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Aortico-left ventricular tunnel and sinus of Valsalva aneurysm
Aortico-Ieft ventricular tunnel and sinus of Valsalva aneurysm Case report with operative repair Aortico-left ventricular tunnel (ALVT) is a rare anomaly, only 2l such cases having appeared in the literature. This report describes a case of ALVT in which there were features characteristic of sinus of Valsalva aneurysm (SVA). Details of the operative repair are discussed.
Eric W. Spooner, M.D.,* Jeffrey M. Dunn, M.D.,** and Douglas M. Behrendt, M.D.,** Ann Arbor, Mich.
Levy and associates! first described three cases of aortico-left ventricular tunnel (ALVT) in 1963. Since then, an additional 18 cases have been reported.v !' All five patients treated medically died, whereas 12 of 16 patients having surgical intervention survived, with one late death. The high mortality rate in patients without operative correction stresses the necessity for early diagnosis and surgical treatment. Despite a fairly typical clinical profile of children with ALVT, 9 differentiation from other types of congenital heart disease may be difficult. The purpose of this report is to describe a case of ALVT presenting with features characteristic of sinus of Valsalva aneurysm (SVA) and thus requiring a modified repair.
Case report Patient K. P. was transferred to the University of Michigan Medical Center on the first day of life for the evaluation of a heart murmur. Pregnancy and delivery were uncomplicated, and the infant had only mild difficulty with feedings. Physical examination on admission revealed an acyanotic, normalappearing, vigorous white male infant in no distress with a blood pressure of 86/44 mm. Hg, a pulse rate of 100 beats per minute, a respiratory rate of 82 breaths per minute, and a weight of 3.4 kilograms. The chest was clear. Cardiac examFromthe University of Michigan Medical Center,AnnArbor, Mich. Received for publication May 17, 1977. Accepted for publication Oct. 7, 1977. Address for reprints: Eric W. Spooner,M.D., Department ofPediatrics, Section of Pediatric Cardiology, Albany Medical College, New Scotland Avenue, Albany, N. Y. 12208. *Department of Pediatrics, Section of Pediatric Cardiology. **Department of Surgery, Section of Thoracic Surgery.
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ination revealed a regular rhythm with an active precordium, an apical lift, and no thrills. The point of maximal impulse was in the fifth intercostal space at the midclavicular line. The first and second sounds were normal. A Grade 2/6 short medium-pitched midsystolic ejection murmur was heard at the second intercostal space on the right. A Grade 3/6 highpitched decrescendo murmur starting with S2 and extending throughout diastole was heard best at the second and third right intercostal spaces and radiated toward the apex. Abdominal examination disclosed no abnormalities, and the extremities were acyanotic with normal pulses. Chest roentgenogram revealed a widened superior mediastinum (initially interpreted as a large thymus) and left ventricular enlargement. Electrocardiogram showed left ventricular hypertrophy with normal T waves. The initial echocardiogram demonstrated a markedly dilated left ventricle with good ventricular contractility. There was fine high-frequency fluttering of the anterior mitral valve leaflet on diastole, suggesting aortic insufficiency. Despite multiple scans from the aorta to the left ventricle, no structural abnormalities were noted." The aortic root appeared normal. Results of these and other examinations appear in Table I. Percutaneous cardiac catheterization on the second day of life demonstrated normal hemodynamics of both the left and right sides of the heart, including normal aortic pulse pressure and left ventricular end-diastolic pressure (Table II). However, biplane roentgenograms of the ascending aorta (Fig. I) showed dilation of the ascending aorta with severe aortic insufficiency. Additionally, a saccular aneurysm protruded from the right coronary sinus of Valsalva anteriorly and inferiorly toward the right ventricle. The right coronary artery arose from the aneurysm and was markedly dilated but tapered rapidly to normal diameter. There was no fistula at the tip of the aneurysm into any other cardiac chamber. No tunnel into the left ventricle could be seen on either posteroanterior or lateral views. The aortic insufficiency did not appear to be peripheral to the aortic valve itself. There was no systolic washout of the aneurysm. The most likely diagnosis was be0022-5223/78/0275-0232$00.50/0 © 1978 The C. V. Mosby Co.
