Impact of Blood Sampling Methods on Blood Loss and Transfusion After Pediatric Cardiac Surgery: An Observational Study.

OBJECTIVES: The aims of this study were to assess the impact of the closed-loop sampling method on blood loss and the need for blood transfusion in pediatric patients following cardiac surgery. DESIGN: Retrospective observational study. SETTING: A single tertiary center. PARTICIPANTS: All pediatric patients younger than 4 years old who were admitted to the pediatric intensive care unit (PICU) after cardiac surgery were enrolled. The study included 100 pediatric patients in the conservative (postimplementation) group and 43 pediatric patients in the nonconservative group (preimplementation). INTERVENTIONS: Observational. MEASUREMENTS: The primary outcome was the volume of blood loss during the PICU follow-up period. The secondary outcomes were the requirement for blood transfusion in each group, duration of mechanical ventilation, length of intensive care unit (ICU) stay, length of hospital stay, and mortality. MAIN RESULTS: In the conservative (postimplementation) group, blood loss during the follow-up period was 0.67 (0.33-1.16) mL/kg/d, while it was 0.95 (0.50-2.30) mL/kg/d in the nonconservative (preimplementation) group, demonstrating a significant reduction in blood loss in the conservative group (p = 0.012). The groups showed no significant differences in terms of the required blood transfusion volume postoperatively during the first 24 hours, first 48 hours, or after 48 hours (p = 0.061, 0.536, 0.442, respectively). The frequency of blood transfusion was comparable between the groups during the first 24 hours, first 48 hours, or after 48 hours postoperatively (p = 0.277, 0.639, 0.075, respectively). In addition, the groups did not show significant differences in the duration of mechanical ventilation, length of ICU stay, length of hospital stay, or mortality. CONCLUSIONS: The closed-loop sampling method can be efficient in decreasing blood loss during postoperative PICU follow-up for pediatric patients after cardiac surgeries. However, its application did not reduce the frequency or the volume of blood transfusion in these patients.

Early echocardiographic signs of cardiovascular affection in pediatric familial hypercholesterolemia.

Familial hypercholesterolemia (FH) is a rare autosomal dominant genetic disorder caused by defective low-density lipoprotein (LDL) receptors or abnormal apolipoprotein B. FH raises the risk of premature atherosclerotic disease and cardiovascular death in young adults. However, cardiovascular affection in children needs to be more adequately studied. Our study aimed to evaluate the effect of hypercholesterolemia on the cardiovascular system of pediatric patients with homozygous FH using conventional and advanced echocardiographic parameters such as tissue Doppler imaging (TDI) and 2-dimensional speckle-tracking echocardiography (2D-STE). This case-control study matched 25 healthy children with 21 patients with homozygous FH. Both groups had conventional echocardiography, TDI, and 2D-STE. Myocardial velocities of the left and right ventricles, left ventricular strain, and aortic stiffness parameters were measured. The FH group had greater systolic blood pressure, dilated coronary arteries, and hypertrophied left ventricle (LV) compared to the control (P = 0.0001, P = 0.001, P = 0.01, respectively). The mitral E/E' ratio was higher in the patient group than in the control group (P = 0.007), indicating LV diastolic dysfunction in patients. At the same time, LV systolic function evaluated by 2D-STE was comparable to that in the control group. The abdominal aorta circumferential strain and ascending aorta M-mode-derived strain were significantly lower in patients compared to those in the control (P = 0.024, P = 0.0001, respectively), indicating increased aortic stiffness in the patients' group; moreover, 85.7% of patients had mild aortic insufficiency.  Conclusion: Mild aortic insufficiency, coronary artery dilatation, left ventricular (LV) diastolic dysfunction, and increased aortic stiffness are among early cardiovascular markers in pediatric patients with homozygous FH before impaired LV systolic function. What is Known: • Familial hypercholesterolemia (FH) in adults is associated with accelerated atherosclerosis, aortic valvopathy, dilated coronary arteries, ischemic heart disease, and premature cardiovascular death. • The cardiovascular effects of FH in children require additional research. What is New: • Pediatric patients with familial hypercholesterolemia tend to have an early affection for left ventricular diastolic function before the affection for the systolic function. • The diastolic dysfunction associated with pediatric FH is correlated to the aortic stiffness and low-density lipoprotein levels.

