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Late development of left ventricular outflow tract obstruction after repair of double-outlet right ventricle
Late development of left ventricular outflow tract obstruction after repair of double-outlet right ventricle A 22-year-old woman had exertional chest pain and dyspnea secondary to left ventricular outflow tract obstruction more than a decade after repair of a type I double-outlet right ventricle. The obstruction was the result of (1) failure of the ventricular septal defect and the tunnel created between the left ventricle and aorta to enlarge commensurate with growth of the heart and (2) a markedly hypertrophied parietal band of the crista supraventricularis which surrounded and narrowed the aortic orifice. At reoperation, the ventricular septal defect and the tunnel were enlarged and the hypertrophied crista supraventricularis was partially resected. The patient is asymptomatic 6 months after operation.
Bernard R. Chaitman, M.D., Claude M. Grondin, M . D ..,, Pierre Theroux, M . D . , and Martial G. Bourassa, M.D., Montreal, Quebec, Canada
.L/ouble-outlet right ventricle is a rare cardiac anomaly frequently associated with other congenital heart defects. 1 ~3 In this condition, the ventricular septal defect represents the sole exit of blood from the left ventricle. Thus a small ventricular septal defect may result in significant obstruction to left ventricular outflow and affect the natural history of the disease both in operated and nonoperated p a t i e n t s . 4 6 In most cases, surgical repair is possible. 7 " 10 Late morbidity and deaths following repair of uncomplicated type I double-outlet right ventricle are related to the severity of preoperative pulmonary artery hypertension, to arrhythmias, and to residual shunts. 7 ' 8 Left ventricular outflow tract obstruction may occur either early 8 or late following surgical correction. The purpose of the present report is to describe the case of a 22-year-old woman in whom left ventricular outflow tract obstruction developed more than a decade following successful repair. She was reoperated upon for relief of the obstruction. From the Montreal Heart Institute and the Departments of Medicine and Surgery, Universite de Montreal Medical School, Montreal, Quebec. Canada. Supported by the Jean-Louis Levesque Foundation. Received for publication April 6, 1976. Accepted for publication May 27, 1976. Address for reprints: Martial G. Bourassa, M.D., Montreal Heart Institute, 5000 East Belanger Street, Montreal HIT 1C8, Quebec, Canada.
Case report The patient, now a 22-year-old woman, had had a right heart catheterization performed in March, 1960, because of failure to thrive, hypoxic episodes, and a heart murmur. Right ventricular, pulmonary artery, and brachial arterypressures were 75/5, 70/30 and 90/62 mm. Hg, respectively. Dye-dilution curves indicated a 45 per cent left-to-right shunt and a small 2 per cent right-to-left shunt at the ventricular level. Surgical correction of the defect was performed in July, 1960. At operation, both great vessels were found to arise from the right ventricle in a side-to-side manner. The aortic root was to the right of the pulmonary artery and there was an infracristal (subaortic) ventricular septal defect which measured 1.5 cm. An oval Teflon patch was placed between the lower margin of the ventricular septal defect and the right anterior aspect of the aortic root, with fixation of the prosthesis posteriorly near the tricuspid valve ring and anteriorly under the crista supraventricularis. Following repair, left and right ventricular pressures were 108/0 and 77/4 mm. Hg, respectively. The right brachial artery pressure by sphygmomanometry was 110 mm. Hg. The postoperative course was uneventful. Physical examination prior to discharge revealed a systolic thrill at the left border of the sternum and a Grade 5/6 systolic murmur maximal at the left sternal border.10 Three years later, physical findings were essentially unchanged from those noted early after the operation. At cardiac catheterization, right ventricular, pulmonary artery, femoral artery, and mean pulmonary capillary wedge pressures were 64/4, 66/28, 104/56, and 10 mm. Hg, respectively. The cardiac index was 5.8 L. per minute per square meter. Dye curves showed a 20 per cent left-to-right shunt at the ventricular level. The remainder of her adolescence was uneventful with no limitation of activity. 265
