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Abstract Background Factors affecting the outcomes after Fontan palliation are still controversial. Thus, this study aimed to compare hospital and long-term outcomes after Fontan palliation in patients with left-dominant, right-dominant, and co-dominant ventricles. Moreover, the study sought to identify factors associated with long-term surgical reintervention, catheter-based interventions, and morality. This retrospective cohort study was conducted in a single tertiary referral center between 2010 and 2024 and included all patients who had Fontan palliation (n = 247). The patients were grouped according to the dominant ventricle into three groups: left-dominant (n = 105), right-dominant (n = 108), and codominant (n = 36). Results No significant differences were found in demographic variables among groups. Heterotaxy was more common in codominant patients who exhibited higher preoperative saturation levels. Early postoperative complications were more frequent in right-dominant patients, with low cardiac output significantly lower in left-dominant patients. Codominant patients experienced more arrhythmias and complete heart block, while operative mortality rates were similar across groups. During a median follow-up of 80 months, the freedom from surgical reintervention at 5, 10, and 15 years was highest in the left-dominant group (97%) compared to right dominance (93%, 78%) and codominance (97%, 87%). Mortality was significantly higher in the right-dominant and codominant groups, with survival rates at 5, 10, and 15 years being 97% for left dominance, 92% for right dominance, and 91% for codominance. Factors influencing long-term mortality were older age, right dominance, and elevated pulmonary artery pressure. Conclusions Patients with left dominance exhibited superior survival rates and fewer complications compared to those with right dominance and codominance. The findings emphasize the importance of anatomical considerations in risk stratification and clinical decision-making. Young age at the time of palliation could improve the outcomes of the Fontan procedure.

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

Background: Factors affecting the outcomes after Fontan palliation are still controversial. Thus, this study aimed to compare hospital and long-term outcomes after Fontan palliation in patients with left-dominant, right-dominant, and co-dominant ventricles. Moreover, the study sought to identify factors associated with long-term surgical reintervention, catheter-based interventions, and morality. This retrospective cohort study was conducted in a single tertiary referral center between 2010 and 2024 and included all patients who had Fontan palliation (n = 247). The patients were grouped according to the dominant ventricle into three groups: left-dominant (n = 105), right-dominant (n = 108), and codominant (n = 36). Results: No significant differences were found in demographic variables among groups. Heterotaxy was more common in codominant patients who exhibited higher preoperative saturation levels. Early postoperative complications were more frequent in right-dominant patients, with low cardiac output significantly lower in left-dominant patients. Codominant patients experienced more arrhythmias and complete heart block, while operative mortality rates were similar across groups. During a median follow-up of 80 months, the freedom from surgical reintervention at 5, 10, and 15 years was highest in the left-dominant group (97%) compared to right dominance (93%, 78%) and codominance (97%, 87%). Mortality was significantly higher in the right-dominant and codominant groups, with survival rates at 5, 10, and 15 years being 97% for left dominance, 92% for right dominance, and 91% for codominance. Factors influencing long-term mortality were older age, right dominance, and elevated pulmonary artery pressure. Conclusions: Patients with left dominance exhibited superior survival rates and fewer complications compared to those with right dominance and codominance. The findings emphasize the importance of anatomical considerations in risk stratification and clinical decision-making. Young age at the time of palliation could improve the outcomes of the Fontan procedure. [ABSTRACT FROM AUTHOR]

Introduction: The evidence about the optimal revascularization strategy in patients with left main coronary artery (LMCA) disease and impaired renal function is limited. Thus, we aimed to compare the outcomes of LMCA disease revascularization (percutaneous coronary intervention [PCI] vs. coronary artery bypass grafting [CABG]) in patients with and without impaired renal function. Methods: This retrospective cohort study included 2,138 patients recruited from 14 centers between 2015 and 2,019. We compared patients with impaired renal function who had PCI (n= 316) to those who had CABG (n = 121) and compared patients with normal renal function who had PCI (n = 906) to those who had CABG (n = 795). The study outcomes were in-hospital and follow-up major adverse cardiovascular and cerebrovascular events (MACCE). Results: Multivariable logistic regression analysis showed that the risk of in-hospital MACCE was significantly higher in CABG compared to PCI in patients with impaired renal function (odds ratio [OR]: 8.13 [95% CI: 4.19–15.76], p < 0.001) and normal renal function (OR: 2.59 [95% CI: 1.79–3.73]; p < 0.001). There were no differences in follow-up MACCE between CABG and PCI in patients with impaired renal function (HR: 1.14 [95% CI: 0.71–1.81], p = 0.585) and normal renal function (HR: 1.12 [0.90–1.39], p = 0.312). Conclusions: PCI could have an advantage over CABG in revascularization of LMCA disease in patients with impaired renal function regarding in-hospital MACCE. The follow-up MACCE was comparable between PCI and CABG in patients with impaired and normal renal function.

