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Extensive coronary arterial fistula development in patients with univentricular circulation
Colin J McMahon1, David Coleman, Paul Oslizlok
1Department of Paediatric Cardiology, Crumlin, Dublin, 12, Ireland.
Insights
Two children with single ventricle circulation and high pulmonary pressure developed coronary arterial fistulae. Medical treatment may cause coronary vasculopathy, potentially requiring heart transplantation.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Vascular Biology
Background:
- Univentricular circulation presents complex challenges in pediatric cardiology.
- Elevated pulmonary arterial pressure is a significant concern in these patients.
- Coronary arterial abnormalities can complicate single ventricle physiology.
Observation:
- Two pediatric patients with univentricular circulation exhibited elevated pulmonary arterial pressure.
- Cardiac catheterization revealed extensive coronary arterial fistulae in both children.
- These patients were treated with phosphodiesterase-5 inhibitors and endothelin antagonists.
Findings:
- Medical management with pulmonary vasodilators was initiated for elevated pulmonary arterial pressure.
- Chronic hypoxemia and elevated filling pressures were noted.
- The combination of factors may lead to coronary endothelial dysfunction and vasculopathy.
Implications:
- Coronary vasculopathy is a potential complication in children with single ventricle circulation and pulmonary hypertension.
- Endothelial dysfunction may be exacerbated by pulmonary vasodilator therapy.
- Early consideration for orthotopic cardiac transplantation may be necessary for affected children.
Abstract:
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.
