The Outcomes of Cardiac Surgery in Children With DiGeorge Syndrome in a Single Center Experience: A Retrospective Cohort Study.

Background DiGeorge syndrome, a common genetic microdeletion syndrome, is associated with multiple congenital anomalies, including congenital cardiac diseases. This study aims to identify the short and midterm outcomes of cardiac surgery performed on children with DiGeorge syndrome. Methods A retrospective cohort study was conducted between the period of 2018-2022, which included children divided into two groups with a 1:2 ratio. Group one included DiGeorge syndrome patients who were diagnosed using fluorescence in situ hybridization (FISH). Group two included the control group of patients who were clear of genetic syndromes. The two groups were matched based on similar cardiac surgery, age of surgery, and Risk Adjustment in Congenital Heart Surgery (RACHS-1) score. The two groups were compared based on the demographical data and postoperative complications. Results The study consisted of 81 children; 27 were DiGeorge syndrome patients, and 54 were in the control group. DiGeorge syndrome patients showed an increase in mechanical ventilation duration (p=0.0047), intensive care unit (ICU) length of stay (p=0.0012), and hospital length of stay (p=0.0391). Moreover, they showed an increased risk for bacteremia (p=0.0414), ventilator-associated pneumonia (VAP; p=0.0036), urinary tract infections (UTI; p=0.0064), and surgical site infection (SSI; p≤0.0001). They were also more susceptible to postoperative seizures (p=0.0049). Furthermore, patients with DiGeorge syndrome had a higher prevalence of congenital renal anomalies. However, there was no mortality in either group.  Conclusion This study shows a variability in the postoperative outcomes between the two groups. The study demonstrates that patients with DiGeorge syndrome have higher risks of infections and longer hospital stay during the postoperative period. Further research with a larger sample is needed to confirm our findings.

Red Blood Cell Distribution Width as a Predictive Biomarker for Postoperative Infections in Children Who Underwent Cardiac Surgery: A Single-Center Retrospective Study.

Background It has been investigated that red blood cell distribution width (RDW) is associated with the clinical outcomes of patients following surgeries and is used as a prognostic biomarker for postsurgical complications. In this study, we aimed to assess the value of RDW as a predictor of postoperative complications in children after cardiac surgeries. Methods Three hundred fifty-five pediatric patients who underwent cardiac surgery between 2017 and 2018 were enrolled, and preoperative and postoperative RDW values were determined. Data collected included demographics; incidence of postsurgical complications, including sepsis, surgical site infections (SSIs), urinary tract infections (UTIs), and ventilator-associated pneumonia (VAP); length of hospital and pediatric cardiac intensive care unit (PCICU) stay; and ventilator duration. Results Among children who underwent cardiac surgery, 29 (8.2%) of the cases developed bloodstream infections (BSIs), while urinary tract infections (UTIs) were observed in 32 (9.0%) of the cases, and ventilator-associated pneumonia (VAP) was observed in 36 of the cases (10.1%). Of all cases, surgical site infections (SSIs) were reported in 13 patients (3.7%). Significantly higher postoperative RDW levels were observed on days three (p-value=0.028), five (p-value=0.041), and seven (p-value=0.042) in cases of BSI. For UTI cases, only preoperative RDW levels (p<0.001) and postoperative day three RDW levels (p<0.049) were significantly higher than their counterparts. VAP cases had significantly higher RDW levels pre-operatively (p-value=0.002), which was also observed in postoperative RDW levels on days three (p-value=0.033), five (p-value=0.031), and seven (p-value=0.021) in comparison to their analogs (p-values<0.05). Furthermore, a significant relationship was found between preoperative RDW and length of intensive care unit (ICU) stay (95% CI 0.685-3.221, p-value=0.003, R2=0.104) and duration of mechanical ventilation (95% CI 0.549-1.938, p-value=0.001, R2=0.102). Conclusion RDW is a significant factor in predicting complications in pediatric patients' post-cardiac surgeries, including BSI, UTI, and VAP, which would consequently anticipate patients' clinical state after cardiac procedures.