Volume 75 Number 2 February. 1978
Aortico-left ventricular tunnel
233
Table I. Echocardiographic data Age
Diameter at end systole (mm.) Aorta Left atrium Diameter at end diastole (mm.) Right ventricle Left ventricle Ejection fraction* (%)
I day
4 weeks
3 months
Normal
12.0 11.7
13.8 9.5
15.4 12.6
10.5-13 9.0-13.5
6.0 30.0 74
8.3 24.0 72
8.4 27.3 80
12 -16 18 -23.5 65 -85
'Echo estimation of ejection fraction calculated assuming volume of left ventricle = (diameter)'.
lieved to be congenital SVA with aortic insufficiency secondary to distortion of the valve leaflets. AL VT could not be ruled out from these studies. Because of marked left ventricular dilatation and tachypnea, he was started on a regimen of digitalis, with a reduction in left ventricular size on echocardiographic examination 2 weeks after discharge (Table I). When he was 3lh months old the murmur became louder (Grade 4/6), the pulse pressure widened (blood pressure 132/40 mm. Hg), and the echocardiogram revealed increasing ventricular dilatation. Although remaining clinically well compensated and asymptomatic, he was admitted for repeat catheterization. This study showed a wide aortic pulse pressure, an aneurysm that had become larger, and still no definite evidence of an ALVT. Because of his deteriorating physical condition and catheterization findings and a strong suspicion that the defect was AL VT, operative repair was performed when the child was 4 months of age. Operative findings. The heart appeared to be normal except for moderate enlargement of the left ventricle, aneurysmal dilatation of the right coronary sinus of Valsalva, and dilatation of the common pulmonary artery and its bifurcation. The aneurysm was approximately 1.5 em. in external diameter, about equal to the size of the aortic annulus. It occupied the area of the right coronary sinus of Valsalva and extended anteriorly over the superior portion of the right ventricle. Internally, no thrombus was identified in the aneurysm. The dilated origin of the right coronary artery arose just to the right of the apex of the aneurysm and the vessel rapidly tapered to normal size. To the left of the origin of the right coronary artery there was a slitlike communication arising from the floor of the aneurysm, which crossed anteriorly and inferiorly to the aortic annulus. This opening tunneled into the left ventricle immediately below the aortic valve (Fig. 2). The aortic valve was tricuspid and appeared to be normal. The aortic orifice of the aneurysm was above the level of the aortic valve annulus in the right coronary sinus of Valsalva. Operative procedure. Correction of the SVA and fistula was performed with the use of hypothermic total arrest. Surface cooling to 28° C. was accomplished. Cardiopulmonary bypass was discontinued, so that there was a quiet, bloodless field. A transverse aortotomy was made and continued inferiorly through the area of the aneurysm. The fistulous tract to the left ventricle was closed with a double layer of mattressed 4-0 Tevdek sutures, and the ventricular septum was brought up to the aortic annulus. Part of the redundant
Table II. Cardiac catheterization data at 2 days of age Pressure (mm. Hg)
Oxygen sat. (%)
Superior vena cava Right atrium Inferior vena cava Right ventricle Mainpulmonary artery Left pulmonary vein* Left atrium* Left ventricle Ascending aorta
72 66 80 76 76 93.5 92
93.5 93.5
2.5 mean 2.5 mean 31/0 31/8, mean 20 5.5 mean 3.7 mean 81/0, LVED 6 81/42, mean 58
Legend: LVED, Left ventricular end-diastolic pressure. 'Reached via patent foramen ovale.
aneurysmal sac was resected, the remainder being plicated to create a tunnel between the aorta and the origin of the right coronary artery. The aortotomy was closed and cardiopulmonary bypass was reinstituted. Electrical defibrillation was necessary, but the patient was weaned from cardiopulmonary bypass without difficulty and without the need of cardiotonic agents. The patient's postoperative course was benign, and he was discharged on the tenth postoperative day. At follow-up 6 weeks later a minimal Grade 1/6 systolic ejection murmur, no diastolic murmurs, and a blood pressure of 86/52 mm. Hg were found. He was asymptomatic and growing well, with normal developmental milestones 6 months postoperatively, and he had no diastolic murmurs.
