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Coarctation of the abdominal aorta
Coarctation SIAVOSH BAHABOZORGUI,
From
the Department
of Surgery
of the Abdominal
M.D. AND PAUL NEMIR, JR.,M.D.,Philadelphia,Pennsylvania
fundoscopy. The chest, heart, and abdomen were essentially normal on physical examination. A systolic bruit was heard over the right side of the back at the level of the first and second lumbar vertebrae. No pulses could be felt at the level of the common femoral arteries or further down in either leg. Both legs were cool but otherwise not remarkable. Pulses in the arms and in the neck were normal. No trophic changes were present in either leg. A left retrograde brachial aortogram was performed and revealed complete obstruction of the abdominal aorta at the level of the interspace of the first and second lumbar vertebrae. (Fig. 1.) A large superior mesenteric artery filled from the abdominal aorta while the inferior mesenteric artery filled through a large collateral marginal artery of Drummond. Both hypogastric arteries were visualized from collateral circulation through the superior hemorrhoidal and the marginal artery of Drummond. There was no visualization of the distal abdominal aorta or the iliac, or femoral arteries. In view of the configuration on aortography, it was thought that the patient had a coarctation of the aorta immediately distal to the level of the renal arteries and that the progressing symptoms were due to gradual thrombosis in the atretic distal abdominal aorta. At operation, the left and right common femoral arteries which were first exposed were found to be soft and of normal caliber. The abdominal aorta was exposed through a midline abdominal incision. Beginning at the level of the renal arteries, the abdominal aorta was markedly narrowed, measuring approximately 1 cm. in diameter. This narrow diameter continued to within 3 cm. of the bifurcation at which level the abdominal aorta became much larger, measuring at least 2.5 cm. This dilated part of the aorta continued down to the bifurcation. (Fig. 2.) The dilated portion of the abdominal aorta was involved in a rather severe atherosclerotic process. The common iliac vessels were also involved with atherosclerosis, but for the most part these vessels were quite satisfactory. There was no periaortic fibrosis around the narrowed portion of this vessel as ordinarily observed in atherosclerosis obliterans or constriction of the aorta due to an inflammatory process.
and the Graduate HosPhiladelphia,
pital of the University of Pennsylvania, Pennsylvan,ania.
OF THE AORTA iS usually located in the aortic isthmus, distal or proximal to the ligamentum arteriosum [1,2]. Although the abdominal aorta is a rare site for coarctation, more instances have been reported in the last fifteen years than in the hundred years after the first description by Quain in 1847 [3]. About fifty cases are reported in the world literature and only about twenty patients have undergone reconstructive surgery [4,5]. A greater awareness of the condition and more refined diagnostic technics have played an important role in more clearly delineating this disease as a cause of hypertension. The purpose of this paper is to report on a patient having an area of coarctation just below the level of the renal arteries, who presented with hypertension and intermittent claudication, and in whom there was complete relief of signs and symptoms after reconstructive sur-
C
Aorta
OARCTATION
gery. CASEREPORT A fifty-three year old man (J. F., GHUP No. 308066) was admitted to the Graduate Hospital of the University of Pennsylvania because of intermittent claudication involving the right leg. Approximately ten months prior to admission, he began to experience pain in the posterior aspect of the right thigh and calf upon walking only half a block. Occasionally, similar symptoms developed in the left leg which were always less severe than those on the right. History was essentially noncontributory except that for two years prior to admission, he had been having decreased libido. Physical examination revealed the blood pressure to be 175/100 mm. Hg in the right arm, 170/105 mm. Hg in the left arm, and unobtainable in both legs. He had a grade I hypertensive retinopathy on 224
American
Journal of Surgery
Coarctation
of Abdominal
Aorta
225
~;rc. 1. Left retrograde brachial aortogram. Note the complete obstruction of the abdominal aorta at the level of the interspace of the first and second lumbar vertebrae. A large superior mesenteric arterv tillrd from the abdominal aorta. The inferior mesenteric artery fills through a large collateral mar&r1 artery of Drummond. There was no visualization of the distal abdominal aorta, iliac, or femoral arteries. FIG. 2. Schematic reproduction of the essential findings at operation. Beginning at the level of the renal arteries, the abdominal aorta was markedly narrowed measuring approximately 1 cm. in diameter, At approximately 3 cm. above the bifurcation the abdominal aorta became dilated measuring at least 2.5 cm. across, This dilated part continued down to the bifurcation. FIG. 3. Diagrammatic drawing of operative procedure. A Teflon graft was anastomosed end to side to the endarterectomized narrowed abdominal aorta approximately 1 cm. below the renal arteries, and both limbs were anastomosed to the common femoral arteries with end to side anastomosis.
