Isolated Biventricular Noncompaction in an adult with severe pulmonary hypertension: An association reviewed
A 13-year-old man with chronic heart failure was referred for diagnostic assessment. There was a history of progressively worsening effort intolerance and dyspnoea for the last year. There was no family history of heart disease or sudden death. On admission, his blood pressure was 100/64 mmHg and the pulse rate was76 beats/min. The 12-lead ECG showed sinus rhythm. Chronic interstitial lung congestion and massive cardiomegaly was found on chest X-ray. Transthoracic echocardiography revealed a normally sized, hypokinetic left ventricle (ejection fraction 37%) accompanied by massive enlargement of the right and left atria. There was spontaneous echo contrast in both ventricles The apical portions of both ventricles had a markedly trabeculated, spongy appearance, indicating biventricular noncompaction. The maximum end-systolic ratio of the noncompacted endocardial layer to the compacted myocardium was more than 2. Doppler examination showed color flow between the prominent trabeculations. Moreover, there was severe tricuspid regurgitation. The calculated peak systolic pulmonary artery pressure by tricuspid regurgitation (TR: gradient 75 mmHg) revealed severe pulmonary hypertension. The restrictive left ventricle inflow and decreased myocardial velocities suggested significantly elevated left ventricle end-diastolic pressure. The child was started on oral decongestants and anticoagulation.