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.

Are Pediatric Residents in Saudi Arabia Equipped to Provide Breastfeeding Care? A Cross-Sectional Study.

Background: There is a progressive reduction in breastfeeding rates in Saudi Arabia. Counseling and support from health-care providers are useful in overcoming barriers to continuing breastfeeding. However, medical education and residency programs often do not adequately provide breastfeeding training. Objective: To determine the knowledge, comfort level, perception, and clinical practices of pediatric residents regarding breastfeeding in Saudi Arabia and to measure the level and type of education received during their residency training. Materials and Methods: This cross-sectional study included pediatric residents from across Saudi Arabia who were registered with the Saudi Commission for Health Sciences and was conducted from February 2021 to January 2022. A validated self-reported questionnaire was used to elicit information from the respondents. Knowledge score was calculated as a percentage of correct answers. Results: A total of 253 residents completed the survey. The mean knowledge score was 58.4% ± 22.7%, which was lower than the cut-off threshold of good knowledge. Almost half of the residents (49.4%) were confident about addressing breastfeeding-related concerns. Although nearly all residents (91.7%) agreed that breastfeeding promotion is part of their role, 35% never or rarely met the mother before birth to discuss breastfeeding. Didactic teaching was the most prevalent educational tool during their training (34.3%); however, most residents preferred learning through interactive workshops (83.7%) and following lactation consultants (82.8%). Conclusion: Despite positive perceptions and confidence in providing breastfeeding care, pediatric residents in Saudi Arabia lack optimal knowledge of breastfeeding. These findings indicate the need for enhancing breastfeeding curricula in pediatric residency programs to improve breastfeeding consultation and management.

Comparison of Cardiac Auscultation Features on Four Different Simulation Mannequins Performed by Pediatric Residents.

INTRODUCTION: Cardiac murmurs are a common problem in pediatric clinical practice. Studies demonstrated low accuracy in detecting and diagnosing various cardiac murmurs at all levels of medical training. So, supplementary training methods started to evolve, including simulation for auscultation skills training. Over the years, mannequins have evolved with different types of technology. Therefore, we decided to compare cardiac auscultation accuracy among high-fidelity mannequins as the primary objective and compare the performance of various postgraduate-level residents as a secondary objective. METHOD: Pediatric residents at King Abdulaziz University Hospital were given a lecture on the basics of cardiac auscultation and then requested to auscultate four mannequins, namely SimJumior® (Laerdal Medical, Stavanger, Norway), SimBaby™ (Laerdal Medical), Pediatric HAL® (Gaumard Scientific, Miami, FL, USA), and Cardiac Patient Simulator K-Plus (Kyoto Kagaku Co. Ltd., Kyoto, Japan). The accuracies of murmur type, diagnosis, and auscultation time were compared.  Results: A total of 56 pediatric residents were enrolled. Median murmur accuracy ranged from 50% to 53% (p-value 0.79), and median diagnosis accuracy ranged from 33% to 36% (p-value 0.77), with a nonsignificant difference between mannequins. Comparing resident levels in all mannequins, median murmur accuracy ranged from 49% to 56% (p-value 0.70), and median diagnosis accuracy ranged from 29% to 41% (p-value 0.09). While the median average auscultation time was between 41 and 50 seconds (p-value 0.34). CONCLUSION: Auscultation skills can be taught through simulation on any mannequin used in this comparison, not necessarily the cardiac one. For better accuracy, future comparisons might include more advanced cardiac mannequins based on cardiac auscultation expertise (i.e., consultant level). The introduction of an auscultation program from the undergraduate level throughout the training process and monitoring of these skills are mandated.

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.

Outpatient pediatric cardiology consultations in a tertiary academic hospital.

