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Aortico—left ventricular tunnel
Aortico-left ventricular tunnel Clinical profile, diagnostic features, and surgical considerations Case histories of 2 patients with aortico-left ventricular tunnel are reported. A review of the literature on this subject shows a unique clinical profile. The electrocardiographic (ECG) and roentgenographic features as well as the hemodynamic and angiographic data of 18 previously reported patients are reviewed. The anatomy and pathophysiology of the defect are discussed and surgical management is described. It is recommended that surgical correction be carried out as soon as the diagnosis is established. Edwin 0 . Okoroma, M.D., Lowell W. Perry, M.D., Lewis P. Scott III, M.D., and James E. McClenathan, M.D., Washington, D. C.
v 3 i n c e 1963, when Levy and his associates 1 described the entity of aortico-left ventricular tunnel in 3 patients, 15 additional patients have been reported. 2 " 10 Although a clinical profile has emerged from a review of these patients, very little detailed description of the surgical problems has been included in these reports. Of these 18 patients, all 5 who had no surgery and 4 of 13 who had surgery died. This 50 per cent mortality rate underscores the need for early surgical intervention in this lesion, which appears readily amenable to total correction. At our institution, between 1964 and 1974, 1,754 cases of congenital heart disease documented by cardiac catheterization, surgery, and/or postmortem examination were seen. 11 Among these were 2 cases of aortico-left ventricular tunnel. The purpose of this report is to describe these 2 patients, both female, who have had successful surgical repair of this defect and to stress the anatomy, pathophysiology, diagnostic features, and surgical consideration in this defect.
From the Departments of Cardiology and Cardiovascular Surgery, Children's Hospital National Medical Center, and The George Washington University Medical School, Department of Child Health and Development. Received for publication May 12, 1975. Address for reprints: James E. McClenathan, M.D., Department of Cardiovascular Surgery, Children's Hospital National Medical Center, Washington, D. C. 20009. 238
Case summaries CASE 1. C. T., a 2 '/2-year-old white girl, was referred for evaluation of a to-and-fro murmur that was first noted at age 4 days. Although she was asymptomatic, the peripheral pulses were bounding and clinical evaluation suggested aortic regurgitation. The blood pressure was 100/20 mm. Hg. The electrocardiogram (ECG) was abnormal, showing left ventricular hypertrophy with inverted T waves in the left precordial leads (Fig. 1). The chest roentgenograms showed a wide mediastinum and cardiomegaly (Fig. 2). Initial catheterization suggested a left coronary-left ventricular fistula. A repeat catheterization 5 months later, done because of onset of symptoms and progressive left ventricular enlargement on chest roentgenogram, demonstrated an aortico-left ventricular communication arising above and anterior to the right sinus of Valsalva. At operation this was confirmed. Evaluation 4 years after operation revealed a Grade 2/6 diastolic murmur considered to be due to minimal aortic valve regurgitation. The blood pressure was 104/60 mm. Hg. CASE 2. K. B. was a white girl who presented at birth with severe congestive heart failure (see Fig. 4/1). She was treated medically with good result. Following stabilization, a cardiac catheterization at age 3 days showed moderately severe aortic regurgitation through an aortico-left ventricular tunnel. She was discharged taking digoxin. A repeat catheterization at age 18 months confirmed the initial diagnosis. At this time, the blood pressure was 90/20 mm. Hg, a to-and-fro murmur with a diastolic thrill was noted, the ECG showed an abnormal QS pattern in the right precordial leads suggesting abnormal septal depolarization (Fig. 3), and the chest roentgenogram showed a prominent ascending aorta and left ventricular enlargement (Fig. AB). At age 2 years she underwent operative correction because of progressive congestive heart failure. In the immediate postoperative
Volume 71 Number 2 February, 1976
