We describe two children, each with a univentricular circulation who had evidence of elevated pulmonary arterial pressure at cardiac catheterization. Both children developed extensive coronary arterial fistulae detected at cardiac catherization. Each child was medically treated with a combination of phosphodiesterase-5 inhibitor and endothelin antagonist pulmonary vasodilators. The presence of chronic hypoxaemia, elevated filling pressures, and the use of pulmonary vasodilators may contribute to coronary endothelial dysfunction, which may result in coronary vasculopathy. This may necessitate early referral for orthotopic cardiac transplantation. © 2013 Wiley Periodicals, Inc.
Keywords: coronary artery; fistula; pulmonary arterial hypertension; univentricular circulation; vasculopathy.
© 2013 Wiley Periodicals, Inc.