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J Thorac Cardiovasc Surg. 2014 Apr;147(4):1291-1298.e2. doi: 10.1016/j.jtcvs.2013.06.018. Epub 2013 Jul 22.

Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction.

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Children's National Heart Institute, Children's National Medical Center, Washington, DC. Electronic address:
Office of Biostatistics Research, National Institutes of Health, Bethesda, Md.
Howard Hughes Medical Institute, National Institutes of Health, Bethesda, Md.
Department of Pediatric Pulmonology, Children's National Medical Center, Washington, DC.
Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pa.
Children's National Heart Institute, Children's National Medical Center, Washington, DC.
National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, Md.



Congenital heart disease (CHD) and heterotaxy patients have increased postoperative and respiratory complications. We recently showed CHD-heterotaxy patients can have respiratory ciliary dysfunction (CD) similar to that associated with primary ciliary dyskinesia, including low nasal nitric oxide and abnormal ciliary motion. In this study, we investigated whether CHD-heterotaxy patients with CD may have worse postsurgical outcomes.


We examined postsurgical outcome in 13 heterotaxy-CHD patients with CD (25 surgeries), compared with 14 heterotaxy-CHD patients without CD (27 surgeries). Outcome data were collected for each surgery, including respiratory complications, tracheostomy, use of inhaled β-agonists or nitric oxide, length of hospital stay, days on ventilator, and death.


The CD versus the no-CD CHD cohorts had similar Risk Adjustment in Congenital Heart Surgery-1 risk categories, repair track, age at surgery, and follow-up evaluation times. Respiratory complications (76% vs 37%; P = .006), need for tracheostomy (16% vs 0%; P = .047), and use of inhaled β-agonists (64% vs 11%; P = .0001) all were increased significantly in heterotaxy-CHD patients with CD. No significant differences were detected in postoperative hospital stay, days on mechanical ventilation, or surgical mortality. A trend toward increased mortality for the CD group beyond the postoperative period was observed (33% vs 0%; P = .055) in patients younger than age 10 years.


Our findings showed that heterotaxy-CHD patients with CD may have increased risks for respiratory deficiencies. Overall, there was a trend toward increased mortality in CD patients with intermediate follow-up evaluation. Because β-agonists are known to increase ciliary beat frequency, presurgical screening for CD and perioperative treatment of CD patients with inhaled β-agonists may improve postoperative outcomes and survival.

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