Eugen Sandica’s research while affiliated with Herz- und Diabeteszentrum Nordrhein-Westfalen and other places

Background The aim of this analysis was to clarify the complexity of CHD in neonates, which are referred to a high-volume pediatric heart center. Does mode or place of delivery within different CHD types influence outcome parameters. Methods Observation period for this analysis was 10 years (2011–2021). All neonates younger 28 days with CHD were included and divided into 5 groups according to expected hemodynamic instability using the AHA Level of Care (LoC) Assignment. Results Of 1210 neonates, 825 met the inclusion criteria. 397 infants were delivered locally (group A). 428 neonates were transferred from distant hospitals (> 15 km) (group B). The predominant LoC grades were LoC 2 (46%) and LoC 1 (28%). LoC grades 3, 4 and P (palliative care) accounted for 26%. A prenatal diagnosis was available in 54% of cases. The concordance rate with postnatal diagnosis was 88%. 92% of locally delivered infants had a prenatal diagnosis, but only 19% of transferred newborns. There were no differences in mode of delivery, birth weight, and APGAR scores between the different delivery locations. The proportion of univentricular heart defects was 51% in group A at LOC 2 and only 24% in group B. The overall mortality rate was 8.4% at LOC 2, with the highest mortality rate of 12.8% at LOC 4. Conclusion 25% of our neonates fulfilled the LoC criteria of an increased risk for hemodynamic instability (LoC 3–4). Comparative studies show no differences in perinatal outcomes. Newborns with single ventricle morphology have an increased mortality rate.

OBJECTIVES The use of ventricular assist devices in children is increasing. However, absolute numbers in individual centers and countries remain small. Collaborative efforts such as the Paedi-EUROMACS are therefore essential in order to combine international experience with paediatric ventricular assist devices. In this paper, the results from the fourth Paedi-EUROMACS report are presented. METHODS All paediatric (<19 years) patients supported by a ventricular assist device from the EUROMACS database were included. Patients are stratified into a congenital heart disease group and a group with a non-congenital aetiology. Endpoints included mortality, transplantation and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident, and pump thrombosis. RESULTS 590 primary implantations were included. The congenital group was significantly younger (2.5 versus 8.0 years respectively, p < 0.001) and were more commonly supported by a pulsatile flow device (73.5% vs 59.9%, p < 0.001). Mortality was significantly higher in the congenital group (30.8 vs 20.4%; p = 0.009) than in the non-congenital group. However, in multivariable analyses, congenital heart disease was not significantly associated with mortality (HR 1.285, CI 0.8111–2.036, p = 0.740). Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with BSA (HR 0.524 CI 0.333–0.823, p = 0.005), congenital heart disease (HR 1.641 CI 1.054–2.555, p = 0.028) and pulsatile flow support (HR 2.345 CI 1.406–3.910, p = 0.001) in multivariable analyses. CONCLUSIONS This fourth Paedi-EUROMACS report highlights the increasing use of paediatric ventricular assist devices. The patient populations with congenital and non-congenital aetiology exhibit distinct characteristics and clinical outcomes.

Objectives: Restrictive cardiomyopathy is rare and is generally associated with worse clinical outcomes compared to other cardiomyopathies. Ventricular assist device (VAD) support for these children is seldom applied and often hampered by the surgical difficulties. Methods: All paediatric (<19 years) patients with a restricted cardiomyopathy supported by a VAD from the EUROMACS database were included and compared to patients with a dilated cardiomyopathy (retrospective database analyses). Participating centres were retrospectively contacted to provide additional detailed echo and Swan Ganz measurements to analyse the effect of VAD support on pulmonary artery pressure and right ventricular function. Results: Forty-four paediatric VAD-supported patients diagnosed with restricted cardiomyopathy were included, with a median age at implantation of 5.0 years. Twenty-six of the 44 patient with a restricted cardiomyopathy survived to transplantation (59.1%), 16 died (36.4%) and 2 are still on ongoing VAD support (4.5%) after a median duration of support of 95.5 days (interquartile range 33.3-217.8). Transplantation probability after 1 and 2 years of VAD support in patients with a restricted cardiomyopathy were comparable to patients with a dilated cardiomyopathy (52.3% vs 51.4% and 59.5% vs 60.1%, P = 0.868). However, mortality probability was higher in the restricted cardiomyopathy cohort (35.8% vs 17.0% and 35.8% vs 19.0%, P = 0.005). Adverse event rates were high (cerebrovascular accident in 31.8%, pump thrombosis in 29.5%, major bleeding 25.0%, eventual biventricular support in 59.1%). In the atrially cannulated group, cerebrovascular accident and pump thrombosis occurred in twice as much patients (21.1% vs 40.0%, P = 0.595 and 15.8% vs 40.0%, P = 0.464; probably non-significant due to the small numbers). Pulmonary arterial pressures improved after implantation of a VAD, and 6 patients who were initially labelled as ineligible due to pulmonary hypertension could eventually be transplanted. Conclusions: VAD support in children with a restricted cardiomyopathy is rarely performed. Mortality and adverse event rates are high. On the other hand, survival to cardiac transplantation was 59.1% with all patients surviving the 1st 30 days after cardiac transplantation. Pulmonary arterial pressures improved while on support, potentially making cardiac transplantation a viable option for previously ineligible children.

