In the postnatal period, extensive peripheral arteriovenous malformations (AVM) are associated with high morbidity, especially when localized in the liver. Their urgent treatment is always a challenging problem in neonates and infants. We analyzed four consecutive children aged three days to three years who underwent eight liquid embolization procedures with ethylene-vinyl alcohol copolymer. The AVM were situated on the thoracic wall, in the liver, and on the lower leg. In three cases, the malformations showed total regression. The tibial AVM degenerated widely. If impaired beforehand, cardiac or hepatic function normalized after the interventions. There were no embolization-associated complications such as nontarget embolization or tissue ischemia. We conclude that application of ethylene-vinyl alcohol copolymer seems to be a safe therapeutic option and can be used in neonates and infants with peripheral AVM in consideration of the agent’s characteristics. Nevertheless, there are still hardly any data concerning young children.

Objective: The objective of this study is to evaluate the clinical application and primary outcome of transcatheter embolization using Amplatzer™ Vascular Plug (AVP) Type 2 and Type 4 in different congenital cardiovascular malformations.Design: This is a single‐center retrospective observational cohort study.Methods: We analyzed clinical and imaging data of 36 patients retrospectively who received transcatheter embolizations of the following malformations using AVP: systemic‐to‐pulmonary collateral arteries (SPCA), patent ductus arteriosus (PDA), ventricular septal defects (VSD), and aberrant pulmonary sequestration arteries (PSA). We included all patients treated in our institution from January 2010 to July 2023.Results: In 36 patients (median age 40.0 months, range 0.5 months–42.0 years; 56.8% male), 44 AVPs were implanted in 37 procedures. The target lesions were SPCA in n = 15 procedures, PDA in n = 9, VSD in n = 9, and PSA in n = 4. Thirty‐four AVP Type 2 and 10 AVP Type 4 were applied, the latter only in SPCA and PSA. SPCA was most common in complex congenital heart disease with univentricular physiology (75.0%). VSD were associated with additional cardiac malformations in 33.3%, PDA were associated with prematurity (55.6%), and all pulmonary sequestrations occurred in scimitar syndrome. Primary total or subtotal occlusion succeeded in n38/44 (86.3%). For residual PDA, an additional occluder was implanted in one patient. In one case, pulmonary sequestration had to be treated surgically. One premature infant with PDA closure sustained a relevant obstruction of the left pulmonary artery by the outer AVP disc which required surgical correction 4 months later.Conclusion: Embolization using AVP is a suitable approach for closure of various cardiovascular malformations with a high primary success rate and low complication rate. It should be considered in treatment of different irregular vessel anomalies and in selected VSD.

Congenital critical aortic valve stenosis (CAVS) is a life-threatening disease requiring urgent treatment. First-line therapy is still controversial. The aim of our study was (1) to analyze retrospectively the patients of our institution who underwent balloon aortic valvuloplasty (BAV) due to CAVS and (2) describe the techniques for improved feasibility of intervention using microcatheters and retrieval loops. Twelve patients underwent 23 BAVs: 1 BAV was performed in 3 patients, 2 BAVs were performed in 7 patients, and 3 BAVs were performed in 2 patients. The peak trans-valvular pressure gradient (Δp) and left ventricular shortening fraction (LVSF) improved significantly in the first two interventions. In the first BAV, Δp decreased from 73.7 ± 34.5 mmHg to 39.8 ± 11.9 mmHg (p = 0.003), and the LVSF improved from 22.3 ± 13.5% to 31.6 ± 10.2% (p = 0.001). In the second BAV, Δp decreased from 73.2 ± 33.3 mmHg to 35.0 ± 20.2 mmHg (p < 0.001), and the LVSF increased from 26.7 ± 9.6% to 33.3 ± 7.4% (p = 0.004). Cardiac surgery during the neonatal period was avoided for all children. The median time to valve surgery was 5.75 years. Few complications occurred, namely mild-to-moderate aortic regurgitation, one remediable air embolism, and one intimal injury to the ascending aorta. We conclude that BAV is a successful emergency treatment for CAVS, resulting in left ventricular relief, clinical stabilization, and a time gain until cardiac surgery.

