History
Most children with small coronary artery fistulae (CAF) are asymptomatic, and continuous murmur may be audible on routine examinations if the fistulae are moderate to large in size. In infants, angina may be recognized by symptoms such as irritability, diaphoresis, pallor, tachypnea, and tachycardia. Most infants present at age 2-3 months after the pulmonary vascular resistance has decreased with heart failure symptoms, such as tiredness during feeding, tachypnea and excessive diaphoresis during feeds, wheezing, episodic pallor, and failure to thrive. Thus, in infancy, they can present with signs of low-output congestive heart failure.
Older patients may present with signs of low-output congestive heart failure, arrhythmias, syncope, chest pain, and, rarely, endocarditis. Patients with large fistulae may present with high-output congestive heart failure, although rarely. In older patients, symptoms may include the following:
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Dyspnea on exertion
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Angina
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Fatigue
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Palpitations
Physical Examination
Most patients are asymptomatic, especially when the fistulas are small. Note the following:
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A coronary artery fistula is suspected following detection of a continuous murmur upon routine examination. Upon clinical examination, the murmur is suggestive of patent ductus arteriosus but is heard lower on the sternal border than usual; thus, the location is often atypical for a patent ductus arteriosus. In addition, the murmur may have an unusual diastolic accentuation, and the continuous murmur of a coronary artery fistula often peaks in mid-to-late diastole, which is uncharacteristic of the systolic accentuation in a patient with patent ductus arteriosus.
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If the fistula connects to the left ventricle, only an early diastolic murmur may be heard, as little coronary flow is evident during the period of systole.
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Some patients with fistulae with a large shunt may present with signs of congestive heart failure and angina.
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Wide pulse pressure and collapsing pulse may be noted.
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The apex beat is diffuse with a palpable or audible third heart sound (S3) gallop in a large fistula. Heart sounds are often reduced in intensity.
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A holosystolic murmur of mitral valve insufficiency is audible at the apex.
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Selective left coronary artery (LCA) injection demonstrating a markedly enlarged left main (*) with normal size circumflex (CX) and left anterior descending (LAD) branches. The fistula continues across the right ventricle free wall to the atrioventricular groove where it terminates at the crux of the heart in the right atrium (straight arrow). (Reproduced from Congenital Heart Disease, Textbook of Angiocardiography.)
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Retrograde aortic root injection, dilated left main (LCA) and circumflex (CX) vessels with the fistulous connection to the right ventricle (arrow). (Reproduced from Congenital Heart Disease, Textbook of Angiocardiography.)
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Three-dimensional multidetector row computed tomographic image showing a circumflex artery fistula. The left main stem is greatly dilated (arrow) and a dilated, tortuous circumflex artery becomes aneurysmal (An) before draining into the coronary sinus. Note also the left anterior descending (LAD) branches arising from this dilated vessel (arrowhead). (Image courtesy of Manghat NE, Morgan-Hughes GJ, Marshall AJ, Roobottom CA. Multidetector row computed tomography: imaging congenital coronary artery anomalies in adults. Heart. Dec 2005;91(12):1515-22.)
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Selective left coronary angiogram immediately after transcatheter coil occlusion of the circumflex coronary fistula (4 7-mm X 70-mm target coils). A tiny residual leak and the proximal circumflex coronary dilatation are shown. Image courtesy of Texas Heart Institute. (Reproduced from McMahon CJ, Nihill MR, Kovalchin JP, et al. Coronary artery fistula. Management and intermediate-term outcome after transcatheter coil occlusion. Tex Heart Inst J. 2001;28(1):21-5.)
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Coronary artery fistula from right coronary artery to right ventricle. B. No antegrade flow in the fistula after coil placement.
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Large coronary artery fistula (A) before Amplatzer vascular plug and (B) after Amplatzer vascular plug placement.