Abstract Background Surgical closure of multiple ventricular septal defects (VSDs) is challenging and associated with a high complication rate. Several factors may affect the outcomes after surgical repair of multiple VSDs. We aimed to report the outcomes after surgical repair of multiple VSDs before and after 1 year and identify the factors affecting the outcomes. We have studied forty-eight patients between 2016 and 2017 who had surgical repair of multiple VSDs. We grouped them according to the age at the time of repair. Study outcomes were hospital complications, prolonged hospital stay, and reoperation. Results There were 18 females (60%) in group 1 and 13 (72.22%) in group 2 (P = 0.39). There were no differences in the operative outcomes between the groups. Prolonged postoperative stay was associated with group 1 (OR 0.23 (0.055–0.96); P = 0.04) and lower body weight (OR 0.76 (0.59–0.97); P = 0.03). Hospital mortality occurred in 2 patients (6.67%) in group 1 and 1 patient (5.56%) in group 2 (P > 0.99). Five patients had reoperations: two for residual VSDs, two for subaortic membrane resection, and one for epicardial pacemaker implantation. All reoperations occurred in group 1 (log-rank P = 0.08). Two patients had transcatheter closure of the residual muscular VSDs; both were in group 2. Conclusions Surgical repair of multiple VSDs was associated with good hospital outcomes. The outcomes were comparable in patients younger or older than 1 year of age. Young age at repair could lead to prolonged postoperative stay and a higher reoperation rate.

Abstract Data on mitral valve replacement (MVR) in young children is still limited. Our objective was to evaluate MVR in children below 5 years and identify factors affecting the outcomes. This retrospective study included 29 patients who had MVR from 2002 to 2020. We grouped the patients into two groups according to their age: age ≤ 24 months (n = 18) and > 24 months (n = 11). Primary cardiac diagnoses were Shone complex (n = 7; 24%), isolated congenital mitral valve abnormality (n = 11; 38%), and complete atrioventricular septal defect (n = 3; 10%). The median age was 19 month (25th–75th percentile: 11–32) and 59% were females (n = 17). The hemodynamic lesions were mitral regurgitation in 66%, mitral stenosis in 10%, and combined mitral stenosis and regurgitation in 24% of the patients. St. Jude mitral valve was the most common valve implanted (n = 19, 66%), followed by CarboMedics in 21% of the patients (n = 6). The mitral valve was implanted in the supra-annular position in 6 cases (21%). Preoperative and operative data were comparable between both groups. There was no association between valve size and position with postoperative heart block (P > 0.99, for both). The median follow-up duration was 19.4 months (8.6–102.5). Nine patients had mitral valve reoperation, six had MVR, and three had clot removal from the mitral valve. There was no effect for age group on reoperation (SHR 0.89 (95% CI 0.27–2.87), P = 0.84). Valve size significantly affected reoperation (SHR 0.39 (95% CI 0.18–0.87), P = 0.02). The supra-annular position was associated with an increased risk of reoperation (SHR 3.1 (95% CI 1.003–9.4), P = 0.049). There was no difference in survival according to the age (Log-rank P = 0.57) or valve size (Log-rank P = 0.66). Mitral valve replacement in children is associated with low morbidity and mortality. The risk of reoperation could be affected by the valve size and position rather than the age.

Abstract 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.