Objectives: Cardiac diseases in the pediatric population can be congenital or acquired. If the diagnosis and treatment are early, the chance for survival increases. Thus, this study aimed to determine the indications for pediatric cardiology consultations in a single tertiary hospital in Jeddah, Saudi Arabia. Materials and Methods: This study was conducted in 2020-2021 at a tertiary center in Jeddah, Saudi Arabia. Patients younger than 14 years of age who were referred by outpatient clinics or those who presented to the emergency department and needed outpatient cardiac evaluation were included in this study. Inpatient referrals were excluded. The Statistical Package for the Social Sciences version 21 was used for statistical analyses. Results: A total of 416 referred patients were included in this study. New patients accounted for 74% of the referrals, while known patients accounted for 26%. The median age was 2.728 years, with 56.3% being male participants. The three most common reasons for referral were: evaluation of cardiac function (21.6%), follow-up evaluation of fetal/neonatal diagnosis (19.5%), and heart murmurs (16.8%). Conclusion: Most of the referrals were new patients. Of those who underwent echocardiography, 48.2% had abnormal results. We recommend further studies to help guide the direction of the residents' education and to provide better patient healthcare services.

Factors influencing blood pressure fluctuation in pediatric patients with sickle cell disease in Saudi Arabia: A retrospective single-center cohort study.

OBJECTIVES: To investigate changes in blood pressure (BP) among pediatric patients with sickle cell disease (SCD) and determine the variables that might influence these changes. METHODS: A total of 100 pediatric patients with SCD who followed up in the pediatric outpatient clinic were recruited for this retrospective cohort study. Clinical data included anthropometric measures, average systolic and diastolic BP recorded during multiple follow-up visits, hemoglobin (Hb) level, serum creatinine, and hemoglobin S percentage. Blood pressure measurements were categorized according to the guidelines of the American Academy of Pediatrics (AAP, 2017). RESULTS: In this cohort, 68% of the patients had normal systolic BP, 13% had elevated systolic BP, 17% had stage 1 hypertension (HTN), while only 2% reported stage 2 HTN. Patients who were overweight had relatively high systolic BP compared to patients who were underweight (p=0.034) or had normal weight (p=0.023). The average systolic BP significantly correlates with body mass index (r= 0.377, p<0.001) and serum creatinine (r=0.369, p<0.001). CONCLUSION: Pediatric overweight SCD patients exhibited higher average systolic BP than those underweight or normal weight. Body mass index and serum creatinine significantly influenced the average systolic BP more than the Hb level or Hb S percentage.

Reversible Cardiomyopathy, What Should the Clinicians Keep in Mind? A Case Report.

Primary carnitine deficiency (PCD) is an autosomal recessive disorder characterized by decreased carnitine levels essential for Beta oxidation in various organs, including the heart. Early diagnosis and treatment of PCD can revert cardiomyopathy. A 13-year-old girl presented with heart failure due to dilated cardiomyopathy and severe cardiac dysfunction; following L carnitine treatment, the patient's clinical conditions improved, and cardiac functions returned to normal within weeks. Investigations revealed PCD; regular L carnitine has been provided, all cardiac medications are discontinued, and the patient is doing well. We believe PCD should be ruled out in every patient with cardiomyopathy.

Clinical Presentation of Myocarditis in the Pediatric Age Group and Predictors of Poor Early and Late Outcomes: Academic Hospital Experience.

BACKGROUND:  Myocarditis is a leading cause of morbidity and mortality in the pediatric age group and contributes to a wide range of complications, including dilated cardiomyopathy, congestive heart failure, and even death, so early identification and comprehensive management are essential for a favorable outcome. OBJECTIVES:  Summarize the presenting clinical signs and symptoms of pediatric patients with a diagnosis of myocarditis and a poor outcome and correlate the clinical presentation and laboratory and radiographic findings to identify possible predictors of a poor outcome. METHODS:  This retrospective cohort study included all patients who were diagnosed with myocarditis and followed up at King Abdulaziz University Hospital, Jeddah, Saudi Arabia over the 13 years between January 01, 2007, and December 31, 2019. Information on patient demographics, clinical presentation, and non-invasive investigations was obtained. Poor outcomes were defined as death or evidence of left ventricular dysfunction on echocardiography which was evaluated at two points in time as an early outcome and a late outcome. RESULTS:  Seventeen patients (male 52.9%, female 47.1%) with a median age of 4 ± 4.31 years are included. The most frequent initial complaints were exercise or feeding intolerance, respiratory symptoms, and shortness of breath. On the other hand, hepatomegaly and respiratory distress were the most common clinical signs. All the patients were admitted to the pediatric intensive care unit but only 41% required mechanical ventilation. The presence of a murmur at presentation was significantly correlated with a poor early outcome. Ischemic changes on the electrocardiogram and moderate left ventricular dysfunction on the echocardiogram were significantly correlated with a poor late outcome. CONCLUSION:  Diagnosis of myocarditis can be established by a combination of clinical presentation and investigative tools. A murmur, ischemic changes on the electrocardiogram, and left ventricular dysfunction are important predictors of myocarditis in children.

