Clinical Importance of Concomitant Cleft Lip/Palate in the Surgical Management of Patients With Congenital Heart Disease.

Background: Congenital heart disease (CHD) frequently occurs in conjunction with extracardiac developmental anomalies, including cleft malformations. The clinical impact of concomitant cleft disease on the surgical management of CHD has not been studied. We evaluated cardiac surgical outcomes in patients with concomitant CHD and cleft lip and/or palate (CL/P).

Methods: Patients with CHD + CL/P managed at our institution between January 2004 and December 2018 were included. Demographic, operative, and follow-up data were retrospectively collected and analyzed using SAS 9.4. Chi-square tests were used for categorical variables and test or Wilcoxon rank sum tests for continuous variables. Significance of < .05 was used.

Results: There were 127 patients with CHD + CL/P; 63 (50%) were boys. Compared to the general CHD population, patients with CHD + CL/P demonstrated an enrichment of atrial septal defects (10.5% vs 34%), tetralogy of Fallot/double outlet right ventricle (6.4% vs 15.7%), arch defects (4.5% vs 10.2%), truncus arteriosus (1.2% vs 3.1%), and total anomalous pulmonary venous return (1.0% vs 2.4%). Of 63 patients who underwent CHD repair, 58 (92%) did so prior to CL/P repair at 21.5 (6-114) days of age. Compared to CHD lesion-matched patients undergoing cardiac surgical repair at our institution, patients with CL/P had a 2- to 3.7-fold longer intensive care stay, 1.8- to 2.6-fold longer hospital stay, and 6- to 13.5-fold increase in major morbidity, without a significant difference in mortality.

Conclusions: Cardiac outflow tract defects are particularly overrepresented in CL/P patients. The presence of CL/P increases the complexity of postoperative care after CHD surgery, without a significant impact on mortality.