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Published on: August 2, 2019
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Clinical-Physiological Considerations in Patients Undergoing Staged Palliation for a Functionally Single Ventricle
Daniel J Penny1, Rajesh Krishnamurthy
11Department of Pediatrics, Section of Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX. 2Department of Radiology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX.
Summary
Patients with a functionally univentricular heart have unique circulatory physiology. Understanding these distinct characteristics is crucial for managing complex congenital heart disease patients effectively.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- The circulation in functionally univentricular hearts differs significantly from normal biventricular circulation.
- Profound differences exist in ventricular function, myocardial architecture, and interventricular interactions.
Purpose of the Study:
- To discuss the pathophysiology of circulation in functionally univentricular hearts.
- To highlight unique physiologic characteristics guiding patient management.
Main Methods:
- Literature review of MEDLINE and PubMed databases.
Main Results:
- Unique features in systemic ventricle-aorta coupling and Fontan anastomosis orientation impact cardiac output.
- The "portal" pulmonary arterial bed is a critical determinant of cardiovascular function.
Conclusions:
- Disease-specific strategies are essential for optimizing cardiovascular function at all care stages.
- Management requires a deep understanding of the distinct physiology in these complex patients.
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