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Ann Thorac Surg 2006;82:2214-2220
© 2006 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Pulmonary Atresia With Intact Ventricular Septum: Initial Management

^a Division of Cardiothoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
^b Department of Surgery, University of Cincinnati, Cincinnati, Ohio

Accepted for publication June 28, 2006.

^_* Address correspondence to Dr Pearl, Cincinnati Children’s Hospital Medical Center, Division of Cardiothoracic Surgery, 3333 Burnet Ave, ML 2004, Cincinnati, OH 45229 (Email: jeffrey.pearl{at}cchmc.org).

Presented at the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 30–Feb 1, 2006.

BACKGROUND: Outcomes for pulmonary atresia with intact ventricular septum are suboptimal, while initial management remains controversial. This study was undertaken to determine the effect of catheter-based therapy on the need for early surgical intervention.

METHODS: A single-institution retrospective chart review was made of all 25 neonates with pulmonary atresia with intact ventricular septum from 1999 to 2005.

RESULTS: Mean age at first intervention was 3.1 ± 2.2 days, mean weight 3.3 ± 0.5 kg. Right ventricular hypoplasia varied: 20% normal, 16% mild, 28% moderate, 28% moderately severe or severe, 8% not classified. Median tricuspid valve z-score was –2.3 ± 2.6. First intervention was catheter-based therapy in 56% (14 of 25), operative in 36% (9 of 25), and no therapy in 2. Technically adequate valvotomy was achieved in 79% (11 of 14). Serious catheter-related complications occurred in 3 of 14 (21%). Only 5 of 14 (36%) with catheter-based therapy weaned from prostaglandins without surgery. Of these, 2 required surgery for cyanosis at 1 and 3 months. Surgery after catheter-based therapy consisted of right ventricular outflow patch in 36% (4 of 11), systemic to pulmonary shunt in 64% (7 of 11). Median time between catheter-based therapy and surgery was 8.5 days (range, 1 to 89). Only 3 of the 23 treated patients avoided operation during infancy. There was 1 early and 1 late death after operation after initial catheter-based therapy, and 1 late death after primary surgery alone during a mean follow-up of 33 months (range, 1.5 to 79).

CONCLUSIONS: Balloon valvotomy alone for pulmonary atresia with intact ventricular septum rarely obviates the need for an additional source of pulmonary blood flow—either shunt or ductal stenting.

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				H. Zhang, S.-j. Li, Y.-q. Li, H. Wang, and S.-s. Hu Hybrid procedure for the neonatal management of pulmonary atresia with intact ventricular septum J. Thorac. Cardiovasc. Surg., June 1, 2007; 133(6): 1654 - 1656. [Full Text] [PDF]