I solated ventricular noncompaction (IVNC) is a rare genetic cardiomyopathy characterized by excessively prominent ventricular trabeculations and deep intertrabecular recesses. It is thought to arise in utero from arrested compaction of a loose myocardial meshwork. The major clinical presentations of left ventricular noncompaction (LVNC) are heart failure, arrhythmias, and thromboembolism. The diagnosis is established with use of 2-dimensional echocardiography or cardiac magnetic resonance (CMR).We report the case of a patient with dilated cardiomyopathy and LVNC, and we discuss the effects of standard heart failure therapy on his condition. Case ReportIn March 2011, a 35-year-old man presented at a cardiology outpatient clinic with a several-week history of palpitations. His medical history yielded nothing relevant, and routine laboratory test results were normal. His chest radiograph showed mild cardiomegaly and normal lung fields. Physical examination revealed normal heart sounds and a mild systolic murmur heard best at the apex. A 12-lead electrocardiogram (ECG) showed sinus rhythm with poor R-wave progression and T-wave inversion in the precordial leads. A transthoracic echocardiogram (TTE) revealed a dilated left ventricle (LV), a depressed LV ejection fraction (LVEF) of 0.25, and moderate mitral regurgitation. A coronary angiogram revealed normal results. A 24-hour ECG showed frequent premature ventricular complexes and runs of nonsustained ventricular tachycardia.Results of a CMR study included a markedly dilated LV with an LVEF of 0.19, an end-diastolic volume of 395 mL, and an end-systolic volume of 319 mL (Fig. 1). Prominent trabeculations were seen in the apical segments and lateral wall of the LV (Fig. 2). The ratio of noncompacted-to-compacted myocardium was 3.35:1. The indexed LV mass was 76 g/m 2 with no evidence of hypertrophy. Right ventricular (RV) size, function, and wall structure were normal. Delayed myocardial gadolinium enhancement produced subtle mid-wall stripes along the anterior and lateral LV wall and the inferior half of the interventricular septum. There was also marked myocardial wall-thinning, particularly of the apical segments. The left atrial diameter was 36 mm. The diagnosis was LVNC with heart failure. The patient was started on perindopril and carvedilol, with gradual upward titration.Because of the patient's ventricular tachycardia and low LVEF, we referred him for implantable cardioverter-defibrillator placement. However, according to the im- Case ReportsKyriacos Papadopoulos, MD Petros M. Petrou, MBChB, MRCP (UK) Demos Michaelides, FRCR, FRCP
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