Impact of Blood Sampling Methods on Blood Loss and Transfusion After Pediatric Cardiac Surgery: An Observational Study.

OBJECTIVES: The aims of this study were to assess the impact of the closed-loop sampling method on blood loss and the need for blood transfusion in pediatric patients following cardiac surgery. DESIGN: Retrospective observational study. SETTING: A single tertiary center. PARTICIPANTS: All pediatric patients younger than 4 years old who were admitted to the pediatric intensive care unit (PICU) after cardiac surgery were enrolled. The study included 100 pediatric patients in the conservative (postimplementation) group and 43 pediatric patients in the nonconservative group (preimplementation). INTERVENTIONS: Observational. MEASUREMENTS: The primary outcome was the volume of blood loss during the PICU follow-up period. The secondary outcomes were the requirement for blood transfusion in each group, duration of mechanical ventilation, length of intensive care unit (ICU) stay, length of hospital stay, and mortality. MAIN RESULTS: In the conservative (postimplementation) group, blood loss during the follow-up period was 0.67 (0.33-1.16) mL/kg/d, while it was 0.95 (0.50-2.30) mL/kg/d in the nonconservative (preimplementation) group, demonstrating a significant reduction in blood loss in the conservative group (p = 0.012). The groups showed no significant differences in terms of the required blood transfusion volume postoperatively during the first 24 hours, first 48 hours, or after 48 hours (p = 0.061, 0.536, 0.442, respectively). The frequency of blood transfusion was comparable between the groups during the first 24 hours, first 48 hours, or after 48 hours postoperatively (p = 0.277, 0.639, 0.075, respectively). In addition, the groups did not show significant differences in the duration of mechanical ventilation, length of ICU stay, length of hospital stay, or mortality. CONCLUSIONS: The closed-loop sampling method can be efficient in decreasing blood loss during postoperative PICU follow-up for pediatric patients after cardiac surgeries. However, its application did not reduce the frequency or the volume of blood transfusion in these patients.

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.

Percutaneous coronary stent implantation in children and young infants following surgical repair of congenital heart disease.

Background: Coronary artery stent implantation (CSI) in the pediatric population is rare. Only a few reports were published on managing postoperative coronary artery obstruction using coronary stents following surgical repair of congenital heart diseases (CHD). This study aimed to analyze the feasibility, indications, procedural technique, risk factors, and short-term outcomes of CSI after pediatric cardiac surgery. Methods: In this retrospective cohort study, we reviewed all pediatric patients who underwent surgical repair of CHD requiring postoperative CSI in two cardiac centers (King Abdulaziz University Hospital and King Faisal Specialist Hospital and Research Center) between 2012 and 2022. Survival to hospital discharge was the study's primary outcome. The secondary outcomes included procedural success, duration of mechanical ventilation, intensive care unit (ICU) stay, hospital stay, need for coronary reintervention, and late mortality. A descriptive analysis was performed for the collected data from the patients' medical records. Results: Eleven patients who underwent postoperative CSI were identified. The most common anatomic diagnosis was congenital aortic valve stenosis. All patients underwent cardiac catheterization on extracorporeal membrane oxygenation support except one patient, who presented with chest pain after cardiac surgery. Procedural success was achieved in all patients with excellent revascularization documented by post-procedural angiograms. Both patients who had late coronary events after cardiac surgery survived hospital discharge. There was no in-hospital mortality among the two patients who required stenting of only the right coronary artery. The four patients who required more than 120 minutes to complete the procedure had early mortality. After CSI, the median duration of mechanical ventilation and ICU stay was 12 and 17 days, respectively. Six patients (54.5%) survived hospital discharge post-CSI; they did not require re-intervention during the follow-up period (38-1,695 days). Conclusions: CSI in pediatric patients can be performed with excellent procedural success for treating coronary artery stenosis after cardiac surgery. It could be considered a potential treatment strategy for this population.

Factors influencing blood pressure fluctuation in pediatric patients with sickle cell disease in Saudi Arabia: A retrospective single-center cohort study.