To obtain control of the aorta above the renal arteries, the left side of the chest was entered through the seventh interspace, and the lower thoracic aorta was encircled with a tape. A longitudinal incision was made in the narrowed aorta just distal to the origin of the renal arteries, and moderately soft, organizing, grumous material was removed from about the orifices of the renal artery and then downwards for the length of the 2.5 cm. incision. A bifurcation Teflon@ prosthesis was anastomosed end to side to the narrowed abdominal aorta approximately 1 cm. below the renal arteries, and both limbs were anastomosed to the common femoral arteries at the level of the bifurcation. (Fig. 3.) The postoperative course was entirely uneventful. At the time of his discharge blood pressure in both arms was 130/70 mm. Hg, excellent dorsalis pedis and posterior tibia1 pulsations were present bilaterally, and the patient was completely free of symptoms. COMMENTS
Features which differentiate coarctation of the abdominal aorta from coarctation of the thoracic aorta are a systolic bruit heard best in the abdominal and lumbar region and complaints of intermittent claudication. Vol. 111, Februavy 1966
Etiology and Anatomy. Whether coarctation of the abdominal aorta is congenital or acquired is controversial. Maycock [6] suggested two possible causes: (1) the failure of the dorsal aortas to fuse equally at one point with consequent obliteration or loss of one of them and (2) kinking of the fused aortas with consequent localized increase in longitudinal tension producing a permanent narrowing. He considered the first explanation to be more probable. Bahnson, Cooley, and Sloan [7 ] believed that in their case stenosis was probably of long duration and congenital in origin, although the abrupt onset of symptoms related to deficient circulation in the lower extremities suggested thrombosis of the narrowed se,ment of the aorta. Coarctation of the abdominal aorta associated with Recklinghausen’s disease has been reported in two cases [8,9], and pathologic evidence of neurofibromatosis was found in tissue removed from the site of toarctation and elsewhere in both patients. Sen and his associates [IO] reported four
226
Bahabozorgui
cases at unusual sites of the aorta in which the coarctation or narrowing was caused by a stenosing panarteritis. All their patients were under the age of twenty and three of them were female patients. They mentioned that the diffuse character of narrowing, involvement of subclavian arteries in two cases, marked periaortic adhesions, and a characteristic histologic picture definitely pointed to an acquired lesion of inflammatory origin. They concluded that the lesion was probably of the nature of an allergic aortitis, and the over-all picture appeared to differ from that of giant cell arteritis, polyarteritis nodosa, and Buerger’s disease. Milloy and Fell [ll ] reported on four patients who had undergone surgery for elongated coarctation of the aorta. They described several differences between the usually encountered adult type of short coarctation of congenital origin and the elongated type of coarctation, which evidently may be congenital or acquired. The elongated type has been reported more commonly in female patients. The usual location of this type is below the isthmus and, when the vessel has been divided at surgery, the intimal and medial layers of the aortic wall have been observed to split apart with ease. D’Abreu, Rob, and Vollmar [12] divided coarctation of the abdominal aorta into two groups : segmentary coarctation and hypoplastic coarctation. These two groups are further divided into three subgroups: suprarenal, interrenal, and infrarenal coarctations according to the site of the beginning of the narrowed aortic segment. In the hypoplastic type there is no actual coarctation, but the abdominal aorta becomes narrow at a certain level and then remains hypoplastic down to the bifurcation. We believe that our case may be categorized under congenital type of abdominal coarctation. The chief collateral anastomoses in cases of coarctation of the abdominal aorta orginate from the lower intercostal arteries and internal mammary arteries to the caudal epigastric and lumbar arteries. Collateral network may also develop between visceral arteries from both sides of the coarctation, especially between the superior and inferior mesenteric artery and inferior mesenteric artery with hypogastric branches as in our case. Microscopically, Fisher and Corcoran [l3 1 observed thickening of the aortic intima and abnormal narrowing of the media at the site
and Nemir of coarctation. Focal cystic necrosis of the aortic wall occurred in the cases described by Albanese and Lazzarini [la]. The renal arteries and other major visceral arteries may originate from the narrowed segment, and they may be stenosed [4,13,15,16], hypoplastic [7,11,14,17], or occluded by thrombosis [18]. An aortic aneurysm distal to the coarctation has been noted [4,19], and aneurysms of the right renal artery in association with the coarctation have been reported [8,20]. The mechanism of aneurysm formation distal to coarctation of the aorta may be due to congenital weakness of the aortic wall which is aggravated by the turbulence, cavitation, and increased lateral pressure beyond the coarctation [4]. The signs and symptoms vary with the location and severity of the lesion, but those most frequently encountered are hypertension, heart failure, and circulatory insufficiency of the lower extremities. The patient with coarctation of the upper abdominal aorta tends to have severe hypertension with increased incidence of cerebrovascular accidents [21]. This is frequently due to added renal hypertension associated with involvement of the renal arteries by the occlusive process [22]. Moreover, this level of involvement appears to be associated with an increased propensity for cardiac failure and uremia [22]. Leg weakness and intermittent claudication are rare in coarctation of the upper abdominal aorta. The greater frequency of claudication in cases of coarctation of the lower abdominal aorta may be due to an inadequate development of the collateral circulation. Degeneration and thrombotic lesions in the stenosed aortic segment and in collateral anastomoses may have significance in the development of a circulatory insufficiency in the lower extremities. In two cases [16,18] in which intermittent claudication did occur, thrombotic obstruction of the aorta at the site of coarctation was revealed at autopsy. Intermittent claudication of relatively recent onset as observed in the patient herein reported was undoubtedly related to the thrombotic process in the stenotic segment . Other symptoms include headache, pericordial or epigastric pain, vertigo, palpitation, and slight dyspnea on exertion. Attacks closely simulating those caused by pheochromocytoma have been reported [23]. Anevican