Discussion
ALVT is a rare entity of obscure origin. Although a relatively distinct clinical profile has emerged,'? difficulties in diagnosis have been encountered. In most reported cases of ALVT, there has been an aortic communication separate from but lateral" or superior to l O the right coronary ostium. This orifice develops into a dilated ampulla. From this ampulla a slitlike tunnel arises and passes anteroinferiorly within 2 to 3 mm. of the aortic valve annulus to enter the left ventricle immediately below the aortic valve. The prox-
The Journal of
234
Spooner, Dunn, Behrendt
Thoracic and Cardiovascular
Surgery
Fig. 1. Roentgenogram of ascending aorta: A, Frontal projection of aortogram showing contrast-filled aneurysm, dilated origin of right coronary artery, contrast in left ventricle, and dilated ascending aorta. B. Lateral projection of aortogram showing filling of large left ventricle and protrusion of large saccular aneurysm from right coronary sinus of Valsalva. Right coronary artery arises from aneurysm and has dilated orifice . AsAo, Ascending aorta. Aneur, Aneurysm. LCA , Left coronary artery. LV, Left ventricular cavity. RCA, Right coronary artery .
imity of the tunnel to the valve annulus may compromise valvular integrity and result in aortic valve insufficiency in many cases .!? Histologically, the ampulla and aortic end of the tunnel contain elastic fibers which become more scarce and are replaced by collagen and endothelium as the tunnel enters the left ventricle." 3. 8 S VA is reported to occur more frequently in bOyS,13. 14 as is the case with AL VT. 10 The most frequent site of involvement in both anomalies is the right
coronary sinus, and both can be present at birth. The coronary ostia may be involved in S VA, 13 although such involvement has not been previously reported with ALVT. Abnormalities of the aortic valve have been reported commonly with both. The fact that our case demonstrated features of both AL VT and S VA suggests a common development. We encountered an extremely large aneurysmal sac protruding from the right coronary sinus of Valsalva. In contrast to findings described in all previous reports,
Volume 75 Number 2 February, 1978
Aortico-left ventricular tunnel
235
F.
Fig. 2. Intraoperative findings: A, External view showing aneurysmarising from right coronary sinus of Valsalva and dilated origin of right coronary artery (RCA). Dashed line indicates incision for aortotorny. B, Internal view through aortotomyshowing orifice of fistula (F) in floor of aneurysm and its proximity to the aortic annulus. The aortic valve cusps (AVC) as well as the right coronary artery ostium (RCA) can be seen. C, Diagrammatic view of sagittal section through the aortic root (AR). aneurysm, and fistula into the left ventricle (LV) . Arrow s show direction of blood flow in diastole. the right coronary artery arose from the aneurysm with marked dilatation of its ostium. The involvement of the right coronary artery, the presence of an aneurysm which enlarged over 4 months , and dilatation of the main pulmonary artery all suggest a generalized process . There was no counter-clockwise rotation of the aortic valve, as reported by Somerville's group." A slitlike tunnel arose from the floor of the aneurysm next to the aortic annulus and communicated with the left ventricle. We can only speculate that an SVA occurring early in utero could develop communication with the myocardial sinusoids and result in an S VA from which a tunnel into the left ventricle arises. The pathological overlap between S VA with aortic
insufficiency and AL VT in our case produced difficulty in preoperative diagnosis. The fact that there was angiographic evidence of right coronary artery involvement , a large aneurysm which became still larger, no systolic washout of contrast in the aneurysm, and no tunnel seen on either posteroanterior or lateral films all suggested SVA, as did the high -pitched quality of the murmur, the absence of a tunnel on echo studies, and the asymptomatic status of the patient. The age of the patient," the severity of the aortic insufficiency, a good dichrotic notch on the aortic tracing," and the typical chest roentgenogram" all suggested AL VT. The anatomic overlap also made it impossible to repair this lesion in a standard fashion for AL VT. Closure of the aortic orifice of the aneurysm would have
236
Spooner, Dunn, Behrendt
The Journal of Thoracic and Cardiovascular Surgery
sacrificed the right coronary artery. Closure of the entrance of the tunnel in the floor of the aneurysm was accomplished with plication of the aneurysm to preserve the patency of the right coronary artery. Whether or not aneurysmal dilatation will recur remains to be seen.
