Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts.Methods: All infants with cleft lip and palate referred to the Children’s Medical Center and Bahramy; the teaching Hospitals of the Tehran University of Medical Sciences from 1991 to 2005 were prospectively enrolled in this study group. All patients were examined and noted by an academic cleft team contain; a pediatrician and a pediatric surgeon, and received cardiac consultation and echocardiography by a pediatric cardiologist. Non cardiac associated anomalies, still born and patients without echocardiography were excluded from the study. Data including age, gender, exposure to contagions and high risk elements, consanguinity and familial history of oral cleft, type of oral cleft, results of cardiac consultation and echocardiography and associated cardiac anomalies were cumulated and analyzed by SSPS version 13.5.Results: Among the 284 infants with oral clefts, 162 were male (57%) and 122 were female (43%). Seventy-nine patients (27.8%) had cleft lip, 84 (29.5%) had cleft palate and 121 (42.6%) had both cleft lip and palate. Of all the patients, 21.1% had congenital heart defects. The most common type of these congenital heart defects (28.3%) was atrial septal defect.Conclusions: For patients with cleft lip and palate, we recommend preoperative cardiac consultation, careful examination and routine echocardiography for associated cardiac anomalies, as well as appropriate management and prophylactic antibiotic therapy for those with associated congenital heart anomaly.

Background: Congenital heart defect (CHD) is one of the leading causes of neonatal death. Although the majority of CHDs are isolated, a significant number of them are associated with noncardiac anomalies. Esophageal Atresia (EA)/ Tracheoesophageal Fistula (TEF) is the most common congenital disorder of the upper GI tract. It is estimated that up to 70% of EA/TEF infants have other associated congenital anomalies such as CHD. This study determined the proportion of heart anomalies among the diseases of the upper GI tract in Imam Reza Hospital of Mashhad. Methods: The records of 38 infants with upper GI obstruction who were referred to the Pediatric Cardiology Clinic of Imam Reza Hospital in Mashhad between 2001 and 2017 were evaluated in this retrospective study. Data were coded and entered into SPSS software (version 16) and analyzed using Chi-square and T-test. Results: In this study, 38 babies with upper GI obstruction were evaluated (20 patients were female, 52. 6%), and the average birth weight was 2. 390 +-0. 870 gr. Among the parents, 13 patients (34. 2%) were relative (third-degree or more) and 25 patients (65. 8%) were nonrelative. The initial and final diagnosis was different at 14 pt (36. 8%) that was confirmed with echocardiographic findings. CHDs were divided into two groups in this study. Malformations such as PFO (patent foramen ovale) or FMV without MR (floppy mitral valve without mitral regurgitation) considered as non-important congenital heart diseases. Other malformations that require interventional or medical management such as VSD, ASD, TOF, or other CHDs are considered important CHDs. Nineteen pt (50%) had important CHD and 16 pt (42. 1%) had non-important CHD and 3 pt (7. 9%) had normal echocardiographic findings. Conclusion: The heart defect is the most common associated anomaly in children with EA/TEF, which is divided into two subgroups. The first important one is CHD, which is effective in gastric surgery and management, and VSD is the most common type. The other group is non important CHD such as PFO or FMV without MR that are not effective in their management. The patients with EA/TEF are at risk for low birth weight and preterm delivery.

Background: Various extracardiac disorders are associated with congenital heart defect (CHD) at varying prevalence rates (7%–50%). Over the years, numerous studies worldwide have investigated these associations. This study aimed to examine the prevalence of extracardiac anomalies in children with CHD in Isfahan, one of Iran’s largest cities. Methods: This cross-sectional study was conducted in Isfahan, Iran, from 2020 through 2022, involving 750 infants under 1 year old diagnosed with CHD. Pediatric cardiologists performed echocardiography to evaluate the cardiovascular system and detect CHD. Most participants were referred for cardiac examinations due to abnormalities detected during physical examinations of skin, cerebral, spinal cord, abdominal, and urinary tract regions. Patients exhibiting signs of a syndromic disorder were also referred for CHD evaluation. Results: Out of 750 infants with confirmed CHD, 241 (32.13%) presented at least 1 extracardiac malformation. Ninety (37.7%) had craniofacial malformations, with 66.7% having cleft palate with or without cleft lip. Forty-eight patients (19.9%) had genetic syndromes, most commonly Down syndrome (56.5%), and 46 (19.8%) had gastrointestinal abnormalities, including intestinal or esophageal atresia. Conclusions: The prevalence of extracardiac anomalies in patients with CHD is significant, and these patients are at an increased risk of mortality and morbidity throughout their lives. Implementing a screening program could effectively prevent further complications associated with the late diagnosis of these anomalies.

Introduction: Midline cleft of mandible, classified as Tessier 30 clefts is extremely rare, with less than 100 reported cases in the latest studies. Variations in severity and associated malformations have been reported before.Case Presentation: In this report, we present the first documented Iranian case of Tessier 30 with median cleft of lower lip and bifid tongue concomitant with congenital heart defects.Conclusions: We explain embryologic origin, differential diagnosis, other associated anomalies and its treatment by reviewing literature.

Background Dextrocardia is a malposition of the heart defined as the right-sided development of the heart. It can increase the likelihood of congenital heart defects or diseases (CHD) and the risk of related morbidities and mortalities. We aimed to determine the frequency of CHDs among Dextrocardia patients. Materials and Methods In a retrospective cross-sectional study the records of patients with Dextrocardia who referred to Imam Reza Hospital (tertiary referral center) of Mashhad between 2006 and 2016 were studied. Data were analyzed using SPSS software version 16. 0. Results In total 163 patients, 85 of whom (54%) were males were studied. Their mean age was 11. 41+ 0. 326 years. The frequency of major Dextrocardia subtypes were: Situs inversus in 77 (47%), Situs Solitus in 59 (36%), and Situs ambiguous in 28 (17%) patients. The frequency of associated complex congenital heart defects (CHD) was 55% in Situs inversus, 77% in Situs solitus and 100% in Situs ambiguous. Isolated associated CHD had a greater frequency in the Situs inversus and Situs solitus groups. The most common isolated associated CHD was septal defects. Conclusion More than 90% of all patients with Dextrocardia, had CHD whereas all patients with Dextrocardia and Situs ambiguous had complex CHD.