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.

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.

Growth of left ventricular outflow tract and predictors of future re-intervention after repair for ventricular septal defect and aortic arch obstruction.

Ventricular septal defect and aortic arch obstruction are usually associated with a narrow left ventricular outflow tract. The aim of the present study was to analyse the growth and predictors of future obstruction of the left ventricular outflow tract after surgical repair. METHODS: We carried out a retrospective review of patients who underwent repair for ventricular septal defect and aortic arch obstruction - coarctation or interrupted aortic arch - between July, 2002 and June, 2013. Echocardiographic data were reviewed, and the need for re-intervention was evaluated. RESULTS: A total of 89 patients were included in this study. A significant left ventricular outflow tract growth was noticed after surgical repair. Preoperatively, the mean left ventricular outflow tract Z-score was -1.46±1 (range -5.5 to 1.1) and increased to a mean value of -0.7±1.3 (range -2.7 to 3.2) at last follow-up (p=0.0001), demonstrating relevant growth of the left ventricular outflow tract after repair for ventricular septal defect and aortic arch obstruction. After primary repair, 11 patients (12.3%) required re-intervention with surgical repair for left ventricular outflow tract obstruction after a mean period of 36±21 months. There were no significant differences in age, weight, and indexed aortic valve and left ventricular outflow tract measurements between those who developed obstruction and those who did not. CONCLUSION: Significant left ventricular outflow tract growth is expected after repair of ventricular septal defect and aortic arch obstruction. Small aortic valve and left ventricular outflow tract at diagnosis are not risk factors to predict the need for surgical re-intervention for left ventricular outflow tract obstruction in future.

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.

Ventilator-associated pneumonia in children after cardiac surgery.

Ventilator-associated pneumonia (VAP) is a nosocomially acquired infection that has a significant burden on intensive care units (ICUs). We investigated the incidence of VAP in children after cardiac surgery and its impact on morbidity and mortality. A prospective cross-sectional review was performed in the postoperative cardiac patients in pediatric cardiac intensive care unit (PCICU) patients from March 2010 until the end of September 2010. The patients were divided into two groups: the VAP group and the non-VAP group, Demographic data and perioperative risk variables were collected for all patients. One hundred thirty-seven patients were recruited, 65 (48%) female and 72 (52%) male. VAP occurred in 9 patients (6.6%). Average body weights in the VAP and non-VAP groups were 5.9 ± 1.24 and 7.3 ± 0.52 kg, respectively. In our PCICU, the mechanical ventilation (MV) use ratio was 26% with a VAP-density rate of 29/1000 ventilator days. Univariate analyses showed that the risk variables to develop VAP are as follows: prolonged cardiopulmonary bypass (CPB) time, use of total parenteral nutrition (TPN), and prolonged ICU stay (p < 0.002 for all). Thirty-three percent of VAP patients had Gram-negative bacilli (GNB). VAP Patients require more MV hours, longer stay, and more inhaled nitric oxide. Mortality in the VAP group was 11% and in the non-VAP group was 0.7 % (p = 0.28). VAP incidence is high in children after cardiac surgery mainly by GNB. VAP increases with longer CPB time, administration of TPN, and longer PCICU stay. VAP increases morbidity in postoperative cardiac patients.

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.

The impact of child-specific characteristics on warfarin dosing requirements.

Background: The influence of child characteristics on warfarin dosing has been reported; however, there is no consensus on the nature and extent of this effect. Objectives: To investigate the impacts of the demographic and clinical characteristics of children on the warfarin dose required to achieve a therapeutic international normalization ratio (INR). Methods: This retrospective cohort study included children aged 3 months to 14 years old who were prescribed warfarin for 3 months or longer with a "stable INR." The primary outcome was the total daily dose (TDD) and total weekly dose of warfarin required to achieve a therapeutic INR target. Results: We included 127 patients with a mean age of 7.7 ± 3.7 years and a median weight of 22 (IQR, 16-33) kg. Of the sample, 55 patients (43.3%) required a TDD of ≤0.1 mg/kg. The TDD for children younger than 5 years, 5 to 10 years, and older than 10 years were 0.14 ± 0.06 mg/kg, 0.12 ± 0.05 mg/kg, and 0.096 ± 0.04 mg/kg, respectively (P = .002). Overweight and obese children required a smaller TDD than normal-weight children: 0.09 ± 0.05 vs 0.13 ± 0.05 mg/kg (P = .004), which was similar for underweight children. A lower body surface area (<0.5 m2) required a higher dose. All the other variables did not affect warfarin doses. The incidence of a subtherapeutic or supratherapeutic INR was independent of demographic or clinical variables. Conclusion: The study confirmed that the patient demographics affect the daily warfarin dose required to achieve the INR target. However, they do not have any predictive value for the incidence of out-of-range-INR.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs. METHODS: In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included. RESULTS: Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity. CONCLUSIONS: PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Is Transposition of the Great Arteries Associated With Shortening of the Intrapericardial Portions of the Great Arterial Trunks? An Echocardiographic Analysis on Newborn Infants With Simple Transposition of the Great Arteries to Explore an Animal Model-Based Hypothesis on Human Beings.

