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.

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.

The influence of age and weight on the outcomes of complete atrioventricular septal defect repair.

BACKGROUND: The appropriate age and weight for surgical repair of atrioventricular septal defect (AVSD) is an area of controversy. We aimed to study the effect of weight and age at the time of surgical repair for complete AVSD in children less than 2 years of age on postoperative outcomes. A retrospective data review was performed for patients who underwent the AVSD repair from 2012 to 2019 at our institutions. Our primary outcome was the postoperative in-hospital length of stay (LOS). Secondary outcomes included total positive pressure ventilation (PPV), ventilation time, maximum vasoactive-inotropic score (max VIS), and other postoperative complications. RESULTS: The study included fifty patients. The median age was 191 days, and the median weight was 4.38 kg at the time of surgery. Weight < 4 kg was associated with longer PPV time and postoperative in-hospital LOS (p value of 0.033 and 0.015, respectively). Additionally, they had higher max VIS at 24 h and 48 h than the other groups with bodyweight 4-5.9 kg or ≥ 6 kg (p value of 0.05 and 0.027, respectively). Patients with older age or lower weight at operation had a longer in-hospital LOS and total length of PPV after surgery. There were no postoperative in-hospital deaths. CONCLUSIONS: Older age and lower weight at the time of surgical repair of atrioventricular septal defect could be independent predictors of prolonged postoperative in-hospital length of stay and total length of positive pressure ventilation.

Postoperative Intracardiac Thrombus in a Child with Nephrotic Syndrome.

Background: Thromboembolic events are a known complication of nephrotic syndrome in children. Intracardiac thrombi are a rare location for thrombus formation but can lead to severe clinical consequences. Case Presentation: We report an intracardiac left atrial thrombus in a child with new onset steroid-resistant nephrotic syndrome and recently repaired atrial septal defect and cor triatriatum. The thrombus was successfully treated with surgical excision. Conclusion: Intracardiac thrombus is a rare but serious complication of nephrotic syndrome in children, especially in children with risk factors for potentially increased intracardiac thrombogenicity such as history of recent intervention.

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.

Paediatric supraventricular tachycardia patients potentially more at risk of developing psychological difficulties compared to healthy peers.

AIM: Assess executive and socio-emotional/behavioural functioning in paediatric supraventricular tachycardia (SVT) patients. METHODS: SVT patients aged 7-17 who had not undergone catheter ablation were included. Parents completed the Child Behaviour Checklist (CBCL/6-18) and the Behavior Rating Inventory of Executive Functioning (BRIEF). Participants age 11-17 years completed the Youth Self-Report (YSR/11-18) and the BRIEF Self-Report (BRIEF-SR). One-sample z test was used to compare questionnaire results to the average t-score range (M = 50, SD = 10). RESULTS: Thirty (18 female) children/adolescents participated (M = 12.6 years old, SD = 3.2 years) with a mean SVT onset age of 7 years (SD = 4.3 years). BRIEF and BRIEF-SR results suggested no difference in executive functioning from average. Mean t-scores of CBCL/6-18 and YSR/11-18 subscales Anxious/Depressed, Withdrawn/Depressed, Somatic Complaints, Thought Problems, Diagnostic and Statistical Manual of Mental Disorders (DSM) Affective Problems, DSM Anxiety Problems and DSM Somatic Problems were significantly elevated compared to average. YSR/11-18 subscales Social Problems, Attention Problems, Internalizing Problems, DSM ADHD Problems and DSM Oppositional Defiant Problems had elevated mean t-scores compared to average. Effect sizes were small to medium (0.2 ≤ d ≤ 0.8). CONCLUSION: Paediatric patients with SVT potentially have a greater risk of developing behaviour, especially internalizing, problems compared to similarly aged children/adolescents without SVT.

Saudi Heart Rhythm Society Task Force on Management of Potential Arrhythmogenicity Associated with Pharmacotherapy for COVID-19.

Evidence of cardiovascular complications associated with the COVID-19 global pandemic continues to evolve. These include direct and indirect myocardial injury with subsequent acute myocardial ischemia, and cardiac arrhythmia. Some results from a limited number of trials of antiviral medications, along with chloroquine/hydroxychloroquine and azithromycin, have been beneficial. However, these pharmacotherapies may cause drug-induced QT prolongation leading to ventricular arrhythmias and sudden cardiac death. Mitigation of the potential risk in these susceptible patients may prove exceptionally challenging. The Saudi Heart Rhythm Society established a task force to perform a review of this subject based on has recently published reports, and studies and recommendations from major medical organizations. The objective of this review is to identify high-risk patients, and to set clear guidelines for management of patients receiving these pharmacotherapies.

