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Congenital Heart Anomalies in Patients with Clefts of the Lip and/or Palate

Journal of the Korean Pediatric Society 2000;43(4):520-525.
Published online April 15, 2000.
Congenital Heart Anomalies in Patients with Clefts of the Lip and/or Palate
Jin Ho Yu1, Jeong Jin Yu1, June Huh2, Chung Il Noh1, Jung Yun Choi1, Yong Soo Yu1
1Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
2Department of Pediatrics, College of Medicine, Hallym university, Chuncheon, Korea
구순열과 구개열에서의 선천성 심기형
유진호1, 유정진1, 허준2, 노정일1, 최정연1, 윤용수1
1서울대학교 의과대학 소아과학교실
2한림대학교 의과대학 소아과학교실
Abstract
Purpose
: The prevalence of congenital heart anomalies is known to be higher in patients with clefts of the lip and/or palate(CL/P). The purpose of this study was to determine the prevalence and type of congenital heart anomalies in patients with CL/P.
Methods
: We investigated congenital heart anomalies in 756 patients presented with CL/P from January 1986 to December 1997 by reviewing their clinical records.
Results
: The prevalence rate of congenital heart anomalies in patients with CL/P was 4.2%(32 of 756). Congenital heart anomalies in those were ventricular septal defect(15 of 32), atrial septal defect(4 of 32), tetralogy of Fallot(3 of 32), patent ductus arteriosus(2 of 32), double outlet right ventricle(2 of 32), pulmonary stenosis(1 of 32), transposition of the great arteries(1 of 32), pulmonary atresia(1 of 32), coarctation of aorta(1 of 32), anomalous systemic venous drainage(1 of 32), and aortic aneurysm with patent ductus arteriosus(1 of 32). It was significant that the prevalence rate of congenital heart anomalies in cleft palate with or without cleft lip(CP+/-L) was 6.8%(30 of 442), because the prevalence rate of congenital heart anomalies in cleft lip alone was not higher than in normal population(0.6%; 2 of 314). Of the 30 patients with congenital heart anomalies, 12 patients(40 %) had conotruncal defects.
Conclusion
: The prevalence of congenital heart anomalies in patients with CP+/-L was much higher than normal population. Cardiac defects were predominantly conotruncal. Predominance of conotruncal defects among congenital heart anomalies in those was associated with abnormalities of neural crest cell proliferation and migration developing into conotruncus and palate.
Key Words: Congenital heart anomaly, Conotruncal defect, Cleft lip, Cleft palate


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