is generally an accidental finding during pregnancy, and usually presents without any symptoms or need for therapeutic intervention. However, it can sometimes imitate dysplasia or carcinoma. We report a case of a 34-year-old G2P0, with a history of cervical dysplasia, presenting at 11 weeks of gestation, with vaginal blood loss. During examination, lesions mimicking dysplasia were found on the cervix. Histological examination reported cervical deciduosis. Deciduosis is a benign change during pregnancy and will resolve spontaneously. With the increasing use of cytology and colposcopy, the reported incidence is growing. When it is hard to differentiate between dysplasia and deciduosis, histological confirmation should be considered.

  3. Right Ventricular Function After Acute Myocardial Infarction Treated With Primary Percutaneous Coronary Intervention (from the Glycometabolic Intervention as Adjunct toPrimary Percutaneous Coronary Intervention in ST-Segment Elevation Myocardial Infarction III Trial)

    NARCIS (Netherlands)

    Gorter, Thomas M; Lexis, Chris P H; Hummel, Yoran M; Lipsic, Erik; Nijveldt, Robin; Willems, Tineke P; van der Horst, Iwan C C; van der Harst, Pim; van Melle, Joost P; van Veldhuisen, Dirk J

    2016-01-01

    Right ventricular (RV) dysfunction is a powerful risk marker after acute myocardial infarction (MI). Primary percutaneous coronary intervention (PCI) has markedly reduced myocardial damage of the left ventricle, but reliable data on RV damage using cardiac magnetic resonance imaging (MRI) are scarce

  4. Gracile bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)