BACKGROUND: In Behçet\'s disease (BD), mild-to-severe valvular regurgitation (VR) poses a serious complication that contributes significantly to heart failure and eventually death. The accurate prediction of VR is crucial in the early stages of BD subjects for improved prognosis. Accordingly, this study aimed to develop a nomogram that can detect VR early in the course of BD.
METHODS: One hundred seventy-two patients diagnosed with Behçet\'s disease (BD) were conducted to assess cardiac valve regurgitation as the primary outcome. The severity of regurgitation was classified as mild, moderate, or severe. The parameters related to the diagnostic criteria were used to develop model 1. The combination of stepAIC, best subset, and random forest approaches was employed to identify the independent predictors of VR and thus establish model 2 and create a nomogram for predicting the probability of VR in BD. Receiver operating characteristics (ROC) and decision curve analysis (DCA) were used to evaluate the model performance.
RESULTS: Thirty-four patients experienced mild-to-severe VR events. Model 2 was established using five variables, including arterial involvement, sex, age at hospitalization, mean arterial pressure, and skin lesions. In comparison with model 1 (0.635, 95% CI: 0.512-0.757), the ROC of model 2 (0.879, 95% CI: 0.793-0.966) was improved significantly. DCA suggested that model 2 was more feasible and clinically applicable than model 1.
CONCLUSIONS: A predictive model and a nomogram for predicting the VR of patients with Behçet\'s disease were developed. The good performance of this model can help us identify potential high-risk groups for heart failure. Key Points • In this study, the predictors of VR in BD were evaluated, and a risk prediction model was developed for the early prediction of the occurrence of VR in patients with BD. • The VR prediction model included the following indexes: arterial involvement, sex, age at hospitalization, mean arterial pressure, and skin lesions. • The risk model that we developed was better and more optimized than the models built with diagnostic criteria parameters, and visualizing and personalizing the model, a nomogram, provided clinicians with an easy and intuitive tool for practical prediction.

OBJECTIVE: We aimed to determine the prevalence of cardiovascular involvement in our Blau syndrome (BS) cohort and provide detailed analysis of their cardiovascular manifestations and outcome. We also tried to find out the risk factors for developing cardiovascular involvement.
METHODS: Clinical manifestations, laboratory findings, and treatments were reviewed. Clinical features were compared between children with cardiovascular involvement and those without angiocardiopathy.
RESULTS: A total of 38 BS children were eligible for final analysis. Among them, 13 (34.2%) developed Takayasu-like vasculitis and/or cardiopathy. Compared with those without angiocardiopathy, recurrent fever was more frequent in BS patients with cardiovascular involvement (p < 0.001). What is more, tumor necrosis factor alpha antagonists (anti-TNF) were more urgently needed in children with cardiovascular involvement (p = 0.015). BS patients with cardiovascular involvement include 4 with Takayasu-like vasculitis and 9 with cardiopathy. The onset of cardiovascular manifestations ranged from 0.75 to 18.5 years of age, with most cases occurring before school period. Symptoms were elusive and lacked specificity, such as dizziness, short of breath, and edema. Some patients were even identified because of the unexpected hypertension during follow-up. Cardiopathy and vasculitis occurred in patients with different genotypes. Imaging changes were discovered before the presentation of the typical triad in 3/4 patients with Takayasu-like vasculitis. Three children developed left ventricular dysfunction with decreased left ventricular ejection fraction. Combination of glucocorticoids and methotrexate with anti-TNF agents is a common treatment option for these BS patients. In the cohort, BS-related cardiovascular involvement was controlled well, with cardiac structural and functional abnormalities completely recovered and slower progression of vasculitis lesions.
CONCLUSIONS: Cardiovascular manifestations is not rare in BS patients. Because of its insidious onset, a systematic and comprehensive assessment of cardiovascular involvement should be performed in newly diagnosed patients with BS. Aggressive initiation of anti-TNF agents may be beneficial to improve the prognosis. Key Points • About 34.2% patients with Blau syndrome developed Takayasu-like vasculitis and/or cardiopathy. • Compared with those without angiocardiopathy, recurrent fever and application of anti-TNF agents were more frequent in BS patients with cardiovascular involvement (p < 0.001, p = 0.015) • Regular assessment of cardiovascular involvement is extremely necessary because of its insidious onset.