Local pulmonary administration of factor VIIa (rFVIIa) in massive pulmonary haemorrhage in post-operative cardiac infant.

Diffuse pulmonary haemorrhage is an ominous condition that has a high paediatric mortality rate. Recombinant activated factor VIIa (rFVIIa) is a powerful haemostatic agent which has been used intravenously in life-threatening haemorrhage in variety of conditions in which conventional medical or surgical therapy are unsuccessful. We report off-label successful use of endotracheal rFVIIa for massive life-threatening respiratory haemorrhage following aspiration and cardiopulmonary resuscitation in a 3-month-old infant who was anticoagulated with enoxaparin following corrective cardiac surgery with other comorbidities. Off-label administration of endotracheal rFVIIa permitted rapid safe control of massive pulmonary haemorrhage and prevented further detrimental decline in respiratory function with satisfactory outcome.

The Role of the Disrupted Podosome Adaptor Protein (SH3PXD2B) in Frank-Ter Haar Syndrome.

Frank-Ter Haar syndrome (FTHS), sometimes referred to as Ter Haar syndrome, is a rare hereditary disorder that manifests in skeletal, cardiac, and ocular anomalies, including hypertelorism, glaucoma, prominent eyes, and facial abnormalities. In this study, we performed whole-exome sequencing (WES) to identify the genetic component responsible for the phenotype of the index patient, a male infant born to a consanguineous family from Saudi Arabia. The analysis revealed a homozygous missense variant, c.280C>G, in the SH3PXD2B gene, which cosegregates with the familial phenotype with a plausible autosomal-recessive mode of inheritance, indicating a potential disease-causing association. The SH3PXD2B gene encodes a TKS4 podosome adaptor protein that regulates the epidermal growth factor signaling pathway. This study validates the critical function of the TKS4 podosome protein by suggesting a common mechanism underlying the pathogenesis of FTHS.

Ultrasound-guided post-pyloric feeding tube insertion in peri-operative cardiac infants.

Delivery of enteral nutrition in critical infants post-paediatric cardiac surgery is sometimes hampered, necessitating direct feeding into the small intestine. This study is highlighting the role of ultrasound-guided post-pyloric feeding tube insertion performed by the paediatric cardiac ICU intensivist in critically ill infants. METHODS: We carried out a prospective pilot observational experimental study in peri-operative cardiac infants with feeding intolerance between 2019 and 2021. Feeding tube insertion depends on a combination of ultrasound and gastric insufflation with air-saline mixture. Insertion was confirmed by bedside abdominal X-ray. RESULTS: Out of 500 peri-operative cardiac infants, 15 needed post-pyloric feeding tube insertion in median 15 postoperative day. All were under 6 months of age with average weight of 3 ± 0.2 kg. Median Risk Adjustment for Congenital Heart Surgery Categories was 4. Median insertion time was 15 minutes. No complications have been reported. First pass success rate was 87%, while a second successful insertion attempt was needed in 2 cases (13%). Target daily calorie intake was achieved within average of 3.5 ± 0.4 days. Mean post-pyloric feeding tube stay was 20 ± 3 days. Out of 15 infants, 3 patients died, 1 patient needed gastrostomy tube, and 11 patients were discharged home on oral feeds. CONCLUSIONS: Ultrasound-guided post-pyloric feeding tube insertion using gastric insufflation with air-saline mixture in peri-operative cardiac infants with feeding intolerance is a useful and practical bedside tool, and it can be performed by a trained paediatric cardiac ICU intensivist. It may have potential positive effects on morbidity and outcome.

Surgical outcome of Yasui procedure for preserving biventricular function: single centre experience.

