Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum. (September 2018)

• Record Type: Journal Article
• Authors: Gleich, Stephen; Latham, Gregory J.; Joffe, Denise; Ross, Faith J.
• Is Part Of: Seminars in cardiothoracic and vascular anesthesia. Volume 22:Number 3(2018)
• Page Start: 256
• Page End: 264
• Publication Date: 2018-09
• Subjects: cardiac anesthesia -- congenital heart disease -- children -- intraoperative assessment -- cardiac surgery -- cardiopulmonary bypass surgery; Cardiovascular system -- Surgery -- Methods -- Periodicals; Chest -- Surgery -- Methods -- Periodicals; Anesthesia -- Periodicals; Anesthetics -- Periodicals; Anesthesia -- Periodicals; Anesthetics -- Periodicals; Cardiovascular Surgical Procedures -- methods -- Periodicals; Thoracic Surgical Procedures -- methods -- Periodicals; 617.96
• DOI: 10.1177/1089253217737180
• Languages: English
• ISSNs: 1089-2532

Abstract

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation. Cardiac transplantation is recommended in the most severe cases. This review will discuss the perioperative and anesthetic management of patients with PA/IVS and highlight the challenges in management.