Background The pathogenesis of transposition of the great arteries (TGA) as a congenital heart defect of the outflow tract with discordant ventriculoarterial connections remains an enigma. TGA usually have parallel great arteries suggesting that deficient torsion of the embryonic arterial heart pole might cause discordant ventriculoarterial connections. It has been speculated that deficient elongation of the embryonic outflow tract might prevent its normal torsion resulting in TGA. The aim of our study was to clarify whether the intrapericardial portions of the great arteries in human patients with TGA might be indeed shorter than in normal hearts. Methods and Results Thirty-four newborns with simple TGA and 35 newborns with normal hearts were analyzed by using images of the outflow tract in their echocardiograms and the following defined lengths of the great arteries were measured: aortic length 1, (AoL-1) and aortic length 2 (AoL-2) = distance between left and right aortic valve level and origin of the brachiocephalic artery, respectively. Pulmonary trunk length 1 (PTL-1) and pulmonary trunk length 2 (PTL 2) = distance between left and right pulmonary valve level and origin of left and right pulmonary artery, respectively. All measurements of the AoL were significantly shorter in TGA compared to normal hearts (AoL-1: 1.6±0.2 versus 2.05±0.1; P<0.0001; AoL-2: 1.55±0.2 versus 2.13±0.1; P<0.0001). With regard to the pulmonary trunk (PT), PTL-1 and PTL-2 were found to be shorter and longer, respectively, in TGA compared with normal hearts, reflecting the differences in the spatial arrangement of the PT between the 2 groups as in TGA the PT is showing a mirror image of the normal anatomy. However, the overall length of the PT between the 2 groups did not differ. Conclusions Our data demonstrate that, compared with normal newborns, the ascending aorta is significantly shorter in newborns with TGA whereas the overall length of the PT does not differ between the 2 groups. This finding is in accord with the animal model-based hypothesis that TGA may result from a growth deficit at the arterial pole of the embryonic heart.

Stent or Shunt, What Could be Better for Children with Duct Dependent Pulmonary Circulation?

BACKGROUND: Systemic to pulmonary shunt (Shunt) is offered for children with duct dependent pulmonary circulation to augment pulmonary flow. Recently patent ductus arteriosus (PDA) stent (Stent) is widely used as an alternative method. We aimed to compare post intervention outcomes in children underwent either procedure. METHODS: Infants under 3 months who had an initial palliation by Shunt or Stent were retrospectively reviewed between 2008 and 2016, then followed till the second intervention or 1 year whichever earlier. RESULTS: 187 patients (110 Shunt and 77 Stent) were included. Initial weight and pulmonary artery (PA) branches size were similar between the groups. Shunt patients had more shock preoperatively and required more emergency intervention. Stent group showed less ICU stay 4 (1-8) vs 13 (7-23) days, p < 0.0001 and less positive pressure ventilation days 1 (0-2) vs 5.5 (3-11), p < 0.0001. However, Stent group had more symptomatic arterial and deep venous thromboses. In Stent patients the branch PAs growth was better and more homogeneous. At follow-up, no difference between groups regarding cumulative readmission days to hospital, hemoglobin levels and the weight percentile for age. Mortality was not different with a tendency to be higher in the Shunt group (13%) compared to the Stent group (5%), p 0.1. CONCLUSIONS: The implantation of PDA stent in patients with duct dependent pulmonary circulation results in a smoother ICU course and a shorter hospital stay, with higher risk of vascular injury. Shunt and Stent procedures have a good outcome for PA growth, somatic growth and survival.

Long-Term Outcome of the Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (ALCAPA) in Children After Cardiac Surgery: A Single-Center Experience.

