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Atrial septostomy for pulmonary hypertension
volume Numbor
116 3
nutrient blood supply of veins in younger individuals may be more dependent on their more extensive vasa vasorum and penetrating vascular loops, and thus may be subject to greater degrees of mural ischemia during transplantation.16 These mechanisms may have been operative in our patients. In addition, a greater amount of shear stress would be expected in grafts connecting the aorta to a lower pressure vessel such as the pulmonary artery, with resultant higher velocity blood flow in the interposed segment. Sequelae of vein graft aneurysms have included occlusive6s7v10and nonocclusive thrombus,4s’2, l3 and rupture, witha-lo or without4 pseudoaneurysm formation. Stasis of blood within the aneurysm will predispose to thrombus formation. Rupture has been associated with larger aneurysms, as would be expected from Laplace’s law. The presence of a vein graft aneurysm can be suspected from physical examination, when it is situated in superficial locations such as the carotidI and femoraP3 sites. Aneurysms of grafts placed in the thoracic cavity can often be suspected on the basis of chest roentgenographic abnormalities,4~6~8*g as were present in our patients. For definitive diagnosis, angiography has usually been employed. However, computed tomography may serve as a diagnostic alternative. This technique better delineated the extent of a pseudoaneurysm than did angiography in one patient with an aneurysm of a coronary artery bypass graft,6 and ascertained the presence of two aneurysmal coronary bypass grafts in another patient in whom severe renal failure precluded the use of intravenous contrast material.g In our second case, magnetic resonance imaging was necessary to delineate the exact nature of the paracardisc mass, despite an initial attempt to do so by angiography. Additional information concerning blood flow and the presence or absence of thrombus was also obtained. Echocardiography was unable to visualize this paracardiac structure. Magnetic resonance imaging may therefore be extremely useful in diagnosis and follow-up of this entity, even when other procedures are of limited diagnostic quality. The demonstration of massive aneurysmal dilatation of systemic-pulmonary artery shunts placed in two young adults at our institution should prompt careful observation in others who are undergoing or who have already undergone this procedure. The diagnosis of massive aneurysmal dilatation can be made noninvasively, as is evident from our second case. Any abnormal paracardiac mass in individuals who have had a saphenous vein systemicpulmonary artery shunt should raise the possibility of a vein graft aneurysm and should be further investigated.
REFERENCES
1. Danilowicz
D, Ishmael RG, Doyle EF, Ison OW, Colvin SB, Greco MA. Use of saphenous vein allografts for aortopulmonary artery anastomoses in neonates with complex cyanotic congenital heart disease. Pediatr Cardiol 1984;5:13-8. 2. Gaylis H. Pathogenesis of anastomotic aneurysms. Surgery 1981;90:509-15.
Brief
3. Vlodaver
nary
Z, Edwards JE. Pathological arterial saphenous vein grafts.
Communications
changes Circulation
873
in aortocoro1971;44:719-
28. 4.
5.
6.
I.
8.
9.
10.
11.
12.
13.
14. 15.
16.
17.
