New Screening Tool for Aortic Root Dilation in Children with Marfan Syndrome and Marfan-Like Disorders.

One of the roles of a pediatric cardiologist who suspects or diagnoses a genetically determined connective tissue disease (e.g., Marfan, Ehlers-Danlos, and Loeys-Dietz syndromes) is to assess whether the aortic root is dilated. The aortic root diameter is affected by the patient's age, sex, and body surface area. Therefore, the aortic root diameter needs to be determined and expressed as a z-score. Calculation of the z-score is time-consuming and problematic if used infrequently. This study aimed to introduce a simple screening method for identifying aortic root dilation in children. The study population consisted of 190 children who were diagnosed with Marfan syndrome or Marfan-like disorders. The aortic root ratio (ARr) was formulated. The value of the ARr was compared in each patient with the results in z-scores, which were obtained using on-line calculators based on the most widespread nomograms. The optimal cut-off value of the ARr was ≥ 18.7. At this cut-off point, the sensitivity of the ARr ranged from 88.3% to 100% and the specificity ranged from 94% to 97.8%. All of the patients in whom the ARr failed to identify aortic root dilation were also divergently classified by different nomograms. At the ARr cut-off point of ≥ 18.0, a sensitivity of 100% was achieved for all nomograms with minimal reduction in specificity. The ARr allows for rapid and precise screening for aortic root dilation in children. Unlike classic analysis, the ARr does not require nomograms or on-line calculations.

Safety and efficacy of self-apposing Stentys drug-eluting stent in left main coronary artery PCI: Multicentre LM-STENTYS registry.

BACKGROUND: There is a paucity of data on left main (LM) percutaneous coronary intervention (PCI) therapy with dedicated DES platforms. The LM-STENTYS is a multicenter registry aimed at evaluating clinical outcome after PCI of LM performed with a self-apposing Stentys DES implantation. METHODS: The registry consists of 175 consecutive patients treated with Stentys DES implanted to LM. The primary endpoint was the composite of major adverse cardiac and cerebral events (MACCE) defined as cardiac death, myocardial infarction (MI), target lesion revascularization (TLR), and stroke assessed after 1 year. The secondary endpoint was stent thrombosis (ST) at 1 year. RESULTS: The median age was 69 years (IQR, 62-78 years). Acute coronary syndrome (ACS) was the presenting diagnosis in 117 (66.9%) patients [74 (63.2%) unstable angina, 31 (26.5%) NSTEMI, 12 (10.3%) STEMI] and stable angina (SA) was present in 58 (33.1%) patients. The median SYNTAX score was 23.0 (IQR, 18.7-32.2) in the SA group and 25.0 (IQR, 20.0-30.7) in the ACS group. During 1-year follow-up in the SA group two (3.4%) MACCE occurred, both of them were cardiac deaths. In ACS patients there were 19 (16.2%) MACCE [9 (7.7%) cardiac deaths, 11 (9.4%) MIs, 11(9.4%) TLR, 1(0.9%) stroke]. Altogether, three (1.7%) cases of acute ST were noted, all of them in ACS subset. CONCLUSION: LM PCI using self-apposing Stentys DES showed favorable clinical outcomes at 1-year in patients with SA. Events of ST in the ACS group warrant further research.

Paradoxical brain embolism in a young man: is it only a patent foramen ovale?

Paradoxical embolism is considered the major cause of cerebral ischemic events in young patients. The most common cause of paradoxical embolism, which has been widely described, is right-to-left shunting (RLS) at cardiac level through a patent foramen ovale (PFO). Rarely paradoxical embolism can also be caused by RLS at pulmonary level due to pulmonary arteriovenous fistula (PAVF). Herein, we present a case of a young man, who experienced transient ischemic attack (TIA) due to paradoxical embolism, in whom both abovementioned abnormalities coexisted. This coincidence is very rare (noted in only 1% of patients with cryptogenic stroke or TIA), but it highlights the importance of searching for extracardiac RLS in patients with cryptogenic stroke, even if a PFO has been detected.

ST-segment elevation myocardial infarction as a COVID-19 manifestation in a recent heart transplant recipient.

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How to Distinguish Marfan Syndrome from Marfanoid Habitus in a Physical Examination-Comparison of External Features in Patients with Marfan Syndrome and Marfanoid Habitus.

Marfan Syndrome (MFS) is a systemic disorder caused by mutations in fibrillin-1. The most common cause of mortality in MFS is dissection and rupture of the aorta. Due to a highly variable and age-dependent clinical spectrum, the diagnosis of MFS still remains sophisticated. The aim of the study was to determine if there exist phenotypic features that can play the role of "red flags" in cases of MFS suspicion. The study population included 306 patients (199 children and 107 adults) who were referred to the Department of Pediatric Cardiology due to suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 109 patients and marfanoid habitus in 168 patients. The study excluded 29 patients with other hereditary thoracic aneurysm syndromes. Comparative analysis between patients with Marfan syndrome and marfanoid habitus was performed. Symptoms with high prevalence and high positive likelihood ratio were identified (pectus carinatum, reduced elbow extension, hindfoot deformity, gothic palate, downslanting palpebral fissures, lens subluxation, myopia ≥ 3 dioptres remarkably high stature). The differentiation between patients with MFS and marfanoid body habitus is not possible by only assessing external body features; however, "red flags" could be helpful in the screening phase.

Long-term outcomes following drug-eluting balloon or thin-strut drug-eluting stents for treatment of in-stent restenosis stratified by duration of dual antiplatelet therapy (DEB-Dragon Registry).