Using 2D speckle-tracking echocardiography to localize the accessory pathway and evaluate cardiac function and dyssynchrony in pediatric patients with Wolf-Parkinson-White syndrome.

Wolf-Parkinson-White (WPW) accessory pathway (AP) may be associated with reentry supraventricular tachycardia (SVT) in addition to ventricular dyssynchrony and cardiac dysfunction. Electrophysiological studies (EPS) are the gold standard for the localization of the AP; however, 2D speckle-tracking echocardiography (2D-STE) may help in the localization of the AP noninvasively. Our study aims to evaluate the capability of 2D-STE for AP localization and the identification of AP-related contractile abnormalities and dyssynchrony in pediatric patients with WPW syndrome. This prospective multicenter cohort study involved 18 pediatric patients with ventricular preexcitation from January 2021 to January 2023. Tissue Doppler imaging (TDI), conventional echocardiography, and 2D-STE were done. Myocardial velocities, myocardial performance index (MPI), the global and segmental longitudinal strain of the left ventricle (LV), and time-to-peak longitudinal strain (TPLS) were measured before and after ablation. The longitudinal strain of the LV segments supplied by the AP, or the nearby segments close to the AP, was significantly impaired and improved after ablation (P = 0.0001). The abnormal strain pattern in the affected segments could predict the location of the AP. The TPLS of the affected segments significantly increased after ablation (P = 0.0001), denoting improved dyssynchrony. The ejection time and the LV MPI measured at the basal septum improved significantly after ablation. CONCLUSIONS: 2D STE may be used for noninvasive localization of the AP and to evaluate cardiac function and dyssynchrony in patients with WPW. Further research on more patients is necessary to validate this method for AP localization. WHAT IS KNOWN: • Accessory pathways (AP) associated with the Wolf-Parkinson-White (WPW) syndrome have been linked to supraventricular tachycardia (SVT). Even without SVT, WPW can cause left ventricular dyssynchrony, contractile dysfunction, and cardiomyopathy. • Electrophysiology study is the gold standard for the localization of the AP in WPW syndrome. WHAT IS NEW: • The combination of 2D-speckle-tracking echocardiography (2D-STE) and the modified Arruda algorithm can precisely localize the AP associated with WPW syndrome. • 2D-STE can potentially assess cardiac function and dyssynchrony related to WPW syndrome. Additionally, 2D-STE can be utilized to evaluate the effectiveness of ablation in restoring cardiac function and dyssynchrony.

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.

Functional recovery of cardiomyopathy induced by atrial tachycardia in children: Insight from cardiac strain imaging.

AIM: To evaluate systolic and diastolic cardiac function in children who had cardiomyopathy induced by ectopic atrial tachycardia (EAT). METHODS: Twenty-two pediatric patients who had cardiomyopathy induced by EAT and 25 age-matched controls were recruited in this case-control study. The patients were examined after rhythm control and normalization of their left ventricular systolic function. Different echocardiographic modalities including tissue Doppler imaging and two-dimension speckle tracking echocardiography were utilized to assess the ventricular and atrial function. RESULTS: The patients' median age was 51 months (interquartile range: 28.5-84 months). The median time interval required for normalization of left ventricular ejection fraction (EF) among patients was 1.5 months (interquartile range: 1.5-2.12 months). Compared to controls, patients had a significantly higher median left ventricular myocardial performance index (MPI) at the interventricular septum (0.44 vs. 0.38, p = .001) and left ventricular lateral wall (0.46 vs. 0.32, p = .0001). The median right ventricular MPI of the patients' group was significantly higher when compared to the control group (0.34 vs. 0.26, p = .0001). The median right atrial (RA) reservoir function in patients was significantly reduced compared to controls (30% vs. 36.63%, p = .007). CONCLUSIONS: Shortly after rhythm normalization and restoration of left ventricular EF, using tissue Doppler imaging and two-dimension speckle tracking echocardiography, children who had cardiomyopathy induced by EAT continue to have left ventricular diastolic dysfunction, right ventricular dysfunction, and reduced RA reservoir function.

