Dutch survey of congenital coronary artery fistulas in adults

Abstract

Aims: This Dutch survey focused on the clinical presentation, noninvasive and invasive diagnostic methods, and treatment modalities of adult patients with congenital coronary artery fistulas (CAFs).

Methods: Between 1996 and 2003, the initiative was taken to start a registry on congenital CAFs in adults. In total 71 patients from a diagnostic coronary angiographic population of 30,829 at 28 hospitals were collected from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques and treatment options were retrospectively collected and analysed.

Results: Out of 71 patients with angiographically proven CAFs, 51 (72%) had 63 congenital solitary fistulas and 20 (28%) had 31 congenital coronary-ventricular multiple microfistulas. Patients with pseudofistulas were excluded from the registry. Coronary angiograms were independently re-analysed for morphology and specific fistula details. The majority (72%) of the fistulas were unilateral, 24% were bilateral and only 4% were multilateral. The morphological characteristics of these 94 fistulas were as follows: the origin was multiple in 47% and single in 53%; the termination was multiple in 52% and single in 48%; and the pathway of the fistulous vessels was tortuous/multiple in 66%, tortuous/single in 28%, straight/multiple in 3% and straight/single in 3%. Percutaneous transluminal embolisation (PTE) was performed in two (3%) patients; surgical ligation was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conservative medical management. The total mortality was 6% (4/71) at a mean follow-up period of approximately five years. Cardiac mortality accounted for 4% (3/71); in all three patients, death could possibly be attributed to the presence of the fistula.

Conclusion: Registry of congenital coronary artery fistulas in adults in the Netherlands is feasible. In spite of restrictions imposed by the Dutch Privacy Law, it was possible to include 71 adult patients with congenital coronary artery fistulas who were eligible for thorough evaluation.

Keywords: adults; coronary arteriography; coronary artery; fistula; morphology; survey.