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Ann Thorac Surg 2009;87:1214-1219. doi:10.1016/j.athoracsur.2008.11.032
© 2009 The Society of Thoracic Surgeons
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Original Articles: Pediatric Cardiac

Right Ventricular Performance in the Fetus With Hypoplastic Left Heart Syndrome

Anita Szwast, MDa,b, Zhiyun Tian, MDa, Margaret McCann, RDMSa, Denise Donaghue, RN, MSNa, Jack Rychik, MDa,b,*

a The Fetal Heart Program at the Cardiac Center at The Children's Hospital of Philadelphia, Pennsylvania
b Division of Cardiology, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

Accepted for publication November 12, 2008.

* Address correspondence to Dr Rychik, The Fetal Heart Program at The Cardiac Center at The Children's Hospital of Philadelphia, 6th Floor Main Building, 34th and Civic Center Blvd, Philadelphia, PA 19104 (Email: rychik{at}email.chop.edu).

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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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Invited Commentary
Jennifer C. Hirsch
Ann. Thorac. Surg. 2009 87: 1219. [Extract] [Full Text] [PDF]



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J. C. Hirsch
Invited Commentary
Ann. Thorac. Surg., April 1, 2009; 87(4): 1219 - 1219.
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