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Fig. 1. A, Left ventriculogram, right anterior oblique view. There is a subaortic stenosis (St) between the aorta (Ao) and left ventricle (LV). B, Left ventriculogram, left lateral view. Injection of contrast medium into the left ventricular (LV) outflow tract shows subaortic stenosis (St) and opacification of the right ventricle (RV) through a ventricular septal defect. The small VSD is at the upper limit of the patch (P) and the aorta (Ao) is displaced anteriorly and superiorly. The aortic and mitral valves (MV) are separated by the parietal band of the crista supraventricularis. In 1973, she experienced two brief episodes of confusion associated with fatigue and dizziness. During the next 2 years she noted exertional chest pain and a progressive decrease in exercise tolerance. For these reasons, she was hospitalized for evaluation. Physical examination revealed a systolic thrill maximal at the fourth left intercostal space, a diminished second heart sound, and a Grade 6/6 holosystolic murmur maximal at the fourth left intercostal space. There was no cyanosis or clubbing. An electrocardiogram and vectorcardiogram showed right bundle branch block. The frontal axis was +90 degrees. Roentgenograms of the chest disclosed mild enlargement of the right heart chambers and calcification of the Teflon patch. An echocardiogram revealed anterior aortic displacement, diastolic fluttering of the mitral valve, and thickness of the posterior left ventricular wall which was at the upper limits of normal. At cardiac catheterization, right ventricular, pulmonary artery, and mean pulmonary capillary wedge pressures of 80/10, 80/30, and 10 mm Hg, respectively, were recorded. Retrograde catheterization of the left ventricle revealed left ventricular and ascending aortic pressures of 255/10 and 110/70 mm. Hg, respectively. Dye curves showed a 25 per cent left-to-right shunt at the ventricular level. The left ventriculogram demonstrated subvalvular aortic stenosis and a small residual ventricular septal defect (Fig. 1). The right ventriculogram was within normal limits. A supravalvular aortogram demonstrated mild aortic regurgitation and a tricuspid aortic valve. At operation in June of 1975, the Teflon patch, which was
partially calcified and covered with a thick layer of fibrous tissue, was resected. The surgeon's index finger (approximately 1.5 cm.) could not be introduced through either the aortic orifice or the ventricular septal defect. The latter was enlarged by incising its anterior rim obliquely toward the pulmonary valve. The narrowed aortic orifice was secondary to a hypertrophied parietal band of the crista supraventricularis (Fig. 2). The parietal band of the crista supraventricularis in double-outlet right ventricle divides into an anterior limb, which surrounds the anterior half of the aortic orifice, and a posterior limb, which accounts for the discontinuity between the aortic and mitral valves. Copious amounts of muscle were resected from both limbs of the parietal band so that the diameters of the ventricular septal defect and the subaortic orifice were approximately equal (2.5 cm.) to that of the ascending aorta. A Teflon patch was then sewn into place to bridge the gap between the ventricular septal defect and the aorta, as is done in repair of type I double-outlet right ventricle.9 Prior to closure of the chest, simultaneously measured systolic left ventricular and aortic pressures were 125 mm. Hg. Six months postoperatively, the patient was asymptomatic. Cardiac examination revealed a Grade 3/6 systolic murmur maximal at the third left intercostal space, a Grade 2/6 short decrescendo diastolic murmur which commenced with the second heart sound, and a loud pulmonic closure sound. A carotid pulse tracing was within normal limits except for a slightly prolonged left ventricular ejection time. Echocardiographic findings remained unchanged.
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© Fig. 2. Anatomic relations. A, The aorta (Ao) and pulmonary artery (PA) originate from the right ventricle (RV). The ventricular septal defect (VSD) is located below the crista supraventricularisfCSKJ. B, In this magnified view of the subaortic region, the anterior and posterior limbs of the parietal band of the CSV are seen encircling the Ao. The mitral valve (MV) is visualized through the VSD and is separated from the Ao by the posterior limb of the parietal band of the CSV. The aortic orifice and the VSD were enlarged at operation.
