coarctation in 15 patients aged 50 to 63 years (mean, 54 years) ..... the beneficial effect of coarctation repair in patients older than 50 ... providing the illustration.
Benefits of Surgical Repair of Coarctation of the Aorta in Patients Older Than 50 Years Matthias Bauer, MD, Vladimir V. Alexi-Meskishvili, MD, PhD, Ulrike Bauer, MD, Diab Alfaouri, MD, Peter E. Lange, MD, PhD, and Roland Hetzer, MD, PhD Departments of Cardiothoracic and Vascular Surgery, and Congenital Heart Diseases, Deutsches Herzzentrum Berlin, Berlin, Germany
Background. Most patients with unrepaired coarctation of the aorta die before the age of 50 years. In patients who present at an older age, the indications for surgical treatment are controversial because the benefits of operating are unclear. Methods. At follow-up investigation from 0.5 to 11.5 years (mean, 4 years) after primary surgical correction of coarctation in 15 patients aged 50 to 63 years (mean, 54 years), we analyzed the preoperative and postoperative complications, symptoms, need for antihypertensive drugs, and blood pressure at rest and during exercise. Results. Preoperatively no patient had normal blood pressure at rest despite combined antihypertensive med-
ication. There was no significant mortality or morbidity after repair. At follow-up examination only 3 patients had at rest mild hypertension, the other 12 patients were normotensive. Of the 11 tested patients, 8 displayed systolic arterial hypertension during exercise. Conclusions. Surgical correction of coarctation can be performed after the age of 50 years with low surgical risk. Operation reduces systolic hypertension at rest and permits more effective medical treatment. Despite persistence of the hypertension during exercise, symptomatic improvement occurs in most patients. (Ann Thorac Surg 2001;72:2060 – 4) © 2001 by The Society of Thoracic Surgeons
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nosis of the coarctation was established before 10 years of age in 5 patients, between 11 and 30 years in 5 patients, between 31 and 50 years in 2 patients, and after 50 years of age in 3. Nine patients (60%) had associated cardiac diseases. The clinical data of the patients are presented in Table 1. Before the operation coronary angiography was performed in all patients to confirm the diagnosis of aortic coarctation. The operation was delayed in 12 patients: 7 patients initially had refused the operation, and in 5 patients conservative treatment only was recommended in other facilities. Surgical repair of the coarctation in 13 patients was performed by the use of a 16-mm to 22-mm prosthetic graft bypass from the left subclavian artery to the descending aorta without aortic cross-clamping. In 2 patients, because of the relatively small size of the left subclavian artery (⬍ 60% of the diameter of the descending aorta at the diaphragm), patch isthmoplasty was performed (aortic cross-clamp time was 10 and 12 minutes). Neither cardiopulmonary bypass nor intraaortic shunts were used. All 15 patients underwent follow-up examination in our institution 0.5 to 11.5 years (mean, 4 years) after operation. The recent follow-up data, which included clinical examination, interview about symptoms, and the need for antihypertensive medication, as well as blood pressure measurement at rest and during bicycle exercise testing, were analyzed. The oldest patient at follow-up examination was 71 years old. Systemic hypertension was defined when the right arm blood pressure exceeded 140/90 mm Hg. A residual gradient was defined as a systolic blood pressure gradi-
oarctation of the aorta has specific diagnostic and surgical problems that are typical for the infant, child, and adult. Because of the development of hypertensive heart and vascular disease, with severe local changes, produced by the coarctation, most older patients with uncorrected coarctation of the aorta die before the age of 50 years from complications such as congestive heart failure, myocardial infarction, aortic rupture, stroke, or infective endocarditis. They also have a higher surgical risk than younger patients without such complications [1, 2]. Although operation for coarctation of the aorta has been successfully performed for 55 years [3], many questions with regard to long-term survival, fate of arterial hypertension, and relief of symptoms remain unanswered, especially in older patients. There are only a few reports that deal with patients who were operated on at more than 50 years of age [4 – 6]. In this report we analyzed the long-term results of surgical repair of coarctation of the aorta in 15 patients older than 50 years of age.
Patients and Methods From April 1988 to August 2000, 15 patients aged 50 to 63 years (mean, 54 years), underwent primary surgical repair of coarctation of the aorta in our institution. DiagAccepted for publication July 16, 2001. Address reprint requests to Dr Matthias Bauer, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany; e-mail: mbauer@ dhzb.de.
