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Hypoplastic Left Heart Syndrome and Other Shunt-Dependent Single Ventricles

Tabbutt, Sarah MD, PhD1; Tweddell, James S. MD2; Ghanayem, Nancy MD3

Pediatric Critical Care Medicine: August 2016 - Volume 17 - Issue 8 - p S318-S322
doi: 10.1097/PCC.0000000000000783
Congenital Heart Disease

Objectives: In this review, we discuss the pathophysiology, treatment, and outcomes of patients with the hypoplastic left heart syndrome and other single ventricle variants prior to and following surgery.

Data Source: MEDLINE and PubMed.

Conclusions: Patients with shunted single ventricle physiology are at increased risk for acute hemodynamic decompensation owing to the increased myocardial workload, the dynamic balance between systemic and pulmonary circulations, and the potential for shunt obstruction. Understanding of the physiology and anticipatory management are critical to prevent hemodynamic compromise and cardiac arrest.

1Division of Cardiology and Critical Care Medicine, Department of Pediatrics, University of California San Francisco School of Medicine, University of California San Francisco, Benioff Children’s Hospital, San Francisco, CA.

2Department of Surgery, Heart Institute, Cincinnati College of Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH.

3Division of Critical Care Medicine, Medical College of Wisconsin, Children’s Hospital of Wisconsin, Milwaukee, WI.

The authors have disclosed that they do not have any potential conflicts of interest.

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Copyright © 2016 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies