Title of article :
An Unusual Cause of Hypoxia: Ventricular Septal Defect, Pulmonary Artery Atresia, and Major Aortopulmonary Collaterals Diagnosed in the Adult Cardiac Catheterization Lab
Author/Authors :
Bravo-Jaimes, Katia Division of Cardiology - University of Texas Health Science Center at Houston, Houston, Texas, USA , Walton, Brian Division of Cardiology - University of Texas Health Science Center at Houston, Houston, Texas, USA , Tung, Poyee Division of Cardiology - University of Texas Health Science Center at Houston, Houston, Texas, USA , Smalling, Richard W. Division of Cardiology - University of Texas Health Science Center at Houston, Houston, Texas, USA
Abstract :
The association of pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA) is an
extreme form of tetralogy of Fallot (TOF). It carries a high mortality risk if not intervened on during infancy with only 20% of
unoperated patients surviving into adulthood. We present the case of a 40-year-old man who presented for evaluation prior to
retinal surgery and was found to have hypoxia and a loud murmur. Cardiac catheterization was performed in the general
catheterization laboratory, demonstrating a membranous VSD, pulmonary atresia, and MAPCA. We highlight the challenges
and limitations that an adult interventional cardiologist may have when encountering these patients.
Keywords :
An Unusual Cause of Hypoxia , Ventricular Septal Defect , Pulmonary Artery Atresia , Major Aortopulmonary Collaterals