2
3
4
5 6
REFERENCES Levy MJ, Lillehei CW, Anderson RC, et al: Aortico-Ieft ventricular tunnel. Circulation 27:841, 1963 Morgan RI, Mazur JH: Congenital aneurysm of aortic root with fistula to left ventricle. A case report with autopsy findings. Circulation 28:589, 1963 Cooley RN, Harris LC, Rodin AE: Abnormal communication between the aorta and left ventricle. Aortico-Ieft ventricular tunnel. Circulation 31:564, 1965 Roberts WC, Morrow AG: Aortico-Ieft ventricular tunnel. A case of massive aortic regurgitation and intracardiac aneurysms. Am J Med 39:662, 1965 Bove KE, Schwartz DC: Aortico-Ieft ventricular tunnel. A new concept. Am J Cardiol 19:696, 1967 Bernhard WF, Plauth W, Fyler D: Unusual abnormalities of the aortic root or valve necessitating surgical correction in early childhood. N Engl J Med 282:68, 1970
7 Fishbone G, DeLeuchtenberg N, Stansel HC: Aorticoleft ventricular tunnel. Radiology 98:579, 1971 8 Perez-Martinez V, Quero M, Castro C, Moreno F, Brito JM, Merino G: Aortic-left ventricular tunnel. Am Heart J 85:237, 1973 9 Somerville J, English T, Ross DN: Aortico-Ieft ventricular tunnel. Clinical features and surgical management. Br Heart J 36:321, 1974 IO Okorama EO, Perry LW, Scott LP, McClenathan JE: Aortico-Ieft ventricular tunnel. J THORAC CARDIOVASC SURG 71:238, 1976 II Nichols GM, Lees MH, Henken DP, et al: Aortico-Ieft ventricular tunnel. Chest 70: I, 1976 12 Rothbaum DA, Dillon JC, Chang S, Feigenbaum H: Echocardiographic manifestations of right sinus of Valsalva aneurysm. Circulation 49:768, 1974 13 Meyer J, Wukasch DC, Hallman GL, Cooley DA: Aneurysm and fistula of the sinus of Valsalva. Ann Thorac Surg 19:170, 1975 14 Howard RJ, MollerJ, Castaneda AR, Varco RL, Nicoloff DM: Surgical correction of sinus of Valsalva aneurysm. J THORAC CARDIOVASC SURG 66:420, 1973
Information for authors Most of the provisions of the Copyright Act of 1976 became effective on January I, 1978. Therefore, all manuscripts must be accompanied by the following statement, signed by each author: "The undersigned author(s) transfers all copyright ownership of the manuscript entitled (title of article) to The C. V. Mosby Company in the event the work is published. The author(s) warrants that the article is original, is not under consideration by another journal, and has not been previously published." Authors will be consulted, when possible, regarding republication of their material.
Eric W. Spooner, M.D.,* Jeffrey M. Dunn, M.D.,** and Douglas M. Behrendt, M.D.,** Ann Arbor, Mich.
Levy and associates! first described three cases of aortico-left ventricular tunnel (ALVT) in 1963. Since then, an additional 18 cases have been reported.v !' All five patients treated medically died, whereas 12 of 16 patients having surgical intervention survived, with one late death. The high mortality rate in patients without operative correction stresses the necessity for early diagnosis and surgical treatment. Despite a fairly typical clinical profile of children with ALVT, 9 differentiation from other types of congenital heart disease may be difficult. The purpose of this report is to describe a case of ALVT presenting with features characteristic of sinus of Valsalva aneurysm (SVA) and thus requiring a modified repair.
Case report Patient K. P. was transferred to the University of Michigan Medical Center on the first day of life for the evaluation of a heart murmur. Pregnancy and delivery were uncomplicated, and the infant had only mild difficulty with feedings. Physical examination on admission revealed an acyanotic, normalappearing, vigorous white male infant in no distress with a blood pressure of 86/44 mm. Hg, a pulse rate of 100 beats per minute, a respiratory rate of 82 breaths per minute, and a weight of 3.4 kilograms. The chest was clear. Cardiac examFromthe University of Michigan Medical Center,AnnArbor, Mich. Received for publication May 17, 1977. Accepted for publication Oct. 7, 1977. Address for reprints: Eric W. Spooner,M.D., Department ofPediatrics, Section of Pediatric Cardiology, Albany Medical College, New Scotland Avenue, Albany, N. Y. 12208. *Department of Pediatrics, Section of Pediatric Cardiology. **Department of Surgery, Section of Thoracic Surgery.
