Patient-tailored platelet transfusion practices for children supported by extracorporeal membrane oxygenation.

BACKGROUND AND OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) serves as cardiopulmonary therapy in critically ill patients with respiratory/heart failure and often necessitates multiple blood product transfusions. The administration of platelet transfusions during ECMO is triggered by the presence or risk of significant bleeding. Most paediatric ECMO programmes follow guidelines that recommend a platelet transfusion threshold of 80-100 × 109/L. To reduce exposure to platelets, we developed a practice to dynamically lower the threshold to ~20 × 109/L. We describe our experience with patient-tailored platelet thresholds and related bleeding outcomes. MATERIALS AND METHODS: We retrospectively evaluated our platelet transfusion policy, bleeding complications and patient outcome in 229 ECMO-supported paediatric patients in our unit. RESULTS: We found that more than 97.4% of patients had a platelet count <100 × 109/L at some point during their ECMO course. Platelets were transfused only on 28.5% of ECMO days; and 19.2% of patients never required a platelet transfusion. The median lowest platelet count in children who had bleeding events was 25 × 109/L as compared to 33 × 109/L in children who did not bleed (p < 0.001). Our patients received fewer platelet transfusions and did not require more red blood cell transfusions, nor did they experience more haemorrhagic complications. CONCLUSION: We have shown that a restrictive, 'patient-tailored' rather than 'goal-directed' platelet transfusion policy is feasible and safe, which can greatly reduce the use of platelet products. Although there was a difference in the lowest platelet counts in children who bled versus those who did not, the median counts were much lower than current recommendations.

Three-Patch Reconstruction of Hourglass Supravalvar Pulmonary Artery Stenosis Mid-Term Results.

BACKGROUND: Some patients with pulmonary stenosis present with a complex multilevel disease that involves the valve, the leaflets, and the sinotubular junction (STJ) forming an hourglass appearance. We herein report the mid-term results of our experience with the reconstruction of the supravalvar narrowing using three pericardial patches. MATERIAL AND METHODS: Retrospective analysis of patient charts and echocardiography studies of patients who underwent three-patch reconstruction of the pulmonary valve (PV) from 2013 to 2022. After PV transection distal to STJ, vertical incisions into the sinuses were performed, and leaflets were trimmed and thinned. The three sinuses were augmented using three pericardial patches. RESULTS: Nineteen patients underwent repair of hourglass supravalvar pulmonary stenosis. Mean weight at surgery was 9.4 kg (median 7.2, range 4.7-35); 16 patients underwent previous catheterization with unsuccessful balloon dilatation of the PV (13 pts.). Preoperative aortic/pulmonary annulus ratio was 1.02 (median 1, range 0.89-1.25). After surgery, gradients across the PV were significantly reduced (94 ± 26 vs 29 ± 9 mm Hg, P = .02). Postoperatively, 14 patients had mild or no pulmonary insufficiency (PI) and five had mild to moderate PI. At a mean follow-up of 71 months (median 78 months, range 8-137), gradients continued to decrease (29 ± 9 vs 15 ± 5 mm Hg, P < .001). CONCLUSION: The three-patch technique for the repair of supravalvar pulmonary stenosis is simple, reproducible, and achieves excellent and long-standing relief of the right ventricular outflow tract gradient.

Pediatric Mechanical Circulatory Support: Introduction and Schneider's Experience.

BACKGROUND: Ventricular assist devices (VADs) play a critical and increasing role in treating end-stage heart failure in pediatric patients. A growing number of patients are supported by VADs as a bridge to heart transplantation. Experience with VADs in the pediatric population is limited, and experience in Israel has not been published. OBJECTIVES: To describe this life-saving technology and our experience with VAD implantation in children with heart failure, including characteristics and outcomes. METHODS: We conducted a retrospective chart review of all patients who underwent VAD implantation at Schneider Children's Medical Center from 2018 to 2023. RESULTS: We analyzed results of 15 children who underwent VAD implantation. The youngest was 2.5 years old and weighed 11 kg at implantation. In eight patients, HeartMate 3, a continuous-flow device, was implanted. Seven patients received Berlin Heart, a pulsatile-flow device. Three children required biventricular support; 11 underwent heart transplants after a median duration of 169 days. Two patients died due to complications while awaiting a transplant; two were still on VAD support at the time of submission of this article. Successful VAD support was achieved in 86.6% of patients. In the last 5 years,79% of our heart transplant patients received VAD support prior to transplant. CONCLUSIONS: Circulatory assist devices are an excellent bridge to transplantation for pediatric patients reaching end-stage heart failure. VADs should be carefully selected, and implantation techniques tailored to patient's weight and diagnosis at a centralized pediatric cardiac transplantation center. Israeli healthcare providers should be cognizant of this therapeutic alternative.