BACKGROUND: Yasui procedure is surgical repair intended to preserve biventricular function for patients with left ventricle outflow tract obstruction associated with aortic arch lesions and ventricular septal defect. METHODS: Retrospective chart review analysis of all patients who had Yasui procedure (2008-2020) comparing midterm outcome of one versus two stage repair. RESULTS: Twenty patients (70% female) underwent Yasui procedure in our center. Eight patients (40%) had left ventricle outflow tract obstruction /interrupted aortic arch, 7 patients (35%) had left ventricle outflow tract obstruction /coarctation of aorta, 3 patients (15%) had double outlet ventricle and ventricular septal defect that were unattainable for tunneling to one of the semilunar valves without creating obstruction, and 2 patients (10%) had aortic atresia with hypoplastic aortic arch. All patients had associated ventricular septal defect. Fifteen patients (75%) had one-stage repair and 5 patients (25%) had two-stage repair. Means age and weight for one and two-stage repair were 1.3 ± 2 months, 13.4 ± 11.5 months and 3.3 ± 0.6 kg, 7.8 ± 3.4 kg, respectively. During follow up, 8 patients (40%) required re- intervention, mainly for right ventricle-pulmonary artery conduit either dilation or replacement. The average duration of follow up was 5 years with nil mortality. CONCLUSION: Yasui procedure is effective approach for children who have left ventricle outflow tract obstruction associated with aortic arch anomalies and ventricular septal defect. Survival rate with single or staged repair is comparably good. During the first 5 year of follow up, nearly 40% of operated patients required re-intervention.

Does interatrial communication affect post-operative course of children undergoing tetralogy of Fallot repair? Single centre retrospective cohort study: propensity score matching.

INTRODUCTION: During tetralogy of Fallot repair, leaving or even create an interatrial communication may facilitate post-operative course particularly with right ventricle restrictive physiology. The aim of our study is to assess the influence of atrial communication on post-operative course of tetralogy of Fallot repair. METHODS: Retrospectively, we studied all children who had tetralogy of Fallot repair (2003-2018). We divided them into two groups: tetralogy of Fallot repair with interatrial communication (TOFASD) group and tetralogy of Fallot repair with intact atrial septum (TOFIAS) group. We performed propensity match score for specific pre- or intra-operative variables and compared groups for post-operative outcome variables. Secondarily, we looked for right ventricle restrictive physiology incidence and influence of early repair performed before 3 months of age on post-operative course. RESULTS: One hundred and sixty children underwent tetralogy of Fallot repair including (93) cases of TOFIAS (58%) and (67) cases of TOFASD (42%). With propensity matching score, 52 patients from each group were compared. Post-operative course was indifferent in term of positive pressure ventilation time, vasoactive inotropic score, creatinine and lactic acid levels, duration and amount of chest drainage and length of intensive care unit and hospital stay. Right ventricle restrictive physiology occurred in 38% of patients with no effects on outcome. 12/104 patients (12%) with early repair needed longer pressure ventilation time (p = 0.003) and intensive care unit stay (p = 0.02). CONCLUSION: Leaving interatrial communication in tetralogy of Fallot repair did not affect post-operative course. As well, right ventricle restrictive physiology did not affect post-operative course. Infants undergoing early tetralogy of Fallot repair may require longer duration of positive pressure ventilation time and intensive care unit stay.

Neurological complications in children with infective endocarditis: Incidence, risk factors, and outcome: A 10-year single-center experience.

