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Original Articles: Pediatric Cardiac

Midterm Results for Collaborative Treatment of Pulmonary Atresia With Intact Ventricular Septum

^a Department of Cardiovascular Surgery, Congenital Heart Institute at Miami Children's Hospital, Miami, Florida
^b Department of Cardiology, Congenital Heart Institute at Miami Children's Hospital, Miami, Florida
^c Department of Pediatric Cardiology, Arnold Palmer Hospital for Children, Orlando, Florida

Accepted for publication January 16, 2009.

^_* Address correspondence to Dr Hannan, Division of Cardiovascular Surgery, Miami Children's Hospital, 3100 SW 62nd Ave, Miami, FL 33155 (Email: rhannan001{at}aol.com).

Presented at the Poster Session of the Forty-fourth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008.

Background: We report a single-institution experience using a collaborative surgical and catheter-based approach to the initial treatment of pulmonary atresia with intact ventricular septum.

Methods: A retrospective review was conducted of all neonates admitted with pulmonary atresia with intact ventricular septum from 1996 to March 2007.

Results: We identified 24 patients with a mean age at first intervention of 4.5 days with mean follow-up of 6.05 years (range, 1.9 to 12.7 years). Initial palliation was determined by right ventricular size, morphology, and presence or absence of right ventricular–dependent coronary circulation. Initial catheter-based pulmonary valve perforation and valvuloplasty was performed in 41.7% (10 of 24 patients; group A), and 58.3% (14 of 24 patients) had an initial systemic-to-pulmonary artery shunt (group B). Tricuspid valve size was significantly smaller in group B (median z-score, –0.52 group A versus –2.40 group B; p < 0.001). Placement of a shunt after valvuloplasty in group A was required in 70.0% (7 of 10 patients). There was no mortality in group A, and 70.0% (7 of 10 patients) are in a two-ventricle pathway and 30.0% (3 of 10 patients) are in a 1.5-ventricle pathway. Group B had mortality of 14.3% (2 of 14 patients), both within 5 days of surgery. All group B patients remain in a single-ventricle pathway. Overall survival is 91.7% (22 of 24 patients).

Conclusions: An individualized approach to this complex lesion has good results. If the right ventricle can be safely decompressed and appears usable, the need for a shunt after valvuloplasty does not preclude two-ventricle (or 1.5-ventricle) repair. Anatomy mandating a shunt as initial palliation has substantial early mortality.