Aorto-left ventricular tunnel, also known as aorto-left ventricular fistula, is a rare CHD characterised by an abnormal connection between the aorta and the left ventricle. This article presents a comprehensive case report on aorto-left ventricular tunnel, encompassing clinical presentation, diagnostic approaches, treatment options, and outcomes, with a specific focus on the necessity of early surgical intervention.

An aorto-ventricular tunnel is a rare congenital cardiac defect, where a channel connects the lumen of the ascending aorta to the left or right ventricle. Four patients presented with an aorto-left ventricular tunnel over two decades at a median age of 8 months (range 0.1–10 months). Two patients (50%) had associated cardiac anomalies including hypoplastic left heart syndrome and left ventricular noncompaction/hypertrophic cardiomyopathy with aortic/pulmonary valve dysplasia in one patient each. Although traditionally surgical treatment has addressed this problem, management has evolved to transcatheter closure with excellent outcomes in appropriately selected patients at our national centre.

Aorto-left ventricular tunnel is an exceedingly rare congenital cardiac defect. Early surgical closure is the treatment of choice. Residual or recurrent tunnel and aortic valve insufficiency are well-recognised complications after surgical repair. In this article, we report on successful transcatheter closure of a residual aorto-left ventricular tunnel using an Amplatzer duct occluder in a 7-year-old boy. The outcome after 6 years of follow-up is encouraging.

Aorto-left ventricular tunnel is a rare congenital heart lesion, with an incidence of <0.1% of all CHD. We present a unique case of a valvar aorto-left ventricular tunnel in a neonate, in belief that our findings may shed some light on the developmental origins of this lesion.

A case is presented of a 3.5-year-old boy with aorto-left ventricular tunnel. A diagnosis of isolated aortic regurgitation had been made at the age of 11 months following cardiac catheterization with selective left ventricular angiography and aortography. The correct diagnosis was established 2.5 years later following cross-sectional echocardiographic examination combined with color Doppler investigation.

An asymptomatic 4-year-old child was referred to our Institution with a provisional diagnosis of severe aortic valvar regurgitation in association with a ventricular septal defect. Intraoperatively, the aortic valve appeared bicuspid, and a tunnel of 7 mm diameter extending between the aorta and the left ventricle was found dividing the anterior commissure into two hemi-commissures. Repair was achieved by reconstructing the anterior commissure by direct reapproximation, and simultaneous obliteration of the aortic side of the tunnel. The aortic valvar leaflet was resuspended onto the arterial wall. This combined approach will hopefully reinforce the poorly supported aortic sinus, and may maintain aortic valvar competence over the long term. Our intraoperative diagnosis of aorto-left ventricular tunnel suggests consideration of this diagnosis when evaluating any patient with suspected aortic valvar incompetence.