BACKGROUND AND OBJECTIVE: Despite improvement in medical management, infective endocarditis (IE) remains a serious disease that may affect children with and without preexisting cardiac conditions with significant morbidity and mortality. Neurological complications of IE represent the worst with guarded prognosis. The aim of this study is to describe the incidence, etiology, characteristics, risk factors, and outcome of children with neurological complications associated with IE. MATERIAL AND METHODS: A retrospective cohort study was conducted from 2009 to 2019 where all pediatric patients who fulfilled the modified Duke criteria for IE were included. We divided the cases into 2 groups: IE with neurological complications and IE without neurological complications control group. We compared the two groups statistically and analyzed the results. RESULTS: We identified 31 (17 male, 14 female) patients with IE. Neurological complications occurred in 7/31 (23%) patients, mainly in the form of a stroke. Gram-positive microbes were the main causative agents for IE (52%) followed by gram-negative (14%), then fungal organisms (3%). Univariate analysis identified the following risk factors for neurological complications: lower body weight, higher C- reactive protein (CRP) level, and left-sided valvular lesions with P values of (0.0003, 0.0001, and 0.04), respectively.Although mortality was higher in the neurological complications group, it was 43% in comparison to 21% in the control group and it did not reach statistical significance (P = .49). Large vegetation size (more than 10 mm) was seen in 57% of patients with neurological complications as compared to 16% in the control group (P = .052). CONCLUSION: Neurological complications occurred in almost a quarter of children with IE. Possible risk factors include lower body weight, left-sided valvular lesion, and higher levels of inflammatory markers (CRP). Stroke was the most common neurological complication encountered with possible increased risk of mortality.

Novel Autosomal Recessive Splice-Altering Variant in PRKD1 Is Associated with Congenital Heart Disease.

Congenital heart defects (CHDs) are the most common types of birth defects, and global incidence of CHDs is on the rise. Despite the prevalence of CHDs, the genetic determinants of the defects are still in the process of being identified. Herein, we report a consanguineous Saudi family with three CHD affected daughters. We used whole exome sequencing (WES) to investigate the genetic cause of CHDs in the affected daughters. We found that all affected individuals were homozygous for a novel splice-altering variant (NM_001330069.1: c.265-1G>T) of PRKD1, which encodes a calcium/calmodulin-dependent protein kinase in the heart. The homozygous variant was found in the affected patients with Pulmonary Stenosis (PS), Truncus Arteriosis (TA), and Atrial Septal Defect (ASD). Based on the family's pedigree, the variant acts in an autosomal recessive manner, which makes it the second autosomal recessive variant of PRKD1 to be identified with a link to CHDs, while all other previously described variants act dominantly. Interestingly, the father of the affected daughters was also homozygous for the variant, though he was asymptomatic of CHDs himself. Since both of his sisters had CHDs as well, this raises the possibility that the novel PRKD1 variant may undergo autosomal recessive inheritance mode with gender limitation. This finding confirms that CHD can be associated with both dominant and recessive mutations of the PRKD1 gene, and it provides a new insight to genotype-phenotype association between PRKD1 and CHDs. To our knowledge, this is the first report of this specific PRKD1 mutation associated with CHDs.

Aorto-left ventricular tunnel: case series of a rare disease.

INTRODUCTION: Aorto-left ventricular tunnel is a rare disease that can cause significant morbidity early in life due to volume overload and left ventricular failure. Surgical intervention is usually curative with minimal early complications. However, long-term effects and outcome are not fully determined. OBJECTIVE: We are reporting a case series of this rare CHD with its long-term outcome. METHODS: We conducted a retrospective analysis of all children from birth to 14 years of age who were admitted between 2001 and 2020 with the diagnosis of aorto-left ventricular tunnel. Demographic, echocardiographic, and perioperative data were collected and reviewed. The pre-operative data were compared with data reviewed on the last outpatient follow-up. RESULTS: Total of five patients fulfilled our inclusion criteria. Three patients were diagnosed after auscultating an incidental murmur, one had symptoms of congestive heart failure, and one had an abnormal fetal echocardiogram. Echocardiography demonstrated stenotic and regurgitant aortic valve with severely depressed left ventricle function in two patients, one of them with also single left coronary artery. The other three patients had normal aortic valve structure and normal ventricular function. All five patients had surgical repair, two by patch closure at aortic end of aorto-left ventricular tunnel, two by patch closure at both aortic and left ventricular ends, and one by aortic root replacement using a homograft. During follow-up, there was no residual aorto-left ventricular tunnel in any of our five cases, two had moderate aortic regurgitation and one had moderate residual aortic stenosis. CONCLUSIONS: Spectrum of presentation for aorto-left ventricular tunnel varies from an occult lesion to frank left heart failure due to volume or less commonly, pressure overload. Early surgical repair is recommended and is usually associated with complete resolution. Long-term follow-up is recommended for aortic root dilatation and aortic valve competency, as valve function need to be addressed in a timely manner to avoid further complications.