Jouvnal
of Surwy
Coarctation
of Abdominal
On physical examination, elevation of blood pressure in the arm, both systolic and diastolic, is significant. In the case reported by Kondo and associates [24] the blood pressure in the left arm was 40 mm. Hg and in the right arm was unobtainable because both subclavian arteries were hypoplastic. The blood pressure is lower in the legs than in the arms, but in cases of mild coarctation it may be the same in both upper and lower extremities. Femoral pulsations are usually diminished or absent. An important sign observed in some patients is the presence of strong aortic pulsations in the epigastrium and its disappearance more distally. This sign is especially valuable in the differential diagnosis of coarctations of the abdominal and the thoracic aorta [25]. A systolic murmur in the lower dorsal region and/or in the upper part of the abdomen may be of some help but is not always heard. Pathogenesis of Hypertension. The pathogenesis of hypertension in coarctation of the abdominal aorta is not clear. Patients with coarctation of the upper abdominal aorta seem to have more severe hypertension than patients with coarctations localized to the thoracic or lower abdominal aorta. This would suggest an association with renal hypertension of the Goldblatt type [26]. Deterioration of the clinical condition of these patients may begin at an early age [22]. Kirkendall, Culbertson, and Eckstein [27] believe that the mechanical factor of aortic coarctation is the most important in the genesis of the arterial hypertension. Impaired renal function has not been a significant factor in a series of patients with renal hypertension due to partial obstruction of the renal arteries of atheromatous-thrombotic [ZZ] or congenital origin, and normal kidney function need not rule out renal hypertension. Although aortography and intravenous pyelography may provide some indications of the renal functional state, they cannot be relied upon to provide the knowledge requisite to deciding the cause of hypertension. Neither the filtration rate nor the rate of renal blood ilow is ascertainable from the nephrogram or pyelogram. Knowledge of the individual renal functional capacities is important not only in the identification of an ischemic kidney but also in ascertaining the functional capacity of a nonischemic kidney upon which renal function will alone depend after removal of the ischemic one [28]. Vol. 111. February
1966
Aorta
227
The fact that hypertension may occur in cases of coarctation of the abdominal aorta in which coarctation is located below the level of the renal arteries supports the belief that impairment of renal blood flow is not the cause of hypertension associated with coarctation. Diagnosis and Treatment. An unusual location of the coarctation should be suspected when, in the presence of clinical signs of coarctation of the aorta, the typical vascular deformity of the upper part of the mediastinum is lacking in conventional roentgenologic examination of the chest. Notching of the ribs is usually absent, but if it does occur it involves only the lower two or three ribs. Retrograde or translumbar aortography appears to be the best diagnostic method [29-311. Angiocardiography has been employed but does not give sufficient contrast filling of the abdominal aorta and may lead to an erroneous diagnosis. Aortography provides accurate delineation of the site of coarctation, its relationship to the origin of the renal arteries, and the length of the narrowed segment, information which is essential to the surgeon. In those cases presenting involvement of the renal arteries, a preoperative evaluation of renal function is important. The results of these investigations may well have a bearing on the type of surgical repair. Differentiation between coarctation of the abdominal aorta and coarctation of the thoracic aorta is not difficult if the possibility of the abdominal localization is kept in mind. This is important in view of the surgical treatment since the patient may be subjected to unnecessary thoracotomy [32,33 1. Acquired conditions producing stenosis or occlusion of the abdominal aorta must also be considered in differential diagnosis. These include atherosclerotic obstructive lesions with or without thrombosis, simple thrombosis or embolic occlusion, aneurysm or dissection involving the abdominal aorta and external pressure from a tumor. Acquired long-standing obstructive processes may sometimes give rise to the development of extensive collateral circulation, as shown by Bayliss [19]. Surgical Treatment. Glenn et al. [S] in 1952 performed the first successful corrective operation in a patient in whom the narrowed segment of the aorta was immediately proximal to the celiac axis and thus did not involve the renal arteries; the operation utilized the splenic
228
Bahabozorgui
artery as a bypass around the coarcted segment of the aorta. Since then, different surgical technics for this disease have been performed. In most instances, because of the location of the coarctation and its relation to the major visceral arteries, only bypassing of the coarcted segment has been attempted [34]. In a few cases, bypass graft to renal arteries has been performed [18,26,35]. In patients with coarctation of the abdominal aorta adjacent to renal arteries and probably also with coarctation located immediately above or below the renal arteries, surgical correction by application of a patch graft may be possible [35-371. In our case, endarterectomy above and below the renal arteries was performed after controlling the aorta above the renal arteries. Because of the close relation of the coarcted segment to the renal arteries, it was not feasible to resect the narrowed segment and insert a prosthesis. In several cases [7,8,12] a bilateral thoracolumbar sympathectomy or splanicectomy was performed before the diagnosis of coarctation was made. Similarly, lumbar sympathectomy was carried out in one case [23], and bilateral lumbar sympathectomy has been attempted in a few cases [7,38]. No definite benefit resulted from these operations. SUMMARY
and N emir
5.
