Myocardial Fistulisation and Coronary Arterial Ectasia in Children with Univentricular Circulation: An Under-Recognised Problem

Among 241 children with univentricular circulation who underwent total cavopulmonary connection (Glenn or Fontan) over a 17 years period (January 2000–April 2017), we found two patients who had diffuse coronary fistulisation of their myocardium (0.8%). We also report an additional third case from Oak Lawn, Illinois, USA. One of the children had documented normal coronary arteries and myocardium at catheterisation prior to development of the fistulisation process. The same child also developed extensive veno-venous collaterals. All children had evidence of elevated pulmonary arterial pressures and were treated with a combination of pulmonary vasodilators. The presence of chronic hypoxaemia and elevated filling pressures could potentially have contributed to coronary endothelial dysfunction, thereby giving rise to the coronary vasculopathy and myocardial fistulisation. Alternatively, this myocardial fistulisation process may be present from early in life and not recognised. This may necessitate early referral for orthotopic cardiac transplantation. Diffuse fistulisation of the myocardium in children and young adults with univentricular circulation may be an under-recognised occurrence and coronary angiography should be considered in these patients.