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Congenital Heart Diseases, Associated Malformations, and Syndromes in Thai Children with Orofacial Clefts: A 10-Year
Krispijyakorn Sangharn1, Poonsak Pisek1,2, Araya Pisek3
1Division of Orthodontics, Department of Preventive Dentistry, Faculty of Dentistry, Khon Kaen University, Khon Kaen, Thailand.
Objective:
The study aimed to determine the distribution of congenital heart diseases (CHDs), associated congenital malformations, and syndromes among children with orofacial clefts (OFCs) at Tawanchai Cleft Center, Khon Kaen, Thailand from 2011 to 2020.
Methods:
The retrospective study reviewed medical records of 1187 children with OFCs at a tertiary cleft center from 2011 to 2020. A total of 95 eligible cases met the following inclusion criteria: diagnosis of cleft palate (CP), cleft lip (CL), or cleft lip and palate (CLP); age from birth to 3 years; confirmed CHD diagnosis by a cardiologist; confirmed diagnosis of congenital malformations or syndromes by a geneticist; and complete medical records. Distributions were described using frequencies and percentages. Fisher's exact test and binary logistic regression (unadjusted and sex-adjusted) were used for inferential analysis.
Results:
Among 95 patients, CP was the most frequent OFC type (48.4%), followed by CLP (44.2%) and CL (7.4%). The sex distribution was 48.4% male and 51.6% female, and all patients were of Thai ethnicity. The observed proportion of CHDs (8.0%) fell in the mid-range of prior studies. The most common CHDs were atrial septal defect (ASD, 25.3%), patent ductus arteriosus (PDA, 20.0%), and ventricular septal defect (VSD, 18.9%). Pierre Robin sequence (PRS) was the most common associated congenital malformation (10.4%). Syndromic clefts occurred in 38 cases (40.0%), predominantly involving 22q11.2 deletion syndrome (18.4%), vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities association (13.1%), and Down syndrome (7.9%). Binary logistic regression revealed significantly lower odds of VSD in patients with unilateral CLP compared to those with CL (sex-adjusted odds ratio = 0.07, 95% confidence interval = 0.01-0.61, p = 0.02).
Conclusion:
ASD, PDA, and VSD are the most common CHDs among patients with OFCs. These CHDs frequently co-occur with PRS and 22q11.2 deletion syndrome. These findings underscore the importance of comprehensive cardiac screening in children with OFCs to support interdisciplinary cleft care management.
