Predicting Heart Failure in Arrhythmogenic Right Ventricular Cardiomyopathy

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.

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

10.1093/med/9780198784906.003.0361 ◽

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.

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

Heart Rhythm ◽

10.1016/j.hrthm.2012.01.024 ◽

2012 ◽

Vol 9 (6) ◽

pp. 961-967 ◽

Cited By ~ 31

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

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Congestive heart failure in the natural history of arrhythmogenic right ventricular cardiomyopathy

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(98)81293-x ◽

1998 ◽

Vol 31 ◽

pp. 149

Author(s):

C. Basso ◽

A. Angelini ◽

U. Livi ◽

A. Nava ◽

G. Thiene

Keyword(s):

Heart Failure ◽

Congestive Heart Failure ◽

Natural History ◽

Right Ventricular ◽

Arrhythmogenic Right Ventricular Cardiomyopathy ◽

Ventricular Cardiomyopathy ◽

History Of

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

International Journal of Cardiology ◽

10.1016/j.ijcard.2019.01.065 ◽

2019 ◽

Vol 286 ◽

pp. 99-103 ◽

Cited By ~ 2

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

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

10.21203/rs.2.16672/v1 ◽

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

ESC CardioMed ◽

10.1093/med/9780198784906.003.0361_update_001 ◽

2018 ◽

pp. 1502-1505

Author(s):

Alexandros Protonotarios ◽

Perry Elliott

Keyword(s):

Heart Failure ◽

Ventricular Tachycardia ◽

Right Ventricular ◽

Ventricular Arrhythmias ◽

Ventricular Dysfunction ◽

Beta Blockers ◽

Arrhythmogenic Right Ventricular Cardiomyopathy ◽

Pharmacological Therapy ◽

Sustained Ventricular Tachycardia ◽

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.

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Arrhythmogenic right ventricular cardiomyopathy in a dog : case report

Journal of the South African Veterinary Association ◽

10.4102/jsava.v71i2.695 ◽

2000 ◽

Vol 71 (2) ◽

Cited By ~ 6

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.

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