Simultaneous repair of diaphragmatic hernia and ventricular septal defect with postoperative complication in a Down syndrome child.

Morgagni hernia (MH) is a rare form of congenital diaphragmatic hernia, typically occurring predominantly on the right side and exhibiting a higher prevalence in females. Usually diagnosed incidentally, MH may coexist with congenital heart defects, chest wall abnormalities and certain genetic syndromes such as Down syndrome. A 4-year-old boy with Down syndrome underwent simultaneous repair of MH and closure of a ventricular septal defect (VSD). A vertical midline sternotomy was performed, and the VSD was repaired using the right atrium approach. Subsequently, MH repair was conducted. Three weeks after the surgery, this patient developed a complete heart block, which lead to the implantation of a VVI pacemaker.

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.

Custodiol versus blood cardioplegia in pediatric cardiac surgery: a randomized controlled trial.

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.

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 affecting the outcomes after bidirectional Glenn shunt: two decades of experience from a tertiary referral center.

BACKGROUND: Despite the improved management of patients with a single ventricle, the long-term outcomes are not optimal. We reported the outcomes of the bidirectional Glenn procedure (BDG) and factors affecting the length of hospital stay, operative mortality, and Nakata index before Fontan completion. RESULTS: This retrospective study included 259 patients who underwent BDG shunt from 2002 to 2020. The primary study outcomes were operative mortality, duration of hospital stay, and Nakata index before Fontan. Mortality occurred in 10 patients after BDG shunt (3.86%). By univariable logistic regression analysis, postoperative mortality after BDG shunt was associated with high preoperative mean pulmonary artery pressure (OR: 1.06 (95% CI 1.01-1.23); P = 0.02). The median duration of hospital stay after BDG shunt was 12 (9-19) days. Multivariable analysis indicated that Norwood palliation before BDG shunt was significantly associated with prolonged hospital stay (ß: 0.53 (95% CI 0.12-0.95), P = 0.01). Fontan completion was performed in 144 patients (50.03%), and the pre-Fontan Nataka index was 173 (130.92-225.34) mm2/m2. Norwood palliation (ß: - 0.61 (95% CI 62.63-20.18), P = 0.003) and preoperative saturation (ß: - 2.38 (95% CI - 4.49-0.26), P = 0.03) were inversely associated with pre-Fontan Nakata index in patients who had Fontan completion. CONCLUSIONS: BDG had a low mortality rate. Pulmonary artery pressure, Norwood palliation, cardiopulmonary bypass time, and pre-BDG shunt saturation were key factors associated with post-BDG outcomes in our series.

Congenital giant left ventricular aneurysm.

We report a case of congenital giant left ventricular aneurysm with severely depressed systolic cardiac function who underwent early surgical resection with subsequent recovery of left ventricular systolic function.

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.

Combined Norwood and cavopulmonary shunt as the first palliation in late presenters with hypoplastic left heart syndrome and single-ventricle lesions.

OBJECTIVE: A primary cavopulmonary shunt as a component of the initial Norwood palliation could be an option in patients with hypoplastic left heart syndrome and single-ventricle lesions. We present our initial experience with this approach in carefully selected patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance. METHODS: The study included 16 patients; the mean age was 137.9 ± 84.2 days. All patients underwent a Norwood palliation consisting of atrial septectomy, Damus-Kaye-Stansel connection, and arch augmentation in addition to the cavopulmonary shunt as the initial palliation. RESULTS: The mean preoperative pulmonary to systemic blood flow (Qp/Qs) ratio on room air (n = 9) and with 100% oxygen (n = 8) was 5.3 ± 3.2 and 8.6 ± 4.3, respectively. The mean pulmonary vascular resistance on room air (n = 10) and 100% oxygen (n = 9) was 4.8 ± 3.1 and 1.7 ± 0.97 WU/m2, respectively. Delayed chest closure was needed in 12 patients, and 6 patients required postoperative inhaled nitric oxide. One patient underwent takedown of the cavopulmonary shunt and construction of the right ventricle to pulmonary artery conduit after 1 month. The mean intensive care unit stay was 18.9 ± 15.4 days. There were 2 in-hospital deaths (48 hours and 8 days after surgery) and 2 postdischarge deaths (6 months and 2 years after hospital discharge). Seven patients have undergone the Fontan completion successfully, and 5 patients await further surgery. CONCLUSIONS: First-stage Norwood palliation with cavopulmonary shunt for patients with hypoplastic left heart syndrome or single-ventricle lesions is feasible in late presenters with low pulmonary vascular resistance.

Outcomes of biventricular repair for shone's complex.

