Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum

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 ventriculoc...

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Veröffentlicht in:Seminars in cardiothoracic and vascular anesthesia 2018-09, Vol.22 (3), p.256-264
Hauptverfasser: Gleich, Stephen, Latham, Gregory J., Joffe, Denise, Ross, Faith J.
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container_title Seminars in cardiothoracic and vascular anesthesia
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creator Gleich, Stephen
Latham, Gregory J.
Joffe, Denise
Ross, Faith J.
description 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.
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subjects Anesthesia - methods
Coronary Circulation
Heart Defects, Congenital - diagnostic imaging
Heart Defects, Congenital - physiopathology
Heart Defects, Congenital - surgery
Humans
Perioperative Care
Pulmonary Atresia - diagnostic imaging
Pulmonary Atresia - physiopathology
Pulmonary Atresia - surgery
title Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum
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