COVID-19 infection in patients with history of pediatric heart transplant in Germany, Austria, and Switzerland.

COVID-19 is a heterogenous infection-asymptomatic to fatal. While the course of pediatric COVID-19 infections is usually mild or even asymptomatic, individuals after adult heart transplantation are at high risk of a severe infection. We conducted a retrospective, multicenter survey of 16 pediatric heart transplant centers in Germany, Austria and Switzerland to evaluate the risk of a severe COVID-19 infection after pediatric heart transplantation between 02/2020 and 06/2021. Twenty-six subjects (11 male) with a median age of 9.77 years at time of transplantation and a median of 4.65 years after transplantation suffered from COVID-19 infection. The median age at time of COVID-10 infection was 17.20 years. Fourteen subjects had an asymptomatic COVID-19 infection. The most frequent symptoms were myalgia/fatigue (n = 6), cough (n = 5), rhinitis (n = 5), and loss of taste (n = 5). Only one subject showed dyspnea. Eleven individuals needed therapy in an outpatient setting, four subjects were hospitalized. One person needed oxygen supply, none of the subjects needed non-invasive or invasive mechanical ventilation. No specific signs for graft dysfunction were found by non-invasive testing. In pediatric heart transplant subjects, COVID-19 infection was mostly asymptomatic or mild. There were no SARS-CoV-2 associated myocardial dysfunction in heart transplant individuals.

First-in-man use of the EXCOR Venous Cannula for combined cavopulmonary and systemic ventricular support in Fontan circulation failure.

BACKGROUND AND AIM: The Berlin Heart EXCOR system has been developed for mechanical circulatory support (MCS) of pediatric patients with terminal heart failure. A recently introduced iteration of the system (EXCOR Venous Cannula, Berlin Heart GmbH, Berlin, Germany) is dedicated to support patients with univentricular physiologies by facilitating implantation of the EXCOR device into the Fontan pathway. CASE PRESENTATION: We report the worldwide first successful implantation of the EXCOR Venous Cannula in a biventricular support concept for a 12-year-old boy (140 cm, 42.7 kg, body surface area 1.29 m2, Pedimacs Level 2) with severe systemic ventricle dysfunction and failing Fontan circulation. Surgery comprised of standard Berlin Heart EXCOR implantation to support the failing ventricle (12 mm apex / staged 12/9 mm arterial cannula / 50 ml ventricle). Cannulation for subpulmonary EXCOR support was achieved by performing a total cavopulmonary connection takedown with subsequent anastomosis of a staged 12/9 mm outflow cannula to the pulmonary artery and implantation of a 14/18 mm EXCOR Venous Cannula as subpulmonary inflow graft, which was connected to the superior vena cava and Fontan tunnel using GORE-TEX grafts. In the postoperative course, cardiac output and central venous pressures rapidly improved with hepatic and renal functions restoring to age- and condition-specific norm values. CONCLUSION: The Berlin Heart EXCOR Venous Cannula is the first system for standardized mechanical support of Fontan circulatory failure. In our patient, subpulmonary support restoring a biventricular circulation combined with systemic MCS normalized hemodynamics and reversed end-organ dysfunction.

Characteristics, management, and outcome of pediatric patients with post-transplant lymphoproliferative disease-A 20 years' experience from Austria.

BACKGROUND: Management of pediatric post-transplantation lymphoproliferative disorder (PTLD) after hematopoietic stem cell (HSCT) and solid organ transplantation (SOT) is challenging. AIM: This study of 34 PTLD patients up to 19-years old diagnosed in Austria from 2000 to 2018 aimed at assessing initial characteristics, therapy, response, and outcome as well as prognostic markers of this rare pediatric disease. METHODS AND RESULTS: A retrospective data analysis was performed. Types of allografts were kidney (n = 12), liver (n = 7), heart (n = 5), hematopoietic stem cells (n = 4), lungs (n = 2), multi-visceral (n = 2), small intestine (n = 1), and vessels (n = 1). Eighteen/34 were classified as monomorphic PTLD, with DLBCL accounting for 15 cases. Polymorphic disease occurred in nine, and non-destructive lesions in six cases. One patient had a non-classifiable PTLD. Thirteen/34 patients are surviving event-free in first remission (non-destructive, n = 4/6; polymorphic, n = 4/9; monomorphic, n = 6/18). Fourteen/34 patients lacked complete response to first-line therapy, of whom seven died. Four/34 patients relapsed, of whom two died. In 3/34 patients, death occurred as a first event. The 5-year overall and event-free survival rates were 64% ± 9% and 35% ± 9% for the whole cohort. Among all parameters analyzed, only malignant disease as the indication for transplantation had a significantly poor influence on survival. CONCLUSIONS: This study shows PTLD still to be a major cause of mortality following SOT or HSCT in children. A continued understanding of the molecular biology of the disease shall allow to decrease treatment intensity for lower risk patients and to identify patients who may benefit from newer therapy approaches to improve outcome and decrease morbidity.