Catheter ablation of supraventricular tachycardia after tricuspid valve surgery in patients with congenital heart disease: A multicenter comparative study.

BACKGROUND: Tricuspid valve (TV) surgery is often required for adult congenital heart disease (ACHD), but may hinder catheter ablation when an artificial material or imbricated tissue covers the tricuspid annulus. OBJECTIVE: The purpose of this study was to determine the outcomes of catheter ablation after TV surgery in a large ACHD cohort. METHODS: An international retrospective study involving 7 centers was conducted. Patients who did and did not undergo TV surgery were matched for age, lesion classification, and postsurgical duration. TV operations were classified as valve ring/replacement vs repair. RESULTS: One hundred thirty-six patients (42 ring/replacement, 39 repair, and 55 no TV surgery; median 32 years [IQR 20 - 46]) underwent 180 procedures targeting 239 tachycardias (cavotricuspid-isthmus dependent intraatrial reentrant tachycardia 36%, other intraatrial reentrant tachycardia 29%, focal atrial tachycardia 18%, and other supraventricular tachyarrhythmia 17%). Post-TV surgery, procedures were longer (4.3 hours vs 3.3 hours; P = .003) and required longer fluoroscopy time (31 minutes vs 18 minutes; P = .001). At least partial acute success was achieved in 81% of procedures in the TV ring/replacement group vs 94% in both TV repair and no TV surgery groups (P = .03). The difference was driven mainly by ablation of annular substrates, with acute success in 73% of TV ring/replacement, 92% of TV repair, and 94% of no TV surgery groups (P = .01). Over a median of 3.0 years, tachycardia recurred after 26% of procedures. TV ring/replacement predicted recurrence in the multivariable analysis (hazard ratio 2.4; 95% confidence interval 1.2-5.2; P = .009). CONCLUSION: After surgery for ACHD, catheter ablation success was lower and tachycardia recurrence was higher after TV valve ring/replacement surgery. The findings of this retrospective report support future larger multicenter series and prospective evaluation to determine the role of empirical annular substrate ablation.

Cardiovascular Collapse with Intravenous Amiodarone in Children: A Multi-Center Retrospective Cohort Study.

OBJECTIVE: To determine the incidence of cardiovascular collapse in children receiving intravenous (IV) amiodarone and to identify the population at risk. DESIGN: A multicenter study of patients ≤ 18 years of age who received intravenous amiodarone between January 2005 and December 2015. A retrospective analysis was performed to identify patients who developed cardiovascular collapse (bradycardia and/or hypotension). RESULTS: Of 456 patients who received amiodarone, cardiovascular collapse occurred in 47 patients (10%). Patient risk factors for collapse in a univariate analysis were as follows: age < 3 months (p = 0.04), depressed cardiac function (p < 0.001), blood pressure below 3rd percentile (p < 0.001), high lactate at baseline (p < 0.001). Administration risk factors included bolus administration (p = 0.04), and bolus administration over ≤ 20 min (p = 0.04). In multivariate analysis, age, baseline blood pressure less than 3rd percentile, and rapid bolus delivery were independent risk factors for cardiovascular collapse in the study group. The mortality rate was significantly higher in the collapse group (28% versus 8%). CONCLUSION: We found an association between IV amiodarone administration and the risk of developing cardiovascular collapse in a significant subset of children. Extreme caution and careful hemodynamic monitoring is recommended when using IV amiodarone in this population, especially in young infants, hemodynamically compromised patients, and in patients receiving rapid amiodarone bolus administration.

Morbidities in the ultra-athlete and marathoner.

The cardiovascular benefits of habitual exercise are well documented. In the current era, more of the population is exceeding the recommendations for physical activity as the popularity of endurance events increases. Recent data have proposed a U-shaped relationship between exercise intensity and cardiovascular outcomes. Regular participation in endurance activities has been shown to result in structural and functional changes in the heart. This re-modelling may be the substrate for cardiac dysfunction or arrhythmias. The risk of sudden cardiac death may also be elevated; however, in most cases of sudden cardiac death, the cause can be linked to an underlying cardiac pathology where exercise acted as the trigger for a lethal arrhythmia. This article serves to review whether excessive exercise may result in harm in some athletes.

Valvar aortico-ventricular tunnel: an insight into the development of the great arteries.

Aorto-left ventricular tunnel is a rare congenital heart lesion, with an incidence of <0.1% of all CHD. We present a unique case of a valvar aorto-left ventricular tunnel in a neonate, in belief that our findings may shed some light on the developmental origins of this lesion.