Percutaneous Transcatheter Closure of Coronary-Pulmonary Artery Fistula Using Polytetrafluoroethylene-Covered Graft Stents

Coronary fistulas are mostly congenital, solitary or multiple communications between coronary vessels and atrium, ventricle, pulmonary artery, superior vena cava or coronary sinus.1,2 Their reported prevalence is 0.007–0.26%.3–6 The right coronary artery (RCA) or its branches are the site of the fistula in about 55% of cases; the left coronary artery is involved in about 35%, and both coronaries in 5%. Most fistulas drain into the right ventricle, right atrium or coronary sinus. Fistulous communications to the pulmonary artery, left atrium or left ventricle are less frequent. Occasionally, coronary fistulas can be acquired, for example after cardiac surgery,5,7 myocardial biopsy,8 pacemaker implantation,9 percutaneous coronary intervention10 or following trauma.11 Usually, symptoms occur when patients reach adulthood even when the fistula is congenital. Occasionally, heart failure or electrocardiographic (ECG) abnormalities can occur in a newborn infant. In adult patients, approximately 50% show symptoms due to heart failure, myocardial ischemia (angina pectoris) combined with arrhythmia or acute myocardial infarction. The indication to close a fistula is given when a significant left-to-right shunt occurs or when there are signs of myocardial ischemia in the associated distributions of the coronary artery concerned. The fistula is often closed in asymptomatic patients to prevent the occurrence of future symptoms or complications, such as infective endocarditis and rupture due to aneurysm dilation. Case Report. A 16-year-old foreign exchange student was referred to a pediatric cardiology clinic in the United States for evaluation of hyperventilation spells and chest pain. In the previous year, the patient had approximately 5 syncopal episodes in his native country, Switzerland. These episodes did not occur during exercise. While staying in the United States, he had 2 episodes of extreme shortness of breath, chest pain and near syncope after physical exertion. Otherwise, the patient had no known health problems. As a child, he had placement of tympanotomy tubes and a phimosis operation. He was not taking any medications. He had 2 siblings who were in good health and there was no known family history of congenital heart disease. On physical examination, there was a normal blood pressure in the upper extremities and no significant difference between left and right pressures. The oxygen saturation of the room air was 97%. Investigation of the heart revealed regular rate and rhythm, with a normal first heart sound (S1) and a physiologically split S2. No continuous systolo-diastolic murmur could be heard on examination. ECG showed a normal sinus rhythm with a non-specific interventricular conduction delay and no signs of ischemia. The transthoracic echocardiography revealed a coronary fistula originating from a coronary artery and draining into the pulmonary artery. This fistula drained laterally along the main pulmonary artery and entered into the lateral wall of the pulmonary artery 2 cm distal to the pulmonary valve. The left coronary artery was normal in dimension. Otherwise, the heart was anatomically normal and a good ventricular contractility was seen without segmental wall motion abnormalities. No ST changes occurred during the exercise stress test. During recovery, there were T inversions in the lateral leads. To verify the existence of the coronary fistula, the referring cardiologists recommended a coronary angiography with additional closing of the fistula. The patient interrupted his year as a foreign exchange student and returned to Switzerland in order to complete the tests. He was sent to us as an outpatient, in order to plan the therapeutic management. The exercise stress test with increasing load up to 300 W and recovery were normal this time, as was the ambulatory ECG monitoring and the signal-averaged ECG. In the additional transesophageal echocardiography, the fistula could be seen as described, and showed an irrelevant left-to-right shunt (less than 5%) at rest. The maximal diastolic flow velocity in the coronary fistula was 80 cm per second. There were no valve anomalies and the coronary arteries had normal dimensions in left dominance. The coronary angiography showed the coronary fistula as arising from the proximal left anterior descending (LAD) coronary artery and draining into the pulmonary artery (Figure 1). The right coronary artery (RCA) was normal. Pressure measurements during right- and left-heart catheterization were all normal. No arrhythmia could be induced during electrophysiological studies. A rhythm disturbance cause for the previous syncopes could therefore be excluded. It seems probable that this otherwise healthy, young patient, with the unusual symptoms of dyspnea and chest pain, developed a steal phenomenon during exercise. The indication to close the fistula was given on one hand due to the occurrence of a myocardial ischemia after exercise stress testing, and on the other hand to enable the student to return to the United States. After an interdisciplinary discussion, closure of the coronary fistula was performed with two graft stents. Generally, indications for the use of graft stents are coronary aneurysm, perforation, rupture and coronary fistula sealing. Aspirin, clopidogrel and heparin were given before and during intervention. Device deployment was performed retrograde (via the femoral artery). A Judkins left 4 catheter was positioned in the main left coronary