Exceptional case of aortic atresia associated with atypical double aortic arch.

We present the unique case of atretic aortic valve associated with hypoplastic ascending aorta and double aortic arch of unusual configuration supplying retrograde blood flow to the coronary arteries.

Paediatric and adult congenital cardiology education and training in Europe.

BACKGROUND: Limited data exist on training of European paediatric and adult congenital cardiologists. METHODS: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. RESULTS: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41). CONCLUSION: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

Antiarrhythmic Medication in Neonates and Infants with Supraventricular Tachycardia.

Supraventricular tachycardia (SVT) is the most common arrhythmia in neonates and infants, and pharmacological therapy is recommended to prevent recurrent episodes. This retrospective study aims to describe and analyze the practice patterns, effectiveness, and outcome of drug therapy for SVT in patients within the first year of life. Among the 67 patients analyzed, 48 presented with atrioventricular re-entrant tachycardia, 18 with focal atrial, and one with atrioventricular nodal re-entrant. Fetal tachycardia was reported in 27%. Antiarrhythmic treatment consisted of beta-receptor blocking agents in 42 patients, propafenone in 20, amiodarone in 20, and digoxin in 5. Arrhythmia control was achieved with single drug therapy in 70% of the patients, 21% needed dual therapy, and 6% triple. Propafenone was discontinued in 7 infants due to widening of the QRS complex. After 12 months (6-60), 75% of surviving patients were tachycardia-free and discontinued prophylactic treatment. Patients with fetal tachycardia had a significantly higher risk of persistent tachycardia (p: 0.007). Prophylactic antiarrhythmic medication for SVT in infancy is safe and well tolerated. Arrhythmia control is often achieved with single medication, and after cessation, most patients are free of arrhythmias. Infants with SVT and a history of fetal tachycardia are more prone to suffer from persistent SVT and relapses after cessation of prophylactic antiarrhythmic medication than infants with the first episode of SVT after birth.

Extracorporeal membrane oxygenation support in pediatrics.

Extracorporeal membrane oxygenation (ECMO) is a general term that describes the short- or long-term support of the heart and/or lungs in neonates, children and adults. Due to favorable results and a steady decline in absolute contraindications, its use is increasing worldwide. Indications in children differ from those in adults. The ECMO circuit as well as cannulation strategies also are individualized, considering their implications in children. The aim of this article is to review the clinical indications, different circuits, and cannulation strategies for ECMO. We also present our institutional experience with 92 pediatric ECMO patients (34 neonates, 58 pediatric) with the majority (80%) of veno-arterial placements between 2014 until 2018. We further to also highlight ECMO use in the setting of cardiac arrest [extracorporeal cardiopulmonary resuscitation (CPR) or eCPR].

Prenatal Diagnosis of Single Ventricle Physiology Impacts on Cardiac Morbidity and Mortality.

We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10 mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.

Prevention of Low Cardiac Output Syndrome After Pediatric Cardiac Surgery: A Double-Blind Randomized Clinical Pilot Study Comparing Dobutamine and Milrinone.

OBJECTIVES: Dobutamine and milrinone are commonly used after open-heart surgery to prevent or treat low cardiac output syndrome. We sought to compare efficacy and safety of these drugs in pediatric patients. DESIGN: Prospective, single-center, double-blinded, randomized clinical pilot study. SETTING: Tertiary-care university children's hospital postoperative pediatric cardiac ICU. PATIENTS: After written consent, 50 consecutive patients (age, 0.2-14.2 yr; median, 1.2 yr) undergoing open-heart surgery for congenital malformations were included. INTERVENTIONS: After cardiopulmonary bypass, a continuous infusion of either dobutamine or milrinone was administered for the first 36 postoperative hours. Maximum dose: dobutamine 6 µg/kg/min, milrinone 0.75 µg/kg/min. MEASUREMENTS AND MAIN RESULTS: There were no significant differences in demographic data, complexity of surgery, and intraoperative characteristics between the two study groups (dobutamine vs milrinone). Efficacy was defined as need for additional vasoactive support, which did not differ between groups (dobutamine 61% vs milrinone 67%; p = 0.71). Sodium nitroprusside was used more often in the dobutamine group (42% vs 13%; p = 0.019). Systolic blood pressure showed a trend toward higher values in the dobutamine group, whereas both drugs increased heart rate early postoperatively. Echocardiography demonstrated a consistently good cardiac function in both groups. Central venous oxygen saturation, serum lactate levels, urine output, time to chest tube removal, length of mechanical ventilation, ICU, and hospital stay were similar in both groups. Both drugs were well tolerated, no serious adverse events occurred. CONCLUSIONS: Dobutamine and milrinone are safe, well tolerated, and equally effective in prevention of low cardiac output syndrome after pediatric cardiac surgery. The hemodynamic response of the two drugs is comparable. In uncomplicated cases, a trend toward the more cost-saving dobutamine might be anticipated; however, milrinone demonstrated a trend toward higher efficacy in afterload reduction.

