Familial Case of Arrhythmogenic Right Ventricular Cardiomyopathy with Desmoplakin Mutation Diagnosed by Right Heart Failure

2014 ◽  
Vol 20 (10) ◽  
pp. S188
Author(s):  
Takahiro Kamada ◽  
Takashi Konishi ◽  
Mitiya Tachiiri ◽  
Yukiko Matsumura ◽  
Tihiro Ota ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Heart Failure ◽  
Familial Case ◽  
Ventricular Cardiomyopathy ◽  
Right Heart

scholarly journals Arrhythmogenic right ventricular cardiomyopathy in a dog : case report

2000 ◽  
Vol 71 (2) ◽  
Author(s):  
A.J. Möhr ◽  
R.M. Kirberger
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Clinical Signs ◽  
Right Heart Failure ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Ventricular Enlargement ◽  
Ventricular Cardiomyopathy ◽  
Right Heart

An 8-month-old Labrador retriever bitch was evaluated for sudden-onset, progressive abdominal distension. Physical examination revealed an exaggerated inspiratory effort, severe ascites, bilateral jugular vein distension, and hypokinetic femoral arterial pulses. Thoracic auscultation detected tachycardia with muffled heart sounds, without audible cardiac murmurs. Thoracic radiographs identified severe right ventricular enlargement and pleural effusion. The electrocardiogram was consistent with incomplete right bundle branch block or right ventricular enlargement. Echocardiography demonstrated severe right ventricular and atrial dilation, secondary tricuspid regurgitation, and thinning and hypocontractility of the right ventricular myocardium. Left heart chamber sizes were slightly decreased, with normal left ventricular contractility. Adiagnosis of arrhythmogenic right ventricular cardiomyopathy was reached, based on the characteristic clinical, electrocardiographic, radiographic and echocardiographic findings, and the exclusion of other causes of isolated right ventricular failure. Treatment effected good control of clinical signs, until acutely decompensated congestive right heart failure led to euthanasia after 4 months. Arrhythmogenic right ventricular cardiomyopathy is a well-described clinical entity in humans, and has previously been documented in 3 male dogs. The condition is characterised by progressive fibro-adipose replacement of right ventricular myocardium, while the left ventricle usually remains unaffected. It should be considered a differential diagnosis in any young dog presented with isolated right heart failure, syncope, or unexplained ventricular tachyarrhythmias. This article reports the 1st case of arrhythmogenic right ventricular cardiomyopathy in a female dog, and highlights its echocardiographic features.

Evidence-based management of arrhythmogenic right ventricular cardiomyopathy in pregnancy

2020 ◽  
Author(s):  
Jagjit Khosla ◽  
Reshma Golamari ◽  
Alice Cai ◽  
Jamal Benson ◽  
Wilbert S Aronow ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Retrospective Studies ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Genetic Disorder ◽  
Ventricular Myocardium ◽  
Evidence Based ◽  
Ventricular Cardiomyopathy ◽  
Genes Encoding ◽  
In Pregnancy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder resulting in fibrofatty replacement of the myocardium. Genetic mutations in genes encoding for desmosome proteins result in a ventricular myocardium prone to arrhythmias and heart failure. Although ARVC is known for a few decades, most of the outcomes in pregnancy are reported recently. Pregnancy leads to significant physiological changes with excess mechanical stress on the myocardium. All the retrospective studies suggest that pregnancy is well tolerated in these patients despite the high risk of arrhythmias and heart failure. Our review focuses on the most up-to-date evidence on the management of ARVC patients during the antepartum and postpartum period.

scholarly journals Usefulness of right ventricular contraction pressure index to predict short-term mortality and right heart failure in patients who underwent continuous-flow left ventricular assist device implantation

2019 ◽  
Vol 43 (1) ◽  
pp. 25-36 ◽  
Author(s):  
Koray Kalenderoğlu ◽  
Tolga Sinan Güvenç ◽  
Müge Taşdemir Mete ◽  
Hüseyin Kuplay ◽  
Semra Ağustos ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Right Heart Failure ◽  
Left Ventricular ◽  
Ventricular Contraction ◽  
Short Term ◽  
Right Heart ◽  
Ventricular Assist ◽  
Pressure Index ◽  
Short Term Mortality

