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Pulmonary Atresia with Intact Ventricular Septum

  • Daniel J. Sisti
  • Karim T. Rafaat
Chapter

Abstract

A newborn with previously unrecognized pulmonary atresia and intact ventricular septum (PA/IVS) presents a medical and surgical emergency if the ductus arteriosus closes, the only source of pulmonary blood flow in this form of congenital heart disease. PA/IVS is a rare form of congenital heart disease, and it is characterized by severe stenosis or atresia of the pulmonary valve or right ventricular outflow tract. Without another shunt such as a ventricular septal defect, the only source of pulmonary blood flow is a patent ductus arteriosus. The ductus can be kept open with a prostaglandin infusion until the infant is ready for surgical correction. Without ductus patency, the newborn will become progressively hypoxemic and ultimately die from cardiovascular collapse. Generally speaking, patients with PA/IVS have two potential surgical outcomes. If the right ventricle (RV) is not small or dysplastic, they may undergo primary repair and have a normal biventricular heart. If the RV is hypoplastic, then the patient will undergo surgical palliation. The first stage of palliation is the Norwood procedure, which establishes a parallel pulmonary and systemic circulation. The second stage is the bidirectional Glenn or hemi-Fontan, which unloads the ventricle and prepares the patient for the third and final stage of palliation, the Fontan procedure. With the Fontan procedure, all pulmonary blood flow flows passively through the lungs, while the single ventricle pumps only the systemic blood flow. Each stage of palliation presents unique challenges to the anesthesiologist, requiring techniques to modulate pulmonary vascular resistance and to optimize systemic oxygen delivery.

Keywords

Congenital heart disease Prostaglandin Norwood procedure Bidirectional Glenn Fontan procedure 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Daniel J. Sisti
    • 1
  • Karim T. Rafaat
    • 1
  1. 1.Department of AnesthesiologyUniversity of CaliforniaSan DiegoUSA

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