This article has Open Peer Review reports available.
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.
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.
- Gregg DE, Fisher LC: Blood supply to the heart. Handbook of Physiology. 1963, Washington DC: American Physiological SocietyGoogle Scholar
- Hauser M: Congenital Anomalies of the Coronary Arteries. Heart. 2005, 91: 1240-1245.View ArticlePubMedPubMed CentralGoogle Scholar
- Levin DC, Fellows KE, Abrams HL: Hemodynamically significant primary anomalies of the coronary arteries:angiographic aspects. Circulation. 1978, 58: 25-34.View ArticlePubMedGoogle Scholar
- Neufeld HN, Schneeweiss A: Coronary artery disease in infant and children. 1983, 189-Philadelphia: Lea & FebigerGoogle Scholar
- Krause W: Über den Ursprung einer akzessorischen A. coronaria cordis aus der A. pulmonalis. Z Ratl Med. 1865, 24: 225-227.Google Scholar
- Liberthson RR, Sagar K, Berkoben : Congenital coronary arteriovenous fistula. Report of 13 patients, review of literature and delineation of management. Circulation. 1979, 59 (5): 849-54.View ArticlePubMedGoogle Scholar
- Barbosa MM, Katina T, Oliveria HG: Doppler echocardiographic features of coronary artery fistula: report of eight cases. J Am Soc Echocardiogr. 1999, 12: 149-154.View ArticlePubMedGoogle Scholar
- Malekahmadi M, Shahmohammadi A: Surgical Outcome of Coronary Artery Fistulas Repair in Children. Pediatr Cardiol. 2005, 26 (4): 328-30.View ArticlePubMedGoogle Scholar
- Wong KT, Menahem S: Coronary arterial fistulas in childhood. Cardiol Young. 2000, 10: 15-20.View ArticlePubMedGoogle Scholar
- Sherwood MC, Rockenmacher S, Colan SD: Prognostic Significance of Clinically Silent Coronary Artery Fistulas. Am J Cardiol. 1999, 83: 407-411.View ArticlePubMedGoogle Scholar
- Sunder KR, Balakrishnan KG, Tharakan JA: Coronary Artery Fistula in children and adults: a review of 25 cases with long-term observations. Int J Cardiol. 1997, 58: 47-53.View ArticlePubMedGoogle Scholar
- Rittenhouse EA, Doty DB, Ehrenhaft JL: Congenital coronary artery- chamber fistula. Review of operative management. Ann Thorac Surg. 1975, 20: 468-View ArticlePubMedGoogle Scholar
- Hong GJ, Lin CY, Loh CY: Congenital Coronary Artery Fistulas:Clinical Considerations and Surgical Treatment. ANZ J Surg. 2004, 74: 350-355.View ArticlePubMedGoogle Scholar
- Armsby LR, Keane JF, Sherwood MC: Management of Coronary Artery Fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol. 2002, 39 (6): 1026-32.View ArticlePubMedGoogle Scholar
- Mavroudis C, Backer CL, Rocchini AP: Coronary artery fistulas in infants and children: A surgical review and discussion of coil embolisation. Ann Thorac Surg. 1997, 63 (5): 1235-1242.View ArticlePubMedGoogle Scholar
- Kamineni R, Butman SM, Rockow JP, Zamora R: An unusual case of an accessory coronary Artery to pulmonary artery fistula: successful closure with transcatheter coil embolisation. J Intervent Cardiol. 2004, 17 (1): 59-63.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.