Three cases of multiple coronary arterial fistulae

  • Dr Hiromichi Nakajima, Chiba children’s Hospital, Japan
  • Dr Hiroyuki Aotsuka, Chiba children’s Hospital, Japan
  • Dr Shunsuke Tatebe, Chiba children’s Hospital, Japan
  • Dr Ryouta Ebata, Chiba children’s Hospital, Japan
  • Dr Mitsuru Aoki, Chiba children’s Hospital, Japan
  • Dr Tadashi Fujiwara, Chiba children’s Hospital, Japan

    • Multiple coronary arterial fistulae (CAF) is a very rare malformation. We present three cases of this anomaly.
    The first patient was a 7-year-old girl with tricuspid atresia type I-A and persistent left superior vena cava (PLSVC). After Modified Blalock-Taussig shunt in neonatal period, total cavo-pulmonary connection (TCPC) was done at 1.5 years of age. The coronary sinus (CS) was confirmed and the PLSVC was ligated. Catheter examination after TCPC showed multiple diffuse CAF from both coronary arteries to the left ventricle. The coronary diameter was enlarged up to 5 mm. LVEDV was 192% of normal. She was stable using ACE inhibitor.
    The second patient was a 4-year-old girl with large muscular VSD. PAB was performed at the age of 8 months. Catheter examination showed diffuse CAF from both coronary to the right ventricle. She was died soon after VSD patch closure because of left ventricular dysfunction. An autopsy revealed septal infarction and stenotic orifice of CS.
    The third patient was 2-year-old male with double outlet right ventricle and PDA. PAB and PDA ligation was performed at neonatal period. He also had minor chromosomal anomaly (46XY(add(9) (q34,3)). Angiography showed diffuse CAF mainly to the right ventricle. Concerning about possible ischemia by right ventricular decompression, we avoided biventricular repair and performed bidirectional Glenn procedure successfully.
    [Conclusion]
    The etiology of diffuse type CAF is still unknown. Stenosis of CS might be a cause of this disease. A risk of ischemia after the pressure reduction of CAF should be considered in planning intra-cardiac surgery.