Aorto-Left Ventricular Tunnel: The First Systematic Review of An Uncommon Entity (177 Worldwide Cases from 1965 to 2024)

Background: The study was aimed at assessing clinical status and outcome of patients affected by aorto-left ventricular tunnel (ALVT). Methods: 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. Results: 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. Conclusions: 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.