Murphy JP Jr, Shabb B, Nishikawa A, Adams PR, Walker WE. Rupture of an aortocoronary saphenous vein graft aneurysm. Am J Cardiol 1986;58:555-‘7. Benchimol A, Harris CL, Desser KB, Fleming H. Aneurysms of an aorto-coronary artery saphenous vein bupass graft-a case report. Vast Surg 1975;9:261-4. Riahi M, Vasu CM, Tomatis LA, Schlosser RJ, Zimmerman G. Aneurysm of saphenous vein bypass graft to coronary artery. J Thorac Cardiovasc Surg 1975;70:358-9. Pintar K, Barboriak JJ, Johnson WD, Co ED. Atherosclerotic aneurysm in aortocoronary vein graft. Arch Path01 Lab Med 1978;102:287-8. Shapeero LG, Guthaner DF, Swerdlow CD, Wexler L. Rupture-of a coronary bypass graft aneurysm: CT evaluation and coil occlusion theranv. AJR 1983:141:1060-2. Yousem D, Scott W-Jr, Fishman EK, Watson AJ, Trail1 T, Gimenez L. Saphenous vein graft aneurysms demonstrated by computed tomography. J Comput Assist Tomogr 1986; l&526-8. Denton MJ, McGowen MA, Scott DF. True aneurysm formation in femoropopliteal autogenous vein bypass grafts: two cases. Aust NZ J Surg 1983;53:317-20. De La Rocha AG, Peixoto RS, Baird RJ. Atherosclerosis and aneurysm formation in a saphenous vein-graft. Br J Surg 1973;60:72-3. Davidson ED, DePalma RG. Atherosclerotic aneurysm occurring in an autogenous vein graft. Am J Surg 1972;124:11214. Cloud W, Handte G, Kron IL. Atherosclerotic aneurysm formation in a femoropopliteal saphenous vein graft. South Med J 1984;77:652-7. Carrasquilla C, Weaver AW. Aneurysm of a saphenous vein graft to the common carotid artery. Vast Surg 1972;6:66-8. Friedman SA, Cerruti MM, Amadeo B. Spontaneous necrosis of a functioning saphenous vein graft. Surgery 1969;66: 1022-5. Dean RH, Wilson JP, Burko H, Foster JH. Saphenous vein aortorenal bypass grafts: serial arteriographic study. Ann Surg 1974;180:469-77. Stanley JC, Ernst CB, Fry WJ. Fate of 100 aortorenal vein grafts: characteristics of late graft expansion, aneurysmal dilatation, and stenosis. Surgery 1973;74:931-44.
Atrial septostomy hypertension*
for pulmonary
Tyrone J. Collins, MD, MAJ, MC, John W. Moore, MD, LTC, MC, and William C. Kirby, MD, LTC, MC. Washington, D.C., and Bethesda, Md.
Recurrent thromboembolic disease related to ventriculoatria1 shunting for hydrocephalus may result in irreversFrom Walter Reed Army Medical Center and Uniformed Services University of the Health Sciences. Reprint requests: Tyrone J. Collins, MD, MAJ, MC, Cardiology Service, Walter Reed Army Medical Center, Washington, DC 20307. *The opinions expressed are those of the authors and are not to he construed as official or as reflecting the views of the Department of the Army or the Department of Defense.
874
Brief
Communications
ible pulmonary hypertension.1,2 Moreover, pulmonary hypertension in the presence of intact atria1 and ventricular septa may cause heart failure that responds poorly to medical therapy. We report a case of intractable car pulmonale after ventriculoatrial shunting that was successfully palliated with blade and balloon atria1 septostomy. The patient was born prematurely and required mechanical ventilation. Secondary hydrocephalus was treated with a ventriculoatrial shunt. The shunt was replaced by a ventriculo-pleural shunt at 5 years of age and was removed at 10 years of age. At 11 years of age, the onset of right heart failure prompted the patient’s admission to Walter Reed Army Medical Center. The admission physical examination was remarkable for cardiac murmurs consistent with tricuspid regurgitation and pulmonary regurgitation, ascites, hepatomegaly, and pedal edema. The chest radiograph demonstrated cardiomegaly, bilateral pleural effusions, and prominent central pulmonary vascular markings. The electrocardiogram (ECG) showed right ventricular hypertrophy. Echocardiography demonstrated a dilated right atrium, an enlarged right ventricle, and paradoxical septal motion. Doppler evaluation confirmed tricuspid and pulmonary insufficiency. The patient’s right-sided heart failure did not respond to a lo-day inpatient course of therapy consisting of oxygen, digoxin, and diuretics. Cardiac catheterization was performed 2 weeks after admission. There were suprasystemic right ventricular and pulmonary arterial pressures (pulmonary artery mean pressure, 84 mm Hg). Pulmonary vascular resistance was calculated to be 44 Wood units. There was no significant change in pulmonary pressures or resistances related to the administration of oxygen or