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Ann Thorac Surg 2005;79:596-606
© 2005 The Society of Thoracic Surgeons
a Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
b Department of Cardiovascular Surgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
c Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
d Department of Biostatistics, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
Accepted for publication July 6, 2004.
* Address reprint requests to Dr Hancock Friesen, #2269 New Halifax Infirmary, 1796 Summer St, Halifax, NS B3H 3A7, Canada (E-mail: camillehf{at}hotmail.com).
Presented at the Fortieth Annual Meeting of The Society of Thoracic Surgeons, San Antonio, TX, Jan 26–28, 2004.
BACKGROUND: Repair of total anomalous pulmonary venous connection (TAPVC) continues to be associated with significant mortality. We reviewed patients undergoing consecutive TAPVC repairs over a 10-year period at Children's Hospital Boston. The impact of current surgical and perioperative management strategies on short-term outcomes (postrepair pulmonary venous obstruction and mortality) is evaluated.
METHODS: All patients with surgically corrected TAPVC from November 1989 to December 2000 were included. Charts were reviewed for patient demographics, operation variables, and postoperative course.
RESULTS: There were 123 patients in the cohort, of which 72 (59%) were male. The median age and weight at operation were 10 days and 3.6 kg, respectively. Sixty-eight (55%) patients presented with pulmonary venous obstruction, and 65 (53%) underwent emergent TAPVC repair. Thirty-nine (32%) had single-ventricle anatomy, and 84 (68%) had two-ventricle anatomy. Thirty patients (24%) died. Kaplan-Meier survival at 1 month was 65% (95% confidence interval [CI], 55% to 75%) for single-ventricle patients versus 90% (95% CI, 90% to 100%) for two-ventricle patients; at 36 months it was 47% (95% CI, 35% to 59%) versus 87% (95% CI, 81% to 93%), respectively. By Cox multivariable regression analysis, a single ventricle (p < 0.001, hazard ratio, 4.8; 95% CI, 2.5 to 9.2) was an independent mortality risk factor. Prerepair pulmonary venous obstruction was a multivariate risk factor for death among single-ventricle patients. Postrepair pulmonary venous obstruction occurred in 11%. If year of operation is used as a predictor, two-ventricle patient survival has significantly improved (p < 0.05).
CONCLUSIONS: Despite current interventions, single-ventricle patients continue to have a worse prognosis than two-ventricle patients.
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