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Abstract 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.

Abstract Background The optimal revascularization strategy in patients with left main coronary artery (LMCA) disease in the emergency setting is still controversial. Thus, we aimed to compare the outcomes of percutaneous coronary interventions (PCI) vs. coronary artery bypass grafting (CABG) in patients with and without emergent LMCA disease. Methods This retrospective cohort study included 2138 patients recruited from 14 centers between 2015 and 2019. We compared patients with emergent LMCA revascularization who underwent PCI (n = 264) to patients who underwent CABG (n = 196) and patients with non-emergent LMCA revascularization with PCI (n = 958) to those who underwent CABG (n = 720). The study outcomes were in-hospital and follow-up all-cause mortality and major adverse cardiovascular and cerebrovascular events (MACCE). Results Emergency PCI patients were older and had a significantly higher prevalence of chronic kidney disease, lower ejection fraction, and higher EuroSCORE than CABG patients. CABG patients had significantly higher SYNTAX scores, multivessel disease, and ostial lesions. In patients presenting with arrest, PCI had significantly lower MACCE (P = 0.017) and in-hospital mortality (P = 0.016) than CABG. In non-emergent revascularization, PCI was associated with lower MACCE in patients with low (P = 0.015) and intermediate (P

Abstract Background Blood-based cardioplegia is the standard myocardial protection strategy in pediatric cardiac surgery. Custadiol (histidine-tryptophan-ketoglutarate), an alternative, may have some advantages but is potentially less effective at myocardial protection. This study aimed to test whether custadiol is not inferior to blood-based cardioplegia in pediatric cardiac surgery. Methods The study was designed as a randomized controlled trial with a blinded outcome assessment. All pediatric patients undergoing cardiac surgery with cardiopulmonary bypass and cardioplegia, including neonates, were eligible. Emergency surgery was excluded. The primary outcome was a composite of death within 30 days, an ICU stay longer than 5 days, or arrhythmia requiring intervention. Secondary endpoints included total hospital stay, inotropic score, cardiac troponin levels, ventricular function, and extended survival postdischarge. The sample size was determined a priori for a noninferiority design with an expected primary outcome of 40% and a clinical significance difference of 20%. Results Between January 2018 and January 2021, 226 patients, divided into the Custodiol cardioplegia (CC) group (n = 107) and the blood cardioplegia (BC) group (n = 119), completed the study protocol. There was no difference in the composite endpoint between the CC and BC groups, 65 (60.75%) vs. 71 (59.66%), respectively (P = 0.87). The total length of stay in the hospital was 14 (Q2–Q3: 10–19) days in the CC group vs. 13 (10–21) days in the BC group (P = 0.85). The inotropic score was not significantly different between the CC and BC groups, 5 (2.6–7.45) vs. 5 (2.6–7.5), respectively (P = 0.82). The cardiac troponin level and ventricular function did not differ significantly between the two groups (P = 0.34 and P = 0.85, respectively). The median duration of follow-up was 32.75 (Q2–Q3: 18.73–41.53) months, and there was no difference in survival between the two groups (log-rank P = 0.55). Conclusions Custodial cardioplegia is not inferior to blood cardioplegia for myocardial protection in pediatric patients. Trial registration The trial was registered in Clinicaltrials.gov, and the ClinicalTrials.gov Identifier number is NCT03082716 Date: 17/03/2017

Abstract Background Intra-cardiac masses are rare and challenging lesions with an overall incidence ranging of 0.02–0.2%. Minimally invasive approaches have been recently introduced for surgical resection of these lesions. Here, we evaluated our early experience using minimally invasive techniques in addressing intra-cardiac lesions. Methodology This is a retrospective descriptive study conducted between April 2018 to December 2020. All patients were diagnosed with cardiac tumors and treated via a right mini-thoracotomy with cardiopulmonary bypass through femoral cannulation at King Faisal Specialist Hospital and Research Centre, Jeddah. Results Myxoma was the most common pathology representing 46% of cases followed by thrombus (27%), leiomyoma (9%), lipoma (9%) and angiosarcoma (9%). All tumors were resected with negative margins. One patient was converted to open sternotomy. Tumor locations were in the right atrium, left atrium, and left ventricle in 5, 3, and 3 patients, respectively. The median ICU stay was 1.33 days. The median length of hospitalization was 5.7 days. There was no 30-days hospital mortality recorded in this cohort. Conclusion Our early experience shows that minimally invasive resection can be performed safely and effectively for intra-cardiac masses. The minimally invasive approach using a mini-thoracotomy with percutaneous femoral cannulation can be an effective alternative in resecting intra-cardiac masses that achieves clear margin resection, quick post-operative recovery, and low rates of recurrence for benign lesions.

