Background: The reports on prevalence rates of congenital heart diseases (CHDs) in patients with orofacial clefts (OFCs)
have varied widely.
Objective: To systematically review the prevalence rates of CHDs in patients with OFCs.
Material and Method: A computer search was conducted through the PubMed from 1950 to June 2015 using key words or
search terms of congenital heart diseases, orofacial clefts, cleft lip/palate and prevalence.
Results: The search resulted in nine studies with 598 CHDs cases identified in 5,707 patients with OFCs. The prevalence of
CHDs in patients with OFCs ranged from 3.9% to 23.9%. The five prospective studies had prevalence rates of 12.0% (95%
confidence interval [CI]: 10.9 to 13.2) whilst the four retrospective studies had prevalence rates of 8.6% (95% CI: 7.5 to 9.8). Concerning the prospective studies, the newborn study had a higher prevalence than those of other childhood studies [23.9% vs. 11.5% (95% CI: 10.4 to 12.7)]. The newborn study with the use of echocardiography had a higher prevalence than those without using echocardiography (23.9% vs. 12.8%). Atrial septal defect was the most frequent CHD found.
Conclusion: CHD is commonly found in a patient with OFC. Echocardiography should be used to assess CHD in patients
with OFCs.

Keywords: Congenital heart diseases, Orofacial clefts, Cleft lip, Cleft lip and palate, Cleft palate, Prevalence, Incidence