Hybrid therapy for pulmonary atresia with intact ventricular septum

In patients with pulmonary atresia with intact ventricular septum without right ventricular-dependent coronary circulation, catheter techniques, including the use of a stiff wire, lasers, and radiofrequency, have been most widely used for initial therapy; however, percutaneous perforation and balloon valvuloplasty have been associated with higher rates of procedural failure and serious complications. We report our experience with a hybrid approach involving the combination of surgery and interventional catheterization techniques for right ventricular decompression.

Between March 2005 and April 2010, we performed a hybrid procedure in 30 newborns and infants (age, 1 day to 2 years; median age, 3 months) with favorable anatomy. The heart was exposed by performing midline sternotomy. A purse-string suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then, a 16-gauge intravenous catheter was placed periventricular with the guidance of echocardiography to perforate the atretic pulmonary ventricle. Subsequently, a guide wire was inserted into the sheath and used to guide a balloon across the pulmonary ventricle. Sequential dilations were performed under the guidance of epicardial echocardiography until full opening of the pulmonary ventricle was obtained. In newborns, ductal ligation was performed, followed by modified Blalock-Taussig (mBT) shunt placement. Patients aged greater than 1 month were treated mostly with ductal ligation, and an mBT shunt was inserted if severe systemic oxygen desaturation occurred after ductal ligation; bidirectional Glenn shunt placement was performed if a patient showed severe hypoplasia.

The hybrid procedure was successfully performed in all patients. Patent ductus arteriosus ligation was simultaneously performed in 27 cases. Six newborns were treated with mBT shunt placement after pulmonary valvuloplasty and patent ductus arteriosus ligation, and 2 patients aged greater than 1 month were treated with mBT shunt placement alone. Another 2 patients were selected for univentricular palliation with bidirectional Glenn procedure because of a diminutive monopartite right ventricle. No pericardial effusion or cardiac tamponade was observed. One patient in whom ductal ligation could not be performed was treated with mBT shunt placement because of hypoxemia 3 days after the hybrid procedure; the other patients were discharged without any further surgical intervention. During the follow-up period of 1.5 months to 5 years, 5 patients died; 25 (83.3%) survived and all were in New York Heart Association functional class 1. Saturation of peripheral oxygen in the survivors increased from 73.1±8.5% to 94±3.5% (p<001). Two-ventricle circulation was achieved in 24 patients, whereas 1 patient had a single-ventricle pathway.

In conclusion, periventricular balloon pulmonary valvuloplasty using a hybrid approach was safe and feasible for patients with pulmonary atresia with intact ventricular septum.