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Fig. 1. ECG's of C. T. (Case 1) (upper) preoperatively and (lower) 4 years postoperatively. The left ventricular hypertrophy noted preoperatively is no longer present. period she had recurrent episodes of supraventricular tachycardia which were controlled with digoxin. The blood pressure remained 100/60 mm. Hg. Although she has continued to do well postoperatively, she still has a Grade 2-3/6 diastolic murmur. A repeat catheterization 3 years after operation showed minimal aortic valve regurgitation, a dilated ascending aorta, and left ventricular enlargement (Fig. AC). Discussion Clinical profile. From a review of the literature, it appears that aortico-left ventricular tunnel is a congenital heart defect that usually presents in early infancy with a characteristic loud to-and-fro murmur. This has been demonstrated well by phonocardiograms.8' 9 Of the 20 reported cases, including our own 2 patients,
this characteristic murmur was first heard during the first week of life in 15 patients, and in another 4 it was heard first between 2 and 9 months. The clinical history was not available in 1 patient.10 Ejection clicks, although rare, may be present. Congestive heart failure develops frequently. Nine patients developed congestive heart failure within the first year of life; only 2 patients have been described as being completely asymptomatic by the time they were operated upon. Presumably, a 26-year-old man, the oldest patient thus far described,9 was also asymptomatic at the time of surgery. Cyanosis has been rare, having been described in only 3 patients,1, 5 2 of whom were in profound congestive heart failure. Fourteen of the 20 patients have been male.
240
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Okoroma et al.
presentation.6 Postoperatively, there has been usually a regression of the LVH on the ECG (Fig. 1). Chest roentgenograms. The chest roentgenogram is characteristic. Virtually all cases have shown a wide mediastinum resulting from dilatation of the ascending aorta and cardiomegaly with left ventricular configuration. A characteristic anterior indentation of the esophagus has been described2 and was present in both our patients (Fig. 5). Pulmonary vascularity has been normal or slightly increased (Figs. 2 and 4). Catheterization and angiography data. Catheterization data are available in 15 patients. Most have had normal right heart hemodynamics, the exceptions being those patients with significant right ventricular outflow obstruction. All have shown markedly wide aortic pulse pressures. None has had any intracardiac shunts. Aortic root angiograms are necessary to make the diagnosis. In 1 of our patients (Case 1) the diagnosis was not made during the initial study. The reason for failure to make the diagnosis was the view in which the angiograms were taken. Either a lateral or right anterior oblique aortic root angiogram is necessary to demonstrate the defect. In 1 of our patients, there was both antegrade and retrograde blood flow through the tunnel during systole and diastole, respectively. Postoperative catheterization data are available in only 2 patients, 1 of Somerville, Sughish, and Ross,9 and our Case 2. Both have mild aortic valve regurgitation. Our patient still has a persistently dilated ascending aorta (Fig. AC). Anatomy. The tunnel seems to be constant both in its origin above the right coronary sinus, separate and distinct from the right coronary artery orifice, and in its
ECG's. The ECG has been described in 17 of the 20 patients. Fifteen had left ventricular hypertrophy: 2 of these in addition had right ventricular hypertrophy. One of our patients had septal Q in the right precordial leads, indicating abnormal septal depolarization. Only 1 patient has had a normal ECG at the time of his initial
Fig. 2. Preoperative chest roentgenogram of C. T. (Case 1) which demonstrates wide mediastinum and left ventricular enlargement.
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Volume 71 Number 2 February, 1976
Aortico-left ventricular tunnel
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Fig. 4C. Postoperative aortic root angiogram of K. B. (Case 2). The ascending aorta is still dilated 3 years later. Fig. 4A. Chest roentgenogram of K. B. (Case 2) at birth, showing wide mediastinum, cardiomegaly, and passive pulmonary congestion.
Table I. Associated cardiac defects in aortico-left ventricular tunnel Cardiac defects Bicuspid aortic valve1, *• 5l I0 Aortic valve stenosis1, *• 6 ' 10 Right ventricular outflow obstruction: Infundibular5, a Valvular1 Patent ductus arteriosus3 Absent right coronary artery9
Fig. 4B. Chest roentgenogram of K. B. (Case 2) at the age of 18 months.
