Replacement of Regurgitant Bicuspid Aortic Valve in a Dilated,Non-Compacted Left Ventricle

Abstract

Bicuspid aortic valve (BAV) is the most common form of congenital heart disease, with 20% of asymptomatic adults with BAV presenting with significant valve insufficiency. Yet, limited data exist regarding surgical indications and outcomes when BAV is accompanied by left ventricular dilation, systolic dysfunction, or left ventricle non-compaction (LVNC) syndrome. We present a case of dilated cardiomyopathy due to severe BAV regurgitation and partial LVNC syndrome and the decision to undergo aortic valve replacement. Our patient represents the most extreme documented case of regurgitant BAV with dilated, dysfunctional, and partially non-compacted left ventricle. Yet, surgical intervention provided improvement in systolic performance and ventricular dimensions.

Keywords

bicuspid aortic valve systolic dysfunction non-compaction left ventricle surgical outcome

Get full access to this article

View all access options for this article.

References

Lewin

Otto

. The bicuspid aortic valve: adverse outcomes from infancy to old age. Circulation. 2005;111(7):832–834.

Agarwal

Khandheria

Paterick

Treiber

Bush

Tajik

. Left ventricular noncompaction in patients with bicuspid aortic valve. J Am Soc Echocardiogr. 2013;26(11):1306–1313.

Nishimura

Otto

Bonow

. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Am Coll Cardiol. 2014;63(22):e57–e185.

Ashikhmina

Sundt

Dearani

Connolly

Schaff

. Repair of the bicuspid aortic valve: a viable alternative to replacement with a bioprosthesis. J Thorac Cardiovasc Surg. 2010;139(6):1395–1401.

Oechslin

Attenhofer

Jost CH

Rojas

Kaufmann

Jenni

. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 2000;36(2):493–500.

Cavusoglu

Ata

Timuralp

. Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement. Echocardiography. 2003;20(4):379–383.

Wrigley

Rosin

Banerjee

. Replacement of a congenital bicuspid aortic valve in a patient with left ventricular noncompaction. Tex Heart Inst J. 2009;36(3):241–243.

Ohki

Moriyama

Mohara

Kimura

Sata

Miyahara

. Aortic valve replacement for aortic regurgitation in a patient with left ventricular noncompaction. Ann Thorac Surg. 2009;87(1):290–292.

Chaliki

Mohty

Avierinos

. Outcomes after aortic valve replacement in patients with severe aortic regurgitation and markedly reduced left ventricular function. Circulation. 2002;106(21):2687–2693.