Left ventricular noncompaction cardiomyopathy is a rare type of congenital cardiomyopathy characterized by prematurely arrested compaction of the endocardial and myocardial fibers and the progressive deterioration of left ventricular contractility. This entity is a genetically heterogeneous disorder and has a wide spectrum of presentation from no symptoms to critical disabling congestive heart failure, which can appear at any age. The prognosis is therefore varied. An elderly patient with left ventricular noncompaction underwent aortic valve replacement for associated aortic regurgitation. Follow-up at two years after surgery revealed an improved clinical condition and recovered cardiac function. This is the fourth known aortic valve replacement in a patient with left ventricular noncompaction.Keywords: cardiomyopathy, ventricular noncompaction, aortic valve replacement
IntroductionLeft ventricular noncompaction cardiomyopathy (LVNC) is believed to be a morphogenetic abnormality involving arrested compaction of the loose myocardial meshwork during early embryogenesis.
1The World Health Organization categorizes LVNC as an unclassified cardiomyopathy.2 The pathophysiology of this abnormality is the progressive deterioration of LV systolic function, 1 but the cause remains controversial. A relationship between coronary microcirculatory dysfunction and subendocardial ischemia is suspected.3 4 The triad of heart failure, systemic embolism and arrhythmias is the major clinical presentation when the LV systolic function deteriorates. used to diagnose LVNC including echocardiography, magnetic resonance imaging and computed tomography. Left ventricular noncompaction was initially considered as one cause of critical congestive heart failure in a pediatric population, 1 but this abnormality has been identified in an adult population due to advances in imaging techniques. 6 Cardiac surgery for adult patients with LVNC has rarely been reported. Here we describe aortic valve replacement to treat aortic regurgitation (AR) in a patient with LVNC.
Case ReportA 74-year-old woman without a family history of cardiovascular disease or sudden death was referred to our hospital with dyspnea that had persisted for three months. The physical findings were unremarkable except for a diastolic murmur at the left sternal edge. A chest x-ray showed cardiomegaly with a cardiothoracic ratio of 70%. Plasma levels of brain natriuretic peptide (BNP) and noradrenalin were elevated to 1540 and 481 pg/ml, respectively. Transthoracic echocardiography revealed extreme regurgitation of the tricuspid aortic valve and diffuse left ventricular (LV) hypokinesis that was particularly severe in the apex, lateral and posterior walls at the mid-ventricular and basal level. The aortic annulus was 23 mm in Left ventricular noncompaction cardiomyopathy is a rare type of congenital cardiomyopathy characterized by prematurely arrested compaction of the endocardial and myocardial fibers and the progressive deterioration of left ventricular contractility. This entity...