Prognostic value of troponin in infants with hypoplastic left heart syndrome between Stage I and II of palliation.

BACKGROUND: The period between stage I and II procedure for treatment of hypoplastic left heart syndrome (HLHS) bears high mortality and morbidity. METHODS: We sought to analyze the prognostic value of Troponin T/I (Trop), a well-recognized marker for myocardial damage and heart failure, for predicting outcome in a retrospective analysis of 70 infants with HLHS at our institution between March 2001 and October 2014. RESULTS: Stage I procedure consisted of Norwood I operation in 35 (50%) and Hybrid-approach in 22 (31%) patients. Palliative care was chosen for 13 (19%) patients. Trop values were collected from clinical charts and were analyzed in relation to the overall outcome. Trop was significantly higher after Norwood I operation in comparison to Hybrid-approach (median 7.1 µg/l (0.7-20.9), vs 1.2 µg/l (0.3-17.9), P < 0.001). Overall mortality of treated patients was 39% (22 patients). Survival was 54% (19 patients) after Norwood and 73% (16 patients) after Hybrid-approach. Independently from the procedure used, maximal Trop and initial lactate values were significantly higher in non-survivors than in survivors, with median Trop of 9 µg/l (0.6-18.8) vs. 3.4 µg/l (0.4-20.9), P 0.007, and median lactate of 3.7 mmol/L (1.6-25) vs. 2.9 mmol/L (0.3-14.6), p 0.03. Reinterventions were required in 17 (30%) patients, 4 (11%) after Norwood and 13 (59%) after Hybrid procedure. No correlation was found between the need for reintervention and Trop levels in the interstage period. CONCLUSIONS: Patients with HLHS have significantly higher Trop levels after Norwood procedure than after Hybrid-approach. Maximal Trop values were related to mortality, but did not correlate with the need for reinterventions.

Cardiac transplantation in a neonate-First case in Switzerland and European overview.

Twenty-four percent of pediatric heart transplantations (pHTx) are carried out in infants. Neonatal heart transplantation is both rarely performed and challenging. We report on a newborn baby girl suffering from cardiac failure due to a huge tumor (24×52 mm) within the free wall of the left ventricle (LV) and subtotal obstruction of the main left bronchus. Following a surgical tumor resection, a Berlin Heart EXCOR left ventricular assist device was implanted as the bridge to the transplantation. In spite of an organ donor/recipient mismatch of >200%, both heart transplantation and the postoperative course were successful. In addition to this case report, the authors also present data from a survey on performed infant and neonatal transplantations in Western Europe. As neonatal heart transplantation is a rare event in Europe, the authors think it is of crucial importance to share this limited experience. We discuss an alternative strategy-namely, palliative surgical correction using the Fontan pathway. The challenges of donor/recipient weight mismatch and the possibilities of overcoming infant donor organ shortage as a postoperative immunosuppressive regimen are discussed as well.

Biventricular intracorporeal ventricular assist device in a 10-year-old child.

PURPOSE: The pediatric population has benefited greatly by the evolution of ventricular assist devices (VADs) leading to miniaturization. Device design like the HeartWare® VAD allows for implantation in smaller patients or even as an implantable biventricular assist device. CASE REPORT: A ten-year-old female patient (body surface area: 1.02 m2, 27 kg) waiting for 7 months for heart transplantation was admitted to the intensive care unit due to terminal heart failure. Transthoracal echocardiography revealed heart failure with an estimated ejection fraction of 15%, dilated left ventricle, moderate tricuspid and mitral regurgitation. Despite maximal medical therapy (including milrinone and 1 course of Levosimendan intravenous), her condition further worsened and mechanical circulatory support was indicated.After implantation of a HeartWare HVAD® device as an LVAD, a second pump was implanted as a right VAD (RVAD). The sewing ring of the HVAD was attached to the lateral wall of the right atrium. The device was implanted so that the axis of the inflow cannula was pointing towards the tricuspid valve. The outflow graft was anastomosed to the pulmonary artery truncus; the outflow graft diameter was not narrowed. Anticoagulation with IV heparin was started 8 hours postoperatively with a target antifactor Xa level of 0.3 to 0.6 IU/ml. The patient was extubated after 26 hours and fully mobilized. BVAD settings were: 2,300 revolutions per minute, calculated flow: 3.2 liters per minute and 2.7 W for the left side and 2,080 revolutions per minute, calculated flow: 3.3 L per minute and 2.1 W for the right side.

