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Ann Thorac Surg 2010;89:1805-1814. doi:10.1016/j.athoracsur.2010.02.004
© 2010 The Society of Thoracic Surgeons
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Edward Hickey
Constantine Mavroudis
Jeffrey P. Jacobs
Marshall L. Jacobs
Carl L. Backer
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Constantine D. Mavroudis
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Right arrow Congenital - cyanotic

Original Articles: Pediatric Cardiac

Impact of Noncardiac Congenital and Genetic Abnormalities on Outcomes in Hypoplastic Left Heart Syndrome

Angira Patel, MD, MPHa, Edward Hickey, MDc, Constantine Mavroudis, MDd,*, Jeffrey P. Jacobs, MDe, Marshall L. Jacobs, MDf, Carl L. Backer, MDb, Melanie Gevitz, BAb, Constantine D. Mavroudis, BAg

a Division of Cardiology, Children's Memorial Hospital, Northwestern University, Chicago IL
b Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Northwestern University, Chicago IL
c Department of Surgery, CHSS Data Center and University of Toronto, Toronto General Hospital, Toronto, Canada
d Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, OH
e Division of Thoracic and Cardiovascular Surgery, All Children's Hospital, University of South Florida College of Medicine, St. Petersburg, FL
f Department of Cardiothoracic Surgery, Drexel University College of Medicine, Philadelphia, PA
g Loyola University Chicago-Stritch School of Medicine, Maywood, IL

Accepted for publication February 3, 2010.

* Address correspondence to Constantine Mavroudis, MD, Cleveland Clinic Foundation, 9500 Euclid Ave/M41-02, Cleveland, OH 44195 (Email: mavrouc{at}ccf.org).

Presented at the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.

Background: Hypoplastic left heart syndrome may coexist with noncardiac congenital defects or genetic syndromes. We explored the impact of such lesions on outcomes after staged univentricular palliation.

Methods: Society of Thoracic Surgeons database 2002 to 2006: Children diagnosed with hypoplastic left heart syndrome who underwent stage 1 Norwood (n = 1,236), stage 2 superior cavopulmonary anastamosis (n = 702) or stage 3 Fontan (n = 553) procedures were studied. In-hospital mortality, postoperative complications, and length of stay were compared at each stage between those with and without noncardiac-genetic defects. Congenital Heart Surgeons' Society database 1994 to 2001: All 703 infants enrolled in the Congenital Heart Surgeons' Society critical left ventricular outflow tract obstruction study who underwent primary stage 1 palliation were reviewed. The impact of noncardiac defects-syndromes on survival was explored using multivariable parametric models with bootstrap bagging.

Results: Society of Thoracic Surgeons database: Stage 1 in-hospital mortality (26% vs 20%, p = 0.04) and mean postoperative length of stay (42 versus 31 days, p < 0.0001) were greater, and postoperative complications significantly more prevalent in infants with noncardiac-genetic defects. Congenital Heart Surgeons' Society database: Noncardiac-genetic defects were present in 55 (8%). Early hazard for death after Norwood was significantly worse in infants with noncardiac defects-syndromes (p = 0.008). Chromosomal defects (n = 14) were highly unfavorable: the early risk of death was doubled (10-year survival 25 ± 9% vs 54 ± 2%, p = 0.005). Turner syndrome accounted for the majority of chromosomal defects in this population (11 of 14, 79%). Mode of death was rarely attributable to the noncardiac-genetic defect.

Conclusions: Survival in hypoplastic left heart syndrome is strongly influenced by the presence of noncardiac abnormalities. Strategies to improve mortality in infants with noncardiac abnormalities should be explored. Presence of chromosomal defects, especially Turner syndrome, should enter decision-management options for parents and physicians.




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