Pulmonary atresia with ventricular septal defect

Frank L. Hanley, V. Mohan Reddy, Mark Rodefeld

Research output: Chapter in Book/Report/Conference proceedingChapter


Pulmonary atresia with ventricular septal defect (PA-VSD) is an uncommon complex congenital cardiac lesion in which no luminal continuity exists between the right ventricle (RV) and the pulmonary arteries. PA-VSD represents the most severe end of the spectrum of tetralogy of Fallot and is often referred to as ‘tetralogy of Fallot with pulmonary atresia.’ The intracardiac anatomy is consistent and similar to that of tetralogy of Fallot with an anteriorly malaligned VSD, a welldeveloped left ventricle (LV), and a variable degree of RV infundibular hypoplasia. The extracardiac pulmonary blood supply, however, has great morphological variability as a hallmark characteristic.

Original languageEnglish (US)
Title of host publicationOperative Cardiac Surgery, Fifth Edition
PublisherCRC Press
Number of pages15
ISBN (Electronic)9781444114171
ISBN (Print)9780340759745
StatePublished - Jan 1 2004


ASJC Scopus subject areas

  • Medicine(all)

Cite this

Hanley, F. L., Reddy, V. M., & Rodefeld, M. (2004). Pulmonary atresia with ventricular septal defect. In Operative Cardiac Surgery, Fifth Edition (pp. 705-719). CRC Press.