https://imcjms.com/public Ibrahim Medical College Journal of Medical Science https://imcjms.com/public/registration/journal_full_text/6 Sat, 23 Jul 2016 09:05:08 +0000 Abstract Dr. MZ Chowdhury, Department of Cardiology, Ibrahim Medical College & BIRDEM, 122 Kazi Nazrul Islam Avenue, Shahbag, Dhaka-1000   A congenital coronary arteriovenous fistula is an anomaly that consists of a communication between a coronary artery and either atrium or ventricle (coronary cameral fistula) or coronary sinus, superior venaecava or pulmonary artery (coronary arteriovenous fistula). Sometimes both of these groups are included under one or the other name¹. It is observed in 0.1% to 0.2% of coronary angiography studies². Case report     Congenital abnormalities of coronary artery occur in 1 - 2% of general population. These abnormalities may be of origin, distribution and termination. Coronary artery fistulae (CAF) are considered to be termination abnormalities3. CAF accounts for 0.2- 0.4% of congenital cardiac anomalies. Approximately 5% of coronary anomalies are CAF4. Common sites of fistulae are right coronary artery or its branches (55%), left coronary artery or its branches (35%) or both (5%)5. Embryologicaly, persistence of some of the inter-trabecular spaces in the primitive myocardium lead to coronary cameral fistula. On the other hand communication between the primitive coronary artery and mediastinal plexus of vessels gives rise to fistulae from coronary artery or pulmonary artery or bronchial artery or mediastinal artery. The fistulous coronary artery may be small or large and tortuous, and it enters the effected chamber by one or several openings. Small fistula does not cause any haemodynamic effect but large fistula causes left to right shunt large enough to cause volume overload though significant pulmonary hypertension is rare. Significant run off through the fistula may decrease flow through more distal coronary artery and cause a coronary steal1. Male and female is equally effected. Small fistula is asymptomatic. In large fistulae, symptoms are rare before 20 years of age. Symptoms include fatigue, exertional dyspnoea and chest pain. The latter is usually seen in 80% of the patients over 50 years. On examination there is thrill and continuous murmur on the mid left or right sternal border louder in mid diastole. It may be absent if LCx is involved. There may be features of complications like congestive cardiac failure, pulmonary hypertension, myocardial ischemia, bacterial endocarditis and saccular aneurysmal dilation rarely leading to spontaneous rupture. For diagnosis electrocardiography, chest radiography, transthoracic echocardiography (2-D & Doppler colour flow), transoesophageal echocardiography, coronary angiogram or laid back aortogram or CT scan can be done5. Except for the very small, most coronary arteriovenous fistulae should be closed by ligation, microparticle embolization or wire coils6. Prognosis is excellent if closure is done early before development of any complication.   1.  Hoffman JIE. Congenital Anomalies of the Coronary vessels and the aortic root. In: Moss and Adam. Eds. Heart Disease in Infants, Children and Adolescents. 4th edition. Williams and Wilkins, Baltimore, 1989: 769-790. 3.  Darwazah AZ, Hussein IH, Hawari MH. Congenital Circumflex Coronary arteriovenous Fistula. Texas Heart Institute Journal 2005; 32: 56-59. 5.  Friedman WF, Silverman N. Congenital Heart Disease in infancy and childhood. In: Braundwald E, Zipes DP, Libby P. Eds. Heart disease 6th edition WB Saunders, Philadelphia, 2001: 1505-1582. 2026 Ibrahim Medical College. All rights reserved.