Background: Right ventricular stroke work index is a useful but invasively measured parameter that can be used to predict right heart failure following continuous-flow left ventricular assist device implantation. Right ventricular contraction pressure index is a novel parameter that was developed to measure right ventricular stroke work index with echocardiography. We aimed to investigate the clinical usefulness of right ventricular contraction pressure index to predict short-term mortality and right heart failure in patients who underwent continuous-flow left ventricular assist device implantation. Methods: A total of 49 patients who participated in institutional advanced heart failure registry and underwent continuous-flow left ventricular assist device implantation with a bridge-to-candidacy indication were analyzed retrospectively. Right ventricular contraction pressure index was calculated using offline measurements. Demographic, clinical and outcome data were obtained from the registry data. Patients were grouped according to right ventricular contraction pressure index quartiles. Results: Patients within the lowest right ventricular contraction pressure index quartile had a trend toward higher short-term mortality (46.2%, p = 0.056) and combined short-term mortality and definitive right heart failure (53.8%, p = 0.054) at 15th day postoperatively. Similarly, short-term survival or survival free of definite right heart failure were significantly lower in the lowest right ventricular contraction pressure index quartile (log-rank p = 0.045 and log-rank p = 0.03, respectively). In a proportional hazards model that included echocardiographic parameters, right ventricular contraction pressure index was an independent predictor for short-term mortality (odds ratio: 6.777, 95% confidence interval: 1.118–41.098, p = 0.037), but not for combined short-term mortality and definite right heart failure. No such associations were found for long-term mortality. Right ventricular contraction pressure index had a statistically significant correlation with invasively measured pulmonary capillary wedge pressure, pulmonary vascular resistance, mean pulmonary pressure, and right ventricular stroke work index. Conclusion: Right ventricular contraction pressure index was found as a useful parameter for determining short-term postoperative mortality in patients undergoing continuous-flow left ventricular assist device implantation.

scholarly journals Metastatic adenocarcinoma involving the right ventricle and pulmonary artery leading to right heart failure: case report

2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Ventricular Outflow Tract ◽  
Right Heart Failure ◽  
Ventricular Outflow Tract ◽  
Right Heart ◽  
The Right

ABSTRACT CONTEXT: Obstruction of the right ventricular outflow tract due to metastatic disease is rare. Clinical recognition of cardiac metastatic tumors is rare and continues to present a diagnostic and therapeutic challenge. CASE REPORT: We present the case of a patient who had severe respiratory insufficiency and whose clinical examinations revealed a giant tumor mass extending from the right ventricle to the pulmonary artery. We discuss the diagnostic and therapeutic options. CONCLUSION: In patients presenting with acute right heart failure, right ventricular masses should be kept in mind. Transthoracic echocardiography appears to be the most easily available, noninvasive, cost-effective and useful technique in making the differential diagnosis.

The Hemodynamic Characteristics of Right Ventricle After Gradient Pulmonary Artery Banding in Rats

2020 ◽  
Author(s):  
Song Jiyang ◽  
Wan Nan ◽  
Shen Shutong ◽  
Wei Ying ◽  
Cao Yunshan
Keyword(s):  
Heart Failure ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Heart Failure ◽  
Therapeutic Strategies ◽  
Pulmonary Artery Banding ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
The Right

Abstract Background: Right ventricular (RV) failure induced by sustained pressure overload is a major contributor to morbidity and mortality in several cardiopulmonary disorders. Reliable and reproducible animal models of RV failure are important in order to investigate disease mechanisms and effects of potential therapeutic strategies. To establish a rat model of RV failure perfectly, we observed the right ventricle and carotid artery hemodynamics characteristics in different degrees of pulmonary artery banding of rats of different body weights. Methods: Rats were subjected to 6 groups：control(0%, n=5)(pulmonary arterial banding 0%), PAB(1-30%, n=4)(pulmonary arterial banding1-30%), PAB(31-60%, n=6)(pulmonary arterial banding31-60%),PAB(61-70%, n=5)(pulmonary arterial bandin61-70%), PAB(71-80%,n=4)(pulmonary arterial banding71-80%), PAB(100%, n=3)(pulmonary arterial banding 100%). We measured the right ventricular pressure(RVP) by right heart catheterization when the pulmonary arterial was ligated. Results: The RVP gradually increased with increasing degree of banding, but when occlusion level exceeding 70%, high pressure state can be only maintained for a few minutes or seconds, and then the RVP drops rapidly until it falls below the normal pressure, which in Group F particularly evident.Conclusions: RVP have different reactions when the occlusion level is not the same, and the extent of more than 70% ligation is a successful model of acute right heart failure. These results may have important consequences for therapeutic strategies to prevent acute right heart failure.

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