Ann Thorac Surg 2006;82:1886-1888
© 2006 The Society of Thoracic Surgeons
Case Reports
Bilateral Coronary-to-Pulmonary Artery Fistulas
Yao-Kuang Huang, MDa,*,
Meng-Huan Lei, MDb,
Ming-Shian Lu, MDc,
Chi-Nan Tseng, MDc,
Jen-Ping Chang, MDc,
Jaw-Ji Chu, MDc
a Division of Cardiovascular Surgery, Lotung Poh-Ai Hospital, Ilan, Taiwan
b Division of Cardiology, Lotung Poh-Ai Hospital, Ilan, Taiwan
c Division of Thoracic and Cardiovascular Surgery, Chang Gung Memorial Hospital, Linkou and Kaohsiung, Taiwan
Accepted for publication February 16, 2006.
* Address correspondence to Dr Huang, Division of Cardiovascular Surgery, Lotung Poh-Ai Hospital, 83 Nan Chang Rd, Lotung, Ilan, 265 Taiwan. (Email: huang137{at}cgmh.org.tw).
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Abstract
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Coronary angiography and multi-detector computed tomography provide crucial information for anatomical correlation and surgical planning for coronary vessel malformation. This study reports a successful experience of bilateral coronary-to-pulmonary artery fistula with a vascular malformation.
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Introduction
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Coronary arteriovenous fistulas (CAVFs), as first described by Krause [1] in 1865, are considered a major coronary anomaly by Ogden's classification. This anomaly comprises half of all coronary anomalies and it is present in 0.002% of the general population. The coronary-to-pulmonary artery fistulas (CPAFs) constitute 15% to 30% of all CAVFs. Multiple fistulas occur in 10.7% to 16% of all CAVFs, whereas both coronary arteries are involved in accounting for 5% of the cases [2]. The bilateral coronary-to-pulmonary artery fistula presented with an additional communication in a vascular malformation is very unusual.
Informed consent from the patient was obtained and the institutional review board approved the presentation of this case. A 56-year-old woman was seen at a medical clinic 2 months prior to admission after having a 1-year history of progressive dyspnea and chest tightness. At admission, the patient was well, except for a continuous murmur over the upper anterior mediastinum. Transthoracic echocardiography documented a continuing shunt in front of the ascending aorta and the main pulmonary trunk. Selective coronary angiography revealed a bilateral CPAF (Fig 1). One fistula originated from the conal branch of the right coronary artery. This fistula went across the right ventricular outlet tract, drained into the main pulmonary trunk, and had branches supplying an extensive vascular malformation lying on the main pulmonary trunk. The second fistula originated from a diagonal branch of the left anterior descending artery, comprising branches from the left main coronary artery and draining laterally into the main pulmonary trunk. The left anterior descending artery and circumflex artery distal to the fistula were normal in caliber. The distal part of the right coronary artery was visualized, but was poorly opacified, which was attributed to either a coronary steal phenomenon or Venturi effect.
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Fig 1. Selective coronary angiography. (A) The left coronary-to pulmonary artery fistula originates from the proximal left descending artery and drains into the main pulmonary trunk. (B) The right coronary-to-pulmonary artery fistula travels across the right ventricular outlet tract and drains into the main pulmonary artery. (LAD = left anterior descending coronary artery; LCPAF = left coronary-to-pulmonary artery fistula; LCX = left circumflex artery; LM = left main coronary artery; outlet = entry of right coronary-to-pulmonary artery fistula; RCPAF = right coronary-to-pulmonary artery fistula; VM = vascular malformation.)
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Further study was performed using 16-slice, multi-detector computed tomography, and 0.75-mm slice collimation was preset for the scan. The image was reviewed using a three-dimensional reconstruction technique (Fig 2). The course of the CPAF and a vascular malformation over the main pulmonary trunk were compatible with conventional coronary angiography.
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Fig 2. Multi-detector computed tomographic scan with three-dimensional reconstruction. (A) The left coronary-to-pulmonary artery fistula (arrow) and (B) the right coronary-to-pulmonary artery fistula are comparable with the selective coronary angiography. (LAD = left anterior descending coronary artery; LCPAF = left coronary-to-pulmonary artery fistula; RCPAF = right coronary-to-pulmonary artery fistula; VM = vascular malformation [arrow].)
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Surgery was started through a median sternotomy. The large right coronary-to-pulmonary artery fistula was visible at the surface of the right ventricular outlet tract, and a continuous thrill was palpated over it (Fig 3). The other fistula was also identified at their origin from the proximal left anterior descending artery to the lateral side of the main pulmonary trunk. A vascular malformation (roughly 2 x 2 x 2 cm3 in size) that received drainage from the collateral branches of both fistulas was located on the anterior surface of the main pulmonary trunk.
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Fig 3. Operative photograph clearly demonstrating the bilateral coronary-to-pulmonary artery fistulas and vascular malformation. (LCPAF = left coronary-to-pulmonary artery fistula; RCPAF = right coronary-to-pulmonary artery fistula; VM = vascular malformation.)
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Both fistulas were dissected near their origins and were temporarily occluded with tourniquets until the thrill disappeared for 20 minutes. During this period, the hemodynamic condition and electrocardiography remained unchanged. Fistulas were ligated proximally, and the thrill disappeared.