Transthoracic intracardiac lines-A double edged sword.

OBJECTIVE: Routine use of central venous access is needed in children undergoing open heart surgery for pressure monitoring and inotrope infusion. We sought to evaluate the efficiency and safety of routine use of transthoracic intracardiac lines (ICLs) in patients undergoing cardiac surgery and to compare them to patients who have been previously treated with traditional central venous lines (non-ICLs). METHODS: Retrospective review of charts of all patients who underwent cardiac surgery and had an ICL inserted in the operating room. Case control matching was done with similar patient in which ICL was not inserted. Patients characteristics, diagnosis, operative, and intensive care data were collected for each patient and analyzed. RESULTS: A total number of 376 patient records were reviewed (198 ICL patients and 178 non-ICL patients). Umbilical line and non-ICL durations were longer in the non-ICL group. ICL duration was the longest of all lines, averaging 12.87 ± 10.82 days. The necessity for multiple line insertions (˃2 insertions) was significantly higher in the non-ICL group, with a relative risk ratio of 3.24 (95% confidence interval: 1.617-6.428). There was no statistical difference of infections rate and line complications between the two groups. CONCLUSION: ICLs are safe in infants undergoing cardiac surgery and can be kept in place for a long period of time with a low rate of line complications and infection. Routine use of ICLs reduces the number of central venous catheter placement in this complex patient population.

Mid-term results of vascular ring surgery.

BACKGROUND: Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. METHODS: Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time. RESULTS: Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. CONCLUSIONS: Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

Clinical Features and Comparison of Kingella and Non-Kingella Endocarditis in Children, Israel.

Kingella spp. have emerged as an important cause of invasive pediatric diseases. Data on Kingella infective endocarditis (KIE) in children are scarce. We compared the clinical features of pediatric KIE cases with those of Streptococcus species IE (StIE) and Staphylococcus aureus IE (SaIE). A total of 60 patients were included in the study. Throughout the study period, a rise in incidence of KIE was noted. KIE patients were significantly younger than those with StIE and SaIE, were predominately boys, and had higher temperature at admission, history of oral aphthae before IE diagnosis, and higher lymphocyte count (p<0.05). Pediatric KIE exhibits unique features compared with StIE and SaIE. Therefore, in young healthy children <36 months of age, especially boys, with or without a congenital heart defect, with a recent history of oral aphthae, and experiencing signs and symptoms compatible with endocarditis, Kingella should be suspected as the causative pathogen.

Extracorporeal Membrane Oxygenation as a Rescue Therapy for Postoperative Diastolic Dysfunction and Refractory Chylothorax.

This is the first published case, as far as we know, of a term neonate with refractory chylothorax secondary to diastolic dysfunction in the cardiac postoperative period, where extracorporeal membrane oxygenation (ECMO) was used to improve the physiologic derangements, thus allowing resolution of the chylous effusion. The infant was prenatally diagnosed with d-transposition of the great arteries. He was started on prostaglandin infusion and underwent balloon atrial septostomy followed by arterial switch operation. After surgery, he developed anasarca and high-volume chylothorax that did not respond to medical management and fasting. Cardiac catheterization demonstrated severe diastolic dysfunction and pulmonary hypertension. On postoperative day 19, he was placed on veno-arterial (VA) ECMO and had gradual regression of the chylothorax and edema. After 13 days on ECMO support, he was decannulated with small, self-limiting, reaccumulation of chylous effusion. He was discharged home on postoperative day 57, and has since been thriving with no evidence of reaccumulation of the chylous effusion. In summary, VA ECMO support could be considered as a rescue modality for patients with uncontrollable refractory high-volume chylous effusion, after other treatment options have been pursued.

Transposition of the Great Arteries-Are We Doing Better? Correlating Outcome to Change in Renal Function Over 2 Decades of Arterial Switch Operation.

