Aorto-Right Ventricular Tunnel: A Case Series and Literature Review

Aorto-right ventricular tunnel (ARVT) is a rare cardiac congenital anomaly where an extracardiac channel connects the ascending aorta above the sinutubular junction to the right ventricle. This defect is caused by an abnormal development of the cushions of the aorto-pulmonary outflow tract. A case series and literature review are described. Two cases of ARVT are described. A literature review was conducted, in which 31 cases were reported. In our 2 cases, both ARVTs connected the ascending aorta above the left aortic sinus to the right ventricle (one to the right ventricular outflow tract and one to the right ventricular apex). Both patients underwent successful surgical correction by patch closure of both tunnel orifices, with uneventful postoperative course. Of the 31 ARVT cases described in our review, only 10 patients (32.3%) had an anatomy similar to the 2 cases described. Coronary artery anomalies can be associated, as reported in our 2 patients and in 16 cases (51.6%) in the review. Surgical correction can be achieved by direct closure or, more often, by patch closure of one or both tunnel orifices, depending mostly on coronary anatomy. Two cases of transcatheter device closure were described in literature, in favorable anatomy cases. Careful attention is required during repair to avoid coronary lesions, due to the high incidence of comorbid coronary anomalies.