No abstract

Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient’s age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group “pulmonary hypertension, heart failure and transplantation” has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

This study aims to evaluate the long-term efficacy and reintervention rate after primary percutaneous portal vein stent angioplasty for portal vein stenosis (PVS) in pediatric liver transplantation (LT) recipients. From 2004 to 2020, a total of 470 pediatric LTs were performed in our center. All cases were screened for interventional PVS treatment and analyzed retrospectively. We identified 44 patients with 46 percutaneous angioplasties for posttransplantation PVS. The median interval from LT to percutaneous catheter intervention was 5 months (16 days-104 months) with a median follow-up (f/u) period after catheter intervention of 5.7 years (2-156 months). In 40 patients, an endovascular stent was placed as primary (n = 38) or secondary (n = 2) intervention. The median age at stent placement was 23 (6-179) months with a median weight of 10 kg (6-46 kg).Technical success and relief of PVS were achieved in all patients irrespective of age or weight. Adverse events occurred peri-interventionally in two patients and were resolved with standard care. All primary portal vein (PV) stents remained patent until the end of f/u. Reinterventions have been successfully performed in 10 patients for suspected or proven restenosis, resulting in a primary patency rate of 75% and an assisted patency rate of 25%. The median time to reintervention was 6.2 years (range 1-10 years). The need for reintervention was independent of age or weight at both transplantation and initial angioplasty as well as of additional risk factors due to portal hypertension. Percutaneous transhepatic PV stent angioplasty in children is safe and effective in all age groups, with excellent long-term patency. Primary stent angioplasty should be considered as first-line treatment for PVS after pediatric LT.

BackgroundCoronavirus disease 2019 (COVID-19) in children is rarely severe. However, severe courses occur, especially in the presence of risk factors. A minority of children develop pediatric inflammatory multisystem syndrome (PIMS) with substantial morbidity. While the importance of cardiac involvement after PIMS is well established, its role after severe acute COVID-19 remains unclear. We aim to compare cardiac sequelae of children after severe acute COVID-19 using cardiac MRI and compare them with patients after PIMS.MethodsFor this prospective cohort study, we recruited patients with acute COVID or PIMS in a single center. Clinical follow-up, lab work, ECG, and echocardiography were done within 2 days after disease onset and 3–6 months after discharge. At the last visit 3–6 months later, cardiac MRI (CMR) with late gadolinium enhancement (LGE) was performed to evaluate cardiac sequelae and compare both groups.ResultsData were obtained from n = 14 patients with PIMS and n = 7 patients with severe acute COVID-19. At the start of the respective disease, left ventricular (LV) ejection fraction was reduced in seven patients with PIMS but none in the acute COVID-19 group. Transient mitral valve insufficiency was present in 38% of patients, of whom PIMS accounted for 7/8 cases. Eight patients (38%) with PIMS presented coronary artery abnormalities, with normalization in 7/8 patients. A significant decrease in LV mass index 3–6 months after disease onset was observed in both groups. MRI follow-up revealed non-ischemic myocardial pattern of LGE in 12/21 patients- in all (6/6) after severe acute COVID-19 and in less than half (6/14) after PIMS. Normal body weight-adjusted stroke volumes and end-diastolic volumes were found in 20/21 patients.ConclusionsWe show that children suffering from severe acute COVID-19 have a similar, or worse, cardiac risk profile as patients with PIMS. Both patient groups should therefore receive close pediatric cardiac follow-up examinations. Cardiac MRI is the technique of choice, as most patients presented with delayed LGE as a sign of persistent cardiac injury despite normalization of laboratory and echocardiographic findings.

To the editor,We thank Marra and colleagues for their critical comments on our publication. Their statement reflects the ongoing controversy concerning the best strategy for posttransplant portal vein stenosis in children as long as there are no controlled multi-center data but single-center experiences. Therefore, we look forward to the publication of the PORTAL registry data.As Marra and colleagues pointed out, there are promising data on balloon angioplasty for portal vein stenosis after pediatric liver transplantation. [1,2] There are definitely patients who might be cured with balloon angioplasty alone. However, the cohorts differ substantially. The patients reported by Naik and colleagues were much older than our cohort, with a longer post transplant interval before catheter intervention. The study of Yabuta et al. [2] has been discussed in our manuscript. A substantial number of their patients received multiple reinterventions (25% of their patients with up to 5 procedures!) for long-term portal vein patency. The review of Kyaw et al. [3] stated a recurrence rate of 22% for balloon angioplasty and lower success rates in infants and toddlers.Both Kyaw et al. [3] and Shim et al. [4] concluded that "clinical success and long-term patency were superior in the primary stent group" compared with balloon angioplasty alone.The potential harm of portal vein stents concerning future transplantation and the potential risk of stent migration are traditional surgical arguments against long self-expanding stents used in the 1990s, which can nowadays be avoided using modern stent technology. The technical challenges during potential retransplantation after portal vein stenting should be manageable by an experienced surgical team.Our data have demonstrated that primary stent angioplasty of portal vein stenosis may avoid early restenosis in small children. Potential reinterventions in school-aged children or adolescents to reach adult-size portal vein diameters will be tolerated much better. In our center, we strictly try to avoid multiple invasive procedures needing general anesthesia in small children. Therefore, we aim for the most effective primary treatment option with the lowest recurrence rates for posttransplant portal vein stenosis. CONFLICT OF INTERESTNothing to report.