Does superior caval vein pressure impact head growth in Fontan circulation?

BACKGROUND: Patients with bidirectional cavopulmonary anastomosis have unphysiologically high superior caval vein pressure as it equals pulmonary artery pressure. Elevated superior caval vein pressure may cause communicating hydrocephalus and macrocephaly. This study analysed whether there exists an association between head circumference and superior caval vein pressure in patients with single ventricle physiology. METHODS: We carried out a retrospective analysis of infants undergoing Fontan completion at our institution from 2007 to 2013. Superior caval vein pressures were measured during routine catheterisation before bidirectional cavopulmonary anastomosis and Fontan completion as well as head circumference, adjusted to longitudinal age-dependent percentiles. RESULTS: We included 74 infants in our study. Median ages at bidirectional cavopulmonary anastomosis and Fontan were 4.8 (1.6-12) and 27.9 (7-40.6) months, respectively. Head circumference showed significant growth from bidirectional cavopulmonary anastomosis until Fontan completion (7th (0-100th) versus 20th (0-100th) percentile). There was no correlation between superior caval vein pressure and head circumference before Fontan (R2=0.001). Children with lower differences in superior caval vein pressures between pre-bidirectional cavopulmonary anastomosis and pre-Fontan catheterisations showed increased growth of head circumference (R2=0.19). CONCLUSIONS: Patients with moderately elevated superior caval vein pressure associated with single ventricle physiology did not have a tendency to develop macrocephaly. There is no correlation between superior caval vein pressure before Fontan and head circumference, but between bidirectional cavopulmonary anastomosis and Fontan head circumference increases significantly. This may be explained by catch-up growth of head circumference in patients with more favourable haemodynamics and concomitant venous pressures in the lower range. Further studies with focus on high superior caval vein pressures are needed to exclude or prove a correlation.

Pediatric heart transplantation.

Pediatric heart transplantation (pHTx) represents a small (14%) but very important and particular part in the field of cardiac transplantation. This treatment has lifelong impact on children. To achieve the best short and especially long-term survival with adequate quality of life, which is of crucial importance for this young patient population, one has to realize and understand the differences with adult HTx. Indication for transplantation, waitlist management including ABO incompatible (ABOi) transplantation and immunosuppression differ. Although young transplant recipients are ultimately likely to be considered for re-transplantation. One has to distinguish between myopathy and complex congenital heart disease (CHD). The differences in anatomy and physiology make the surgical procedure much more complex and create unique challenges. These recipients need a well-organized and educated team with pediatric cardiologists and intensivists, including a high skilled surgeon, which is dedicated to pHTx. Therefore, these types of transplants are best concentrated in specialized centers to achieve promising outcome.

VAD as bridge to recovery in anthracycline-induced cardiomyopathy and HHV6 myocarditis.

This report describes an 8-year-old child with acute anthracycline-induced cardiomyopathy triggered by human herpesvirus 6 and the subsequent implantation of an intracorporeal continuous-flow left ventricular assist device (LVAD) and the process to discharge the child from the hospital. After barely 3 months on mechanical support, the device was explanted after thorough examination. Experiences regarding LVAD removal are limited, and no guidelines for echocardiographic and hemodynamic criteria for LVAD removal in children have been published thus far. We present our institutional algorithm for device selection, surveillance in an ambulatory setting, and testing for myocardial recovery, as well as our criteria for LVAD explantation in children.

Acute chemotherapy-induced cardiomyopathy treated with intracorporeal left ventricular assist device in an 8-year-old child.

Evolution of ventricular assist devices (VADs) leading to miniaturization has made intracorporeal implantation in children feasible. Ventricular assist device therapy for anthracycline-induced cardiomyopathy (CMP) in adults has been reported. We report the case of an 8-year-old child (body surface area 0.97 m) presenting with anthracycline-induced CMP being successfully treated with an intracorporeal left ventricular assist device (LVAD) as a bridge to candidacy/recovery. We present our institutional algorithm, which advises intracorporeal LVAD implantation for long-term ventricular assist, in children with a body surface area >0.6 m. Advantages are better mobilization and the possibility to discharge home, leading to enhanced quality of life.

