Anna Cavigelli-Brunner

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 m2) 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 m2. Advantages are better mobilization and the possibility to discharge home, leading to enhanced quality of life.

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.

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.

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.

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.

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.

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.