Coronary-cameral fistulas in adults (first of two parts)

Abstract

This is a case series and review of the literature adding 11 new cases. Coronary-cameral fistulas (CCFs) are infrequent anomalies which are in general co-incidentally found during diagnostic coronary angiography (CAG). To delineate the characteristics of congenital and acquired CCFs in adults, we performed a PubMed search for papers dealing with congenital or acquired CCFs in adults. Publications on coronary-vascular fistulas or paediatric subjects were not included. From the world literature, a total of 243 adult patients were identified who had congenital (65%) or acquired (35%) CCFs. In this review, which is part one of a two-part series on CCFs, we describe and discuss the congenital fistulas, give an overview on the published literature and report details of our own series of 11 patients with MMFs and solitary macro CCFs. Of the congenital group, 85% were small or large solitary macro CCFs (cut-off 1.5 mm) and 15% were coronary artery-ventricular multiple micro-fistulas (MMFs). Apical hypertrophic cardiomyopathy was reported in some of the reviewed subjects with MMFs (3/24 = 13%) but not was seen in our own series. Conservative medical management was generally the treatment of choice in congenital MMFs; prophylactic implantable cardioverter defibrillators (ICD) were implanted in 2/24 (8%) of subjects, especially when extensive micro-fistulisations were involved. None of the patients of our own series required an ICD, as the MMFs were of limited size. Congenital or acquired CCFs in adults are infrequent anomalies having a wide spectrum of clinical presentation may varies from asymptomatic to severely devastating states requiring different treatment modalities.

Keywords: Congenital coronary artery-ventricular multiple micro-fistulas; Congenital coronary-cameral fistulas; Congenital heart defect; Coronary angiography.

Figure 1

From the distal segment. A: The left anterior descending coronary artery/diagonal branch multiple micro-fistulas (red circle) to the left ventricle (LV) lumen are visible; B: The right coronary artery multiple fistulas (arrow) to the LV cavity. Dual endocardial pacing leads are appreciated.

Figure 2

Dilated fistulous vessel (arrow head) originating from the proximal segment of the right coronary artery (solid arrow) and terminating into the right atrium. The mitral valve ring is visible (hollow arrow). RA : Right atrium.