Percutaneous angioplasty is the method of choice for treatment of a recoarctation. The rate of complications like acute dissection or aortic rupture is extremely low, although …

Mucopolysaccharidosis II (MPS II, Hunter syndrome) is a rare, X‐linked lysosomal storage disease caused by reduced activity of iduronate‐2‐sulfatase (I2S), with subsequent cellular accumulation of the glycosaminoglycans (GAGs), heparan sulfate, and dermatan sulfate (DS). DS is a major component of the extracellular matrix of heart valves, which can be affected in MPS II. We investigated the natural history of valve disease in MPS II and the impact of long‐term intravenous enzyme replacement therapy (ERT) with recombinant I2S (idursulfase). In total, 604 cardiac examinations were assessed from serial follow‐up of 80 male patients (49 neuronopathic). Valve disease was classified according to standard practice from hemodynamic features evident from echocardiography. The natural history group comprised 48 patients (up to 14.8 years of follow‐up; median, 2.6 years; 24 patients started ERT during the study); 56 patients were treated (up to 14.2 years of follow‐up; median, 6.2 years). Lifetime GAG burden (calculated from urinary GAG measurements) correlated significantly with the degree of valve disease. Onset of moderate‐to‐severe valve disease was significantly delayed in treated (median age at onset, 29.1 ± 2 [95% CI: 25.2–32.9] years; Kaplan–Meier estimation) versus untreated patients (17.6 ± 1 [95% Cl: 15.8–19.4] years; p < 0.0001). Cox regression modeling found that long‐term ERT reduced the probability of developing severe valve disease (χ², 32.736; significant after 5 years of ERT). Overall, this study found that valve disease severity in MPS II correlates with GAG burden and that progression is delayed by long‐term ERT.

Background: A 24 h Holter study in children after transcatheter secundum ASD (ASD II) closure was conducted to detect the prevalence of defects and/or device-related late atrial arrhythmias (LAAs). ASD II closure with an Amplatzer septal occluder (ASO) is an established procedure. Little is known about LAAs after device implantation. Methods: The eligible participants were children who had undergone ASO implantation, with a follow-up of ≥5 years, as well as one pre- and at least one post-procedural Holter ECG. Results: In total, 161 patients (mean age: 6.2 ± 4.3 years), with a mean follow-up of 12.9 ± 3.1 years (range 5–19), were included. A median of four Holter ECGs per patient were available. LAAs occurred before intervention in four patients (2.5%), and it was peri-interventional in four patients (2.5%), sustained in three patients (1.9%), and developed in three patients (1.9%). In patients with pre- and peri-interventional LAAs, the Qp/Qs ratio was higher (6.4 ± 3.9 vs. non-AA: 2.0 ± 1.1 (p = 0.002)) and the IAS/ASO ratio was lower (1.18 ± 0.27 vs. non-AA: 1.7 ± 0.4 (p < 0.001)). The patients with LAAs differed from those without LAAs in their Qp/Qs (6.8 ± 3.5 vs. 2.0 ± 1.3; p < 0.0001) and IAS/ASO ratios (1.14 ± 0.19 vs. 1.73 ± 0.45; p < 0.001). The patients with LAAs had a Qp/Qs ratio ≥2.94:1, and those who developed LAAs had an IAS/ASO ratio <1.15. Conclusions: LAAs occurred in 1.9% of patients and were sustained in another 1.9% of patients but persisted in those with large shunt defects and large occluders in relation to the atrial septal length. The predisposing factors for LAAs after ASD closure were a high Qp/Qs ratio, pre-existing atrial arrhythmias, and a low IAS/ASO ratio.

Background/Objectives: There is an increasing need for cross-sectional imaging in pediatric patients with congenital heart disease. This patient group is highly sensitive to ionizing radiation. The purpose of this article was to evaluate differences in radiation dose and image quality between a first-generation photon-counting (PC)-CT system and a 256-slice single-source energy-integrating detector (EID)-CT in these patients. Methods: We retrospectively assessed effective dose, CT dose index-volume (CTDI_vol), dose length product (DLP), and image quality of all prospectively electrocardiography-gated CTs of the thorax in all patients < 18 years of age examined between February 2021 and August 2024 (n = 43). Two independent observers subjectively scored image quality, vascular contrast, and noise on a 5-point Likert scale. In addition, we assessed the signal-to-noise-ratio (SNR) and contrast-to-noise-ratio (CNR) quantitatively. Results: All dose parameters were significantly lower in the PC-CT (n = 27) versus the EID-CT (n = 16) group (mean effective dose: 0.8 ± 0.64 versus 2.2 ± 0.88 mSv, p < 0.001; mean CTDI_vol: 1.22 ± 0.96 versus 4.8 ± 1.08 mGy, p < 0.001; mean DLP: 30.7 ± 31.9 versus 73.7 ± 50.7 mGy*cm, p < 0.001). Overall subjective image quality and contrast were rated higher in the PC-CT group (p = 0.046 and < 0.001, respectively). Quantitative CNR was significantly higher in the PC-CT group (mean 39.1 ± 12.9 versus 26.2 ± 10.8, p = 0.002). Conclusions: PC-CT enables high-quality examinations for the evaluation of congenital heart disease with a highly significant dose reduction compared with a 256-slice single-source EID-CT.