OBJECTIVES: To investigate changes in blood pressure (BP) among pediatric patients with sickle cell disease (SCD) and determine the variables that might influence these changes. METHODS: A total of 100 pediatric patients with SCD who followed up in the pediatric outpatient clinic were recruited for this retrospective cohort study. Clinical data included anthropometric measures, average systolic and diastolic BP recorded during multiple follow-up visits, hemoglobin (Hb) level, serum creatinine, and hemoglobin S percentage. Blood pressure measurements were categorized according to the guidelines of the American Academy of Pediatrics (AAP, 2017). RESULTS: In this cohort, 68% of the patients had normal systolic BP, 13% had elevated systolic BP, 17% had stage 1 hypertension (HTN), while only 2% reported stage 2 HTN. Patients who were overweight had relatively high systolic BP compared to patients who were underweight (p=0.034) or had normal weight (p=0.023). The average systolic BP significantly correlates with body mass index (r= 0.377, p<0.001) and serum creatinine (r=0.369, p<0.001). CONCLUSION: Pediatric overweight SCD patients exhibited higher average systolic BP than those underweight or normal weight. Body mass index and serum creatinine significantly influenced the average systolic BP more than the Hb level or Hb S percentage.

Early echocardiographic signs of cardiovascular affection in pediatric familial hypercholesterolemia.

Familial hypercholesterolemia (FH) is a rare autosomal dominant genetic disorder caused by defective low-density lipoprotein (LDL) receptors or abnormal apolipoprotein B. FH raises the risk of premature atherosclerotic disease and cardiovascular death in young adults. However, cardiovascular affection in children needs to be more adequately studied. Our study aimed to evaluate the effect of hypercholesterolemia on the cardiovascular system of pediatric patients with homozygous FH using conventional and advanced echocardiographic parameters such as tissue Doppler imaging (TDI) and 2-dimensional speckle-tracking echocardiography (2D-STE). This case-control study matched 25 healthy children with 21 patients with homozygous FH. Both groups had conventional echocardiography, TDI, and 2D-STE. Myocardial velocities of the left and right ventricles, left ventricular strain, and aortic stiffness parameters were measured. The FH group had greater systolic blood pressure, dilated coronary arteries, and hypertrophied left ventricle (LV) compared to the control (P = 0.0001, P = 0.001, P = 0.01, respectively). The mitral E/E' ratio was higher in the patient group than in the control group (P = 0.007), indicating LV diastolic dysfunction in patients. At the same time, LV systolic function evaluated by 2D-STE was comparable to that in the control group. The abdominal aorta circumferential strain and ascending aorta M-mode-derived strain were significantly lower in patients compared to those in the control (P = 0.024, P = 0.0001, respectively), indicating increased aortic stiffness in the patients' group; moreover, 85.7% of patients had mild aortic insufficiency.  Conclusion: Mild aortic insufficiency, coronary artery dilatation, left ventricular (LV) diastolic dysfunction, and increased aortic stiffness are among early cardiovascular markers in pediatric patients with homozygous FH before impaired LV systolic function. What is Known: • Familial hypercholesterolemia (FH) in adults is associated with accelerated atherosclerosis, aortic valvopathy, dilated coronary arteries, ischemic heart disease, and premature cardiovascular death. • The cardiovascular effects of FH in children require additional research. What is New: • Pediatric patients with familial hypercholesterolemia tend to have an early affection for left ventricular diastolic function before the affection for the systolic function. • The diastolic dysfunction associated with pediatric FH is correlated to the aortic stiffness and low-density lipoprotein levels.

Reversible Cardiomyopathy, What Should the Clinicians Keep in Mind? A Case Report.

Primary carnitine deficiency (PCD) is an autosomal recessive disorder characterized by decreased carnitine levels essential for Beta oxidation in various organs, including the heart. Early diagnosis and treatment of PCD can revert cardiomyopathy. A 13-year-old girl presented with heart failure due to dilated cardiomyopathy and severe cardiac dysfunction; following L carnitine treatment, the patient's clinical conditions improved, and cardiac functions returned to normal within weeks. Investigations revealed PCD; regular L carnitine has been provided, all cardiac medications are discontinued, and the patient is doing well. We believe PCD should be ruled out in every patient with cardiomyopathy.

Using 2D speckle-tracking echocardiography to localize the accessory pathway and evaluate cardiac function and dyssynchrony in pediatric patients with Wolf-Parkinson-White syndrome.

