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Ann Thorac Surg 2006;82:1629-1636
© 2006 The Society of Thoracic Surgeons

Original Articles: Cardiovascular

Heterotaxy Syndrome with Functional Single Ventricle: Does Prenatal Diagnosis Improve Survival?

Meryl S. Cohen, MDa,*, Amy H. Schultz, MDa, Zhi-Yun Tian, MDa, Denise D. Donaghue, RNa, Paul M. Weinberg, MDa, J. William Gaynor, MDb, Jack Rychik, MDa

a Divisions of Cardiology and Cardiothoracic Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
b Departments of Pediatrics and Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

Accepted for publication May 11, 2006.

* Address correspondence to Dr Cohen, Division of Cardiology, The Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104 (Email: cohenm{at}email.chop.edu).

BACKGROUND: Despite improved outcome for many single ventricle lesions, staged reconstruction for heterotaxy syndrome with a functional single ventricle continues to have a high mortality. Prenatal identification of heterotaxy syndrome may improve long-term survival.

METHODS: Our database was reviewed from January 1996 to December 2004 for patients with heterotaxy syndrome. Assessment was made for prenatal diagnosis and echocardiographic characteristics of heterotaxy syndrome. We sought to assess the accuracy of fetal echocardiography in the diagnosis of heterotaxy syndrome and determine whether prenatal diagnosis and other risk factors have an impact on survival in patients with heterotaxy syndrome.

RESULTS: Of 81 patients that met criteria, 43 (53%) had prenatal diagnosis. Prenatal diagnosis had high specificity and positive predictive value for all findings but had low sensitivity for anomalous pulmonary veins. Among the 70 patients born alive, survival was 60% with median follow-up of 51.4 months (range, 6.5 to 109.7 months). Prenatal diagnosis did not improve survival (p = 0.09). None of the 11 patients with complete heart block (CHB) survived past 3 months of age. Two patients underwent heart transplantation as their first intervention and have survived. CHB and anomalous pulmonary venous connection were associated with shorter duration of survival.

CONCLUSIONS: Prenatal diagnosis of heterotaxy syndrome does not improve survival in patients who undergo single ventricle reconstruction. The most potent risk factors for poor outcome (CHB, anomalous pulmonary veins) are likely not impacted by identification in utero. In light of the poor outcome, cardiac transplantation as an initial therapy may be a viable option for some patients.






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