Hemopericardium with cardiac tamponade as a rare presentation of a massive aortic aneurysm in a young child with autosomal recessive cutis laxa.

Aortic aneurysms are rare in the pediatric age group and are commonly caused by genetic disorders associated with vasculopathy, weakness and fragility of arterial walls with progressive dilatation or even rupture. We reported a giant aortic aneurysm involving the ascending aorta and aortic arch in a 20-month-old girl with autosomal recessive cutis laxa type 1B (ARCL1B) who presented with hemorrhagic pericardial effusion and tamponade (impending rupture). Successful surgical repair has been done through excision of the aneurysmal part and replacement by Hemashield graft with preservation of the aortic valve.

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).

Subtle cardiac dysfunction in nephropathic cystinosis: insight from tissue Doppler imaging and 2D speckle tracking echocardiography.

BACKGROUND: Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disorder that initially affects the kidney progressing to multi-organ failure due to accumulation of cystine in all tissue compartments. OBJECTIVE: The main objective of this study is the evaluation of cardiac function in cystinosis patients using non-conventional echocardiographic modalities like pulsed wave tissue Doppler imaging (PW-TDI) and 2D speckle tracking echocardiography (2D-STE). METHODS: This is a case control study conducted on fifteen patients with cystinosis and 15 normal controls. Echocardiography was done for all participants and PW-TDI was performed for measurement of S', E', A' velocities and myocardial performance index (MPI) at basal parts of septal, left ventricle (LV), and right ventricle (RV) free walls. 2D-STE was done for evaluation of global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) of LV. Mitral E and A velocities and tricuspid annular plane systolic excursion (TAPSE) were also measured. RESULTS: The GLS, GRS, and S' velocity at basal septum and LV lateral wall were significantly lower in patients denoting LV systolic dysfunction (p = 0.005, p < 0.0001, p = 0.001, p = 0.006, respectively), while E/E' were significantly higher in patients group denoting LV diastolic dysfunction (p < 0.001). For RV function, TAPSE, S', and E' velocity were significantly lower in patients group (p 0.013, p < 0.01, p = 0.05, respectively) indicating RV systolic and diastolic dysfunction. The TDI-derived MPI for both LV and RV were significantly higher in patients group (p < 0.0001, p < 0.01, respectively) indicating both ventricular systolic and diastolic dysfunction. For prediction of cardiac dysfunction among patients, the receiver operating characteristic (ROC) curve showed that GRS ≤ 29% had sensitivity 93.3% and specificity 100%, GLS > - 20.1% had sensitivity 66.7% and specificity 93.3%, LV-E/E' >7.87 had sensitivity 73.3% and specificity 93.3%, and MPI-LV > 0.36 had sensitivity 100% and specificity 93.3% while MPI-RV > 0.29 had sensitivity 80% and specificity 93.3% and TAPSE ≤ 19 mm had sensitivity 80% and specificity 73.3%. CONCLUSIONS: Patients with cystinosis have significant both left and right ventricular dysfunction, which can be better evaluated using the non-conventional echocardiographic modalities like TDI and 2D-STE for early detection of subtle cardiac dysfunction.

Serum vitamin D status following pediatric cardiac surgery and association with clinical outcome.

Our aim is to determine the prevalence of vitamin D deficiency in children with congenital heart disease (CHD). In addition, we demonstrated the effect of cardiopulmonary bypass (CPB) on vitamin D, parathyroid hormone (PTH), and calcium levels. The association between perioperative vitamin D levels and postoperative clinical outcomes has been explored. A prospective observational study was conducted from February 2018 to June 2019 on 69 children undergoing elective surgery for CHD under CPB. Blood samples were collected preoperatively, immediate postoperatively, and 24 h postoperatively. Vitamin D deficiency was present in 34 (49.3%) patients preoperatively and 63 (91.3%) patients immediately postoperative. We identified 42.03% decline of 25(OH)D immediately postoperative. Changes in ionized calcium (iCa) concentrations were accompanied by reciprocal alterations in PTH concentrations. Lower postoperative 25(OH)D was associated with higher maximum vasoactive inotropic score (VIS) in the first 24 h postoperative (r = - 0.259, p = 0.03).Conclusion: Vitamin D deficiency is common in children with CHD and the majority are vitamin D deficient following cardiac surgery with acute decline of serum 25(OH)D after CPB. Lower postoperative vitamin D levels in children undergoing cardiac surgery are associated with the need for increasing the inotropic support.What is Known:• Vitamin D is a pleiotropic hormone, important for calcium homeostasis.• Vitamin D deficiency might affect the outcome in critically ill patients.What is New:• Cardiopulmonary bypass causes acute decline of vitamin D in children.• Lower postoperative vitamin D is associated with higher inotropic support.

