Coronary Artery Z-scores in Febrile Children with Suspected Kawasaki's Disease-The Value of Serial Echocardiography.

BACKGROUND: Progressive enlargement of the coronary artery (CA) diameters on serial echocardiography can support diagnosis of Kawasaki's disease (KD) even CA dimensions are within the normal range. METHODS: A single-center, retrospective study compared mean Z-scores of the proximal CA internal diameters in children hospitalized with non-KD febrile illnesses (FCs) with those of KD patients. RESULTS: A total of 223 patients with suspicion of KD have been admitted over a period of 16 years and data were evaluable for 176 children. Distributions for age, sex, and body surface area were similar for both groups. FC had a significantly shorter duration of hospitalization, higher levels of hemoglobin, lower levels of liver transaminases, and segmented neutrophils, respectively. The majority of FC patients (75/82, 91.5%) had normal CA Z-scores (p < 0.001) and only 3 (3.7%) had CA Z-score ≥2.5 standard deviation (SD). In KD, subjects (46/94, 49.5%) had a CA dilation (Z-score ≥2.5 SD) and the maximum CA Z-score (Zmax) was significantly higher compared with FC patients (p < 0.001). On serial echocardiograms, FC patients showed a mild decrease, whereas KD patients developed a significant increase of CA Zmax (p < 0.001). Seven KD patients had a segmental dilation of a CA which has been confirmed by cardiac catheter. In FC, no segmental dilation of any CA was documented by echocardiography. CONCLUSION: This study found that mean CA dimensions in FCs were smaller and did not increase in serial echocardiograms compared with KD patients.

Value of serial echocardiography in diagnosing Kawasaki's disease.

Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. Early diagnosis and treatment is mandatory in incomplete KD to reduce the risk of coronary involvement. Between 2002 and 2018, 124 patients have been diagnosed suffering from KD at the University Children's Hospital Regensburg (KUNO). We assessed luminal diameters of both CAs normalized as Z-scores by 2D-echocardiography. A total of 94 patients were analyzed. Of them, 31 (33%) were affected by an incomplete form of KD. In 24 children (26%), serial echocardiography was necessary in order to confirm diagnosis. Mean Z-scores for the left main coronary artery (LMCA), right main coronary artery (RMCA), and left anterior descending artery increased significantly between the initial (LMCA 0.79z, RMCA 0.15z, LAD 0.49z) and second (LMCA 1.69z, RMCA 0.99z, LAD 1.69z) examination (p < 0.05).Conclusion:To confirm diagnosis of KD, it might not be necessary to detect dilation or aneurysms. Our observation suggests that patients suspected having KD should be monitored with serial echocardiography in order to detect a possible enlargement of the CA diameters, even if Z-scores are within the normal range. What is Known: • Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. • Due to less classic clinical criteria in patients with incomplete KD, the risk for CA pathology is even higher. What is New: • A significant progression of patients' CA Z-scores in serial echocardiographic measurements may be helpful to ensure diagnosis of KD early even if Z-scores are within the normal range. • Twenty-seven patients (90%) with incomplete KD could be diagnosed within 10 days of fever, early enough to prevent significantly higher rates of CA aneurysm.