Unilateral and multilateral congenital coronary-pulmonary fistulas in adults: clinical presentation, diagnostic modalities, and management with a brief review of the literature

Abstract

Background: Congenital coronary-pulmonary fistulas (CPFs) are commonly unilateral, but bilateral and multilateral fistulas may occur. In multilateral CPFs, the value of a multidetector computed tomography (MDCT) imaging technique as an adjuvant to coronary angiography (CAG) is eminent. The purpose of this study was to describe the clinical presentation, diagnostic modalities, and management of coincidentally detected congenital CPFs.

Hypothesis: Unilateral and multilateral coronary-pulmonary fistulas are increasingly detected due to the wide speard application of multidetector computed tomography which might be a supplementary or replacing to conventional coronary angiography.

Methods: We evaluated 14 adult patients with congenital coronary artery fistulas (CAFs) who were identified from several Dutch cardiology departments.

Results: Fourteen adult patients (5 female and 9 male), with a mean age of 57.5 years (range, 24-80 years) had the following abnormal findings: audible systolic cardiac murmur (n = 4), chronic atrial fibrillation (n = 2), nonsustained ventricular tachycardia (n = 1), and cardiomegaly on chest x-ray (n = 2). Echocardiography revealed normal findings with trivial valvular abnormalities (n = 9), depressed left ventricle systolic function (n = 3), and severe mitral regurgitation and atrial dilatation (n = 2). The findings in the rest of the patients were unremarkable. CAG and MDCT were used as a diagnostic imaging techniques either alone (CAG, n = 6; MDCT, n = 1) or in combination (n = 7). Single modality and multimodality diagnostic methods revealed 22 fistulas including CPFs (n = 15), coronary cameral fistulas terminating into the right (n = 2) and the left atrium (n = 1), and systemic-pulmonary fistulas (n = 4). Of all of the fistulas, 10 were unilateral, 6 were bilateral, and 6 was hexalateral. (13) N-ammonia positron emission tomography-computed tomography was performed in 3 patients revealing decreased myocardial perfusion reserve.

Conclusions: CAG remains the gold standard for detection of CPFs. An adjuvant technique using MDCT provides full anatomical details of the fistulas.

Figure 1

Patient 1: (A) Coronary angiographic frame of the left coronary artery demonstrating fistulous vessels from the LAD and LM to the PT (arrows). (B, C) Myocardial perfusion imaging and positron emission tomography‐computed tomography scanning demonstrating reversible myocardial ischemic changes in several segments. (D, E) Schematic representation of multidetector computed tomography of the heart showing a hexalateral coronary artery fistula. Arabic numerals indicate anomalous vessels arising from the ascending aorta (1, 4), posterior aspect of the transverse portion of the aortic arch (5, 6), LM (2), and LAD (3). These vessels bifurcated and anastomosed, and finally terminated into the PT (asterisk). Abbreviations: AO, aorta; GCV, great cardiac vein; LAD, left anterior descending artery; LB, lateral branch; LCx, left circumflex artery; LMCA, left main coronary artery; MA, median artery; PT, pulmonary trunk; RA, right atrium; RCA, right coronary artery; RM, right marginal branch; SVC, superior vena cava.

Figure 2

Patient 2: (A) Coronary angiographic frame of the left coronary artery showing the fistula from the circumflex coronary artery (Cx) ending in the left atrium (LA). The Cx turned into complete dilated fistulous channel (white arrow) turnicating the LA. (B) Residual fistula after coiling (black arrow) on repeat coronary angiography 7 months later.

Figure 3

Patient 6: Coronary angiographic frame of the left (A) and right (B) coronary arteries in bilateral fistulas forming a crown‐like figure (black arrows). The left anterior descending coronary artery‐pulmonary artery fistula shows multiple origin, pathway, and outflow. The right coronary artery‐pulmonary artery fistula has a single termination (white arrow).

Figure 4

Patient 14: Coronary angiographic frame of the right coronary artery (RCA) demonstrating a severely dilated and tortuous RCA with slow flow and hypoperfusion of the distal segments (arrow). The fistula has a single origin, pathway, and outflow.