The Outcome of Congenital Cardiac Surgery in Patients with Down Syndrome: Single-Center Experience.

OBJECTIVES: This study aimed to describe the outcomes of Down syndrome patients who underwent cardiac surgery for congenital heart defects and to develop risk prediction models for in-hospital mortality, recurrent hospital admission, and the need for catheter intervention among a cohort of patients. METHODS: This single-centre retrospective cohort study included consecutive Down syndrome patients who underwent cardiac surgery for congenital heart defects between January 2018 and December 2021. We reviewed the electronic medical records. Two hundred patients fulfilled the eligibility criteria with complete data reporting. The patients' perioperative data and outcomes were recorded. RESULTS: Females constituted 56.5%. Most (78.5%) patients showed accepted recovery. The incidence of all-cause in-hospital mortality was 3.0%. The rates of the need for a second operation, heart failure management, and permanent pacemaker insertion were 3.0%, 2.0%, and 2.5%, respectively. Only 8 (4.0%) patients stayed in the hospital for a long duration after chylothorax or tracheostomy (if intubated more than 2 weeks). The model had an accuracy of 99% and included the intraoperative transesophageal echocardiography (TEE) abnormalities (residual heart lesions) (adjusted odds ratio [AOR]: 26.541, p = 0.033), the duration of mechanical ventilation following the operation (AOR: 1.152, p = 0.009), and the occurrence of postoperative heart block (AOR: 76.447, p = 0.005). CONCLUSION: Surgical treatment of congenital heart defects in Down syndrome patients had good outcomes with accepted recovery (without intra-hospital or during follow-up mortality or morbidity) of 78.5% and a 3% incidence of in-hospital mortality. Though, the occurrence of chylothorax was considerably high, and resulted in a long hospital stay (more than 10 days). Repair of tetralogy of Fallot and coarctation of the aorta were associated with an increased likelihood of catheter intervention following the operation.

Predictors of major adverse events and complications after ventricular septal defects surgical closure in children less than 10 kg.

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.

The efficacy and safety of percutaneous balloon angioplasty for aortic coarctation in children. Acute and mid-term results in a single center experience.

OBJECTIVES: To assess the efficacy and safety of balloon angioplasty (BAP) procedure for treatment of coarctation of the aorta (CoA) in children. Methods: A retrospective study included 27 consecutive children, underwent BAP for either native-CoA (Na-CoA) or recoarctation (Re-CoA). Medical records, echocardiographic findings, angiographic and hemodynamic data were collected from the hospital database. Follow­up was scheduled at 1, 3, 6, 12 months after the procedure. The study took place over a period of 4.5 years, from April 2014 to January 2019, in Madinah Cardiac Center, Madinah, Northwest region, Saudi Arabia. RESULTS: The mean age of patients was 11.86±8.96 months. Seven children had Na-CoA and 20 children had Re-CoA. The success rate of the procedure was achieved in 23 children (85%), as BAP reduced the mean systolic pressure gradient across the CoA (Na-CoA: from 45.28± 18.3 to 9.8± 6.57 mm Hg, p=0.0009), and in Re-CoA groups (from 42.48±16.7 to 10.9±8.5 mm Hg, p less than 0.0001). In mid-term follow-up, the need for re-intervention occurred in 8 children of the cohort (3 children [42.8%] from the Na-CoA group, and 5 children [25%] from the Re-CoA group).  Conclusions: Balloon angioplasty is considered a safe procedure for the management of CoA, but its efficacy remains questionable especially for young infants with Na-CoA type. However, it is a reliable option for managing Re-CoA children, with a lower rate of future re-intervention.

Transcatheter device closure of ventricular septal defects in children: a retrospective study at a single cardiac center.

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.

The trends and patterns of congenital heart diseases at Madinah Cardiac Center, Madinah, Saudi Arabia.

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  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.

Risk factors for prolonged mechanical ventilation after surgical repair of congenital heart disease. Experience from a single cardiac center.

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 greater than 72 hours of ventilation. Results: A total of 257 patients were included, among whom 219 (85.2%) were intubated for greater than 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.

The spectrum of congenital heart diseases in down syndrome. A retrospective study from Northwest Saudi Arabia.

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.

Pediatric heart diseases in Madina, Saudi Arabia. Current status and future expectations.

OBJECTIVE: To demonstrate the magnitude of pediatric heart disease problem in Madina, current situation of heart service, obstacles and future expectations. METHODS: We conducted this cross sectional study in Madina Maternity and Children Hospital, Madina, Kingdom of Saudi Arabia from January 2007 to June 2008. The study was approved by the Ethical Committee. A computer based program was established to register the data of all children attended the pediatric cardiology unit. RESULTS: A total of 4348 children were seen in the study period; 2301 were follow up cases while 2047 were new cases. Of the new cases, 794 (38.8%) were found to have heart diseases. Of this, 705 have CHD (cardiomyopathies 34, rheumatic heart disease 26, mitral valve prolapse 15, Kawasaki disease 9, and arrhythmia 5). Ventricular septal defect was the most common CHD (34.5%), and followed by atrial septal defect (8.9%). Other CHDs were as follows pulmonary stenosis (7.9%), patent ductus arteriosus (6%), atrioventricular septal defect (3.8%), aortic stenosis (3.5%), dextro-transposition of great arteries (3.5%), tetralogy of fallot (3%), coarctation of aorta (2.8%), and others (26%). Three hundred and ten patients required referral to the cardiac centers in Riyadh for catheterization or surgical intervention. Urgent referral was required in 128 patients. All patients were transferred via medical evacuation service. CONCLUSION: Pediatric heart disease constitutes a major health problem in Madina. The majority of patients have CHD. The need for cardiac centre in Madina was emphasized to avoid problems of transportations.

Splenectomized versus non-splenectomized patients with thalassemia major. Echocardiographic comparison.

OBJECTIVE: To study the effect of splenectomy in patients with thalassemia major on the cardiovascular system through echocardiographic study. METHODS: A prospective, cross sectional study was carried out from December 2006 to December 2007. Patients from the Thalassemia Center in the Maternity and Children's Hospital, Madina, Kingdom of Saudi Arabia, were screened by means of history, physical examination, laboratory studies, and echocardiography. RESULTS: Fifty-seven patients were studied: 36 were non-splenectomized, while 21 were surgically splenectomized. The 2 study groups were well matched for age, gender, height, and weight. The total amount of blood given during the previous year (6577.1+/-206.9 ml versus 5390.5+/-220.2 ml, p=0.0005), and the annual transfusion index (200.9+/-11.3 versus 134.1+/-7.3, p=0.0001) were significantly lower in the splenectomized group. There was no significant difference between the 2 groups regarding laboratory studies. Left ventricular systolic function shows no difference regarding fraction shortening between the 2 groups. The mitral valve E/A ratio was significantly higher in the splenectomized group (1.6+/-0.2 versus 1.4+/-0.2, p=0.02). The pulmonary artery pressure was higher in the splenectomized group (34.2+/-9.1 versus 20.8+/-9.2 mm Hg, p=0.0001). There was a significantly higher number of patients with pulmonary hypertension in the splenectomized group (14 [66.7%] versus 6 [16.7%], p=0.0004). CONCLUSION: Splenectomized patients with thalassemia major are at high risk of having impaired diastolic left ventricular function and pulmonary hypertension.