© 2001 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
0003-4975/01/$20.00 PII S0003-4975(01)03094-6
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Table 1. Preoperative Clinical and Surgical Data
Patient No. Age (y)
Sex
Preoperative Upper Body Preoperative BP (cuff) PG (inv.) (mm Hg) (mm Hg)
1 2
53 61
Male Male
170/90 220/130
65 60
3 4 5 6 7
53 54 50 63 52
Male Male Female Female Female
225/100 145/70 180/100 210/130 230/110
116 60 40 50 80
8 9 10
53 50 52
Male Male Male
190/80 180/90 190/80
70 90 110
11 12 13
50 50 55
Male Male Male
160/100 150/80 200/100
40 30 80
14 15
54 60
Male Male
155/80 155/75
75 50
Associated CVD AI, AS* ...
Preoperative Preoperative Preoperative Antihypertensive Complications NYHA Class Medication Type of Operation
... LV dysfunction CAD Stroke ... ... ... Stroke AS, MI, CAD Endocarditis AS* LV dysfunction AS, MI Claudication CAD ... CAD LV dysfunction ... Claudication AS* ... ... LV dysfunction ... ... AI, VSD Claudication
II IV
A, C C, D, N
AP PB (16 mm)
II II III III IV
B, C, D, N A A, B, D A, D B, D
II II IV
A, B A, B, N ...
PB (18 mm) PB (16 mm) PB (18 mm)
II III IV
A, B A, B A, B, C, D
PB (22 mm) PB (22 mm) PB (18 mm)
II III
A, B, C, D A, B, C
PB (20 mm) PB (20 mm)
PB PB PB PB
(18 mm) (18 mm) (18 mm) (16 mm) AP
A ⫽ angiotensin-converting enzyme; AI ⫽ aortic valve insufficiency; AP ⫽ aortoplasty; AS ⫽ aortic valve stenosis; AS* ⫽ aortic valve BP ⫽ blood pressure; C ⫽ calcium antagonist; CAD ⫽ coronary artery stenosis with bicuspid aortic valve; B ⫽ -adrenergic blocker; disease; CVD ⫽ cardiovascular disease; D ⫽ diuretics; inv. ⫽ invasive; LV ⫽ left ventricle; MI ⫽ mitral valve insufficiency; N⫽ nitrate; NYHA ⫽ New York Heart Association; PB ⫽ prosthetic bypass; PG ⫽ pressure gradient; VSD ⫽ ventricular septal defect.
ent between the right arm and either leg of more than 20 mm Hg at rest.
Results Preoperatively all patients had exhibited arterial hypertension at rest. Medical control of hypertension was not possible despite triple or even quadruple antihypertensive drug regimens applied in most patients. Only 1 patient received no preoperative antihypertensive medication. All other patients were administered antihypertensive drugs to prevent cerebrovascular insults and cardiac events from extreme hypertension. The dosage of the medication was adjusted so that severe peripheral malperfusion did not occur. Several patients were treated with antihypertensive drugs by their general practitioners without coarctation having been diagnosed. In 9 patients (69%), systolic hypertension was severe (systolic blood pressure ⬎ 180 mm Hg). An invasively measured pressure gradient at the coarctation (30 to 116 mm Hg, mean 67.7 mm Hg) was observed preoperatively in all 15 patients (Table 1). The following complications occurred preoperatively in 7 patients (46%): 1 patient experienced aortic valve endocarditis, 2 patients had a cerebrovascular insult (in both cases without residual defects), and 4 patients had congestive heart failure (Table 1). There was no early or late mortality or neurologic complications after coarctation repair. Twelve patients (80%) exhibited paradoxical hypertension early after the operation, which was controlled in all
instances with sodium nitroprusside infusion and -adrenergic blockers. Other complications were not observed. Blood pressure before discharge was normal in 13 patients. Only 2 patients had mild hypertension (systolic blood pressure measured at right arm was 150/80 and 155/80 mm Hg). During the follow-up period there were no complications related to the operation or hypertension in any of the 15 patients. After operation there was no pressure gradient between upper and lower extremities at rest in 3 patients, whereas a mild gradient of less than 20 mm Hg was found in 12 patients (Table 2). Owing to severe aortic valve stenosis, 1 patient underwent aortic valve replacement with a mechanical prosthesis 4 years after the coarctation repair, and another patient underwent