Endocarditic sinus of valsalva fistulae to right ventricular outflow tract in adult ventricular septal defects

•Perioperative management of adult congenital cardiac patient with left to right shunting who underwent successful repair•Necessary preoperative testing to determine if surgical closure of intracardiac shunt is feasible•Long-term outcomes of those who underwent repair and those who did not

Surgical repair of congenital ventricular septal defects (VSDs) in adults is quite rare. Most congenital VSDs are repaired in children. Of those adult patients diagnosed as having VSDs, many are not repaired due to irreversible pulmonary vascular disease. Repair in a patient with a VSD and fistula is even more uncommon. From a review of the literature, we found no other case reports with our unique combination of echocardiographic and surgical findings: a supracristal VSD, right and left sinus of Valsalva fistulas into the right ventricular outflow tract, and a pulmonary artery to pulmonary vein fistula in the context of an aseptic endocarditis lesion. We review the important aspects of anesthetic management in an adult with an intracardiac shunt. An adult patient with unrepaired congenital VSD may develop multiple fistulas as a consequence of endocarditis. This patient refused surgery until the progressive dyspnea was worsened by the endocarditis and the fistulas. At the time of surgery, his ventricular ejection fraction measured 47%, the ventricular chambers were enlarged, and the pulmonary to systemic flow ratio measured 2:1. He did well clinically after the VSD and fistulae repair.