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Tetralogy of Fallot with rheumatic stenosis of the aortic valve
Case Reports
Tetralogy
of Fallot With
Rh eumatic
Stenosis of the Aortic
N. Perryman Collins, M.D., Andrew G. M&row, Eugene Braunwald, M.D., Bethesda, Md.
Valve
M.D., and
The incidence of rheumatic valvulitis in patients with congenital heart disease is difficult to ascertain. In patients having congenitally bicuspid aortic valves, the incidence of all rheumatic infections of the heart has been variously reported from 16 to 62 per cent,‘J and in those with atria1 septal defects it has been as high as 13 per cent.3 Gelfman and Levine,4 in examining 181 autopsy cases of congenital cardiac malformations, found 2.5 (14 per cent) in which there was evidence of superimposed rheumatic infection. One was a patient with tetralogy of Fallot but no details were given as to the specific rheumatic lesion in the heart. Since their study, little has been written concerning the occurrence of rheumatic heart disease in patients with congenital defects. A patient recently studied at the National Heart Institute was shown to have the tetralogy of Fallot and calcific aortic stenosis of rheumatic origin. To our knowledge, no description of a patient with these combined lesions has been recorded, and in view of the sparsity of information on the subject a description of the clinical, hemodynamic, and anatomic findings in this patient was considered worth while. CASE
REPORT
F. P. (No. 02-lo-20), a 25year-old female student, was admitted to the National Heart Institute on Aug. 17, 1958. Cyanosis and a heart murmur were known to have been present since birth. She had always had exertional dyspnea and was said to have squatted to rest during childhood. The history also revealed that at 4, 7, and 12 years of age she had had attakcs of joint effusions with pain associated with fever and an elevated erythrocyte sedimentation rate. On each occasion the diagnosis of acute rheumatic fever was made and she was hospitalized. The patient’s dyspnea was more severe after the last such illness. Ten years prior to admission an attempt had been made, at another hospital, to perform a right subclavian-pulmonary artery anastomosis but the procedure was abandoned because of the small size of the right pulmonary artery. She had remained on almost complete bed rest for 10 years, and for the 2 months immediately prior to admission had been hospitalized because of marked dyspnea. From the Clinic of Surgery, National Heart Received for publication Feb. 1, 1960.
Institute, 624
Bethesda.
Md.
E-L%: “4”
TETRALOGY
OF FALLOT
WITH
RHEUMATIC
AORTIC
STENOSIS
625
Physical examination revealed the patient to be well developed and slightly obese; there was marked generalized cyanosis. The blood pressure was 106/75 mm. Hg and the pulse 104. A healed right posterolateral thoracotomy incision was present. A left ventricular tap and a pronounced right ventricular lift were palpable over the precordium. A Grade 4/6 ejection type of systolic murmur was audible over the entire precordium but was maximal at the base of the heart and was transmitted to the carotid vessels. A Grade 2/6 murmur was also present over the entire thorax during diastole, and the two murmurs were considered to be continuous. There was a single second heart sound at the base. Normal sinus rhythm was present and the peripheral arterial pulsations were normal. There was marked clubbing and cyanosis of all digits. Routine laboratory examinations were normal except that the hemoglobin was elevated to 22.8 Gm. per 100 ml. and the hematocrit was 69 per cent. Fluoroscopic and radiographic examinations revealed right ventricular enlargement, decreased pulmonary vascularity, and calcification in the region of the aortic valve (Fig. 1). The electrocardiogram demonstrated right axis deviation, right atrial enlargement, and right ventricular hypertrophy.