Discussion Pathological and clinical features of double-outlet right ventricle have been well described. 1-3 This defect can be repaired in most patients with this anomaly.7-10 In 18 patients without coexistent pulmonic stenosis, Gomes and co-workers8 have reported an operative mortality rate of 22 per cent. Follow-up over an average of 6 years revealed a late mortality rate of 29 per cent. Severe systemic pulmonary hypertension was frequent in those who died early or late after operation. The patient presented herein was operated upon at the age of 7 years. The pulmonary artery pressure, which was elevated before operation, remained significantly increased 15 years postoperatively. It is unlikely that the small recurrent or residual ventricular septal defect could account for the persistent pulmonary hypertension. For these reasons, Kirklin7 and others8 recommend operation at an early age prior to the development of irreversible pulmonary hypertension. Postoperative amelioration of symptoms is frequent.8, 9 Late morbidity is related to severe pulmonary hypertension, arrhythmias, residual shunts, and incomplete correction of associated congenital cardiac defects. 7-9 Recur-
rent or residual left-to-right shunts are not infrequent following closure of a complex ventricular septal defect with a prosthesis, and they usually occur at the superior attachment of the patch.11 Our patient had a small left-to-right ventricular shunt which was documented 3 years following the initial repair and which did not change significantly over the ensuing 12 years. The loud cardiac murmur which persisted throughout childhood and adolescence were attributed to the small ventricular septal defect. The initial operation offered excellent symptomatic relief and allowed the patient to reach a normal adult height and weight. The size of the left ventricular outflow tract in double-outlet right ventricle is in great part dependent upon the size of the ventricular septal defect. A small septal defect can result in significant obstruction.4-6 Reconstruction of the left ventricular outflow tract may involve enlargement of the septal defect and/or resection of excess muscle bundles surrounding the aortic root.8 Occasionally, a small residual gradient between the left ventricle and aorta will persist or develop in the early postoperative period.8 The patient presented herein did not require enlargement of the ventricular
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septal defect at the initial operation, as there was no left ventricular gradient early after repair. The late development of severe left ventricular outflow tract obstruction more than a decade postoperatively was suggested by the appearance of chest pain and pre-syncopal attacks. The location and severity of obstruction were documented by left heart catheterization and angiographic study. The severity of obstruction was not suspected from the electrocardiogram or vectorcardiogram, both showing right bundle branch block, nor from the chest roentgenograms. Although left ventricular outflow tract obstruction has been described preoperatively 4 ~ 6 and early after repair of double-outlet right ventricle, 8 there are no reports of late development of this condition. The cause of the obstruction was believed to be twofold: (1) growth of the heart without concomitant increase in the size of the ventricular septal defect and tunnel created between the left ventricle and aorta and (2) markedly hypertrophied crista supraventricularis which surrounded the aortic orifice. The second surgical intervention involved partial resection of the hypertrophied crista supraventricularis, enlargement of the ventricular septal defect, and replacement of the prosthetic patch. Echocardiographic features of double-outlet right ventricle include displacement of the anterior aortic wall from the interventricular septum and the demonstration of mitral-semilunar valve displacement. 12 The echocardiogram in our patient revealed these findings and in addition showed diastolic fluttering of the mitral valve, indicative of aortic insufficiency. The mild aortic insufficiency demonstrated by echocardiograms and angiograms before operation persisted after operation in our patient. Various causes of aortic insufficiency in patients with double-outlet right ventricle include bacterial endocarditis, 13 bicuspid aortic valve, 14 and herniation of an aortic cusp into the ventricular septal defect. 15 The patient presented in this report had none of these findings, and the cause of her aortic insufficiency remains obscure. The late development of left ventricular outflow tract obstruction is a distinct possibility in patients who have had surgical correction of type I double-outlet right ventricle, because of the anatomic relationships of this condition after repair and because of the presence of a subaortic conus. 1 6 These patients should be followed closely and should undergo repeat left heart catheterization when necessary. We are indebted to Drs. Ihor Dyrda and Andre Pasternac for performing the cardiac catheterizations. REFERENCES 1 Neufeld, H. N., DuShane, J. W., Wood, E. H., Kirklin. J. W., and Edwards, J. E.: Origin of Both Great Vessels
The Journal of Thoracic and Cardiovascular Surgery
From the Right Ventricle. I. Without Pulmonary Stenosis, Circulation 23: 399, 1961. 2 Lev, M., Bharati, S., Meng, C. L., Liberthson, R. R., Paul, M. H., and Idriss, F.: A Concept of Double-Outlet Right Ventricle, J. THORAC. CARDIOVASC. SURG. 64:
271, 1972. 3 Neufeld, H.N., Lucas, R. V., Jr., Lester, R. G., Adams. P., Jr., Anderson, R. C , and Edwards, J. E.: Origin of Both Great Vessels From the Right Ventricle Without Pulmonary Stenosis, Br. Heart J. 24: 393, 1962. 4 Lavoie, R., Sestier, F., Gilbert, G., Chameides, L., Van Praagh, R., and Grondin, P.: Double Outlet Right Ventricle With Left Ventricular Outflow Tract Obstruction Due to Small Ventricular Septal Defect, Am. Heart J. 82: 290, 1971. 5 Serratto, M., Arevalo, F., Goldman, E. J., Hastreiter. A., and Miller, R. A.: Obstructive Ventricular Septal Defect in Double Outlet Right Ventricle, Am. J. Cardiol. 19: 457, 1967. 6 Mason, D. T., Morrow, A. G., Elkins, R. C , and Friedman, W. F.: Origin of Both Great Vessels From the Right Ventricle Associated With Severe Obstruction to Left Ventricular Outflow, Am. J. Cardiol. 24: 1 18. 1969. 7 Kirklin, J. W.: Evaluating the Results of CardiacSurgery, Circulation 48: 232, 1973. 8 Gomes, M. M. R., Weidman, W. H., McGoon, D. C . and Danielson, G. K.: Double-Outlet Right Ventricle Without Pulmonic Stenosis, Circulation 43, 44: 31, 1971 (Suppl. I). 9 Kirklin, J. W., Harp, R. A., and McGoon. D. C : Surgical Treatment of Origin of Both Vessels From Right Ventricle, Including Cases of Pulmonary Stenosis. J. THORAC. CARDIOVASC. SURG. 48: 1026,
1964.
