Coronary Arteriovenous Fistula and Kawasaki Disease

  • Bo Sang Kwon, Korea
  • Gi Beom Kim, Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea, Korea
  • Eun Jung Bae, Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea, Korea
  • Chung Il Noh, Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea, Korea
  • Yong Soo Yun, Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea, Korea
  • Jung Yun Choi, Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea, Korea

    • Introduction: Coronary arteriovenous fistula (CAVF) from the coronary artery to the pulmonary artery is a rare anomaly; however, its natural history and clinical course, especially related to Kawasaki disease (KD), have been still unclear. The purpose of this study was to identify the natural history and the relationship to Kawasaki disease.
    Methods: We studied retrospective analysis of the patients diagnosed as CAVF from 1996 to 2007 in a tertiary center, excluding the iatrogenic cases.
    Results: The mean age of 36 patients(M:F=20:16) was 5.4±5.4(range:0.1-22.4) years. The mean age at diagnosis and duration of follow-up was 3.9±4.8(0.1-18.4) years and 1.8±1.8(0.1-5.3) years, respectively. The fistulous opening sites were the main pulmonary artery in 35 and the left pulmonary artery in 1. Initial presentations were cardiac murmur in 7, incidental detection in 24 including 6 KD patients (17%), chest pain in 2, and congestive heart failure in 1. Fistulous opening in 8 patients spontaneously disappeared after 2.4±2.2 (0.67-5) years but that in 28 has persisted for 1.6±1.8(0.05-4) years. In KD, the duration of febrile illness was 6.3±2.4 (5-10) days and coronary artery size was nearly normal(n=3) and 3~3.5 mm–sized dilatation(n=3). All KD patients were typical Kawasaki diseases. Although only 1 patient had abnormal myocardial SPECT findings, decreased anterior wall perfusion defect, he had no ischemic symptoms and normal ECG.
    Conclusion: CAVF was frequently observed in KD patients. Although KD might be one of causes in CAVF, further studies are needed in order to search the etiology or causal relationship with KD.