Asian Cardiovasc Thorac Ann 2000;8:56-58
© 2000 Asia Publishing EXchange Pte Ltd
Aorto-Left Ventricular Communication Through Extracardiac Tunnel
Gökhan Ipek, MD,
Kaan Kirali, MD,
Altu
Tuncer, MD,
Necmettin Yakut, MD,
Suat Ömerolu, MD,
Ali Gürbüz, MD,
Cevat Yakut, MD
Department of Cardiovascular Surgery
Kos uyolu Heart and Research Hospital
Istanbul, Turkey
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For reprint information contact: Kaan Kirali, MD Tel: 90 216 325 5457 Fax: 90 216 339 0441 email: kosuyolu{at}superonline.com, Department of Cardiovascular Surgery, Kosuyolu Heart and Research Hospital, Kadiköy, Istanbul 81020, Turkey.
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Abstract
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An asymptomatic 24-year-old man underwent surgical correction of an aorto-left ventricular tunnel. A 1-cm opening was found 1 cm above the commissure of the left and right coronary cusps, which communicated with the left ventricle. It was successfully closed by direct suture.
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Introduction
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Aorto-left ventricular tunnel (ALVT) was described in 1963 by Levy and colleagues1 as an abnormal paravalvular communication between the aorta and left ventricular cavity, bypassing the aortic valve. Since then, approxi-mately 70 cases have been reported.2 The true incidence of ALVT is unknown but Okoroma and colleagues3 found 2 cases among 1754 congenital heart disease patients. High mortality in patients without operative correction highlights the need for early diagnosis and surgical treatment.
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Case Report
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An asymptomatic 24-year-old man was referred for evaluation of a murmur that was first noted one year previously during examination for an upper respiratory tract infection. An easily discernible thrill associated with a 4/6 diastolic murmur was detected. Chest radiography showed a cardiothoracic ratio of 0.7. An electrocardiogram indicated left ventricular enlargement with diastolic overload. Echocardiography and cardiac catheterization (Figures 1 and 2
) identified an ALVT arising immediately above the right sinus of Valsalva. The left ventricular end-diastolic diameter was 53 mm (normal, 38 to 56 mm) and the end-systolic diameter was 38 mm (normal, 20 to 41 mm). The blood pressure in the aorta was 135/60 mm Hg.
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Figure 1. Two-dimensional echocardiogram showing the aorto-left ventricular tunnel. LCC = left coronary cusp, NCC = noncoronary cusp, RCC = right coronary cusp.
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At operation, the ascending aorta appeared normal, except for an aneurysmal bulge of approximately 2 x 3 cm arising from the right side of the aortic root at the right ventricular outflow tract. After crossclamping the aorta, the right ventricle was opened but no defect was detected. On incising the aorta, a 1-cm opening was found 1 cm above the commissure of the left and right coronary cusps (Figure 3). A probe revealed that the tunnel led into the left ventricle. The aortic valve was normal and the coronary ostia were in the normal area. The tunnel orifice was closed with 3 interrupted mattress sutures of 4/0 polypropylene and reinforced with an over-and-over continuous 4/0 polypropylene stitch as a second layer. Cardiopulmonary bypass was gradually discontinued and the thrill was no longer detected over the aortic root. Preoperative transesophageal echocardio-graphy showed no communication between the aorta and left ventricle.
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Figure 3. After aortotomy, the aortic orifice of the aorto-left ventricular tunnel was seen 1 cm above the level of the commissure of the left and right coronary cusps.