232
ination revealed a regular rhythm with an active precordium, an apical lift, and no thrills. The point of maximal impulse was in the fifth intercostal space at the midclavicular line. The first and second sounds were normal. A Grade 2/6 short medium-pitched midsystolic ejection murmur was heard at the second intercostal space on the right. A Grade 3/6 highpitched decrescendo murmur starting with S2 and extending throughout diastole was heard best at the second and third right intercostal spaces and radiated toward the apex. Abdominal examination disclosed no abnormalities, and the extremities were acyanotic with normal pulses. Chest roentgenogram revealed a widened superior mediastinum (initially interpreted as a large thymus) and left ventricular enlargement. Electrocardiogram showed left ventricular hypertrophy with normal T waves. The initial echocardiogram demonstrated a markedly dilated left ventricle with good ventricular contractility. There was fine high-frequency fluttering of the anterior mitral valve leaflet on diastole, suggesting aortic insufficiency. Despite multiple scans from the aorta to the left ventricle, no structural abnormalities were noted." The aortic root appeared normal. Results of these and other examinations appear in Table I. Percutaneous cardiac catheterization on the second day of life demonstrated normal hemodynamics of both the left and right sides of the heart, including normal aortic pulse pressure and left ventricular end-diastolic pressure (Table II). However, biplane roentgenograms of the ascending aorta (Fig. I) showed dilation of the ascending aorta with severe aortic insufficiency. Additionally, a saccular aneurysm protruded from the right coronary sinus of Valsalva anteriorly and inferiorly toward the right ventricle. The right coronary artery arose from the aneurysm and was markedly dilated but tapered rapidly to normal diameter. There was no fistula at the tip of the aneurysm into any other cardiac chamber. No tunnel into the left ventricle could be seen on either posteroanterior or lateral views. The aortic insufficiency did not appear to be peripheral to the aortic valve itself. There was no systolic washout of the aneurysm. The most likely diagnosis was be0022-5223/78/0275-0232$00.50/0 © 1978 The C. V. Mosby Co.
Volume 75 Number 2 February. 1978
Aortico-left ventricular tunnel
233
Table I. Echocardiographic data Age
Diameter at end systole (mm.) Aorta Left atrium Diameter at end diastole (mm.) Right ventricle Left ventricle Ejection fraction* (%)
I day
4 weeks
3 months
Normal
12.0 11.7
13.8 9.5
15.4 12.6
10.5-13 9.0-13.5
6.0 30.0 74
8.3 24.0 72
8.4 27.3 80
12 -16 18 -23.5 65 -85
'Echo estimation of ejection fraction calculated assuming volume of left ventricle = (diameter)'.
lieved to be congenital SVA with aortic insufficiency secondary to distortion of the valve leaflets. AL VT could not be ruled out from these studies. Because of marked left ventricular dilatation and tachypnea, he was started on a regimen of digitalis, with a reduction in left ventricular size on echocardiographic examination 2 weeks after discharge (Table I). When he was 3lh months old the murmur became louder (Grade 4/6), the pulse pressure widened (blood pressure 132/40 mm. Hg), and the echocardiogram revealed increasing ventricular dilatation. Although remaining clinically well compensated and asymptomatic, he was admitted for repeat catheterization. This study showed a wide aortic pulse pressure, an aneurysm that had become larger, and still no definite evidence of an ALVT. Because of his deteriorating physical condition and catheterization findings and a strong suspicion that the defect was AL VT, operative repair was performed when the child was 4 months of age. Operative findings. The heart appeared to be normal except for moderate enlargement of the left ventricle, aneurysmal dilatation of the right coronary sinus of Valsalva, and dilatation of the common pulmonary artery and its bifurcation. The aneurysm was approximately 1.5 em. in external diameter, about equal to the size of the aortic annulus. It occupied the area of the right coronary sinus of Valsalva and extended anteriorly over the superior portion of the right ventricle. Internally, no thrombus was identified in the aneurysm. The dilated origin of the right coronary artery arose just to the right of the apex of the aneurysm and the vessel rapidly tapered to normal size. To the left of the origin of the right coronary artery there was a slitlike communication arising from the floor of the aneurysm, which crossed anteriorly and inferiorly to the aortic annulus. This opening tunneled into the left ventricle immediately below the aortic valve (Fig. 2). The aortic valve was tricuspid and appeared to be normal. The aortic orifice of the aneurysm was above the level of the aortic valve annulus in the right coronary sinus of Valsalva. Operative procedure. Correction of the SVA and fistula was performed with the use of hypothermic total arrest. Surface cooling to 28° C. was accomplished. Cardiopulmonary bypass was discontinued, so that there was a quiet, bloodless field. A transverse aortotomy was made and continued inferiorly through the area of the aneurysm. The fistulous tract to the left ventricle was closed with a double layer of mattressed 4-0 Tevdek sutures, and the ventricular septum was brought up to the aortic annulus. Part of the redundant
Table II. Cardiac catheterization data at 2 days of age Pressure (mm. Hg)
Oxygen sat. (%)
Superior vena cava Right atrium Inferior vena cava Right ventricle Mainpulmonary artery Left pulmonary vein* Left atrium* Left ventricle Ascending aorta
72 66 80 76 76 93.5 92
93.5 93.5
2.5 mean 2.5 mean 31/0 31/8, mean 20 5.5 mean 3.7 mean 81/0, LVED 6 81/42, mean 58
Legend: LVED, Left ventricular end-diastolic pressure. 'Reached via patent foramen ovale.