No. of patients 4 4 2 1 1 1
ventricular entrance. The tunnel passes anteroinferiorly, is aneurysmal in size and shape, elevating the right ventricular outflow tract, and enters the left ventricle at a point high in the septum immediately beneath the annulus of the aortic valve (Fig. 6). The elevation of the outflow tract has been considered the cause of the right ventricular outflow tract obstruction in 2 patients.5' 8 One point not previously stressed is that the medial wall of the aneurysmal channel is within 2 to 3 mm. of the annulus of the aortic valve. In the 2 cases presented here, this fact was very evident at the time of surgery and this proximity appeared to compromise the integrity of the aortic valve. Histologic studies2, 3 ' 8 have shown the presence of elastic fibers in that portion of the tunnel near the aortic orifice which seemingly dissipate as the ventricular aspect is approached. At the ventricular end the tunnel wall is primarily collagen and endothelial lining. Surgical considerations. The surgical repair in the 2 cases presented was done with standard cardiopulmo-
The Journal of Thoracic and Cardiovascular Surgery
2 4 2 Okoroma et al.
Fig. 5. Chest roentgenograms with esophagogram demonstrating anterior indentation of the esophagus: A, C. T. (Case 1); B, K. B. (Case 2). nary bypass. After the body temperature has been lowered to 30° C , the aorta was cross-clamped. In Case 2 (K. B.) the tunnel was so large that it was incised transversely near its origin on the aorta, thus giving good access to the aortic aspect. The aortic origin was carefully closed with buttressed mattress sutures, reattaching the superior margin of the aortic annulus to the aortic wall. The fistula was then further opened inferiorly and the ventricular component exposed and this was similarly closed with buttressed mattress sutures to effectively close the communication near its entrance to the ventricular cavity. The central portion of the large communication was then obliterated by running sutures. In Case 1 (C. T.), in order to effect a more accurate closure of the aortic aspect, it was necessary to open the aorta superiorly to the coronary ostium. Since one margin of the aortic orifice of the tunnel approached within 3 mm. of the right coronary ostium, this was felt to be a safer method for repair of the aortic ostium. The ventricular and central portions of the tunnel were closed in a manner similar to that used in Case 2. In both cases the aortic valve was tricuspid; however, surgical closure of the aortic ostium of the tunnel in each case somewhat distorted the normal contour of the aortic valve. This may have contributed to the residual minimal aortic valve incompetence. Immediately postoperative pressure measurements
revealed no systolic gradient across the aortic valve. Diastolic aortic pressures rose from a preoperative average of 40 to 80 mm. Hg postoperatively. The pulse contour became normal. Comment Aortico-left ventricular tunnel is a rare congenital cardiac defect.2'4 Its incidence prior to this report is not known. Only 2 cases were observed among 1,754 cases of documented CHD over a 10 year period.11 The embryology is still obscure. While Levy and associates1 suggested that the tunnel represents an anomalous vessel, Somerville and colleagues9 believed that a disturbance in the development and incorporation of the distal bulbus cordis may be the cause. It would seem reasonable that this lesion is a developmental anomaly in which a weak spot in the aortic wall superior to the right coronary artery permits communication with ventricular sinusoids which are still in communication with the ventricular cavity. In time the tunnel then develops endothelial lining. Initially it was felt that associated defects were quite frequent 4 ' 5 ' 8 but Table I shows that aortico-left ventricular tunnel is seldom associated with other complex cardiac lesions. Aortic valve lesions are the most common among these. Although the clinical profile seems unique, it appears that the most distinguishing clinical feature of