nitroglycerin. Pulmonary artery wedge angiography demonstrated diffuse pruning of small vessels and poor capillary blushes, consistent with severe pulmonary vascular disease.3 Because of refractory heart failure, repeat catheterization and blade and balloon atria1 septostomy were performed approximately 1 month after admission. Transseptal catheterization was initially performed, and it was followed by blade septostomy with a 1.5 cm Park blade septostomy catheter (Cook Inc., Bloomington, Ind.) (completing three passes). The septostomy was then dilated with a 20 mm diameter Mansfield balloon valvuloplasty catheter (Mansfield Scientific Inc., Mansfield, Mass.) that was inflated after positioning its midpoint across the atrial septum. Systemic arterial oxygen saturation decreased from 95% before septostomy to 91% after septostomy. Pressures before and after septostomy respectively were: mean right atria1 pressure 16 mm Hg and 14 mm Hg; mean left atria1 pressure 3 mm Hg and 14 mm Hg; and pulmonary artery pressure 100/50 mm Hg and 80/50 mm Hg. Femoral artery pressure was 100/50 mm Hg before and after septostomy. Immediately after atria1 septostomy, the patient was treated with intraveA.clUU C&s dm,i a dobutamine infusion. Significant symptomatic improvement was noted and the patient’s hepatomegaly, ascites, pleural effusions, and
peripheral edema resolved. Contrast echocardiography performed 2 months and 10 months later revealed rightto-left shunting at the atrial level. The patient has been maintained on digoxin, furosemide, and aspirin. At 12 months follow-up, he shows no recurrent signs of rightsided heart failure. Primary pulmonary hypertension (pulmonary arterial hypertension of unknown cause) has been associated with: pulmonary veno-occlusive disease, recurrent thromboembolism, and plexogenic arteriopathy.4 Moreover, Noonan, Ehmke’ and Friedman et al.* have reported pulmonary vascular disease developing in patients with ventriculoatrial shunting for hydrocephalus. There is no effective medical therapy for right-sided heart failure related to irreversible pulmonary vascular disease.5 However, Austen et al6 demonstrated beneficial effects (decompression of the hypertensive right ventricle and augmentation of the systemic blood flow) from experimentally created rightto-left shunting in a dog model of pulmonary hypertension. Rich and Lam7 reported early favorable hemodynamic results in a 22-year-old woman with primary pulmonary hypertension in whom right-to-left shunting was created by septostomy. Nihill et al.& have presented favorable results in eight patients treated with blade and balloon atria1 septostomy for terminal car pulmonale. Interestingly, patients referred for heart-lung transplantation have survived longer if they have a patent foramen ovale.9 Our case provides further evidence that palliation for refractory right-sided heart failure from pulmonary hypertension may be achieved by atria1 septostomy. We believe that atria1 septostomy may prove to be an important adjunct to the therapy for pulmonary hypertension and associated right-sided heart failure. REFERENCES
1. Noonan JA, Ehmke DA. Complications of ventriculovenous shunts for control of hydrocephalus. N Engl J Med 1963; 2695’0. 2. Friedman S, Zita-Gozum C, Chatten J. Pulmonary vascular changes complicating shunting for hydrocephslus. J Pediatr 1964;64:305. 3. Nihill MR, McNamara DG. Magnification pulmonary wedge angiography in the evaluation of children with congenital heart disease and pulmonary hypertension. Circulation 1978;58:1094. 4. Jones DK, Higenbottam TW, Wallwork J. Treatment of primary pulmonary hypertension with intravenous epoprostenol (prbstacyclin). Br Heart J 1987;57:270. 5. Timmis GC. Westveer DC. Hauser AM. Stewart JR. Dressendorfer RH, ‘O’Neill WW. ‘Cardiovascular review. Baltimore: Grune & Stratton, Inc., 1985683-7. 6. Austen WG. Morrow AG. Berrv WB. Exnerimental studies of the surgical treatment of pr&ary pulionary hypertension. J Thorac Cardiovasc Surg 1964;48:448. 7. Rich S, Lam W. Atria1 septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol 1983;51:1560. 8. Nihill MR, O’Laughlin MP, Mullins CE. Blade and balloon atrial septostomy is effective palliation for terminal car pulmonale. [Abstract]. Am J Cardiol 1987;60:638. 9. Glanville AR, Burke CM, Theodore J, Robin ED. Primary pulmonary hypertension; length of survival in patients referred for heart-lung transplantation. Chest 1987;91:675.
