Jochen Pfeifer

Background: For infants who underwent surgical coarctation repair and required balloon angioplasty for recoarctation within the first year of life, data are limited—especially regarding safety, mid-term efficacy, and reintervention risk factors. Methods: This retrospective study included the data of 33 infants who underwent surgical correction of CoA within their first three months of life and whose first cardiac catheterization for recoarctation—defined by a systolic echocardiographic gradient of ≥20 mmHg—was performed within the first year of life. Results: The median age at repair was 21 days, and that at angioplasty was 77 days. The peak systolic gradient decreased from 40.0 to 21.0 mmHg (p < 0.001), and the mean gradient decreased from 13.9 to 6.7 mmHg (p = 0.02). The aortic stenotic diameter increased from 3.2 to 4.1 mm (p < 0.001), and the z score increased from −4.2 to −2.7 (p < 0.001). Right arm systolic blood pressure dropped from 111.8 to 88.2 mmHg (p < 0.001); 7% of cases remained hypertensive. Complications occurred in 12% of patients, and reintervention was needed in 39.4%. Association with other congenital heart disease increased reintervention risk (OR 4.51). Follow-up gradients were as follows: 27.4 ± 10.2 mmHg (1 year), 24.6 ± 8.3 mmHg (2 years), 32.8 ± 18.8 mmHg (5 years), and 35.4 ± 23.2 mmHg (7 years). Conclusions: Early balloon angioplasty for postoperative recoarctation is safe, effective, and repeatable, with a high procedural success rate and sustained hemodynamic improvements. Reinterventions were more common in patients with additional CHD, though outcomes remained favorable.

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.

Port catheters provide a reliable, long‐term venous access option in children for repeated administration of medications or parenteral nutrition. A cardiac catheterization procedure was performed in a 7‐year‐old girl in whom lateral chest radiography revealed posterior deviation suggestive of azygos vein malposition. To avoid surgical replacement, a long loop snare technique was employed to safely withdraw the catheter into the superior vena cava with restoration of catheter function. This case highlights that endovascular long loop snare repositioning offers a feasible, minimally invasive alternative to surgical intervention for rare port catheter malpositions in pediatric patients.

We report on a 6-year-old boy with underlying hypoplastic left heart syndrome and a total cavopulmonary connection (Fontan circulation) with a diagnosis of plastic bronchitis. After an initial good response to therapy, his productive cough became significantly stronger again. Four months later, the patient’s mother brought a preserving jar containing an extensive bronchial cast to the clinic, the size of which is rarely seen in small children. Plastic bronchitis is a rare but dreaded complication in patients with Fontan circulation as well as in infectious or inflammatory diseases; its treatment is challenging.

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.

Background: Systemic-to-pulmonary collaterals (SPCs) are common in congenital heart disease (CHD). Particularly in single ventricle anatomy and Fontan circulation, SPC can both complicate the postoperative course and lead to clinical deterioration in the long term. The treatment of SPC is controversial. The aim of our study was (1) to retrospectively analyse patients who underwent SPC embolization using Concerto™ Helix nylon-fibred microcoils (CHMs) and (2) to describe the interventional technique. Methods: In this single-centre retrospective observational cohort study, we analysed clinical and imaging data of all patients who underwent transcatheter embolization of SPCs using CHMs from January 2016 to December 2023. Results: In 38 consecutive patients (65.8% male, median age 41 months, range 2–490), a total number of 141 CHMs had been implanted into 64 SPCs in 49 procedures. The majority were arterial SPCs (n = 59/64) originating from the thoracic aorta or its branches; 5/64 were veno-venous SPCs. Primary closure succeeded in all procedures. The CHM diameters ranged from 3 to 8 mm, with 5 mm being the most commonly used diameter. The mean coil/SPC ratio was 2.6 (range 1.3–5.3). CHM implantation was performed via four French sheaths. Both detachment and stable positioning were simple and safe. Neither non-target embolization nor coil migration occurred. One complication was a vascular injury with resulting extravasation of contrast medium. In 18/49 procedures (36.7%), coils other than CHMs or vascular plugs were additionally inserted into separate SPCs. Conclusions: CHMs are appropriate for SPC embolization in all age groups, including infants, with a low complication rate. The coils are particularly suitable for the closure of collaterals with a small diameter or tortuous course. They can be used in combination with other embolization devices to achieve comprehensive collateral closure.

Hypertrophic cardiomyopathy (HCM) is the most common hereditary cardiomyopathy. It is often caused by mutations of genes encoding for sarcomeric or sarcomere-associated proteins. Despite its clinical importance, divergent definitions are published by major cardiology societies. Some regard HCM as a specific genetic disease, whereas others define it as a broad ‘spectrum of the thick heart’. The present narrative review aimed to assess both definitions from a pathoanatomical perspective. As a conjoint interdisciplinary and translational approach is needed to further increase knowledge and improve the understanding of HCM, the PubMed database was searched using several advanced search algorithms to explore the perspectives of the (forensic) pathologist, clinician, and basic researcher regarding the difference between the definitions of HCM. This discrepancy between definitions can impact critical data, such as prevalence and mortality rate, and complicate the understanding of the disease. For example, due to the different definitions, research findings regarding molecular changes from studies applying the narrow definition cannot be simply extended to the ‘spectrum’ of HCM.

Background: Systemic-to pulmonary collaterals (SPC) are common in congenital heart disease (CHD). Particularly in single ventricle anatomy and Fontan circulation, SPC can both complicate the postoperative course and lead to clinical deterioration in the long term. Treatment of SPC is controversial. The aim of our study was (1) to analyze retrospectively patients who underwent SPC embolization using Concerto™ Helix nylon-fibred microcoils (CHM) and (2) to describe the interventional technique. Methods: In this a single-center retrospective observational cohort study, we analyzed clinical and imaging data of all patients who underwent transcatheter embolization of SPC using CHM from January 2016 to December 2023. Results: In 38 consecutive patients (65.8 % male, median age 41 months, range 2 – 490), a total number of 141 CHM had been implanted into 64 SPC in 49 procedures. The majority were arterial SPC (n = 59/64) originating from the thoracic aorta or its branches; 5/64 were veno-venous SPC. Primary closure succeeded in all procedures. The CHM diameters ranged from 3 to 8 mm, with 5 mm being the most commonly used diameter. The mean coil / SPC ratio was 2.6 (range 1.3 – 5.3). CHM implantation was performed via 4 French sheaths. Both detachment and stable positioning was simple and safe. Neither non-target embolization nor coil migration occurred. One complication occurred with a minor vascular injury without clinical relevance. Conclusion: CHM are easy to use and appropriate for SPC embolization in all age groups, including infants, with a low complication rate. The coils are particularly suitable for closure of collaterals with small diameter or tortuous course.