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Therefore, patients often are referred for surgical correction. Intraoperatively, because of the tortuosity and aneurysmal dilation, it can be difficult to identify the origin and termination of the CAFs.
In this report, we describe a patient with a long and tortuous left anterior descending (LAD) artery-to-PA fistula who underwent intraoperative epicardial sonography and transesophageal echocardiography (TEE) to image the fistula followed by 2-point ligation without the use of cardiopulmonary bypass (CPB).
A 65-year-old man with a history of hypertension underwent an exercise stress test that was positive for ischemia in the LAD artery distribution. A subsequent cardiac catheterization revealed an extensive vascular anomaly arising from the mid-LAD artery. His coronary vessels otherwise were free of disease. A computed tomography angiogram defined the origin from the LAD artery and the termination into the PA. Reconstructed views of the computed tomography angiogram confirmed it to be a very long, tortuous fistula with flow into the PA (Fig 1). Because of the anatomic anomaly combined with the evidence of ischemia, he was referred for surgery.
Fig 1Computed tomography angiogram reconstruction showing a tortuous LAD artery-to-PA fistula. (Color version of figure is available online.)
Intraoperatively, numerous loops of vascular tissue were found overlying the proximal LAD artery distribution and on the anterior surface of the PA. Bundles of this tissue also were identified laterally to the left side of the heart in the space between the PA and the left atrial appendage and superiorly to the bifurcation of the PA, complicating the identification of the ends of the fistula. Intraoperative TEE showed flow from the LAD artery into the vascular plexus. A mechanical stabilizer (Maquet Cardiovascular, Wayne, NJ) was placed on the LAD artery to help displace and retract the LAD artery to assist in identifying the neck of the fistula. The origin of the fistula then was ligated securely with a silk suture. There was no evidence of myocardial ischemia by either electrocardiography or TEE.
With the aid of intraoperative epicardial scanning, the distal insertion of the fistula onto the PA was identified (Fig 2A and B). The end of the tract was controlled carefully. After the surrounding fistulous tissue had been dissected free, the pulmonary arterial end was ligated, divided, and oversewn. TEE confirmed the closure of the insertion into the PA (Fig 2C).
Fig 2(A) Intraoperative epicardial imaging of the PA. The distal insertion of the fistula (white arrow) is seen entering the anterior aspect of the PA. (B) Color-flow Doppler showing significant flow from the fistula into the PA. (C) Intraoperative epicardial imaging of the PA. Flow from the fistula to the PA is absent after ligation. (Color version of figure is available online.)
The patient was never placed on CPB and required no coronary grafting. Postoperatively, the patient had an uneventful recovery. A postoperative exercise stress test was negative, and he remains asymptomatic.
CAFs are very rare and occur in an estimated 0.002% of the population.
They may originate from either coronary system with approximately 50% originating from the right coronary artery, 42% from the left coronary artery, 5% from both coronary arteries, and 3% unspecified.
Symptoms generally are related either to ischemia or congestive heart failure because of left-to-right shunting. Ischemia can occur because of a mechanical impediment to distal coronary flow (such as thrombus or spasm) or from coronary “steal” because of the diversion of blood via the fistula into the low-pressure PA system and away from the distal coronary bed.
The management of CAFs is somewhat controversial, especially for younger, asymptomatic patients. Although observation with endocarditis prophylaxis may be warranted in select cases, it generally is agreed that symptomatic lesions should be repaired once they are discovered.
CAFs can be treated in a variety of ways, including transcatheter coil embolization and operative ligation (epicardial or endocardial) with or without the use of CPB.
revealed a higher mortality rate for transcatheter techniques (2.2%) than for surgical treatment (1.4%). Coronary dissection, myocardial infarction and incomplete closure of the fistulae also have been described with transcatheter techniques.
During the perioperative period, care must be taken to avoid excessive decreases in pulmonary vascular resistance to prevent worsening of left-to-right shunting. Ligation of a CAF can be performed via an epicardial approach and/or from within the cardiac chamber into which the fistula terminates. CPB with cardioplegia allows for controlled isolation of the fistula origin (often engulfed by friable fistulous vasculature) as well as the option for endocardial repair of the distal fistula. When the proximal and distal extents of the lesions are anatomically accessible, off-pump techniques, as applied in our case, are appropriate. The use of epicardial scanning in our patient allowed for the safe identification of the ends of the fistula and for the documentation of effective ligation. Given the general availability of echocardiographic equipment in cardiac operating rooms, we would strongly recommend the use of this technique for CAFs. This has been described by other authors as well.
With great interest, we read the report of Takahashi et al1 titled “Intraoperative Imaging and Off-Pump Ligation of Coronary Artery Fistula.” The management of coronary artery fistula (CAF) is indeed somewhat controversial, especially in younger, asymptomatic patients. It generally is agreed that symptomatic lesions and larger fistulae with significant shunting should be treated. Surgical ligation2 generally is accepted as the treatment of choice in CAF.
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