Fig. I.-Posterior-anterior (A) and lateral (B) projections of the chest x-ray. The heart is grossly enlarged, particularly the right ventricle, and no main pulmonary artery is visible. Pulmonary vascularity is decreased. The ring of calcification in the region of the aortic valve is indicated by arrows in the lateral view. Right heart catheterization was carried out on Aug. 18, 19.58. The pressure in the right ventricle was 160/S mm. Hg, and in the brachial artery it was 90/63 mm. Hg. Arterial indicatordilution curves recorded after right atrial and right ventricular injection revealed a large right-toleft shunt which originated from the right ventricle. The peripheral arterial. saturation was 61 per cent. The nitrous-oxide index6 was 0 per cent in the right atrium and 100 per cent in the right ventricle; the latter value indicated that the catheter had sampled left ventricular blood immediately beneath the aortic valve. The catheter could not be manipulated into the pulmonary artery or aorta. A selective angiocardiogram with right ventricular injection revealed a large ventricular septal defect and a large single vessel arising from the base of the heart (Fig. 2). Two ventricles were demonstrated but the pulmonary arteries were never opacified. The angiocardiographic findings were thought to indicate pulmonary atresia (pseudotruncus arteriosus) or a severe form of tetralogy of Fallot. Since the ventricles were functioning with a common ejectile force, the systolic pressure gradient of 70 mm. Hg between the right ventricle and brachial artery indicated the presence of associated aortic stenosis. To confirm this diagnosis and to localize the site of outflow obstruction, a retrograde arterial catheterization was performed. The catheter entered
626
COLLINS,
MORROW,
AND BRAUNWXLD
the left ventricle from the aorta, and the pressures recorded as it was withdrawn valve proved the presence of valvular aortic stenosis (Fig. 3).
Am. Heart J. October, 1960 across the aortic
Because of the severe deformity of the right ventricular outflow tract and the absence of pulmonary arterial opacification demonstrated by the angiocardiogram, the patient’s lesions were not considered to be amenable to complete correction. Therefore, a systemic pulmonary anastomosis and aortic valvulotomy were decided upon. At the time of operation it was clear that the congenital lesion was a severe form of the tetralogy of Fallot; there was marked dextroposition of the aorta, and the main pulmonary artery was hypoplastic. There was an intense systolic thrill in the ascending aorta, which was dilated. The left pulmonary artery was small, but it was possible to perform an anastomosis between the end of the left subclavian artery and the side of the left pulmonary artery. After the anastomosis had been opened, an aortic valvulotomy was carried out by closed transventricular dilatation. Measurements of ventricular and systemic arterial pressures before and after the valvulotomy revealed almost complete abolition of the pressure gradient across the aortic valve. In the immediate postoperative period the patient was hypotensive and refractory to the administration of blood and vasopressor agents. Approximately 2% hours after the conclusion of the operation, cardiac arrest occurred and resuscitation was unsuccessful.
Fig. 2.-Anteroposterior (A) and lateral (B) views of the selective angiocardiogram. The injection was made into the right ventricle. Both the right ventricle (R V) and left ventricle (LV) are filled, and the dilated aorta (Ao) is seen in both views. The outflow tract of the right ventricle appears to end blindly (arrow), and the pulmonary arteries do not fill. Pathologic Findings.-The heart weighed 700 grams, there was hypertrophy of both ventricles, and the ventricular cavities were small. There was severe stenosis of the right ventricular infundibulum, which was due to myocardial and endocardial thickening; the opening in the outflow was only 1 cm. in diameter. The pulmonic valve had three cusps, was stenotic as well as hypoplastic, and measured 3 cm. in circumference. The subclavian-pulmonary artery anastomosis was patent. A defect in the membranous portion of the ventricular septum was present; the mitral ring was calcified. The aorta was dextroposed, and there was dilatation of the ascending aorta. The aortic valve had three normally situated cusps. There was marked calcification of the aortic annulus and cusps, and there were numerous calcific deposits on the aortic side of the leaflets and to a lesser extent on their ventricular side (Fig. 4). The valve had little or no mobility. The right and left coronary cusps were fused near the ring; the other commissures showed evidence of having been recently opened.