10 McGoon, D. C : Origin of Both Great Vessels From the Right Ventricle, Surg. Clin. North Am. 41: 1113, 1961. 11 Gersony, W. M., and Krongrad, E.: Evaluation and Management of Patients After Surgical Repair of Congenital Heart Diseases, Progr. Cardiovasc. Dis. 18: 39, 1975. 12 French, J. W., and Popp, R.: Variability of Echocardiographic Discontinuity in Double Outlet Right Ventricle and Truncus Arteriosus, Circulation 51: 848. 1975. 13 Engle, M. A., Steinberg, I., Lukas. D. S., and Goldberg, H. P.: Acyanotic Ventricular Septal Defect With Both Great Vessels From the Right Ventricle, Am. Heart J. 66: 755, 1963. 14 Levy, M. J., Dewall, R., Elliott, L. P., and Cuello. L.: Origin of Both Great Arteries From the Right Ventricle and Pulmonic Stenosis, Chest 42: 372, 1962. 15 Khattri, H. N., Misra, K. P., and Dutta, B. N.: Double Outlet Right Ventricle With Long Survival, Br. Heart J. 30: 569, 1968. 16 Van Praagh, R., and Van Praagh, S.: Isolated Ventricular Inversion With a Consideration of the Morphogenesis. Definition and Diagnosis of Nontransposed and Transposed Great Arteries, Am. J. Cardiol. 17: 395. 1966.
Bernard R. Chaitman, M.D., Claude M. Grondin, M . D ..,, Pierre Theroux, M . D . , and Martial G. Bourassa, M.D., Montreal, Quebec, Canada
.L/ouble-outlet right ventricle is a rare cardiac anomaly frequently associated with other congenital heart defects. 1 ~3 In this condition, the ventricular septal defect represents the sole exit of blood from the left ventricle. Thus a small ventricular septal defect may result in significant obstruction to left ventricular outflow and affect the natural history of the disease both in operated and nonoperated p a t i e n t s . 4 6 In most cases, surgical repair is possible. 7 " 10 Late morbidity and deaths following repair of uncomplicated type I double-outlet right ventricle are related to the severity of preoperative pulmonary artery hypertension, to arrhythmias, and to residual shunts. 7 ' 8 Left ventricular outflow tract obstruction may occur either early 8 or late following surgical correction. The purpose of the present report is to describe the case of a 22-year-old woman in whom left ventricular outflow tract obstruction developed more than a decade following successful repair. She was reoperated upon for relief of the obstruction. From the Montreal Heart Institute and the Departments of Medicine and Surgery, Universite de Montreal Medical School, Montreal, Quebec. Canada. Supported by the Jean-Louis Levesque Foundation. Received for publication April 6, 1976. Accepted for publication May 27, 1976. Address for reprints: Martial G. Bourassa, M.D., Montreal Heart Institute, 5000 East Belanger Street, Montreal HIT 1C8, Quebec, Canada.