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

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

Excess nonhemorrhagic pleural drainage after surgery for congenital heart diseases: Single center experience.

OBJECTIVE: Chylothorax after surgery for congenital heart disease is an uncommon but serious complication that adversely affects surgical outcomes. The aim of our study was presenting our experience for the management of postoperative chylothorax and excess nonhemorrhagic pleural drainage. METHODS: Medical records of patients with excess nonhemorrhagic pleural drainage were retrospectively reviewed and the collected data included demographics, surgical procedures, drainage characteristics, methods of postoperative management, and outcome. RESULTS: From March 2011 to May 2018, 52 patients with excess postoperative pleural drainage were identified from a total of 816 pediatric patients operated upon for congenital cardiac disease, giving an incidence of 63.7%. Tetralogy of Fallot and single ventricle morphology were the most common cardiac pathology. The serum triglyceride level was checked and found elevated in 30 patients (53.5%). The maximum daily drainage was 136.25 ± 109.7 mL/day and the mean duration of drainage was 32.23 ± 35.7 days. Medium-chain triglyceride formula was given for 27 patients (51.9%), octreotide for 22 (42.3%) for a mean duration of 8.07 ± 28.3 days. Total parenteral nutrition was needed for 11 patients (21.1%) for a mean duration of 3.13 ± 7.63 days. The success rate for conservative management was 94.2%. Thoracic duct ligation performed for three patients. The mean duration of mechanical ventilation was 7.4 ± 3.6 days, mean intensive care unit stay was 29.6 ± 35.1 days, and mean total hospital stay was 20.9 ± 17.5 days. We had six cases of hospital mortality (11.53%). CONCLUSION: Initiation of a stepwise approach for excess nonhemorrhagic pleural drainage based on the amount and rate of drainage achieve a favorable outcomes.

Evolution of ventricular function in children with permanent right ventricular pacing after tetralogy of Fallot repair: A midterm follow-up.

OBJECTIVE: We aim to evaluate the midterm effect of chronic apical right ventricular (RV) pacing on right and left ventricular (LV) function using different modalities of echocardiography including conventional echocardiography, tissue Doppler imaging and two-dimension speckle tracking echocardiography. METHODS: This case-control study enrolled 49 patients divided into two groups: a paced group and a nonpaced group. The paced group included 23 patients that underwent tetralogy of Fallot (TOF) repair and required permanent pacemaker insertion for postoperative complete heart block. The nonpaced group included 26 patients that had TOF repair at the same period. RESULTS: The median age for the paced and nonpaced groups was 6 and 8 years, respectively (P = .169). The follow-up duration after TOF surgical repair was 4 years for the paced patients and 5 years for nonpaced patients (P = .411). In the nonpaced group, the QRS duration increased and LV global longitudinal strain (GLS) decreased significantly with increasing duration after TOF repair, P value was .006 and .042, respectively. In the paced group, tricuspid annular systolic plane excursion (TAPSE) was significantly correlated with age (r = .578; P = .004) and duration following TOF correction (r = .724; P < .001). CONCLUSION: Chronic RV apical pacing in children after TOF repair was associated with better clinical status, preservation of RV systolic function, and prevention of progressive QRS prolongation. RV pacing was not associated with progressive deterioration of LV systolic function with increasing the time interval following TOF repair. Therefore, RV pacing can be beneficial in corrected TOF patients presenting with RV failure.

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.

Myocardial Protection by Blood-Based Del Nido versus St. Thomas Cardioplegia in Cardiac Surgery for Adults and Children.