BACKGROUND: In multisystem inflammatory syndrome (MIS-C), children typically present high-grade fever, gastrointestinal symptoms, Kawasaki-like symptoms, and even a toxic shock-like syndrome days to weeks after recovering from SARS-CoV-2 infection. It is important to raise awareness of this condition in order to have early diagnosis and immediate treatment of patients. We have, herein, reported 44 cases of MIS-C with various risk factors and symptoms. Furthermore, we have emphasized the efficacy of experience in treating children with MIS-C with high-dose corticosteroids as an alternative to immunoglobulin in low-income countries.
METHODS: We conducted a targeted survey of MIS-C from early May 2020 to October 2022 on 44 children and adolescents with characteristics of multisystem inflammatory syndrome admitted to the pediatric department of the university hospital center in Oujda, Morocco, to which patients diagnosed with MIS-C were referred. The case definition included six criteria: serious illness leading to hospitalization, age under 18 years, fever of at least 24 hours, laboratory evidence of inflammation, multi-organ involvement, biological inflammatory syndrome, and evidence of coronavirus infection based on polymerase chain reaction, antibody testing or exposure to people with COVID-19 in the past month. The criteria used to diagnose myocarditis were impaired left ventricular function, central mitral leak, and elevation of BNP or pro-BNP. Coronary involvement was assessed by the z-score and the criteria for its presence was a z-score equal to or greater than 2.5.
RESULTS: Our study included 44 children and adolescents with MIS-C in our hospital, with male predominance (79%) and a median age of six years. Cardiovascular involvement was present in 91%, mucocutaneous in 78%, gastrointestinal in 70%, hematologic in 84%, and respiratory in 2% of patients. Coronary abnormalities (z-score ≥ 2.5) were documented in 21 cases (48%). Glucocorticoids were frequently used in comparison to immunoglobulin, which were uncommonly available and expensive.
CONCLUSIONS: The therapeutic protocol that was adopted was high doses of short-term prednisone (Cortancyl) at 4mg/kg/day for 4 days. Favorable outcome was noted in all patients over a 2-year period.

Background and objectives: Multisystem inflammatory syndrome in children (MIS-C) poses challenges to the healthcare system, especially with frequent heart involvement. The current retrospective observational study aims to summarize the type and degree of cardiovascular involvement in children with MISC and to find possible associations between laboratory, inflammatory, and imaging abnormalities and the predominant clinical phenotype using a cluster analysis. Material and methods: We present a retrospective observational single-center study including 51 children meeting the MIS-C criteria. Results: Fifty-three percent of subjects presented with at least one sign of cardiovascular involvement (i.e., arterial hypotension, heart failure, pericardial effusion, myocardial dysfunction, pericarditis without effusion, myocarditis, coronaritis, palpitations, and ECG abnormalities). Acute pericarditis was found in 30/41 of the children (73%) assessed using imaging: 14/30 (46.7%) with small pericardial effusion and 16/30 (53.3%) without pericardial effusion. The levels of CRP were significantly elevated in the children with pericarditis (21.6 ± 13 mg/dL vs. 13.9 ± 11 mg/dL, p = 0.035), and the serum levels of IL-6 were higher in the children with small pericardial effusion compared to those without (191 ± 53 ng/L vs. 88 ± 27 ng/L, p = 0.041). Pericarditis with detectable pericardial effusion was significantly more frequent in the female vs. male subjects, 72% vs. 30% (p = 0.007). The hierarchical clustering analysis showed two clusters: Cluster 1 includes the children without cardiovascular symptoms, and Cluster 2 generalizes the MIS-C children with mild and severe cardiovascular involvement, combining pericarditis, myocarditis, heart failure, and low blood pressure. Also, subjects from Cluster 2 displayed significantly elevated levels of fibrinogen (5.7 ± 0.3 vs. 4.6 ± 0.3, p = 0.03) and IL-6 (158 ± 36 ng/mL vs. 66 ± 22 ng/mL, p = 0.032), inflammatory markers suggestive of a cytokine storm. Conclusions: Our results confirm that children with oligosymptomatic MIS-C or those suspected of long COVID-19 should be screened for possible cardiological involvement.

OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis and is characterized by inflammation of blood vessels. The aim of the present study was to assess cardiac valvular changes in patients with GPA in a cohort of 105 patients followed for a mean of six years.
METHODS: We followed 105 patients (mean age 50.4 years, 67 female) for a mean of 6.2±1.3 years. Echocardiography and laboratory tests were performed in all patients.
RESULTS: At baseline, 43% of patients were diagnosed with aortic regurgitation (AR), which was the most common valvular lesion. Moreover, it was the only valvular involvement that significantly increased during observation (p=0.01). In a multivariate model, only D-dimer level was a predictor of AR in this group of patients (OR 8.0 (95% CI: 1.7-38.2, p=0.01).
CONCLUSIONS: Involvement of the heart valves is a common finding in patients with GPA, but significant valvular disease is a rare complication. The most common valvular disease in this group of patients is AR. Aortic valves are also the most prone to degeneration in the course of the vasculitis.

UNASSIGNED: Lysosomal storage diseases (LSDs) are rare, progressive, multi-organ disorders caused by inherited enzyme deficiencies. Gaucher\'s disease (GD) is the most prevalent form of LSDs.
UNASSIGNED: A 19-year-old Caucasian male presented with exertional dyspnoea. Physical examination revealed a Grade III/VI systolic diamond murmur at the heart base and a Grade IV/VI systolic murmur at the apex. Electrocardiogram showed signs of left ventricular hypertrophy (LVH). Trans-thoracic echocardiography (TTE) and trans-oesophageal echocardiography (TEE) demonstrated moderate LVH, severe aortic valve stenosis, severe supra-valvular aortic stenosis, and moderate mitral stenosis with severe degenerative mitral valve regurgitation. Bone marrow biopsy and aspiration confirmed the presence of characteristic Gaucher\'s cells. The patient underwent the Bentall procedure and mitral valve replacement and was discharged in good condition.
UNASSIGNED: Gaucher\'s disease exhibits three clinical phenotypes, and cardiovascular involvement is commonly seen in GD Type III. Valvular calcification and ascending aorta involvement are frequent cardiovascular manifestations. Although severe valvular heart involvement is rare in GD, cardiac valve surgery has shown favourable outcomes in previous studies and our case.

UNASSIGNED: Behcet\'s disease (BD) is a multi-systemic inflammatory vasculitis which may be life-threatening if combined with cardiovascular problems. The aim of the study was to identify potential risk factors associated with cardiovascular involvement in BD.
UNASSIGNED: We reviewed the medical databases of a single center. All BD patients identified as fulfilling the 1990 International Study Group criteria or the International Criteria for Behcet\'s Disease criteria. Cardiovascular involvement, clinical manifestations, laboratory features, and treatments were recorded. The relationship between parameters and cardiovascular involvement was analyzed.
UNASSIGNED: 111 BD patients were included: 21 (18.9%) had documented cardiovascular involvement (CV BD group) and 99 (81.1%) had no cardiovascular involvement (non-CV BD group). Compared with non-CV BD, the proportion of males and smokers were significantly increased in CV BD (p = 0.024 and p < 0.001, respectively). Levels of activated partial thromboplastin time (APTT), cardiac troponin I and C-reactive protein were significantly higher (p = 0.001, p = 0.031, and p = 0.034, respectively) in the CV BD group. Cardiovascular involvement was associated with smoking state, the presence of papulopustular lesions, and higher APTT in multivariate analyzed (p = 0.029, p = 0.021, and p = 0.006, respectively). The ROC curve showed that APTT predicts the risk of cardiovascular involvement (p < 0.01) at a cut-off value of 33.15 s with a sensitivity of 57.1% and specificity of 82.2%.
UNASSIGNED: Cardiovascular involvement in BD patients was associated with gender, smoking state, the presence of papulopustular lesions, and higher APTT. All patients newly diagnosed with BD should be systematically screened for cardiovascular involvement.

