Rare case of left-dominant arrhythmogenic right ventricular cardiomyopathy with dramatic reverse remodeling after cardiac resynchronization as an adjunct to pharmacological therapy

Heart & Lung ◽  
2012 ◽  
Vol 41 (6) ◽  
pp. e39-e43 ◽  
Author(s):  
Chih-Chung Hsiao ◽  
Jen-Yuan Kuo ◽  
Chun-Ho Yun ◽  
Chung-Lieh Hung ◽  
Cheng-Ho Tsai ◽  
...  
ESC CardioMed ◽  
2018 ◽  
pp. 1502-1505
Author(s):  
Alexandros Protonotarios ◽  
Perry Elliott

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.


2020 ◽  
Vol 9 (2) ◽  
pp. 86-88
Author(s):  
Suman Adhikari ◽  
Sunil Chandra Jha ◽  
Ratna Mani Gajurel ◽  
Chandra Mani Paudel ◽  
Subarna Mani Acharya ◽  
...  

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder. Its hallmark is the fibrofatty replacement of myocardium leading to cardiomyopathy. It can have various presentations which can range from silent disease to sudden cardiac death. Here, we report a case of ARVC in a 42 years old female who presented to us with dizziness as her first clinical presentation. We clinched the diagnosis with the help of electrocardiogram, echocardiography and cardiac magnetic resonance imaging (CMR).


2020 ◽  
Author(s):  
Jagjit Khosla ◽  
Reshma Golamari ◽  
Alice Cai ◽  
Jamal Benson ◽  
Wilbert S Aronow ◽  
...  

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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