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 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.

Arrhythmogenic Right Ventricular Cardiomyopathy: Progress Toward Personalized Management

2019 ◽  
Vol 70 (1) ◽  
pp. 1-18 ◽  
Author(s):  
Cynthia A. James ◽  
Hugh Calkins
Keyword(s):  
At Risk ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Family Members ◽  
Ventricular Myocardium ◽  
Ventricular Cardiomyopathy ◽  
Pathogenic Variants ◽  
Natural History Studies ◽  
Genes Encoding

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart disease characterized by fibrofatty replacement of the ventricular myocardium, a high risk of ventricular arrhythmias, and progressive ventricular dysfunction. The clinical course is highly variable, and optimal approaches to management remain undefined. ARVC is associated with pathogenic variants in genes encoding the cardiac desmosome. Genetic testing facilitates identification of at-risk family members, but penetrance of ARVC in pathogenic variant carriers is difficult to predict. Participation in endurance exercise is a known key risk factor. However, there remains significant uncertainty about which family member will develop disease and how best to approach longitudinal screening. Our clinically focused review describes how new insights gained from natural history studies, improved understanding of pathogenic mechanisms, and appreciation of genetic and environmental modifiers have set the stage for developing personalized approaches to managing both ARVC patients and their at-risk family members.

Myocyte Transdifferentiation

2000 ◽  
Vol 124 (2) ◽  
pp. 287-290 ◽  
Author(s):  
Giulia d'Amati ◽  
Cira R. T. di Gioia ◽  
Carla Giordano ◽  
Pietro Gallo
Keyword(s):  
Right Ventricular ◽  
Transplant Patient ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Ventricular Cardiomyopathy ◽  
Familial Dilated Cardiomyopathy ◽  
Familial Cardiomyopathy ◽  
New Type ◽  
The Right ◽  
Gross Examination

Abstract Adipose substitution of ventricular myocardium is characteristic of arrhythmogenic right ventricular cardiomyopathy, but is also found in other heart conditions. It is thought to be a consequence of myocyte loss due to myocarditis or other noxious stimuli. We describe a unique case of cardiomyopathy with a morphologic pattern suggestive of transdifferentiation from myocytes to mature adipocytes. Gross, histologic, and ultrastructural examination were performed on the heart of a female transplant patient with a clinical diagnosis of familial dilated cardiomyopathy. Gross examination showed fibroadipose substitution of the left ventricle and adipose replacement of the right. Histology, immunohistochemistry, and ultrastructure were highly suggestive of transdifferentiation from cardiac muscle to adipose tissue. Myocyte transdifferentiation could represent an alternative pathogenetic pathway to the myocyte-loss and adipose-replacement mechanism in arrhythmogenic right ventricular cardiomyopathy, or it could be the basis of a new type of familial cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy: management of symptoms and prevention of sudden cardiac death

2018 ◽  
Author(s):  
Perry Elliott ◽  
Alexandros Protonotarios
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Ventricular Dysfunction ◽  
Beta Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Sustained Ventricular Tachycardia ◽  
Carrier Status ◽  
Ventricular Cardiomyopathy

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have arrhythmia-related symptoms or are identified during screening of an affected family. Heart failure symptoms occur late in the disease’s natural history. As strenuous exercise has been associated with disease acceleration and worsening of ventricular arrhythmias, lifestyle modification with restricted athletic activities is recommended upon disease diagnosis or even identification of mutation carrier status. An episode of an haemodynamically unstable, sustained ventricular tachycardia or ventricular fibrillation as well as severe systolic ventricular dysfunction constitute definitive indications for implantable cardioverter defibrillator (ICD) implantation, which should also be considered following tolerated sustained or non-sustained ventricular tachycardia episodes, syncope, or in the presence of moderate ventricular dysfunction. Antiarrhythmic medications are used as an adjunct to device therapy. Catheter ablation is recommended for incessant ventricular tachycardia or frequent appropriate ICD interventions despite maximal pharmacological therapy. Amiodarone alone or in combination with beta blockers is most effective for symptomatic ventricular arrhythmias. Beta blockers are considered for use in all patients with a definite diagnosis but evidence for their prognostic benefit is sparse. Heart failure symptoms are managed using standard protocols and heart transplantation is considered for severe ventricular dysfunction or much less commonly uncontrollable ventricular arrhythmias.

