title: Endocarditic sinus of valsalva fistulae to right ventricular outflow tract in adult ventricular septal defects.
abstract: 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.
authors.affiliation: Department of Anesthesiology, Division of Cardiothoracic Anesthesiology, New York University Langone Medical Center, 550 First Ave, TH 530, New York, NY 10016, USA.
authors.forename: Natacha
authors.initials: N
authors.lastname: Zamor
authors.affiliation: Department of Pediatrics, Division of Cardiology, New York University Langone Medical Center, New York, NY, USA; NYU Pediatric Cardiology Associates, 23 160 East 32nd St, 2nd fl 24, New York, NY 10016, USA.
authors.forename: Colin
authors.initials: C
authors.lastname: Phoon
authors.affiliation: Department of Cardiothoracic Surgery, Division of Pediatric and Adult Congenital Cardiac Surgery, New York University Langone Medical Center, 530 First Ave, Suite 9 V, New York, NY 10016, USA.
authors.forename: Sunil
authors.initials: S
authors.lastname: Malhotra
authors.affiliation: Department of Anesthesiology, Division of Cardiothoracic Anesthesiology, New York University Langone Medical Center, 550 First Ave, TH 530, New York, NY 10016, USA. Electronic address: jennie.ngai@nyumc.org.
authors.forename: Jennie
authors.initials: J
authors.lastname: Ngai
mesh_terms: D000328:Adult; D000758:Anesthesia / Q000379:methods; D004417:Dyspnea / Q000209:etiology; D004696:Endocarditis / Q000473:pathology*; D006345:Heart Septal Defects, Ventricular / Q000473:pathology / Q000601:surgery*; D006352:Heart Ventricles / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D012850:Sinus of Valsalva / Q000473:pathology* / Q000601:surgery; D016157:Vascular Fistula / Q000473:pathology* / Q000601:surgery
keywords: Adult congenital cardiac surgery; Anesthetic management of VSD; Sinus of Valsalva fistula; VSD; Ventricular septal defect