aneurysmal sac was resected, the remainder being plicated to create a tunnel between the aorta and the origin of the right coronary artery. The aortotomy was closed and cardiopulmonary bypass was reinstituted. Electrical defibrillation was necessary, but the patient was weaned from cardiopulmonary bypass without difficulty and without the need of cardiotonic agents. The patient's postoperative course was benign, and he was discharged on the tenth postoperative day. At follow-up 6 weeks later a minimal Grade 1/6 systolic ejection murmur, no diastolic murmurs, and a blood pressure of 86/52 mm. Hg were found. He was asymptomatic and growing well, with normal developmental milestones 6 months postoperatively, and he had no diastolic murmurs.
Discussion
ALVT is a rare entity of obscure origin. Although a relatively distinct clinical profile has emerged,'? difficulties in diagnosis have been encountered. In most reported cases of ALVT, there has been an aortic communication separate from but lateral" or superior to l O the right coronary ostium. This orifice develops into a dilated ampulla. From this ampulla a slitlike tunnel arises and passes anteroinferiorly within 2 to 3 mm. of the aortic valve annulus to enter the left ventricle immediately below the aortic valve. The prox-
The Journal of
234
Spooner, Dunn, Behrendt
Thoracic and Cardiovascular
Surgery
Fig. 1. Roentgenogram of ascending aorta: A, Frontal projection of aortogram showing contrast-filled aneurysm, dilated origin of right coronary artery, contrast in left ventricle, and dilated ascending aorta. B. Lateral projection of aortogram showing filling of large left ventricle and protrusion of large saccular aneurysm from right coronary sinus of Valsalva. Right coronary artery arises from aneurysm and has dilated orifice . AsAo, Ascending aorta. Aneur, Aneurysm. LCA , Left coronary artery. LV, Left ventricular cavity. RCA, Right coronary artery .
imity of the tunnel to the valve annulus may compromise valvular integrity and result in aortic valve insufficiency in many cases .!? Histologically, the ampulla and aortic end of the tunnel contain elastic fibers which become more scarce and are replaced by collagen and endothelium as the tunnel enters the left ventricle." 3. 8 S VA is reported to occur more frequently in bOyS,13. 14 as is the case with AL VT. 10 The most frequent site of involvement in both anomalies is the right
coronary sinus, and both can be present at birth. The coronary ostia may be involved in S VA, 13 although such involvement has not been previously reported with ALVT. Abnormalities of the aortic valve have been reported commonly with both. The fact that our case demonstrated features of both AL VT and S VA suggests a common development. We encountered an extremely large aneurysmal sac protruding from the right coronary sinus of Valsalva. In contrast to findings described in all previous reports,
Volume 75 Number 2 February, 1978
Aortico-left ventricular tunnel
235
F.
