Ann Thorac Surg 1998;65:1780-1783
© 1998 The Society of Thoracic Surgeons
Case Report
Repair of Aortico–Left Ventricular Tunnel Originating From the Left Aortic Sinus
Guido Michielon, MDa,
Carlo Sorbara, MDb,
Dino Carlo Casarotto, MDa
a Department of Cardiac Surgery, University of Padua Medical School, Padua, Italy
b Department of Cardiovascular Anesthesia, University of Padua Medical School, Padua, Italy
Accepted for publication December 24, 1997.
Address reprint requests to Dr Michielon, Division of Cardiac Surgery, Venice-Mirano, Via Mariutto 13, 30035 Venice-Mirano, Italy
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Abstract
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We report on a case of an 11-year-old asymptomatic child with aortico–left ventricular tunnel arising from the left aortic sinus. Preoperative transesophageal echocardiography showed a dilated aortic root with mild aortic valve incompetence and demonstrated the course of the tunnel, which originated from the left coronary sinus entering the outlet portion of the left ventricular outflow tract. Patch closure of the aortic end of the tunnel eliminated left ventricular volume overload with immediate marked reduction of cardiomegaly. At 10-month follow-up the child is asymptomatic and receiving no oral medications. Control two-dimensional Doppler echocardiography shows trivial central aortic valve incompetence.
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Introduction
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Aortico–left ventricular tunnel (ALVT) is a rare congenital heart disease, originally described by Edwards in 1961 [1] as an abnormal paravalvular communication between the aorta and the left ventricle. Most commonly the tunnel is located above the right coronary sinus; however, a connection between the left aortic sinus and the left ventricle has been previously reported in 1 case [2]. The aorto–ventricular communication leads to heart failure, mimicking aortic valve incompetence. No long-term survival has been reported with medical treatment alone; therefore, surgical treatment is mandatory.
An 11-year-old boy with no previous cardiac history was admitted to a referring hospital because of recent onset of fever (39°C) and diarrhea. A "new" heart murmur was detected, prompting an echocardiographic evaluation, which was significant for "aortic valve incompetence." An aortic valve infective endocarditis was suspected, and serial blood cultures were obtained and found negative for bacterial growth. Serology was positive at high dilutions for anti-O and anti-H Salmonella typhi antibodies.
The child was transferred to our institution after 4 weeks of intravenous antibiotic therapy for further evaluation. On admission he was asymptomatic and afebrile. Blood pressure was 90/35 mm Hg. A 4/6 to-and-fro diastolic murmur was heard on his hyperactive precordium. Peripheral pulses were bouncing. A 12-lead electrocardiogram showed normal sinus rhythm, heart rate of 85 beats/min with QRS axis +30 degrees and evidence of left ventricular hypertrophy. Chest roentgenogram showed an increased heart size (cardiothoracic ratio, 0.65) and broad upper mediastinal shadow due to an enlarged ascending aorta. Transesophageal echocardiography revealed marked aortic root dilatation (diameter, 3 cm) and left ventricular end-diastolic diameter of 5.16 cm. These findings were in conflict with the recent discovery of a "new" heart murmur, suggesting a congenital disease of the aortic root. The aortic valve was tricuspid with partial fusion of the intercoronary commissure. A short-axis view demonstrated a paravalvular communication arising from the ascending aorta above the left coronary sinus, 5 mm aside the aortic annulus, entering the left ventricular outflow tract in the outlet portion. Interrogation by color Doppler echocardiography suggested the presence of anterograde systolic and retrograde diastolic flow across the tunnel, with only trivial central aortic valve incompetence (Fig 1). The transesophageal echocardiographic study was conclusive for type 1 ALVT [3] arising above the left coronary sinus. Angiographic confirmation was thought to be unnecessary, and surgical intervention was scheduled thereafter.
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Fig 1. Short-axis (A) and long-axis (B, C) transesophageal echocardiographic view demonstrating the course of the aortico–left ventricular tunnel as a paravalvular communication external to the aortic valve annulus (C) and the rapid aortic runoff in diastole (A, B).
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The operation was performed during cardiopulmonary bypass and moderate hypothermia (28°C) with single venous uptake and left ventricular venting through the right superior pulmonary vein. After aortic cross-clamping and cardioplegic arrest, the aortic valve was inspected. There was no evidence of cusp prolapse or signs of previous endocarditis. The aortic entrance of the tunnel was identified external to the aortic valve annulus, 5 mm to the left of the left coronary ostium (Fig 2). Closure of the aortic entrance of the tunnel was accomplished using an autologous pericardial patch. Doppler interrogation by intraoperative transesophageal echocardiography after surgical repair demonstrated a complete closure of the ALVT with trivial residual central aortic valve incompetence. No gradient was detected across the left ventricular outflow tract. The postoperative course was uneventful. The patient was discharged home on the seventh postoperative day, receiving no medications, with marked reduction of his cardiomegaly on the predischarge chest roentgenogram (cardiothoracic ratio, 0.5). A transthoracic two-dimensional echocardiogram at 20 months of follow-up demonstrated reduction of left ventricular end-diastolic diameter to 4.02 cm and trivial central aortic valve incompetence.
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Fig 2. (A) Operative view of the aortico–left ventricular tunnel (black arrow). (B) Surgical correction by pericardial patch closure of its aortic entrance next to the intercoronary commissure. The head of the patient is on the left.
