Myocardial Fistulisation and Coronary Arterial Ectasia in Children with Univentricular Circulation: An
Daragh Finn1, Kevin Walsh1, David Roberson2
1Department of Paediatric Cardiology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
Insights
Diffuse coronary fistulisation, a rare condition affecting the heart
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Vascular Biology
Background:
- Univentricular circulation presents complex challenges in pediatric cardiac care.
- Total cavopulmonary connection (TCPC) procedures like Glenn and Fontan are common surgical interventions.
- Coronary artery abnormalities can occur post-TCPC, impacting patient outcomes.
Purpose of the Study:
- To investigate the incidence and characteristics of diffuse coronary fistulisation in children post-TCPC.
- To explore potential contributing factors and clinical implications of this rare complication.
- To raise awareness and suggest diagnostic considerations for this under-recognized condition.
Main Methods:
- Retrospective review of 241 children undergoing TCPC (Glenn or Fontan) between January 2000 and April 2017.
- Case identification of patients with diffuse coronary fistulisation.
- Analysis of clinical data, including pre- and post-operative coronary assessments, pulmonary pressures, and treatments.
Main Results:
- Two cases (0.8%) of diffuse coronary fistulisation of the myocardium were identified among 241 children post-TCPC.
- An additional third case was reported, with one patient having normal initial coronary arteries.
- All affected children exhibited elevated pulmonary arterial pressures and received pulmonary vasodilators.
Conclusions:
- Diffuse coronary fistulisation is a potentially under-recognized complication in children with univentricular circulation after TCPC.
- Chronic hypoxemia and elevated filling pressures may contribute to coronary endothelial dysfunction and vasculopathy.
- Coronary angiography should be considered in these patients, and early cardiac transplantation may be necessary.
Abstract:
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.
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