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Isolated congenital coronary arteriovenous fistula
V Chopra1, A Saxena, S S Kothari
1Department of Cardiology, All India Institute of Medical Sciences, New Delhi.
Insights
Congenital coronary arteriovenous fistula (CAVF) can occur at any age, often originating from the right coronary artery with small shunts. Surgical ligation or coil embolization are safe treatment options for CAVF.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Congenital coronary arteriovenous fistula (CAVF) is a rare condition.
- Diagnosis and management of CAVF can be challenging.
- Understanding the presentation and outcomes of CAVF is crucial for effective treatment.
Purpose of the Study:
- To review the clinical characteristics, management, and outcomes of patients diagnosed with isolated congenital coronary arteriovenous fistula (CAVF) at a single center.
- To evaluate the safety and efficacy of surgical ligation and coil embolization for CAVF treatment.
- To identify factors influencing patient outcomes and inform clinical decision-making.
Main Methods:
- Retrospective review of nine patients diagnosed with isolated CAVF over eight years.
- Analysis of patient demographics, fistula origin and drainage, hemodynamic parameters, and treatment modalities.
- Assessment of clinical outcomes and follow-up data.
Main Results:
- Nine patients (7 male, 2 female) aged 11 days to 22 years were diagnosed with CAVF.
- Most fistulae originated from the right coronary artery and drained into the right ventricle.
- Surgical ligation and coil embolization were successfully employed, with all patients remaining asymptomatic post-treatment.
Conclusions:
- Congenital coronary arteriovenous fistula (CAVF) can present across a wide age range, with a predilection for the right coronary artery and small left-to-right shunts.
- Both surgical ligation and coil embolization are safe and effective treatment options for CAVF.
- Conservative management may be suitable for selected cases, with all patients achieving an asymptomatic status.
Abstract:
During the last eight years a total of nine cases of isolated congenital coronary arteriovenous fistula (CAVF) have been diagnosed at our centre. Age of these patients ranged from 11 days to 22 years (mean 6.4 +/- 4.2 years). There were seven male and two female patients. Right and left coronary arteries were the sites of origin in seven and three cases respectively. One patient had two fistulae, one each originating from right coronary artery and obtuse marginal artery. The drainage sites were right ventricle in seven cases and right atrium in two. The pulmonary artery pressure was normal in eight cases and elevated in one case. The pulmonary artery to systemic flow ratio ranged from 1.1:1-2.4:1 in all except the 11 day-old-baby in whom it was 4:1. Pulmonary vascular resistance (PVRI) was below 3.5 in all cases (range 1.0-3.5 units). Surgical ligation of fistula was done in four cases and two patients underwent coil embolisation of fistula. Follow-up is available in five out of eight patients including three cases in whom CAVF was closed. Two other young patients are being followed-up conservatively and all the patients are asymptomatic. CAVF can present at any age; majority of the cases have small left to right shunts and more commonly CAVF arise from right coronary artery. Surgical ligation of fistula is safe in older patients, non-surgical embolisation using coils may be an alternative to surgery in selected cases.