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Fig. 6. Illustration of aortico-left ventricular tunnel (T) showing its aneurysmal size and, in the sagittal section, its relationship to the right coronary artery and the aortic valve. AOT, Aortic ostium of the tunnel. VOT, Ventricular ostium of the tunnel. RC, Right coronary artery. T, Tunnel. this lesion is the time of onset of symptoms. Although other cardiac defects such as aortic valve regurgitation, coronary artery fistula, ventricular septal defect with aortic regurgitation, and ruptured sinus of Valsalva may have similar clinical features, they produce symptoms much later in life. Aortico-left ventricular tunnel usually produces signs and symptoms in the newborn period or shortly therafter. Eighteen of the 19 patients with available clinical history presented with a characteristic to-and-fro murmur and/or congestive heart failure within the first 3 months of life. The early presentation of symptoms is most likely related to the large volume of blood regurgitation through the tunnel in these patients. Our 2 patients had 3+ to 4 + regurgitation through the tunnel on cineangiograms; however, despite this large volume, Case 1 was clinically asymptomatic. Finally, from a surgical standpoint it is necessary to close accurately the aortic aspect of this lesion with carefully buttressed sutures in such a manner as neither to induce major distortion of the anterior aortic cusps nor to compromise the right coronary orifice. Whether the ventricular aspect can be closed at the level of its entrance into the ventricular cavity is unanswered. Our feeling is that surgical manipulation at or near the level of its entrance into the ventricle may seriously disturb the conduction system and for that
reason this was not attempted in our cases. Rather, the closure of the ventricular aspect was performed approximately 0.5 to 1 cm. proximal to its entrance into the ventricle. Somerville and associates9 have proposed 5 years as the ideal age for surgery. In view of the high incidence of residual aortic valve regurgitation which has persisted in those patients surviving surgery (6 of 8 patients in whom it was commented upon), we would certainly recommend a much earlier repair. Because we feel that long-standing regurgitation through the tunnel with its attendant left ventricular dilatation contributes to dilatation of the aortic valve ring, and therefore to aortic regurgitation, surgical correction should be carried out as soon as the diagnosis is established. REFERENCES 1 Levy, M. J., Lillehei, C. W., Anderson, R. C , Amplatz, K., and Edwards, J. E.: Aortico-Left Ventricular Tunnel, Circulation 27: 841, 1963. 2 Morgan, R. I., and Mazur, J. H.: Congenital Aneurysm of Aortic Root With Fistula to Left Ventricle. A Case Report With Autopsy Findings, Circulation 28: 589, 1963. 3 Cooley, R. N., Harris, L. C , and Rodin, A. E.: Abnormal Communication Between the Aorta and Left Ventricle. Aortico-Left Ventricular Tunnel, Circulation 31: 564, 1965.
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4 Roberts, W. C , and Morrow, A. G.: Aortico-Left Ventricular Tunnel. A Case of Massive Aortic Regurgitation and of Intracardiac Aneurysms, Am. J. Med. 39: 662, 1965. 5 Bove, K. E., and Schwarts, D. C : Aortico-Left Ventricular Tunnel. A New Concept. Am. J. Cardiol. 19: 696, 1967. 6 Burnhard, W. F., Plantts, W., and 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., and Stangel, H. C : Aortico-Left Ventricular Tunnel, Radiology 98: 579, 1971.
The Journal of Thoracic and Cardiovascular Surgery
8 Perez-Martinez, V., Queno, M., Cesho, C , Moreno, F., Bito, J. M., and Merino, G.: Aortico-Left Ventricular Tunnel, Am. Heart J. 85: 237, 1973. 9 Somerville, J., Sughish, T., and Ross, D.: Aortico-Left Ventricular Tunnel. Clinical Features and Surgical Management, Br. Heart J. 36: 321, 1974. 10 Edwards, J. E.: Atlas of Acquired Diseases of the Heart and Great Vessels, Vol. II, Philadelphia, 1961, W. B. Saunders Company, p. 1142. 11 Perry, L. W., Scott, L. P., Shapiro, S. R., McClenathan, J. E., Chandra, R., and Walsh, B. J.: Documented Heart Disease at Children's Hospital National Medical Center, 1964-1974: Report of 1754 Cases, Clin. Proc. CHNMC 31: 73, 1975.