Wolf-Parkinson-White (WPW) accessory pathway (AP) may be associated with reentry supraventricular tachycardia (SVT) in addition to ventricular dyssynchrony and cardiac dysfunction. Electrophysiological studies (EPS) are the gold standard for the localization of the AP; however, 2D speckle-tracking echocardiography (2D-STE) may help in the localization of the AP noninvasively. Our study aims to evaluate the capability of 2D-STE for AP localization and the identification of AP-related contractile abnormalities and dyssynchrony in pediatric patients with WPW syndrome. This prospective multicenter cohort study involved 18 pediatric patients with ventricular preexcitation from January 2021 to January 2023. Tissue Doppler imaging (TDI), conventional echocardiography, and 2D-STE were done. Myocardial velocities, myocardial performance index (MPI), the global and segmental longitudinal strain of the left ventricle (LV), and time-to-peak longitudinal strain (TPLS) were measured before and after ablation. The longitudinal strain of the LV segments supplied by the AP, or the nearby segments close to the AP, was significantly impaired and improved after ablation (P = 0.0001). The abnormal strain pattern in the affected segments could predict the location of the AP. The TPLS of the affected segments significantly increased after ablation (P = 0.0001), denoting improved dyssynchrony. The ejection time and the LV MPI measured at the basal septum improved significantly after ablation. CONCLUSIONS: 2D STE may be used for noninvasive localization of the AP and to evaluate cardiac function and dyssynchrony in patients with WPW. Further research on more patients is necessary to validate this method for AP localization. WHAT IS KNOWN: • Accessory pathways (AP) associated with the Wolf-Parkinson-White (WPW) syndrome have been linked to supraventricular tachycardia (SVT). Even without SVT, WPW can cause left ventricular dyssynchrony, contractile dysfunction, and cardiomyopathy. • Electrophysiology study is the gold standard for the localization of the AP in WPW syndrome. WHAT IS NEW: • The combination of 2D-speckle-tracking echocardiography (2D-STE) and the modified Arruda algorithm can precisely localize the AP associated with WPW syndrome. • 2D-STE can potentially assess cardiac function and dyssynchrony related to WPW syndrome. Additionally, 2D-STE can be utilized to evaluate the effectiveness of ablation in restoring cardiac function and dyssynchrony.

Clinical deterioration following arterial switch surgery due to extensive aortopulmonary collaterals, unusual condition but worth considering: case report.

BACKGROUND: The occurrence of major aortopulmonary collateral arteries (MAPCAs) is infrequent in patients with D-transposition of great arteries (D-TGA) with intact ventricular septum (IVS). Hemodynamically significant MAPCAs may complicate the postoperative course of these patients after arterial switch operation (ASO). CASE PRESENTATION: We present a rare case of neonatal D-TGA-IVS associated with extensive MAPCAs. After the ASO, the patient developed pulmonary hemorrhage, chest wall edema, and deterioration of lung compliance with the need for high-frequency ventilation (HFV). The patient also had a significant capillary leak with skin edema, high chest tube drainage, and high peritoneal drainage. Cardiac catheterization revealed extensive MAPCAs supplying the whole lung segments. After the catheter closure of most of these MAPCAs, the patient had clinical improvement. CONCLUSIONS: Although the occurrence of MAPCAs with D-TGA-IVS is infrequent, clinicians should suspect their presence in cases with unexplained heart failure, pulmonary hemorrhage, or cardiovascular compromise after ASO. Catheter closure of MAPCAs is feasible with an acceptable short-term outcome.

Risk factors of early postoperative cardiac arrhythmia after pediatric cardiac surgery: A single-center experience.

OBJECTIVES: To evaluate the incidence of arrhythmia in the early postoperative period and to identify its risk factors among pediatric patients following cardiac surgery at King Abdulaziz University Hospital (KAUH), Jeddah, Saudi Arabia, between 2015-2020. METHODS: Out of 1242 patients, a total of 821 aged <18 years who underwent cardiac surgery were included in this retrospective cohort carried out in June 2021 at KAUH, Jeddah, Saudi Arabia. Information retrieved from the hospital medical records had patients' demographics, types of arrhythmias, hemodynamic stability, electrolyte disturbances, cardiopulmonary bypass (CPB), and aortic cross-clamp (AXC) durations. Univariate and multivariate logistic regression analyses were used to evaluate the possible risk factors associated with postoperative arrhythmia. RESULTS: Of the 821 patients, 140 (17.1%) developed arrhythmia postoperatively. The most common arrhythmias were junctional ectopic tachycardia (JET, 51.4%), atrioventricular block (27.1%), and supraventricular tachycardia (10%). The majority of cases occurred on the first day postoperatively (79.3%). Patients with postoperative arrhythmias had a more prolonged CPB (p=0.0001) and AXC (p=0.005) time, electrolytes disturbances (p=0.021), and hemodynamic instability (p=0.0001) than other patients. CONCLUSION: Postoperative arrhythmia, especially JET, is common after pediatric cardiac surgery. Prolonged cardiopulmonary bypass, prolonged aortic cross-clamping, electrolytes disturbances, and hemodynamic instability are possible risk factors for postoperative cardiac arrhythmias.