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Discussion
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As originally described, ALVT is characterized by a tract that originates in the ascending aorta and courses in the visceral pericardium to penetrate the infundibular septum, terminating in the left ventricle inferior to the right aspect of the aortic root. The aortic orifice of the ALVT is anterior and just below the level of the commissure of the aortic valve.4 Patients with ALVT usually have severe aortic regurgitation into the left ventricle. They present with congestive heart failure, marked cardiomegaly, and signs of severe aortic regurgitation.5 The age at presenta-tion and the severity of symptoms are related to the area of the tunnel and the degree of aortic regurgitation. Some patients present as neonates with cardiomegaly and severe heart failure, others with a smaller tunnel may present in childhood without symptoms.2,6 Rarely do patients present beyond the second or third decade of life.6 When symp-toms are present in infancy and surgical repair is not accomplished, death usually occurs within a few months.
The diagnosis can be made by two-dimensional echo-cardiography and cardiac catheterization.7 A loud systolic and diastolic murmur can be detected in all patients; 57% have a murmur at birth and 41% have a murmur detected in the first 4 years.6 The anomaly may be associated with other cardiac defects such as bicuspid aortic valve, aortic stenosis, patent ductus arteriosus, patent foramen ovale, pulmonary stenosis, ventricular septal defect, or absent right coronary artery.6
The etiology of ALVT remains unknown but there are several theories to explain the pathogenesis, including an abnormal coronary artery, early or intrauterine rupture of a sinus of Valsalva aneurysm, malformation of the distal bulbus cordis, early aortic dissection similar to Marfan syndrome (abundance of mucopolysaccharides in the aortic and tunnel walls), or persistence of embryonic rests of the fifth aortic arch.6 Hovaguimian and colleagues6 proposed a classification based on local anatomical findings: type 1, a simple tunnel with a slit-like opening at the aortic end and no aortic valve distortion; type 2, a large extracardiac aortic wall aneurysm of the tunnel with an oval opening at the aortic end, with or without valvular distortion; type 3, intracardiac aneurysm of the septal portion of the tunnel, with or without right ventricular outflow tract obstruction; and type 4, a combination of types 1 and 2. Our patient was type 2.
The treatment of choice is surgical repair. Patients who are treated medically generally die of congestive heart failure. The risk of hospital death has been noted as 5% to 20%.4,6 Surgical techniques are: direct closure; closure with a patch; and closure of both the aortic and ventricular ends of the tunnel with obliteration of the tunnel. A reoperation rate of 3% has been reported.6 We believe ALVT should be treated surgically as soon as the diagnosis is made, to prevent deterioration of left ventricular function, sudden death, or distortion of the aortic root and valve. Direct closure can be used, except in the case of a large aortic opening where patch material should be employed to prevent valvular distortion.
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References
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Levy MJ, Lillehei CW, Anderson RC, Amplatz K, Edwards JE. Aortico-left ventricular tunnel. Circulation 1963; 27:841–53.[Abstract/Free Full Text]
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Sousa-Uva M, Touchot A, Fermont L, Piot D, Delezoide AL, Serraf A, et al. Aortico-left ventricular tunnel in fetuses and infants. Ann Thorac Surg 1996;61:1805–10.[Abstract/Free Full Text]
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Okoroma EO, Perry LW, Scott LP, McClenathan EM. Aortico-left ventricular tunnel. J Thorac Cardiovasc Surg 1976;71:238–44.[Abstract]
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Kirklin JW, Barratt-Boyes BG. Congenital aneurysm of the sinus of Valsalva. Cardiac surgery. Vol 1. New York: Churchill Livingstone, 1993:835–6.
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Levy MJ, Sehachner A, Blieden LC. Aortico-left ventricular tunnel. Collective review. J Thorac Cardiovasc Surg 1982;84:102–9.[Abstract]
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Hovaguimian H, Çobano
lu A, Starr A. Aortico-left ventricular tunnel: a clinical review and new surgical classification. Ann Thorac Surg 1988;45:106–12.[Abstract]
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Cook AC, Fagg NL, Ho SY, Groves AM, Sharland GK, Anderson RH, et al. Echocardiographic anatomical correlations in aorto-left ventricular tunnel. Br Heart J 1995;74:443–8.[Abstract/Free Full Text]
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Abstract
Introduction
