Bidirectional glenn surgery without palliative pulmonary artery banding in univentricular heart with unrestricted pulmonary flow. Retrospective multicenter experience.

BACKGROUND: Although pulmonary artery banding (PAB) has been generally acknowledged as an initial palliative treatment for patients having single ventricle (SV) physiology and unrestrictive pulmonary blood flow (UPBF), it may result in unfavorable outcomes. Performing bidirectional Glenn (BDG) surgery without initial PAB in some selected cases may avoid the complications associated with PAB and reduce the number of operative procedures for these patients. This research aimed to assess the outcome of BDG surgery performed directly without doing initial PAB in patients with SV-UPBF. METHODS: This Multicenter retrospective cohort includes all patients with SV-UPBF who had BDG surgery. Patients were separated into two groups. Patients in Group 1 included patients who survived till they received BDG (20 Patients) after initial PAB (28 patients), whereas patients in Group 2 got direct BDG surgery without first performing PAB (16 patients). Cardiac catheterization was done for all patients before BDG surgery. Patients with indexed pulmonary vascular resistance (PVRi) ≥ 5 WU.m2 at baseline or > 3 WU.m2 after vasoreactivity testing were excluded. RESULTS: Compared with patients who had direct BDG surgery, PAB patients had a higher cumulative mortality rate (32% vs. 0%, P = 0.016), with eight deaths after PAB and one mortality after BDG. There were no statistically significant differences between the patient groups who underwent BDG surgery regarding pulmonary vascular resistance, pulmonary artery pressure, postoperative usage of sildenafil or nitric oxide, intensive care unit stay, or hospital stay after BDG surgery. However, the cumulative durations in the intensive care unit (ICU) and hospital were more prolonged in patients with BDG after PAB (P = 0.003, P = 0.001respectively). CONCLUSION: Direct BDG surgery without the first PAB is related to improved survival and shorter hospital stays in some selected SV-UPBF patients.

Percutaneous closure of postoperative residual ventricular septal defects, including dehiscence of surgical patches.

Background: Percutaneous closure of residual ventricular septal defects (VSDs) after congenital heart surgery may provide a safer and more efficient alternative to redo surgery. This study aimed to evaluate the outcome of transcatheter closure of residual postoperative VSD. Methods: This multicenter retrospective cohort study was conducted at the tertiary care institutions of King Faisal Specialist Hospital and King Abdulaziz University Hospital, Saudi Arabia, from March 2012 to March 2022. All patients who underwent transcatheter closure of postoperative residual VSD were included. As catheter closure of VSD related to surgical patches is challenging, patients were divided into two groups. Group 1 comprised patients with VSD related to the surgical patches, while Group 2 included residual muscular VSD. Various occluders and approaches were utilized based on the patient's weight and the VSD type, size, and proximity to the cardiac valves. Demographic, echocardiographic, catheterization, and outcome data were collected and analyzed using descriptive and comparative statistics. Results: Thirty-three patients underwent 37 VSD catheter closure procedures. Twenty-two procedures were done to close residual VSD related to the surgical patch, while fifteen were done for additional muscular VSD. The median age of the patients was 3.3 years, and the interquartile range (IQR) ranged between 9 months and 7 years. The median weight was 13.1 kilograms, with an IQR of 5.1 to 16.8 kilograms. The median pulmonary to systemic flow ratio (QP/QS) was 1.6 with an IQR of 1.5 to 2.44; the median systolic pulmonary pressure was 46 mmHg with an IQR of 32 to 54 mmHg. The median procedure duration was 120 minutes, with an IQR of 90 to 160 minutes. Patients in Group 1 were older and had a lower mean pulmonary pressure than Group 2 (P=0.02, P=0.007, respectively). Of the 37 procedures, 35 (94.6%) were done successfully, while two patients had redo surgery due to failed procedures (one had device embolization). Ten successful catheterizations were performed for infants weighing ≤5 kilograms. The functional heart failure class improved significantly after the closure of the residual VSD. There were three documented mortalities, none related to the procedure. No significant difference between patient groups regarding hospital stay or survival (P=0.660, P=0.791, respectively). Conclusions: After congenital heart surgery, transcatheter closure of residual VSD may be a safe and effective alternative to surgical closure. It can be applied to various residual VSD using a variety of occluders with satisfactory results. Moreover, using specific approaches can close residual VSD, even in small infants.