v 3 i n c e 1963, when Levy and his associates 1 described the entity of aortico-left ventricular tunnel in 3 patients, 15 additional patients have been reported. 2 " 10 Although a clinical profile has emerged from a review of these patients, very little detailed description of the surgical problems has been included in these reports. Of these 18 patients, all 5 who had no surgery and 4 of 13 who had surgery died. This 50 per cent mortality rate underscores the need for early surgical intervention in this lesion, which appears readily amenable to total correction. At our institution, between 1964 and 1974, 1,754 cases of congenital heart disease documented by cardiac catheterization, surgery, and/or postmortem examination were seen. 11 Among these were 2 cases of aortico-left ventricular tunnel. The purpose of this report is to describe these 2 patients, both female, who have had successful surgical repair of this defect and to stress the anatomy, pathophysiology, diagnostic features, and surgical consideration in this defect.
From the Departments of Cardiology and Cardiovascular Surgery, Children's Hospital National Medical Center, and The George Washington University Medical School, Department of Child Health and Development. Received for publication May 12, 1975. Address for reprints: James E. McClenathan, M.D., Department of Cardiovascular Surgery, Children's Hospital National Medical Center, Washington, D. C. 20009. 238
Case summaries CASE 1. C. T., a 2 '/2-year-old white girl, was referred for evaluation of a to-and-fro murmur that was first noted at age 4 days. Although she was asymptomatic, the peripheral pulses were bounding and clinical evaluation suggested aortic regurgitation. The blood pressure was 100/20 mm. Hg. The electrocardiogram (ECG) was abnormal, showing left ventricular hypertrophy with inverted T waves in the left precordial leads (Fig. 1). The chest roentgenograms showed a wide mediastinum and cardiomegaly (Fig. 2). Initial catheterization suggested a left coronary-left ventricular fistula. A repeat catheterization 5 months later, done because of onset of symptoms and progressive left ventricular enlargement on chest roentgenogram, demonstrated an aortico-left ventricular communication arising above and anterior to the right sinus of Valsalva. At operation this was confirmed. Evaluation 4 years after operation revealed a Grade 2/6 diastolic murmur considered to be due to minimal aortic valve regurgitation. The blood pressure was 104/60 mm. Hg. CASE 2. K. B. was a white girl who presented at birth with severe congestive heart failure (see Fig. 4/1). She was treated medically with good result. Following stabilization, a cardiac catheterization at age 3 days showed moderately severe aortic regurgitation through an aortico-left ventricular tunnel. She was discharged taking digoxin. A repeat catheterization at age 18 months confirmed the initial diagnosis. At this time, the blood pressure was 90/20 mm. Hg, a to-and-fro murmur with a diastolic thrill was noted, the ECG showed an abnormal QS pattern in the right precordial leads suggesting abnormal septal depolarization (Fig. 3), and the chest roentgenogram showed a prominent ascending aorta and left ventricular enlargement (Fig. AB). At age 2 years she underwent operative correction because of progressive congestive heart failure. In the immediate postoperative
Volume 71 Number 2 February, 1976
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Fig. 1. ECG's of C. T. (Case 1) (upper) preoperatively and (lower) 4 years postoperatively. The left ventricular hypertrophy noted preoperatively is no longer present. period she had recurrent episodes of supraventricular tachycardia which were controlled with digoxin. The blood pressure remained 100/60 mm. Hg. Although she has continued to do well postoperatively, she still has a Grade 2-3/6 diastolic murmur. A repeat catheterization 3 years after operation showed minimal aortic valve regurgitation, a dilated ascending aorta, and left ventricular enlargement (Fig. AC). Discussion Clinical profile. From a review of the literature, it appears that aortico-left ventricular tunnel is a congenital heart defect that usually presents in early infancy with a characteristic loud to-and-fro murmur. This has been demonstrated well by phonocardiograms.8' 9 Of the 20 reported cases, including our own 2 patients,
this characteristic murmur was first heard during the first week of life in 15 patients, and in another 4 it was heard first between 2 and 9 months. The clinical history was not available in 1 patient.10 Ejection clicks, although rare, may be present. Congestive heart failure develops frequently. Nine patients developed congestive heart failure within the first year of life; only 2 patients have been described as being completely asymptomatic by the time they were operated upon. Presumably, a 26-year-old man, the oldest patient thus far described,9 was also asymptomatic at the time of surgery. Cyanosis has been rare, having been described in only 3 patients,1, 5 2 of whom were in profound congestive heart failure. Fourteen of the 20 patients have been male.
