Congenital Coronary Artery Fistula in Infancy and Childhood: Diagnostic and Therapeutic Aspects

Abstract

Clinical symptoms and age at manifestation of a congenital coronary artery fistula may vary considerably. They depend on the underlying anatomy and also on the size of the fistulous connection to the left or right side of the heart. Using colour Doppler echocardiography for direct visualization of the entire course of the fistulous vessel, including the site of termination, succeeds only in a small number of cases. Furthermore, regular coronary vessels branching off proximally and distally of the coronary artery fistula usually are not recognizable by this method. Only selective angiography provides this information and is unchallenged the most important and indispensable diagnostic technique, especially with regard to surgical treatment. This publication presents physical, echocardiographic, and angiographic data of 15 patients, who were admitted to the German Heart Center Munich between 1970 and 1993. By an invasive diagnostic approach the following arteriovenous fistulous connections were found: from right coronary artery to right atrium (3 patients) or to right ventricle (3 patients), from left coronary artery to right atrium or coronary sinus (3 patients), from left coronary artery to right ventricle (4 patients) and from right and left coronary artery to right ventricle (2 patients). In 5 patients a "proximal" form of coronary artery fistula ("side-to-side pattern") was found, in 8 patients a "distal" form ("end-artery type"), and in 2 patients a combination of both forms. In 14 patients surgical closure was performed (6 symptomatic infants, mean age at surgery = 95 days, and 8 asymptomatic children, mean age at surgery = 7.1 years): 13 patients survived surgery. On an average of 5 years after surgery all of these 13 patients are in excellent condition (NYHA functional class I). The experiences in surgical treatment verify the importance of an exact angiographic visualization of the anatomy of a coronary artery fistula and the regular coronary vessels branching off proximally and distally of the fistula. Closure of coronary artery fistulas at the time of diagnosis is recommended also in asymptomatic patients, since perioperative morbidity and mortality increases in older patients.

Imaging of Coronary Artery Fistulas by Multidetector Computed Tomography: Is Multidetector Computed Tomography Sensitive?

“…Even if a patient is initially asymptomatic, symptoms such as fatigue, The closure of CAFs at the time of diagnosis is recommended even in asymptomatic patients, because of perioperative morbidity and mortality increases in older patients 7 and long-term results are excellent. 8 Patients with CAFs which had a low flow from the coronary artery to other vascular structures or cardiac chambers have benign prognosis.…”

Evaluation of patients with coronary artery anomalies

“…However, ECHO can sometimes offer limited information about the termination site of the fistula, when the course is atypical. [9] Therefore, further evaluations are needed to identify the definite diagnosis with other imaging modalities. Conventional angiography is considered as gold standard in CAFs.…”

“…Conventional angiography is considered as gold standard in CAFs. [9] Coronary angiography is invasive; therefore, it carries a risk, although it provides the most detailed anatomy of the fistula giving information about the size, course, origin, presence of any stenosis and the drainage site, and it helps to rule out various anomalies and defects, including but not limited to patent ductus arteriosus, ventricular septal defects with aortic incompetence, and arteriovenous fistulas in the lungs or chest wall. [16] It is, thus, of utmost importance to have an image of the related coronary artery branches originating proximally and distally of the fistula.…”

“…[7] The majority of the cases are asymptomatic; however, with exercise, symptoms such as chest pain, dyspnea, and syncope can be manifested. [7][8][9][10] In CAFs, clinical symptoms and the age of onset are associated with the underlying anatomy and the diameter of the fistula. [8] When calibration of the fistula is small, clinical findings may not be evident.…”

Coronary Artery Fistula and Its Imaging Modalities—A Review Article

“…When a normal coronary artery is studied, the termination is described as broom-like arborisation, penetrating into the myocardium [16]. Normally the communications between coronary arteries with great vessels and cardiac chamber takes place through sinusoids; and on the course of development, these sinusoids transform into a normally calibrated capillary network.…”