BACKGROUND: St. Thomas (ST) and Del Nido (DN) cardioplegic solutions are widely used for myocardial protection during cardiac surgery. In 2016, our university hospital shifted from modified St. Thomas to Del Nido solution for both adult and pediatric cardiac surgery. This retrospective study was conducted to compare ST and DN solutions regarding surgical workflow and clinical outcome in pediatric and adult patients undergoing cardiac surgery. METHODS: We reviewed 220 patients who underwent cardiac surgery requiring cardioplegic arrest. Patients were categorized in 2 groups: ST (n = 110) and DN (n = 110). Each group included 60 pediatric and 50 adult patients. Demographic, intraoperative, and postoperative variables were collected. RESULTS: In pediatric patients, no significant difference was found between the 2 groups regarding clamping time, bypass time, need for defibrillation, inotropic score, postoperative ejection fraction (EF), period of mechanical ventilation, intensive care unit stay, or postoperative arrhythmias. One patient in the ST group required mechanical support by extracorporeal membrane oxygenation. We had 5 cases of pediatric mortality (3 in DN and 2 in ST, P = .64). In adult patients, significantly fewer patients in the DN group needed defibrillation than in the ST group. No significant difference was found regarding clamping time, inotropic score, or intraaortic balloon pump use. Mortality in adult patients was 6 cases (4 in ST group and 2 in DN group). CONCLUSION: DN cardioplegia solution is as safe as ST solution in pediatric and adult cardiac surgery. It has comparable results of myocardial protection and clinical outcome, with superiority regarding uninterrupted surgery and lower rate of defibrillation.

Delayed Sternal Closure in Congenital Heart Surgery: A Risk-Benefit Analysis.

BACKGROUND: Delayed sternal closure commonly is used after pediatric cardiac surgery. Its benefits include relieving cardiac compression and stabilizing postoperative critically ill patients. METHODS: We retrospectively reviewed the records of 72 patients, who had undergone delayed sternal closure, among 1,254 patients operated for congenital heart diseases. Indications of delayed sternal closure, perioperative hemodynamic and metabolic status, postoperative infection, and mortality were reported. RESULTS: Transposition of great arteries was the most common preoperative cardiac pathology (26.3%). Bleeding and hemodynamic instability were the most frequent indications for delayed sternal closure, representing 38.8% and 34.7%, respectively. The mean duration of open chest was 3.45 days ± 1.46 days. The mean duration of ICU stay was 20.95 days ± 20.06 days. Two patients had deep sternal wound infection. Sepsis was found in 39 patients (54.1%), and the most common causative organism was coagulase negative (30.5%). ICU stay was a significant risk factor for sepsis (P = .003); duration of open sternum, period of mechanical ventilation (MV), and total hospital stay were not statistically significant risk factors. Sternal closure time (SCT) was affected by period of hemodynamic instability (P = .036). Bypass time, clamping time, and nonsurgical bleeding did not significantly affect SCT. The mortality rate was 15.2% (N = 11). CONCLUSION: Delayed sternal closure is a simple and effective technique that could prevent postoperative cardiac compression in hemodynamic instability states after pediatric cardiac operations.

Factors affecting the outcome of extracorporeal membrane oxygenation following paediatric cardiac surgery.

BACKGROUND: Extracorporeal membrane oxygenation has been widely used after paediatric cardiac surgery due to increasing complex surgical repairs in neonates and infants having complex CHDs. MATERIALS AND METHODS: We reviewed retrospectively the medical records of all patients with CHD requiring corrective or palliative cardiac surgery at King Abdulaziz University Hospital that needed extracorporeal membrane oxygenation support between November 2015 and November 2018. RESULTS: The extracorporeal membrane oxygenation population was 30 patients, which represented 4% of 746 children who had cardiac surgery during this period. The patients' age range was from 1 day to 20.33 years, with a median age of 6.5 months. Median weight was 5 kg (range from 2 to 53 kg). Twenty patients were successfully decannulated (66.67%), and 12 patients (40%) were survived to hospital discharge. Patients with biventricular repair tended to have better survival rate compared with those with single ventricle palliation (55.55 versus 16.66%, p-value 0.058). During the first 24 hours of extracorporeal membrane oxygenation support, the flow rate was significantly reduced after 4 hours of extracorporeal membrane oxygenation connection in successfully decannulated patients. CONCLUSION: Survival to hospital discharge in patients requiring extracorporeal membrane oxygenation support after paediatric cardiac surgery was better in those who underwent biventricular repair than in those who had univentricular palliation. Capillary leak on extracorporeal membrane oxygenation could be a risk of mortality in patients after paediatric cardiac surgery.