scholarly journals Plasma BIN1 correlates with heart failure and predicts arrhythmia in patients with arrhythmogenic right ventricular cardiomyopathy

Heart Rhythm ◽  
2012 ◽  
Vol 9 (6) ◽  
pp. 961-967 ◽  
Author(s):  
Ting-Ting Hong ◽  
Rebecca Cogswell ◽  
Cynthia A. James ◽  
Guson Kang ◽  
Clive R. Pullinger ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy

scholarly journals Possibility of the Subcutaneous Implantable Cardioverter-Defibrillator for Prevention of Sudden Cardiac Death in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Author(s):  
Shingo Sasaki
Keyword(s):  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Young Patients ◽  
Ventricular Myocardium ◽  
Hereditary Disease ◽  
Ventricular Cardiomyopathy ◽  
Cardioverter Defibrillator

The EMBLEM™ entirely subcutaneous implantable cardioverter-defibrillator (S-ICD) system (Boston Scientific, Marlborough, Massachusetts, USA) was introduced as a new alternative to the conventional transvenous implantable cardioverter-defibrillator and has been expected to reduce device-related complications, especially in young patients who require long-term lead placement. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a well-known hereditary disease recognized as a cause of sudden cardiac death (SCD) in young adults. However, the precise clinical role of S-ICD in patients with ARVC remains to be defined because of the low QRS amplitude of subcutaneous electrocardiogram (S-ECG) followed by the high incidence of inappropriate shock (IAS) delivery due to oversensing. It is well known that the sensing of S-ICD is largely dependent on the QRS/T ratio of S-ECG. The decrease in the QRS amplitude is more likely to lead to oversensing such as T wave or myopotential oversensing. In patients with ARVC, the decrease in the QRS amplitude due to degeneration of the right ventricular myocardium progresses overtime. In this chapter, we would like to discuss the usefulness of S-ICD lead repositioning for young adult patients with ARVC based on our experience of patients with IAS.

scholarly journals Heart failure in patients with arrhythmogenic right ventricular cardiomyopathy: Genetic characteristics

2019 ◽  
Vol 286 ◽  
pp. 99-103 ◽  
Author(s):  
Annina S. Vischer ◽  
Silvia Castelletti ◽  
Petros Syrris ◽  
William J. McKenna ◽  
Antonios Pantazis
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Genetic Characteristics

Different arrhythmic prognosis in high-risk arrhythmogenic right ventricular cardiomyopathy according to the indication of the defibrillator

2019 ◽  
Author(s):  
Amalio Ruiz-Salas ◽  
Isabel Navarro-Arce ◽  
Carmen Medina-Palomo ◽  
Alberto Barrera-Cordero ◽  
Manuel Jiménez-Navarro ◽  
...  
Keyword(s):  
Heart Failure ◽  
Primary Prevention ◽  
High Risk ◽  
Secondary Prevention ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Risk Category ◽  
Ventricular Cardiomyopathy ◽  
The Impact

Abstract Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC/D) is an inherited cardiomyopathy characterized by ventricular arrhythmias and heart failure. The aim of our study was to analyze the impact of the ICD indication in the prognosis of patients with high-risk ARVC/D according to the consensus document. Methods The high-risk category includes patients who experienced cardiac arrest due to sustained ventricular tachycardia or ventricular fibrillation and patients with severe right or left ventricular dysfunction. We included 41 patients with high-risk ARVC/D: 33 in secondary prevention and 8 in primary prevention. Results We followed 41 patients during 6.37 ± 4.88 years. Twenty-six patients (63.4%) had at least one appropriate arrhythmic event: 24 p (72.7%) in secondary prevention and 2 p (25%) in primary prevention; p=0.02. Twenty-four patients (72.7%) in secondary prevention and five (62.5%) in primary prevention had a cardiovascular event such as arrhythmias, admission due to heart failure, heart transplantation or cardiovascular death. Conclusions High-risk ARVC/D patients had a high number of cardiovascular events, but their nature and treatment were different. Arrhythmic prognosis was worse in secondary prevention and most of the events found in primary prevention were related to heart failure and, therefore, without benefit of the ICD.

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