Noncompaction cardiomyopathy in the State of Qatar

OBJECTIVE: To study the development of noncompaction of the ventricular myocardium (NCCM) in the state of Qatar and to highlight the prognostic parameters in those patients.

METHODS: We conducted this study from 2000 to 2004 on patients who were referred to Hamad General Hospital with questionable echocardiographic features of cardiomyopathy with or without clinical manifestations of heart failure and were found to have NCCM. The diagnosis of NCCM was made according to echocardiographic criteria in 12 cases and those patients are followed up for 2-5 years.

RESULTS: The mean age at diagnosis of NCCM was 6.5 years. Among them, 4 were males and 8 were females. Family history of NCCM was reported in 5 cases. Normal ejection fraction was detected in 5 patients; in this group pulsed-Tissue Doppler Imaging revealed evidence of subclinical systolic dysfunction in 4 cases. All patients showed variable degrees of diastolic dysfunction. Severely impaired ejection fraction was found in 3 cases. Progression to dilated cardiomyopathy occurred in 4 cases. Site of noncompaction included left ventricle apex in all cases, inferoposterior in 11 cases, and lateral wall in 11 cases while biventricular noncompaction was noted in 4 cases. Electrocardiogram findings included right bundle branch blocker (3) patients, left bundle branch blocker (2), left ventricular hypertrophy (6) and right ventricular hypertrophy in 3 cases. Atrial tachyarrhythmias developed in 4 cases. Wolff-Parkinson-White syndrome was detected in one patient. Associated congenital anomalies included ventricular septal defect, pulmonary stenosis, aortic coarctation, and Ebstein anomaly. The overall mortality rate was 25%.

CONCLUSION: Noncompaction cardiomyopathy is so rare to be easily missed. The prognosis is poor in symptomatic cases; however, detection of subclinical systolic dysfunction is needed.