Ann Thorac Surg 2001;72:911-913
© 2001 The Society of Thoracic Surgeons
Case report
Congenital aortocaval fistula to the superior vena cava
Abraham Oomman, MDa,
Robert Mao, MDa,
Prasad Krishnan, MCha,
Maligail Ramakrishna Girinath, FRACSa
a Apollo Hospital, Chennai, India
Accepted for publication September 22, 2000.
Address reprint requests to Dr Oomman, Apollo Hospital, Graems Rd, Chennai, India 600006
e-mail: dr-oomman{at}lycos.com
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Abstract
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Aortocaval fistula is a rare cause of left-to-right shunt. Common causes are congenital, traumatic, and dissecting aneurysm. We report a case of congenital aortocaval fistula to the superior vena cava, the proximal end of which was in close relation to the ostium of the right coronary artery.
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Introduction
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Communications between aortic root or coronary arteries and right heart structures are well known. Common causes are ruptured sinus of Valsalva and coronary arteriovenous fistula. Common chambers to which drainage occurs are the right atrium, right ventricle, and pulmonary artery. The superior vena cava (SVC) is an uncommon site of drainage. We report a case of congenital aortocaval fistula draining into the SVC. The proximal end of the fistula was in close relation to the right coronary artery ostium. The fistula was closed successfully.
A 14-year-old boy was admitted for cardiac evaluation. He was the product of a nonconsanguineous marriage, and the delivery was normal. There was no significant illness in the neonatal period or infancy. There were no recurrent lower respiratory tract infections and no history of trauma. He was evaluated 2 years earlier for cough with expectoration and on examination was found to have a cardiac abnormality and was advised to have cardiac evaluation. He continued to participate actively in all sports.
Clinical examination showed a normally built and nourished boy. He did not have any features of Marfan syndrome. There was no pallor, clubbing, cyanosis, or edema. Pulse was 90 beats per minute, regular and high volume. All peripheral pulses were palpable. Blood pressure was 120/70 mm Hg. There was no cardiomegaly. S1 and S2 were normal. A soft S3 was audible. A continuous murmur was audible in the second and third right intercostal spaces. Clinical diagnosis was coronary arteriovenous fistula or ruptured sinus of Valsalva to right atrium. Results of electrocardiogram and chest x-ray were noncontributory. Echocardiogram with color flow mapping showed continuous color and Doppler flow from aorta to SVC with a systolic gradient of 91 mm Hg and diastolic gradient of 19 mm Hg. There was trivial aortic regurgitation.
Oxymetry showed increased oxygen saturation of 83.4% at low SVC. There was no systemic desaturation. Right and left ventricular filling pressures and pulmonary artery pressures were normal. QP/QS was 1.7:1. Left ventricular angiogram in the left anterior oblique artery showed a normal-sized left ventricle with normal contractility. There was a fistulous communication between right aortic sinus and SVC, which was greatly dilated. This was confirmed by aortic root angiogram (Fig 1) of the left anterior oblique artery at 70 degrees. The communication was above the level of right coronary artery, which could be hooked separately and separate origin of the fistula and the right coronary artery could be visualized (Fig 2). Both coronary arteries were normal. There was no dilated or ruptured sinus of Valsalva. Hand injection to SVC showed aneurysmally dilated SVC opacifying the right atrium.
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Fig 1. Aortic root angiogram showing fistula between right aortic sinus and superior vena cava, which is greatly dilated.
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Fig 2. Aortic root angiogram showing separate origins of the aortocaval fistula and the right coronary artery (arrow).
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Preoperatively, there was a massively dilated SVC measuring about 4 to 5 cm in diameter with a continuous thrill palpable over it. The SVC end of the aortocaval fistula was visible as a 5- to 7-mm diameter opening in the posterior wall of the SVC just above the SVC-to-right atrial junction, and it was directly closed. The ascending aorta was opened by transverse aortotomy. The origin of the fistula from the aorta was 1 cm in diameter just above the right coronary sinus. The right coronary artery was in close relation to the fistula a few millimeters from the aortic wall. Closure of the aortic end of the fistula would have compromised the orifice of the right coronary artery. To preserve the native right coronary artery, the aortic end of the fistula was not closed. The patient had an uneventful postoperative course, and predischarge echocardiogram showed no flow into the SVC.
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Comment
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Communication between the aorta and right side of the heart includes ruptured sinus of Valsalva, coronary arterial fistula, congenital tunnel to right ventricle or right atrium, aortocaval fistula, rupture of a dissecting aneurysm of the ascending aorta [1], and pseudoaneurysm of the right coronary artery followed by formation of a fistula between the aneurysm and right atrium. Aneurysmal aorto–right atrial communication has been previously reported [2, 3]. Patients with aorto-right atrial communication were asymptomatic. A distinctive appearance was noted on the angiograms, with a large tortuous tunnel seen superior to the left sinus of Valsalva, passing posterior to the aortic root before terminating near the right atrial-SVC junction. Anatomic distinction must be made between sinus of Valsalva aneurysm, which occurs below the level of coronary arteries, and aortico-left ventricular tunnel, which occurs above their origin.
A congenital coronary artery fistula is an abnormal, direct communication between any coronary artery and any of the cardiac chambers, as well as SVC, coronary sinus, pulmonary artery, and pulmonary veins [4]. Although coronary artery fistulas commonly involve right heart structures, drainage into the SVC is rare. Congenital fistula with large aneurysmal sacculations draining into the SVC from both coronaries has been reported [5, 6]. Coronary arteriovenous fistula has a definite abnormal feature on selective coronary angiography. Surgical or transcatheter [7] closure are the therapeutic options.
Aortocameral fistulas are uncommon entities and can drain into the SVC or inferior vena cava. The causes of aorto-SVC fistula include dissecting aneurysm in Marfan syndrome [1] and trauma [8]. Wong and associates [9] reported congenital myxedema and an aberrant right subclavian artery to SVC. Soler and colleagues [10] described an unusual case of congenital systemic arteriovenous fistula arising from the descending aorta and draining separately into the SVC, azygos vein, and innominate vein. These cases of aortocaval fistulas of probable congenital origin are fundamentally different from our case. Clinically these patients resemble any left-to-right shunt conditions, with a continuous murmur on the right sternal border; surgical treatment is curative.
Our case had distinctive features. The origin of the fistula was just above the right coronary sinus. The origin of the right coronary artery was closely related to the dilated proximal part of the fistula. However, the right coronary artery could be hooked separately during cardiac catheterization and was normal. MEDLINE search did not find any similar reports.
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References
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Rosenberg H., Williams W.G., Trusler G.A., Smallhorn J., Rowe R.D., Moes C.A., et al. Congenital aortico-right atrial communications. The dilemma of differentiation from coronary-cameral fistula. J Thorac Cardiovasc Surg 1986;91:841-847.[Abstract]
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Wong C.K., Lau C.P., Leung W.H. An arteriovenous fistula from an aberrent right subclavian artery to the superior caval vein in a congenitally myxedematous adult. Int J Cardiol 1989;25:126-129.[Medline]
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Abstract
Introduction