Impact of stent of ductus arteriosus and modified Blalock-Taussig shunt on pulmonary arteries growth and second-stage surgery in infants with ductus-dependent pulmonary circulation.

INTRODUCTION: Ducts-dependent pulmonary circulation is spectrum of congenital heart diseases that need urgent intervention to augment pulmonary blood. Systemic to pulmonary shunt is the classical surgical management. Stenting of ductus arteriosus emerged in the last 2 decades as an alternative plausible intervention. OBJECTIVES: To evaluate and compare the short and midterm effects of PDA stenting in compared to surgically placed shunt for augmentation of pulmonary blood flow looking to pulmonary artery (PA) branches growth, oxygen saturation and suitability for second stage repair. METHODS: We conducted this prospective study in Cardiac Surgical Intensive Care Unit. Cases were divided into "stent group" and "surgical shunt" group. Results were compared between two groups regarding oxygen saturation, mechanical ventilation duration, intensive care stay, mortality and morbidity. Growth of PA branches was assessed during follow up by echocardiograph. Nakata index score was calculated by angiogram before second stage surgery and was compared between both groups. RESULTS: 43 patients were included. Forty-two cases were offered stent as initial management. 6/42 cases failed stenting (14%) and 3/42 (7%) required late BT shunt after PDA stenting. 10/43 cases ended up receiving BT shunt and were counted as "surgical shunt group". Stent group (33 cases) needed less mechanical ventilation (2.08 ± 0.65 vs.7.8 ± 4 days with p = 0.014), and less ICU stay compared with surgical shunt group (6.2 ± 1.02 vs. 14 ± 4.5 days, P = 0.009). Both groups achieved similar growth of pulmonary artery branches (p = 0.6 for Z score of left pulmonary artery and P = 0.8 for Z score for right pulmonary artery). Although "stent group" reached second stage surgery with lower O2 saturation 67.6 ± 4.6 vs. 80 ± 4.2 in "surgical shunt" group with P value = 0.0002). Majority of patients in both groups had some PA distortion and needed surgical reconstruction in main pulmonary artery or in its main branches during second stage repair. 3 cases (7.1%) died soon post stenting versus none in surgical shunt group (p = value 0.57). CONCLUSIONS: In neonates with ductus-dependent pulmonary circulation PDA stenting can be introduced as safe first possible option to augment pulmonary blood flow with good outcome and suitable preparation for second stage palliation.

Perioperative management of Wilms' tumor with intracardiac extension: Report of two cases with review of literature.

Wilms' tumor (WT) is one of the solid tumor that affects children. It involves the kidney and may extend to the lungs and liver. WT conquers the adjacent blood vessels such as renal veins and inferior vena cava (IVC); its extension to IVC and right-sided heart chambers is unusual. Furthermore, when the tumor extends to heart chambers, its surgical management becomes challenging and demands multidisciplinary medical and surgical specialties including pediatric cardiac surgery. In this report, we discuss the surgical management and perioperative treatment of two unusual cases of WT with IVC and intracardiac extension.

