Key aspects of the medical management of Kawasaki disease (KD) are not yet supported by a high evidence level, thus making room for individual recommendations. We performed a structured comparison of existing international KD guidelines to analyze potential differences in the implementation of evidence-based KD recommendations regarding diagnosis and therapy. To identify country-specific guidelines, we took a multilateral approach including a comprehensive PubMed literature, online research, and directly contacting national pediatric associations. We then ran a structured guidelines’ analysis and evaluated the diagnostic and therapeutic differences in the context of evidence-based medicine. In this structured guideline analysis, we identified nine national and one European guidelines. According to them all, the diagnosis of KD still relies on its clinical presentation with no reliable biomarker recommended. First-line treatment consistently involves only intravenous immunoglobulin (IVIG) therapy. Recommendations in terms of acetylsalicylic acid, corticosteroids, and additional therapeutic options vary considerably.Conclusion: According to all guidelines, KD is diagnosed clinically with some variance in defining incomplete KD and being a non-responder to treatment. First-line treatment consistently includes IVIG. Recommendations for additional therapeutic strategies are more heterogeneous. What is Known:• The diagnosis of KD relies on the clinical presentation, entailing challenges in timely diagnosis.• Other treatment options then IVIG are not supported by a high evidence level, making room for individual recommendations. What is New:• Definition of incomplete KD and being non-responsive to an initial treatment vary to some extent between the national guidelines.• Only IVIG is consistently proposed throughout all guidelines, further therapeutic recommendations vary between the national recommendations.

We report the long-term effect after successfully implanting an 8 mm Atrial-flow-regulator (AFR) device in a 7-year-old girl with idiopathic pulmonary hypertension with persistent syncope under triple therapy with significant improvement after implantation and absence of any further syncope. Early Implantation of the AFR device (Occlutech, Germany) can be efficient and safe interventional therapy option for pulmonary arterial hypertension with a history of syncope.

Background: Pulmonary hypertension (PH) is diagnosed based on an invasive evaluation of the mean pulmonary artery (PA) pressure. The morphological assessment of the pulmonary arteries was only recently not feasible. With the advent of optical coherence tomography (OCT)-imaging, an accessible tool allows to study PA morphology longitudinally. The primary hypothesis was that OCT distincts the PA structure of PH patients from control subjects. The secondary hypothesis was that PA wall thickness (WT) correlates with the progression of PH. Methods: This is a retrospective monocentric study of 28 paediatric patients with (PH group) and without PH (control group) who had undergone cardiac catheterisation including OCT imaging of the PA branches. OCT parameters analysed were WT and the quotient of WT and diameter (WT/DM) and those were compared between the PH group and the control group. In addition, the OCT parameters were aligned with the haemodynamic parameters to evaluate the potential of OCT as a risk factor for patients with PH. Results: WT and WT/DM in the PH group were significantly higher compared to the control group {WT: 0.150 [0.230, range (R): 0.100–0.330] vs. 0.100 [0.050, R: 0.080–0.130] mm, P<0.001; WT/DM: 0.06 [0.05] vs. 0.03 [0.01], P=0.006}. There were highly significant correlations between WT and WT/DM with the haemodynamic parameters mean pulmonary arterial pressure (mPAP) [Spearman correlation coefficient (r_s) =0.702, P<0.001; r_s=0.621, P<0.001], systolic pulmonary arterial pressure (sPAP) (r_s=0.668, P<0.001; r_s=0.658, P<0.001) and WT and pulmonary vascular resistance (PVR) (r_s=0.590, P=0.02). Also, there was a significant correlation between WT and WT/DM and the risk factors quotient of mPAP and mean systemic arterial pressure (mSAP) (mPAP/mSAP) (r_s=0.686, P<0.001; r_s=0.644, P<0.001) and pulmonary vascular resistance index (PVRI) (r_s=0.758, P=0.002; r_s=0.594, P=0.02). Conclusions: OCT can detect significant differences in WT of the PA in patients with PH. Furthermore, the OCT parameters correlate significantly with haemodynamic parameters and risk factors for patients with PH. More investigations are required to evaluate to what extent the impact of OCT can contribute to the clinical care of children with PH.

May–Thurner syndrome is a venous compression syndrome of the pelvic vessels that represents a relevant risk factor for thrombus formation. The standard procedure to secure a diagnosis is venography, followed by endovascular therapy as the preferred treatment choice if the patient is symptomatic. In our case series, there are three related patients with May–Thurner syndrome. A 16-year-old female was admitted with pulmonary embolism, dyspnoea and hip pain. The compression syndrome was diagnosed with interventional venography, and the patient received venous stent implantation. Due to her family history, we also suspected her mother to be affected by the syndrome and elucidated the diagnosis shortly afterwards by invasive venography. Subsequently, we examined the patient’s 19-year-old brother, and magnetic resonance imaging confirmed May–Thurner syndrome. A similar case series has not been published before. In this case, the family relation indicates a possible hereditary aspect of May–Thurner syndrome. This hypothesis should be the subject of further research. In conclusion, it is essential to assess family history thoroughly when treating patients with May–Thurner syndrome.

Background: Kawasaki Disease (KD) is a generalized vasculitis in childhood with possible long-term impact on cardiovascular health besides the presence of coronary artery lesions. Standard vascular parameters such as carotid intima–media thickness (cIMT) have not been established as reliable markers of vascular anomalies after KD. The carotid intima–media roughness (cIMR) representing carotid intimal surface structure is considered a promising surrogate marker for predicting cardiovascular risk even beyond cIMT. We therefore measured cIMR in patients with a history of KD in comparison to healthy controls to investigate whether KD itself and/or KD key clinical aspects are associated with cIMR alterations in the long-term. Methods: We assessed cIMR in this case-control study (44 KD, mean age in years (SD); 13.4 (7.5); 36 controls, mean age 12.1 (5.3)) approximately matched by sex and age. Different clinical outcomes such as the coronary artery status and acute phase inflammation data were analyzed in association with cIMR values. Results: When comparing all patients with KD to healthy controls, we detected no significant difference in cIMR. None of the clinical parameters indicating the disease severity, such as the persistence of coronary artery aneurysm, were significantly associated with our cIMR values. However, according to our marginally significant findings (p = 0.044), we postulate that the end-diastolic cIMR may be rougher than the end-systolic values in KD patients. Conclusions: We detected no significant differences in cIMR between KD patients and controls that could confirm any evidence that KD predisposes patients to a subsequent general arteriopathy. Our results, however, need to be interpreted in the light of the low number of study participants.