Diagnostic and therapeutic approach of congenital solitary coronary artery fistulas in adults: Dutch case series and review of literature

Abstract

Background: Coronary artery fistulas (CAFs) are infrequent anomalies, coincidentally detected during coronary angiography (CAG).

Aim: To elucidate the currently used diagnostic imaging modalities and applied therapeutic approaches.

Materials and methods: Five Dutch patients were found to have CAFs. A total of 170 reviewed subjects were subdivided into two comparable groups of 85 each, treated with either percutaneous 'therapeutic' embolisation (PTE group) or surgical ligation (SL group).

Results: In our series, the fistulas were visualised with several diagnostic imaging tests using echocardiography, multidetector computed tomography, and CAG. Four fistulas were unilateral and one was bilateral; five originated from the left and one originated from the right coronary artery. Among the reviewed subjects, high success rates were found in both treatment groups (SL: 97% and PTE: 93%). Associated congenital or acquired cardiovascular disorders were frequently present in the SL group (23%). Bilateral fistulas were present in 11% of the SL group versus 1% of the PTE group. The fistula was ligated surgically in one and abolished percutaneously in another. Medical treatment including metoprolol was conducted in two, and watchful waiting follow-up was performed in one.

Conclusions: Several diagnostic imaging techniques are available for assessment of the anatomical and functional characteristics of CAFs.

Fig. 1

a Left anterior oblique (LAO) frame with cranial angulations shows CAFs from the Cx to the CS (arrow). b Right anterior oblique (RAO) frame with cranial angulations shows CAFs from Cx to CS (arrow); ventricular lead of the pacemaker is well appreciated. c RAO caudal angiographic view illustrates a fistula from the distal segment of the Cx terminating into the CS (arrow)

Fig. 2

a RAO angiographic view shows an LAD-PA fistula (arrows). b RCA-PA fistula. Filling of the pulmonary artery occurs via a common channel (arrowheads). c and d ‘Asian dragon’: The fistula’s resemblance to an Asian dragon shape can be appreciated. The morphological anatomy of the fistulous vessels from the LAD to the PA shows the appearance of an ‘Asian dragon’ with a serpiginous vessel with multiple twists and turns terminating in the pulmonary artery via a dilated common channel

Fig. 3

Left lateral angiographic view shows an LAD-PA fistula (arrow)

Fig. 4

a Normal findings on the rest ¹³N-ammonia polar map (left panel) and a large absolute perfusion defect (dark blue) in the inferior wall on adenosine ¹³N-ammonia polar map (right panel). b Three-dimensional volume-rendered computed tomography coronary angiography demonstrates CAFs from Cx to CS (arrow). c After coiling of the fistula, abnormal flow was abolished (arrow)