A young female with an unusual cause of dyspnea

International Journal of Cardiology 122 (2007) e21 – e22 www.elsevier.com/locate/ijcard Letter to the Editor A young female with an unusual cause of dyspnea Elie Zoghbi a,⁎, Fadi Seif b , Mounir Obeid c , Gilbert Abou Nader a , Jaber Sawaya a a Division of Cardiology — American University of Beirut Medical Center, Beirut, Lebanon Department of Internal Medicine — American University of Beirut Medical Center, Beirut, Lebanon Department of Cardiothoracic surgery — American University of Beirut Medical Center, Beirut, Lebanon b c Received 24 August 2006; accepted 2 November 2006 Available online 25 January 2007 Abstract Within the context of coronary artery anomalies, coronary fistulas are classified by termination. A coronary artery fistula (CAF) involves a sizable communication between a coronary artery and a heart chamber (coronary–cameral fistula) or a segment of the systemic or pulmonary system (coronary artery arteriovenous fistula). These are often due to deviations from normal embryological development. CAF may be present in patients at any age, but is usually suspected in early childhood and accounts for 0.08–0.4% of congenital cardiac anomalies. They may also be acquired by trauma or from invasive cardiac procedures. The majority of CAF arises from the right coronary artery (55%) and the left anterior descending coronary artery, with the circumflex rarely involved. The outcome of these connections depends upon the termination site. The pathophysiology of these lesions is identical. Coronary artery anomalies are difficult to detect clinically. Most are benign but some may produce symptoms that can be life threatening. We present a female with an unusual cause of dyspnea found to have a continuous heart murmur, cardiac catheterization showed an evidence of a long tract arising from the origin of the right coronary artery with flow directed towards the right atrium and the right atrial appendage. © 2007 Published by Elsevier Ireland Ltd. Keywords: Dyspnea; Continuous heart murmur; Coronary artery; Fistula 1. Case presentation A 38 year old female presented to our center for investigation of progressive dyspnea. The patient has been complaining of dyspnea for many years however she reports that recently she started to have decrease in exercise tolerance associated with shortness of breath even with mild exertion. She also reports some episodes of shortness of breath at rest. She has no history of chest pain, paroxysmal nocturnal dyspnea, orthopnea or peripheral edema. She also reports mild episodic palpitations that are usually self-limited. She is a non-smoker and her family history is negative for coronary artery disease, congenital heart diseases or sudden cardiac death. She has no history of a significant chest wall trauma or any invasive cardiac intervention. She is a school teacher and ⁎ Corresponding author. American University of Beirut, Riad El-Solh, P.O. Box 110236, Beirut, Lebanon. Tel.: +961 3 864975. E-mail address: [email protected] (E. Zoghbi). 0167-5273/$ - see front matter © 2007 Published by Elsevier Ireland Ltd. doi:10.1016/j.ijcard.2006.11.068 a mother of two children. Her physical examination revealed a continuous machinery murmur grade III/VI with both a systolic and a diastolic component with the diastolic being greater than the systolic; the murmur is best heard over the aortic area and right third intercostals space with no radiation to the carotids, to the axilla or to the back. An echocardiography was done showing a normal left ventricular function with an ejection fraction of 65% and no significant valvular pathology, but a significant dilatation of the proximal right coronary artery was seen with increased color flow to it. Left heart catheterization was performed showing normal coronaries with a dominant circumflex; however an evidence of a long tract arising from the origin of the right coronary artery with flow directed towards the right atrium and the right atrial appendage was seen. Lab studies revealed hemoglobin = 12 mg/dl (normal 11–16); hematocrit = 36% (33–48); creatinine = 0.6 mg/dl (0.5–1.2); EKG revealed a normal sinus rhythm. The diagnosis of a fistula arises from the right coronary artery to the right atrium with hemodynamic evidence of left 22 E. Zoghbi et al. / International Journal of Cardiology 122 (2007) e21–e22 to right shunt. An attempt to close the tract by a percutaneous coiling of the fistula failed because of a common origin of this fistula with the ostium of the right coronary artery. The patient was discharged home for a later follow up of her dyspnea, however a few months later her dyspnea became more severe so the patient was readmitted to our center for an open heart closure of this fistula. Surgery was performed by ligating the distal portion of the fistula. 