Intracranial Pressure and Cerebral Hemodynamics in Infants Before and After Glenn Procedure.

OBJECTIVES: This prospective cohort study aimed to investigate changes in intracranial pressure (ICP) and cerebral hemodynamics in infants with congenital heart disease undergoing the Glenn procedure, focusing on the relationship between superior vena cava pressure and estimated ICP. DESIGN: A single-center prospective cohort study. SETTING: The study was conducted in a cardiac center over 4 years (2019-2022). PATIENTS: Twenty-seven infants with congenital heart disease scheduled for the Glenn procedure were included in the study, and detailed patient demographics and primary diagnoses were recorded. INTERVENTIONS: Transcranial Doppler (TCD) ultrasound examinations were performed at three time points: baseline (preoperatively), postoperative while ventilated (within 24-48 hr), and at discharge. TCD parameters, blood pressure, and pulmonary artery pressure were measured. MEASUREMENTS AND MAIN RESULTS: TCD parameters included systolic flow velocity, diastolic flow velocity (dFV), mean flow velocity (mFV), pulsatility index (PI), and resistance index. Estimated ICP and cerebral perfusion pressure (CPP) were calculated using established formulas. There was a significant postoperative increase in estimated ICP from 11 mm Hg (interquartile range [IQR], 10-16 mm Hg) to 15 mm Hg (IQR, 12-21 mm Hg) postoperatively (p = 0.002) with a trend toward higher CPP from 22 mm Hg (IQR, 14-30 mm Hg) to 28 mm Hg (IQR, 22-38 mm Hg) postoperatively (p = 0.1). TCD indices reflected alterations in cerebral hemodynamics, including decreased dFV and mFV and increased PI. Intracranial hemodynamics while on positive airway pressure and after extubation were similar. CONCLUSIONS: Glenn procedure substantially increases estimated ICP while showing a trend toward higher CPP. These findings underscore the intricate interaction between venous pressure and cerebral hemodynamics in infants undergoing the Glenn procedure. They also highlight the remarkable complexity of cerebrovascular autoregulation in maintaining stable brain perfusion under these circumstances.

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.

Outcome of truncus arteriosus repair: 20 years of single-center experience comparing early versus late surgical repair.

BACKGROUND: Truncus arteriosus is a rare CHD. Neonatal and early infancy repair is recommended though some cases may present late. The aim of our study is to investigate the current results of truncus arteriosus repair and to analyse the differences in outcome and reintervention need between early versus late truncus arteriosus surgical repair. MATERIAL AND METHODS: In this cohort study, we reviewed all children who underwent truncus arteriosus repair from 2001 till 2021. We divided patients into two groups; early repair group including patients repaired at age less than 3 months and late repair group including patients who had repair at 3 months of age and later. We compared both groups for outcome variables. RESULTS: Sixty-four children had truncus arteriosus repair including 48(75%) patients in early repair and 16(25%) patients in late repair groups. Peri-operative course was comparable between both groups. Post-surgery, we observed pulmonary hypertension in 6(12%) patients in early repair group comparing with 11(69%) patients in late repair group (p = 0.0001). In the last follow-up visit, pulmonary hypertension resolved in all early repair group patients while 6(37.5%) patients in late repair group continued to have pulmonary hypertension (p = 0.0001). Twenty-three(36%) patients required reintervention including 22(48%) in early repair group versus 1(6%) in late repair group (p = 0.007). CONCLUSION: In general, the outcome of early truncus arteriosus repair is excellent with resolution of pulmonary hypertension following early repair. Late repair caries higher risk of persistent pulmonary hypertension (37.5%). About one-third of the patients who had truncus arteriosus repair will require re-intervention within 38±38.4 months after initial surgery.

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.

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.

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.

Bedside ultrasonography screening for congenital renal anomalies in children with congenital heart diseases undergoing cardiac repair.

INTRODUCTION: Ultrasound (US) assessment of renal anomalies in children requiring pediatric cardiac surgery is not a standard practice. This study is highlighting the role of bedside US performed by intensivist to detect occult renal anomalies associated with congenital heart disease (CHD). METHODS: A cross sectional study for 100 consecutive children with CHD admitted to Pediatric Cardiac Intensive Care Unit (PCICU) in 2015. US of kidneys screening was performed by trained pediatric cardiac intensivists to ascertain the presence of both kidneys in renal fossae without gross anomalies and to investigate if early detection of occult kidney anomaly would have any impact on outcome. RESULTS: After screening of 100 consecutive children with CHD with renal US, we identified in 94 cases (94%) normal right and left kidney in the standard sonographer shape within the renal fossae. In 6 cases further investigation revealed ectopic kidney in 3 patients (50%), solitary functional kidney in 2 patients (33%) and bilateral grade IV hydronephrosis in one patient (17%). Urinary tract infection developed peri-operatively in 66% of the cases with kidney anomalies with statistical significance compared to patients with normal renal US (P: 0.0011). No significant renal impairment was noted in these patients post-surgery. We observed no specific association between the type of renal anomaly and specific CHD. CONCLUSION: Routine renal US in children with CHD demonstrated prevalence of associated congenital renal anomalies in 6% of children undergoing cardiac surgery. The presence of occult renal anomalies was associated with higher UTI risk. Performing routine renal US as a standard practice in children with CHD is justifiable.

Ultrasound for diaphragmatic dysfunction in postoperative cardiac children.

Introduction The use of ultrasound for assessing diaphragmatic dysfunction after paediatric cardiac surgery may be under-utilised. This study aimed to evaluate the role of bedside ultrasound performed by an intensivist to diagnose diaphragmatic dysfunction and the need for plication after paediatric cardiac surgery. METHODS: We carried out a retrospective cohort study on prospectively collected data of postoperative children admitted to the paediatric cardiac ICU during 2013. Diaphragmatic dysfunction was suspected based on difficulties in weaning from positive pressure ventilation or chest X-ray findings. Ultrasound studies were performed by the paediatric cardiac ICU intensivist and confirmed by a qualified radiologist. RESULTS: Out of 344 postoperative patients, 32 needed diaphragm ultrasound for suspected dysfunction. Ultrasound studies confirmed diaphragmatic dysfunction in 17/32 (53%) patients with an average age and weight of 10.8±3.8 months and 6±1 kg, respectively. The incidence rate of diaphragmatic dysfunction was 4.9% in relation to the whole population. Diaphragmatic plication was needed in 9/17 cases (53%), with a rate of 2.6% in postoperative cardiac children. The mean plication time was 15.1±1.3 days after surgery. All patients who underwent plication were under 4 months of age. After plication, they were discharged with mean paediatric cardiac ICU and hospital stay of 19±3.5 and 42±8 days, respectively. CONCLUSIONS: Critical-care ultrasound assessment of diaphragmatic movement is a useful and practical bedside tool that can be performed by a trained paediatric cardiac ICU intensivist. It may help in the early detection and management of diaphragmatic dysfunction after paediatric cardiac surgery through a decision-making algorithm that may have potential positive effects on morbidity and outcome.

Ebstein cardiac anomaly, functional pulmonary atresia and isovaleric acidemia: A case report.

In this report, we present a rare association between Ebstein anomaly (EA) and isovaleric acidemia (IVA) in a newborn who was admitted to our cardiac center. He underwent for PDA stenting to maintain adequate pulmonary blood flow, later he developed recurrent metabolic acidosis, prominent sweaty feet odor, neutropenia and thrombocytopenia. His organic acids profile in the urine confirmed the diagnosis of IVA. To the best of our knowledge, there is no association between these two rare diseases. We are presenting this case report to highlight this rare association.