Isolated left ventricular non-compaction: an unusual indication for heart transplantation

Case report In 2003, a 43-year-old woman was referred to our cardiology clinic with a diagnosis of hypertrophic cardiomyopathy after family screening using transthoracic echocardiography (TTE). Her 14-year-old son had been diagnosed with hypertrophic cardiomyopathy 2 years beforehand. The family history revealed that the patient’s mother suffered sudden cardiac death at the age of 56 years. A repeat TTE, however, showed the presence of numerous prominent trabeculations and deep sinusoids, especially in the apical and posterior wall. The left ventricle’s systolic function was impaired with an ejection fraction estimated at 32%. Cardiac catheterization showed normal coronary arteries. The cardiac output (CO) and cardiac index (CI), measured using thermodilution, were 3.2 L/min and 2.25 L/min/m 2 respectively. She had no other cardiac abnormalities. Cardiac magnetic resonance (CMR) imaging confirmed extensive areas of prominent trabeculation and inter-trabecular recesses of the left ventricle with relative sparing of the basal septum. The delayed contrast images revealed diffuse patchy endocardial, epicardial and mid-wall myocardial enhancement at the non-compacted left ventricular walls, suggestive of extensive myocardial fibrosis (Fig 1). These areas matched the irreversibly hypoperfused sites detected by the myocardial perfusion imaging. Hence, a diagnosis of isolated left ventricular non-compaction cardiomyopathy (LVNC) was made. A CMR imaging subsequently performed on her son also showed features diagnostic of LVNC rather than hypertrophic cardiomyopathy.