Outcomes of bidirectional Glenn surgery done without prior cardiac catheterization.

BACKGROUND: Cardiac catheterization is usually done routinely in patients with univentricular hearts before palliative Bidirectional Glenn (BDG) surgery. The objective of this study was to compare the outcomes of patients with physiological univentricular hearts and restrictive pulmonary flow that did not undergo routine cardiac catheterization before BDG with the patients that did have cardiac catheterization done. We retrospectively reviewed the data of all patients with single ventricle physiology and restrictive pulmonary blood flow who underwent BDG surgery from January 2016 till December 2020. Patients were divided into two groups: the catheterization and the non-catheterization groups. RESULTS: Out of 93 patients, 25 (27%) underwent BDG surgery without prior cardiac catheterization. The median age of patients was ten months, interquartile range (IQR) was 5-18 months. Tricuspid atresia represented 36% of the non-catheterization group, while unbalanced atrioventricular septal defect and hypoplastic left heart syndrome represented 19% and 17.6% of the catheterization group. No patients in the catheterization group were excluded from further BDG surgery based on the catheterization data. Moreover, no significant differences were found between the patients' groups regarding the length of hospital stay, length of intensive care unit stay, postoperative oxygen saturation, or survival (P = 0.266, P = 0.763, P = 0.543, P = 0456). CONCLUSIONS: Although pre-BDG cardiac catheterization is the routine and standard practice, in certain situations, some patients with single ventricle physiology and restrictive pulmonary blood flow may go directly to BDG without cardiac catheterization if noninvasive imaging is satisfactory on a case-by-case basis and according to center experience. Pre-BDG catheterization could be reserved for patients with limited echocardiographic studies, high-risk patients, or those indicated for catheter intervention before BDG surgery.

Neonatal congenital heart surgery: contemporary outcomes and risk profile.

OBJECTIVE: Many studies still dispute the identification of independent risk factors that influence outcome after neonatal cardiac surgery. We present our study to announce the contemporary outcomes and risk profile of neonatal cardiac surgery at our institute. METHODS: We designed a retrospective study of neonatal patients who underwent surgery for congenital heart diseases between June 2011 and April 2020. Demographic, operative, and postoperative data were collected from medical records and surgical databases. The primary outcome was the operative mortality (in-hospital death) and secondary outcomes included hospital length of stay, intensive care unit stay, duration of mechanical ventilation. RESULTS: In total, 1155 cardiac surgeries in children were identified; of these, 136 (11.8%) were performed in neonates. Arterial switch operations (48 cases) were the most frequent procedures. Postoperatively, 11 (8.1%) patients required extracorporeal membrane oxygenation, and 4 (2.9%) patients had complete heart block. Postoperative in-hospital mortality was 11%. The median postoperative duration of mechanical ventilation, intensive care unit stay, and hospital length of stay were 6, 18, and 24 days, respectively. CONCLUSION: The early outcomes of neonatal cardiac surgery are encouraging. The requirement of postoperative extracorporeal membrane oxygenation support, postoperative intracranial hemorrhage, and acute kidney were identified as independent risk factors of mortality following surgery for congenital heart defects in neonates.

Margin between success and failure of PDA stenting for duct-dependent pulmonary circulation.