Volume 60 Number 4
TETRALOGY
OF FALLOT
WITH
RHEI’MATIC
AORTIC
STENOSIS
+I.0 sec.3
Fig. 3.-Pressures recorded during retrograde arterial catheterization as the catheter was withdrawn from the left ventricle into the aorta. A peak systolic gradient of 40 to 50 mm. Hg is evident,. The patient was anesthetized during this study.
Fig. 4.-The appearance of the heart and great vessels at autopsy. The dextroposition of the aorta and the diminutive main and left pulmonary arteries are shown. The site of the patent left subclavisn-pulmonary anastomosis and the extreme dilatation of the aorta are also evident. The relations of the calcified aortic valve to the ventricular septum are shown in the insert.
628
COLLINS,
MORROW,
AND BRAUNWALD
Am. Heart J. October, 1960
Microscopic examination of the aortic valve showed it to be greatly thickened and to contain lobulated areas of calcium surrounded by dense fibrous tissue and focally dense chronic inflammatory cellular infiltrates (Fig. 5). The valve was prominently vascularized. Other histologic examinations revealed that the mitral valve was thickened and there was fibrous tissue as well as many thick-walled vessels deep in its substance. There were widely scattered focal areas of scarring in the myocardium of both ventricles and extensive endocardial calcification.
Fig. 5.-Photomicrograph of a vertical section through an aortic leaflet. The leaflet (AL) is extensively infiltrated with fibrous tissue and chronic inflammatory cells. The clear areas represent sites in which calcium was present. Numerous vascular channels are evident in the base of the leaflet. (Hematoxylin and eosin; X 14, reduced %.) COMMENT
There can be little doubt regarding the cause of the aortic stenosis in this patient. The episodes of acute rheumatic fever were well documented and the presence of chronic inflammation in both the mitral and aortic valves provided anatomic proof of rheumatic disease. There was nothing to suggest a congenital deformity of the aortic valve. Because of the combination of defects in this patient, there were unusual problems in diagnosis and treatment. Since the main pulmonary artery and its branches did not opacify in the selective angiocardiogram, it was concluded preoperatively that the congenital lesion was probably pulmonary atresia. It may be difficult to distinguish this malformation from the severe forms of tetralogy of Fallot. A large portion of the pulmonary blood flow in this patient was undoubtedly from systemic arterial collaterals; these were probably responsible for the continuous murmur heard over most of the thorax. In the presence of a large ventricular septal defect, as in this patient, the systolic pressures in the right ventricle, left ventricle, and aorta are ordinarily equal. However, the systolic pressure gradient between the right ventricle and brachial artery indicated the presence of obstruction, which was subsequently
Volume 60 Number 4
TETRALOGY
OF FALLOT
WITH
RHEUMATIC
AORTIC
STENOSIS
529
proved to be at the aortic valve. Since the usual anatomic defects of the tetralogy of Fallot were present in addition to the aortic stenosis, there was obstruction to the outflow of blood from both ventricles. An increased burden on the left ventricle was produced in much the same manner, but to a lesser degree, as ilr the right ventricle. It seems likely, also, that blood flow through the hypoplast ic. right ventricular outflow tract and small pulmonary artery was increased somewhat by the abnormally high ventricular pressure. It is clear, however, that there was an added work load on the right ventricle besides that which resulted from the pulmonic stenosis, because the presence of aortic stenosis further increased the resistance to right ventricular ejection. The degree of anatomic narrowing of the aortic valve in this patient, if present as an isolated lesion, would not have been considered alarming, but since it represented obstruction to the only route of emptying for both ventricles, it assumed greater significance. Since the aorta was receiving all the blood from the left ventricle and most of that from the right, the flow across the valve was greatly increased and a larger aortic gradient was present in this patient than would have been present in a patient having isolated aortic stenosis with the same degree of anatomic narrowing. When surgical treatment for this patient was considered, a completeI>, corrective procedure was thought not to be feasible because of