Isolated left ventricular noncompaction: unusual cause of decompensated heart failure and indication of heart transplantation in the early infancy: case report and literature review

A female infant, aged 10 months, arrived at our hospi-tal and was admitted with clinical signs of heart failure anda history of a previous admission at Intensive Care Unitfor decompensated heart failure and cardiac arrest onemonth before. She was born at term of healthy, unrelatedparents and was asymptomatic until the age of two months,when she presented with shortness of breath, poor appe-tite and weight loss. She weighed 6.8 Kg and was 75.5 cmtall. Cardiovascular examination revealed a decreased-vol-ume peripheral pulse and a third heart sound. There wasalso hepatomegaly, 4 cm below the right costal margin. Nodysmorphic features were observed. There was no sign ofarrhythmia noted while awaiting transplantation.The patient evolved with refractory heart failure as aresult of severely deteriorated left ventricular function andbecame a candidate for heart transplantation. On admis-sion, the infant’s medications were aldactone 1.5 mg/kg/day, captopril 1.5 mg/kg/day, furosemide 3.5 mg/kg/dayand digoxin 10 mg/kg/day.During the pre-transplantation exams, the chest X-rayrevealed substantial cardiomegaly. The electrocardiographyshowed left ventricular overload, but no signs of ventricu-lar arrhythmias. The echocardiogram showed dilated car-diac chambers (important enlargement of left atrium andleft ventricle). There was a severe left ventricular systolicdysfunction (shortening fraction of 7.3%) and a discrete leftatrioventricular valve insufficiency, with no signs of throm-bosis. The dimension of the septum and left ventricular wallwas 6 mm thick. Gallium-67 scintigraphy for myocardialinflammatory process was negative. An endomyocardialbiopsy revealed no evidence of myocarditis. Cardiac cath-eterization showed a systolic pulmonary pressure of 28mmHg, diastolic pulmonary pressure of 18 mmHg, meanpulmonary pressure of 21 mmHg, capillary wedge pressureof 15 mmHg, pulmonary vascular resistance index of 1.8Woods/m