Congenital heart defects in children with cleft lips and/or palates at an academic hospital in central South Africa

Background: Globally, cleft deformities are the most common craniofacial anomalies and show an association with congenital heart defects. Little research on cleft lips and/or palates (CL/P) and congenital heart defects has been reported from Africa, and none from South Africa. In 2001, it was proposed that CL/P be listed as one of six priority conditions for monitoring and notification to South African health authorities. This goal of creating a national registry has not been achieved. A near-fatal anaesthetic incident following a missed cardiac lesion in a child with a cleft lip and palate prompted this study. Objectives: To describe the prevalence of congenital heart defects diagnosed in children with CL/P presenting for corrective surgery during the Smile Week over three consecutive years (2013-2015) at an academic hospital in South Africa. Methods: A retrospective, descriptive file review of 62 patients with CL/P was performed. Since 2013, echocardiography has been performed on all patients with CL/P. Results: Twenty-three, 21 and 18 patients were operated in 2013, 2014 and 2015, respectively. Of these patients, 85.5% (n = 53) had no clinical evidence of a cardiac defect, of which eight did have clinically significant cardiac defects on echocardiography. Sixteen patients (25.8%) in total (n = 16/62) with a cleft deformity had a clinically significant congenital heart lesion. Of the 16 patients with a cardiac defect on transthoracic echocardiography, only four had clinical evidence of cardiac defect. Therefore, sensitivity of clinical examination was 25%, whereas the specificity was 89.1%. Three of the four patients with a syndrome had a clinically significant echocardiographic finding. uConclusion: A national guideline for the preoperative care of patients with CL/P, including routine echocardiography, is needed. Furthermore, a national registry is required for patients with CL/P with associated congenital anomalies.