Voiding dysfunction in children causes, management, and prognosis: A single-center retrospective study.

OBJECTIVES: To review voiding dysfunction caused by 3 different etiologies; dysfunction voiding syndrome (DVS), neurogenic bladder secondary to spinal dysraphisim (NB), and valve bladder syndrome (VBS). METHODS: A single-center retrospective study on children with voiding dysfunction followed up at King Abdulaziz University Hospital, Jeddah, Saudi Arabia from 2005 to 2017. RESULTS: One hundred and ninety-nine children (67.3% boys) were included: Group 1 (n=75, DVS), Group 2 (n=64, NB), and Group 3 (n=60, VBS). Further classification according to the age at presentation; infants (46%), toddlers (27%) and school aged (28%). Management categories: 31% children needed observation only, 25% needed clean intermittent catheterization (CIC), 13% needed only surgery and 31% needed both surgery and CIC. Associated comorbidities: hydronephrosis (81%), vesicoureteral reflux (47%), pyelonephritis (37%) and renal scar (60%), all have negative impact on estimated glomerular filtration rate (eGFR). Urodynamic studies revealed poor bladder compliance in 57.6% and atonic bladder in 1.1%, progression to chronic kidney disease (22%), commenced on renal replacement therapy 11.5% and 4% died with ESKD. Overall improvement in the last eGFR is observed (p<0.001), but VBS group was the least to improve (p=0.021). There was a negative correlation between the last eGFR and age at presentation (p=0.002). CONCLUSION: Early diagnosis and management of childhood voiding dysfunction was associated with better prognosis. Children managed conservatively have better preservation of kidney function than those who needed surgery.

The prevalence of renal stones among local residents in Saudi Arabia.

BACKGROUND: Urolithiasis is the most common urological problem worldwide. It is a recurrent multifactorial problem that is caused by the interaction of several environmental and genetic factors. This study aimed to assess the prevalence of renal stones among local residents in Saudi Arabia in order to renew the statistics of renal stones occurrence in the current Saudi population. METHODS: A cross-sectional study was conducted using an electronic questionnairethat was distributed randomly through phones and social media to reach the local residents in Saudi Arabia. We then reviewed the published papers in Saudi journals for patients with renal stones. RESULTS: From a total of 580 responders to the electronic questionnaire, the prevalence of renal stones was 9.1% (n = 64). The median age at diagnosis was 29 years and the mean age at diagnosis was 36.91 years (SD = 18.66, Range of 20-99). Two peaks of age were observed, the first peak was at the (21-25) age group representing 34.4% of the kidney stones patients. The second peak was older than 47 years. The majority of those diagnosed with kidney stones had normal BMI (n = 29, 45.3%), and the family history of kidney stones among first degree relatives was found in 35.9% of the cases (n = 23). CONCLUSION: Kidney stones is a common health problem with the local incidence being underreported. In our sample, the prevalence was 9.1%. We also observed a relatively high percentage of positive family history among renal stone patients (34.9%) that could be attributed to the high rates of consanguinity. We encourage more local epidemiological studies to describe the patterns and the contributing factors of the development of kidney stones.

Clinical characteristics and outcomes of children with COVID-19 in Saudi Arabia.

OBJECTIVES: To determine the demographic and clinical characteristics, underlying comorbidities, and outcomes of children with coronavirus disease 2019 (COVID-19) infection. METHODS: In this retrospective study, we reported 62 pediatric patients (age <14 years) with confirmed COVID-19 between March 2 and July 1, 2020, at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. RESULTS: Comorbid conditions, including cardiac, neurological, respiratory, and malignant disorders, were reported in 9 patients (14.5%). The most prominent presenting complaints were fever (80.6%) and cough (48.4%). Most of our patients (80.6%) had mild disease, 11.3% had moderate disease, and 8.1% exhibited severe and critical illness. Twenty-one patients (33.9%) were hospitalized, with 4 patients (6.5%) admitted to the pediatric intensive care unit, and 3 (4.8%) patients died. CONCLUSION: All pediatric age groups are susceptible to COVID-19, with no gender difference. COVID-19 infection may result in critical illness and even mortality in subsets of pediatric patients.

Coronavirus disease in children: A multicentre study from the Kingdom of Saudi Arabia.