Coronary-pulmonary arterial fistula in a neonate with pulmonary atresia-ventricular septal defect and single coronary artery.

In cases of pulmonary atresia with ventricular septal defect (PA-VSD), coronary-pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA-VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS). Successful surgical correction with VSD closure and right ventricle (RV) to the pulmonary artery (PA) conduit was made.

Early Ventricular Dysfunction After Anthracycline Chemotherapy in Children.

The aim of this study was to determine the effect of induction dose of anthracycline chemotherapy on the biventricular function among children with acute hematological malignancies (AHM) using tissue Doppler imaging (TDI) and 2D speckle tracking echocardiography (2D-STE). Thirty pediatric patients with AHM and a mean age of 9.24 ± 4.14 years performed conventional echocardiography, TDI and 2D-STE. After induction chemotherapy, the RV showed mainly a diastolic alteration in its function manifested in significant reduction in the tricuspid TDI-derived E' and E'/A' ratio compared with the baseline (20.40 ± 3.81 vs. 17.47 ± 3.87 cm/s, p = 0.001, 1.29 ± 0.27 vs. 1.03 ± 0.37, p < 0.01, respectively), while the TDI-derived RV MPI and isovolumetric relaxation time of RV were significantly increased (0.32 ± 0.06 vs. 0.36 ± 0.08, p < 0.01, 24.73 ± 8.62 vs. 28.47 ± 11.51 ms, p < 0.05, respectively). The LV showed post-chemotherapy mainly an alteration in its longitudinal systolic function in the form of a reduction in MAPSE (13.61 ± 2.00 vs. 11.95 ± 1.75 mm; p < 0.001), TDI-derived systolic velocity of lateral mitral annulus (10.98 ± 2.34 vs. 10.03 ± 1.83 cm/s, p < 0.05), 2D-STE-derived global longitudinal strain (-21.58 ± 2.54 vs. -19.18 ± 3.59 %, p = 0.001) and 2D-STE-derived global longitudinal strain rate (-1.76 ± 0.22 vs. 1.55 ± 0.29 1/s, p < 0.05), with preservation of LV diastolic function when compared to baseline. TDI and 2D-STE could be used for early detection of anthracycline-induced cardiotoxicity in the pediatric age group. Early after induction chemotherapy, the RV develops mainly diastolic dysfunction, while the LV showed a relative longitudinal systolic impairment.

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.

Clinical deterioration following arterial switch surgery due to extensive aortopulmonary collaterals, unusual condition but worth considering: case report.

BACKGROUND: The occurrence of major aortopulmonary collateral arteries (MAPCAs) is infrequent in patients with D-transposition of great arteries (D-TGA) with intact ventricular septum (IVS). Hemodynamically significant MAPCAs may complicate the postoperative course of these patients after arterial switch operation (ASO). CASE PRESENTATION: We present a rare case of neonatal D-TGA-IVS associated with extensive MAPCAs. After the ASO, the patient developed pulmonary hemorrhage, chest wall edema, and deterioration of lung compliance with the need for high-frequency ventilation (HFV). The patient also had a significant capillary leak with skin edema, high chest tube drainage, and high peritoneal drainage. Cardiac catheterization revealed extensive MAPCAs supplying the whole lung segments. After the catheter closure of most of these MAPCAs, the patient had clinical improvement. CONCLUSIONS: Although the occurrence of MAPCAs with D-TGA-IVS is infrequent, clinicians should suspect their presence in cases with unexplained heart failure, pulmonary hemorrhage, or cardiovascular compromise after ASO. Catheter closure of MAPCAs is feasible with an acceptable short-term outcome.