116 3
nutrient blood supply of veins in younger individuals may be more dependent on their more extensive vasa vasorum and penetrating vascular loops, and thus may be subject to greater degrees of mural ischemia during transplantation.16 These mechanisms may have been operative in our patients. In addition, a greater amount of shear stress would be expected in grafts connecting the aorta to a lower pressure vessel such as the pulmonary artery, with resultant higher velocity blood flow in the interposed segment. Sequelae of vein graft aneurysms have included occlusive6s7v10and nonocclusive thrombus,4s’2, l3 and rupture, witha-lo or without4 pseudoaneurysm formation. Stasis of blood within the aneurysm will predispose to thrombus formation. Rupture has been associated with larger aneurysms, as would be expected from Laplace’s law. The presence of a vein graft aneurysm can be suspected from physical examination, when it is situated in superficial locations such as the carotidI and femoraP3 sites. Aneurysms of grafts placed in the thoracic cavity can often be suspected on the basis of chest roentgenographic abnormalities,4~6~8*g as were present in our patients. For definitive diagnosis, angiography has usually been employed. However, computed tomography may serve as a diagnostic alternative. This technique better delineated the extent of a pseudoaneurysm than did angiography in one patient with an aneurysm of a coronary artery bypass graft,6 and ascertained the presence of two aneurysmal coronary bypass grafts in another patient in whom severe renal failure precluded the use of intravenous contrast material.g In our second case, magnetic resonance imaging was necessary to delineate the exact nature of the paracardisc mass, despite an initial attempt to do so by angiography. Additional information concerning blood flow and the presence or absence of thrombus was also obtained. Echocardiography was unable to visualize this paracardiac structure. Magnetic resonance imaging may therefore be extremely useful in diagnosis and follow-up of this entity, even when other procedures are of limited diagnostic quality. The demonstration of massive aneurysmal dilatation of systemic-pulmonary artery shunts placed in two young adults at our institution should prompt careful observation in others who are undergoing or who have already undergone this procedure. The diagnosis of massive aneurysmal dilatation can be made noninvasively, as is evident from our second case. Any abnormal paracardiac mass in individuals who have had a saphenous vein systemicpulmonary artery shunt should raise the possibility of a vein graft aneurysm and should be further investigated.
REFERENCES
1. Danilowicz
D, Ishmael RG, Doyle EF, Ison OW, Colvin SB, Greco MA. Use of saphenous vein allografts for aortopulmonary artery anastomoses in neonates with complex cyanotic congenital heart disease. Pediatr Cardiol 1984;5:13-8. 2. Gaylis H. Pathogenesis of anastomotic aneurysms. Surgery 1981;90:509-15.
Brief
3. Vlodaver
nary
Z, Edwards JE. Pathological arterial saphenous vein grafts.
Communications
changes Circulation
873
in aortocoro1971;44:719-
28. 4.
5.
6.
I.
8.
9.
10.
11.
12.
13.
14. 15.
16.
17.