BACKGROUND: The COVID-19 global pandemic caused by severe acute respiratory syndrome coronavirus 2 infection, warranted attention for whether it has unique manifestations in children. Children tend to develop less severe disease with a small percentage present with clinical manifestations of paediatric multisystem inflammatory syndrome and have poor prognosis. We studied the characteristics of COVID-19 in children requiring hospitalisation in the Kingdom of Saudi Arabia and assessed the clinical presentation and the risk factors for mortality, morbidity, and paediatric intensive care (PICU) admission. METHODS: We conducted a retrospective analysis of COVID-19 patients under 15 years hospitalised at three tertiary academic hospitals between 1 March and 30 June 2020. RESULTS: Eighty-eight children were enrolled (>20% were infants). Seven (8%) were in critical condition and required PICU admission, and 4 (4.5%) died of which 3 met the full diagnostic criteria of multi-system inflammatory syndrome and had a high Paediatric Risk of Mortality (PRISM) score at the time of admission. The initial polymerase chain reaction (PCR) test result was positive for COVID-19 in most patients (97.7%), and the remaining two patients had positive result in the repeated confirmatory test. In a subset of patients (20 subjects), repeated PCR testing was performed until conversion to negative result, and the average duration for conversion was 8 (95% CI: 5.2-10.5) days Children requiring PICU admission presented with signs of respiratory distress, dehydration, and heart failure. Most had fever (71.4%) and tonsillitis; 61.4% were discharged within 7 days of hospitalisation. Risk factors for mortality included skin rash, hypotension, hypoxia, signs of heart failure, chest radiograph suggestive of acute respiratory distress syndrome, anaemia, leucocytosis, hypernatraemia, abnormal liver enzymes, and high troponin I, and risk factors for prolonged hospitalisation (>7 days) included the presence of comorbidities, leucopaenia, hyponatraemia, and elevated C-reactive protein. CONCLUSIONS: The majority of hospitalised children had a brief febrile illness and made a full recovery, but a minority had severe disease.

Outcomes of Primary Bidirectional Glenn in Children with Single Ventricle Physiology and Increased Pulmonary Blood Flow.

BACKGROUND: We reported our experience in managing patients with single ventricle (SV) physiology and increased pulmonary blood flow (PBF), aiming to assess if it is feasible to proceed with primary Bidirectional Glenn (BDG) without a prior operation to limit PBF. MATERIALS AND METHODS: This is a retrospective study with 51 consecutive patients who underwent BDG operation as a primary operation or a second stage prior to the definitive Fontan operation at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia between 2010 and 2018. Patients were categorized into two groups based on their PBF prior to the operation: Patients who had SV physiology and increased PBF (seven patients) vs. patients with SV physiology and restricted PBF (44 patients). RESULTS: The median age for the increased PBF group was 9.9 months [interquartile range (IQR): 2-16.9 months], and the median age for the restricted PBF group was 15.3 months (IQR: 6.7-42.6 months). Although the length of hospital stay was longer in patients with increased PBF (P = 0.039), we couldn't find a statistically significant difference in early mortality, duration of mechanical ventilation, length of pleural drainage, and length of intensive care unit (ICU) stay between the groups. CONCLUSION: In our experience, we found that primary BDG could be done safely for patients having SV physiology and increased PBF with acceptable short-term outcomes. It might further reduce the morbidity and mortality for those patients by avoiding the risk of initial pulmonary artery banding or aortopulmonary shunts.

2-Methoxyestradiol ameliorates metabolic syndrome-induced hypertension and catechol-O-methyltransferase inhibited expression and activity in rats.

