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 In Caucasian Kawasaki disease (KD) patients, distal coronary artery aneurysms (CAA) are usually diagnosed in association with proximal CAAs. Additional imaging such as conventional coronary artery angiography may therefore be unnecessary, if no aneurysms are detectable echocardiographically. In this context, potentially unnecessary CCs hopefully will reduce in future. Not only the CAA diameters, but also CAA length and CAA count may influence cardiac related morbidity and should be considered in long-term follow-up care. The age at the acute phase of KD, such as gender and location of CAA within the coronary arteries seems to not affect CAA dimensions. 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