240
The Journal of Thoracic and Cardiovascular Surgery
Okoroma et al.
presentation.6 Postoperatively, there has been usually a regression of the LVH on the ECG (Fig. 1). Chest roentgenograms. The chest roentgenogram is characteristic. Virtually all cases have shown a wide mediastinum resulting from dilatation of the ascending aorta and cardiomegaly with left ventricular configuration. A characteristic anterior indentation of the esophagus has been described2 and was present in both our patients (Fig. 5). Pulmonary vascularity has been normal or slightly increased (Figs. 2 and 4). Catheterization and angiography data. Catheterization data are available in 15 patients. Most have had normal right heart hemodynamics, the exceptions being those patients with significant right ventricular outflow obstruction. All have shown markedly wide aortic pulse pressures. None has had any intracardiac shunts. Aortic root angiograms are necessary to make the diagnosis. In 1 of our patients (Case 1) the diagnosis was not made during the initial study. The reason for failure to make the diagnosis was the view in which the angiograms were taken. Either a lateral or right anterior oblique aortic root angiogram is necessary to demonstrate the defect. In 1 of our patients, there was both antegrade and retrograde blood flow through the tunnel during systole and diastole, respectively. Postoperative catheterization data are available in only 2 patients, 1 of Somerville, Sughish, and Ross,9 and our Case 2. Both have mild aortic valve regurgitation. Our patient still has a persistently dilated ascending aorta (Fig. AC). Anatomy. The tunnel seems to be constant both in its origin above the right coronary sinus, separate and distinct from the right coronary artery orifice, and in its
ECG's. The ECG has been described in 17 of the 20 patients. Fifteen had left ventricular hypertrophy: 2 of these in addition had right ventricular hypertrophy. One of our patients had septal Q in the right precordial leads, indicating abnormal septal depolarization. Only 1 patient has had a normal ECG at the time of his initial
Fig. 2. Preoperative chest roentgenogram of C. T. (Case 1) which demonstrates wide mediastinum and left ventricular enlargement.
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Volume 71 Number 2 February, 1976
Aortico-left ventricular tunnel
24 1
Fig. 4C. Postoperative aortic root angiogram of K. B. (Case 2). The ascending aorta is still dilated 3 years later. Fig. 4A. Chest roentgenogram of K. B. (Case 2) at birth, showing wide mediastinum, cardiomegaly, and passive pulmonary congestion.
Table I. Associated cardiac defects in aortico-left ventricular tunnel Cardiac defects Bicuspid aortic valve1, *• 5l I0 Aortic valve stenosis1, *• 6 ' 10 Right ventricular outflow obstruction: Infundibular5, a Valvular1 Patent ductus arteriosus3 Absent right coronary artery9
Fig. 4B. Chest roentgenogram of K. B. (Case 2) at the age of 18 months.