Aortopulmonary window: Types, associated cardiovascular anomalies, and surgical outcome. Retrospective analysis of a single center experience.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. It occurs as an isolated cardiac lesion or in association with other cardiac anomalies and rarely with abnormal coronary arteries. The spectrum of cardiovascular anomalies associated with APW and overall management and outcome in the current era were reviewed. METHODS: Between 2001 and 2018, all patients diagnosed with APW were included. Based on associated cardiovascular anomalies, those patients were divided into 2 groups: simple APW group and complex APW group (APW with associated other cardiovascular anomalies). All cases were followed longitudinally. The outcomes are described. RESULT: Twenty patients underwent APW repair including 2 (10%) in simple APW group and 18 (90%) in complex APW group. Their mean age and weight were 4.8 ± 1.8 months and 4 ± 0.4 kg, respectively. APW Type I was confirmed in 65% followed by Type III in 20% and then Type II in 15% of the patients. In the complex APW group, atrial septal defect was the commonest associated cardiac lesion occurring in 8/20 (40%), followed by ventricular septal defect, interrupted aortic arch, and pulmonary artery anomalies in 25% of each. The presence of patent ductus arteriosus (PDA) was found in 40% of APW cases with 2/3rd of them in association with interrupted aortic arch. Two patients (10%) had unusual coronary anomalies that required repair, both with APW Type I. Associated non-cardiac anomalies were found in 30% of cases. Risk Adjustment for Congenital Heart Surgery (RACHS-1) score frequencies were between 2 and 4. Only one patient had reactive pulmonary hypertension related to chronic lung disease. All patients underwent surgical correction with median age of 2 month at the time of repair (interquartile range, 2 weeks to 4.5 months). Mean duration of mechanical ventilation, pediatric cardiac ICU and hospital length of stay were 2.8 ± 0.5, 9 ± 3 and 26 ± 6 days, respectively. All patients survived with no residual APW with mean follow-up duration of 4.5 years. CONCLUSION: Majority of APW are associated with other cardiovascular anomalies (90%) including coronary abnormalities (10%). Early surgical repair of APW and associated lesions showed excellent survival rate, freedom from re-intervention need within an average of 4.5 years of follow up and no evidence of persistent pulmonary hypertension post repair.

Complicated community-acquired methicillin-resistant Staphylococcus aureus pancarditis with cardiac pseudoaneurysm in a healthy child: A case report.

INTRODUCTION: Infective endocarditis is more prevalent among children with congenital heart diseases as compared to healthy children. Community-acquired methicillin-resistant Staphylococcus aureus is a causative pathogen of infective endocarditis, and it rarely causes pancarditis in healthy children. The clinical decision for surgical intervention of left-sided heart vegetation is challenging despite the availability of management guidelines. PRESENTATION OF CASE: We report a case of a previously healthy 12-year-old girl who presented with aggressive endocarditis secondary to community-acquired methicillin-resistant Staphylococcus aureus infection, with left-sided vegetation, mitral valve regurgitation, pancarditis, brain abscess, and stroke. She underwent an emergency vegetectomy and mitral valve repair. Three weeks after the first surgery, she developed left ventricular pseudoaneurysm that required life-saving surgical intervention. The child gradually recovered and was discharged home with acceptable cardiac function and mild neurological deficit. DISCUSSION: Pancarditis, especially with an aggressive progression resulting in intracardiac pseudoaneurysm, is rarely reported in healthy children. The definition of the optimal timing of surgical intervention in pediatric infective endocarditis management is lacking and the clinical decision-making process remains challenging. The development of left ventricular pseudoaneurysm is serious and also needs an immediate intervention, given the high risk of its rupture and subsequent devastating outcomes. CONCLUSION: Community-acquired methicillin-resistant Staphylococcus aureus is an etiology for aggressive infective pancarditis in a healthy child, leading to an intracardiac pseudoaneurysm. Emergency surgical interventions should be considered in children with left-sided vegetation to prevent devastating consequences.

ADAMTS19-associated heart valve defects: Novel genetic variants consolidating a recognizable cardiac phenotype.