OBJECTIVES: It is believed that management of neonates with dextro-transposition of the great arteries is constantly improving. Renal function may play a role in the prognosis of patients after congenital heart surgery. The aim of this study was to describe the outcome of neonates who underwent arterial switch operation during the past 2 decades using renal function as a surrogate marker for morbidity and mortality. DESIGN: Retrospective cohort study. SETTING: Dedicated cardiac ICU of a university-affiliated pediatric medical center. PATIENTS: Infants who underwent arterial switch surgery in 1993-2015. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The cohort included 336 infants who underwent arterial switch operation for dextro-transposition of the great arteries (n = 169, 50%), transposition of the great arteries/ventricular septal defect (n = 133, 40%), or Taussig-Bing anomaly (n = 34, 10%). Between 1993-1998 and 2012-2015, the mean minimal postoperative estimated glomerular filtration rate rose from 30 mL/min/1.73 m to 40 mL/min/1.73 m (p < 0.05), and the proportion of patients with estimated glomerular filtration rate less than 30 mL/min/1.73 m decreased from 56% to 23% (p < 0.05). The daily furosemide dosage decreased from 4 mg/kg/d to 0.5 mg/kg/d (p < 0.05). Urinary output on operative day 0 decreased over time, but urinary output on operative day 2 significantly increased. Maximal lactate levels and time to lactate normalization decreased steadily. Dialysis was performed in only a few patients in the early periods, and in none in the last 6 years. The mean mortality rate of patients with dextro-transposition of the great arteries and transposition of the great arteries/ventricular septal defect decreased to 2.7% in the last 6 years. The odds ratio of a prolonged hospital stay (≥ 28 d) in a patient with estimated glomerular filtration rate less than 30 mL/min/1.73 m was 18.79, and in a patient with transposition of the great arteries/ventricular septal defect, 3.39. The odds ratio of dying after Rashkind atrial septostomy was 4.42. CONCLUSIONS: During the past 2 decades, there has been significant improvement in outcome of patients undergoing transposition of the great arteries repair. Renal function was found to be a good prognostic marker of morbidity and mortality.

The Utility of Albumin Level as a Marker of Postoperative Course in Infants Undergoing Repair of Congenital Heart Disease.

We sought to examine the role of preoperative and 2nd postoperative day albumin levels as predictors for postoperative course in infants undergoing repair of congenital heart disease. This retrospective, single-center, observational study comprised consecutive infants younger than 1 year who had undergone repair of tetralogy of Fallot, ventricular septal defect, complete atrioventricular canal or transposition of the great arteries over a 25 months period. We correlated preoperative and postoperative day (POD) #2 albumin level to vaso-inotropic score (VIS) and intensive care unit (ICU) length of stay (LOS) as markers for degree and duration of postoperative cardiac support. A composite outcome was defined as maximal vaso-inotropic score of > 10 and ICU LOS > 96 h. Preoperative albumin level negatively correlated with VIS and ICU LOS. Compared to preoperative albumin level of > 4 g/dL, the relative risk of meeting composite criteria was 1.5 for preoperative albumin of 3.1-4 g/dL and 2.6 for preoperative albumin ≤ 3 g/dL. Compared to POD#2 albumin level > 3 g/dL, the relative risk of meeting composite criteria was 1.8 for albumin of 2.6-3 g/dL, and 2.5 for albumin ≤ 2.5 g/dL. In summary, we found that preoperative and POD#2 albumin levels predicted prolonged and complicated postoperative course. These finding may help clinicians to inform the patient's parents, early in the ICU hospitalization, as to the predicted risks and difficulties of their infant's postoperative course.

Pediatric Extracorporeal Membrane Oxygenation Reach-Out Program: Successes and Insights.