6. 7.
8.
9.
10.
11.
12.
13.
14.
15.
AND CONCLUSIONS
A case is reported of coarctation of the abdominal aorta just below the renal artery manifested by intermittent claudication and hypertension. Obstruction was relieved by endarterectomy and bifurcation prosthesis. Etiology, clinical features, pathogenesis of hypertension, diagnosis, and surgical treatment of coarctation of the abdominal aorta are discussed. Coarctation of the aorta in unusual sites is being reported with greater frequency. An awareness of the possibility of this condition will lead to more accurate diagnosis and better management.
16. 17.
18.
19.
20.
21.
REFERENCES
1. GROSS, R. E. Coarctation of the aorta. Circulation, 7: 757, 1953. 2. SCHUSTER, S. R. and GROSS, R. E. Surgery for coarctation of the aorta. A review of 500 cases. J. Tkoracic & Cardiovas. Surg., 43: 54, 1962. 3. QUAIN, R. Partial coarctation of the abdominal aorta. Tr. Patk. SOG.London, 1: 244, 1847. 4. BAIRD, R. J., EVANS, J. R., and LABROSSE, C. L.
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Coarctation of the abdominal aorta. drck. Surg., 89: 466, 1964. PYORALA, K., HEINONEN, O., KOSKELO, P., and HEIKEL, P. Coarctation of the abdominal aorta. Review of twenty-seven cases. Am. J. Cardiol., 6: 650, 1960. MAYCOCK, W. D’il. Congenital stenosis of the abdominal aorta. Am. Heavl J., 13: 633, 1937. BAHNSON, H. T., COOLEY, R. N., and SLOAN, R. D. Coarctation of the aorta at unusual sites. Report of two cases with angiocardiographic and operative findings. Am. Heart J.. 38: 905. 1949. GLENN, F., “KEEFER, E. E:, SPEED, D. S., and DOTTER, C. T. Coarctation of the lower thoracic and abdominal aorta immediately proximal to celiac axis. Surg., Gynec. b O&t., 94: 561, 1952. BLOOR, K. and WILLIAMS, R. T. Neurofibromatosis and coarctation of the abdominal aorta with renal artery involvement. Brit. J. SW-~., 50: 811, 1963. SEN, P. K., KINARE, S. G., KULKARNI, T. P., and PARULKAR, G. B. Stenosing aortitrs of unknown etiology. Surgery, 51: 317, 1962. MILLOY, F. and FELL, E. H. Elongated coarctation of the aorta. Arch. Surg., 78: 759, 1959. D’ABREU, A. L., ROB, C. G., and VOLLMAR, J. F. Die Coarctio Aortae abdominalis Langenbecks. Arch. k&n. Ckir.. 290: 52. 1959. FISHER, E. R. and CORCO~AN, A. C. Congenital coarctation of the abdominal aorta with resultant renal hypertension. Arch. Int. Med., 89: 943, 1952. ALBANESE, A. R. and LAZZARINI, A. A. Coarctation of abdominal aorta-graft of preserved aorta. Angiology, 4: 429, 1953. BRUST, A. A., HOWARD, J. M., BRYANT, M. R., and GODWIN, J. T. Coarctation of the abdominal aorta with stenosis of the renal arteries and hypertension. Clinical and pathologic study of two cases and review of the literature. Am. J. Med., 27: 793, 1959. SHAPIRO, M. J. Coarctation of the abdominal aorta. Am. J. Cardiol., 4: 547, 1959. BJBRK, V. 0. and INTONTI, F. Coarctation of abdominal aorta with right renal artery stenosis. Ann. Surg., 160: 54, 1964. INMON, T. W. and POLLOCK, B. E. Coarctation of the abdominal aorta. Review of the literature. Am. Heart J., 52: 314, 1956. BAYLISS, G. J. Collateral circulation following obstruction of the abdominal aorta. Anat. Rec., 75: 405, 1939. SENNING, A. and JOHANSSON,L. Coarctation of the abdominal aorta. J. Tkoracic & Cardiovas. Surg., 40: 517, 1960. KAUFMAN. S. F. and MARKHAM. _ T. W. Coarctation of the abdominal aorta with death from rupture of an aneurysm of a cerebral artery. Ann. Int. Med., 43: 418, 1955. MORRIS. G. C., DEBAKEY, M. E., COOLEY, D. A., and CRAWFORD, E. S. Subisthmic aortic stenosis and occlusive disease. Arch. Sum., 80: 87, 1960. GOLDZIEHER,J. W., MCMAHON, Hy E., and-GOLDZIEHER, M. A. Coarctation of the abdominal aorta, simulating pheochromocytoma. Arch. Int. Med., 88: 835, 1951.