Recently, ADAMTS19 was identified as a novel causative gene for autosomal recessive heart valve disease (HVD), affecting mainly the aortic and pulmonary valves. Exome sequencing and data repository (CentoMD) analyses were performed to identify patients with ADAMTS19 variants (two families). A third family was recognized based on cardiac phenotypic similarities and SNP array homozygosity. Three novel loss of function (LoF) variants were identified in six patients from three families. Clinically, all patients presented anomalies of the aortic/pulmonary valves, which included thickening of valve leaflets, stenosis and insufficiency. Three patients had (recurrent) subaortic membrane, suggesting that ADAMTS19 is the first gene identified related to discrete subaortic stenosis. One case presented a bi-commissural pulmonary valve. All patients displayed some degree of atrioventricular valve insufficiency. Other cardiac anomalies included atrial/ventricular septal defects, persistent ductus arteriosus, and mild dilated ascending aorta. Our findings confirm that biallelic LoF variants in ADAMTS19 are causative of a specific and recognizable cardiac phenotype. We recommend considering ADAMTS19 genetic testing in all patients with multiple semilunar valve abnormalities, particularly in the presence of subaortic membrane. ADAMTS19 screening in patients with semilunar valve abnormalities is needed to estimate the frequency of the HVD related phenotype, which might be not so rare.

Predictors of Reopening the Sternum in Children After Cardiac Surgery.

OBJECTIVES: Capillary leak syndrome can be severe in children after open-heart surgery which may hinder sternum closure and described as mediastinal tamponade. Reopening the sternum postoperatively may help maintaining hemodynamics and respiratory function. We looked for predictors that indicate the need for reopening the sternum. DESIGN: A retrospective cohort study. SETTING: A single cardiac center experience from 2009 to end of 2015. PATIENTS: All children who required emergent reopening the sternum in the pediatric cardiac ICU after cardiac surgery were grouped as index cases and matched to a control group for age, body weight, cardiac diagnosis, and type of repair (single vs biventricular). INTERVENTIONS: Emergent reopening the sternum. MEASUREMENTS AND MAIN RESULTS: With a ratio of two control cases for each index case, variables related to cardiac output (predictors) were collected in a time line of 12, 6 hours, and just before reopening the sternum. Morbidities and mortality were also reviewed. Thirty-three index cases were compared with 63 control cases. Hospital stay and hospital-acquired infections were the same between the groups. Ventilation hours were longer in the index cases. Temperature gap more than 3°C, inotropic score more than 14, and acute kidney injury indicated by doubled blood urea nitrogen and creatinine were higher in the index group 6 hours before reopening the sternum. Mortality was more in the reopening sternum group with higher risk when extracorporeal membrane oxygenation was needed. CONCLUSIONS: Low cardiac output after cardiac surgery in children in form of temperature gap more than 3°C, inotropic score more than 14, and acute kidney injury may predict the need of reopening the sternum. Rate of mortality was higher in the reopening sternum group when extracorporeal membrane oxygenation was needed.

Five-year survival, performance, and neurodevelopmental outcome following cardiopulmonary resuscitation after pediatric cardiac surgery, preliminary investigation in a single-center experience.

BACKGROUND AND AIM: Children who suffer cardiopulmonary arrest (CPA) after cardiac surgery frequently survive with return of spontaneous circulation. However, their neurodevelopmental outcomes and performance are still unclear. The aim of this study is to evaluate the midterm neurodevelopmental outcome and overall performance of children who survived CPA following cardiac surgery. MATERIALS AND METHODS: In this cohort study, we followed-up children who received cardiopulmonary resuscitation (CPR) post cardiac surgery during 2012-2013. We assessed their 5-year survival, functional, and neurodevelopmental outcomes using two performance scales: Pediatric Cerebral Performance Category (PCPC) and Pediatric Overall Performance Category (POPC). Both scales ranged from 1 for normal to 6 for brain death/death. We compared CPR group with a matching group (1:1) that had similar characteristics and conditions but no CPR. RESULTS: Out of 758 postoperative cardiac children, 15 (2%) children had 19 episodes of CPA. Their median age was 10 months (0.5-168). Survival rates were 12/15 (80%) on hospital discharge and 10/15 (66%) after 5 years. Among 12 survivors, two patients (17%) scored 6, one (8%) scored 4, five (42%) scored 2, and four (33%) scored 1 on both PCPC and POPC. The median PCPC and POPC scores were [2, (interquartile range: 1-6) and 1, (interquartile range: 1-3, p = 0.018] for CPR and matching group, respectively. Regression analysis identifies duration of CPR, number of CPR session, and late-occurring CPA as risk factors for poor outcome. CONCLUSION: Two-thirds of children requiring CPR post cardiac surgery survived after 5 years. Their neurodevelopmental and functional evaluation demonstrated worse outcome in comparison with their matching cases. CPR duration, number of CPA events, and late CPA were risk factors for poor outcome. Rehabilitation and special education programs might be needed for these groups of children with special needs.

