Clinical Characterization of Left Ventricular Noncompaction: A Case Series of Patients

Background-Non-compaction of ventricular myocardium (LVNC), also known as LVHT (left ventricular hypertrabeculation), is a rare embryonic cardiomyopathy that is thought to be a consequence of intrauterine arrest of compaction. It is characterized by an excessively prominent trabecular meshwork, which is accompanied by depressed ventricular function, systemic embolism and ventricular arrhythmia. This study was conducted to clarify the clinical features of patients with left ventricular noncompaction (LVNC) who were diagnosed in Shahid Rajaei Cardiovascular Medical Center. Methods and Results- We retrospectively reviewed patients with LVNC between December 2004 and December 2005. A total of twenty-four patients were identified. In 4 patients there were associated cardiac lesions. They consisted of 6 females and 18 males with a mean age of 38.2 years (age range: 13-62 years). The average ejection fraction was 23.3%.The extension of noncompacted myocardium that was observed on 2-D echocardiography, was predominantly at the apex. There were two patients with systemic emboli, one with nonsustained ventricular tachycardia (VT), and one with Wolf-Parkinson-White sydnrome. The most common abnormality in the the electrocardiogram was left bundle branch block. Conclusions- LVNC is most frequently diagnosed primarily by echocardiography and its prevalence seems to be increased with the improvement of cardiac imaging; so echocardiographers should be aware and trained to recognize this abnormality (Iranian Heart Journal 2007; 8 (4): 35-42).