Risk factors of early postoperative cardiac arrhythmia after pediatric cardiac surgery: A single-center experience.

OBJECTIVES: To evaluate the incidence of arrhythmia in the early postoperative period and to identify its risk factors among pediatric patients following cardiac surgery at King Abdulaziz University Hospital (KAUH), Jeddah, Saudi Arabia, between 2015-2020. METHODS: Out of 1242 patients, a total of 821 aged <18 years who underwent cardiac surgery were included in this retrospective cohort carried out in June 2021 at KAUH, Jeddah, Saudi Arabia. Information retrieved from the hospital medical records had patients' demographics, types of arrhythmias, hemodynamic stability, electrolyte disturbances, cardiopulmonary bypass (CPB), and aortic cross-clamp (AXC) durations. Univariate and multivariate logistic regression analyses were used to evaluate the possible risk factors associated with postoperative arrhythmia. RESULTS: Of the 821 patients, 140 (17.1%) developed arrhythmia postoperatively. The most common arrhythmias were junctional ectopic tachycardia (JET, 51.4%), atrioventricular block (27.1%), and supraventricular tachycardia (10%). The majority of cases occurred on the first day postoperatively (79.3%). Patients with postoperative arrhythmias had a more prolonged CPB (p=0.0001) and AXC (p=0.005) time, electrolytes disturbances (p=0.021), and hemodynamic instability (p=0.0001) than other patients. CONCLUSION: Postoperative arrhythmia, especially JET, is common after pediatric cardiac surgery. Prolonged cardiopulmonary bypass, prolonged aortic cross-clamping, electrolytes disturbances, and hemodynamic instability are possible risk factors for postoperative cardiac arrhythmias.

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.

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.

Outcome of ductus arteriosus stenting including vertical tubular and convoluted tortuous ducts with emphasis on technical considerations.

BACKGROUND: Ductal stenting is the preferred method of securing adequate pulmonary blood flow in patients with duct-dependent pulmonary circulation. The main limitation in most centers is the difficult vertical tubular or convoluted ducts that represent real challenges to interventional pediatric cardiologists. We present our experience in patent ductus arteriosus (PDA) stenting with some technical tips to overcome difficulties, especially in stenting tortuous or long tubular ducts. This study was conducted on all patients with cyanotic congenital heart disease who underwent PDA stenting between January 2011 and December 2018. RESULTS: We attempted to stent the PDA in 43 patients, with a success rate of 93% (40 patients) and only one procedural mortality. There was also one stent migration that needed to be treated with urgent surgery. Three-fourths of the patients had difficult ductal morphology and origin. One stent was used to cover the PDA in 27 patients (62.8%), two stents were used in 13 (30.2%), and three stents were used in 2 patients (4.6%). In-stent stenosis rate was 12.5% (5 patients) and the development of progressive left pulmonary artery stenosis was seen in two patients (5%). Pulmonary artery growth was adequate in all patients. CONCLUSIONS: PDA stenting is an effective method of palliation for patients with duct-dependent pulmonary circulation. It has low morbidity and mortality rates. Stenting difficult ducts have become more feasible with evolving materials and techniques.

Impact of coronavirus disease 2019 in a child who underwent ventricular septal defect device closure: a case report.

BACKGROUND: Studies about the incidence and severity of coronavirus disease 2019 (COVID-19) in children are still significantly lower than those in adults. Moreover, data on the effect of COVID-19 in children with congenital heart disease (CHD) are limited. To the best of our knowledge, this study first reported mortality in a child with CHD who acquired COVID-19. CASE SUMMARY: A 16-month-old boy presented to the emergency department due to shortness of breath, fever, cough, and poor oral intake. He tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). He required mechanical ventilation for rapidly progressing respiratory failure. The patient had a large mid-muscular ventricular septal defect (VSD) that was closed percutaneously at the age of 13 months. Moreover, we followed his hospital sequelae from admission to death. DISCUSSION: This child had multiple risk factors, including malnutrition and persistent pulmonary hypertension (PH) after late closure of the VSD. The pre-existing PH could have been aggravated by the lung condition associated with COVID-19 and the respiratory failure triggered by SARS-CoV-2 infection. The patient presented with ventricular systolic dysfunction, elevated troponin serum levels and newly developed trifascicular block, which were indicative of myocardial injury. The elevated inflammatory markers and multi-organ dysfunction seem to corroborate multisystem inflammatory syndrome in children, which was described recently among paediatric patients with COVID-19.

