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Surgical Management of Hypoplastic Left Heart Syndrome

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Pediatric Critical Care

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Abstract

Congenital heart disease is the most common birth defect. The most common severe form of congenital heart disease is hypoplastic left heart syndrome (HLHS), affecting almost 1000 US births per year. Hypoplastic left heart syndrome is defined by underdevelopment of the left side of the heart and obstruction to systemic outflow. This consists of mitral stenosis or atresia, a non-apex-forming hypoplastic left ventricle, aortic stenosis or atresia, a hypoplastic ascending and arch aorta, coarctation of the aorta, and a patent ductus arteriosus.

Current traditional surgical management of HLHS consists of staged palliation to a Fontan circulation. This is composed of the Norwood procedure at birth, second-stage superior cavopulmonary connection at typically 4–6 months of age, and a completion Fontan procedure at 18–48 months of age.

Based on this standard management, outcomes have dramatically improved. However, despite these advances, there is still significant morbidity and mortality associated with HLHS. This has led to investigation into new and alternative therapies. As a result, there is substantial practice pattern variation among institutions and between individual surgeons, cardiologists, and intensivists. This has therefore led to multiple current controversies regarding surgical management of HLHS.

Current controversies include regionalization of care to centers of excellence, the role of fetal cardiac intervention, appropriate management at each stage of palliation, and the role of mechanical circulatory support and transplantation. Regarding first-stage palliation with the Norwood procedure, the appropriate shunt type, use of deep hypothermic circulatory arrest or regional cerebral perfusion, need for delayed sternal closure, and differences in postoperative management remain debated. Lastly, the evolving role of the hybrid Norwood procedure and subsequent management is also being studied.

Advancements in understanding and continued collaboration will be paramount to establish best practices and resolve these current controversies. The hope is this will translate to continued improvement in outcomes for this challenging group of patients.

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  1. Department of Cardiac Surgery, Section of Pediatric Cardiovascular Surgery, University of Michigan C.S. Mott Children’s Hospital, Ann Arbor, MI, USA

    Peter Sassalos & Richard G. Ohye

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  1. Peter Sassalos
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  1. Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA

    Christopher W. Mastropietro

  2. Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA

    Kevin M. Valentine

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Sassalos, P., Ohye, R.G. (2019). Surgical Management of Hypoplastic Left Heart Syndrome. In: Mastropietro, C., Valentine, K. (eds) Pediatric Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-319-96499-7_7

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