Aortico-left ventricular tunnel in fetuses and infants

Background Aortico-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle presenting in early childhood as aortic regurgitation and cardiac failure. This condition has rarely been reported in fetuses. Operation is the only treatment, and postoperative aortic incompetence could be related to the age or the type of repair. Methods We conducted a retrospective, two-institution review, from 1983 to 1995, of aortico-left ventricular tunnel diagnosed in utero and before 6 months of age. Results Three cases of aortico-left ventricular tunnel were diagnosed in utero by Doppler echocardiography between 22 and 24 weeks' gestation. Prenatal aortico-left ventricular tunnel was associated with severe left ventricular dysfunction, aortic valve anomalies, and fetal hydrops. One death occurred in utero and one immediately after birth, and in 1 case pregnancy was interrupted. In these 3 cases the diagnosis was confirmed by autopsy. Three neonates and 2 infants had the diagnosis of aortico-left ventricular tunnel made after birth and underwent successful surgical repair. At short and midterm follow-up all patients are alive and aortic valve regurgitation is absent or trivial. Conclusions This series shows that aortico-left ventricular tunnel covers an anatomic spectrum of lesions. Cases diagnosed in utero by Doppler echocardiography are characterized by severe ventricular dysfunction, associated aortic valve lesions, and poor outcome. Postnatal cases represent the more favorable end of the spectrum, with no associated lesions, and can be repaired without mortality and with good functional results.