Murphy JP Jr, Shabb B, Nishikawa A, Adams PR, Walker WE. Rupture of an aortocoronary saphenous vein graft aneurysm. Am J Cardiol 1986;58:555-‘7. Benchimol A, Harris CL, Desser KB, Fleming H. Aneurysms of an aorto-coronary artery saphenous vein bupass graft-a case report. Vast Surg 1975;9:261-4. Riahi M, Vasu CM, Tomatis LA, Schlosser RJ, Zimmerman G. Aneurysm of saphenous vein bypass graft to coronary artery. J Thorac Cardiovasc Surg 1975;70:358-9. Pintar K, Barboriak JJ, Johnson WD, Co ED. Atherosclerotic aneurysm in aortocoronary vein graft. Arch Path01 Lab Med 1978;102:287-8. Shapeero LG, Guthaner DF, Swerdlow CD, Wexler L. Rupture-of a coronary bypass graft aneurysm: CT evaluation and coil occlusion theranv. AJR 1983:141:1060-2. Yousem D, Scott W-Jr, Fishman EK, Watson AJ, Trail1 T, Gimenez L. Saphenous vein graft aneurysms demonstrated by computed tomography. J Comput Assist Tomogr 1986; l&526-8. Denton MJ, McGowen MA, Scott DF. True aneurysm formation in femoropopliteal autogenous vein bypass grafts: two cases. Aust NZ J Surg 1983;53:317-20. De La Rocha AG, Peixoto RS, Baird RJ. Atherosclerosis and aneurysm formation in a saphenous vein-graft. Br J Surg 1973;60:72-3. Davidson ED, DePalma RG. Atherosclerotic aneurysm occurring in an autogenous vein graft. Am J Surg 1972;124:11214. Cloud W, Handte G, Kron IL. Atherosclerotic aneurysm formation in a femoropopliteal saphenous vein graft. South Med J 1984;77:652-7. Carrasquilla C, Weaver AW. Aneurysm of a saphenous vein graft to the common carotid artery. Vast Surg 1972;6:66-8. Friedman SA, Cerruti MM, Amadeo B. Spontaneous necrosis of a functioning saphenous vein graft. Surgery 1969;66: 1022-5. Dean RH, Wilson JP, Burko H, Foster JH. Saphenous vein aortorenal bypass grafts: serial arteriographic study. Ann Surg 1974;180:469-77. Stanley JC, Ernst CB, Fry WJ. Fate of 100 aortorenal vein grafts: characteristics of late graft expansion, aneurysmal dilatation, and stenosis. Surgery 1973;74:931-44.
Atrial septostomy hypertension*
for pulmonary
Tyrone J. Collins, MD, MAJ, MC, John W. Moore, MD, LTC, MC, and William C. Kirby, MD, LTC, MC. Washington, D.C., and Bethesda, Md.
Recurrent thromboembolic disease related to ventriculoatria1 shunting for hydrocephalus may result in irreversFrom Walter Reed Army Medical Center and Uniformed Services University of the Health Sciences. Reprint requests: Tyrone J. Collins, MD, MAJ, MC, Cardiology Service, Walter Reed Army Medical Center, Washington, DC 20307. *The opinions expressed are those of the authors and are not to he construed as official or as reflecting the views of the Department of the Army or the Department of Defense.
874
Brief
Communications
ible pulmonary hypertension.1,2 Moreover, pulmonary hypertension in the presence of intact atria1 and ventricular septa may cause heart failure that responds poorly to medical therapy. We report a case of intractable car pulmonale after ventriculoatrial shunting that was successfully palliated with blade and balloon atria1 septostomy. The patient was born prematurely and required mechanical ventilation. Secondary hydrocephalus was treated with a ventriculoatrial shunt. The shunt was replaced by a ventriculo-pleural shunt at 5 years of age and was removed at 10 years of age. At 11 years of age, the onset of right heart failure prompted the patient’s admission to Walter Reed Army Medical Center. The admission physical examination was remarkable for cardiac murmurs consistent with tricuspid regurgitation and pulmonary regurgitation, ascites, hepatomegaly, and pedal edema. The chest radiograph demonstrated cardiomegaly, bilateral pleural effusions, and prominent central pulmonary vascular markings. The electrocardiogram (ECG) showed right ventricular hypertrophy. Echocardiography demonstrated a dilated right atrium, an enlarged right ventricle, and paradoxical septal motion. Doppler evaluation confirmed tricuspid and pulmonary insufficiency. The patient’s right-sided heart failure did not respond to a lo-day inpatient course of therapy consisting of oxygen, digoxin, and diuretics. Cardiac catheterization was performed 2 weeks after admission. There were suprasystemic right ventricular and pulmonary arterial pressures (pulmonary artery mean pressure, 84 mm Hg). Pulmonary vascular resistance was calculated to be 44 Wood units. There was no significant change in pulmonary pressures or resistances related to the administration of oxygen or