Left ventricular pseudoaneurysm and α1-antitrypsin enzyme deficiency: another pathological correlation

Alpha1-antitrypsin deficiency (A1ATD) is usually linked to chronic lung dysfunction and chronic liver disease. Recent evidence have shown involvement also of the cardiovascular system. Indeed, dissection of the thoracic aorta or of the coronary arteries, and intra-cerebral aneurysms has been shown to represent concomitant vascular adverse events and apparently related to the impaired vascular wall integrity, strictly related to the elastase levels which are usually modulated by the A1AT. The case herein reported describes a patient affected by A1ATD associated with a large pseudoaneurysm of the left ventricle without any known predisposing factors to pseudoaneurysm development (coronary artery disease, acute myocardial infarction, taruma and myocarditis) indicating that the connective tissue impairment documented in A1ATD may also affect the myocardial structure, ultimately leading to weakened wall integrity, sudden rupture and pseudoaneurysm formation successfully managed by a surgical approach.