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 Surgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ventricle, and heterotaxy syndromes are thought to influence short- and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting. Results Between 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson’s test, Wilcoxson’s test, and generalized linear regression with Poisson distribution were used. Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein stenosis occurred in three patients, and reoperation was done in one patient. Survival free from reoperation was 98%, 98%, and 90% at 1, 24, and 60 months in the absence of pulmonary vein stenosis. However, all three patients who developed pulmonary vein stenosis died, at 0.5, 2.7, and 6.3 months of follow-up, respectively. We were unable to detect a significant effect of anatomical type, preoperative obstruction, associated single ventricle, or heterodoxy syndrome on operative mortality or long-term freedom from death or reoperation. Patients who presented with obstruction and infracardiac or supracardiac TAPVD had longer hospital stay. Conclusion TAPVD repair outcomes are excellent except for patients who develop postoperative pulmonary venous stenosis. Anatomical type, obstructed presentation, associated single ventricle, or heterotaxy are not significant predictors of survival.

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 Postoperative pain has negative consequences on patients’ outcomes after cardiac surgery. Routine management with opioid and or non-steroidal anti-inflammatory medications has several disadvantages. Dexmedetomidine is a selective α2 agonist used for sedation and analgesia. The use of dexmedetomidine for postoperative pain management and decreasing delirium and agitation in cardiac surgery patients is a matter of debate. Our objective was to determine the role of an early administration of dexmedetomidine in decreasing opioid use post-cardiac surgery and its effects on the quality of postoperative recovery. Results Medical records of 120 patients admitted to the cardiac surgery intensive care unit (CSICU) after coronary artery bypass grafting (CABG) in two cardiac centers between December 2015 and December 2016 were reviewed. Patients were divided into two groups. Group A included 55 patients who received dexmedetomidine in a dose of 0.2–0.4 mcg/kg/h on admission to CSICU, and group B included 65 patients who did not receive dexmedetomidine. The primary outcome was the pain score immediately after extubation, and the secondary outcomes included post-extubation sedation and pain scores for 12 h. There were significant decrease of the pain scores in dexmedetomidine group that continues through the 3rd, 6th, 8th, and 12th hour readings after surgery with mean modified Ramsay scores 0.1 ± 0.0, 0.89 ± 2.05, 0.35 ± 0.1, and 0.12 ± 1.1 respectively compared to 0.46 ± 1.15, 3.46 ± 2.93, 0.98 ± 1.90, and 0.12 ± 1.1 in group B (p

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.

Objective Surgical site infection (SSI) is a serious complication after coronary artery bypass grafting (CABG). This study was performed to evaluate evidence-based practice and structured problem-solving to reduce SSI after CABG. Methods An infection control strategy including supervised chlorhexidine gluconate (CHG) showers was implemented from January 2017 to March 2018 for 119 patients undergoing CABG. The controls comprised 244 patients who underwent CABG from 2014 to 2016. Risk factors for SSI were identified, and a problem-focused strategy was used to control SSI. Propensity score matching was used to study the effect of CHG showers on SSI. Results SSI occurred in 25 patients (10.25%) in the control group, and the significant risk factors were the postoperative blood glucose level, transfer from an outside hospital, emergency operation, redo sternotomy, a higher American Society of Anesthesiologists score, and the duration of surgery. After implementation of the program, the SSI rate significantly decreased to 3.36%. Patients who had undergone preoperative CHG showers had a significantly lower SSI rate (1.69%) than the matched controls (13.56%). Conclusion SSI after CABG can be reduced using evidence-based practice and structured problem-solving to identify risk factors. A preoperative CHG shower is associated with a lower SSI rate after CABG.

Currently, gender is not considered in the choice of the revascularization strategy for patients with unprotected left main coronary artery (ULMCA) disease. This study analyzed the effect of gender on the outcomes of percutaneous coronary intervention (PCI) vs coronary artery bypass grafting (CABG) in patients with ULMCA disease. Females who had PCI (n = 328) were compared with females who had CABG (n = 132) and PCI in males (n = 894) was compared with CABG (n = 784). Females with CABG had higher overall hospital mortality and major adverse cardiovascular events (MACE) than females with PCI. Male patients with CABG had higher MACE; however, mortality did not differ between males with CABG vs PCI. In female patients, follow-up mortality was significantly higher in CABG patients, and target lesion revascularization was higher in patients with PCI. Male patients had no difference in mortality and MACE between groups; however, MI was higher with CABG, and congestive heart failure was higher with PCI. In conclusion, women with ULMCA disease treated with PCI could have better survival with lower MACE compared with CABG. These differences were not evident in males treated with either CABG or PCI. PCI could be the preferred revascularization strategy in women with ULMCA disease. [ABSTRACT FROM AUTHOR]