Amevican
Journal
of Suwry
Coarctation
of Abdominal Aorta
?A. Kosno, b., W.r~s;o~, I., RAULSTON, B. O., and KTKOI\\A, D. Congenital coarctation of the abdominal aorta; a theoretically reversible type of cardiac disease. .-ltil. Heart J., 39: 306, 1950. 25. STOKES, J. M., WOHLTI\IASN,H., and CARLSON, E. Coarctation of the abdominal aorta and renal artery stenosis corrected by surgical treatment. ‘The imlxxtance of individual renal function tests in selection of proper management. Ann. Surg., 152: 856, 1960. 26. TOONE, \V. M. and LITHERLAND, H. K. X case of coarctation of the abdominal aorta associated with renal ischemia and hypertension. &fed. Sere. J. Canada, 18: 659, 1962. 27. KIRKENDALL, W. J., CULBERTSON, J. W., and ECKSTEIN, J. W. Renal hemodynamics in patients with coarctation of the aorta. J. Lab. & Gin. Xed., 53: 6, 1959. 28. BRUKIN, M. Hypertension and unilateral renal ischemia caused by coarctation of the abdominal aorta. Soulh African M. J., 36: 962, 1962. 29. BUNNAG, T. S. Coarctation of the abdominal aorta at the origin of the inferior mesenteric artery. Report of a case diagnosed by translumbar aortography. Am. J. Roentgenok, 78: 1003, 1957. 30. KITTREDGE, R. D. and _%NDERSON, J. W. Coarcta-
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1966
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tion of the lower thoracic and abdotninal aorta. Radiology, 70: 799, 1962. M~RTIS, J. F. and YOUNT, E. H. Coarctation of the abdominal aorta. dm. J. Roextgencl., 76: 782, 1956. GERBASI, F. S., RIBLER, R. S., and MARGILETH, ;i. X Coarctation of the abdominal aorta. J. I’ediat., 52: 191, 1958. SCHUSTER, S. R. Coarctation of the abdominal aorta. Ann. Surg., 158: 1012, 1963. DILLON, M. and POSTLETHWAIT, R. W. Coarctation of the abdominal aorta. Report of a case and review of the surgical procedures. Soathern iv. J., 54: 295, 1961. SOSDERGAARD,T. and OTTOSEN, P. Coarctation of the abdominal aorta. Acta. chir. scandinav. (su~~l.), 285: 194, 1962. HANSON, J., IKKES, D., JOHANSSON,L., RUDHE, U., and SENNING, 0. Coarctation of the abdominal aorta. Acta. chir. scandinav. (suppl.), 245: 315, 1959. KING, H., KAISER, G., and KING, R. Repair of coarctation of the aorta by patch grafting. J. Thoracic & Cardiovas. Surg., 43: 792, 1962. RITCHIE, H. D. and DOUGLAS, D. M. Xtresia of the abdominal aorta. Brit. M. J., 1: 144, 1956.
From
the Department
of Surgery
of the Abdominal
M.D. AND PAUL NEMIR, JR.,M.D.,Philadelphia,Pennsylvania
fundoscopy. The chest, heart, and abdomen were essentially normal on physical examination. A systolic bruit was heard over the right side of the back at the level of the first and second lumbar vertebrae. No pulses could be felt at the level of the common femoral arteries or further down in either leg. Both legs were cool but otherwise not remarkable. Pulses in the arms and in the neck were normal. No trophic changes were present in either leg. A left retrograde brachial aortogram was performed and revealed complete obstruction of the abdominal aorta at the level of the interspace of the first and second lumbar vertebrae. (Fig. 1.) A large superior mesenteric artery filled from the abdominal aorta while the inferior mesenteric artery filled through a large collateral marginal artery of Drummond. Both hypogastric arteries were visualized from collateral circulation through the superior hemorrhoidal and the marginal artery of Drummond. There was no visualization of the distal abdominal aorta or the iliac, or femoral arteries. In view of the configuration on aortography, it was thought that the patient had a coarctation of the aorta immediately distal to the level of the renal arteries and that the progressing symptoms were due to gradual thrombosis in the atretic distal abdominal aorta. At operation, the left and right common femoral arteries which were first exposed were found to be soft and of normal caliber. The abdominal aorta was exposed through a midline abdominal incision. Beginning at the level of the renal arteries, the abdominal aorta was markedly narrowed, measuring approximately 1 cm. in diameter. This narrow diameter continued to within 3 cm. of the bifurcation at which level the abdominal aorta became much larger, measuring at least 2.5 cm. This dilated part of the aorta continued down to the bifurcation. (Fig. 2.) The dilated portion of the abdominal aorta was involved in a rather severe atherosclerotic process. The common iliac vessels were also involved with atherosclerosis, but for the most part these vessels were quite satisfactory. There was no periaortic fibrosis around the narrowed portion of this vessel as ordinarily observed in atherosclerosis obliterans or constriction of the aorta due to an inflammatory process.