Novel approach for Recanalization and Limb Saving Following Acute Thrombosis of Upper Limb Arteries in Two Neonates After Congenital Heart Surgery.

Neonates with congenital heart disease are at a high risk of vascular thrombosis. Thrombosis may occur due to vascular injury, increased blood viscosity secondary to polycythemia associated with congenital cyanotic heart diseases, or stasis of blood flow associated with low cardiac output (Schmidt B & Andrew M., Pediatrics 1995; 96: 939-943. Veldman A et al.,Vasc Health Risk Manag 2008; 4: 1337-1348).

Cardiac catheterization addressing early post-operative complications in congenital heart surgery-a single-center experience.

BACKGROUND: Cardiac catheterization after congenital heart surgery may play an important role in the diagnosis and management of patients with a complicated or unusual post-operative course. The main objective of this study was to evaluate the safety, efficacy, and outcome of cardiac catheterization performed in the early post-operative period following congenital heart surgery. All patients who underwent cardiac catheterization after congenital heart surgery during the same admission of cardiac surgery from November 2015 to May 2018 were included in the study. RESULTS: Thirty procedures were performed for 27 patients (20 interventional and 10 diagnostic). The median age of the patients was 15 months (15 days to 20 years), median weight was 8.2 kg (3.4 to 53 kg), and median time from surgery was 3 days (0-32 days). Eleven procedures were performed for 11 patients on extracorporeal membrane oxygenation (ECMO) support. The main indications for catheterization included the inability to wean from ECMO (10 procedures) and cyanosis (10 procedures). Interventional procedures included angioplasty using stents (10 procedures, success rate of 90%), angioplasty using only balloons (2 procedures, success rate of 50%), and occlusion for residual shunts (8 procedures, success rate of 100%). No mortality was recorded during any procedure. Vasoactive-inotropic score had significantly decreased 48 h after catheterization when compared to pre-catheterization scores (p = 0.0001). Moreover, 72% of patients connected to ECMO support were successfully weaned from ECMO after catheterization. Procedural complications were recorded in 3 interventional procedures. Survival to hospital discharge was 55.5% and overall survival was 52%. Patients on ECMO support had a higher mortality than other patients. CONCLUSION: Cardiac catheterization can be performed safely in the early post-operative period, and it could improve the outcome of the patient (depending on the complexity of the cardiac lesions involved).

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.

Venovenous malformation: a common finding after Kawashima operation.

OBJECTIVES: It has been reported that systemic venovenous malformation (VVM) can develop in patients with interrupted inferior vena cava (IVC) and univentricular type of congenital heart disease who undergo superior vena cava to pulmonary artery connection (Kawashima operation). These malformations can lead to profound systemic desaturation postoperatively. However, there have been few reports that characterise the prevalence, anatomic details and clinical correlations of these systemic VVM arising after Kawashima operation. In this study, we describe our experience with VVM after Kawashima operation, and discuss issues regarding their evaluation and postoperative management. METHODS: Eight patients with median age 19 months (range: 5-238) who underwent Kawashima operation were subjected to postoperative angiography, prospectively. Sites of VVM origin and entry, as well as their course, were documented. The presence of pulmonary arteriovenous malformations (AVMs) was also documented. RESULTS: At median follow-up of 31 months (range: 16-72 months), a total of 14 VVM were found in different supra- and infra-diaphragmatic sites in six patients (75%); four of them had concomitant pulmonary AVM while the remaining two patients had only pulmonary AVM. CONCLUSIONS: Our findings suggest that systemic VVM can occur frequently after Kawashima operation and can produce significant desaturation postoperatively, and hence we support hepatic incorporation. Performing detailed angiographic studies of the supra- and infra-diaphragmatic systemic veins in routine assessment of patients before Kawashima operation is, probably, warranted.