Beina Azadgoli1, Naikhoba C O Munabi1, Artur Fahradyan2, Allyn Auslander2, Meghan McCullough1, Nikki Aflatooni3, Sally L Davidson Ward4, S Ram Kumar5,6, Pedro A Sanchez-Lara7, Jordan Swanson2,8, William P Magee2. 1. Division of Plastic and Reconstructive Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA. 2. Division of Plastic and Maxillofacial Surgery, Children's Hospital Los Angeles, CA, USA. 3. University of Southern California, Los Angeles, CA, USA. 4. Division of Pediatric Pulmonology, Children's Hospital Los Angeles, CA, USA. 5. Heart Institute, Children's Hospital Los Angeles, CA, USA. 6. Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA. 7. Division of Pediatric Genetics, Children's Hospital Los Angeles, CA, USA. 8. Division of Research and Outcomes, Operation Smile International, Virginia Beach, VA, USA.
Abstract
OBJECTIVE: To evaluate characteristics of congenital heart disease (CHD) in patients with cleft lip and/or palate (CL/P) and assess potential associations with cleft outcomes. DESIGN: Retrospective review of all patients with CL/P who underwent primary cleft treatment from 2009 to 2015. SETTING: Children's Hospital Los Angeles, a tertiary hospital. PATIENTS: Exclusion criteria included microform cleft lip diagnosis, international patients, and patients presenting for secondary repair or revision after primary repair at another institution. MAIN OUTCOMES MEASURED: Patient demographics, prenatal and birth characteristics, CL/P characteristics, syndromic status, postoperative complications, and other outcomes were analyzed relative to CHD diagnoses and management. Patients with CL/P with (+CHD) were compared to those without (-CHD) CHD using χ2 tests and analysis of variance. RESULTS: Among 575 patients with CL/P, 83 (14.4%) had CHD. Congenital heart disease rates were significantly higher in patients with cleft palate (CP) compared to other cleft types (χ2, P = .009). Eighty-one (97.6%) out of 83 +CHD patients were diagnosed prior to initial CL/P surgical assessment. Twenty-three (27.7%) +CHD patients required surgical repair of 10 cardiac anomalies prior to cleft care. Congenital heart disease was associated with delayed CP repair and increased rates of fistula in isolated patients with CP. CONCLUSIONS: Congenital heart disease is known to be more prevalent in patients with CL/P. These data suggest the condition is particularly increased in patients with CP. Severe forms of CHD are diagnosed and treated prior to cleft care however postoperative fistula may be more common in patients with CHD. Therefore, careful attention is required for patient optimization and palatal flap dissection in patients with coexisting CHD and CL/P.
OBJECTIVE: To evaluate characteristics of congenital heart disease (CHD) in patients with cleft lip and/or palate (CL/P) and assess potential associations with cleft outcomes. DESIGN: Retrospective review of all patients with CL/P who underwent primary cleft treatment from 2009 to 2015. SETTING:Children's Hospital Los Angeles, a tertiary hospital. PATIENTS: Exclusion criteria included microform cleft lip diagnosis, international patients, and patients presenting for secondary repair or revision after primary repair at another institution. MAIN OUTCOMES MEASURED: Patient demographics, prenatal and birth characteristics, CL/P characteristics, syndromic status, postoperative complications, and other outcomes were analyzed relative to CHD diagnoses and management. Patients with CL/P with (+CHD) were compared to those without (-CHD) CHD using χ2 tests and analysis of variance. RESULTS: Among 575 patients with CL/P, 83 (14.4%) had CHD. Congenital heart disease rates were significantly higher in patients with cleft palate (CP) compared to other cleft types (χ2, P = .009). Eighty-one (97.6%) out of 83 +CHD patients were diagnosed prior to initial CL/P surgical assessment. Twenty-three (27.7%) +CHD patients required surgical repair of 10 cardiac anomalies prior to cleft care. Congenital heart disease was associated with delayed CP repair and increased rates of fistula in isolated patients with CP. CONCLUSIONS:Congenital heart disease is known to be more prevalent in patients with CL/P. These data suggest the condition is particularly increased in patients with CP. Severe forms of CHD are diagnosed and treated prior to cleft care however postoperative fistula may be more common in patients with CHD. Therefore, careful attention is required for patient optimization and palatal flap dissection in patients with coexisting CHD and CL/P.
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