The shortage of dedicated pediatric extracorporeal membrane oxygenation (ECMO) centers and the expanding indications for pediatric ECMO necessitate a regional program for transport of ECMO-supported patients. Data about feasibly and safety of pediatric ECMO transport are scarce. Our aim is to describe our experience with a pediatric ECMO reach-out program and review pertinent literature. Demographic, clinical, and outcome data were collected retrospectively from the charts of all patients cannulated onto ECMO at referring centers and transported to our center from 2003 to 2018. Similar data were recorded for patients who were referred for ECMO support from within the hospital. The cohort included 80 patients cannulated at 17 referring centers. The transport team included a senior pediatric cardiac surgeon and an ECMO specialist. All transfers but one were done by special emergency medical service ambulance. No major complications or deaths occurred during transport, and all patients were stable upon arrival to our unit. Mortality was lower in the ECMO reach-out cohort than in-house patients referred for ECMO support. This is the first study from Israel and one of the largest to date describing a dedicated pediatric ECMO transport program. Extracorporeal membrane oxygenation transport appears to be feasible and safe when conducted by a small, highly skilled mobile team. Successful reach-out program requires open communication between the referring physician and the accepting center. As survival correlates with ECMO volume, maintaining a large ECMO center with 24/7 retrieval capabilities may be the best strategy for pediatric mechanical circulatory support program.

Alveolar Dead-Space Fraction and Arterial Saturation Predict Postoperative Course in Fontan Patients.

OBJECTIVES: Fontan surgery, the final surgical stage in single ventricle palliation, redirects systemic venous blood into the pulmonary circulation for gas exchange. A decrease in pulmonary blood flow can lead to major complications and grave outcomes. Alveolar dead-space fraction represents the portion of inhaled air that does not participate in gas exchange and hence quantifies ventilation-perfusion abnormalities in the lung. Increased alveolar dead-space fraction has been associated with prolonged mechanical ventilation and worse outcome after congenital heart surgery. The association of alveolar dead-space fraction with clinical outcomes in patients undergoing Fontan operation has not been reported. INTERVENTIONS: None. DESIGN, SETTING, AND PATIENTS: A retrospective charts review of all pediatric patients who underwent Fontan surgery during June 2010-November 2018 in a tertiary-care pediatric hospital. Associations between alveolar dead-space fraction and arterial oxyhemoglobin saturation to a composite outcome (surgical or catheter-based intervention, extracorporeal membrane oxygenation use, prolonged ventilation, prolonged hospital length of stay, or death) were explored. Secondary endpoints were parameters of severity of illness, chest drainage duration, and length of stay. MEASUREMENTS AND MAIN RESULTS: Of 128 patients undergoing Fontan operation, 34 met criteria for composite outcome. Alveolar dead-space fraction was significantly higher in the composite (0.33 ± 0.14) versus control (0.25 ± 0.26; p = 0.016) group. Alveolar dead-space fraction greater than or equal to 0.29 indicated a 37% increase in risk to meet composite criteria. Admission arterial oxygen saturation was significantly lower in composite versus control group (93.4% vs 97.1%; p = 0.005). Alveolar dead-space fraction was significantly associated with increased durations of mechanical ventilation, ICU length of stay, duration of thoracic drainage, and parameters of severity of illness. CONCLUSIONS: Alveolar dead-space fraction and arterial saturation may predict complicated postoperative course in patients undergoing the Fontan operation.

Urgent Surgical Treatment of Aortic Endocarditis in Infants and Children.

Infective endocarditis (IE) in the pediatric population can present as a life-threatening condition. Optimal timing for surgical intervention should consider surgical risks versus the risk of neurologic complications. We herein report our experience with this group of critically ill children. Retrospective analysis of patient charts of all patients who underwent urgent surgical treatment of aortic IE from 1994 to 2014 was performed. Nine patients with acute storming IE of the aortic valve or the ascending aorta were urgently operated (eight normal heart, one congenital aortic stenosis), age ranged from 8 weeks to 4.2 years. Causative organisms were Staphylococcus aureus (2), Staphylococcus coagulase negative (1), Kingella kingae (2), Streptococcus pneumoniae (2), or culture negative (2). Presenting symptoms other than hemodynamic instability were neurologic decompensation (5) coronary embolization (1) and cardiogenic shock due to scalded skin syndrome (1). CT and MRI demonstrated significant brain infarcts in four patients. Operations performed were the Ross operation (7) and ascending aortic reconstruction (2). There were no operative deaths. At a median follow-up of 6 years (range 2-196 months), all patients are alive and well. Re-intervention included homograft replacement (2) and transcatheter Melody valve implantation (1). At their last follow-up, the neo-aortic valve was functional in all patients with minimal regurgitation and all had full resolution of the neurological deficits. Urgent surgical treatment for aortic valve IE in infants is challenging. Although surgery is complex and pre-disposing conditions such as sepsis, neurologic and cardiac decompensations are prevalent, operative results are excellent and gradual and significant neurologic improvement was noted over time.