Background EXCOR ventricular assist device (VAD) is the gold standard circulatory support for children with end-stage heart failure. Until recently, the only available driving unit was the stationary Ikus. This study (NCT04634708) investigates the impact of the novel mobile EXCOR Active driving unit on patients’ mobility and the quality of life (QoL) of their families. Methods Children on EXCOR VAD support with the Ikus who were mobilizable outside the hospital room were eligible for the prospective study. Patients remained on Ikus for 7 days, then switched to Excor Active, and were observed for another 45 days. The end-points were the rates of clinically relevant device exchanges (i.e., exchanges that could raise safety concerns for the patient) and successful patient outcomes, respectively. QoL of patients’ families was recorded through 2 validated questionnaires: the Pediatric Quality of Life Inventory Family Impact Module and Depression, Anxiety and Stress Scale 21. Patients’ mobility and activity levels were monitored through diary entries. Results A total of 24 patients were enrolled of which 23 patients completed the study. No clinically relevant device exchanges occurred and there was a successful outcome in 22/23 (95.7%) patients. Changing from the Ikus to the EXCOR Active improved QoL as seen in family impact total score (from 53.8 ± 19.8-66.5 ± 20.8, p = 0.005) and mental health (depression: moderate to mild, stress moderate to normal, both p < 0.05; anxiety: mild to normal, n.s.). Activity levels raised in both, mean time (from 100-300 min/day, p = 0.011) and distance (from 30-1,110 m/day, p = 0.002). Conclusions The EXCOR Active driving unit is safe and performs equivalent to Ikus. This study indicates considerable improvement in patients’ mobility. In addition, data suggest that families’ QoL is positively affected by means of the novel driving unit.

Background Increasing regulations and requirements of advisory bodies, in particular the Joint Federal Committee and the Medical Service of the health insurance funds, make it necessary to employ only demonstrably well-trained perfusionists. The minimum requirement for this staff is EBCP certification. Currently there is limited availability of such specialists on the German market. Therefore, the qualification of young people in this area is of central importance. The aim of this paper is to strengthen the training of perfusionists at our centre, to standardise the process and to provide the respective student with a “roadmap” to their internship. Material & Methods The structure is based on a rough division of the 24 weeks of internship. This is described in detail in the following and is backed up with the learning objectives for the respective time periods. Results At our centre, practical training has been standardized and clear responsibilities have been defined. Furthermore, as a centre of maximum care in the field of cardiac surgery, we can offer students the necessary number of perfusions in just six months to meet the requirements of the ECBP for practical training. According to this concept, 20 perfusionists have been successfully trained in the last 8 years. All of them have passed the exams and have been certified according to EBCP. Conclusion The aim of the practical semester is for the student to be in a position at the end of the semester to independently supervise simple cardiac surgery procedures with the aid of the Extra- Corporal Circulation (ECC) and to carry this out in accordance with the currently valid guidelines and directives (1–8) and the departmental procedural instructions based on them. Great emphasis is placed to the students becoming aware of their competence to act, knowing their limits and being able to assess when these limits have been reached and the involvement of experienced colleagues is necessary to ensure patient safety.

