Right Ventricular Performance in the Fetus With Hypoplastic Left Heart Syndrome

The Fetal Heart Program, Cardiac Center, Children's Hospital of Philadelphia, Pennsylvania 19104, USA.
The Annals of thoracic surgery (Impact Factor: 3.85). 05/2009; 87(4):1214-9. DOI: 10.1016/j.athoracsur.2008.11.032
Source: PubMed


In the fetus with hypoplastic left heart syndrome (HLHS), the single right ventricle (RV) pumps the entire cardiac output in utero. By investigating RV performance in utero, we sought to determine the inherent capabilities of a single RV before the increased metabolic demands of postnatal life and surgical palliation. In addition, we sought to determine whether the presence or absence of a left ventricular cavity impacts on RV performance in fetal life.
Between November 2004 and December 2006, Doppler flow-derived measures of ventricular performance were obtained with echocardiography in 76 fetuses with normal cardiovascular system and in 48 age-matched fetuses with HLHS from 17 weeks until 40 weeks of gestation. The myocardial performance index, ventricular ejection force, and cardiac output were determined for both groups and compared using unpaired Student's t tests and regression analysis.
In fetuses with HLHS, cardiac output was diminished by 20%, RV ejection force was elevated, and RV myocardial performance index was elevated compared with those of normal fetuses. The presence of a left ventricular cavity did not impact on RV performance in utero.
Fetuses with HLHS have preserved systolic performance but impaired diastolic performance compared with normal fetuses. The heart of a fetus with HLHS is less efficient than the normal heart in that ejection force of the RV is increased, but overall delivery of cardiac output is lower than normal. We conclude that patients with HLHS have inherent limitations in cardiac performance even before birth.

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Available from: Anita Szwast, May 12, 2014
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    • "Cardiac output [32], pulmonary vascular resistance [33], and cerebral vascular resistance [34] have been measured in fetuses with HLHS using Doppler and are discussed below. The accurate identification of a restrictive atrial septum in HLHS by PV VTI ratio is an example of the clinical utility of fetal hemodynamic assessment in CHD, as this enables accurate identification of fetuses that may require emergency neonatal intervention [35,36]. "
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