However, the vascular malformation, which receives collateral branches from both fistulas, remained engorged after occlusion of both fistulas. Pulmonary artery exploration was performed under noncardioplegic cardiopulmonary bypass support. A defect (1 x 1 cm2) was identified at the base of that vascular malformation and was subsequently closed with a pericardial patch.
The postoperative echocardiography revealed no residual shunt and the patient recovered uneventfully.
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Comment
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Coronary arteriovenous fistulas are rare and may be congenital or acquired (traumatic, infectious, or iatrogenic). Fistulas originating from the right coronary artery account for 50% to 58% of cases, with 25% of cases from the left anterior descending artery, 18.3% from the circumflex artery, 1.9% from the diagonal branch, and 0.7% from the left main coronary artery or circumflex-marginal branch. The drainage site is the pulmonary artery in 29.8% to 43% of cases, the right ventricle in 14% to 40%, the right atrium in 19% to 20.2%, the left ventricle in 5.8% to 19%, and the left atrium in 5%. Multiple fistulas occur in 10.7% to 16% of all CAVFs, whereas both coronary arteries are involved in 5% of cases [2–4].
Symptoms depend on the magnitude of the shunt and the structures involved. Fistulas into the right heart chambers typically produce pulmonary congestion. If a fistula drains to the left heart chambers, it can cause left ventricular volume overloading. However, most patients are asymptomatic. Congestive heart failure and endocarditis each develop in 20% of patients. Angina pectoris and myocardial infarction, due to coronary artery steal, occurs in 3% to 7%. In extremity, coronary to pulmonary artery fistula presents as spontaneous rupture, causing hemopericardium and cardiac tamponade [5].
Aortography and selective coronary angiography can determine the shunt size, the precise features of the anomalous vessels, the run off of the coronary artery, and the coexisting cardiac abnormalities. Multi-detector computed tomography with three-dimensional reconstruction is an emerging option for the investigation of coronary artery disease. Although multi-detector computed tomography remains inferior in arrhythmia, temporal resolution, and concomitant intervention to conventional coronary angiography, it provides good noninvasive anatomical correlation with conventional coronary angiography and surgery [6].
General agreement exists that symptomatic patients should be treated. Current treatment options comprise observation, transcatheter embolization, and surgical ligation with or without pulmonary bypass. Surgical ligation has been very successful with a mortality rate of less than 1.5%. Complex fistulas require cardiopulmonary bypass and even cardioplegic arrest [3]. Although some authors have successfully occluded the bilateral CPAF without cardiopulmonary bypass [4], we still recommended routine exploration of the pulmonary artery using cardiopulmonary bypass in such patients, especially in the case of a bilateral CPAF in combination with a vascular malformation. Omitted communication in the vascular malformation can cause late recurrence after the dominant left-to-right shunt is resolved.
Transcatheter closure of the CAVF has been an option since 1983, and results have improved ever since. In a recent report, transcatheter closure established complete closure of the CAVF in 82% of cases [7]. Nevertheless, there are still some anatomic restrictions for transcatheter embolization, including multiple drainage sites and vessel tortuosity, as in the present case. Versatile therapeutic recommendations for CAVF (including surgery and transcatheter embolization) have been proposed [7, 8]. However, further study is necessary to determinate the optimal treatment modality in such cases.
In summary, this study reports a successful experience of bilateral CPAF with a vascular malformation and multiple drainage sites. Coronary angiography and multi-detector computed tomography can provide crucial information for anatomical correlation and surgical planning. We recommend routine pulmonary arterial exploration in such complex cases, even when detailed preoperative images exist.
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References
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- Krause W. Ueber den Ursprung einer akzessorischen A. coronaria aus der A. pulmonalis Z Ratl Med 1865;24:225-229.
- Dodge-Khatami A, Mavroudis C, Backer CL. Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries Ann Thorac Surg 2000;69:S270-S297.[Abstract/Free Full Text]
- Liberthson RR, Sagar K, Berkoben JP, et al. Congenital coronary arteriovenous fistulaReport of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849-854.[Abstract/Free Full Text]
- Olearchyk AS, Runk DM, Alavi M, et al. Congenital bilateral coronary-to-pulmonary artery fistulas Ann Thorac Surg 1997;64:233-235.[Abstract/Free Full Text]
- Bauer HH, Allmendinger PD, Flaherty J, et al. Congenital coronary arteriovenous fistula: spontaneous rupture and cardiac tamponade Ann Thorac Surg 1996;62:1521-1523.[Abstract/Free Full Text]
- Chan MSM, Chan IYF, Fung KH, et al. Demonstration of complex coronary-pulmonary artery fistula by MDCT and correlation with coronary angiography Am J Roentg 2005;184:S28-S32.[Free Full Text]
- Armsby LR, Keane JF, Sherwood MC, et al. Management of coronary artery fistulaePatient selection and results of transcatheter closure. J Am Coll Cardiol 2002;39:1026-1032.[Abstract/Free Full Text]
- Goldberg SL, Makkar R, Duckwiler G. New strategies in the percutaneous management of coronary artery fistulae: a case report Catheter Cardiovasc Interv 2004;61:227-232.[Medline]
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Abstract
Introduction
