Naikhoba C O Munabi1, Jordan Swanson, Allyn Auslander, Pedro A Sanchez-Lara, Sally L Davidson Ward, William P Magee. 1. From the *Division of Plastic and Reconstructive Surgery, Keck School of Medicine of the University of Southern California, †Division of Plastic and Reconstructive Surgery, Children's Hospital Los Angeles; ‡Department of Pathology & Laboratory Medicine and Pediatrics, Keck School of Medicine of the University of Southern California; §Center for Personalized Medicine, and ∥Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, Los Angeles, CA.
Abstract
BACKGROUND: Life-threatening anomalies, such as congenital heart disease (CHD) must be identified in patients with cleft lip and/or palate (CL/P) to minimize perioperative risk. Nevertheless, screening practices vary highly among cleft teams and programs, and little is known about the prevalence and clinical significance of CHD in nonsyndromic CL/P patients. Through a systematic literature review, this study examines the demographics and severity of CHD in the nonsyndromic CL/P population. The implications of concomitant CHD in providing safe and comprehensive cleft care both in the United States and abroad are discussed. METHODS: A systematic review of PubMed literature from 1980 to September 2015 was performed following PRISMA guidelines. Studies describing rates of CHD and severity of lesions specifically in nonsyndromic and all CL/P patients were included. Analysis of cumulative data was performed according to nonsyndromic status and cleft type. RESULTS: Twelve studies were found to meet inclusion criteria. Of the 4055 nonsyndromic CL/P patients who met inclusion criteria, 7.42% (n=301) had CHD, which was significantly greater than the general population (~1%; odds ratio [OR], 7.94; P<0.0001). Congenital heart disease was significantly more common in cleft palate (CP) (OR, 15.1), combined CL and palate (CL+P) (OR, 13.5), and CL (OR, 4.23) compared with the general population. Palatal clefts (CP and CL+P) had significantly increased odds of CHD compared with CL (OR, 3.58 and 3.19, respectively, both P<0.0001). The most common forms of CHD were atrial or ventricular septal defects (n=210, 74.2%), which typically do not require surgical intervention in the general population. Clinical significance of these CHD lesions in CL/P patients is not fully known. CONCLUSIONS: Cleft management programs aim to maximize the number of patients receiving care while maintaining patient safety. Appropriate evaluation of perioperative risk necessitates understanding the prevalence of CHD in CL/P patients and the severity of those lesions. Patients with CL/P, particularly patients with palatal clefts, have significantly higher odds of having CHD than the general population. Congenital heart disease is most likely to present as atrial or ventricular septal defects, which are lesions that are unlikely to impact safety during cleft repair surgery.
BACKGROUND: Life-threatening anomalies, such as congenital heart disease (CHD) must be identified in patients with cleft lip and/or palate (CL/P) to minimize perioperative risk. Nevertheless, screening practices vary highly among cleft teams and programs, and little is known about the prevalence and clinical significance of CHD in nonsyndromic CL/Ppatients. Through a systematic literature review, this study examines the demographics and severity of CHD in the nonsyndromic CL/P population. The implications of concomitant CHD in providing safe and comprehensive cleft care both in the United States and abroad are discussed. METHODS: A systematic review of PubMed literature from 1980 to September 2015 was performed following PRISMA guidelines. Studies describing rates of CHD and severity of lesions specifically in nonsyndromic and all CL/Ppatients were included. Analysis of cumulative data was performed according to nonsyndromic status and cleft type. RESULTS: Twelve studies were found to meet inclusion criteria. Of the 4055 nonsyndromic CL/Ppatients who met inclusion criteria, 7.42% (n=301) had CHD, which was significantly greater than the general population (~1%; odds ratio [OR], 7.94; P<0.0001). Congenital heart disease was significantly more common in cleft palate (CP) (OR, 15.1), combined CL and palate (CL+P) (OR, 13.5), and CL (OR, 4.23) compared with the general population. Palatal clefts (CP and CL+P) had significantly increased odds of CHD compared with CL (OR, 3.58 and 3.19, respectively, both P<0.0001). The most common forms of CHD were atrial or ventricular septal defects (n=210, 74.2%), which typically do not require surgical intervention in the general population. Clinical significance of these CHD lesions in CL/Ppatients is not fully known. CONCLUSIONS:Cleft management programs aim to maximize the number of patients receiving care while maintaining patient safety. Appropriate evaluation of perioperative risk necessitates understanding the prevalence of CHD in CL/Ppatients and the severity of those lesions. Patients with CL/P, particularly patients with palatal clefts, have significantly higher odds of having CHD than the general population. Congenital heart disease is most likely to present as atrial or ventricular septal defects, which are lesions that are unlikely to impact safety during cleft repair surgery.
Authors: Naikhoba C O Munabi; Shady Mikhail; Omar Toubat; Michelle Webb; Allyn Auslander; Pedro A Sanchez-Lara; Zarko Manojlovic; Ryan J Schmidt; David Craig; William P Magee; Subramanyan Ram Kumar Journal: Am J Med Genet A Date: 2022-04-06 Impact factor: 2.578
Authors: Omar Toubat; Demetrios N Mallios; Naikhoba C O Munabi; William P Magee; Vaughn A Starnes; S Ram Kumar Journal: World J Pediatr Congenit Heart Surg Date: 2021-01
Authors: Olufemi A Erinoso; Olutayo James; Ogochukwu J Sokunbi; Olawale O Adamson; Adeola A Adekunle; Olusola F Agbogidi; Ajoke O Ogunlewe; Ekanem N Ekure; Wasiu L Adeyemo; Akinola L Ladeinde; Olugbemiga M Ogunlewe Journal: Afr J Paediatr Surg Date: 2021 Oct-Dec