nitroglycerin. Pulmonary artery wedge angiography demonstrated diffuse pruning of small vessels and poor capillary blushes, consistent with severe pulmonary vascular disease.3 Because of refractory heart failure, repeat catheterization and blade and balloon atria1 septostomy were performed approximately 1 month after admission. Transseptal catheterization was initially performed, and it was followed by blade septostomy with a 1.5 cm Park blade septostomy catheter (Cook Inc., Bloomington, Ind.) (completing three passes). The septostomy was then dilated with a 20 mm diameter Mansfield balloon valvuloplasty catheter (Mansfield Scientific Inc., Mansfield, Mass.) that was inflated after positioning its midpoint across the atrial septum. Systemic arterial oxygen saturation decreased from 95% before septostomy to 91% after septostomy. Pressures before and after septostomy respectively were: mean right atria1 pressure 16 mm Hg and 14 mm Hg; mean left atria1 pressure 3 mm Hg and 14 mm Hg; and pulmonary artery pressure 100/50 mm Hg and 80/50 mm Hg. Femoral artery pressure was 100/50 mm Hg before and after septostomy. Immediately after atria1 septostomy, the patient was treated with intraveA.clUU C&s dm,i a dobutamine infusion. Significant symptomatic improvement was noted and the patient’s hepatomegaly, ascites, pleural effusions, and
peripheral edema resolved. Contrast echocardiography performed 2 months and 10 months later revealed rightto-left shunting at the atrial level. The patient has been maintained on digoxin, furosemide, and aspirin. At 12 months follow-up, he shows no recurrent signs of rightsided heart failure. Primary pulmonary hypertension (pulmonary arterial hypertension of unknown cause) has been associated with: pulmonary veno-occlusive disease, recurrent thromboembolism, and plexogenic arteriopathy.4 Moreover, Noonan, Ehmke’ and Friedman et al.* have reported pulmonary vascular disease developing in patients with ventriculoatrial shunting for hydrocephalus. There is no effective medical therapy for right-sided heart failure related to irreversible pulmonary vascular disease.5 However, Austen et al6 demonstrated beneficial effects (decompression of the hypertensive right ventricle and augmentation of the systemic blood flow) from experimentally created rightto-left shunting in a dog model of pulmonary hypertension. Rich and Lam7 reported early favorable hemodynamic results in a 22-year-old woman with primary pulmonary hypertension in whom right-to-left shunting was created by septostomy. Nihill et al.& have presented favorable results in eight patients treated with blade and balloon atria1 septostomy for terminal car pulmonale. Interestingly, patients referred for heart-lung transplantation have survived longer if they have a patent foramen ovale.9 Our case provides further evidence that palliation for refractory right-sided heart failure from pulmonary hypertension may be achieved by atria1 septostomy. We believe that atria1 septostomy may prove to be an important adjunct to the therapy for pulmonary hypertension and associated right-sided heart failure. REFERENCES
1. Noonan JA, Ehmke DA. Complications of ventriculovenous shunts for control of hydrocephalus. N Engl J Med 1963; 2695’0. 2. Friedman S, Zita-Gozum C, Chatten J. Pulmonary vascular changes complicating shunting for hydrocephslus. J Pediatr 1964;64:305. 3. Nihill MR, McNamara DG. Magnification pulmonary wedge angiography in the evaluation of children with congenital heart disease and pulmonary hypertension. Circulation 1978;58:1094. 4. Jones DK, Higenbottam TW, Wallwork J. Treatment of primary pulmonary hypertension with intravenous epoprostenol (prbstacyclin). Br Heart J 1987;57:270. 5. Timmis GC. Westveer DC. Hauser AM. Stewart JR. Dressendorfer RH, ‘O’Neill WW. ‘Cardiovascular review. Baltimore: Grune & Stratton, Inc., 1985683-7. 6. Austen WG. Morrow AG. Berrv WB. Exnerimental studies of the surgical treatment of pr&ary pulionary hypertension. J Thorac Cardiovasc Surg 1964;48:448. 7. Rich S, Lam W. Atria1 septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol 1983;51:1560. 8. Nihill MR, O’Laughlin MP, Mullins CE. Blade and balloon atrial septostomy is effective palliation for terminal car pulmonale. [Abstract]. Am J Cardiol 1987;60:638. 9. Glanville AR, Burke CM, Theodore J, Robin ED. Primary pulmonary hypertension; length of survival in patients referred for heart-lung transplantation. Chest 1987;91:675.