OBJECTIVES: Percutaneous patent ductus arteriosus (PDA) stenting is a therapeutic modality in patients with duct-dependent pulmonary circulation with reported success rates from 80-100%. The current study aims to assess the outcome and the indicators of success for PDA stenting in different ductal morphologies using various approaches. METHODS: A prospective cohort study from a single tertiary center presented from January 2018 to December 2019 that included 96 consecutive infants with ductal-dependent pulmonary circulation and palliated with PDA stenting. Patients were divided according to PDA origin into 4 groups: Group 1: PDA from proximal descending aorta, Group 2: from undersurface of aortic arch, Group 3: opposite the subclavian artery, Group 4: opposite the innominate/brachiocephalic artery. RESULTS: The median age of patients was 22 days and median weight was 3 kg. The procedure was successful in 78 patients (81.25%). PDA was tortuous in 70 out of 96 patients. Femoral artery was the preferred approach in Group 1 (63/67), while axillary artery access was preferred in the other groups (6/11 in Group 2, 11/17 in Group 3, 1/1 in Group 4, P <0.0001). The main cause of procedural failure was inadequate parked coronary wire inside one of the branch of pulmonary arteries (14 cases; 77.7%), while 2 cases (11.1%) were complicated by acute stent thrombosis, and another 2 cases with stent dislodgment. Other procedural complications comprised femoral artery thrombosis in 7 cases (7.2%). Patients with straight PDA, younger age at procedure and who had larger PDA at pulmonary end had higher odds for success (OR = 8.01, 2.94, 7.40, CI = 1.011-63.68, 0.960-0.99, 1.172-7.40,respectively, P = 0.048, 0.031,0.022 respectively). CONCLUSIONS: The approach for PDA stenting and hence the outcome is markedly determined by the PDA origin and morphology. Patients with straight PDA, younger age at procedure and those who had relatively larger PDA at the pulmonary end had better opportunity for successful procedure.

Cardiac Catheterization During Extracorporeal Membrane Oxygenation After Congenital Cardiac Surgery: A Multi-Center Retrospective Study.

Cardiac catheterization can affect clinical outcomes in patients on extracorporeal membrane oxygenation (ECMO) after congenital heart surgery; however, its effect in this group of patients remains unclear. This study aimed to evaluate the safety and outcome of cardiac catheterization in patients undergoing ECMO after congenital cardiac surgery and determine predictors that influence successful weaning. This retrospective cohort study included pediatric patients who underwent cardiac catheterization while on ECMO after congenital heart surgery in two cardiac centers between November 2012 and February 2020. Predictors of successful weaning from ECMO were studied using univariate and multivariate logistic regression analyses. Of 123 patients on ECMO support after congenital cardiac surgery, 60 patients underwent 60 cardiac catheterizations (31 diagnostic and 29 interventional). Thirty-four (56.7%) and 22 patients (36.7%) underwent successful decannulation from ECMO support and survived after hospital discharge, respectively. Patients who underwent earlier catheterization (within 24 h of ECMO initiation) had more successful weaning from ECMO and survival compared to others. Patients who underwent an interventional procedure (interventional catheterization or redo cardiac surgery after cardiac catheterization) had better survival than those who underwent only diagnostic catheterization (P = 0.038). Shorter durations of ECMO was the most important predictor of successful weaning from ECMO. Early cardiac catheterization greatly impacts successful weaning from ECMO and survival. Patients with correctable lesions amenable either by catheterization or redo surgery are more likely to survive. Shorter durations of ECMO could have a significant influence on successful weaning from ECMO and survival.

Hemopericardium with cardiac tamponade as a rare presentation of a massive aortic aneurysm in a young child with autosomal recessive cutis laxa.

Aortic aneurysms are rare in the pediatric age group and are commonly caused by genetic disorders associated with vasculopathy, weakness and fragility of arterial walls with progressive dilatation or even rupture. We reported a giant aortic aneurysm involving the ascending aorta and aortic arch in a 20-month-old girl with autosomal recessive cutis laxa type 1B (ARCL1B) who presented with hemorrhagic pericardial effusion and tamponade (impending rupture). Successful surgical repair has been done through excision of the aneurysmal part and replacement by Hemashield graft with preservation of the aortic valve.

Outcome of ductus arteriosus stenting including vertical tubular and convoluted tortuous ducts with emphasis on technical considerations.

BACKGROUND: Ductal stenting is the preferred method of securing adequate pulmonary blood flow in patients with duct-dependent pulmonary circulation. The main limitation in most centers is the difficult vertical tubular or convoluted ducts that represent real challenges to interventional pediatric cardiologists. We present our experience in patent ductus arteriosus (PDA) stenting with some technical tips to overcome difficulties, especially in stenting tortuous or long tubular ducts. This study was conducted on all patients with cyanotic congenital heart disease who underwent PDA stenting between January 2011 and December 2018. RESULTS: We attempted to stent the PDA in 43 patients, with a success rate of 93% (40 patients) and only one procedural mortality. There was also one stent migration that needed to be treated with urgent surgery. Three-fourths of the patients had difficult ductal morphology and origin. One stent was used to cover the PDA in 27 patients (62.8%), two stents were used in 13 (30.2%), and three stents were used in 2 patients (4.6%). In-stent stenosis rate was 12.5% (5 patients) and the development of progressive left pulmonary artery stenosis was seen in two patients (5%). Pulmonary artery growth was adequate in all patients. CONCLUSIONS: PDA stenting is an effective method of palliation for patients with duct-dependent pulmonary circulation. It has low morbidity and mortality rates. Stenting difficult ducts have become more feasible with evolving materials and techniques.

