Cardiac Catheter Examination in Kawasaki Disease: Initial Data from the Central European Registry

Background: Patients with a history of Kawasaki disease (KD), especially those with diagnosed coronary artery involvement, are known to require long-term cardiac care. Cardiac cauterization (CC) remaining the gold standard for diagnosing coronary pathology, we conducted an international survey to document and evaluate the clinical impact of CC performed on KD patients. Herewith, we present our first preliminary data.

Method: We used a standardized questionnaire to retrospectively analyze CCs performed on KD children from the year 2010 until today. Acute phase clinical data, detailed morphology, distribution and development of coronary artery pathologies, but also pursued therapeutic strategies are covered in this registry.

Results: A total of 163 patients (65% male) undergoing 246 CCs have been recorded by 14 centers of pediatric cardiology from Germany, Switzerland, and Sweden. Median age during KD's acute phase was 1.7 years with a mean follow-up period of 27 to months, range of 0 month to 21 years (time between acute phase and first CC). Almost 60% of the patients had at least one coronary artery aneurysm (CAA) and up to 8 aneurysms in one single patient have been detected.

The right coronary artery's (RCA) proximal segment was the most frequent to display aneurysms (55/196), and also the one to deliver CAAs with the largest diameters and lengths (mean/range of the CAA diameter × length): 6 mm/1.9–31 mm × 10.7 mm/2.8–62 mm). Cross-table analysis revealed that the distal segments exhibit CAAs almost exclusively, if proximal segments had been also affected; there were only two patients having isolated distal coronary artery involvement.

Furthermore, we found a positive correlation between the maximum diameter (and respective Z-Score) and the total number of aneurysms per patient (RP = 0.54; p < 0.0001).

Conclusion: According to our first data-analysis, the proximal right coronary artery was the most affected vessel. There was a significant positive correlation between the number of CAAs and the maximum CAA diameter. Knowing that distal CAAs occur more often when proximal located CAAs exist, may influence the long-term cardiac follow-up strategies in KD patients.

Publication History

Article published online:
28 January 2023