Congenital left ventricular aneurysms and diverticula: definition, pathophysiology, clinical relevance and treatment.

A congenital left ventricular aneurysm or diverticulum is a rare cardiac malformation; 411 cases have been reported since its first description in 1816, and other cardiac, vascular or thoraco-abdominal abnormalities have been shown in about 70%. It appears to be a developmental anomaly, starting in the 4th embryonic week. Diagnosis can be made after exclusion of coronary artery disease, local or systemic inflammation or traumatic causes as well as cardiomyopathies. Clinically, most congenital left ventricular aneurysms and diverticula are asymptomatic or may cause systemic embolization, heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies such as echocardiography, magnetic resonance imaging or left ventricular angiography, visualizing the structural changes and accompanying abnormalities. Mode of treatment has to be individually tailored and depends on clinical presentation, accompanying abnormalities and possible complications; treatment options include surgical resection especially in symptomatic patients, anticoagulation after systemic embolization, radiofrequency ablation or implantation of an implantable cardioverter defibrillator in case of symptomatic ventricular tachycardia, occasionally combined with class I or III antiarrhythmic drugs. Copyright 2006 S. Karger AG, Basel.