Left ventricular noncompaction: case report and literature review

Isolated left ventricular noncompaction (ILVNC) is a rare cause of dilated cardiomyopathy. It is morphologically characterized by a spongy left ventricle with prominent myocardial trabeculations. Although not proven, it is nowadays widely accepted that ILVNC results from arrest of the normal myocardial compaction process during early embryonic life. ILVNC can occur in a familial form and certain genes coding proteins linking the extracellular matrix and the cytoskeleton have been related to this disorder. Clinically, the disease courses with heart failure, embolic and arrhythmic events being a common cause of morbidity and mortality. Current diagnostic criteria for ILVNC are based on clinical and echocardiographic data. Therapy is based on current heart failure guidelines, with particular emphasis on anticoagulation and prevention of fatal arrhythmias. The authors describe the case of a 29-year-old man admitted to the Cardiology Department with decompensated heart failure (NYHA IV), a dilated left ventricle with severe systolic dysfunction, an apical thrombus and highly trabeculated walls. Clinical evolution was favorable after standard pharmacologic therapy for heart failure and levosimendan, the patient being discharged in NYHA class I-II. Eighteen months later, despite partial recovery of left ventricular systolic function, the patient suffered sudden death. A review and discussion of the literature presented regarding etiopathogenesis, diagnostic criteria and therapeutic options.