and the Graduate HosPhiladelphia,
pital of the University of Pennsylvania, Pennsylvan,ania.
OF THE AORTA iS usually located in the aortic isthmus, distal or proximal to the ligamentum arteriosum [1,2]. Although the abdominal aorta is a rare site for coarctation, more instances have been reported in the last fifteen years than in the hundred years after the first description by Quain in 1847 [3]. About fifty cases are reported in the world literature and only about twenty patients have undergone reconstructive surgery [4,5]. A greater awareness of the condition and more refined diagnostic technics have played an important role in more clearly delineating this disease as a cause of hypertension. The purpose of this paper is to report on a patient having an area of coarctation just below the level of the renal arteries, who presented with hypertension and intermittent claudication, and in whom there was complete relief of signs and symptoms after reconstructive sur-
C
Aorta
OARCTATION
gery. CASEREPORT A fifty-three year old man (J. F., GHUP No. 308066) was admitted to the Graduate Hospital of the University of Pennsylvania because of intermittent claudication involving the right leg. Approximately ten months prior to admission, he began to experience pain in the posterior aspect of the right thigh and calf upon walking only half a block. Occasionally, similar symptoms developed in the left leg which were always less severe than those on the right. History was essentially noncontributory except that for two years prior to admission, he had been having decreased libido. Physical examination revealed the blood pressure to be 175/100 mm. Hg in the right arm, 170/105 mm. Hg in the left arm, and unobtainable in both legs. He had a grade I hypertensive retinopathy on 224
American
Journal of Surgery
Coarctation
of Abdominal
Aorta
225
~;rc. 1. Left retrograde brachial aortogram. Note the complete obstruction of the abdominal aorta at the level of the interspace of the first and second lumbar vertebrae. A large superior mesenteric arterv tillrd from the abdominal aorta. The inferior mesenteric artery fills through a large collateral mar&r1 artery of Drummond. There was no visualization of the distal abdominal aorta, iliac, or femoral arteries. FIG. 2. Schematic reproduction of the essential findings at operation. Beginning at the level of the renal arteries, the abdominal aorta was markedly narrowed measuring approximately 1 cm. in diameter, At approximately 3 cm. above the bifurcation the abdominal aorta became dilated measuring at least 2.5 cm. across, This dilated part continued down to the bifurcation. FIG. 3. Diagrammatic drawing of operative procedure. A Teflon graft was anastomosed end to side to the endarterectomized narrowed abdominal aorta approximately 1 cm. below the renal arteries, and both limbs were anastomosed to the common femoral arteries with end to side anastomosis.
To obtain control of the aorta above the renal arteries, the left side of the chest was entered through the seventh interspace, and the lower thoracic aorta was encircled with a tape. A longitudinal incision was made in the narrowed aorta just distal to the origin of the renal arteries, and moderately soft, organizing, grumous material was removed from about the orifices of the renal artery and then downwards for the length of the 2.5 cm. incision. A bifurcation Teflon@ prosthesis was anastomosed end to side to the narrowed abdominal aorta approximately 1 cm. below the renal arteries, and both limbs were anastomosed to the common femoral arteries at the level of the bifurcation. (Fig. 3.) The postoperative course was entirely uneventful. At the time of his discharge blood pressure in both arms was 130/70 mm. Hg, excellent dorsalis pedis and posterior tibia1 pulsations were present bilaterally, and the patient was completely free of symptoms. COMMENTS
Features which differentiate coarctation of the abdominal aorta from coarctation of the thoracic aorta are a systolic bruit heard best in the abdominal and lumbar region and complaints of intermittent claudication. Vol. 111, Februavy 1966
Etiology and Anatomy. Whether coarctation of the abdominal aorta is congenital or acquired is controversial. Maycock [6] suggested two possible causes: (1) the failure of the dorsal aortas to fuse equally at one point with consequent obliteration or loss of one of them and (2) kinking of the fused aortas with consequent localized increase in longitudinal tension producing a permanent narrowing. He considered the first explanation to be more probable. Bahnson, Cooley, and Sloan [7 ] believed that in their case stenosis was probably of long duration and congenital in origin, although the abrupt onset of symptoms related to deficient circulation in the lower extremities suggested thrombosis of the narrowed se,ment of the aorta. Coarctation of the abdominal aorta associated with Recklinghausen’s disease has been reported in two cases [8,9], and pathologic evidence of neurofibromatosis was found in tissue removed from the site of toarctation and elsewhere in both patients. Sen and his associates [IO] reported four
226
Bahabozorgui