Diagnostic role of whole-body [18F]-FDG positron emission tomography in patients with symptoms suspicious for malignancy after heart transplantation.

BACKGROUND: Increased rates of malignancies and infections occur in transplant patients under immunosuppression, but the resultant clinical symptoms, and results of physical examination, chest X-ray, abdominal ultrasonography and laboratory findings are frequently difficult to interpret or inconclusive. The aim of the present study was to investigate the usefulness of whole-body [(18)F]-FDG PET for investigation of heart transplant patients suffering from suspicious symptoms, with a previously ambiguous diagnosis. METHODS: Seventeen consecutive patients (8 women; 48 ± 22 years) with non-specific symptoms (lymphadenopathy, fever of unknown origin or recurrent febrile temperatures, weight loss, abdominal pain, night sweating, cough or generally reduced physical condition) were evaluated retrospectively. All patients underwent whole-body [(18)F]-FDG examinations by PET (7 patients) or PET/CT (10 patients) at 8 ± 6 (range 0.1 to 21) years after orthotopic heart transplantation (OHT). During a follow-up of 28 ± 25 months, results of bone marrow biopsies, and histologic and/or microbiologic findings were registered and retrospectively compared with the PET results. RESULTS: PET revealed the cause of non-specific symptoms in 9 of 17 patients; there were 5 cases of lymphoproliferative disease (PTLD), 2 carcinomas and 2 cases of infection. Four patients were rated false positive, 1 patient false negative and 3 patients were correctly rated as negative. Sensitivity, specificity and positive and negative predictive values were 0.90, 0.43, 0.69 and 0.75, respectively, giving an overall diagnostic accuracy of 0.71. CONCLUSIONS: A non-invasive strategy of using whole-body [(18)F]-FDG PET or PET/CT in heart transplant recipients with non-specific unexplained symptoms may offer diagnostic stratification for malignancy and infections with a high sensitivity and modest diagnostic accuracy. These findings require prospective confirmation.

Right ventricle-to-pulmonary artery shunt related complications after Norwood procedure.

OBJECTIVE: The right ventricle-to-pulmonary artery (RV-PA) shunt in the Norwood procedure (NP) for children with hypoplastic left-heart syndrome (HLHS) provides stable early hemodynamics and improves survival in many centers. However, lower pulmonary-to-systemic flow ratio causes early cyanosis and may require earlier second-stage procedure. The aim of the study was to present shunt-related results after NP with RV-PA shunt and our technique of RV-PA shunt construction. METHODS: Between June 2001 and August 2010, 236 children with HLHS and variants underwent NP with RV-PA shunt, and were operated on by the same surgeon. The medical records were retrospectively reviewed. RESULTS: To date, 180 children at a mean age of 7.0 ± 1.6 months with a mean weight of 6.4 ± 0.9 kg underwent second-stage procedure. The mean systemic oxygen saturation before stage 2 operation was 74.8 ± 6.6% and mean arterial partial oxygen pressure was 32.8 ± 6.7 mm Hg. These two parameters were significantly lower than after NP (p = 0.029, p < 0.001, respectively). Between stage 1 and 2 operation, three children (1.3%) died due to the shunt obstruction. Four children (1.7%) underwent re-operations due to shunt problems (one of them died), and the other four (1.7%), stent implantation in RV-PA shunt. Two infants (1.1%) developed aneurysm of the right ventricle infundibulum, which was resected during stage 2 without complications. One child required early (before fifth month of age) second-stage procedure due to the shunt obstruction. The patients with right-sided to the neo-aorta course of the RV-PA shunt had significantly more frequent delayed sternal closure after NP than children with left-sided shunt (35.5% vs 14.1%; p = 0.008). CONCLUSIONS: The RV-PA shunt can be a safe and efficient technique in providing optimal pulmonary blood flow in the children with HLHS after Norwood procedure, performed with minimal rate of complications. In our experience, the use of RV-PA shunt in NP does not require earlier second-stage procedure.