the hypoplastic right ventricular outflow tract and pulmonary arteries. The creation of an artificial ductus alone would have increased the burden on the left ventricle by increasing the return of blood to the left side of the heart. A similar situation is seen following the construction of an anastomosis in patients with tetralogy of Fallot in whom the outflow of blood from the left ventricle is impeded by extreme dextroposition of the aorta. Relief of the aortic stenosis, in this patient, would be expected to relieve the excess workload of both ventricles; it is doubtful whether a reduction in ventricular pressure would have significantly decreased the flow of blood through the pulmonary artery after the anastomosis was opened. The cause of death in the immediate postoperative period was most likely related to the poor myocardial function which certainly accompanied the myocardial fibrosis she was shown to have. SUMMARY
A patient is described in whom a severe form of the tetralogy of Fallot was complicated by rheumatic stenosis of the aortic valve. These diagnoses were established by the clinical findings and the results of right and left heart catheterization and selective angiocardiography. An aortic valvulotomy and subclavian-pulmonary anastomosis were carried out, but the patient died in the immediate postoperative period. The pathologic findings are described and a discussion of the hemodynamic alterations which accompanied this unusual combination of defects is presented. REFERENCES
:: 3. 4. 5.
Koletsky S. : Congenital Bicuspid Aortic Valves Arch. Int. Med. 67:129 1941. Gross L ‘: So-Called Congenital Bicuspid Aortic’Valve Arch Path 23:3$0, 1937. Roe&r, ‘H. : Interatrial Septal Defect, Arch. Int. Med.‘54:339, 1934. Gelfman, R., and Levine, S. A.: The Incidence of Acute and Subacute Bacterial Endocarditis in Congemtal Heart Disease, Am. J. M. SC. 204:324, 1942. Morrow, A. G., Sanders, R. J., and Braunwald, E.: The Nitrous Oxide Test. An Improved Method for the Detection of Left-to-Right Shunts, Circulation 17:284, 19.58.
Tetralogy
of Fallot With
Rh eumatic
Stenosis of the Aortic
N. Perryman Collins, M.D., Andrew G. M&row, Eugene Braunwald, M.D., Bethesda, Md.
Valve
M.D., and
The incidence of rheumatic valvulitis in patients with congenital heart disease is difficult to ascertain. In patients having congenitally bicuspid aortic valves, the incidence of all rheumatic infections of the heart has been variously reported from 16 to 62 per cent,‘J and in those with atria1 septal defects it has been as high as 13 per cent.3 Gelfman and Levine,4 in examining 181 autopsy cases of congenital cardiac malformations, found 2.5 (14 per cent) in which there was evidence of superimposed rheumatic infection. One was a patient with tetralogy of Fallot but no details were given as to the specific rheumatic lesion in the heart. Since their study, little has been written concerning the occurrence of rheumatic heart disease in patients with congenital defects. A patient recently studied at the National Heart Institute was shown to have the tetralogy of Fallot and calcific aortic stenosis of rheumatic origin. To our knowledge, no description of a patient with these combined lesions has been recorded, and in view of the sparsity of information on the subject a description of the clinical, hemodynamic, and anatomic findings in this patient was considered worth while. CASE
REPORT
F. P. (No. 02-lo-20), a 25year-old female student, was admitted to the National Heart Institute on Aug. 17, 1958. Cyanosis and a heart murmur were known to have been present since birth. She had always had exertional dyspnea and was said to have squatted to rest during childhood. The history also revealed that at 4, 7, and 12 years of age she had had attakcs of joint effusions with pain associated with fever and an elevated erythrocyte sedimentation rate. On each occasion the diagnosis of acute rheumatic fever was made and she was hospitalized. The patient’s dyspnea was more severe after the last such illness. Ten years prior to admission an attempt had been made, at another hospital, to perform a right subclavian-pulmonary artery anastomosis but the procedure was abandoned because of the small size of the right pulmonary artery. She had remained on almost complete bed rest for 10 years, and for the 2 months immediately prior to admission had been hospitalized because of marked dyspnea. From the Clinic of Surgery, National Heart Received for publication Feb. 1, 1960.