Fig. 2. Intraoperative findings: A, External view showing aneurysmarising from right coronary sinus of Valsalva and dilated origin of right coronary artery (RCA). Dashed line indicates incision for aortotorny. B, Internal view through aortotomyshowing orifice of fistula (F) in floor of aneurysm and its proximity to the aortic annulus. The aortic valve cusps (AVC) as well as the right coronary artery ostium (RCA) can be seen. C, Diagrammatic view of sagittal section through the aortic root (AR). aneurysm, and fistula into the left ventricle (LV) . Arrow s show direction of blood flow in diastole. the right coronary artery arose from the aneurysm with marked dilatation of its ostium. The involvement of the right coronary artery, the presence of an aneurysm which enlarged over 4 months , and dilatation of the main pulmonary artery all suggest a generalized process . There was no counter-clockwise rotation of the aortic valve, as reported by Somerville's group." A slitlike tunnel arose from the floor of the aneurysm next to the aortic annulus and communicated with the left ventricle. We can only speculate that an SVA occurring early in utero could develop communication with the myocardial sinusoids and result in an S VA from which a tunnel into the left ventricle arises. The pathological overlap between S VA with aortic
insufficiency and AL VT in our case produced difficulty in preoperative diagnosis. The fact that there was angiographic evidence of right coronary artery involvement , a large aneurysm which became still larger, no systolic washout of contrast in the aneurysm, and no tunnel seen on either posteroanterior or lateral films all suggested SVA, as did the high -pitched quality of the murmur, the absence of a tunnel on echo studies, and the asymptomatic status of the patient. The age of the patient," the severity of the aortic insufficiency, a good dichrotic notch on the aortic tracing," and the typical chest roentgenogram" all suggested AL VT. The anatomic overlap also made it impossible to repair this lesion in a standard fashion for AL VT. Closure of the aortic orifice of the aneurysm would have
236
Spooner, Dunn, Behrendt
The Journal of Thoracic and Cardiovascular Surgery
sacrificed the right coronary artery. Closure of the entrance of the tunnel in the floor of the aneurysm was accomplished with plication of the aneurysm to preserve the patency of the right coronary artery. Whether or not aneurysmal dilatation will recur remains to be seen.
2
3
4
5 6
REFERENCES Levy MJ, Lillehei CW, Anderson RC, et al: Aortico-Ieft ventricular tunnel. Circulation 27:841, 1963 Morgan RI, Mazur JH: Congenital aneurysm of aortic root with fistula to left ventricle. A case report with autopsy findings. Circulation 28:589, 1963 Cooley RN, Harris LC, Rodin AE: Abnormal communication between the aorta and left ventricle. Aortico-Ieft ventricular tunnel. Circulation 31:564, 1965 Roberts WC, Morrow AG: Aortico-Ieft ventricular tunnel. A case of massive aortic regurgitation and intracardiac aneurysms. Am J Med 39:662, 1965 Bove KE, Schwartz DC: Aortico-Ieft ventricular tunnel. A new concept. Am J Cardiol 19:696, 1967 Bernhard WF, Plauth W, Fyler D: Unusual abnormalities of the aortic root or valve necessitating surgical correction in early childhood. N Engl J Med 282:68, 1970
7 Fishbone G, DeLeuchtenberg N, Stansel HC: Aorticoleft ventricular tunnel. Radiology 98:579, 1971 8 Perez-Martinez V, Quero M, Castro C, Moreno F, Brito JM, Merino G: Aortic-left ventricular tunnel. Am Heart J 85:237, 1973 9 Somerville J, English T, Ross DN: Aortico-Ieft ventricular tunnel. Clinical features and surgical management. Br Heart J 36:321, 1974 IO Okorama EO, Perry LW, Scott LP, McClenathan JE: Aortico-Ieft ventricular tunnel. J THORAC CARDIOVASC SURG 71:238, 1976 II Nichols GM, Lees MH, Henken DP, et al: Aortico-Ieft ventricular tunnel. Chest 70: I, 1976 12 Rothbaum DA, Dillon JC, Chang S, Feigenbaum H: Echocardiographic manifestations of right sinus of Valsalva aneurysm. Circulation 49:768, 1974 13 Meyer J, Wukasch DC, Hallman GL, Cooley DA: Aneurysm and fistula of the sinus of Valsalva. Ann Thorac Surg 19:170, 1975 14 Howard RJ, MollerJ, Castaneda AR, Varco RL, Nicoloff DM: Surgical correction of sinus of Valsalva aneurysm. J THORAC CARDIOVASC SURG 66:420, 1973
Information for authors Most of the provisions of the Copyright Act of 1976 became effective on January I, 1978. Therefore, all manuscripts must be accompanied by the following statement, signed by each author: "The undersigned author(s) transfers all copyright ownership of the manuscript entitled (title of article) to The C. V. Mosby Company in the event the work is published. The author(s) warrants that the article is original, is not under consideration by another journal, and has not been previously published." Authors will be consulted, when possible, regarding republication of their material.