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.

Bovine jugular vein valved xenograft for extracardiac total cavo-pulmonary connection: The risk of thrombosis and the potential liver protection effect.

BACKGROUND: Currently, non-valved conduits are preferred for extracardiac total cavo-pulmonary connection (TCPC). However, previous work has failed to provide objective data comparing the postoperative outcome between non-valved TCPCs and bovine jugular vein valved xenograft (BJV) TCPCs. Hence, the objective of this study is to compare the postoperative outcomes in extracardiac TCPC patients who received BJV vs synthetic non-valved conduits and evaluate the effect of BJV on liver fibrosis. METHODS: Of 206 patients who had extracardiac TCPC from 2002 to 2017 were divided into three groups. Group A (n = 66) received BJV, group B (n = 37) received PET conduits and group C (n = 103) received polytetrafluoroethylene (PTFE) tube. Study endpoints were hospital outcomes, conduits thrombosis, reinterventions, and survival. Liver stiffness and fibrosis were assessed in eight patients with BJV. RESULTS: Preoperative parameters were comparable among groups. Thrombosis was significantly lower in group C (P < .0003) but no difference between groups A and B (P = .951). Reinterventions did not differ significantly among groups (Log-rank P = .598). Hospital deaths occurred in seven patients (3.4%). There was no difference in survival between groups (Log-rank P = .221). The median liver stiffness score was 18.65 kPa and the eight patients had advanced liver fibrosis (grade F3-4) in group A. CONCLUSION: PTFE is the recommended conduit for TCPC with a lower risk of thrombosis compared to BJV and PET. BJV conduits in TCPC circuits may not protect against liver fibrosis. BJV should not be considered as an option for TCPC.

Gastrointestinal presentation of COVID-19 in a pediatric heart transplant recipient.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated coronavirus disease 2019 (COVID-19) most commonly causes a mild respiratory illness; however, there are wide ranges of presenting symptoms and disease severity. It has a mortality rate around 7%. Case presentation: We present a case of a 9-year-old female patient with hypoplastic left heart syndrome status post heart transplantation at age of 7 days. She presented to our emergency room complaining of intermittent fever, chills, fatigue, poor appetite, and diarrhea.A throat swab nucleic acid test was positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Intravenous fluids therapy was used for correction of hydration status. To the best of our knowledge this is the first reported case of non-pulmonary presentation of coronavirus disease-2019 (COVID-19) in a pediatric heart transplant recipient, which was successfully managed conservatively. Conclusions: Gastrointestinal manifestations can be the only presenting symptom in pediatric heart transplant recipients with COVID-19. Conservative treatment could be used successfully. Immunomodulatory medications that are used in heart transplant recipients may have protective value in SARS-CoV-2 infection.

Isolated right superior vena cava draining into the left atrium in a child with vein of Galen aneurysmal malformation-case report.

BACKGROUND: Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described. CASE PRESENTATION: We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully. CONCLUSIONS: The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.