BACKGROUND: Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival. METHODS: This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded. RESULTS: Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01. CONCLUSION: Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.

Outcomes of Primary Bidirectional Glenn in Children with Single Ventricle Physiology and Increased Pulmonary Blood Flow.

BACKGROUND: We reported our experience in managing patients with single ventricle (SV) physiology and increased pulmonary blood flow (PBF), aiming to assess if it is feasible to proceed with primary Bidirectional Glenn (BDG) without a prior operation to limit PBF. MATERIALS AND METHODS: This is a retrospective study with 51 consecutive patients who underwent BDG operation as a primary operation or a second stage prior to the definitive Fontan operation at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia between 2010 and 2018. Patients were categorized into two groups based on their PBF prior to the operation: Patients who had SV physiology and increased PBF (seven patients) vs. patients with SV physiology and restricted PBF (44 patients). RESULTS: The median age for the increased PBF group was 9.9 months [interquartile range (IQR): 2-16.9 months], and the median age for the restricted PBF group was 15.3 months (IQR: 6.7-42.6 months). Although the length of hospital stay was longer in patients with increased PBF (P = 0.039), we couldn't find a statistically significant difference in early mortality, duration of mechanical ventilation, length of pleural drainage, and length of intensive care unit (ICU) stay between the groups. CONCLUSION: In our experience, we found that primary BDG could be done safely for patients having SV physiology and increased PBF with acceptable short-term outcomes. It might further reduce the morbidity and mortality for those patients by avoiding the risk of initial pulmonary artery banding or aortopulmonary shunts.

The outcomes of bidirectional Glenn before and after 4 months of age: A comparative study.

OBJECTIVE: We aim to present our experience with the bidirectional Glenn (BDG) in patients less than 4 months of age and to compare their outcomes with the patients who underwent BDG after the age of 4 months. METHODS: A retrospective review of data was performed for patients who underwent the BDG procedure from 2002 to 2018 at our institutions. We reviewed the patients' demographics, echocardiographic findings, cardiac catheterization data, operative details, postoperative data, and outcome variables. RESULTS: The study was conducted on 213 patients. At the time of the BDG operation, 32 patients were younger than 4 months (younger group) and 181 patients were older than 4 months (older group). The preoperative mean pulmonary artery pressure was significantly higher in the younger group (p = .035) but there were no significant differences between both groups in Qp/Qs, ventricular end-diastolic pressure, indexed pulmonary vascular resistance, and preoperative oxygen saturation. However, the initial postoperative oxygen saturation of the younger group was lower than the older group (p = .007). The duration of mechanical ventilation, duration of pleural drainage, ICU stay, and hospital stay after BDG were significantly longer in the younger group compared to the older group. The early mortality was higher in the younger group, but this difference did not reach statistical significance (p = .283). CONCLUSION: Performing BDG procedure in infants less than 4 months of age is safe, with favorable outcomes. Early BDG is associated with a less-smooth postoperative course without a significant increase in early or late mortality.

A Large Stomach Ulcer Is Associated With Raised Mortality in a Cohort of Patients Who Underwent Open Repair of Perforated Peptic Ulcer: A Five-Year Follow-Up Study.

Introduction Perforated peptic ulcer disease (PPUD) is associated with a high postoperative mortality and morbidity rates especially within the first 90 days. The size and site of the ulcer may contribute to the prognosis of PPUD. In this study, we will describe the association of size and site of PPUD with the overall mortality and in-hospital morbidities in a tertiary care university hospital. Methods A retrospective observational cohort study was conducted at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. A total of 50 patients who had PPUD and underwent open exploratory laparotomy with surgical treatment were analyzed. Patients were divided into two groups: a small ulcer group when the ulcer diameter was less than equal to 1 cm and a large ulcer group when it was more than 1 cm. For the subgroup analysis, patients were categorized according to site into small duodenum, large duodenum, small stomach, and large stomach PPUD. The primary outcome was overall mortality that was measured by survival analysis and Cox regression. Secondary outcomes were intensive care unit (ICU) admission, ICU and hospital length of stay, and in-hospital mortality, which were assessed by stepwise logistics and linear regression. Results Overall mortality at 10, 30, and 90 days was 14% (95% CI: 0.06-0.27), 24% (95% CI: 0.14-0.39), and 34% (95% CI: 0.23-0.49), respectively. Saudi patients had a 72% decreased risk of overall mortality compared to non-Saudi patients (P=0.03) over the follow-up period. Overall, patients who had stomach PPUD had a 2.23-fold increased risk of overall mortality over time compared to those who had duodenum PPUD (P=0.10). Large PPUD, >1 cm, had a 3.20-fold increased risk of overall mortality over time compared to small PPUD (P=0.04). Large stomach PPUD had a 4.22-fold increased risk of overall mortality over time compared to other ulcers (P=0.01). Conclusions Large stomach PPUD is associated with increased overall mortality and morbidity. These findings indicate that patients who have a large stomach PPUD might need careful perioperative and postoperative personalized surgical plans as these patients may eventually undergo complicated surgical procedures.