No. of patients 4 4 2 1 1 1
ventricular entrance. The tunnel passes anteroinferiorly, is aneurysmal in size and shape, elevating the right ventricular outflow tract, and enters the left ventricle at a point high in the septum immediately beneath the annulus of the aortic valve (Fig. 6). The elevation of the outflow tract has been considered the cause of the right ventricular outflow tract obstruction in 2 patients.5' 8 One point not previously stressed is that the medial wall of the aneurysmal channel is within 2 to 3 mm. of the annulus of the aortic valve. In the 2 cases presented here, this fact was very evident at the time of surgery and this proximity appeared to compromise the integrity of the aortic valve. Histologic studies2, 3 ' 8 have shown the presence of elastic fibers in that portion of the tunnel near the aortic orifice which seemingly dissipate as the ventricular aspect is approached. At the ventricular end the tunnel wall is primarily collagen and endothelial lining. Surgical considerations. The surgical repair in the 2 cases presented was done with standard cardiopulmo-
The Journal of Thoracic and Cardiovascular Surgery
2 4 2 Okoroma et al.
Fig. 5. Chest roentgenograms with esophagogram demonstrating anterior indentation of the esophagus: A, C. T. (Case 1); B, K. B. (Case 2). nary bypass. After the body temperature has been lowered to 30° C , the aorta was cross-clamped. In Case 2 (K. B.) the tunnel was so large that it was incised transversely near its origin on the aorta, thus giving good access to the aortic aspect. The aortic origin was carefully closed with buttressed mattress sutures, reattaching the superior margin of the aortic annulus to the aortic wall. The fistula was then further opened inferiorly and the ventricular component exposed and this was similarly closed with buttressed mattress sutures to effectively close the communication near its entrance to the ventricular cavity. The central portion of the large communication was then obliterated by running sutures. In Case 1 (C. T.), in order to effect a more accurate closure of the aortic aspect, it was necessary to open the aorta superiorly to the coronary ostium. Since one margin of the aortic orifice of the tunnel approached within 3 mm. of the right coronary ostium, this was felt to be a safer method for repair of the aortic ostium. The ventricular and central portions of the tunnel were closed in a manner similar to that used in Case 2. In both cases the aortic valve was tricuspid; however, surgical closure of the aortic ostium of the tunnel in each case somewhat distorted the normal contour of the aortic valve. This may have contributed to the residual minimal aortic valve incompetence. Immediately postoperative pressure measurements
revealed no systolic gradient across the aortic valve. Diastolic aortic pressures rose from a preoperative average of 40 to 80 mm. Hg postoperatively. The pulse contour became normal. Comment Aortico-left ventricular tunnel is a rare congenital cardiac defect.2'4 Its incidence prior to this report is not known. Only 2 cases were observed among 1,754 cases of documented CHD over a 10 year period.11 The embryology is still obscure. While Levy and associates1 suggested that the tunnel represents an anomalous vessel, Somerville and colleagues9 believed that a disturbance in the development and incorporation of the distal bulbus cordis may be the cause. It would seem reasonable that this lesion is a developmental anomaly in which a weak spot in the aortic wall superior to the right coronary artery permits communication with ventricular sinusoids which are still in communication with the ventricular cavity. In time the tunnel then develops endothelial lining. Initially it was felt that associated defects were quite frequent 4 ' 5 ' 8 but Table I shows that aortico-left ventricular tunnel is seldom associated with other complex cardiac lesions. Aortic valve lesions are the most common among these. Although the clinical profile seems unique, it appears that the most distinguishing clinical feature of
Volume 71 Number 2 February, 1976
Aortico-left ventricular tunnel