Institute, 624
Bethesda.
Md.
E-L%: “4”
TETRALOGY
OF FALLOT
WITH
RHEUMATIC
AORTIC
STENOSIS
625
Physical examination revealed the patient to be well developed and slightly obese; there was marked generalized cyanosis. The blood pressure was 106/75 mm. Hg and the pulse 104. A healed right posterolateral thoracotomy incision was present. A left ventricular tap and a pronounced right ventricular lift were palpable over the precordium. A Grade 4/6 ejection type of systolic murmur was audible over the entire precordium but was maximal at the base of the heart and was transmitted to the carotid vessels. A Grade 2/6 murmur was also present over the entire thorax during diastole, and the two murmurs were considered to be continuous. There was a single second heart sound at the base. Normal sinus rhythm was present and the peripheral arterial pulsations were normal. There was marked clubbing and cyanosis of all digits. Routine laboratory examinations were normal except that the hemoglobin was elevated to 22.8 Gm. per 100 ml. and the hematocrit was 69 per cent. Fluoroscopic and radiographic examinations revealed right ventricular enlargement, decreased pulmonary vascularity, and calcification in the region of the aortic valve (Fig. 1). The electrocardiogram demonstrated right axis deviation, right atrial enlargement, and right ventricular hypertrophy.
Fig. I.-Posterior-anterior (A) and lateral (B) projections of the chest x-ray. The heart is grossly enlarged, particularly the right ventricle, and no main pulmonary artery is visible. Pulmonary vascularity is decreased. The ring of calcification in the region of the aortic valve is indicated by arrows in the lateral view. Right heart catheterization was carried out on Aug. 18, 19.58. The pressure in the right ventricle was 160/S mm. Hg, and in the brachial artery it was 90/63 mm. Hg. Arterial indicatordilution curves recorded after right atrial and right ventricular injection revealed a large right-toleft shunt which originated from the right ventricle. The peripheral arterial. saturation was 61 per cent. The nitrous-oxide index6 was 0 per cent in the right atrium and 100 per cent in the right ventricle; the latter value indicated that the catheter had sampled left ventricular blood immediately beneath the aortic valve. The catheter could not be manipulated into the pulmonary artery or aorta. A selective angiocardiogram with right ventricular injection revealed a large ventricular septal defect and a large single vessel arising from the base of the heart (Fig. 2). Two ventricles were demonstrated but the pulmonary arteries were never opacified. The angiocardiographic findings were thought to indicate pulmonary atresia (pseudotruncus arteriosus) or a severe form of tetralogy of Fallot. Since the ventricles were functioning with a common ejectile force, the systolic pressure gradient of 70 mm. Hg between the right ventricle and brachial artery indicated the presence of associated aortic stenosis. To confirm this diagnosis and to localize the site of outflow obstruction, a retrograde arterial catheterization was performed. The catheter entered
626
COLLINS,
MORROW,
AND BRAUNWXLD
the left ventricle from the aorta, and the pressures recorded as it was withdrawn valve proved the presence of valvular aortic stenosis (Fig. 3).
