Ann Thorac Surg 1997;63:854-856
© 1997 The Society of Thoracic Surgeons
Case Reports
Aorto–Left Atrial Fistula With Bicuspid Aortic Valve and Coronary Artery Origin Anomaly
Mehmet 
ah Topcuo
lu, MD,
Orhan Kemal Salih, MD,
Mustafa an, MD,
Cem Kayhan, MD,
Tümer Ulus, MD
Departments of Cardiovascular Surgery and Cardiology, Çukurova University, Faculty of Medicine, Adana, Turkey
Accepted for publication October 28, 1996.
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Abstract
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Published reports of aorto–left atrial fistula are very rare. We report a 20-year-old man who had an aorto–left atrial fistula with bicuspid aortic valve and coronary artery origin anomaly. Because acquired etiologic factors were not detected, we believe that the lesions were structural defects of congenital origin.
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Introduction
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Abnormal communications between the ascending aorta and the cardiac chambers are rare. Those most frequently found are due to ruptured aneurysms of the sinus of Valsalva, particularly in the right atrium or right ventricle but very rarely the left atrium [1, 2]. Congenital cardiac structural anomaly and a fistula between the aorta and the right atrium has been reported by Otero Coto and associates [3].
We present a surgically corrected case of a fistula between the aorta and the left atrium of congenital origin, together with bicuspid aortic valve plus coronary artery origin anomaly.
A 20-year-old man, who had been asymptomatic for 2 years before the visit, was admitted to our hospital complaining of retrosternal chest discomfort and progressive exertional dyspnea. His functional capacity (according to the New York Heart Association classification) was class III.
Physical examination showed a regular pulse rate of 92 beats/min and blood pressure of 100/40 mm Hg. A systolic thrill was palpated over the third intercostal space of the left sternal margin. On cardiac oscultation there were an ejection systolic murmur (grade 2/6) and an early diastolic murmur over the aortic area, whereas over the mitral area there was systolodiastolic murmur.
The chest roentgenogram showed cardiomegaly and a slightly dilated ascending aorta. The electrocardiogram showed left-axis deviation and prominent left ventricular hypertrophy. Transthoracic echocardiography and cardiac catheterization revealed ascending aortic enlargement, left ventricular hypertrophy, dilated left atrium, calcified bicuspid aortic valve with moderate aortic regurgitation, and an aorto–left atrial fistula (Fig 1
). In the coronary angiogram, the left coronary artery originated near the right coronary artery, and the left circumflex artery originated from the right coronary artery (Fig 2).
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Fig 1. . Anteroposterior aortogram showing aorto–left atrial fistula ( arrow). (ao = ascending aorta; la = dilated left atrium; lv = left ventricle.)
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Fig 2. . Selective right coronary angiogram showing that the circumflex coronary artery ( cx) originated from the right coronary artery (rc).
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The patient underwent operation for aortic valve replacement and fistula repair. During the operation, the ascending aorta's diameter was approximately 3.5 cm, the left atrium was markedly dilated, and a systolic thrill was palpated over the left atrium.
Aortotomy was performed and the aortic valve was found to have a bicuspid structure with commissural fusion and calcification. No annular ectasia, aneurysm of the sinus of Valsalva, or changes of infective endocarditis or dissecting aneurysm were seen. There was a communication between the aorta and the left atrium where the left main coronary artery should have been present (Fig 3).
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Fig 3. . (A) Photograph and (B) diagram of the calcified bicuspid aortic valve ( black arrow) and the orifice of the aorto–left atrial fistula (white arrow, ALAF).
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Because the aorta and the annulus were macroscopically normal, the aorto–left atrial fistula was closed with several interrupted stitches reinforced with Teflon pledgets and a size 25 CarboMedics (Austin, TX) aortic valve prosthesis was implanted. Before the closure of the aorta, a 1 x 0.2-cm full-thickness aortic tissue sample was excised for histopathologic examination.
The postoperative period was uneventful. Postoperative echocardiographic and Doppler examination did not reveal aorto–left atrial fistula.
Histopathologic examination of the aortic wall revealed cystic degeneration; and therefore, the patient's and his family's histories were reviewed. There were no findings suggestive of Marfan's disease.
Three months after the operation, the patient was in good condition and his functional capacity (according to the New York Heart Association classification) was class I.
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Comment
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Aorto–left atrial fistulas due to rupture of sinus of Valsalva aneurysms are mostly of congenital origin. The main cause seems to be cystic medial necrosis with degeneration of the wall of the ascending aorta, particularly when there is a lack of continuity between the tunica media of aorta and the fibrous valvular ring, as pointed out by Edwards and Burchell [2]. Acquired causes of aorto–left atrial fistula such as aortic root abscess [4, 5], trauma [6], and aortic enlargement (Manouguian procedure) [7] also have been reported.
Otero Coto and associates [3] have reported that absence of symptoms suggestive of rupture and lack of morphologic evidence of aortic dissection may suggest the communication is congenital. In our case the aorto–left atrial communication, bicuspid aortic valve, and left coronary artery origin anomaly were found together and the histopathologic examination of the aortic specimen showed cystic degeneration. Because the acquired etiologic factors were not detected, we believe that these lesions were due to structural defects of congenital origin.
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Footnotes
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Address reprint requests to Dr Topcuolu, Department of Cardiovascular Surgery, Çukurova University, Faculty of Medicine, 01330 Balcali, Adana, Turkey.
Address reprint requests to Dr Simansky, Department of Thoracic Surgery, Sheba Medical Center, Tel Hashomer, Israel 52621.
Address reprint requests to Dr Borges, Division of Pneumology, Medical Department I, Schumannstr 20-21, 10117 Berlin, Germany.
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References
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- Jean R, Fermin H, Velarde H, Laired A. Silva G. Aorto–left atrial fistula. J Cardiovasc Surg 1986;27:355–8.[Medline]
- Edwards JE, Burchell HF. The pathological anatomy of deficiencies between the aortic root and the heart. Thorax 1957;12:125–39.
- Otero Coto E, Caffarena JM, Such M, Marques JL. Aorto–right atrial communication. Report of unusual case. J Thorac Cardiovasc Surg 1980;80:941–4.
- Bedi-HS, Farnsworth AE. Homograft aortic root replacement for destructive prosthetic endocarditis. Ann Thorac Surg 1993;55:386–8.[Abstract/Free Full Text]
- Behnam R. Aortico–left atrial fistula in aortic valve endocarditis. Chest 1992;102:1271–3.[Abstract/Free Full Text]
- Maroni JP, Terdjman M, Montely JM, Ghannem M, Mesnildrey P, Hanania G. Complex cardiac lesion diagnosed more than 20 years after the causal injury. Arch Mal Coeur Vaiss 1994;87:949–52.[Medline]
- Hachida M, Koyanagi H. Hanayama N, Nonoyama M, Nakano K. Successful reconstruction of aorto–left atrial fistula following aortic valve replacement and root enlargement by the Manouguian procedure. J Card Surg 1994;9:392–8.[Medline]
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Abstract
Introduction
