- Case report
- Open Access
- Open Peer Review
Doppler findings in a rare Coronary Artery Fistula
© Jung et al; licensee BioMed Central Ltd. 2007
- Received: 22 January 2007
- Accepted: 09 March 2007
- Published: 09 March 2007
One of the primary forms of congenital anomalies of the coronary arteries is coronary artery fistula (CAF). It is defined as a direct communication between the coronary artery and any surrounding cardiac chamber or vascular structure, which bypasses the myocardial capillary bed. We present a newborn baby with a large coronary artery fistula connecting the left anterior descending (LAD) artery to the left ventricular (LV) apex. Associated cardiac abnormalities were found: a ventricular septal defect (diameter 4 mm), a patent foramen ovale as well as trivial tricuspid and mitral regurgitation. Here we demonstrate the echocardiograms of an extremely rare form of CAF diagnosed within the first days of postnatal life.
- Right Coronary Artery
- Ventricular Septal Defect
- Left Coronary Artery
- Coronary Artery Fistula
- Muscular Ventricular Septal Defect
Hemodynamically significant congenital anomalies of the coronary arteries
Isolated/primary – without CHD:
Secondary – with CHD:
• Congenital coronary artery fistula
• PA + IVS
• Anomalous origin of accessory coronary arteries from the pulmonary artery
• AA + MS
• Ectopic origin of the coronary arteries from aortic sinus
• Absence of a coronary artery
CAF diagnosed in the neonate is very rare. Symptoms of the disease at older age may be continuous systolic-diastolic murmur, congestive heart failure, pulmonary hypertension, bacterial endocarditis, premature arteriosclerotic changes within the fistulae and thromboembolic events. Angina is uncommon and myocardial infarction rare. It is postulated that these ischaemic symptoms are caused by coronary steal . If the fistula drains into a right-side chamber or vessel, including the right atrium, right ventricle, or pulmonary artery, it can cause a left to right shunt with increased flow to the pulmonary circulation and ultimately to the left heart. If the fistula drains into the left atrium and ventricle, it can produce an isolated volume overload of those chambers similar to the overload that occurs in aortic reurgitation . A CAF that has not been detected or closed in childhood has been reported to become symptomatic in adulthood because of this chronic volume load and ischaemia. Therefore it has been recommended that these fistulae be closed in childhood, either surgically or by transcatheter coil occlusion. Liberthson et al. and Sunder et al. found that patients greater than 20 years of age had significantly higher rates of symptoms and complications of CAF [6, 11]. But there is still discussion about recommending the elective closure of asymptomatic patients. A good argument in favour of a conservative approach is the unexpectedly high incidence of spontaneous closure of CAF . On the one hand, the life long risk of the complications of the CAF is not known, but on the other hand any intervention to close such fistulae is associated with risk of morbidity and the benefit of such intervention is questionable.
If the decision is made for elective closure surgical ligation versus coil embolisation should be individualized. Factors that favor surgical ligation include large fistulas, multiple fistulous connections, extreme vessel tortuosity, presence of an aneurysm, need for concomitant distal bypass, or the presence of large branch vessels that can be inadvertently embolized . Factors that favor transcatheter coil embolisation (TCE) include single drain site, older age, presence of a fistula arising as an accessory coronary artery and absence of an adjacent vessel .
Surgical ligation results in a high closure rate if there are not multiple sites, but the major disadvantage is the morbidity associated with the surgery. Some disadvantages associated with catheter closure of the fistulae are transient arrhythmias, coil embolization into the great vessels or recoil into the major coronary artery leading to acute mycardial infarction and occasionally sudden death. Also, complete obliteration of the CAF can be achieved in only 80–85% of the patients .
Differential diagnosis includes persistent ductus arteriosus, pulmonary arteriovenous fistula, ruptured sinus of Valsalva aneurysm, aortopulmonary window, prolapse of the right aortic cusp with a supracristal ventricular septal defect, internal mammary artery to pulmonary artery fistula and systemic arteriovenous fistula.
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