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Comment
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Aortico–left ventricular tunnel is a very rare congenital malformation, accounting for 0.1% of all congenital heart disease. Levy and associates are credited with the first successful surgical correction in 1963. The etiology of ALVT is still unknown, and no pathogenetic theory appears to be conclusive as yet. Based on histologic sections Levy and associates suspected that the ALVT was an abnormal coronary artery. The origin of one coronary artery from the tunnel supports the theory of ALVT as an example of coronary artery fistula [4]. Persistence of embryonic rests of the fifth arch, intrauterine rupture of a sinus of Valsalva aneurysm [5], and early aortic dissections as in Marfan syndrome [6] have been proposed as other pathogenetic theories of ALVT origin. Hovaguimian and associates [3] proposed a classification of ALVT in four anatomic subtypes. In the review of 34 surgically treated cases reported in the English-language literature, they described a slit-like opening at the aortic end, with no valve distortion in 24% of cases (type 1), a large extracardiac aneurysm in 44% of cases (type 2), intracardiac aneurysm of the septal portion of the tunnel, with or without right ventricular outflow tract obstruction, in 24% of cases (type 3), and a combination of type 2 and type 3 in 8% of cases (type 4). Clinical presentation frequently occurs early in infancy (60% at birth) because of rapid aortic run-off, leading to cardiomegaly and congestive heart failure. Although cardiac catheterization remains the gold standard technique in detecting ALVT, definitive diagnosis can be obtained by two-dimensional and Doppler echocardiography [5, 7]. Superb definition of anatomic details is currently available with transesophageal echocardiography. Surgical correction of this lesion is mandatory, to reduce left ventricular volume overload and the progressive annular dilatation.
In our case the ALVT originated from the left aortic sinus in an asymptomatic child. The incidental finding of a heart murmur during hospital admission for recurrent hyperpyrexia in an otherwise asymptomatic child is quite unusual for this rare lesion. The diagnosis of a type 1 ALVT was very well documented by transesophageal echocardiography alone, with no need for angiographic confirmation. The aortic opening of the tunnel in the left aortic sinus represents a very unusual anatomic finding for ALVT.
Early and late occurrence of aortic valve incompetence as well as obstruction of the right ventricular outflow tract by an aneurysmatic tunnel represent the major concerns in surgical correction of ALVT. Serino and associates [8] have suggested that an inherent weakness at the junction between the membranous portion of the ventricular septum and the aortic anulus is the most important factor leading to aortic valve incompetence in ALVT. The rapid aortic runoff through the tunnel, which is interposed between the distal infundibular septum and the aortic root, can change the geometry of the aortic root and compromise structural support of the sinuses, resulting in marked root dilatation and cusp prolapse.
Surgical correction in our case included single patch closure of the aortic opening of the tunnel. This choice, although controversial, was selected because this was a type 1 ALVT. Because aneurysm of the tunnel is generally a presenting anatomic finding of ALVT, we thought that exclusion of the tunnel by patch closure of its aortic opening would have resulted in a blind pouch at high impedence and little potential for ex novo aneurysm development. In conclusion, two-dimensional and color Doppler flow echocardiographic imaging allow accurate noninvasive diagnosis of ALVT. Transesophageal echocardiography, when feasible, provides excellent definition of anatomic details, guiding the surgeon in his or her operative strategy (eg, single patch repair, double patch repair, obliteration of aneurysmatic tunnel). In the absence of intracardiac or extracardiac aneurysm formation (type 1 ALVT), we believe that single patch closure of the aortic end of the tunnel can prevent distortion of the aortic valve, preserving early valve competence. Longer fol-low-up is necessary to evaluate possible late development of aortic valve insufficiency.
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Acknowledgments
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We thank Mr Giancarlo Pengo for his technical assistance in the preparation of the photographic support of the manuscript.
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References
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- Edwards J.E. Atlas of acquired disease of the heart and great vessels, vol 3. Philadelphia: Saunders, 1961:1142.
- Horvath P., Balaji S., Skovranek S., Hucin B., de Leval M.R., Stark J. Surgical treatment of aortico–left ventricular tunnel. Eur J Cardiothorac Surg 1991;5:113-117.[Abstract]
- Hovaguimian H., Cobanoglu A., Starr A. Aortico–left ventricular tunnel: a clinical review and new surgical classification. Ann Thorac Surg 1988;45:106-112.[Abstract]
- Hucin B., Horvath P., Skovranek J., Reich O., Samanek M. Correction of aortico–left ventricular tunnel during the first day of life. Ann Thorac Surg 1989;47:254-256.[Abstract]
- Turley K., Silverman N.H., Teitel D., et al. Repair of aortico–left ventricular tunnel in the neonate: surgical, anatomic and echocardiographic considerations. Circulation 1982;65:1015-1020.[Abstract/Free Full Text]
- Cooley R.N., Harris L.C., Rodin A.E. Abnormal communication between the aorta and left ventricle: aortico–left ventricular tunnel. Circulation 1965;31:564-571.[Abstract/Free Full Text]
- Fripp R.R., Werner J.C., Whitman V., et al. Pulsed Doppler and two dimensional echocardiographic findings in aortico–left ventricular tunnel. J Am Coll Cardiol 1984;4:1012-1014.[Abstract]
- Serino W., Andrade I., Ross D., et al. Aorto–left ventricular communication after closure: late postoperative problems. Br Heart J 1983;49:501-506.[Abstract/Free Full Text]
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510 - 511.
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