Tag Archives: ARVD/C

Cardiology MCQ 26.4.15

All of the following statements about Arrhythmogenic Right Ventricular
Dysplasia/Cardiomyopathy are correct except

A. Pathogenic mutations can be identified in 50% of patients

B. The clinical presentation is between 2nd to 5th decade of life

C. Left dominant arrhythmogenic cardiomyopathy most commonly involves apical septal segment of left ventricle

D.  Left-dominant disease is more commonly seen in patients with desmoplakin mutations

Explanation:

-Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy. Although structural involvement of the right ventricle predominates, a left dominant form of ARVD/C has been described

-Ventricular arrhythmias, increased risk of sudden cardiac death, and abnormalities of right ventricular structure and function characterize this disease
-The pathognomonic features are right ventricular myocyte loss with fibrofatty replacement.
-Because a pathogenic mutation can be identified in approximately 50% of affected individuals

-Patients usually present during the second to fifth decades of life with palpitations, light-headedness, syncope, or sudden death. Patients younger than 12 years and those older than 60 years rarely manifest clinical signs or symptoms of ARVD/C

-Cardiac MRI can be to detect involvement of left ventricle in patients with ARVD/C, especially those with advanced disease

-Left-dominant arrhythmogenic cardiomyopathy also occurs in and is defined by early disease of the LV, often affecting the posterolateral wall, in the absence of significant right ventricle (RV) systolic dysfunction. Left-dominant disease is more commonly seen in patients with desmoplakin mutations.

References:

1. Marcus F, Fontaine G, Guiraudon G, et al: Right ventricular dysplasia: A report of 24 adult cases. Circulation 65:384–398, 1982.
2. Corrado D, Basso C, Thiene G, et al: Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: A multicenter study. J Am Coll Cardiol 30:1512–1520, 1997.
3. Dalal D, Nasir K, Bomma C, et al: Arrhythmogenic right ventricular dysplasia: A United
States experience. Circulation 112:3823–3832,2005.
4. Marcus F, Zareba W, Calkins H, et al: Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: Results from the North American Multidisciplinary Study. Heart Rhythm 6:984–989, 2009.
5. Sen-Chowdhry S, Syrris P, Prasad SK, et al: Leftdominant arrhythmogenic cardiomyopathy: An under-recognized clinical entity. J Am Coll Cardiol 52:2175–2187, 2008.
6. Dalal D, Tandri H, Judge DP, et al: Morphologic variants of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy: A genetics-magnetic resonance imaging correlation study. J Am Coll Cardiol 53:1289–1299, 2009.
7. den Haan A, Tan B, Zikusoka M, et al: Comprehensive desmosome mutation analysis in North Americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Cardiovasc Genet 2:428–435, 2009.

Answer: C

Keywords: Cardiology review, Cardiology, Multiple choice questions, medical tudents, Electrophysiology,  Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, ARVD/C