Novel MYO1D Missense Variant Identified Through Whole Exome Sequencing and Computational Biology Analysis Expands the Spectrum of Causal Genes of Laterality Defects.

Laterality defects (LDs) or asymmetrically positioned organs are a group of rare developmental disorders caused by environmental and/or genetic factors. However, the exact molecular pathophysiology of LD is not yet fully characterised. In this context, studying Arab population presents an ideal opportunity to discover the novel molecular basis of diseases owing to the high rate of consanguinity and genetic disorders. Therefore, in the present study, we studied the molecular basis of LD in Arab patients, using next-generation sequencing method. We discovered an extremely rare novel missense variant in MYO1D gene (Pro765Ser) presenting with visceral heterotaxy and left isomerism with polysplenia syndrome. The proband in this index family has inherited this homozygous variant from her heterozygous parents following the autosomal recessive pattern. This is the first report to show MYO1D genetic variant causing left-right axis defects in humans, besides previous known evidence from zebrafish, frog and Drosophila models. Moreover, our multilevel bioinformatics-based structural (protein variant structural modelling, divergence, and stability) analysis has suggested that Ser765 causes minor structural drifts and stability changes, potentially affecting the biophysical and functional properties of MYO1D protein like calmodulin binding and microfilament motor activities. Functional bioinformatics analysis has shown that MYO1D is ubiquitously expressed across several human tissues and is reported to induce severe phenotypes in knockout mouse models. In conclusion, our findings show the expanded genetic spectrum of LD, which could potentially pave way for the novel drug target identification and development of personalised medicine for high-risk families.

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.

Equine pericardium: a versatile alternative reconstructive material in congenital cardiac surgery.

BACKGROUND: Pericardial patches are often used for repair of congenital cardiac defects. The aim of this study was to describe our initial experience with the use of equine pericardium and its safety and advantages and disadvantages compared to bovine pericardium. METHODS: We designed a retrospective cohort study of 111 patients who were surgically treated for congenital heart disease between 2017 and 2020. Equine pericardium was used in 58 patients and bovine pericardium was used in 53 patients. Recorded variables included demographic data, preoperative cardiac pathology, site of patch insertion, morbidity and mortality. RESULTS: The overall survival rate was 94.5% and no deaths were related to patch insertion. None of our patients were reoperated on for patch related complications. Postoperative transcatheter intervention was needed in 2 patients (1.8%): one for dilatation of aortic arch stenosis after repair of hypoplastic left heart syndrome with equine pericardium and one for dilatation of pulmonary artery branches after repair of tetralogy of Fallot using bovine pericardium. CONCLUSIONS: Equine pericardium is a safe patch material for reconstruction in congenital heart surgery. It may be preferable to bovine pericardium in cases requiring a complex shape or a pliable patch as in in arch reconstruction or for valve reconstruction.

Impact of coronavirus disease 2019 in a child who underwent ventricular septal defect device closure: a case report.

BACKGROUND: Studies about the incidence and severity of coronavirus disease 2019 (COVID-19) in children are still significantly lower than those in adults. Moreover, data on the effect of COVID-19 in children with congenital heart disease (CHD) are limited. To the best of our knowledge, this study first reported mortality in a child with CHD who acquired COVID-19. CASE SUMMARY: A 16-month-old boy presented to the emergency department due to shortness of breath, fever, cough, and poor oral intake. He tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). He required mechanical ventilation for rapidly progressing respiratory failure. The patient had a large mid-muscular ventricular septal defect (VSD) that was closed percutaneously at the age of 13 months. Moreover, we followed his hospital sequelae from admission to death. DISCUSSION: This child had multiple risk factors, including malnutrition and persistent pulmonary hypertension (PH) after late closure of the VSD. The pre-existing PH could have been aggravated by the lung condition associated with COVID-19 and the respiratory failure triggered by SARS-CoV-2 infection. The patient presented with ventricular systolic dysfunction, elevated troponin serum levels and newly developed trifascicular block, which were indicative of myocardial injury. The elevated inflammatory markers and multi-organ dysfunction seem to corroborate multisystem inflammatory syndrome in children, which was described recently among paediatric patients with COVID-19.