triple aortocoronary bypass operation 1 year after coarctation operation. At the last follow-up examination after 0.5 to 11.5 years (mean, 4 years), only 3 patients had mild hypertension; the other 12 patients (80%) were normotensive. Only 1 patient was postoperatively without the need for antihypertensive medication (Table 2). The extent of antihypertensive therapy was postoperatively reduced in all patients. Nine patients (60%) needed only one antihypertensive drug, such as a adrenergic blocker, an angiotensin-converting enzyme inhibitor, or a diuretic for the control of blood pressure (Table 2). Follow-up bicycle exercise testing was possible in 11 patients. In 3 patients exercise testing was not performed because of aortic valve stenosis, and 1 patient had un-
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Table 2. Postoperative Clinical Data BP at End of Exercise Early Duration BP at End of BP at End Late Test Postoperative of Hospital Stay of Hospital Postoperative (mm Hg)–Level Follow-up Postoperative Patient Paradoxical Intubation (arm) Stay (leg) BP of Period Antihypertensive NYHA Class No. Hypertension (d) (mm Hg) (mm Hg) (mm Hg) Exercise (W) (y) Medication Postoperatively 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
X X X X ... X X X X X ... X X X ...
1 1 1 1 1 1 1 1 1 3 1 1 1 1 1
125/80 130/85 150/80 120/70 155/80 120/75 110/60 110/60 110/80 140/80 120/70 120/70 135/70 120/70 130/60
145/90 125/80 145/70 110/70 145/80 120/70 100/60 100/60 120/80 130/80 110/60 115/75 130/60 120/70 125/65
125/80 145/90 150/80 130/80 155/80 125/80 100/70 120/70 120/80 130/85 120/80 120/70 130/70 130/70 135/60
A ⫽ angiotensin-converting enzyme inhibitor; B ⫽ -adrenergic blocker; N ⫽ nitrate; NYHA ⫽ New York Heart Association.
dergone a leg amputation after an accident. Eight of the 11 patients (72.7%) showed a pathologic blood pressure increase at low exercise levels (50 W) or developed severe arterial hypertension at higher exercise levels (systolic blood pressure ⬎ 195 mm Hg), which led to cessation of the exercise testing. In the other 2 patients the exercise testing was stopped because of dyspnea (patient 10) and muscle weakness (patient 11). The symptomatic state of the patients postoperatively significantly improved. Incidence of headache, palpitations, dyspnea, and angina pectoris significantly diminished. Claudication, which was observed preoperatively in 3 patients (age 50, 53, and 60 years), disappeared after the operation (Fig 1). Analysis according to the preoperative and postoperative New York Heart Association (NYHA) classes showed a distinct improvement of the clinical condition of the patients after surgical correction of coarctation.
Fig 1. Preoperative and postoperative symptoms of patients.
210/80–100 200/80–75 200/80–75 195/70–50 215/80–75 ... ... ... 195/80–50 150/80–25 170/70–100 ... 195/70–50 200/80–75 180/65–100 BP ⫽ blood pressure;
9 10 2 4 2 1 11 4 1 4 2 1 1 0,5 0,5
B C, D, N A A A, B B B A, B A A, B, D, N ... A A B, C D
C ⫽ calcium antagonist;
II II II II II II II II II III II II III II II D ⫽ diuretics;
Preoperatively most of the patients were in NYHA classes III or IV. Postoperatively 13 patients (86.6%) were in NYHA class II (Fig 2).
Comment This study contains the largest patient group that has undergone repair of coarctation of the aorta at more than 50 years of age to be reported in the literature to date. Other reports with regard to this age group are only casuistic [4, 5] or only a few patients older than 50 years are included in the reports on coarctation repair in adults [7–11]. It is well known that, to avoid late hypertension and to prevent recoarctation, the optimal age for elective aortic coarctation repair is approximately 1.5 years [12, 13].
Fig 2. New York Heart Association (NYHA) classification of the patients preoperatively and postoperatively.
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Fig 3. Operation field after completion of the subclavian artery to descending aorta bypass.
