Specific morphology of coronary artery aneurysms in mainly Caucasian Kawasaki Disease patients – Initial data from the Cardiac Catheterization in Kawasaki Disease registry

Aims and Background Patients with a history of Kawasaki disease (KD), especially those with diagnosed coronary artery involvement, are known to require long-term cardiac care. However, specific evidence-based recommendations on long-term medical strategies are missing. Cardiac catheterization (CC) is still considered the gold-standard for diagnosing detailed coronary pathology. Therefore, and to better understand coronary artery pathology development in the long-term, we conducted a survey to document and evaluate CC data in a European population. Here we describe initial data on the first catheter examination these patients underwent. Method We administered a standardized questionnaire to retrospectively analyze CC data from KD children from the year 2010 until April 2023. This register covers basic acute phase clinical data and, more importantly, detailed information on morphology, distribution and the development of coronary artery pathologies. Data on participating departments of pediatric cardiology, mainly from Germany, were evaluated, with this study focusing on investigating each patient’s first CC exclusively. Results We analyzed a total of 164, mainly Caucasian, patients (65% male) across 14 pediatric cardiology departments. A relevant number of patients had no coronary artery aneurysm at the CC, facing the fact that distal CAAs were almost exclusively detected alongside proximal CAAs. Patients with multiple CAAs revealed a significant positive correlation between the number of CAAs and their dimensions, in diameter, as in length. Location of the CAA within the coronary artery, age at KD’s onset or gender did not significantly influence CAA diameters, but CAAs were longer in older children and in males. Conclusion The fact of distal CAAs being only present together with proximal ones, will hopefully reduce diagnostic CCs in KD patients without echocardiographically detected proximal CAAs. Furthermore this study gives valuable insights into dimensional specifics of CAAs in KD patients. As an ongoing register, future analyses will further explore long-term outcomes and performed treatments, helping to refine clinical long-term strategies for KD patients. Clinical perspective Clinical Trial Registration Cardiac Catheterization in Kawasaki Disease – data from the central European registry from 2010 – today DRKS-ID: DRKS00031022, Date of Registration: 16.01.2023 ### Competing Interest Statement The authors have declared no competing interest. ### Clinical Trial Cardiac Catheterization in Kawasaki Disease - data from the central European registry from 2010 - today DRKS-ID: DRKS00031022, Date of Registration: 16.01.2023 ### Funding Statement none ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: Ethical consent from the "Ethikkommission LMU Munich", Pettenkoferstrasse 8, 80336 München, Project-Nr:19-997, date of approval 17.03.2020 I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes Data, consistent with the declareation of Helsinki and German data protection law, are available upon request. * KD : Kawasaki Disease CC : Cardiac Catheterization CAA : Coronary Artery Aneurysm AP : Acute Phase IVIG : Intravenous Immunoglobulin AHA : American Heart Association JCS : Japanese Circulation Society IQR : Interquartile Range BSA : Body Surface Area RCA : Right Coronary Artery LCA : Left Coronary Artery LAD : Left Anterior Descending Coronary Artery LCX : Left Circumflex Coronary Artery SNP : Single Nucleotide Polymorphism ITPKC : Inositol 1,4,5-trisphosphate 3-kinase C MMP : Matrix Metalloproteinases