Case report The patient, now a 22-year-old woman, had had a right heart catheterization performed in March, 1960, because of failure to thrive, hypoxic episodes, and a heart murmur. Right ventricular, pulmonary artery, and brachial arterypressures were 75/5, 70/30 and 90/62 mm. Hg, respectively. Dye-dilution curves indicated a 45 per cent left-to-right shunt and a small 2 per cent right-to-left shunt at the ventricular level. Surgical correction of the defect was performed in July, 1960. At operation, both great vessels were found to arise from the right ventricle in a side-to-side manner. The aortic root was to the right of the pulmonary artery and there was an infracristal (subaortic) ventricular septal defect which measured 1.5 cm. An oval Teflon patch was placed between the lower margin of the ventricular septal defect and the right anterior aspect of the aortic root, with fixation of the prosthesis posteriorly near the tricuspid valve ring and anteriorly under the crista supraventricularis. Following repair, left and right ventricular pressures were 108/0 and 77/4 mm. Hg, respectively. The right brachial artery pressure by sphygmomanometry was 110 mm. Hg. The postoperative course was uneventful. Physical examination prior to discharge revealed a systolic thrill at the left border of the sternum and a Grade 5/6 systolic murmur maximal at the left sternal border.10 Three years later, physical findings were essentially unchanged from those noted early after the operation. At cardiac catheterization, right ventricular, pulmonary artery, femoral artery, and mean pulmonary capillary wedge pressures were 64/4, 66/28, 104/56, and 10 mm. Hg, respectively. The cardiac index was 5.8 L. per minute per square meter. Dye curves showed a 20 per cent left-to-right shunt at the ventricular level. The remainder of her adolescence was uneventful with no limitation of activity. 265
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Fig. 1. A, Left ventriculogram, right anterior oblique view. There is a subaortic stenosis (St) between the aorta (Ao) and left ventricle (LV). B, Left ventriculogram, left lateral view. Injection of contrast medium into the left ventricular (LV) outflow tract shows subaortic stenosis (St) and opacification of the right ventricle (RV) through a ventricular septal defect. The small VSD is at the upper limit of the patch (P) and the aorta (Ao) is displaced anteriorly and superiorly. The aortic and mitral valves (MV) are separated by the parietal band of the crista supraventricularis. In 1973, she experienced two brief episodes of confusion associated with fatigue and dizziness. During the next 2 years she noted exertional chest pain and a progressive decrease in exercise tolerance. For these reasons, she was hospitalized for evaluation. Physical examination revealed a systolic thrill maximal at the fourth left intercostal space, a diminished second heart sound, and a Grade 6/6 holosystolic murmur maximal at the fourth left intercostal space. There was no cyanosis or clubbing. An electrocardiogram and vectorcardiogram showed right bundle branch block. The frontal axis was +90 degrees. Roentgenograms of the chest disclosed mild enlargement of the right heart chambers and calcification of the Teflon patch. An echocardiogram revealed anterior aortic displacement, diastolic fluttering of the mitral valve, and thickness of the posterior left ventricular wall which was at the upper limits of normal. At cardiac catheterization, right ventricular, pulmonary artery, and mean pulmonary capillary wedge pressures of 80/10, 80/30, and 10 mm Hg, respectively, were recorded. Retrograde catheterization of the left ventricle revealed left ventricular and ascending aortic pressures of 255/10 and 110/70 mm. Hg, respectively. Dye curves showed a 25 per cent left-to-right shunt at the ventricular level. The left ventriculogram demonstrated subvalvular aortic stenosis and a small residual ventricular septal defect (Fig. 1). The right ventriculogram was within normal limits. A supravalvular aortogram demonstrated mild aortic regurgitation and a tricuspid aortic valve. At operation in June of 1975, the Teflon patch, which was
partially calcified and covered with a thick layer of fibrous tissue, was resected. The surgeon's index finger (approximately 1.5 cm.) could not be introduced through either the aortic orifice or the ventricular septal defect. The latter was enlarged by incising its anterior rim obliquely toward the pulmonary valve. The narrowed aortic orifice was secondary to a hypertrophied parietal band of the crista supraventricularis (Fig. 2). The parietal band of the crista supraventricularis in double-outlet right ventricle divides into an anterior limb, which surrounds the anterior half of the aortic orifice, and a posterior limb, which accounts for the discontinuity between the aortic and mitral valves. Copious amounts of muscle were resected from both limbs of the parietal band so that the diameters of the ventricular septal defect and the subaortic orifice were approximately equal (2.5 cm.) to that of the ascending aorta. A Teflon patch was then sewn into place to bridge the gap between the ventricular septal defect and the aorta, as is done in repair of type I double-outlet right ventricle.9 Prior to closure of the chest, simultaneously measured systolic left ventricular and aortic pressures were 125 mm. Hg. Six months postoperatively, the patient was asymptomatic. Cardiac examination revealed a Grade 3/6 systolic murmur maximal at the third left intercostal space, a Grade 2/6 short decrescendo diastolic murmur which commenced with the second heart sound, and a loud pulmonic closure sound. A carotid pulse tracing was within normal limits except for a slightly prolonged left ventricular ejection time. Echocardiographic findings remained unchanged.