Iatrogenic Coronary Artery Compromise Post Non-Coronary Cardiac Surgery in Patients With Normal Coronaries.

BACKGROUND: Unexpected events in cardiac surgery may increase morbidity and mortality. We present rare complications related to coronary arteries in non-coronary cardiac surgery in adults and pediatrics. PATIENTS AND METHODS: We retrospectively reviewed our surgical left-sided valve procedures and aortic root reconstruction for patients with documented coronary ostial injury or left circumflex artery (LCX) between January 2012 and December 2019. Preoperative echocardiography was the standard investigation for all cases and other specific work ups were ordered, according to each case. Management by surgical or non-surgical intervention was planned, according to each complication. Postoperative hemodynamics and mortality rate were the outcomes of interest. RESULTS: Seven patients were found to have coronary artery compromise post left-sided valve procedures and aortic root reconstruction in adults and children. The details are shown in Table 1. The complications were in 2 patients post-mitral valve (MV) repair, 3 patients post-aortic (AV) replacement, 2 pediatric patients, 1 post-aortic homograft, and the other post-repair of anomalous single coronary arising from the pulmonary artery (ASCAPA). Six patients were hemodynamically unstable. Five patients had intraoperative ischemic changes on electrocardiogram and echocardiography, while ventricular arrhythmias were documented in 3 patients. Two patients were treated with percutaneous coronary intervention to LCX and right coronary artery (RCA), while 4 patients required immediate surgery to graft the obtuse marginal branch of the LCX artery (1) and RCA (3). No revision to valvular procedure was done. With the exception of one, all patients survived. CONCLUSION: A high index of suspicion is required to diagnose and rescue coronary complications post-valvular surgery and aortic root reconstruction.

Surgical repair for persistent truncus arteriosus in neonates and older children.

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 < 0.001) had a longer hospital stay. Surgical re-intervention was required in 8 patients (22.86%), and 11 patients (30.56%) had catheter-based reintervention. Freedom from reintervention was 86% at 1 year, 75% at 2 years and 65% at 3 years. Survival at 1 year was 81% and at 3 years was 76%. High postoperative inotropic score predicted mortality (p = 0.013). CONCLUSION: Repair of the truncus arteriosus can be performed safely with low morbidity and mortality, both in neonates, infants, and older children. Re-intervention is common, preferably through a transcatheter approach.

Arterial-level repair of transposition of great arteries without coronary artery transfer.

Hypoplastic ascending aorta and interrupted aortic arch (IAA) are rarely associated with dextro-transposition of the great arteries (D-TGA). Severe hypoplastic ascending aorta may preclude coronary artery transfer making arterial switch operation problematic. We report a case of D-TGA with a large subpulmonic ventricular septal defect, IAA, and hypoplastic ascending aorta that underwent successful biventricular surgical repair without coronary artery transfer.

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.

Evolution of the Norwood operation outcomes in patients with late presentation.

OBJECTIVES: We present the evolution of Norwood operation outcomes and practice pattern changes over 15 years from a single institution in Saudi Arabia. We intended to identify time trends in patient selection, procedural details, and outcome predictors over time. METHODS: Patients who underwent a Norwood operation (n = 145) between 2003 and 2018 with the use of a Blalock-Taussig shunt (BT group; n = 72), right ventricle to pulmonary artery shunt (Sano group; n = 66), or a primary cavopulmonary shunt (CPS group; n = 7) were included. The study outcomes were operative mortality, long-term survival, and multistate transition to CPS, Fontan, and death. RESULTS: Median age was 29 days. Predictors of operative mortality were lower weight (P = .026), and longer bypass time (P = .014), whereas age, and type of shunt were not. Predictors of improved long-term survival were greater weight at operation (P = .0016), later era (P = .006), and shorter bypass time (P = .001). The multistate model revealed that patients with lower weight were more likely to undergo Sano versus BT (P < .001), and if BT was chosen in such patients, they were more likely to die (P = .027). The likelihood of receiving Sano shunt was 3-fold greater in the recent era (P = .003). CONCLUSIONS: Improved outcomes of the Norwood operation are evident in the recent era and with Sano shunt, especially in patients of smaller weight. Late presentation or older age is not a contraindication to Norwood operation. The incorporation of a primary CPS at stage one operation is feasible in selected patients.