Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect

A severely restrictive aerial septal defect (ASD) in neonates with hypoplas tic left heart syndrome (HLHS) results in pulmonary venous hypertension, pu lmonary edema, ana intractable hypoxia, Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheteri zation at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography, Three underwent blade septostomy with 2 procedure- related deaths, and 1 had balloon aerial septostomy (BAS); all 4 died befor e surgical intervention, Fifteen underwent Brocken-brough atrial septoplast y with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a scent placed a cross the atrial septum after transatrial needle puncture. In the 16 patien ts treated with septoplasty or stent, oxygen saturation increased from 50 /- 4% io 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting trans plantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation, Eight of 14 (57%) survived to hospital discharge. Six of 7 ( 86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in pro found cyanosis demands urgent intervention, BAS is Frequently unsuccessful and blade septostomy has high mortality, Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, all owing stabilization before reconstructive surgery or while awaiting transpl ant. (C) 1999 by Excerpta Medico, Inc.