Background: Kawasaki disease (KD) is an acute febrile systemic vasculitis that affects small and medium blood vessels. Intensified treatments for the most severely affected patients have been proposed recently, and the early identification of KD patients at high risk for coronary artery aneurysms (CAA) is crucial. However, the risk scoring systems developed in Japan have not been validated in European populations, and little data is available concerning the link between initial echocardiogram findings other than high z-scores and cardiac prognosis.

Methods: In order to investigate whether the presence of any abnormalities, other than high z-scores in first echocardiogram, are associated with resistance to IV immunoglobulins and/or subsequent development of CAA, we retrospectively analyzed data from children diagnosed with KD between 2006 and 2016 at a tertiary Hospital in Paris, France.

Results: A total of 157 children were included. The initial echocardiogram was performed after a median of 7 days of fever and was abnormal in 48 cases (31%). The initial presence of any echocardiographic abnormality (coronary artery dilatation, CAA, pericardial effusion, perivascular brightness of the coronary arteries, left-ventricular dysfunction and mitral insufficiency) was strongly associated with resistance to intravenous immunoglobulin (p = 0.005) and development of coronary artery lesions within the first 6 weeks of disease (p = 0.01). All patients (n = 7) with persistent coronary abnormalities at 1 year already had an abnormal initial echocardiogram. Severity scoring systems from Japan had low sensitivity (0-33%) and low specificity (71-82%) for predicting immunoglobulin resistance or cardiac involvement.

Conclusions: In European populations with mixed ethnic backgrounds, the presence of any abnormalities at the initial echocardiogram may contribute to early identification of patients with severe disease.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6052519PMC
http://dx.doi.org/10.1186/s12969-018-0264-7DOI Listing

Publication Analysis

Top Keywords

initial echocardiogram
20
coronary artery
16
associated resistance
12
kawasaki disease
8
abnormal initial
8
echocardiogram associated
8
development coronary
8
artery lesions
8
early identification
8
identification patients
8

Similar Publications

Aortic regurgitation in ankylosing spondylitis-an echocardiography follow-up study.

Clin Rheumatol

January 2025

Department of Rheumatology and Inflammation Research, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, Göteborg, Sweden.

Objectives: To investigate the long-term course of aortic regurgitation (AR) and the width of the proximal ascending aorta (PAA) in patients with ankylosing spondylitis (AS).

Method: This is a follow-up cohort study of patients with AS examined with echocardiography at inclusion (2009 to 2011). Out of the initial 187, a subgroup of 52 patients (54% men, mean age 62 years) was selected for follow-up based on presence/absence of AR at baseline; 26 with AR (18 mild, 7 moderate, 1 severe) and 26 age/sex-matched without AR.

View Article and Find Full Text PDF

is rarely associated with neurological manifestations. This report describes a rare case of endocarditis complicated by a cerebral stroke caused by . We also briefly reviewed the neurological clinical spectrum of disease described in the literature.

View Article and Find Full Text PDF

Comprehensive treatment strategy in a patient with systemic lupus erythematosus-related pulmonary artery hypertension: a case report.

Eur Heart J Case Rep

January 2025

Division of Cardiology, Department of Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa Ward, Tokyo 142-8555, Japan.

Background: Although the prognosis in systemic lupus erythematosus (SLE) has dramatically improved, pulmonary artery hypertension (PAH) is one of the life-threatening comorbidities associated with SLE. The management of the comorbidity is occasionally challenging due to the lack of consensus regarding treatment options including immunosuppressive agents, selective pulmonary vasodilators, and cardiac rehabilitation.

Case Summary: A 28-year-old female who terminated prednisolone after remission of SLE by her own discretion 3 years ago developed dyspnoea on effort.

View Article and Find Full Text PDF

Aims: Early identification of healthy subjects prone to develop cardiac dysfunction may be instrumental to prevention strategies. Our study aimed to evaluate whether circulating levels of growth differentiation factor-15 (GDF-15) could predict adverse changes in echocardiographic indexes of cardiac structure and function in an initially healthy populational familial cohort with a long follow-up (STANISLAS cohort).

Methods And Results: We evaluated 1679 participants (49 ± 14 years, 48% males) included in the fourth visit (V4) of the STANISLAS cohort with available GDF-15 measurements (Olink proteomic analysis) and echocardiographic parameters.

View Article and Find Full Text PDF

Aims: Heart failure (HF) patients may lack improvement of left ventricular (LV) ejection fraction (LVEF) despite optimal HF medication comprising an angiotensin receptor-neprilysin inhibitor (ARNI). Therefore, we aimed to identify key predictors for LV functional enhancement and prognostic reverse cardiac remodelling in HF patients on ARNI treatment.

Methods: We retrospectively analysed 294 consecutive patients with HF with reduced (HFrEF) or mildly reduced (HFmrEF) ejection fraction in our 'EnTruth' patient registry.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!