Bilateral Coronary to Pulmonary Artery Fistulae – 5 ½ Years of
Follow-up with Conservative Treatment.
Anil Kumar, Mohammed Murtaza, Shahriar Yazdanfar
Department of Medicine, Sound Shore Medical Centre of Westchester, New York Medical College,
New Rochelle, New York 10802, USA.
Cardiology Department, Albert Einstein Medical Centre, 5501 Old York Road, Philadelphia, PA 19104, USA.

INTRODUCTION

Coronary artery fistula (CAF) was first described by Krause in 1865 as an anomalous communication between a coronary artery and a cardiac chamber, great vessels or other vascular structures, bypassing the myocardial capillary bed1. Majority of CAFs are congenital but rare cases of traumatic and iatrogenic following coronary artery bypass grafting (CABG) or after endomyocardial biopsy have been reported2. Patients with CAF may remain asymptomatic throughout life and routine check-up may reveal a localized continuous murmur. Patients with large fistulae and sizeable intracardiac shunts become symptomatic and may present with dyspnea on exertion, effort intolerance, angina, palpitations and rarely, sudden cardiac death. Moreover, major complications e.g., myocardial infarction, heart failure, infective endocarditis and in rare occasions, rupture may develop. The incidence of complications increases with advancing age3. The endocarditis risk also remains high at 5% to 10% range, even in small asymptomatic CAFs without secondary changes4. On the basis of the above observations, it is commonly believed that large symptomatic CAFs must be closed surgically or by the percutaneous technique using coil embolization. Our patient with bilateral coronary artery to pulmonary artery fistulae and presenting symptoms of dyspnea and intermittent episodes of atypical chest pain was followed medically by a conservative route for 5½years. Her symptoms remained unchanged and there was no evidence of progression of disease or clinical deterioration. Follow-up echocardiograms showed

normal left ventricle (LV) and right ventricle (RV) size and function with moderate pericardial effusion, but no valvular abnormalities.
CASE SUMMARY

A 57 year old female presented to the hospital with dyspnea on exertion and atypical chest pain for 2 month duration. Past medical history was significant for hypertension, dyslipidemia, hypothyroidism and asthmatic bronchitis. General physical examination was unremarkable and electrocardiogram was within normal l imits. A diagnostic cardiac catheterization study revealed fistulous communication of both right and left coronary arteries with main pulmonary artery, as well as moderate atherosclerotic disease of the mid left anterior descending (LAD) coronary artery and its first diagonal branch (Figure 1, 2). The right heart chambers and the pulmonary artery pressures were normal and oximetry run did not show a detectable step - up at the pulmonary level (Table). She was advised conservative management and was followed up clinically by regular cardiac evaluations and periodic echocardiography. She remained in New York Heart Association (NYHA) class I to II, with no increase in her original presenting symptoms. There was no intervening event, except for a need for escalating the doses of antihypertensive medications for optimal hypertension control. On echocardiography, the right and left ventricular size and function were normal and there was no significant valvular abnormality. A small to moderate circumferential pericardial effusion was noted which remained unchanged on repeat study 6 months later. The patient also had a negative stress test for

Correspondence: Anil Kumar, Department of Medicine, Sound Shore Medical Centre of Westchester, New York Medical College, New Rochelle,New York-10802,USA.
Email: akgothwal@yahoo.com

of cases, the pulmonary trunk is the draining site12,13. In a minority of CAF (approximately 5%), more than 1 fistulous channels have been identified from a single branch or multiple coronary arteries. Bilateral CAFs are a much rarer malformation14,15,16. However, when they occur, more than half of the bilateral CAFs drain into the pulmonary artery. The
majority of patients with a small shunt are clinically asymptomatic. The continuous murmur a hallmark of large fistulae, is absent and the shunt is usually undetectable by oximetry run, the features observed in our patient. The clinical course in these patients is usually benign. In a series of 101 patients with small fistulae followed clinically, no fistula- related symptoms or complications were found for up to 11 years of follow-up6. Serial cardiac catheterization studies in small numbers of these patients documented no increase in the size of fistula or the amount of shunt over time. Despite the reported benign clinical course of the small coronary arterial fistulae, endocarditis risk remains
high in the group reported by Rittenhouse et al4. The symptoms in patients with CAFs are primarily caused by the
hemodynamic abnormalities and the hemodynamic consequences of the fistulae are directly influenced by the size of the intracardiac shunt and the site of the communicating structure. The most common presentations include congestive heart failure, myocardial ischemia and arrhythmias, in the presence of large intracardiac shunts. In patients with coronary artery to pulmonary artery fistulae, although portion of the coronary blood flow is diverted to the pulmonary circulation with lower resistance, the “Steal phenomenon” is rarely considered as the underlying
mechanism of myocardial ischemia, a mechanism well
established in patients with the congenital origin of the left
coronary artery from the pulmonary artery.
MANAGEMENT

There is no general agreement regarding optimal management of patients with CAFs17. The management strategies for the treatment of CAFs should be primarily based on symptoms and shunt size. Although conservative management appears to be best suited for patients with small shunts and mild or no symptoms, patients with significant hemodynamic abnormalities due to large shunts and moderate to severe symptoms should be considered for surgical or percutaneous closure. Generally, surgical closure or percutaneous closure with coil embolization
devices is recommended for symptomatic patients with large shunts and fistulae with significant hemodynamic consequences, especially those with heart failure. Surgery is also recommended to avoid risk of future complications. The optimal management of asymptomatic patients with a small isolated fistula, however, remains controversial. The report by Hobbs et al demonstrated a benign clinical course of the later group in a cohort of 101 patients followed up for up to 11 years. Despite these reports,

general consensus regarding treatment of adult asymptomatic patients with nonsignificant shunting has not been reached. The natural history of patients with bilateral coronary artery to
pulmonary artery fistulae is unknown and to our knowledge, there is no report of long term follow-up with conservative management in such patients. Clinical follow up was available in our patient, who had a favourable clinical course 5½ years after the diagnosis with no symptoms related to her coronary artery fistulae and with normal cardiac structure and function demonstrated on follow- up echocardiographic studies. A radionuclide stress test done at 5½
years also excluded the presence of myocardial ischemia. Advances in echocardiography and other newer emerging imaging technologies, such as Cardiac Computerised Tomography, Magnetic Resourse Imaging and Computerised
tomographic angiography may enhance noninvasive diagnosis and evaluation of the patients with coronary artery fistulae and facilitate an early detection of future complications.
ACKNOWLEDGEMENTS

We gratefully acknowledge the assistance of Basem Bichay (RCIS, RCVT) in the preparation of photographs.
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