Specific Morphology of Coronary Artery Aneurysms in Mainly White Patients With Kawasaki Disease: Initial Data From the Cardiac Catheterization in Kawasaki Disease Registry

Abstract

Background: Patients with Kawasaki disease (KD) with coronary artery involvement require long-term cardiac care. Although respective evidence-based recommendations are missing, cardiac catheterization is still considered the gold standard for diagnosing detailed coronary pathology. Therefore, to better understand coronary artery pathology development, we conducted a survey to document and evaluate cardiac catheterization data in a European population.

Methods and results: We retrospectively analyzed cardiac catheterization data from KD children from the year 2010 until April 2023. This registry covers basic acute-phase clinical data, and more importantly, detailed information on morphology, distribution, and the development of coronary artery pathologies. A total of 164 mainly White patients (65% boys) were included. A relevant number of patients had no coronary artery aneurysm (CAA) at the cardiac catheterization, indicating 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 and in length. Location of the CAA within the coronary artery, age at onset of KD, or natal sex did not significantly influence CAA diameters, but CAAs were longer in older children and in boys.

Conclusions: That distal CAAs were only present together with proximal ones will hopefully reduce diagnostic CCs in patients with KD without echocardiographically detected proximal CAAs. Furthermore, this study gives valuable insights into dimensional specifics of CAAs in patients with KD. As an ongoing registry, future analyses will further explore long-term outcomes and performed treatments, helping to refine clinical long-term strategies for patients with KD.

Registration: URL: https://drks.de/; Unique Identifier: DRKS00031022.

Keywords: Kawasaki disease; White; cardiac catheterization; coronary artery pathology; long‐term cardiac care.

Figure 1. Flowchart.

CAA in the AP/1.CC. Comparison of CAAs present in the AP vs at 1.CC including the median (range) time interval AP‐1.CC in months, indicating that 1.CC was performed within a shorter time interval when CAAs were present (significantly for the CAAs for patients in the AP group). 1.AP indicates acute phase; CAA, coronary artery aneurysm; and CC, first cardiac catheterization.

Figure 2. Number of coronary artery aneurysms related to the AHA classification of coronary artery segments.

A, Illustration of the CA tree: segments 1 to 4: RCA, 5; LMCA, 6 to 10; LAD, 11 to 13; LCX. B, Distribution of CAA per segment (CAA extension over multiple segments included); modified AHA coronary segment classification. AHA indicates American Heart Association; CA, coronary artery; CAA, coronary artery aneurysm; LAD, left anterior descending coronary artery; LCA, left coronary artery; LCX, left circumflex coronary artery; LMCA, left main coronary artery; and RCA, right coronary artery.

Figure 3. Box plots of CAA diameter per segment.

Box plots of CAA diameter per segment of the (A) RCA and (B) LCA/LAD. Respective median/IQR are as follows: RCA segment 1, 10.07/6.90; segment 2, 9.10/7.60; segment 3, 7.51/6.90; segment 4, 4.21/1.40. LCA/LAD segment 5, 7.31/5.97; segment 6, 7.18/6.40; segment 7, 11.49/3.94; segment 8, 8.55/3.06; segment 9, 9.61/4.99; segment 10, 3.46/0.00. CAA indicates coronary artery aneurysm; IQR, interquartile range; LAD, left anterior descending coronary artery; LCA, left coronary artery; and RCA, right coronary artery.

Figure 4. Correlation of CAA count with diameter and length.

Correlation maximum number of CAAs per patient with (A) diameter, correlating index R=0.45 (P=0.0031) and (B) length, correlating index R=0.34 (P<0.001). CAA indicates coronary artery aneurysm.