OBJECTIVES Myocardial recovery in children supported by a durable left ventricular assist device is a rare, but highly desirable outcome because it could potentially eliminate the need for a cardiac transplant and the lifelong need for immunosuppressant therapy and the risk of complications. However, experience with this specific outcome is extremely limited. METHODS All patients < 19 years old supported by a durable left ventricular assist device from the European Registry for Patients with Mechanical Circulatory Support database were included. Participating centres were approached for additional follow-up data after explantation. Associated factors for explantation due to myocardial recovery were explored using Cox proportional hazard models. RESULTS The incidence of recovery in children supported by a durable left ventricular assist device was 11.7% (52/445; median duration of support, 122.0 days). Multivariable analyses showed body surface area (hazard ratio 0.229; confidence interval 0.093–0.565; P = 0.001) and a primary diagnosis of myocarditis (hazard ratio 4.597; confidence interval 2.545–8.303; P < 0.001) to be associated with recovery. Left ventricular end-diastolic diameter in children with myocarditis was not associated with recovery. Follow-up after recovery was obtained for 46 patients (88.5%). Sustained myocardial recovery was reported in 33/46 (71.7%) at the end of the follow-up period (28/33; >2 year). Transplants were performed in 6/46 (11.4%) (in 5 after a ventricular assist device was reimplanted). Death occurred in 7/46 (15.2%). CONCLUSIONS Myocardial recovery occurs in a substantial portion of paediatric patients supported with durable left ventricular assist devices, and sustainable recovery is seen in around three-quarters of them. Even children with severely dilated ventricles due to myocarditis can show recovery. Clinicians should be attentive to (developing) myocardial recovery. These results can be used to develop internationally approved paediatric weaning guidelines.

Introduction: The Berlin Heart EXCOR offers circulatory support across all paediatric ages. Clinically, the necessary care as well as outcomes differ in various age groups. The EUROMACS database was used to study age and size-related outcomes for this specific device. Methods: All patients <19 years of age from the EUROMACS database supported with a Berlin Heart EXCOR between 2000 and November 2021 were included. Maximally Selected Rank statistics were used to determine BSA cut-off values. Multivariable Cox Proportional-Hazard Regression using ridge penalization was performed to identify factors associated with outcomes. Results: In total, 303 patients were included (mean age: 2.0 years (interquartile range: 0.6-8.0, males: 48.5%)). Age and BSA were not significantly associated with mortality (n = 74, p = 0.684, p = 0.679). Factors associated with transplantation (n = 175) were age (HR 1.07, p = 0.006) and aetiology other than congenital heart disease (HR 1.46, p = 0.020). Recovery rates (n = 42) were highest in patients with a BSA <0.53 m2 (21.8% vs 4.3-7.6% at 1 year, p = 0.00534). Patients with a BSA ≥0.73 m2 had a lower risk of early pump thrombosis, but a higher risk of early bleedings compared to children with a BSA <0.73 m2. Conclusions: Mortality rates in Berlin Heart supported patients cannot be predicted by age or BSA. Recovery rates are remarkably high in the smallest patient category (BSA <0.53 m2). This underscores that the Berlin Heart EXCOR is a viable therapeutic option, even for the smallest and youngest patients.

Patients with refractory heart failure due to chronic progressive cardiac myopathy (CM) may require mechanical circulatory support as a bridge to transplantation. A few patients can be weaned from support devices if recovery can be achieved. The identification of these patients is of great importance as recovery may be missed if the heart is unloaded by the ventricular assist device (VAD). Testing the load-bearing capacity of the supported left ventricle (LV) by temporarily and gradually reducing mechanical support during cardiac exercise can help identify responders and potentially aid the recovery process. An exercise training protocol was used in 3 patients (8 months, 18 months and 8 years old) with histological CM findings and myocarditis. They were monitored regularly using clinical information and functional imaging with VAD support. Echocardiographic examination included both conventional real-time 3D echocardiography (RT3DE) and speckle tracking (ST). A daily temporary reduction in pump rate (phase A) was followed by a permanent reduction in rate (phase B). Finally, pump stops of up to 30 min were performed once a week (phase C). The final decision on explantation was based on at least three pump stops. Two patients were weaned and successfully removed from the VAD. One of them was diagnosed with acute viral myocarditis. The other had chronic myocarditis with dilated myopathy and mild interstitial fibrosis. The noninvasive assessment of cardiac output and strain under different loading conditions during VAD therapy is feasible and helps identify candidates for weaning despite severe histological findings. The presented protocol, which incorporates new echocardiographic techniques for determining volume and deformation, can be of great help in positively guiding the process of individual recovery, which may be essential for selecting and increasing the number of patients to be weaned from VAD.