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A 64 year old woman presented with a six month history of progressive exertional dyspnoea and paroxysmal nocturnal dyspnoea. Echocardiography showed severe pulmonary hypertension, concentric left ventricular hypertrophy, normal systolic function, and no evidence of shunting by saline contrast. The 12 lead ECG exhibited atrial fibrillation. Chest radiography and computed tomography (CT) revealed dilated proximal pulmonary arteries without signs of parenchymal lung disease or pulmonary venous abnormalities.
Cardiac catheterisation showed fixed pulmonary hypertension without shunting. Selective coronary angiography demonstrated tortuous, dilated, epicardial coronary arteries (below left) with all three coronary arteries draining into the left ventricle via a diffuse plexus of coronary fistulae. Left coronary angiography (late frame) revealed diastolic opacification of the left ventricle (below right). The coronary sinus was not cannulated during right heart catheterisation, and there was no obvious opacification of it in the delayed phase of coronary angiogram.
Coronary artery fistulae between a coronary artery and a cardiac chamber can occur in 0.2% of diagnostic cardiac catheterisations and usually arise from the right coronary artery to drain into a right heart structure (right atrium, right ventricle or pulmonary artery). Fistulae draining into the left ventricle are uncommon, with less than 20 reported cases of fistulae involving all three coronary arteries. These congenital fistulae are abnormally prominent Thebesian vessels that represent persistence of the intertrabecular vascular network. They result in a diastolic volume overload of the left ventricle (left-to-left shunt), dilated epicardial arteries, and a “coronary steal” with shunting of blood away from the myocardium into the low resistance fistulae.