Metabolic syndrome (MetS) is a common disorder that is associated with hypertension and poses a significant cardiovascular risk. Deactivation of extra-neuronal norepinephrine is mediated by catechol-O-methyltransferase (COMT). Endogenous 2-methoxyestradiol (2ME) is a product of COMT activity. The current study investigated the impact of 2ME on MetS-induced hypertension and the possible alterations in COMT expression and activity in rats. Animals were randomly divided into 4 groups. Group 1 received drinking water and standard food pellets. Groups 2, 3 and 4 were subjected to experimental induction of MetS. Animals in groups 3 and 4 were given daily IP injection of 2ME (25 and 50 mg/kg, respectively). MetS animals showed significant increases in body weight gain and visceral fat, fasting blood glucose and serum insulin and insulin resistance. Meanwhile, MetS was associated with a significant hypertriglyceridemia. Further, MetS significantly increased systolic, diastolic and mean arterial blood pressure. These effects were associated with significant reduction in COMT expression in the liver, kidneys and aorta as well as reduced hepatic activity. 2ME inhibited the alterations in body weight gain, visceral fat accumulation, fasting blood glucose and serum insulin, insulin resistance and serum triglycerides. Elevations in blood pressure were significantly inhibited by 2ME. Also, it attenuated the decrease in liver, kidney and aorta COMT expression and hepatic COMT activity. MetS was associated with elevated epinephrine and norepinephrine levels. Only the higher dose of 2ME significantly mitigated the rise in epinephrine level. In conclusion, 2ME protects against MetS-induced hypertension and averts COMT inhibited expression and activity.

Serum vitamin D status following pediatric cardiac surgery and association with clinical outcome.

Our aim is to determine the prevalence of vitamin D deficiency in children with congenital heart disease (CHD). In addition, we demonstrated the effect of cardiopulmonary bypass (CPB) on vitamin D, parathyroid hormone (PTH), and calcium levels. The association between perioperative vitamin D levels and postoperative clinical outcomes has been explored. A prospective observational study was conducted from February 2018 to June 2019 on 69 children undergoing elective surgery for CHD under CPB. Blood samples were collected preoperatively, immediate postoperatively, and 24 h postoperatively. Vitamin D deficiency was present in 34 (49.3%) patients preoperatively and 63 (91.3%) patients immediately postoperative. We identified 42.03% decline of 25(OH)D immediately postoperative. Changes in ionized calcium (iCa) concentrations were accompanied by reciprocal alterations in PTH concentrations. Lower postoperative 25(OH)D was associated with higher maximum vasoactive inotropic score (VIS) in the first 24 h postoperative (r = - 0.259, p = 0.03).Conclusion: Vitamin D deficiency is common in children with CHD and the majority are vitamin D deficient following cardiac surgery with acute decline of serum 25(OH)D after CPB. Lower postoperative vitamin D levels in children undergoing cardiac surgery are associated with the need for increasing the inotropic support.What is Known:• Vitamin D is a pleiotropic hormone, important for calcium homeostasis.• Vitamin D deficiency might affect the outcome in critically ill patients.What is New:• Cardiopulmonary bypass causes acute decline of vitamin D in children.• Lower postoperative vitamin D is associated with higher inotropic support.

Determinants of successful medical education of caregivers of children with congenital heart diseases.

OBJECTIVE: To assess the implementation and efficiency of medical education among the caregivers of children with congenital heart disease (CHD) and to investigate its success factors. MATERIAL AND METHOD: A cross-sectional study was conducted from February 2014 to March 2014 in a tertiary healthcare center. Study included 120 caregivers of children with CHD visiting the Paediatric Cardiology Clinic that included 93% mothers, aged 31.72 ± 6.67 years, 38.3% Saudi citizens and 45.0% had a high educational level. Caregivers were interviewed through a semi-structured questionnaire that included socio-demographic data of participants and demographic and clinical data of patients; awareness about the diagnosis and self-assessed understanding of thedisease; disease dimensions explained, educational material used andefficacy of whole medical education received in alleviating caregivers' anxiety. RESULTS: Of children who had CHD, around 42.5% were males aged 5.65 ± 3.99 years and 37.3% had ventral septal defect, 14.4% had atrial septal defectand 19.5% had a complex CHD. Study results showed that 83.3% of caregivers were aware of the correct diagnosis and 64.2% had good understanding of the nature and effect of the disease. Frequently explained disease dimensions were impact on child's growth (65.8%), complications (57.5%), and outcomes (55.0%). Least frequently explained dimensions were those related to infective endocarditis (IEC) including risk factors (15.8%), prophylaxis (17.5%), complications (19.2%) and symptoms (22.5%). The whole medical education received was efficient to enable good understanding of the disease and alleviate anxiety in 70.8% and 62.5% of the cases, respectively. Time dedicated to educate, use of illustrating educational materials, explaining various disease dimensions (aetiology, complications, prognosis, long-term management, etc.) and the number of dimensions explained were all significant predictors for both good understanding of the child's disease and anxiety relief among caregivers.