Abstract Objectives Persistent truncus arteriosus represents less than 3% of all congenital heart defects. We aim to analyze mid-term outcomes after primary Truncus arteriosus repair at different ages and to identify the risk factors contributing to mortality and the need for intervention after surgical repair. Methods This retrospective cohort study included 36 children, underwent repair of Truncus arteriosus in the period from January 2011 to December 2018 in two institutions. We recorded the clinical and echocardiographic data for the patients preoperatively, early postoperative, 6 months postoperative, then every year until their last documented follow-up appointment. Results Thirty-six patients had truncus arteriosus repair during the study period. Thirty-one patients had open sternum post-repair, and two patients required extracorporeal membrane oxygenation. Bleeding occurred in 15 patients (41.67%), and operative mortality occurred in 5 patients (14.7%). Patients with truncus arteriosus type 2 (p = 0.008) and 3 (p = 0.001) and who were ventilated preoperatively (p

Abstract Background Rosai-Dorfman disease is a histiocytic lesion that affects lung rarely. Case presentation We present a 52-year-old female diagnosed with right intrabronchial mass invading the bronchial wall and the extrabronchial tissues with lymphadenopathy. Multiple bronchoscopic biopsies were not diagnostic. Pneumonectomy was performed and postoperative histology revealed marked mucin impaction and bronchial dilatation. The pulmonary tissue showed areas of hemorrhage and chronic inflammation. The mass exhibited an excessive number of lymphocytes, plasma cells, and numerous histiocytes engulfing them (lymphocytophagocytosis). These histiocytes were S100 protein and CD68 positive. These features are consistent with Rosai-Dorfman disease. Conclusion Rosai-Dorfman Disease with pulmonary affection can be misdiagnosed as malignancy. Careful histological examination of the specimen for emperipolesis or lymphocytophagocytosis together with S100 protein and CD68 positivity are the clue for proper diagnosis.

Abstract Background Junctional ectopic tachycardia is a serious arrhythmia that frequently occurs after tetralogy of Fallot repair. Arrhythmia prophylaxis is not feasible for all pediatric cardiac surgery patients and identification of high risk patients is required. The objectives of this study were to characterize patients with JET, identify its predictors and subsequent complications and the effect of various treatment strategies on the outcomes in selected TOF patients undergoing total repair before 2 years of age. Methods From 2003 to 2017, 609 patients had Tetralogy of Fallot repair, 322 were included in our study. We excluded patients above 2 years and patients with preoperative arrhythmia. 29.8% of the patients (n = 96) had postoperative JET. Results JET patients were younger and had higher preoperative heart rate. Independent predictors of JET were younger age, higher preoperative heart rate, cyanotic spells, non-use of B-blockers and low Mg and Ca (p = 0.011, 0.018, 0.024, 0.001, 0.004 and 0.001; respectively). JET didn’t affect the duration of mechanical ventilation nor hospital stay (p = 0.12 and 0.2 respectively) but prolonged the ICU stay (p = 0.011). JET resolved in 39.5% (n = 38) of patients responding to conventional measures. Amiodarone was used in 31.25% (n = 30) of patients and its use was associated with longer ICU stay (p = 0.017). Ventricular pacing was required in 4 patients (5.2%). Median duration of JET was 30.5 h and 5 patients had recurrent JET episode. Timing of JET onset didn’t affect ICU (p = 0.43) or hospital stay (p = 0.14) however, long duration of JET increased ICU and hospital stay (p = 0.02 and 0.009; respectively). Conclusion JET increases ICU stay after TOF repair. Preoperative B-blockers significantly reduced JET. Patients with preoperative risk factors could benefit from preoperative arrhythmia prophylaxis and aggressive management of postoperative electrolyte disturbance is essential.

Abstract 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.

Abstract Background Right ventricular (RV) volume overload increases morbidity and mortality after tetralogy of Fallot (TOF) repair. Surgical strategies like pulmonary leaflets sparing and tricuspid valve repair at time of primary repair may decrease RV overload. Our objective is to evaluate early and midterm results of pulmonary leaflets sparing with infundibular preservation and tricuspid valve repair in selected TOF patients with moderate pulmonary annular hypoplasia. Methods From 2011 to 2016; 46 patients with TOF and moderate pulmonary annular hypoplasia had surgical repair with sparing of the pulmonary valve leaflets. Concomitant tricuspid valve repair was performed in 33 patients (71.8%). Mean age was 13.1 ± 4.8 months, 68% were males (n = 31) and mean weight was 9.5 ± 2.3 kg. Preoperative McGoon ratio was 1.9 ± 0.4 and pulmonary valve z-score ranges from − 2 to − 3. Preoperative pressure gradient of RVOT was 80.9 ± 7.7 mmHg and 10.9% had minor coronary anomalies (n = 5). Results All repairs were performed through trans-atrial trans-pulmonary approach. 87% had pulmonary valve commissurotomy (n = 40). Mean cardiopulmonary bypass time was 71 ± 6.3 min and ischemic time 42.4 ± 4.9 min. Hospital mortality occurred in 4.3% (n = 2). Mean RVOT pressure gradient decreased significantly postoperatively (28.8 ± 7.2 mmHg, p-value< .001) and at the last follow up (23.6 ± 1.8 mmHg, p-value< .001). Pulmonary regurgitation progressed by one grade in 2 patients compared to the postoperative grade. 1 patient (2.5%) had late mortality and reintervention was required in 5 patients (12.5%). Conclusion Pulmonary leaflets sparing, and tricuspid valve repair are safe for TOF repair with no added morbidity or mortality. These procedures could contribute to reducing right ventricular volume overload over time after TOF repair.