Congenital heart disease
Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy

https://doi.org/10.1016/j.jtcvs.2010.07.082Get rights and content
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Objective

Patients with heterotaxy and complex congenital heart disease underwent cardiac surgery with high mortality and morbidity. Recent studies have revealed an association among heterotaxy, congenital heart disease, and primary ciliary dyskinesia. We undertook a retrospective review of patients undergoing cardiac surgery at Children’s National Medical Center between 2004 and 2008 to explore the hypothesis that there is increased mortality and respiratory complications in heterotaxy patients.

Methods

Retrospective review was performed on postsurgical outcomes of 87 patients with heterotaxy and congenital heart disease exhibiting the full spectrum of situs abnormalities associated with heterotaxy. As controls patients, 634 cardiac surgical patients with congenital heart disease, but without laterality defects, were selected, and surgical complexities were similar with a median Risk Adjustment in Congenital Heart Surgery-1 score of 3.0 for both groups.

Results

We found the mean length of postoperative hospital stay (17 vs 11 days) and mechanical ventilation (11 vs 4 days) were significantly increased in the heterotaxy patients. Also elevated were rates of tracheostomies (6.9% vs 1.6%; odds ratio, 4.6), extracorporeal membrane oxygenation support (12.6% vs 4.9%: odds ratio, 2.8), prolonged ventilatory courses (23% vs 12.3%; odds ratio, 2.1) and postsurgical deaths (16.1% vs 4.7%; odds ratio, 3.9).

Conclusions

Our findings show heterotaxy patients had more postsurgical events with increased postsurgical mortality and risk for respiratory complications as compared to control patients with similar Risk Adjustment in Congenital Heart Surgery-1 surgical complexity scores. We speculate that increased respiratory complications maybe due to ciliary dysfunction. Further studies are needed to explore the basis for the increased surgical risks for heterotaxy patients undergoing cardiac surgery.

CTSNet classification

15:44:36
20
21

Abbreviations and Acronyms

BiV
biventricular
CHD
congenital heart disease
CNMC
Children’s National Medical Center
ECMO
extracorporeal membrane oxygenation
GEE
generalized estimating equation
PCD
primary ciliary dyskinesia
PDA
patent ductus arteriosus
RACHS-1
Risk Adjustment in Congenital Heart Surgery
SV
single ventricle

Cited by (0)

Supported by funds from the National Institutes of Health (grant No. IH ZO1-HL005701). M. Swisher was generously supported by a fellowship from the Howard Hughes Medical Institute.

Disclosures: Authors have nothing to disclose with regard to commercial support.