Am. Heart J. October, 1960 across the aortic
Because of the severe deformity of the right ventricular outflow tract and the absence of pulmonary arterial opacification demonstrated by the angiocardiogram, the patient’s lesions were not considered to be amenable to complete correction. Therefore, a systemic pulmonary anastomosis and aortic valvulotomy were decided upon. At the time of operation it was clear that the congenital lesion was a severe form of the tetralogy of Fallot; there was marked dextroposition of the aorta, and the main pulmonary artery was hypoplastic. There was an intense systolic thrill in the ascending aorta, which was dilated. The left pulmonary artery was small, but it was possible to perform an anastomosis between the end of the left subclavian artery and the side of the left pulmonary artery. After the anastomosis had been opened, an aortic valvulotomy was carried out by closed transventricular dilatation. Measurements of ventricular and systemic arterial pressures before and after the valvulotomy revealed almost complete abolition of the pressure gradient across the aortic valve. In the immediate postoperative period the patient was hypotensive and refractory to the administration of blood and vasopressor agents. Approximately 2% hours after the conclusion of the operation, cardiac arrest occurred and resuscitation was unsuccessful.
Fig. 2.-Anteroposterior (A) and lateral (B) views of the selective angiocardiogram. The injection was made into the right ventricle. Both the right ventricle (R V) and left ventricle (LV) are filled, and the dilated aorta (Ao) is seen in both views. The outflow tract of the right ventricle appears to end blindly (arrow), and the pulmonary arteries do not fill. Pathologic Findings.-The heart weighed 700 grams, there was hypertrophy of both ventricles, and the ventricular cavities were small. There was severe stenosis of the right ventricular infundibulum, which was due to myocardial and endocardial thickening; the opening in the outflow was only 1 cm. in diameter. The pulmonic valve had three cusps, was stenotic as well as hypoplastic, and measured 3 cm. in circumference. The subclavian-pulmonary artery anastomosis was patent. A defect in the membranous portion of the ventricular septum was present; the mitral ring was calcified. The aorta was dextroposed, and there was dilatation of the ascending aorta. The aortic valve had three normally situated cusps. There was marked calcification of the aortic annulus and cusps, and there were numerous calcific deposits on the aortic side of the leaflets and to a lesser extent on their ventricular side (Fig. 4). The valve had little or no mobility. The right and left coronary cusps were fused near the ring; the other commissures showed evidence of having been recently opened.
Volume 60 Number 4
TETRALOGY
OF FALLOT
WITH
RHEI’MATIC
AORTIC
STENOSIS
+I.0 sec.3
Fig. 3.-Pressures recorded during retrograde arterial catheterization as the catheter was withdrawn from the left ventricle into the aorta. A peak systolic gradient of 40 to 50 mm. Hg is evident,. The patient was anesthetized during this study.
Fig. 4.-The appearance of the heart and great vessels at autopsy. The dextroposition of the aorta and the diminutive main and left pulmonary arteries are shown. The site of the patent left subclavisn-pulmonary anastomosis and the extreme dilatation of the aorta are also evident. The relations of the calcified aortic valve to the ventricular septum are shown in the insert.
628
COLLINS,
MORROW,
AND BRAUNWALD
Am. Heart J. October, 1960
Microscopic examination of the aortic valve showed it to be greatly thickened and to contain lobulated areas of calcium surrounded by dense fibrous tissue and focally dense chronic inflammatory cellular infiltrates (Fig. 5). The valve was prominently vascularized. Other histologic examinations revealed that the mitral valve was thickened and there was fibrous tissue as well as many thick-walled vessels deep in its substance. There were widely scattered focal areas of scarring in the myocardium of both ventricles and extensive endocardial calcification.
