Left ventricular non-compaction cardiomyopathy with coronary artery anomaly complicated by ventricular tachycardia

Non-compaction cardiomyopathy (NCCM) is characterized by prominent trabeculations, deep intertrabecular recesses, and a thick non-compacted endocardial myocardium. Prevalence in the general population remains unclear, but echocardiography series report 0.05%. During fetal development muscle fibers and trabeculae should compact into a solid myocardium and when this fails, NCCM occurs. The condition is genetic, even though acquired forms have been described. Worsening myocardial dysfunction may lead to heart failure and/or arrhythmias.

A 52-year-old man presented with heart failure. The diagnosis of NCCM was confirmed after echocardiography and cardiac magnetic resonance tomography. Interestingly, the angiogram revealed a coronary anomaly, in which the circumflex artery rose aberrantly from the right coronary artery. Due to left ventricular ejection fraction being less than 35% despite optimal pharmacological therapy, an implantable cardioverter defibrillator (ICD) was implanted and four years later a ventricular tachycardia was terminated by antitachycardia pacing.

We describe a case of NCCM with a concomitant coronary anomaly, in which systolic myocardial dysfunction developed. The ICD subsequently terminated a life-threatening ventricular arrhythmia, which supports risk stratification based on low ejection fraction and possibly coronary anomaly.