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Ann Thorac Surg 1996;62:1521-1523
© 1996 The Society of Thoracic Surgeons

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Case Report

Congenital Coronary Arteriovenous Fistula: Spontaneous Rupture and Cardiac Tamponade

Divisions of Cardiology and Cardiothoracic Surgery, Hartford Hospital, University of Connecticut School of Medicine, Hartford, Connecticut

Accepted for publication June 11, 1996.

	Abstract

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Congenital coronary arteriovenous fistula is an unusual, but not rare, coronary anomaly. Management of asymptomatic fistulas is controversial because of great variability in natural history. We describe an 82-year-old female patient with spontaneous rupture of a previously undetected left main coronary artery-to-pulmonary artery coronary arteriovenous fistula, with resulting hemopericardium and cardiac tamponade. Emergent surgical exploration and repair provided successful treatment.

	Introduction

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An 82 year-old woman with a history of hypertension had sudden onset of epigastric and substernal chest pain while knitting. The pain increased with respiration, radiated to the back, and was associated with generalized weakness, shortness of breath, and diaphoresis. Systolic blood pressure was measured by ambulance personnel at 210 mm Hg but had dropped to 60 mm Hg by the time of arrival in the emergency room. With intravenous fluids and dopamine at 10 µg•kg^-1•min^-1, systolic blood pressure fluctuated between 70 and 100 mm Hg. Heart tones were distant, and there was a pulsus paradoxus of 16 mm Hg. Electrocardiography showed sinus rhythm with frequent atrial premature beats, but was otherwise normal. Chest roentgenography revealed new cardiomegaly and clear lungs.

Transthoracic echocardiography was limited by poor acoustic penetration, but showed a moderate-sized pericardial effusion with right ventricular outflow tract compression and right atrial free wall diastolic inversion, suggestive of cardiac tamponade. Over the next hour, there was a significant increase in the size of the pericardial effusion, with the appearance of fibrous strands, suggesting thrombus formation in the pericardial space. The proximal aortic root appeared normal, but there was suspicion of an intimal flap or mobile, ulcerated plaque in the abdominal aorta.

The patient was taken emergently to the operating room with diagnoses of hemopericardium and tamponade, presumptively caused by dissection of the ascending aorta. Intraoperative transesophageal echocardiography revealed a large pericardial effusion with fresh thrombus and a normal-appearing thoracic aorta, without clear evidence of dissection.

Surgical exploration confirmed a large pericardial hematoma and a normal external aorta. A large, lobulated, actively bleeding arteriovenous fistula was noted between the left main coronary artery and the pulmonary artery, with apparent spontaneous rupture (Fig 1). Transesophageal echocardiography was unable to clearly visualize flow through the fistula. The proximal end was surgically clipped, and the drainage site into the pulmonary artery was oversewn.

View larger version (50K): [in this window] [in a new window]   Fig 1. . Course of coronary arteriovenous fistula (CAVF) and site of spontaneous rupture. (LAD = left anterior descending coronary artery; LCX = left circumflex coronary artery.)

View larger version (147K): [in this window] [in a new window]   Fig 2. . Low-power photomicrograph showing cross-section of coronary arteriovenous fistula at site of spontaneous rupture (arrow), with hemorrhage into adventitial tissue (A). The fistula lumen (*) contained extensive atherosclerotic plaque (P) with lipid pools and cholesterol crystals. (Hematoxylin and eosin, van Gieson's elastic stains; x20 before 50% reduction.)

	Comment

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Management of CAVF is controversial because of the variability in natural history. Young patients (less than 20 years old) tend to be asymptomatic, although symptoms may develop later in life due to chronic left-to-right shunt (dyspnea, fatigue), ischemia from coronary steal (angina, myocardial infarction), or endocarditis. Conversely, a substantial majority of patients with CAVF live long, asymptomatic lives (91% of patients with CAVF in angiographic series are asymptomatic, with survival into the ninth decade reported) [1]. Spontaneous closure is rare, with only 9 documented cases. Elective surgical repair is universally advocated for those with attributable symptoms or large shunts (more than 1.5:1), but is more controversial for asymptomatic patients with smaller shunts. In two large surgical series comprising 344 patients [2, 3], there was only one perioperative death and few surgical complications, although older patients were more likely to have complications than those less than 20 years of age. Surgical technique is dictated by anatomy, with single, easily dissected communications ligated distally, without the use of cardiopulmonary bypass. Multiple communications or large, tortuous channels are usually closed by opening the recipient chamber while on cardiopulmonary bypass to ensure closure of all fistulous tracts. Coronary artery bypass grafting is considered if distal coronary flow may be compromised by ligation. Many authors now recommend surgical repair at the time the diagnosis is made, to avoid future complications and the increased risk of surgical repair at an older age [1–5].

