TY - JOUR
T1 - Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction
AU - Harden, Brandon
AU - Tian, Xin
AU - Giese, Rachel
AU - Nakhleh, Nader
AU - Kureshi, Safina
AU - Francis, Richard
AU - Hanumanthaiah, Sridhar
AU - Li, You
AU - Swisher, Matthew
AU - Kuehl, Karen
AU - Sami, Iman
AU - Olivier, Kenneth
AU - Jonas, Richard
AU - Lo, Cecilia W.
AU - Leatherbury, Linda
N1 - Funding Information:
Supported by a National Institutes of Health grant ( ZO1-HL005701 to C.W.L.), Children's National Medical Center intramural funding (RAC) 2010-2011; and Matthew Swisher and Rachel Giese were generously supported by a fellowship from Howard Hughes Medical Institute .
PY - 2014/4
Y1 - 2014/4
N2 - Objective(s) 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. Methods 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. Results 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. Conclusions 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.
AB - Objective(s) 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. Methods 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. Results 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. Conclusions 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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U2 - 10.1016/j.jtcvs.2013.06.018
DO - 10.1016/j.jtcvs.2013.06.018
M3 - Article
C2 - 23886032
AN - SCOPUS:84896543009
SN - 0022-5223
VL - 147
SP - 1291-1298.E2
JO - Journal of Thoracic and Cardiovascular Surgery
JF - Journal of Thoracic and Cardiovascular Surgery
IS - 4
ER -