Congenital coronary artery abnormalities include various types of anomalies such as anomalous aortic origin, anomalous proximal course, anomalous distal connection, anomalous pulmonary arterial origin and anomalous coronary artery size. In this article I will review the most frequent abnormality and clinically significant anomalies: coronary artery fistulae and anomalous origin of the left coronary artery from the pulmonary trunk in adults. Recently, many patients with congenital heart disease (CHD) have reached adulthood and some of them are already in their mid-50s. I will touch on coronary artery sequelae attributed to antecedent Kawasaki disease observed in adult ischaemic heart disease, as well as the recent topic of coronary circulation in adults with cyanotic congenital heart disease (CCHD).
Coronary Artery Fistulae
Coronary artery fistulae are the most prevalent haemodynamically significant congenital anomalies of the coronary artery. Both coronary arteries arise from their assigned aortic sinus, but a fistulous branch of one or more of these arteries communicates directly with a cardiac chamber, pulmonary trunk, coronary sinus, vena cava or pulmonary vein. Adult survival is expected, but the lifespan may not be normal. The clinical manifestation depends largely on the amount of blood flowing through the fistula. Myocardial ischaemia may develop when the fistulous bypass induces coronary artery steel. In some cases of Kawasaki disease, this coronary artery anomaly is accidentally detected by a coronary angiogram.
Occasionally, a coronary artery fistula closes naturally.1 The long-term results of surgical repair are related to the volume of blood through the fistulous communication, the site of the communication and myocardial ischaemia before surgery. However, the results of late surgery are excellent in most cases. Surgery is usually safe; therefore, surgery or coil occlusion is usually recommended unless there is a single isolated fistula with a trivial shunt. Many coronary artery fistulae are very small (usually originating from the proximal left coronary artery and entering the pulmonary trunk).2
- Liberthson RR, Sagar K, Berkoben JP, et al., Congenital coronary arteriovenous fistula. Report of 13 patients. Review of the literature, and delineation of the management, Circulation, 1979;59:849–54.
- Hackett D, Hallidie-Smith KA, Spontaneous closure of coronary artery fistula, Br Heart J, 1984;52:477–9.
- Perloff JK, Survival patterns without cardiac surgery or interventional catheterisation. In: Perloff JK and Child JS (eds.), Congenital Heart Disease in Adults, 2nd ed, 1998;9–12.
- Purut CM, Sabiston DC, Origin of the left coronary artery from the pulmonary artery in older adults, J Thorac Cardiovasc Surg, 1991;102:566–70.
- Moodie DS, Fyfe D, Gill DC, et al., Anomalous origin of the left coronary artery from the pulmonary artery (Bland–White– Garland syndrome) in adult patients: long-term follow-up after surgery, Am Heart J, 1983;106:381–8.
- Kato H, Inoue O, Kawasaki T, et al., Adult coronary artery disease probably due to childhood Kawasaki disease, Lancet, 1992;340:1127–9.
- Burns JC, Shike H, Gordon JB, et al., Sequelae of Kawasaki disease in adolescents and young adults, J Am Coll Cardiol, 1996;28:253–7.
- Enbergs A, Burger R, Reinecke H, et al., Prevalence of coronary artery disease in a general population without suspicion of coronary artery disease, Eur Heart J, 2000;21:45–52.
- Kaemmerere H, Oelert E, Bahlmann J, et al., Arterial hypertension in adults after surgical treatment of aortic coarctation, Thorac Cardiovasc Surg, 1998;46:121–5.
- Cohen M, Foster V, Steeler PM, et al., Coarctation of the aorta: long-term follow-up and prediction of outcome after surgical correction, Circulation, 1989;80:840–45.
- Niwa K, The coronary circulation in adults with congenital heart disease, Intern Med, 2006;45:1199–1200.
- Fyfe A, Perloff JK, Niwa K, et al., Cyanotic congenital heart disease and coronary atherogenesis, Am J Cardiol, 2005;96: 283–90.
- Chugh R, Perloff JK, Fishbein M, et al., Extramural coronary arteries in adults with cyanotic congenital heart disease, Am J Cardiol, 2004;94:1355–7.
- Perloff JK, The coronary circulation in cyanotic congenital heart disease, Int J Cardiol, 2004;97:79–86. 16. Niwa K, Perloff JK, Bhuta S, et al., Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analysis, Circulation, 2001;103;393–400.
- Malinski T, Normal and pathological distribution of nitric oxide in the cardiovascular system, Pol J Pharmacol, 1998;50:387–91.
- Steinberg D, Pasthasarathy S, Carew TE, et al., Beyond cholesterol. Modifications of low density lipoprotein that decreases its atherogeneity, N Engl J Med, 1989;320:915–24.
- Gross SS, Lane P, Physiological reactions of nitric oxide and haemoglobin. A radical rethink, Proc Natl Acad Sci, 1999;96: 9967–99.
- Madhavan M, Wattigney WA, Srinjvasan SR, et al., Serum bilirubin distribution and its relation to cardiovascular risks in children and young adults, Atherosclerosis, 1997;131:107–13.
- Lill MC, Perloff JK, Child JS, Pathogenesis of thrombocytopoenia in cyanotic congenital heart disease, Am J Cardiol, 2006;98: 254–8.