In patients with aorta-right atrial tunnel, 2-dimensional echocardiography and transesophageal echocardiography are enough to establish clinical diagnosis, but ascending aortography is necessary to differentiate from more common clinical conditions, like ruptured sinus of Valsalva aneurysm and coronary cameral fistula. The rarity of this condition is established by the fact that during the same period of time, we have treated in our institution 66 cases of ruptured sinus of Valsalva aneurysm, which is the most common aorta-right atrial communication. Treatment options are simple ligation or ligation with implantation of coronary ostium or coil embolization. The location of the coronary ostium dictates technical details. Follow-up reveals excellent functional recovery.
Background
Persistent left superior vena cava is a well-recognized thoracic venous abnormality, most of which do not present with any symptoms. Its opening into the left atrium is uncommonly encountered and, if present, is noted to be associated with various other complex intracardiac malformations, which further complicates its management. Herein, we highlight a case of an adult with persistent left superior vena cava with a spectrum of other cardiac anomalies rarely encountered with it, which was effectively managed by tailored surgical intervention.
Case presentation
A 26-year-old female on meticulous evaluation was found to have left isomerism with an interrupted inferior vena cava and hemiazygos continuing as a persistent left superior vena cava. It was also associated with common atrium, partial atrioventricular canal defect, and complete heart block. This complex array of anomalies was managed surgically. Common atrium was closed with atrial neo-septation using autologous pericardial patch tailored in a way, such that the persistent left superior vena cava drained into right atrium with left atrial appendage being on right side of neo-septum. Cleft in the left atrioventricular valve was also repaired. Despite preoperative complete heart block, the patient had an uneventful postoperative recovery and was in atrial fibrillation with controlled ventricular rate, thus precluding the need for permanent pacemaker implantation.
Conclusions
A rare spectrum of adult congenital cyanotic heart disease was successfully managed with thorough preoperative evaluation and judicious intraoperative management. Surgical management should be tailored based on specific anatomy for reduction of morbidity and optimum results.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.