Objectives:To to define the frequency and patterns of congenital heart disease (CHD) among children with Down syndrome (DS) in Northwest Saudi Arabia.Methods:We included children with confirmed DS referred to the regional pediatric cardiology unit in Madinah Maternity and Children Hospital between January 2008 and December 2013. Children were identified from the unit’s data-base and the charts were reviewed retrospectively. We excluded term and preterm children with patent ducts arteriosus (PDA) and persistent foramen oval spontaneously resolved during the first 4 weeks of life.Results:A total of 302 children with DS were identified (50.3% male). Of these, 177 (58.6%) had CHD. Atrioventricular septal defect (AVSD) was the most frequent lesion identified in 72/177 (40.7%) followed by mixed left to right shunt defects (14.7%) and secundum atrial septal defect (ASD) (11.8%). Ventricular septal defect was detected in 10.7% and 8.5% had PDA beyond the neonatal period. There was no gender difference in the frequency of CHD (p=0.9) and the presence of CHD was not related to the genetic cause of DS (p=0.9).Conclusion:The frequency of CHD in our DS cohort is comparable with Europe, Asia, and other KSA regions. However its pattern appears to be different from some areas in KSA.

Objectives:To investigate the association between angiotensin-converting enzyme (ACE) insertion/deletion (I/D) polymorphism and rheumatic heart disease (RHD) in Saudi patients.Methods:A case-control study was conducted in Saudi RHD patients. Genomic DNA was isolated from 99 RHD patients attending the Pediatric Cardiology Clinic at the Maternity and Children Hospital, Al-Madinah, Saudi Arabia from March 2013 to June 2014, and from 145 age- and gender-matched controls. Patient clinical records were reviewed to report major and minor modified Jones’ criteria for diagnosis. The diagnosis was confirmed by echocardiography. The ACE I/D polymorphism was identified by polymerase chain reaction.Results:A significant difference in ACE D allele carriage (DD+ID) distribution between RHD cases and controls was identified (p=0.02, odds ratio = 3.6, 95% confidence interval: 1.2-10.8). The D allele carriage was significantly associated with development of mitral valve lesions alone (p=0.03).Conclusion:The ACE I/D polymorphism is associated with an increased risk of RHD in the Saudi population. Further studies are needed to confirm our findings and to understand the molecular mechanisms underlying this association.

Objectives: We studied these predictors at a single cardiac center. Methods: A retrospective cohort study was carried out after obtaining approval from the institutional review board. All patients (age, 0-14 years) who underwent congenital heart disease (CHD) surgery from January 2014 to June 2016 were included. Prolonged mechanical ventilation (PMV) was defined as >72 hours of ventilation. Results: A total of 257 patients were included, among whom 219 (85.2%) were intubated for >72 hours and 38 (14.8%) were intubated for ≥72 hours. Age (29.9 versus 11.95 years), weight (9.6 versus 5.9 kg), cross-clamp time (CCT) (53.6 versus 71.8 min), cardiopulmonary bypass time (CBP) (80.98 versus 124.36 min), length of stay in the pediatric intensive care unit (PICU) (10.4 versus 27.2 days), infection (12.8% versus 42.1%), open sternum (0.9% versus 13.2%), re-intubation (19.2% versus 39.5%), pulmonary hypertension (10.9% versus 31.6%), and impaired heart function (10.1% versus 23.7%) were associated with PMV. In terms of Risk Adjustment in Congenital Heart Surgery (RACHS) classification, only patients with RACHS 4 (18.4%) were associated with the risk for PMV. Conclusions: Age, weight, CBP, CCT, pulmonary hypertension, impaired cardiac function, and sepsis are risk factors for PMV. These factors should be considered when deciding surgery and in providing PICU care.

BACKGROUND: Ventricular septal defect (VSD) is the most common congenital heart disease in the pediatric population. Nowadays, trans-catheter closure is considered a feasible method of therapy for most muscular and some perimembranous types of VSDs. OBJECTIVE: Assess the safety, efficacy and outcome of percutaneous transcatheter closure of VSDs in children. DESIGN: Retrospective, single center study. SETTING: Madinah Cardiac Center, Madinah, Saudi Arabia. PATIENTS AND METHODS: The study included all consecutive children who underwent transcatheter closure of isolated VSD during the period from December 2014 to January 2019. The data were collected from hospital database medical records. Transthoracic echocardiography (TTE) and an electrocardiogram (ECG) were done before and after the procedure in all the patients. The device was implanted by the retrograde or antegrade approach. All patients were subjected to follow-up evaluation at 1, 3, 6, 12 months, and annually thereafter with TTE and ECG. MAIN OUTCOME MEASURES: Procedure success rate, clinical follow-up, TTE. SAMPLE SIZE: 70 children. RESULTS: The mean (standard deviation) age of patients was 10.2 (4.1) years (range: 2-18 years), and their mean body weight was 30.9 (13.9) kg (range: 7.0-57.7 kg). Forty-eight (68.6%) children had muscular VSD (mVSD), and 22 (31.4%) children had perimembranous VSD (pmVSD). The majority of defects were closed via the retrograde approach using the Amplatzer muscular occluder device. At 24 hours after the procedure, the success rate was 90%. Only four (5.7%) cases had major adverse events including complete atrioventricular block, hemolysis, and thrombus formation. CONCLUSION: Transcatheter closure is a safe and feasible procedure in VSDs of various morphologies, with a low adverse event rate. LIMITATIONS: Retrospective design, single-center study, absence of control group. CONFLICT OF INTEREST: None.