Fig. 5.-Photomicrograph of a vertical section through an aortic leaflet. The leaflet (AL) is extensively infiltrated with fibrous tissue and chronic inflammatory cells. The clear areas represent sites in which calcium was present. Numerous vascular channels are evident in the base of the leaflet. (Hematoxylin and eosin; X 14, reduced %.) COMMENT
There can be little doubt regarding the cause of the aortic stenosis in this patient. The episodes of acute rheumatic fever were well documented and the presence of chronic inflammation in both the mitral and aortic valves provided anatomic proof of rheumatic disease. There was nothing to suggest a congenital deformity of the aortic valve. Because of the combination of defects in this patient, there were unusual problems in diagnosis and treatment. Since the main pulmonary artery and its branches did not opacify in the selective angiocardiogram, it was concluded preoperatively that the congenital lesion was probably pulmonary atresia. It may be difficult to distinguish this malformation from the severe forms of tetralogy of Fallot. A large portion of the pulmonary blood flow in this patient was undoubtedly from systemic arterial collaterals; these were probably responsible for the continuous murmur heard over most of the thorax. In the presence of a large ventricular septal defect, as in this patient, the systolic pressures in the right ventricle, left ventricle, and aorta are ordinarily equal. However, the systolic pressure gradient between the right ventricle and brachial artery indicated the presence of obstruction, which was subsequently
Volume 60 Number 4
TETRALOGY
OF FALLOT
WITH
RHEUMATIC
AORTIC
STENOSIS
529
proved to be at the aortic valve. Since the usual anatomic defects of the tetralogy of Fallot were present in addition to the aortic stenosis, there was obstruction to the outflow of blood from both ventricles. An increased burden on the left ventricle was produced in much the same manner, but to a lesser degree, as ilr the right ventricle. It seems likely, also, that blood flow through the hypoplast ic. right ventricular outflow tract and small pulmonary artery was increased somewhat by the abnormally high ventricular pressure. It is clear, however, that there was an added work load on the right ventricle besides that which resulted from the pulmonic stenosis, because the presence of aortic stenosis further increased the resistance to right ventricular ejection. The degree of anatomic narrowing of the aortic valve in this patient, if present as an isolated lesion, would not have been considered alarming, but since it represented obstruction to the only route of emptying for both ventricles, it assumed greater significance. Since the aorta was receiving all the blood from the left ventricle and most of that from the right, the flow across the valve was greatly increased and a larger aortic gradient was present in this patient than would have been present in a patient having isolated aortic stenosis with the same degree of anatomic narrowing. When surgical treatment for this patient was considered, a completeI>, corrective procedure was thought not to be feasible because of the hypoplastic right ventricular outflow tract and pulmonary arteries. The creation of an artificial ductus alone would have increased the burden on the left ventricle by increasing the return of blood to the left side of the heart. A similar situation is seen following the construction of an anastomosis in patients with tetralogy of Fallot in whom the outflow of blood from the left ventricle is impeded by extreme dextroposition of the aorta. Relief of the aortic stenosis, in this patient, would be expected to relieve the excess workload of both ventricles; it is doubtful whether a reduction in ventricular pressure would have significantly decreased the flow of blood through the pulmonary artery after the anastomosis was opened. The cause of death in the immediate postoperative period was most likely related to the poor myocardial function which certainly accompanied the myocardial fibrosis she was shown to have. SUMMARY
A patient is described in whom a severe form of the tetralogy of Fallot was complicated by rheumatic stenosis of the aortic valve. These diagnoses were established by the clinical findings and the results of right and left heart catheterization and selective angiocardiography. An aortic valvulotomy and subclavian-pulmonary anastomosis were carried out, but the patient died in the immediate postoperative period. The pathologic findings are described and a discussion of the hemodynamic alterations which accompanied this unusual combination of defects is presented. REFERENCES
:: 3. 4. 5.
Koletsky S. : Congenital Bicuspid Aortic Valves Arch. Int. Med. 67:129 1941. Gross L ‘: So-Called Congenital Bicuspid Aortic’Valve Arch Path 23:3$0, 1937. Roe&r, ‘H. : Interatrial Septal Defect, Arch. Int. Med.‘54:339, 1934. Gelfman, R., and Levine, S. A.: The Incidence of Acute and Subacute Bacterial Endocarditis in Congemtal Heart Disease, Am. J. M. SC. 204:324, 1942. Morrow, A. G., Sanders, R. J., and Braunwald, E.: The Nitrous Oxide Test. An Improved Method for the Detection of Left-to-Right Shunts, Circulation 17:284, 19.58.