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J Thorac Cardiovasc Surg 2004;127:1000-1008
© 2004 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum

^a Division of Cardiovascular Surgery Hospital for Sick Children, Toronto, Ontario, Canada,
ⁱ Division of Pediatric Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada
^b Department of Surgery, Wake Forest University School of Medicine, Winston-Salem, NC, USA
^c Divisions of Cardiothoracic Surgery and Biostatistics and Epidemiology, Cleveland Clinic Foundation, Cleveland, Ohio, USA
^d Division of Cardiothoracic Surgery, Children's Hospital of Los Angeles, Los Angeles, Calif, USA
^e Department of Cardiac Surgery, The Children's Hospital, Boston, Mass, USA
^f Division of Cardiothoracic Surgery, Children's Hospital of Pittsburgh, Pittsburgh, Pa, USA
^g Division of Pediatric Cardiothoracic Surgery, Children's Hospital Medical Center, Cincinnati, Ohio, USA
^h Department of Cardiac Surgery, Children's Mercy Hospital, Kansas City, Mo, USA

Read at the Eighty-third Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass, May 4-7, 2003.

Received for publication May 9, 2003; revisions received November 11, 2003; accepted for publication November 17, 2003.

^* Address for reprints: William G. Williams, MD, Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada
bill.williams{at}sickkids.ca

OBJECTIVE: We sought to define the prevalence of definitive end states and their determinants in children given a diagnosis of pulmonary atresia and intact ventricular septum during the neonatal period.

METHODS: Between 1987 and 1997, 408 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective study by 33 institutions. Competing risks analysis was used to demonstrate the prevalence of 6 end states. Factors predictive of attaining each end state were identified by means of multivariable analysis with bootstrap validation.

RESULTS: Overall survival was 77% at 1 month, 70% at 6 months, 60% at 5 years, and 58% at 15 years. Prevalence of end states 15 years after entry were as follows: 2-ventricle repair, 33%; Fontan repair, 20%; 1.5-ventricle repair, 5%; heart transplant, 2%; death before reaching definitive repair, 38%; and alive without definitive repair, 2%. Patient-related factors discriminating among end states primarily included adequacy of right-sided heart structures, degree of aberration of coronary circulation, low birth weight, and tricuspid valve regurgitation. After adjusting for these factors, 2 institutions were predictive of 2-ventricle repair, 1 of Fontan repair, and 6 of death before definitive repair. Two institutions were predictive of both 2-ventricle and Fontan repair. These 2 institutions achieved a higher risk-adjusted prevalence of definitive repair and a lower prevalence of prerepair mortality.

CONCLUSIONS: Characteristics of neonates with pulmonary atresia and intact ventricular septum predict type of definitive repair. A morphologically driven institutional protocol emphasizing both 2-ventricle and Fontan pathways might mitigate the negative effect of unfavorable morphology. In the current era, 85% of neonates are likely to reach a definitive surgical end point, with 2-ventricle repair achieved in an estimated 50%.

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