2. Discussion Most adults with coronary artery fistula are diagnosed incidentally. The most common presentation is that of a continuous heart murmur, as seen in our patient. If symptoms develop, most patients present later in life complaining of shortness of breath and evidence of right ventricular enlargement or dysfunction related to progressive enlargement of the fistula and an increase in left to right shunting [1]. CAF can also be associated with myocardial ischemia or infarction due to stealing of coronary blood flow down the fistulous tract, and rarely rupture [1]. Coronary artery fistulas vary widely in their morphological appearance and presentation [2]. These communications are often congenital, however, in certain instances, they may be acquired by trauma (stab, gunshot, projectile injuries…), or from invasive cardiac procedures (pacemaker electrode implantation endomyocardial biopsy, coronary angiography). The major sites of origin are the right coronary artery (55%), then the left coronary artery system (35%), and both coronary arteries (5%). The major termination sites are the right ventricle (40%), right atrium (26%), and the pulmonary arteries (17%). Coronary artery fistulas are rare, and it is difficult to detect coronary anomalies. Clinical manifestations vary considerably and the long term outcome is not fully known [2]. The volume of the shunt varies with the size of the fistula and differences between the systemic resistance and the resistance in the terminating vessel or chamber; flow moves from the coronary arteries to the lower pressure vessel or chamber. Spontaneous Fig. 2. closure has been reported in children. A loud continuous murmur usually located at the lower sternal border is noted. The actual diagnosis can often be made with transthoracic and color Doppler echocardiography in children (Fig. 1). In adults, transesophageal echocardiography may be more sensitive [3]. Nowadays, the use of contrast enhanced computer tomography and three dimensional image reconstruction or magnetic resonance imaging have been implicated [4]. The current management of adults who are asymptomatic is controversial. While all symptomatic fistulas need to be surgically corrected due to the significant mortality and morbidity associated, asymptomatic patients are currently being managed conservatively by some physicians [5]. Regardless, patients with large shunts tend to be symptomatic, whereas asymptomatic adults commonly have minimal shunting and this point becomes inconsequential. In addition, any patient undergoing surgery for another reason should have the fistula corrected at the same time to prevent future complications. The repair is straightforward and can be done without cardiopulmonary bypass in some cases (Fig. 2). Yet, the prevalent use of coronary angiography will continue to find asymptomatic patients with these anomalies [6]. References Fig. 1. [1] Balanescu S, Sangiorgi G, Castelvecchio S, Medda M, Inglese L. Coronary artery fistulas: clinical consequences and methods of closure. A literature review. Ital Heart J 2001;2(9):669–76. [2] Gowda Ramesh M, Vasavada Balendu C, Khan Ijaz A. Coronary artery fistulas: clinical and therapeutic considerations. Int J Cardiol 8 February 2006;107(1):7–10. [3] Vitarelli A, De Curtis G, Conde Y, et al. Assessment of congenital coronary artery fistulas by transesophageal color Doppler echocardiography. Am J Med 2002;113:127–33. [4] Ropers D, Moshage W, Daniel WG, Jessl J, Gottwik M, Achenbach S. Visualization of coronary artery anomalies and their anatomic course by contrast-enhanced electron beam tomography and three-dimensional reconstruction. Am J Cardiol 2001;15:193–7. [5] Sherwood MC, Rockenmacher S, Colan SD, Geva T. Prognostic significance of clinically silent coronary artery fistulas. Am J Cardiol 1999;83:407–11. [6] Kamiya H, Yasuda T, Nagamine H, et al. Surgical treatment of congenital coronary artery fistulas: 27 year experience and a review of the literature. J Card Surg 2002;17:173–7.