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: Hirschsprung's disease (HSCR) may be accompanied by other anomalies, including CONGENITAL heart disease (CHD), resulting in additional complications. This study was performed to evaluate the prevalence and type of concomitant CHD in hospitalized children with HSCR. Methods: All HSCR patients (n=129) admitted to Mofid Children's Hospital in Tehran, Iran, from April 2016 to August 2019 were investigated in a descriptive cross-sectional study. Two-dimensional, M-mode and pulsed, continuous, and color Doppler provided echocardiography were applied to evaluate cardiac structure and function. Results: CHD was observed in 48 (37. 2%) cases, and the most common anomalies were Atrial Septal Defect (ASD) in 20 (15. 5%), Ventricular Septal Defect (VSD) in 1 (0. 8%), Patent Ductus Arteriosus (PDA) in 2 (1. 6%), Tetralogy of Fallot in 3 (2. 3%), ASD and Pulmonary stenosis in 2 (1. 6%), ASD and PDA in 7 (5. 4%), ASD and VSD in 3 (2. 3%), as well as VSD and PDA in 2 (1. 6%) patients. Conclusion: Cardiac anomalies are relatively prevalent in the Iranian HSCR population participating in the present study. In addition, early echocardiographic evaluation in the setting of HSCR is recommended.

Background: CONGENITAL heart DEFECTS (CHDs) are the most common CONGENITAL malformations at birth. Substance abuse has increased dramatically over the past two decades. It also can affect neonates of drug-abusing mothers. Objectives: This study aimed to elucidate the possible association of maternal drug abuse with CHDs in their newborn infants. Patients and Methods: In this study, 72 neonates who were born during 6 years in three teaching hospitals are studied. Echocardiography was performed by a single pediatric cardiologist using two-dimensional and color Doppler echocardiography. The data were analyzed using descriptive statistics. Results: 1) Of 72 included cases, 38 (52. 78%) had abnormal echocardiographic findings; 2) from 38 abnormal echocardiography, 35 (48. 61% of total and 92. 11% of abnormal echoes) had mild CONGENITAL heart defect (CHD), and 3 had complex CHD; 3) There was no significant difference in the prevalence of neonatal CONGENITAL heart defect with the type of misused drugs (opiates or methamphetamines). Conclusions: In our study, the prevalence of CHD in newborns of drug abuser mothers was significantly higher than the normal population of infants. Hence, echocardiographic screening of these newborns seems to be logical.