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© Fig. 2. Anatomic relations. A, The aorta (Ao) and pulmonary artery (PA) originate from the right ventricle (RV). The ventricular septal defect (VSD) is located below the crista supraventricularisfCSKJ. B, In this magnified view of the subaortic region, the anterior and posterior limbs of the parietal band of the CSV are seen encircling the Ao. The mitral valve (MV) is visualized through the VSD and is separated from the Ao by the posterior limb of the parietal band of the CSV. The aortic orifice and the VSD were enlarged at operation.
Discussion Pathological and clinical features of double-outlet right ventricle have been well described. 1-3 This defect can be repaired in most patients with this anomaly.7-10 In 18 patients without coexistent pulmonic stenosis, Gomes and co-workers8 have reported an operative mortality rate of 22 per cent. Follow-up over an average of 6 years revealed a late mortality rate of 29 per cent. Severe systemic pulmonary hypertension was frequent in those who died early or late after operation. The patient presented herein was operated upon at the age of 7 years. The pulmonary artery pressure, which was elevated before operation, remained significantly increased 15 years postoperatively. It is unlikely that the small recurrent or residual ventricular septal defect could account for the persistent pulmonary hypertension. For these reasons, Kirklin7 and others8 recommend operation at an early age prior to the development of irreversible pulmonary hypertension. Postoperative amelioration of symptoms is frequent.8, 9 Late morbidity is related to severe pulmonary hypertension, arrhythmias, residual shunts, and incomplete correction of associated congenital cardiac defects. 7-9 Recur-
rent or residual left-to-right shunts are not infrequent following closure of a complex ventricular septal defect with a prosthesis, and they usually occur at the superior attachment of the patch.11 Our patient had a small left-to-right ventricular shunt which was documented 3 years following the initial repair and which did not change significantly over the ensuing 12 years. The loud cardiac murmur which persisted throughout childhood and adolescence were attributed to the small ventricular septal defect. The initial operation offered excellent symptomatic relief and allowed the patient to reach a normal adult height and weight. The size of the left ventricular outflow tract in double-outlet right ventricle is in great part dependent upon the size of the ventricular septal defect. A small septal defect can result in significant obstruction.4-6 Reconstruction of the left ventricular outflow tract may involve enlargement of the septal defect and/or resection of excess muscle bundles surrounding the aortic root.8 Occasionally, a small residual gradient between the left ventricle and aorta will persist or develop in the early postoperative period.8 The patient presented herein did not require enlargement of the ventricular
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septal defect at the initial operation, as there was no left ventricular gradient early after repair. The late development of severe left ventricular outflow tract obstruction more than a decade postoperatively was suggested by the appearance of chest pain and pre-syncopal attacks. The location and severity of obstruction were documented by left heart catheterization and angiographic study. The severity of obstruction was not suspected from the electrocardiogram or vectorcardiogram, both showing right bundle branch block, nor from the chest roentgenograms. Although left ventricular outflow tract obstruction has been described preoperatively 4 ~ 6 and early after repair of double-outlet right ventricle, 8 there are no reports of late development of this condition. The cause of the obstruction was believed to be twofold: (1) growth of the heart without concomitant increase in the size of the ventricular septal defect and tunnel created between the left ventricle and aorta and (2) markedly hypertrophied crista supraventricularis which surrounded the aortic orifice. The second surgical intervention involved partial resection of the hypertrophied crista supraventricularis, enlargement of the ventricular septal defect, and replacement of the prosthetic patch. Echocardiographic features of double-outlet right ventricle include displacement of the anterior aortic wall from the interventricular septum and the demonstration of mitral-semilunar valve displacement. 