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

Keywords: congenital heart defects, cleft deformities, cleft lip, cleft palate, cleft lip with or without cleft palate

Abstract

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.

Conclusion: 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.

Author Biographies

W J Barrett, University of the Free State

MBChB, MMed (Anes), DA (SA), FCA (SA), Department of Anaesthesiology, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa

B J S Diedericks, University of the Free State

MBChB, MMed (Anes), FCA (SA),  Department of Anaesthesiology, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa

C L Barrett, University of the Free State

MBChB, MMed (Int Med), PG Dip (Transf Med), FCP (SA), Department of Internal Medicine, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa

G Joubert, University of the Free State

BA, MSc, Department of Biostatistics, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa

Edwin W Turton, University of the Free State

MMed (Anes), FCA(SA), Department of Anaesthesiology, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa

Published
2019-03-14
Section
Research Articles