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  • Author
    Gabriella Odudu
  • Co-author

    Hezzy Shmueli, Evan Zahn, Nir Flint, Robert J. Siegel, Rose Thompkins

  • Title

    Coronary Artery Fistula: An Unexpected Cause of Heart Failure in A 58-Year-Old Woman

  • Abstract

    PURPOSE: To present a rare case of heart failure in a 58-year-old female due to undiagnosed, congenital coronary artery fistula (CAF).

    SUMMARY: CAF is an abnormal connection between one or more coronary arteries and a chamber of the heart or major thoracic vessel, typically resulting in a left-to-right shunt. Although CAF can be diagnosed in childhood related to evaluation for an asymptomatic, continuous murmur, symptomatic CAF are more commonly encountered among adults. A majority of CAF are of no clinical consequence as shunt volume is often hemodynamically insignificant and thus, clinically asymptomatic. However, the shunt volume may be of sufficient size to result in progressive right heart volume overload leading to arrhythmia, symptoms of heart failure, including dyspnea and exercise intolerance, and pulmonary hypertension from pulmonary over-circulation. Specific management strategies, which can include surgical repair or catheter-based embolization, have been controversial as there are no guideline-directed indications for CAF closure. Moreover, data on the long-term outcomes following closure of a large CAF in adults is limited. Therefore, we want to highlight the obstacles we faced in this case in order to increase awareness about this unique presentation to early-diagnose & appropriately manage this rare condition. We report a 58-year-old woman with history of rheumatoid arthritis (RA), well-controlled on methotrexate, Von Willebrand disease (VWd) & a ten-year history of paroxysmal atrial fibrillation (pAF), managed with beta blocker therapy, who presented with palpitations & shortness of breath & reported experiencing increasing episodes of pAF, as well as, progressive dyspnea on exertion, reduced exercise tolerance and mild lower extremity edema in the past year. Her examination was notable for 3/6 continuous murmur along the left sternal border and trace bilateral pitting edema to the ankles. Transthoracic echocardiography (TTE) demonstrated normal biventricular systolic function with four chamber dilatation, no significant valvular disease, and massively dilated coronary sinus (CS) and left main coronary artery (LMCA). Continuous color flow from the CS into the right atrium suggested a CAF from the left coronary system to the CS. Coronary computed tomography angiogram (CCTA) confirmed a large CAF from the left circumflex coronary artery (LCX) draining into the CS. Right heart catheterization demonstrated a hemodynamically significant left-to-right intracardiac shunt (Qp:Qs 2.5:1.0), elevated pulmonary artery pressure 50/21 mmHg mean 32 mmHg and a capillary wedge pressure of 16 mmHg. The patient was referred for CAF closure.

    CONCLUSION: CAF is a rare cause of heart failure in an adult, & closure is indicated in cases of hemodynamically significant left-to-right arteriovenous shunting. Data is limited regarding long-term outcome & optimal medical management post-closure, but post-closure myocardial infarction remains a concern. Valente et al. described 76 cases of CAF, of which 40 underwent transcatheter closure & 20 underwent surgical intervention. Long-term complication included MI in 9% of patients & was associated with drainage of the CAF into the CS & older age at time of diagnosis. Thus, systemic anticoagulation post closure may be necessary, especially if the originating coronary artery is significantly dilated. As demonstrated in our case, the patient was older at time of diagnosis with a presumed higher risk of MI post-CAF closure, given a massively dilated LCX and drainage into the CS. Anticoagulation was advised, but decision was complicated by patient’s VWd & history of bleeding. In summation, undiagnosed congenital heart disease, including CAF, should be considered in the differential diagnosis of an adult presenting with heart failure and murmur, to allow for the proper diagnostic work up & timely subsequent treatment to decrease the risk of morbidity & mortality.

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