Infective endocarditis in children with normal heart: Indication for surgical intervention.

Although infective endocarditis is an uncommon condition, it can be fatal if not treated. The new era of infective endocarditis in children with structurally normal heart has become apparent entity. Duke criteria has been established for a long time and gives clear guidelines for diagnosis; however, surgical indication in pediatric population needs to be tailored to individual patients.

Cardiac recovery and outcome of neonates and infants presenting with severe aortic coarctation and depressed cardiac function.

OBJECTIVES: Coarctation of the aorta represents 5-8% of all congenital heart diseases. Children with severe coarctation of the aorta may present with significant depression of myocardial function. The aim of this study is to identify short and midterm outcomes of neonates and infants with isolated coarctation of the aorta and depressed left ventricular systolic function with regard to recovery of their cardiac function. METHODS: All patients with isolated coarctation of the aorta who underwent surgical repair between December 2002 and December 2015 were retrospectively reviewed in a cohort study. The patients were divided into 2 groups: (1) Patients with depressed left ventricle systolic function who were found to have an ejection fraction and fractional shortening less than 55% and 25%, respectively, (2) Patients with coarctation of the aorta and normal left ventricle systolic function (Control Group). We reviewed both groups after surgery and compared them in terms of their cardiac function recovery. RESULTS: 58 patients were included. 25 patients (43%) depressed left ventricle systolic function group, 33 patients (57%) Control Group. There were statistically significant differences in ejection fraction and fractional shortening (p < 0.0001) between the two groups before surgery. Follow-up demonstrated improvement and recovery of ventricular function in most of the patients, six months after surgery there was no more statistical difference between the groups in terms of cardiac function. CONCLUSION: Majority of the patients with isolated coarctation of the aorta and depressed left ventricle systolic function showed improvement of ventricular function within 4 weeks after surgery, except for patients with residual coarctation of the aorta.

Effects of protocol-based management on the post-operative outcome after systemic to pulmonary shunt.

OBJECTIVES: Systemic to pulmonary shunt (commonly known as Modified Blalock-Taussig shunt) is a palliative procedure in cyanotic heart diseases to overcome inadequate blood flow to the lungs. Based on the most recent risk stratification score, the mortality and morbidity of this procedure is still high especially in neonates and over-shunting patients. We developed and implemented protocol-based management in March 2013 to better standardize the management of these patients. The aim of this study is to evaluate the effects of applying this protocol-based management in our center. METHODS: We conducted a retrospective cohort study through chart review analysis.We included all children who underwent MBTS from January 2000 till December 2015. We compared the early postoperative outcome of patients operated after the protocol-based management implementation (March 2013 till December 2015) (protocol group) with patients operated before implementing the MBTS protocoled management (control group). RESULTS: 197 patients underwent MBTS from January 2000 till December 2015. Of the 197 patients, 25 patients were in the protocol group and 172 patients were in the control group. There was a significant improvement in the postoperative course and less morbidity after protocoled management implementation as reflected in ventilation time, reintubation rate, inotropic support duration, intensive care unit ICU stay and significantly lower postoperative complications in the protocol group. Mortality of the control group versus protocol group (19.3% VS 8%) with Standardized Mortality Ratio (SMR) dropped from 2.27 before protocoled management to 0.94 after protocoled management (protocol group). CONCLUSION: The study suggests that protocoled management of patients with MBTS can improve the postoperative course and early outcome.