Published online January 2, 2007
PEDIATRICS Vol. 119 No. 1 January 2007, pp. 109-117 (doi:10.1542/peds.2006-1592)
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STATE-OF-THE-ART REVIEW ARTICLE

New Developments in the Treatment of Hypoplastic Left Heart Syndrome

Bahaaldin Alsoufi, MDa,b, Jayme Bennetts, MDa, Subodh Verma, MD, PhDa and Christopher A. Caldarone, MDa

a Cardiac Centre, Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
b King Faisal Heart Institute at King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

ABSTRACT

In the current decade, the prognosis of newborns with hypoplastic left heart syndrome, previously considered a uniformly fatal condition, has dramatically improved through refinement of rapidly evolving treatment strategies. These strategies include various modifications of staged surgical reconstruction, orthotopic heart transplantation, and hybrid palliation using ductal stenting and bilateral pulmonary artery banding. The variety of treatment approaches are based on different surgical philosophies, and each approach has its unique advantages and disadvantages. Nonetheless, multiple experienced centers have reported improved outcomes in each one of those modalities. The purpose of this review is to outline recent developments in the array of currently available management strategies for neonates with hypoplastic left heart syndrome. Because the vast majority of deaths in this patient population occur within the first months of life, the focus of the review will be evaluation of the impact of these management strategies on survival in the neonatal and infant periods.

Key Words: hypoplastic left heart syndrome • cardiovascular anomalies • heart transplantation

Abbreviations: HLHS—hypoplastic left heart syndrome • RV-PA—right ventricle to pulmonary artery • BDCPA—bidirectional cavopulmonary anastomosis • PAB/DS—pulmonary artery band and ductal stent

Accepted Jul 28, 2006.


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