Aortico-Left Ventricular Tunnel: Report of Two Prenatal Cases and Review of the Literature

Introduction

Aortico-left ventricular tunnel (ALVT) is a rare congenital heart malformation representing approximately 0.001% of all congenital cardiac malformations (Okoroma EO et al. J Thorac Cardiovasc Surg. 1976; 71: 238–44). ALVT was first described as an abnormal communication between the ascending aorta and the left ventricle (Levy MJ et al. Circulation 1963; 27: 841–53). The tunnel begins above the sinus of Valsalva near the right coronary ostium and ends in the left ventricle below the right aortic cusp. The embryological basis is unknown. Diagnosis can be made by fetal echocardiography. Possible features of the lesion during fetal life include a dilated left ventricle, aortic regurgitation and a dilated ascending aorta. Postnatal treatment is early surgical patch closure of the defect. A good long-term outcome postoperatively is reported. However, the outcome is highly dependent on the antenatal presence of heart failure and hydrops (Singh A et al. Pediatr Cardiol 2011; 32: 822–5). The course of an untreated large ALVT is progressive heart failure leading to death. We present two second trimester cases with signs of severe heart failure due to ALVT which were diagnosed by autopsy.