In our patients the preoperative pressure gradients varied from 25 to 118 mm Hg, but the pressure gradient was not decisive as an indication for operation. The goal of operation in this group of patients was to decrease the left ventricular afterload and to increase the possibility of more effective medical treatment of hypertension after relief of coarctation. The surgical approach by means of extraanatomic bypass techniques was first described by Blalock and Park in 1944 [14]. They performed an endto-side anastomosis of the left subclavian artery with the aorta distal to the stenosis. Subclavian artery to descending aorta prosthetic bypass (Fig 3) completely relieves aortic obstruction, can be performed without cardiopulmonary bypass, provides stable long-term results, and seems to be a life-long solution for this group of patients, as also reported in other studies [8, 15]. There is controversial discussion of the role of balloon angioplasty and stent implantation in patients with coarctation at different ages. Long-segment stenosis and complex forms of coarctation are less likely to respond to balloon dilatation. In adults the procedure has a high risk of aneurysm formation and aortic rupture. In this patient group, catheter dilatation should be used in cases with postsurgical recoarctation and patients with discrete coarctation [16, 17]. We found balloon dilatation not suitable in our group of patients with mostly complex forms of coarctation and calcification in the isthmic region in some cases. After operation using the subclavian artery to descending aorta prosthetic bypass, there was a distinct improvement in the clinical status of our patients. This supports the beneficial effect of coarctation repair in patients older than 50 years.
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Cohen and associates [18] found postoperative systolic blood pressure to be an independent predictor of late survival: there was a direct correlation between severity of postoperative systolic hypertension and the probability of premature death. According to published studies, the prevalence of late hypertension after correction of aortic coarctation in adults varies from 12% [7] to 50% [19, 20]. The percentage of patients with systemic hypertension increases with time after surgical repair of coarctation in adults. Presbitero and colleagues [21] reported that blood pressure was normal in most of the patients seen 10 years after operation, but later hypertension gradually became more frequent. Thirty years after coarctation repair only 32% of the patients are expected to be normotensive, which is much lower than the incidence in the normal population. Aris and coworkers [6] showed that all 8 patients in their study older than 50 years who underwent coarctation repair were normotensive after a mean interval of 4 years, and 5 patients did not need antihypertensive medication. Exercise testing was not performed because the authors showed in a previous study [22] that a hypertensive response to exercise only occurs if a residual gradient is present. Twelve of our 15 patients had normal blood pressure at rest postoperatively, and only 3 patients had mild hypertension (systolic pressure, 140 to 160 mm Hg). Exercise tests disclosed that in 8 of 11 patients tested, a pathologic blood pressure profile and exercise hypertension were found, despite the absence of a pressure gradient at the prosthesis. Therefore, residual stenosis may be excluded as a reason for the development of exercise hypertension. Exercise testing is mandatory in every patient after correction of coarctation to reveal latent hypertension and to optimize antihypertensive treatment. Postoperatively the need for antihypertensive drug treatment was significantly reduced in our patients. The study by Kaemmerer and associates [23], which included 41 patients aged 25.0 ⫾ 13.7 years at the time of operation with a mean interval between operation and the study of 11 ⫾ 4.9 years, showed that during exercise testing 20% exhibited pathologic blood pressure behavior, but that no patient with normal blood pressure at rest was hypertensive during exercise. These findings are contrary to ours in the older patients. Postoperatively most of our patients are in NYHA class II. Even 7 preoperatively severely compromised (NYHA classes III and IV) patients improved (Fig 2). Similarly to other groups [7], we found a high incidence of bicuspid aortic valves and coronary artery disease. One patient needed coronary artery bypass grafting, and 1 patient prosthetic aortic valve replacement during the follow-up period. In conclusion, our experience shows that correction of coarctation of the aorta can be performed safely and effectively in patients older than 50 years of age. Despite persistence of hypertension during exercise, symptomatic improvement occurs in most of the patients, and surgical correction of coarctation allowed a more effective medical treatment of residual hypertension. Correction
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BAUER ET AL REPAIR OF COARCTATION IN PATIENTS OLDER THAN 50 YEARS
of coarctation may therefore be performed in the older patient to prevent the complications of chronic arterial hypertension in the further course of the patient’s life. The authors thank Anne M. Gale for editorial assistance, Julia Stein for statistical advice, and Reinhold Giering-Jaensch for providing the illustration.
