Arrhythmogenic Right Ventricular Dysplasia Associated with Bicuspid Aortic Valve

Abstract

A 22-year old boy was diagnosed arrhythmogenic right ventricular dysplasia (ARVD) with bicuspid aortic valve (BAV) after a symptomatic event of ventricular tachycardia originating from the right ventricle. Cardiac magnetic resonance imaging disclosed right ventricular enlargement, diffuse thinning of lateral wall and normal left ventricle. Echocardiography showed globally thinned, moderately enlarged right ventricle and bicuspid aortic valve. Cardiovascular malformations may be present with bicuspid aortic valve. Here we would like to report the first ARVD case with an accompanying BAV.

Keywords

• Arrhythmogenic Right Ventricular Dysplasia, Bicuspid Aortic Valve.

Aritmojenik Sa¤ Ventrikül Displazisi ile Birlikte Olan Biküspit Aort Kapak

Abstract

Sa¤ ventrikül kaynakl› ventriküler taflikardiye ba¤l› semptomu olan 22 yafl›nda erkek bir hastada aritmojenik sa¤ ventrikül displaziye (ASVD) efllik eden bikuspit aortik kapak (BAK) saptad›k. Kardiyak manyetik rezonans görüntülemede sa¤ ventrikül lateral duvarda yayg›n incelme ile geniflleme ve normal sol ventrikül saptand›. Ekokardiyografide ise sa¤ ventrikülde orta derece geniflleme ile global incelme ve BAK saptad›k. Bikuspit aortik kapak di- ¤er kardiyovasküler malformasyon ile birlikte bulunabilmektedir. Biz bu yaz›m›zda ilk defa ASVD'ye efllik eden BAK olgusunu bildirdik.

Keywords

• Aritmojenik Sa¤ Ventrikül Displazisi, Bikuspit Aortik Kapak

References

1. Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation; 1982; 65: 384–98.
2. Sen-Chowdhry S, Prasad SK, Syrris P, Wage R, Ward D, Mer- rifield R, et al. Cardiovascular magnetic resonance in arrhy- thmogenic right ventricular cardiomyopathy revisited: compari- son with task force criteria and genotype. J Am Coll Cardiol; 2006; 48: 2132-40.
3. Ainsworth CD, Skanes AC, Klein GJ, Gula LJ, Yee R, Krahn AD. Differentiating arrhythmogenic right ventricular cardiom- yopathy from right ventricular outflow tract ventricular tach- ycardia using multilead QRS duration and axis. Heart Rhythm; 2006; 4:416-23
4. Hoffman JI, Kaplan S. The incidence of congenital heart di- sease. J Am Coll Cardiol; 2002; 39: 1890–900.
5. Gershoni-Baruch R, Moor EV, Enat R. Marfan syndrome as- sociated with bicuspid aortic valve, premature aging, and pri- mary hypogonadism. Am J Med Genet; 1990; 37: 169–72.
6. Hirose H, Amano A, Takahashi A, Nagano N, Kohmoto T. Ruptured aortic dissecting aneurysm in Turner's syndrome: a case report and review of literature. Ann Thorac Cardiovasc Surg; 2000; 6: 275–80. normal left ventricular function.