12 The echocardiogram in our patient revealed these findings and in addition showed diastolic fluttering of the mitral valve, indicative of aortic insufficiency. The mild aortic insufficiency demonstrated by echocardiograms and angiograms before operation persisted after operation in our patient. Various causes of aortic insufficiency in patients with double-outlet right ventricle include bacterial endocarditis, 13 bicuspid aortic valve, 14 and herniation of an aortic cusp into the ventricular septal defect. 15 The patient presented in this report had none of these findings, and the cause of her aortic insufficiency remains obscure. The late development of left ventricular outflow tract obstruction is a distinct possibility in patients who have had surgical correction of type I double-outlet right ventricle, because of the anatomic relationships of this condition after repair and because of the presence of a subaortic conus. 1 6 These patients should be followed closely and should undergo repeat left heart catheterization when necessary. We are indebted to Drs. Ihor Dyrda and Andre Pasternac for performing the cardiac catheterizations. REFERENCES 1 Neufeld, H. N., DuShane, J. W., Wood, E. H., Kirklin. J. W., and Edwards, J. E.: Origin of Both Great Vessels
The Journal of Thoracic and Cardiovascular Surgery
From the Right Ventricle. I. Without Pulmonary Stenosis, Circulation 23: 399, 1961. 2 Lev, M., Bharati, S., Meng, C. L., Liberthson, R. R., Paul, M. H., and Idriss, F.: A Concept of Double-Outlet Right Ventricle, J. THORAC. CARDIOVASC. SURG. 64:
271, 1972. 3 Neufeld, H.N., Lucas, R. V., Jr., Lester, R. G., Adams. P., Jr., Anderson, R. C , and Edwards, J. E.: Origin of Both Great Vessels From the Right Ventricle Without Pulmonary Stenosis, Br. Heart J. 24: 393, 1962. 4 Lavoie, R., Sestier, F., Gilbert, G., Chameides, L., Van Praagh, R., and Grondin, P.: Double Outlet Right Ventricle With Left Ventricular Outflow Tract Obstruction Due to Small Ventricular Septal Defect, Am. Heart J. 82: 290, 1971. 5 Serratto, M., Arevalo, F., Goldman, E. J., Hastreiter. A., and Miller, R. A.: Obstructive Ventricular Septal Defect in Double Outlet Right Ventricle, Am. J. Cardiol. 19: 457, 1967. 6 Mason, D. T., Morrow, A. G., Elkins, R. C , and Friedman, W. F.: Origin of Both Great Vessels From the Right Ventricle Associated With Severe Obstruction to Left Ventricular Outflow, Am. J. Cardiol. 24: 1 18. 1969. 7 Kirklin, J. W.: Evaluating the Results of CardiacSurgery, Circulation 48: 232, 1973. 8 Gomes, M. M. R., Weidman, W. H., McGoon, D. C . and Danielson, G. K.: Double-Outlet Right Ventricle Without Pulmonic Stenosis, Circulation 43, 44: 31, 1971 (Suppl. I). 9 Kirklin, J. W., Harp, R. A., and McGoon. D. C : Surgical Treatment of Origin of Both Vessels From Right Ventricle, Including Cases of Pulmonary Stenosis. J. THORAC. CARDIOVASC. SURG. 48: 1026,
1964.
10 McGoon, D. C : Origin of Both Great Vessels From the Right Ventricle, Surg. Clin. North Am. 41: 1113, 1961. 11 Gersony, W. M., and Krongrad, E.: Evaluation and Management of Patients After Surgical Repair of Congenital Heart Diseases, Progr. Cardiovasc. Dis. 18: 39, 1975. 12 French, J. W., and Popp, R.: Variability of Echocardiographic Discontinuity in Double Outlet Right Ventricle and Truncus Arteriosus, Circulation 51: 848. 1975. 13 Engle, M. A., Steinberg, I., Lukas. D. S., and Goldberg, H. P.: Acyanotic Ventricular Septal Defect With Both Great Vessels From the Right Ventricle, Am. Heart J. 66: 755, 1963. 14 Levy, M. J., Dewall, R., Elliott, L. P., and Cuello. L.: Origin of Both Great Arteries From the Right Ventricle and Pulmonic Stenosis, Chest 42: 372, 1962. 15 Khattri, H. N., Misra, K. P., and Dutta, B. N.: Double Outlet Right Ventricle With Long Survival, Br. Heart J. 30: 569, 1968. 16 Van Praagh, R., and Van Praagh, S.: Isolated Ventricular Inversion With a Consideration of the Morphogenesis. Definition and Diagnosis of Nontransposed and Transposed Great Arteries, Am. J. Cardiol. 17: 395. 1966.