Objectives: The purpose of this study was to analyze image quality and radiation exposure of thoracic computed tomography angiography (CTA) in children with congenital heart diseases (CHDs) using either manual contrast medium (CM) injection or automated power injectors with bolus tracking. Methods: A total of 137 thoracic CTAs of 120 consecutive pediatric patients were included in this retrospective study. We analyzed the method of CM administration (power injection with bolus tracking (PI) or manual injection (MI)), injection routes, volumes and flow rates of CM. For the evaluation of objective image quality, attenuation values in the heart chambers and great thoracic vessels were determined by region-of-interest (ROI) analysis and signal-to-noise (SNR) and contrast-to-noise (CNR) ratios calculated thereof. Visual image quality was assessed by two blinded readers (four-point Likert-scale) analyzing the presence of artifacts and the depiction of relevant anatomical structures. Effective radiation doses were calculated with dose length products and specific conversion factors. Results: CM administration was performed using PI in 119/137 CTAs, whereas MI was conducted in 18/137. The smallest size of peripheral venous cannulas was 24 gauge in 36/137 (26.3%) cases. Overall mean CM volume was 17 mL ± 16 mL (mean ± SD). In PI, the mean flow rate of CM was 1.52 ± 0.90 mL/s with a range between 0.5 and 5.0 mL/s. When comparing the overall PI population and an age-, size- and weight-matched PI subpopulation (18 cases) with the MI population, attenuation values in Hounsfield units (HU) and CNR values were significantly higher in the PI groups than in the MI group for each relevant cardiac structure (left ventricle, right ventricle, ascending aorta and pulmonary trunk, p = 0.02–0.001). Overall image quality and depiction of cardiac structures were rated significantly better in CTAs with PI (interquartile ranges: “good” to “excellent” (Likert 3–4)) in PI compared with CTAs acquired with MI (interquartile ranges: “fair” to “good” (2–3)) in MI by both readers (p < 0.001). The inter-observer reliability was strong, with a Kendall’s Tau-b correlation coefficient of τ = 0.802 (p < 0.001). The mean effective radiation dose (E) did not differ significantly when comparing the stratified samples (i.e., the matched PI subgroup and the MI group; 0.5 (±0.3) mSv in both, p = 0.76). There were no complications associated with the CM injections for both application approaches. Conclusions: Automated contrast agent applications with power injectors and bolus tracking ensure better image quality in pediatric CTA, even when low volumes and flow rates need to be applied. There is a slight increase in radiation associated with bolus tracking. This approach represents a suitable imaging technique for the work-up of congenital heart disease.

SARS-CoV-2 infection affects multiple immune mechanisms and leads to severe COVID-19 and death, in part related to infection-induced or pre-existing autoantibodies. Here, we describe severe COVID-19 to associate with autoantibodies against interleukin-1 receptor antagonist (IL-1Ra) and progranulin (PGRN), endogenous antagonists of IL-1 and TNF signaling, respectively. These autoantibodies coincide with hyperphosphorylation of IL-1Ra (Thr111) or PGRN (Ser81), form immune complexes independent of phosphorylation, reduce antigen plasma levels, and permit enhanced IL-1 and TNF signaling. Using phage-display selected Fabs specific for hyperphosphorylated isoforms, we track phospho-antigens and autoantibodies in a German national pandemic network cohort. Most seropositive patients show both autoantibodies. Levels peak at baseline and decline over 12 months, with phospho-antigens decreasing before autoantibodies. Seropositivity associates with hyperinflammation and cytokine profiles. Importantly, signaling by key inflammatory cytokines induce IL-1Ra and PGRN hyperphosphorylation in healthy monocytes, but require up to 1000-fold higher doses than in monocytes from previously seropositive severe COVID-19 survivors.