Background The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery anomaly. It induces left ventricular (LV) dysfunction and mitral valve regurgitation (MR). If untreated, survival beyond infancy is rare. The aim of our study was to analyze the outcome in children with ALCAPA after cardiac surgery. Methods We retrospectively reviewed all patients who were diagnosed at our institution with ALCAPA and underwent surgical repair from 1999 to the end of 2018 (for 20 years). We followed LV dimensions, function, the progress of MV regurgitation, and the somatic growth of children after surgical repair. Results Twenty-nine patients underwent ALCAPA repair while 15 (52%) patients were male. The median age at surgical repair was 5.3 (IQR: 3.8-7.4) months and the mean weight was 5.5±2 kg. Surgical repair was performed in form of coronary reimplantation in 26 (90%) patients and Takeuchi repair in three (10%) patients. Intensive care unit (ICU) stay was eight (IQR: 6-17) days and hospital stay was 15 (IQR: 12-21) days. The follow-up duration was 5±3.6 years. Echocardiographic parameters started to improve by six weeks after the repair, and they normalized by one year. At the time of surgery ejection fraction (EF) was 34±17%, fractional shortening (FS) was 15±10%, and LV inner diameter in diastole (LVIDD) z score was 5.7±2.8. These parameters improved by one year after surgery to 66±7%, 34±6%, and 0±1.3, respectively. However, somatic growth started to improve six months after surgical repair. MR was moderate to severe in seven (24%) patients at the time of surgery and regressed to no more moderate nor severe MR at the last follow-up. None of the 29 patients died. Conclusions LV systolic function and dimensions start to improve by six weeks after surgery and reach normal values by one year. MR regresses without intervention in correspondence with the regression of LV dimensional parameters. The somatic growth of children improves six months after repair.

Percutaneous balloon dilatation for congenital aortic stenosis during infancy: A 15-year single-center experience.

BACKGROUND: Congenital aortic stenosis (AS) is a rare disease. Treatment options for newborns are challenging. Newborns may have higher reintervention rate and mortality. OBJECTIVES: The study aimed to identify the factors predictive of reintervention following balloon aortic valvuloplasty (BAV) for AS during infancy. METHODS: Retrospectively, between 2001 and 2016, echocardiography (echo) and cardiac catheterization data for infants with AS were analyzed, including follow-ups and reinterventions. Percentage reduction was defined as the ratio between the drop of aortic valve (AV) peak gradient and the baseline peak gradient. RESULTS: Sixty infants were included and 48 were followed up. Sixteen (27%) patients were neonates. Peak-to-peak gradient at AV was 64 ± 27 mmHg, which was reduced to 27 ± 13 mmHg. Percentage reduction was 53% ±24%. Forty-nine (82%) patients had adequate results (residual AV gradient <35 mmHg). There was no significant aortic insufficiency (AI) before procedure, while 6 (10%) patients had increased AI immediately after BAV. Of 48 patients, 14 (29%) required an additional BAV. Of 48 patients, 8 (17%) required surgical interventions following BAV. Reintervention was associated more with small left ventricular outflow tract (LVOT), high residual AV, and low percentage reduction. Mortality was 8.3%. CONCLUSIONS: BAV in infancy has a reasonable success rate (82%) with high rate of reintervention. Patent ductus arteriosus-dependent neonates carried the highest risk of mortality. Small LVOT, high AV residual gradient, and low percentage reduction resulted in more reinterventions.

Role of intraoperative transesophageal echocardiography in pediatric cardiac surgery.

BACKGROUND: Intraoperative transesophageal echocardiography (TEE) has a major role in detecting residual lesions during and/or after pediatric cardiac surgery. METHODS: All pediatric patients who underwent cardiac surgery between July 2001 and December 2008 were reviewed. The records of surgical procedure, intraoperative TEE, and predischarge transthoracic echocardiograms were reviewed to determine minor and major residual cardiac lesions after surgical repair. RESULTS: During the study period, a total of 2268 pediatric cardiac patients were operated in our center. Mean age was 21 months (from 1 day to 14 years). Of these patients, 1016 (48%) had preoperative TEE and 1036 (46%) were evaluated by intraoperative echocardiography (TEE or epicardial study). We identified variations between TEE and preoperative transthoracic echocardiography in 14 patients (1.3%). Only one surgical procedure was cancelled after atrial septal defect exclusion. The other 13 patients had minor variation from their surgical plan. Major residual lesions requiring surgical revision were detected in 41 patients (3.9%), with the following primary diagnoses: tetralogy of Fallot in 12 patients (29%), atrioventricular septal defect in seven patients (17%), ventricular septal defect in seven patients (17%), double outlet right ventricle in two patients (5%), Shone complex in two patients (5%), subaortic stenosis in two patients (5%), mitral regurgitation in two patients (5%), pulmonary atresia in two patients (5%), and five patients (12%) with other diagnoses. CONCLUSION: Intraoperative TEE has a major impact in pediatric cardiac surgery to detect significant residual lesions. Preoperative TEE has a limited role in case of a high quality preoperative transthoracic echocardiography. We recommend routine use of intraoperative TEE during and/or after intracardiac repair in children.

Ultrasound guided vascular access in pediatric cardiac critical care.

