Abstract

Introduction: Congenital heart disease (CHD) patients with heterotaxy (HTX) have high prevalence of airway ciliary dysfunction (CD). This is associated with higher postoperative morbidity and more respiratory complications. As a high prevalence of airway CD is also observed in CHD patients without HTX, we hypothesize patients with broad spectrum of CHD may have increased postoperative morbidity associated with airway CD. Methods: CHD patients <1 yr undergoing cardiac surgery were recruited. CD testing was conducted with two tests used for primary ciliary dyskinesia (PCD) diagnosis: nasal nitric oxide (nNO) was measured, typically low with PCD, and nasal scrape was obtained for ciliary motion (CM) analysis with videomicroscopy. Data on post-operative outcome parameters were collected. Results: We recruited 55 CHD patients with 76 surgical events; 38% had airway CD (low nNO/ abnormal CM). Demographics and surgical complexity scores were similar between patients with or without CD. In CHD patients with CD, there was significant increase in continuous positive airway pressure ventilation (CPAP) (p = 0.008) and trend for more reintubation (11% non-CD vs. 27% CD patients) (Table 1). There was significant increase in beta-agonist use in CD patients (p = 0.006) and five-fold increase in respiratory medication use (Table 1). No difference was observed in length of stay, ventilator days, overall supportive ventilation (CPAP, high flow nasal cannula, nasal cannula), use of pulmonary vasodilators, chest tube days, effusion, or need for extracorporeal membrane oxygenation (ECMO). Conclusions: CHD patients with airway CD have increased postoperative morbidity, including increased need for CPAP, more use of albuterol and respiratory medications, and more reintubations. These results suggest CHD patients may benefit from presurgical screening for airway CD, with those identified with CD given perioperative respiratory therapy to reduce postoperative morbidity.

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