Cardiac surgical outcomes of patients with heterotaxy syndromeCentral MessagePerspective
Manan H. Desai, MD,
Nicolle M. Ceneri, MD,
Zaenab Dhari, MD,
Aybala Tongut, MD,
Mahmut Ozturk, MD,
Steven J. Staffa, MS,
David Zurakowski, MS, PhD,
David Schidlow, MD, MMus,
Pranava Sinha, MD,
Richard A. Jonas, MD,
Can Yerebakan, MD
Affiliations
Manan H. Desai, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Nicolle M. Ceneri, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Zaenab Dhari, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Aybala Tongut, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Mahmut Ozturk, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Steven J. Staffa, MS
Departments of Anesthesiology and Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass
David Zurakowski, MS, PhD
Departments of Anesthesiology and Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass
David Schidlow, MD, MMus
Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, Mass
Pranava Sinha, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Richard A. Jonas, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC
Can Yerebakan, MD
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC; Address for reprints: Can Yerebakan, MD, Division of Cardiovascular Surgery, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, 111 Michigan Ave NW, Washington, DC 20010.
Objectives: The study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality. Methods: A retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan–Meier analysis for survival were performed. Results: Patient mortality in the cohort was 34% (28/82), including 36% (20/55) for single ventricle palliation and 30% (8/27) for biventricular repair. At 5 years, the probability of survival did not differ between the groups by log-rank testing (P = .829). Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; P < .001), total anomalous pulmonary venous return (hazard ratio, 4.3; 95% confidence interval, 1.7-10.8; P = .002), and birth weight 2.5 kg or less (hazard ratio, 2.4; 95% confidence interval, 1.0-5.4; P = .041) to be independent risk factors for mortality in all-comers. Pulmonary vein stenosis was a univariate predictor of mortality among all patients with heterotaxy (hazard ratio, 3.0; 95% confidence interval, 1.4-6.4; P = .005) and in the subgroup of patients with single ventricles (hazard ratio, 4.0; 95% confidence interval, 1.7-9.7; P = .002). Overall survival was 66% (54/82) at a median follow-up time of 2.2 years (0.4-4.1) from the initial surgery. Conclusions: Outcomes of children with heterotaxy syndrome, irrespective of the operative pathway, remain suboptimal in the current era. Risk factors for mortality in this population include birth weight 2.5 kg or less, extracorporeal membrane oxygenation, pulmonary vein stenosis, and total anomalous pulmonary venous return, which may help to further optimize surgical decision making. Multiorgan system involvment is frequently encountered in these patients.