INTRODUCTION: Safely obtaining vascular access in the pediatric population is challenging. This report highlights our real-world experience in developing a safer approach to obtaining vascular access using ultrasound guidance in children and infants with congenital heart disease. METHODS: As part of a quality initiative, we prospectively monitored outcomes of all vascular access attempts guided by ultrasound from January 2010 to September 2010. Variables monitored included age, weight, the time from first needle puncture to wire insertion, site of insertion, number of attempts, type of line, and complications. RESULTS: There were 77 attempts (15 arterial and 62 venous) to obtain vascular access in 43 patients. The mean age was 15 months (6 days-11 years; median 2.5 months). The mean weight was 7.2 kg (2-46 kg, median 3.8). Success rates were 93% and 95% for arterial and venous cannulation, respectively. Mean time from first needle puncture to wire insertion was 3.9 min (0.5-15 min, median 2 min). Fifty-five (75%) central line cannulations were successful from the first puncture; 17(23%) were successful from the second puncture; and one case (2%) required three punctures. Thirty patients (45%) weighed less than 3.5 kg. This lower body weight did not affect success rate, which was unexpectedly high (96.6%). There were no associated complications. CONCLUSION: Ultrasound guided vascular cannulation in critically ill pediatric patients is safe, effective and efficient. This approach had a high success rate, and was associated with zero complications in our setting.

Impact of chylothorax on the early post operative outcome after pediatric cardiovascular surgery.

BACKGROUND AND AIM: Chylothorax is the accumulation of chyle in the pleural cavity, which usually develops after disruption of the thoracic duct along its intra-thoracic route. In the majority of cases, this rupture is secondary to trauma (including cardio thoracic surgeries). Chylothorax is a potentially serious complication after cardiovascular surgeries that require early diagnosis and adequate management. This study aims to determine the risk factors and the impact of chylothorax on the early postoperative course after pediatric cardiac surgery. METHODS: A retrospective study of all cases complicated with chylothorax after pediatric cardiac surgery was conducted at King Abdulaziz Cardiac Center between January 2007 and December 2009. RESULTS: There were 1135 cases operated on during the study period. Of these, 57 cases (5%) were complicated by chylothorax in the postoperative period. Thirty patients (54%) were males, while 27 (47%) were females. Ages ranged from 4 to 2759 days. The most common surgeries complicated by chylothorax were the single ventricle repair surgeries (15 cases, 27%); arch repairs (10 cases, 18%); ventricular septal defect repairs (10 cases, 18%); atrioventricular septal defect repairs (7 cases, 12%); arterial switch repair (6 cases, 11%), and others (8 cases, 14%). The intensive care unit (ICU) and the length of hospital stays were significantly longer in the chylothorax group. Additionally, some early postoperative parameters such as incidence of sepsis, ventilation time, and inotropes duration and number were higher in the chylothorax group. CONCLUSION: Chylothorax after pediatric cardiac surgery is not a rare complication. It occurs more commonly with single ventricle repair and aortic arch repair surgeries, and has a significant impact on the postoperative course and post operative morbidity.

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.

Ultrasonography assessment of vocal cords mobility in children after cardiac surgery.

OBJECTIVES: Upper airway obstruction after pediatric cardiac surgery is not uncommon. In the cardiac surgical population, an important etiology is vocal cord paresis or paralysis following extubation. In this study, we aimed to evaluate the feasibility and accuracy of ultrasonography (US) assessment of the vocal cords mobility and compare it to fiber-optic laryngoscope (FL). MATERIAL AND METHODS: A prospective pilot study has been conducted in Pediatric Cardiac ICU (PCICU) at King Abdulaziz Cardiac Center (KACC) from the 1st of June 2009 till the end of July 2010. Patients who had cardiac surgery manifested with significant signs of upper airway obstruction were included. Each procedure was performed by different operators who were blinded to each other report. Results of invasive (FL) and non-invasive ultrasonography (US) investigations were compared. RESULTS: Ten patients developed persistent significant upper airway obstruction after cardiac surgery were included in the study. Their mean ± SEM of weight and age were 4.6 ± 0.4 kg and 126.4 ± 51.4 days, respectively. All patients were referred to bedside US screening for vocal cord mobility. The results of US were compared subsequently with FL findings. Results were identical in nine (90%) patients and partially different in one (10%). Six patients showed abnormal glottal movement while the other four patients demonstrated normal vocal cords mobility by FL. Sensitivity of US was 100% and specificity of 80%. CONCLUSION: US assessment of vocal cord is simple, non-invasive and reliable tool to assess vocal cords mobility in the critical care settings. This screening tool requires skills that can be easily obtained.

Dextrocardia, aortopulmonary window with transposition of the great arteries, case report.

Transposition of the great arteries (D-TGA) in combination with aortopulmonary window (APW) is a very rare entity and carries high morbidity and mortality. Only few cases have been reported with this association. We report the first case of D-TGA and APW with mirror image dextrocardia which was repaired successfully.