cases at unusual sites of the aorta in which the coarctation or narrowing was caused by a stenosing panarteritis. All their patients were under the age of twenty and three of them were female patients. They mentioned that the diffuse character of narrowing, involvement of subclavian arteries in two cases, marked periaortic adhesions, and a characteristic histologic picture definitely pointed to an acquired lesion of inflammatory origin. They concluded that the lesion was probably of the nature of an allergic aortitis, and the over-all picture appeared to differ from that of giant cell arteritis, polyarteritis nodosa, and Buerger’s disease. Milloy and Fell [ll ] reported on four patients who had undergone surgery for elongated coarctation of the aorta. They described several differences between the usually encountered adult type of short coarctation of congenital origin and the elongated type of coarctation, which evidently may be congenital or acquired. The elongated type has been reported more commonly in female patients. The usual location of this type is below the isthmus and, when the vessel has been divided at surgery, the intimal and medial layers of the aortic wall have been observed to split apart with ease. D’Abreu, Rob, and Vollmar [12] divided coarctation of the abdominal aorta into two groups : segmentary coarctation and hypoplastic coarctation. These two groups are further divided into three subgroups: suprarenal, interrenal, and infrarenal coarctations according to the site of the beginning of the narrowed aortic segment. In the hypoplastic type there is no actual coarctation, but the abdominal aorta becomes narrow at a certain level and then remains hypoplastic down to the bifurcation. We believe that our case may be categorized under congenital type of abdominal coarctation. The chief collateral anastomoses in cases of coarctation of the abdominal aorta orginate from the lower intercostal arteries and internal mammary arteries to the caudal epigastric and lumbar arteries. Collateral network may also develop between visceral arteries from both sides of the coarctation, especially between the superior and inferior mesenteric artery and inferior mesenteric artery with hypogastric branches as in our case. Microscopically, Fisher and Corcoran [l3 1 observed thickening of the aortic intima and abnormal narrowing of the media at the site
and Nemir of coarctation. Focal cystic necrosis of the aortic wall occurred in the cases described by Albanese and Lazzarini [la]. The renal arteries and other major visceral arteries may originate from the narrowed segment, and they may be stenosed [4,13,15,16], hypoplastic [7,11,14,17], or occluded by thrombosis [18]. An aortic aneurysm distal to the coarctation has been noted [4,19], and aneurysms of the right renal artery in association with the coarctation have been reported [8,20]. The mechanism of aneurysm formation distal to coarctation of the aorta may be due to congenital weakness of the aortic wall which is aggravated by the turbulence, cavitation, and increased lateral pressure beyond the coarctation [4]. The signs and symptoms vary with the location and severity of the lesion, but those most frequently encountered are hypertension, heart failure, and circulatory insufficiency of the lower extremities. The patient with coarctation of the upper abdominal aorta tends to have severe hypertension with increased incidence of cerebrovascular accidents [21]. This is frequently due to added renal hypertension associated with involvement of the renal arteries by the occlusive process [22]. Moreover, this level of involvement appears to be associated with an increased propensity for cardiac failure and uremia [22]. Leg weakness and intermittent claudication are rare in coarctation of the upper abdominal aorta. The greater frequency of claudication in cases of coarctation of the lower abdominal aorta may be due to an inadequate development of the collateral circulation. Degeneration and thrombotic lesions in the stenosed aortic segment and in collateral anastomoses may have significance in the development of a circulatory insufficiency in the lower extremities. In two cases [16,18] in which intermittent claudication did occur, thrombotic obstruction of the aorta at the site of coarctation was revealed at autopsy. Intermittent claudication of relatively recent onset as observed in the patient herein reported was undoubtedly related to the thrombotic process in the stenotic segment . Other symptoms include headache, pericordial or epigastric pain, vertigo, palpitation, and slight dyspnea on exertion. Attacks closely simulating those caused by pheochromocytoma have been reported [23]. Anevican
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On physical examination, elevation of blood pressure in the arm, both systolic and diastolic, is significant. In the case reported by Kondo and associates [24] the blood pressure in the left arm was 40 mm. Hg and in the right arm was unobtainable because both subclavian arteries were hypoplastic. The blood pressure is lower in the legs than in the arms, but in cases of mild coarctation it may be the same in both upper and lower extremities. Femoral pulsations are usually diminished or absent. An important sign observed in some patients is the presence of strong aortic pulsations in the epigastrium and its disappearance more distally. This sign is especially valuable in the differential diagnosis of coarctations of the abdominal and the thoracic aorta [25]. A systolic murmur in the lower dorsal region and/or in the upper part of the abdomen may be of some help but is not always heard. Pathogenesis of Hypertension. The pathogenesis of hypertension in coarctation of the abdominal aorta is not clear. Patients with coarctation of the upper abdominal aorta seem to have more severe hypertension than patients with coarctations localized to the thoracic or lower abdominal aorta. This would suggest an association with renal hypertension of the Goldblatt type [26]. Deterioration of the clinical condition of these patients may begin at an early age [22]. Kirkendall, Culbertson, and Eckstein [27] believe that the mechanical factor of aortic coarctation is the most important in the genesis of the arterial hypertension. Impaired renal function has not been a significant factor in a series of patients with renal hypertension due to partial obstruction of the renal arteries of atheromatous-thrombotic [ZZ] or congenital origin, and normal kidney function need not rule out renal hypertension. Although aortography and intravenous pyelography may provide some indications of the renal functional state, they cannot be relied upon to provide the knowledge requisite to deciding the cause of hypertension. Neither the filtration rate nor the rate of renal blood ilow is ascertainable from the nephrogram or pyelogram. Knowledge of the individual renal functional capacities is important not only in the identification of an ischemic kidney but also in ascertaining the functional capacity of a nonischemic kidney upon which renal function will alone depend after removal of the ischemic one [28]. Vol. 111. February
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The fact that hypertension may occur in cases of coarctation of the abdominal aorta in which coarctation is located below the level of the renal arteries supports the belief that impairment of renal blood flow is not the cause of hypertension associated with coarctation. Diagnosis and Treatment. An unusual location of the coarctation should be suspected when, in the presence of clinical signs of coarctation of the aorta, the typical vascular deformity of the upper part of the mediastinum is lacking in conventional roentgenologic examination of the chest. Notching of the ribs is usually absent, but if it does occur it involves only the lower two or three ribs. Retrograde or translumbar aortography appears to be the best diagnostic method [29-311. Angiocardiography has been employed but does not give sufficient contrast filling of the abdominal aorta and may lead to an erroneous diagnosis. Aortography provides accurate delineation of the site of coarctation, its relationship to the origin of the renal arteries, and the length of the narrowed segment, information which is essential to the surgeon. In those cases presenting involvement of the renal arteries, a preoperative evaluation of renal function is important. The results of these investigations may well have a bearing on the type of surgical repair. Differentiation between coarctation of the abdominal aorta and coarctation of the thoracic aorta is not difficult if the possibility of the abdominal localization is kept in mind. This is important in view of the surgical treatment since the patient may be subjected to unnecessary thoracotomy [32,33 1. Acquired conditions producing stenosis or occlusion of the abdominal aorta must also be considered in differential diagnosis. These include atherosclerotic obstructive lesions with or without thrombosis, simple thrombosis or embolic occlusion, aneurysm or dissection involving the abdominal aorta and external pressure from a tumor. Acquired long-standing obstructive processes may sometimes give rise to the development of extensive collateral circulation, as shown by Bayliss [19]. Surgical Treatment. Glenn et al. [S] in 1952 performed the first successful corrective operation in a patient in whom the narrowed segment of the aorta was immediately proximal to the celiac axis and thus did not involve the renal arteries; the operation utilized the splenic
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artery as a bypass around the coarcted segment of the aorta. Since then, different surgical technics for this disease have been performed. In most instances, because of the location of the coarctation and its relation to the major visceral arteries, only bypassing of the coarcted segment has been attempted [34]. In a few cases, bypass graft to renal arteries has been performed [18,26,35]. In patients with coarctation of the abdominal aorta adjacent to renal arteries and probably also with coarctation located immediately above or below the renal arteries, surgical correction by application of a patch graft may be possible [35-371. In our case, endarterectomy above and below the renal arteries was performed after controlling the aorta above the renal arteries. Because of the close relation of the coarcted segment to the renal arteries, it was not feasible to resect the narrowed segment and insert a prosthesis. In several cases [7,8,12] a bilateral thoracolumbar sympathectomy or splanicectomy was performed before the diagnosis of coarctation was made. Similarly, lumbar sympathectomy was carried out in one case [23], and bilateral lumbar sympathectomy has been attempted in a few cases [7,38]. No definite benefit resulted from these operations. SUMMARY
and N emir
5.
6. 7.
8.
9.
10.
11.
12.
13.
14.
15.
AND CONCLUSIONS
A case is reported of coarctation of the abdominal aorta just below the renal artery manifested by intermittent claudication and hypertension. Obstruction was relieved by endarterectomy and bifurcation prosthesis. Etiology, clinical features, pathogenesis of hypertension, diagnosis, and surgical treatment of coarctation of the abdominal aorta are discussed. Coarctation of the aorta in unusual sites is being reported with greater frequency. An awareness of the possibility of this condition will lead to more accurate diagnosis and better management.
16. 17.
18.
19.
20.
21.
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