Neonatal Cardiac Surgery in the New Era: Lessons learned from 1000 Consecutive Cases.

BACKGROUND: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. OBJECTIVES: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. METHODS: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel. RESULTS: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%). CONCLUSIONS: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members' contributions.

Percutaneous intraperitoneal catheters in neonates following open heart surgery.

OBJECTIVE: Ascitic fluid in the peritoneal cavity may severely impair respiratory and renal function following neonatal heart surgery. It has been our practice to liberally insert percutaneous peritoneal catheters (PPCs) in order to manage fluid balance and thereby improve ventilatory function. We herein report our experience with PPC. METHODS: Retrospective analysis of charts of all surviving neonates that underwent PPC insertion from January 2007 through March 2010. Charts were reviewed for demographic and clinical variables from the preoperative, operative, and postoperative periods. RESULTS: A total of 1268 patients underwent surgery, 292 (23%) were neonates. 17 (5.8%) patients required PPC. Mean age and weight were 16 days and 3.1 kg, respectively. Mean amount drained upon insertion was 55 ± 46 ml. Catheters were maintained for a mean of 5 days and drained an average of 201 ml on the first postinsertion day. Ventilatory settings did not change significantly prior to and postcatheter insertion (respiratory rate [29 ± 3.8 vs 28.7 ± 3.9; P = .93], inspiratory pressures [26.3 ± 3.6 vs 26.1 ± 3.3 cm H2O; P = .34], and fraction of inspired oxygen [0.66 ± 0.21 vs 0.63 ± 0.18; P = .53]). Carbon dioxide values decreased significantly (43.2 ± 9.7 vs 37 ± 4.9 mm Hg; P = .01), and PO2 values increased (78 ± 69 vs 104 ± 57 mm Hg; P = .05). CONCLUSIONS: The PPC insertion can be easily performed at the bedside with minimal complications. Fluid balance management is facilitated, and ventilation is improved. The PPC insertion is a valuable addition to the armamentarium of the physician treating neonates in the intensive care unit after complex congenital heart surgery.

Direct innominate artery cannulation for antegrade cerebral perfusion in neonates undergoing arch reconstruction.

BACKGROUND: Antegrade cerebral perfusion (ACP) is performed in neonates either by direct cannulation (DC) or indirect cannulation (IC) of the innominate artery. IC is achieved by a graft sutured to the innominate artery or advancement of a cannula through the ascending aorta into the innominate artery, whereas DC is performed by directly cannulating the innominate artery. These techniques may be limited by technical problems that can compromise perfusion. The purpose of the present study was to evaluate the flow measurements and safety of DC when compared with IC. METHODS: This was a retrospective chart review of consecutive neonates who underwent ACP from January 2007 to December 2010. Patient characteristics, surgical and hemodynamic measurements, and postoperative neurologic findings were recorded. RESULTS: Seventy neonates underwent ACP during the study period (46 using DC and 24 using IC). The groups were similar in age and weight. Operative variables were similar regarding cardiopulmonary bypass (CPB), cross-clamp times, maximal flow at full CPB, minimal temperature, ACP time, flow and flow index, and upper extremity blood pressure and proximal cannula pressure during ACP. There was a significantly higher flow index at full CPB in the DC group (217 ± 40 mL/kg/min versus 190 ± 46 mL/kg/min; p = 0.013), which correlated with higher proximal cannula pressures at full CPB (172 ± 27 mm Hg versus 158 ± 26 mm Hg; p = 0.04). Sixty-two of the 65 survivors (95%) had normal neurologic evaluations on discharge. CONCLUSIONS: ACP using DC is comparable to that using IC, with appropriate pressures in the proximal aortic line at full CPB and adequate upper extremity pressures during ACP, reflecting suitable flows in the cerebral circulation.

Anomalous origin of the pulmonary artery from the aorta: early diagnosis and repair leading to immediate physiological correction.

INTRODUCTION: Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction. MATERIALS AND METHODS: Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Children's Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed. RESULTS: Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operative mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised. CONCLUSIONS: Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.