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Fig. 6. Illustration of aortico-left ventricular tunnel (T) showing its aneurysmal size and, in the sagittal section, its relationship to the right coronary artery and the aortic valve. AOT, Aortic ostium of the tunnel. VOT, Ventricular ostium of the tunnel. RC, Right coronary artery. T, Tunnel. this lesion is the time of onset of symptoms. Although other cardiac defects such as aortic valve regurgitation, coronary artery fistula, ventricular septal defect with aortic regurgitation, and ruptured sinus of Valsalva may have similar clinical features, they produce symptoms much later in life. Aortico-left ventricular tunnel usually produces signs and symptoms in the newborn period or shortly therafter. Eighteen of the 19 patients with available clinical history presented with a characteristic to-and-fro murmur and/or congestive heart failure within the first 3 months of life. The early presentation of symptoms is most likely related to the large volume of blood regurgitation through the tunnel in these patients. Our 2 patients had 3+ to 4 + regurgitation through the tunnel on cineangiograms; however, despite this large volume, Case 1 was clinically asymptomatic. Finally, from a surgical standpoint it is necessary to close accurately the aortic aspect of this lesion with carefully buttressed sutures in such a manner as neither to induce major distortion of the anterior aortic cusps nor to compromise the right coronary orifice. Whether the ventricular aspect can be closed at the level of its entrance into the ventricular cavity is unanswered. Our feeling is that surgical manipulation at or near the level of its entrance into the ventricle may seriously disturb the conduction system and for that
reason this was not attempted in our cases. Rather, the closure of the ventricular aspect was performed approximately 0.5 to 1 cm. proximal to its entrance into the ventricle. Somerville and associates9 have proposed 5 years as the ideal age for surgery. In view of the high incidence of residual aortic valve regurgitation which has persisted in those patients surviving surgery (6 of 8 patients in whom it was commented upon), we would certainly recommend a much earlier repair. Because we feel that long-standing regurgitation through the tunnel with its attendant left ventricular dilatation contributes to dilatation of the aortic valve ring, and therefore to aortic regurgitation, surgical correction should be carried out as soon as the diagnosis is established. REFERENCES 1 Levy, M. J., Lillehei, C. W., Anderson, R. C , Amplatz, K., and Edwards, J. E.: Aortico-Left Ventricular Tunnel, Circulation 27: 841, 1963. 2 Morgan, R. I., and Mazur, J. H.: Congenital Aneurysm of Aortic Root With Fistula to Left Ventricle. A Case Report With Autopsy Findings, Circulation 28: 589, 1963. 3 Cooley, R. N., Harris, L. C , and Rodin, A. E.: Abnormal Communication Between the Aorta and Left Ventricle. Aortico-Left Ventricular Tunnel, Circulation 31: 564, 1965.
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4 Roberts, W. C , and Morrow, A. G.: Aortico-Left Ventricular Tunnel. A Case of Massive Aortic Regurgitation and of Intracardiac Aneurysms, Am. J. Med. 39: 662, 1965. 5 Bove, K. E., and Schwarts, D. C : Aortico-Left Ventricular Tunnel. A New Concept. Am. J. Cardiol. 19: 696, 1967. 6 Burnhard, W. F., Plantts, W., and 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., and Stangel, H. C : Aortico-Left Ventricular Tunnel, Radiology 98: 579, 1971.
The Journal of Thoracic and Cardiovascular Surgery
8 Perez-Martinez, V., Queno, M., Cesho, C , Moreno, F., Bito, J. M., and Merino, G.: Aortico-Left Ventricular Tunnel, Am. Heart J. 85: 237, 1973. 9 Somerville, J., Sughish, T., and Ross, D.: Aortico-Left Ventricular Tunnel. Clinical Features and Surgical Management, Br. Heart J. 36: 321, 1974. 10 Edwards, J. E.: Atlas of Acquired Diseases of the Heart and Great Vessels, Vol. II, Philadelphia, 1961, W. B. Saunders Company, p. 1142. 11 Perry, L. W., Scott, L. P., Shapiro, S. R., McClenathan, J. E., Chandra, R., and Walsh, B. J.: Documented Heart Disease at Children's Hospital National Medical Center, 1964-1974: Report of 1754 Cases, Clin. Proc. CHNMC 31: 73, 1975.