Objectives: To characterize the pattern of congenital heart diseases (CHDs) in Madinah, Saudi Arabia. Methods: We retrospectively collected and analyzed the demographic and diagnostic details of all patients with CHDs referred at Madinah Cardiac Center (MCC) over a period of 3 years from January 2017 to December 2019. Results: During the study period, 1,127 patients with CHDs were identified. The male to female ratio was 1.1:1, with a mean age of 8.4±2.4 years. The acyanotic CHDs were the predominant lesions, accounting for 84.8% of all cases, while the cyanotic types accounted for 13%. Patent ductus arteriosus (PDA), ventricular Original Article septal defects (VSD), atrial septal defects (ASD), coarctation of the aorta (CoA), and atrioventricular septal defect (AVSD) were the most common acyanotic CHDs and represented 27.9%, 24.8%, 18.9%, 6.4%, and 4.4% of the total cases, respectively. Tetralogy of Fallot (ToF) (8.7%), followed by transposition of the great arteries (TGA) (1.7%) and truncus arteriosus (1.1%), were the most common cyanotic CHDs. There was a male predominance of VSD, ToF, CoA, TGA, and truncus arteriosus. In contrast, PDA, ASD, and AVSD were more common in females. Conclusion: The pattern of CHDs observed in our study and age at which the diagnosis of CHDs was made were different from the other national and international studies, which points to a diagnostic issue along with problems of awareness on the part of the general population.

Introduction Early intervention in patients with congenitally disconnected pulmonary artery improves long‐term outcome. Case presentation We present 3 cases of isolated disconnected pulmonary artery in the absence of associated structural heart disease during a period of 3 years. Conclusion Transcatheter stenting of the feeding ductus arteriosus re‐established pulmonary artery flow and growth. Successful surgical repair was achieved, and normal perfusion and complete function of the ipsilateral lung were regained.

Background Ventricular septal defect (VSD) is the most common congenital cardiac defect for which outcomes are not uniform. There is a lack of consensus on the risk factors for the unfavorable outcomes following surgical VSD closure. Aim The aim of this study was to determine the risk factors and the predictors of major adverse events (MAEs) and complications following surgical closure of VSD in children weighing less than 10 kg. Methods This retrospective cohort study included children less than 10 kg who underwent surgical closure of congenital VSD of any type with or without associated congenital heart diseases. Patients with associated major cardiac anomalies were excluded. Preoperative, operative and postoperative data were collected from medical records. Results This study included 127 patients 52.8% were males, the median age was 8.0 months (IQR = 6.0–11.0 months), and their median weight was 5.7 kg (IQR = 4.8–7.0). Mortality was in one patient (0.8%) Multivariable logistic regression analysis revealed that male sex group (observational data), previous pulmonary artery banding (PAB), and significant intraoperative residual VSD were significant risk factors for the development of MAEs (odds ratios were 3.398, 14.282, and 8.634, respectively). Trisomy 21 syndrome (odds ratio: 5.678) contributed significantly to prolonged ventilation. Pulmonary artery banding (odds ratio: 14.415), significant intraoperative (3 mm) residual VSD (odds ratio: 11.262), and long cross-clamp time (odds ratio: 1.064) were significant predictors of prolonged ICU stay, whereas prolonged hospital stay was observed significantly in male sex group (odds ratio: 12.8281), PAB (odds ratio: 2.669), and significant intraoperative (3 mm) residual VSD (odds ratio: 19.551). Conclusions Surgical VSD repair is considered a safe procedure with very low mortality. Trisomy 21 was a significant risk factor for prolonged ventilation. Further, PAB, significant intraoperative residual of 3 mm or more that required a second pulmonary bypass, and a greater cross-clamp time were significant predictors of MAE and associated complications with prolonged ICU and hospital stay.

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