Outcome of biventricular repair in infants with multiple left heart obstructive lesions.

The decision to perform biventricular repair for infants with multiple obstructive or hypoplastic left heart lesions (LHL) and borderline left ventricle (LV) may be controversial. This study sought to assess the mortality and morbidity of patients with LHL after biventricular repair and to determine the growth of the left-sided cardiac structures. Retrospective analysis of 39 consecutive infants with LHL who underwent biventricular repair was performed. The median age at surgery was 7 days (range 1-225 days), and the median follow-up period was 34 months (range 1-177 months). Between diagnosis and the end of the follow-up period, the size of the aortic annulus (z-score -4.1 ± 2.8 vs. -0.1 ± 2.7) and the LV (LV end-diastolic diameter z-score -1.7 ± 2.8 vs. 0.21 ± 1.7) normalized. During the follow-up period, 23 patients required 39 reinterventions (62%) consisting of redo surgery for 21 patients (57%) and catheter-guided reinterventions for 8 patients (22%). At the end of the follow-up period, 25 of 34 patients were doing subjectively well; 10 children (29%) received cardiac medication; 12 (35%) presented with failure to thrive (weight ≤ P3) and 5 (15%) with pulmonary hypertension. The overall mortality rate was 13%. Biventricular repair for patients with multiple LHL results in sufficient growth of the left-sided cardiac structures. Nevertheless, residual or newly developing obstructive lesions and pulmonary hypertension are frequent, causing significant morbidity that requires reintervention.

Do predictors exist for a successful withdrawal of preoperative prostaglandin E(1) from neonates with d-transposition of the great arteries and intact ventricular septum?

Prostaglandin E(1) (PGE(1)) is given to neonates with d-transposition of the great arteries (d-TGA) to reduce cyanosis by reopening and maintaining the patency of the ductus arteriosus. To avoid side effects, this medication can be stopped for hemodynamically stable patients after balloon atrial septostomy (BAS). A consecutive series of neonates with d-TGA and an intact ventricular septum (IVS) presenting from 2000 through 2005 was analyzed retrospectively to search for side effects of PGE(1) and to identify predictors for a safe preoperative withdrawal. The medication was stopped for hemodynamically stable patients with transcutaneous oxygen saturations higher than 80% after BAS and reinitiated for patients with an oxygen saturation lower than 65%. Patients successfully weaned were compared with those who had failed weaning in terms of atrial septal defect (ASD) size, ductus arteriosus size, and the transcutaneous oxygen saturation. Prostaglandin E(1) was initiated for all 43 neonates with d-TGA. The median maintenance dose of PGE(1) was 0.00625 µg/kg/min (range, 0.00313-0.050 µg/kg/min) for a median duration of 6 days (range, 1-12 days). For 16 patients, PGE(1) was preoperatively withdrawn but then had to be reinitiated for 7 of the 16 patients. No predictors for a successful weaning of PGE(1) were found based on ASD size, ductus arteriosus size, or oxygen saturation. The adverse effects of PGE(1) were apnea in 10 patients and fever in 19 patients. Neither seizures nor necrotizing enterocolitis was documented. Prostaglandin E(1) was successfully withdrawn for a minority of hemodynamically stable patients with d-TGA. No predictors for a successful weaning could be identified. Because apnea and fever are common side effects, withdrawal of PGE(1) after BAS may improve patient safety and comfort. In this patient group, if PGE(1) withdrawal was not well tolerated, it could be safely reinitiated. There were no serious side effects of PGE(1.).

Candida mediastinitis with aortic rupture after congenital heart surgery.

We describe the fatal course of a Candida albicans mediastinitis in a 4-year-old child after truncus arteriosus repair, leading to aortic rupture. The diagnosis was made by computer tomography and confirmed at exploratory sternotomy, with positive swab and blood cultures. After local surgical lavage, Amphotericin B therapy was started, but the child died due to aortic rupture 2 weeks later, despite negative blood cultures. The potential risk of aortic rupture with Candida mediastinitis after congenital heart repair exists, and combined aggressive surgical and medical treatment is essential. Guidelines for the diagnosis and therapy in pediatric patients with post-operative mycotic mediastinitis are required.