Isolated Right Ventricular Outflow Tract Obstruction

Abstract

Isolated right ventricular outflow tract obstruction (RVOTO) is a spectrum diagnosis with heterogeneous morphology and pathophysiology, leading to equally wide choices of surgical and catheter-based therapies, whose goals range from achieving biventricular repair to single ventricular palliation, en passant by the grayer zone of one-and-a-half repair. This chapter addresses those lesions with RVOTO without a ventricular septal defect and normal ventriculo-arterial connections, which include pulmonary atresia with intact ventricular septum (PA-IVS), neonatal critical pulmonary stenosis, supravalvar, valvar, and infundibular pulmonary stenosis. Similar to the broad diagnosis of isolated RVOTO, PA-IVS has an equally diverse pathophysiology leading to complex surgical management decisions pre-, intra-, and postoperatively. Pulmonary stenosis is a common congenital cardiac defect. Pulmonary stenosis can be broken down into four different entities: valvar, subvalvar, supravalvar, and peripheral pulmonary stenosis.

Authors 2

1. Ali Dodge‐Khatami Aachen

   RWTH Aachen University

   Affiliation as printed

   University of Aachen RWTH, Aachen, Germany

2. Christopher E. Greenleaf

   The University of Texas Health Science Center at Houston

   Affiliation as printed

   University of Texas Health Science Center, Houston, TX, USA