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J Thorac Cardiovasc Surg 2010;139:e35-e36
© 2010 The American Association for Thoracic Surgery

Brief Clinical Report

Sex reversal and hypoplastic left heart syndrome

^a Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC
^b Division of Pediatric Endocrinology, Medical University of South Carolina, Charleston, SC

Received for publication August 11, 2008; accepted for publication September 11, 2008.

^_* Address for reprints: Andrew M. Atz, MD, Director, Pediatric Cardiac Intensive Care, Children's Heart Program of South Carolina, Medical University of South Carolina, 165 Ashley Ave, PO Box 250915, Charleston, SC 29425. (Email: atzam@musc.edu).

The first 20% of the full text of this article appears below.

	Introduction

 
The prognosis of congenital heart disease, especially in its most complex forms, is complicated when associated with extracardiac anomalies. Meacham syndrome, or PAGOD syndrome (pulmonary tract and pulmonary artery hypoplasia, agonadism, omphalocele, diaphragmatic defect, and dextrocardia) is a constellation of genital, cardiac, and pulmonary malformations that carries a poor prognosis. We report the case of a patient with sex reversal and hypoplastic left heart syndrome without congenital diaphragmatic hernia who has survived to Fontan completion and is doing well.

	Clinical Summary

 
The patient, whose condition was diagnosed prenatally with fetal ultrasonography, was found at birth to have hypoplasia of the left ventricle and the mitral and aortic valves, with coarctation of the aorta and proximal stenoses of the branch pulmonary arteries. A Norwood procedure with the Sano modification was performed on day 6 after birth. After 2 days of persistent hypoxemia, the patient was taken back to the operating room, where the Sano shunt was . . . [Full Text of this Article]