References 1. Ostermiller WE, Somerndike JM, Hunter J, et al. Coarctation of the aorta in adult patients. J Thorac Cardiocasv Surg 1969;61:125–30. 2. Campbell M. Natural history of coarctation of the aorta. Br Heart J 1970;32:633– 40. 3. Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J Thorac Surg 1945;14:347– 61. 4. Patel Y, Jilani MI, Cho K. Coarctation of the aorta presenting in a 79-year-old male. Thorac Cardiovasc Surg 1998;46:158 – 60. 5. Cayenne S, Sahgal P, Mistra VK, Conrad A, Jonas E. Asymptomatic patient with coarctation of the aorta presenting late in life: an unusual case and review. Cardiovasc Rev Rep 1996;17:48 –53. 6. Aris A, Subirana T, Ferries P, Torner-Sole M. Repair of aortic coarctation in patients more than 50 years of age. Ann Thorac Surg 1999;67:1376 –9. 7. Fraser RS, Stobey J, Rossall RE, Dvorkin J, Taylor RF. Coarctation of the aorta in adults. Can Med Assoc J 1976;115: 415–7. 8. Wells WJ, Prendergast TW, Berdjis F, et al. Repair of coarctation of the aorta in adults: the fate of systolic hypertension. Ann Thorac Surg 1996;61:1168 –71. 9. Lawrie GM, DeBakey ME, Morris GC, Crawford ES, Wagner WF, Glaeser DH. Late repair of coarctation of the descending thoracic aorta in 190 patients. Arch Surg 1981;116:1557– 60. 10. Clarkson PM, Nicholson MR, Barratt-Boyes BG, Neutze JM, Whitlock RM. Results after repair of coarctation f the aorta beyond infancy: a 10 to 28 year follow-up with particular reference to late systemic hypertension. Am J Cardiol 1983; 51:1481– 8.
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11. Vigano M, Ressia L, Gaeta R. Long-term follow-up after repair of coarctation of the aorta in adults. Ann Thorac Surg 1997;63:1827– 8. 12. Brouwer RM, Erasmus ME, Ebels T, Eijgelaar A. Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repair. Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years. J Thorac Cardiovasc Surg 1994;108:525–31. 13. Seirafi PA, Warner KG, Geggel RL, Payne DD, Cleveland RJ. Repair of coarctation of the aorta during infancy minimizes the risk of late hypertension. Ann Thorac Surg 1998;66:1378 – 82. 14. Blalock A, Park EA. Surgical treatment of experimental coarctation (atresia) of the aorta. Ann Surg 1944;119:445–56. 15. Grinda JM, Mace L, Dervanian P, Folliquet TA, Neveux JY. Bypass graft for complex forms of isthmic aortic coarctation in adults. Ann Thorac Surg 1995;60:1299 –302. 16. Gibbs JL. Treatment options for coarctation of the aorta. Heart 2000;84:11–3. 17. Fawzy ME, Sivanandam V, Galal O, et al. One- to ten-year follow-up results of balloon angioplasty of native coarctation of the aorta in adolescents, and adults. J Am Coll Cardiol 1997;30:1542– 6. 18. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction. Circulation 1989; 80:840 –5. 19. Westaby S, Parnell B, Pridie RB. Coarctation of the aorta in adults. Cinical presentation and results of surgery. J Cardiovasc Surg 1987;28:124 –7. 20. Maron BJ, O’Neal-Humphries J, Rowe RD, Melerts ED. Prognosis of surgically corrected coarctation of the aorta: a 20-year postoperative appraisal. Circulation 1973;47:119 –26. 21. Presbitero P, Demarie D, Villani M, et al. Long term results (15–30 years) of surgical repair of aortic coarctation. Br Heart J 1987;57:462–7. 22. Subirana MT, Borras X, Roca J, Serra JR, Torner-Soler M. Evolutio´ n de la hipertensio´ n arterial en pacientes operados de coarctatio´ n de aorta en edad adulta. Rev Esp Cardiol 1987;40(Suppl 1):9. 23. Kaemmerer H, Oelert F, Bahlmann J, Blucher S, Meyer GP, Mugge A. Arterial hypertension in adults after surgical treatment of aortic coarctation. Thorac Cardiovasc Surg 1998;46:121–5.