Although primarily a lung disease, extra-pulmonary tuberculosis (TB) can affect any organ or system. Of these, cardiovascular complications associated with disease or drug toxicity significantly worsen the prognosis. Approximately 60% of patients with TB have a cardiovascular disease, the most common associated pathological entities being pericarditis, myocarditis, and coronary artery disease. We searched the electronic databases PubMed, MEDLINE, and EMBASE for studies that evaluated the impact of TB on the cardiovascular system, from pathophysiological mechanisms to clinical and paraclinical diagnosis of cardiovascular involvement as well as the management of cardiotoxicity associated with antituberculosis medication. The occurrence of pericarditis in all its forms and the possibility of developing constrictive pericarditis, the association of concomitant myocarditis with severe systolic dysfunction and complication with acute heart failure phenomena, and the long-term development of aortic aneurysms with risk of complications, as well as drug-induced toxicity, pose complex additional problems in the management of patients with TB. In the era of multidisciplinarity and polymedication, evidence-based medicine provides various tools that facilitate an integrative management that allows early diagnosis and treatment of cardiac pathologies associated with TB.

A special form of the rare infantile Sweet syndrome (acute febrile neutrophilic dermatosis) is facultative healing in the form of postinflammatory elastolysis with acquired cutis laxa, named \"Marshall\" syndrome after the authors who first described it. We report the case of a 3-year-old child in whom the cutaneous manifestation led to diagnosis of Takayasu arteritis. Postinflammatory elastolysis with acquired cutis laxa is a clinically relevant cutaneous indicator of life-threatening cardiovascular complications such as aortitis, aortic aneurysm, coronary stenosis and heart failure in children with Sweet\'s syndrome. Cutis laxa usually precedes cardiac complications or, as in our case, occurs simultaneously; thus, immediate cardiac and rheumatologic examinations are important to initiate systemic therapy with anti-inflammatory and immunomodulatory agents early to prevent complications.
UNASSIGNED: Eine Besonderheit des seltenen Sweet-Syndroms des Kindesalters ist die fakultative Abheilung in Form einer postinflammatorischen Elastolyse mit erworbener Cutis laxa, die nach dem Erstbeschreiber als Marshall-Syndrom benannt wird. Wir berichten von einem 3‑jährigen Kind, bei dem ein derartiges Sweet-Syndrom zur Erstdiagnose einer Takayasu-Arteriitis führte. Die postinflammatorische Elastolyse mit erworbener Cutis laxa stellt beim kindlichen Sweet-Syndrom einen klinisch relevanten kutanen Indikator für lebensbedrohliche kardiale Gefäßkomplikationen wie Aortitis, Aortenaneurysma, Koronararterienstenose und Herzversagen dar. Da das Cutis-laxa-artig abheilende Sweet-Syndrom den kardialen Komplikationen zumeist zeitlich vorausgeht oder wie in unserem Fall simultan auftritt, sollten die betroffenen Patienten umgehend kardiologisch und rheumatologisch untersucht werden, um bei vaskulärer Beteiligung einen komplikativen Verlauf durch frühe antiinflammatorische und immunmodulierende Systemtherapie zu verhindern.

Coronavirus Disease 2019 (COVID-19) has been a pandemic challenge for the last year. Cardiovascular disease is the most described comorbidity in COVID-19 patients, and it is related to the disease severity and progression. COVID-19 induces direct damage on cardiovascular system, leading to arrhythmias and myocarditis, and indirect damage due to endothelial dysfunction and systemic inflammation with a high inflammatory burden. Indirect damage leads to myocarditis, coagulation abnormalities and venous thromboembolism, Takotsubo cardiomyopathy, Kawasaki-like disease and multisystem inflammatory syndrome in children. Imaging can support the management, assessment and prognostic evaluation of these patients. Ultrasound is the most reliable and easy to use in emergency setting and in the ICU as a first approach. The focused approach is useful in management of these patients due its ability to obtain quick and focused results. This tool is useful to evaluate cardiovascular disease and its interplay with lungs. However, a detailed echocardiography evaluation is necessary in a complete assessment of cardiovascular involvement. Computerized tomography is highly sensitive, but it might not always be available. Cardiovascular magnetic resonance and nuclear imaging may be helpful to evaluate COVID-19-related myocardial injury, but further studies are needed. This review deals with different modalities of imaging evaluation in the management of cardiovascular non-ischaemic manifestations of COVID-19, comparing their use in emergency and in intensive care.