Introduction: Data regarding the duration of dual antiplatelet therapy (DAPT) in patients with drug-eluting stent restenosis (DES-ISR) treated with percutaneous coronary intervention (PCI) and drug-eluting balloons (DEB) or DES are not unambiguous. Aim: To evaluate the relationship between long-term outcomes and the length of DAPT in patients treated with PCI due to DES-ISR with DEB or DES. Material and methods: Overall, a total of 1,367 consecutive patients with DES-ISR, who underwent PCI with DEB or DES between 2008 and 2019 entered the study. The mean length of the follow-up was 1,298.7 ±794 days. We assessed study endpoints according to the duration of DAPT (≤ 3 vs. > 3 and ≤ 6 vs. > 6 months) before and after propensity score matching (PSM): stroke, target lesion revascularisation (TLR), target vessel revascularisation (TVR), myocardial infarction (MI), death and device oriented composite endpoints (DOCE). Kaplan-Meier estimates were created to differentiate long-term outcomes. Results: Pairwise contrast analysis considering type of PCI (DES vs. DEB) and duration of DAPT (≤ 6 vs. > 6 months) before PSM revealed superiority of DES + DAPT > 6 months vs. DEB + DAPT > 6 months for DOCE (p < 0.001), TVR (p = 0.02) and TLR (p = 0.01). Also, DES + DAPT ≤ 6 months was found to be superior compared to DEB + DAPT ≤ 6 months for DOCE (p < 0.001), TVR (p = 0.02) and TLR (p = 0.01). Kaplan-Meier estimate analysis confirmed that DAPT > 6 months is related to a higher stroke rate (p = 0.01) when compared to ≤ 6 months. Conclusions: Treatment with DAPT in patients with DES-ISR is related to better long-term outcomes in the case of PCI with DES than DEB. DAPT > 6 months is related to the greater rate of strokes, independently of the type of treatment (DES and DEB) than DAPT ≤ 6 months.

Long-Term Outcomes Following Drug-Eluting Balloons Versus Thin-Strut Drug-Eluting Stents for Treatment of In-Stent Restenosis (DEB-Dragon-Registry).

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Safety and Efficacy of Embolic Protection Devices in Saphenous Vein Graft Interventions: A Propensity Score Analysis-Multicenter SVG PCI PROTECTA Study.

Background: Evidence concerning the efficacy of the embolic protection devices (EPDs) in saphenous vein graft (SVG) percutaneous coronary intervention (PCI) is sparse. The study was designed to compare major cardiovascular events of all-comer population of SVG PCI with and without EPDs at one year of follow-up. Methods and results: A multi-center registry comparing PCI with and without EPDs in consecutive patients undergoing PCI of SVG. The group comprised 792 patients, among which 266 (33.6%) had myocardial infarction (MI). The primary composite endpoint was major adverse cardiac and cerebrovascular event (MACCE) defined as death, MI, target vessel revascularization (TVR), and stroke assessed at one year. After propensity score analysis, there were no differences in MACCE (21.9% vs. 23.9%; HR 0.91, 95% CI 0.57-1.45, p = 0.681, respectively) nor in secondary endpoints of death, MI, TVR, target lesion revascularization (TLR) and stroke at one year in EPDs PCI group vs. no-EPDs PCI group. Similarly, there were no differences between groups in the study endpoints at 30 days follow-up. Conclusions: There were no clinical benefit for routine use of EPDs during SVG PCI in short and long-term follow-up. Further studies are warranted to explore the effect of individual types of EPDs on clinical outcomes.

New-generation drug eluting stent vs. bare metal stent in saphenous vein graft - 1 year outcomes by a propensity score ascertainment (SVG Baltic Registry).

BACKGROUND: Data regarding the efficacy of the percutaneous coronary intervention (PCI) with new-designed drug-eluting stent (new-DES) vs. bare metal stent (BMS) of saphenous vein grafts (SVG) stenosis is scarce. The primary objective was to compare one-year clinical outcomes of PCI in stenosis of SVG using new-DES vs. BMS in a real-world population. METHODS AND RESULTS: We carried out a multi-center registry comparing new-DES with BMS in all consecutive patients undergoing PCI of SVG. The primary composite endpoint was major adverse cardiac and cerebrovascular events (MACCE) at 1 year. This observation included 792 consecutive patients (mean age 69 ±â€¯8.9y), treated with either new-DES (n = 379, 47.9%) or BMS (n = 413, 52.1%). Among patients treated with new-DES compared with BMS, there was a lower risk of MACCE (21.4% vs. 28.3%, HR = 0.69, 95% CI 0.50-0.95, p = 0.025) as well as myocardial infarction (MI) (6.3% vs. 12.1%; HR 0.49, 95% CI 0.30-0.82, p = 0.005) at 1 year. After propensity score adjustment, the similar, significant reduction in MACCE and MI was observed in favor of new-DES (HR 0.66, 95% CI 0.46-0.96, p = 0.030; and HR 0.53, 95% CI 0.31-0.92, p = 0.020, respectively). CONCLUSION: In patients undergoing PCI of SVG, the use of new-DES is associated with a reduced 1-year rate of MACCE and MI compared to BMS.

[Magnetic resonance imaging in a patient with an implantable cardioverter-defibrillator]. / Badanie rezonansem magnetycznym przeprowadzone bez powiklan u chorego z wszczepionym kardiowerterem-defibrylatorem.

The number of patients with cardiac pacemakers (PM), implantable cardioverter-defibrillators (ICD) and cardiac resynchronisation therapy PM systems is increasing. The number of magnetic resonance imaging (MRI) examinations is also growing and amounts to about 60 million tests per year worldwide. The presence of an ICD is still considered to be an absolute contraindication to MRI by most experts. We present a patient with an implanted ICD who successfully underwent brain MRI with use of special precautions.