Multiple coronary artery-left ventricle microfistulae.

Abstract

A 55-year-old woman with a 26-year history of systemic lupus erythematosus and scleroderma was referred to our hospital, due to shortness of breath. Six years previously, pulmonary hypertension caused by collagen disease was found. Her estimated pulmonary artery systolic pressure was 50 mmHg. She had been treated with an endothelin receptor antagonist (ERA). On examination, her blood pressure was 130/85 mmHg and pulse was regular at 88/min. Diastolic murmur (Levine II/VI) was heard at the right 2nd intercostal space. An electrocardiogram showed normal sinus rhythm. A chest computed tomography (CT) scan showed interstitial lung fibrosis. The diffusing capacity of the lungs for carbon monoxide was 48.6 %. Echocardiography showed normal left ventricle (LV) size and function. The LV wall thickness was normal, and noncompacted LV myocardium was not seen. Doppler echocardiography zooming in on the LV identified multiple diastolic flow signals perpendicular to the epicardium that drain into the LV (Fig. 1). Pulsed-wave Doppler echocardiography showed a diastolic flow with a peak velocity of 1.9 cm/s. Because no other findings including aortic regurgitation inducing the diastolic murmur were detected, the murmur was probably caused by the coronary artery fistulae. Left coronary angiography revealed a bizarre appearance of capillary blush draining into the LV (Fig. 2). Opacification of the LV through a network of microfistulae was seen. The microfistulae were predominantly arising from the diagonal branch. Similar communications arising from the circumflex and right coronary arteries were seen. Thallium 201 adenosine perfusion myocardial scintigraphy showed no perfusion defect. Ventilation/perfusion scintigraphy did not suggest pulmonary embolism. Her symptom was probably caused by pulmonary dysfunction. She required continuous follow-up and regular medications with ERA including prednisolone and azathioprine. Coronary artery fistulae have an incidence of 0.2 % in patients undergoing diagnostic cardiac catheterization [1– 4]. In more than 90 % of these patients, a single fistula drains into the right heart chambers or pulmonary artery. Multiple coronary artery–left ventricular fistulae arising from all 3 major coronary arteries and draining into the LV are extremely rare. These multiple communications, inducing left-to-left shunt, are presumed to result from the persistence of embryonic myocardial thebesian sinusoids [3]. In patients with multiple coronary artery–left ventricle microfistulae, auscultatory patterns are variable. Systolic, diastolic, or no murmur was reported in the literature. The coronary flow pattern on pulsed-wave Doppler echocardiograms was not clearly mentioned [2, 3, 5, 6]. Clinical presentation depends on the hemodynamic significance, though most of the patients are asymptomatic and are found incidentally [2–4]. However, some may present with congestive heart failure or myocardial ischemia caused by Y. Matsumura (&) Y. Ochi Y. Baba K. Tanioka K. Hayashi T. Kubo N. Yamasaki T. Furuno H. Kitaoka Y. Doi Department of Medicine and Geriatrics, Kochi Medical School, Kochi University, Kohasu, Oko-cho, Nankoku, Kochi 783-8505, Japan e-mail: matsumur@kochi-u.ac.jp