Aorto-left ventricular tunnel (ALVT) is a rare congenital anomaly. In this condition, the aorto-ventricular tunnel is a congenital extracardiac channel that connects the ascending aorta to the left ventricle above the sinotubular junction. A 5-year-old boy presented with fatigue during physical activity, leading to visiting a pediatric specialist. Upon examination, a continuous murmur, predominantly diastolic, was detected. Suspecting aortic insufficiency, the patient was referred for further evaluation. Transthoracic echocardiography revealed a congenital ALVT. Although congenital ALVT is a rare congenital heart disease, its clinical symptoms may overlap with other congenital heart diseases, such as tetralogy of Fallot without pulmonary stenosis and patent ductus arteriosus. Differential diagnosis, such as the absence of a wide pulse pressure, can be helpful in distinguishing between these conditions.

Aorto-left ventricular tunnel is a rare congenital cardiac anomaly characterised by an extracardiac channel connecting the ascending aorta directly to the left ventricle. Surgical repair in neonates poses significant challenges due to the diminutive size and close proximity of critical structures such as the aortic valve and coronary ostia. We report the case of a neonate with an aorto-left ventricular tunnel, mild left ventricular dysfunction, and mild aortic insufficiency who underwent successful surgical repair on the sixth day of life. Following cardiopulmonary bypass initiation and cardioplegic arrest, the ascending aorta was transected for direct visualisation. A 5-mm 0° rigid endoscope was employed intraoperatively, providing high-resolution magnification of the aortic valve, right coronary artery, and tunnel anatomy. The tunnel was closed using an autologous pericardial patch, ensuring preservation of the coronary ostium and aortic valve integrity. Postoperative echocardiography demonstrated good left ventricular function, trivial aortic insufficiency, and no residual shunt. The use of rigid endoscopy significantly enhanced the safety and precision of the repair by allowing accurate anatomical delineation and facilitating patch placement. This case highlights the importance of detailed visualisation in neonatal aorto-left ventricular tunnel repair and underscores the utility of rigid endoscopic technology in complex congenital heart surgery. Endoscopic assistance not only aids intraoperative decision-making but also serves as a valuable tool for surgical education and postoperative review.

Using full-flow cardiopulmonary bypass, mild hypothermia and cardioplegic arrest, the aortic orifice of the aorto-left ventricular tunnel is closed with an autologous pericardial patch. After a longitudinal incision into the tunnel, a second patch of polytetrafluoroethylene is placed as far distally into the tunnel as possible to exclude most of its lumen from perfusion.

The study was aimed at assessing clinical status and outcome of patients affected by aorto-left ventricular tunnel (ALVT). A systematic search of keywords relating to ALVT was conducted to identify papers published between 1965 and February 2024 present on Pubmed/Medline and Scopus. A total of 109 studies, which in all consisted of case reports and case series comprising 177 patients (64.2% males, p < 0.02) met the inclusion criteria. The median age of patients was 9.5 ± 8.9 years. Initial diagnosis was based on echocardiographic findings in 86.4% of patients, and confirmed by computed tomography (CT) and/or magnetic resonance imaging (MRI) in 17%. Of the 177 patients identified, 47.1% were diagnosed with a heart murmur and 32.4% with congestive heart failure. Associated cardiac abnormalities were detected in 39.8% (unicuspid/bicuspid aortic valve with or without stenosis/atresia in 14.8%, coronary artery abnormalities in 9.6%). A total of 90.3% of patients underwent surgery, whilst 4.5% were treated by means of transcatheter closure. Outcomes were largely favorable (death was reported in 5.7%). Mild residual aortic regurgitation continued to be present in 22.7% of the sample. In terms of statistics, no risk factors for death were found. ALVT, an extremely rare congenital cardiac abnormality, may be diagnosed in both newborns and adults. Initial diagnostic observations are usually made using echocardiography, and subsequently refined by means of catheterization, CT or MRI. Surgery should be performed as soon as possible following diagnosis, particularly due to the inefficacy of medical treatment. In selected cases, transcatheter closure may represent a valid option. The condition is associated with a high mortality rate. Moreover, complications, particularly in the form of residual aortic valve regurgitation, may hamper postoperative prognosis. Due to the rarity of the disease, the setting up of an international registry is recommended.

Aorto-left ventricular tunnel (ALVT) is a paravalvular communication between aorta and left ventricle. It is one of the rare congenital heart diseases which could present with heart failure. A case of ALVT was diagnosed in infancy. Preliminary assessment was possible using conventional echocardiography; however, functional assessment and accurate anatomy of ALVT were further verified via variable imaging modalities starting from speckle tracking and three-dimensional echocardiography to cardiac CT angiogram. The tunnel was successfully repaired with uneventful recovery. Multimodality imaging can accurately assess cardiac function and demonstrate the anatomy of ALVT noninvasively to plan for successful intervention.