Spontaneous arterial rupture is usually the result of aneurysmal dilatation of an arterial wall weakened by atherosclerosis or congenital defect. A recent angiographic review of 23 CAVFs found 26% to have evidence of aneurysmal dilatation [6]. Shear stress due to increased flow velocity and turbulence may predispose a vessel to accelerated atherosclerosis and thrombosis, resulting in occlusion of distal flow, increased intraluminal pressure, and rupture of the weakened wall. The presence of fresh thrombus, marked atherosclerosis, and mural thinning on pathologic analysis of our patient's CAVF is consistent with this series of events.

Two cases of spontaneous rupture of a CAVF have been previously reported, both of which were fatal. Arraya and associates [7] described an 8-year-old child with uneventful elective surgical repair of an anomalous coronary artery draining into the right atrium. Six months postoperatively, the child died suddenly of aneurysmal dilatation and rupture of the anomalous coronary artery into the pericardium. Habermann and colleagues [8] described a 52-year-old patient who died suddenly of cardiac tamponade. Autopsy study revealed a left circumflex-to-coronary sinus fistula, with thrombosis, necrosis, and perforation of the coronary sinus.

It is unusual for a patient this old to present with new symptoms attributable to a CAVF. In addition, this is only the third reported case of spontaneous rupture. Despite its rarity, spontaneous rupture of either a native or anomalous coronary artery should be considered in the differential diagnosis of sudden hemopericardium without evidence of aortic dissection, in a patient of any age. The importance of emergent surgical exploration is stressed, with the current case illustrating successful treatment. The acute and catastrophic presentation of spontaneous rupture supports the view that congenital CAVF should be electively repaired, preferably before age 20 years, when operative risk is lowest.

	Acknowledgments

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We gratefully acknowledge the assistance of Eleanor Rhodes, Rocky Ackroyd, Dr Richard Muller, Dr Anita Kelsey, and Dr David D. Waters in the preparation of this report.

	Footnotes

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Address reprint requests to Dr Bauer, Division of Cardiology, The Graduate Hospital, One Graduate Plaza, Suite 101, Philadelphia, PA 19146.

	References

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1. Sapin P, Frantz E, Jain A, Nichols TC, Dehmer GJ. Coronary artery fistula: an abnormality affecting all age groups. Medicine 1990;69:101–13.[Medline]
2. Lowe JE, Oldham HN, Sabiston DC. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373–80.[Medline]
3. Urrutia-S CO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983;33:300–7.
4. Rittenhouse EA, Doty DB, Ehrenhaft JL. Congenital coronary artery-cardiac chamber fistula. Ann Thorac Surg 1975;20:468–85.[Abstract]
5. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary artery fistula. Circulation 1979;59:849–54.[Abstract/Free Full Text]
6. Said SAM, El Gamal MIH. Coronary angiographic morphology of congenital coronary arteriovenous fistulas in adults. Cathet Cardiovasc Diagn 1995;35:29–35.[Medline]
7. Arraya I, Oda Y, Yamamoto K, et al. Surgical experience with congenital coronary A-V fistula. Jpn J Thorac Surg 1966;19:281–4.
8. Habermann JH, Howard ML, Johnson ES. Rupture of the coronary sinus with hemopericardium. Circulation 1963;28:1143–7.[Abstract/Free Full Text]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Philip D. Allmendinger Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Bauer, H. H. Articles by Chen, C. Search for Related Content PubMed PubMed Citation Articles by Bauer, H. H. Articles by Chen, C.