Pulmonary hypertension is defined by a mean pulmonary artery pressure ≥25 mmHg at rest, measured during right heart catheterization. Ventricular noncompaction is a genetic cardiomyopathy which mostly effects left ventricle. It is related with deterioration of myocardial embryogenesis and commonly together with other cardiac diseases (1).
Isolated ventricular non-compaction is characterized by modified morphology of myocardial wall, increased trabeculation in ventricular cavity and deep intertrabecular recesses.
A 43-year-old woman presented exercise induced dyspnea and atypical chest pain. She has not any medical history prior. On admission, her 12 lead ECG showed complete righ bundle branch block, her blood pressure was 120/80 and pulse rate 80 per minutes. Transthoracic 2D echocardiogram and magnetic resonance imaging showed dilated and hypertrophied right ventricle with non-compaction of the right ventricular apex. The systolic pulmonary arterial pressure was 80 mmHg on the Doppler echocardiography. The coronary angiography revealed normal coronary arteries. The catheterization was showed pulmonary hypertension, right ventricle non-compaction and negative pulmonary vasoreactivity testing.
Ventricular noncompaction, especially right ventricular noncompaction, complicated by severe pulmonary hypertension is exceptional. Only a few isolated right ventricular noncompaction has been reported but inclusion of pulmonary hypertesion cases are rare subsets .
Diagnosis of pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic ventricular dysfunction secondary to right ventricular noncompaction. Widespread usage of cardiac magnetic resonance imaging, may enhance visual quality and evaluation of ventricular morphology, probably this will provide prevalance increment and clinical outcome improvements. Early diagnosis would bring better results.
Interventional Cardiovascular Magnetic Resonance Imaging (iCMR) in an Adolescent with Pulmonary Hypertension