artery through a 7 French sheath. The diameter of the LAD as measured by IVUS was 4.8 mm (Figure 1). In order to secure the vessel, a guidewire (0.014´´ Balance Heavyweight Guidewire; Guidant Corporation, Temecula, California) was positioned in the LAD (Figure 2). The next step involved the positioning of 1 PTFE-covered graft stent (Jomed 4.0 x 19 mm, Jomed, Europe, Sweden) at the origin of the coronary fistula. We used 2 projections before placement of the graft stent. A control angiography showed the fistula still open, as the graft stent had been placed too far distally in the LAD. A second graft stent was placed slightly proximal to the first (Figure 3). They were expanded with 18 bar. After redilatation with a JOCATH Maestro PTCA Catheter (Jomed 5.0 x 14 mm), the coronary fistula was completely closed (Figure 4) and the flow in the LAD remained normal (TIMI grade 3). The patient developed neither symptoms nor complications during intervention. The continuous ECG control showed no ST segment changes and therefore no signs of an intermittent ischemia. The intervention duration was approximately 30 minutes. The patient was discharged the following day without symptoms. Medication was as follows: aspirin 100 mg per day for 2 years and clopidogrel 75 mg per day for 1 year due to the stents used. The patient was asymptomatic through 4 months of follow-up. He returned to complete his studies in the United States; therefore, we were not able to perform a post-procedural stress test. In this case, the coronary fistula was successfully closed with 2 JoStents (PTFE-covered graft stents). There are few reports in which the closure of a coronary-pulmonary fistula using a graft stent was performed.12–15 Discussion. The majority of fistula patients are asymptomatic; only a small percentage develop symptoms. Coronary fistulas may lead to steal phenomena with reduced myocardial perfusion and ischemia, which may present clinically as angina pectoris, dyspnea or syncope, seldom as heart failure owing to volume overload, myocardial infarction or arrhythmia.3,4,16–18 Symptoms and complications, such as infective endocarditis, or rupture of an aneurysmal fistula depend on shunt size and pressure gradient. The number of patients described in the literature is small, so it is speculative to make definite statements on prognosis and therapy. A recent study with follow-up data on 31 coronary fistula patients, over a period of 2 years, documented the spontaneous closure of the fistula in 23% of the cases and reports of no complications whatsoever.19 For this reason, a conservative approach is generally favored; exceptions are symptomatic young patients, large or multiple, bilateral fistulas (especially with relevant left-to-right shunts), the occurrence of myocardial ischemia during stress tests and fistula complications (aneurysmal dilation and rupture). In these cases, closure of the fistula is indicated.4 In neonates and children, the closure of the fistula is the primary goal due to the fistula’s tendency to progress.20 The history of our patient with syncopal episodes, dyspnea and chest pain, as well as T changes after stress test in the United States, justified the closure of the fistula, although no relevant hemodynamic left-to-right shunt was documented. Otherwise, a relevant increased risk for developing infective endocarditis would have remained for the patient’s lifetime. Percutaneous transcatheter closure was introduced about 20 years ago21 and nowadays various interventional catheter techniques exist. Formerly, surgical ligation with or without separation from the fistula was standard procedure, with a mortality rate of up to 4%, myocardial infarction and reoccurrences.22–25 In a recently published study26 on various percutaneous closure techniques, ninety-seven percent of all fistula closures were successful. In most cases (88%), stainless-steel wire coils were used. The main complication in 17% of the cases was embolization of the occluding devices. The release-mechanism of the conventional stainless-steel coils occasionally caused problems. Today, so-called controlled release microcoils made of platinum are available.26–28 They possess a higher security level, with better control, more reliability of the coil release and more accurate positioning, even in aneurysmally dilated vessels with short tortuosity. In wide and more straight vessels, other common interventional closure systems (cheaper) can be used. Long-term follow-up studies on coil embolization and on other occlusion devices have not yet been published. Short- and medium-term follow-up controls show remarkably high rates of complete closures.26,28,32,41,42 Very few articles have been written on the elective placement of covered stents in native coronary arteries. A study published in May 200343 stated that PTFE-covered stent grafts were deployed with a high success rate. The restenosis rate was 24% after 6 months. However, this study showed that subacute occlusion occurred frequently, and therefore ticlopidine or clopidogrel treatment was recommended for at least 3 months. Two studies44,45 on the placement of PTFE-covered stents in veins also report restenosis rates of 17–25% after 6 months. One of these studies showed a contradictory opinion on the occurrence of periprocedural complications. The therapeutic management of coronary fistulas should still be decided on an individual basis. When the decision is made in favor of conservative therapy, regular follow-up assessments and endocarditis prophylaxis are essential.

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