HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): John C. Mullen Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mullen, J. C. Articles by Bentley, M. J. Search for Related Content PubMed PubMed Citation Articles by Mullen, J. C. Articles by Bentley, M. J.

Ann Thorac Surg 1996;62:1830-1831
© 1996 The Society of Thoracic Surgeons

---

Case Report

Recurrent Aortic Dissection After Orthotopic Heart Transplantation

Division of Cardiothoracic Surgery, The University of Alberta, Edmonton, Alberta, Canada

Accepted for publication June 15, 1996.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
A patient with Marfan's syndrome and previous Bentall repair for aortic dissection required orthotopic cardiac transplantation for end-stage cardiomyopathy. Postoperatively he suffered recurrent aortic dissection involving the transverse and descending aorta leading to tracheal and esophageal compression. He underwent successful surgical replacement of his ascending aorta, transverse arch, and descending aorta.

	Introduction

Top Footnotes Abstract Introduction Comment References

 
A select group of patients with Marfan's syndrome who have undergone surgical correction for aneurysmal dilatation or dissection of the aorta may ultimately require heart transplantation for end-stage heart failure [1–3]. Posttransplantation morbidity is substantial, with a 40% incidence of thoracic aortic dissection in operative survivors [3]. We describe a case of a patient with Marfan's syndrome after previous Bentall repair in whom end-stage cardiomyopathy developed and who underwent successful heart transplantation. This was complicated by recurrent dissection 19 days postoperatively, which led to life-threatening tracheal compression. Surgical repair involved replacement of his ascending aorta, transverse arch, and descending aorta.

In 1983 a 22-year-old man with Marfan's syndrome underwent a Bentall procedure for acute aortic dissection. Congestive heart failure developed in December 1992, and end-stage cardiomyopathy diagnosed. The patient's history included spontaneous pneumothorax, renal calculi, lens dislocation, and a 25 pack-year history of smoking. Cardiac catheterization revealed aneurysmal dilatation of the left main coronary artery, and a localized calcific aneurysm of the innominate artery with a false lumen above the Dacron graft, which was attached to the undersurface of the transverse aortic arch. He had frequent readmissions with congestive heart failure, and suffered a cardiac arrest from which he was resuscitated in July 1994.

In August 1994 he underwent orthotopic heart transplantation. His recovery was uncomplicated; he remained normotensive and was discharged 13 days postoperatively. Six days later, he was readmitted with severe chest pain, presyncope, shortness of breath, and was severely hypertensive. Labetalol and sodium nitroprusside therapy was instituted and computed tomographic scan confirmed acute extension of his chronic dissection. His condition worsened over the next several days: creatinine levels steadily increased (peak, 450 µmol/L), and stridor developed. A repeat computed tomographic scan showed increasing size of the descending aortic dissection with extension to the iliac vessels, and tracheal compression between the transverse arch and descending aortic aneurysms (Fig 1). Dysphagia developed in addition to severe stridor. His creatinine level gradually decreased back to 160 µmol/L, and the decision was made to proceed with replacement of the transverse arch and the ascending and descending aorta.

View larger version (97K): [in this window] [in a new window]   Fig 1. . Computed tomographic scan of the thorax shows aneurysmal enlargement of transverse arch and descending aorta compressing the trachea.

He received antithymocyte globulin for 5 days postoperatively, and cyclosporine was withheld for 2 days. A transesophageal echocardiogram showed excellent myocardial function. He enjoyed a smooth and progressive recovery and was discharged on the 12th postoperative day. Eighteen months postoperatively he continued to do very well, was normotensive, and was enjoying a full and active life.

	Comment

Top Footnotes Abstract Introduction Comment References

 
Aortic root replacement with coronary reimplantation has improved survival in patients with Marfan's syndrome and ascending aortic aneurysms [1–4]. Postoperatively recurrent true aneurysms, persistent dissections, and pseudoaneurysm formation are not infrequent [5]. Despite surgical correction a certain percentage of patients experience progressive cardiac deterioration and become transplant candidates.

Kesler and associates [3] reported a survey of active cardiac transplant centers regarding results of transplantation in patients with Marfan's syndrome. Thirty patients were evaluated, 13 were listed, and 11 received transplants, with an operative mortality of 9.1%. Morbidity was significant, with a 40% incidence of thoracic aortic dissection (one fatal). Concerns over future complications or the presence of significant aortic dilatation were predominant reasons given for refusal to list these patients for cardiac transplantation.

Crawford [6] reported 41 patients with Marfan's syndrome requiring cardiovascular operations. Reoperations were frequent (49%), particularly after treatment of aortic dissection. It was thought that many of the late deaths may have been prevented by more careful follow-up and more aggressive antihypertensive therapy.

The mechanisms of posttransplantation aortic aneurysm formation are unclear. It has been suggested that the increased stress placed on the aorta by the transplanted heart may be a primary factor [3, 7]. Other possible contributors are the collagen-weakening effect of immunosuppression [3], increased tissue collagenase reported to predispose patients to abdominal aortic aneurysms after major operations [8], and hypertension secondary to immunosuppression [3].

In this patient the presence of a strong new donor heart combined with existing chronic aortic dissection led to extensive acute dissection on top of chronic aortic dissection, which progressed despite maximal intensive medical management. The decision to proceed with the operation to save his life was difficult due to the extent of repair required, his renal dysfunction, the requirement for ongoing immunosuppression, the short interval since his heart transplantation (and likelihood of severe adhesions), and the concern over myocardial protection for the new donor heart.

A number of important techniques were employed during the operation to maximize cerebral, myocardial, renal, and spinal cord protection. A clamshell thoracotomy was employed to provide full access to the ascending, transverse, and descending aorta, as well as the heart. He was cooled systemically to 15°C to minimize metabolic requirements. A bridge connection between the femoral arterial line and superior vena caval cannula allowed for continuous retrograde cerebral and distal aortic perfusion. The heart received continuous perfusion by cold antegrade blood cardioplegia, and the left ventricle was vented. Postoperatively the blood pressure was tightly controlled (systolic blood pressure <110 mm Hg), and cyclosporine was withheld for the first several days until normal renal function was assured.

A transplant center's ability to survey for and successfully treat major vascular complications associated with Marfan's syndrome is important to obtain survival statistics sufficient to justify transplantation [3]. The decision to list a patient with Marfan's syndrome with a previous aortic dissection is therefore a difficult one and must be individually determined, based on institutional experience and expertise.

	Footnotes

Top Footnotes Abstract Introduction Comment References

 
Address reprint requests to Dr Mullen, Departments of Surgery and Pediatrics, The University of Alberta Hospital, 2D2.18 WC Mackenzie Health Sciences Centre, 8440 112 St, Edmonton, Alberta T6G 2B7, Canada.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Gott VL, Pyeritz RE, Magovern GJ, Cameron DE, McKusick VA. Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. N Engl J Med 1986;314:1070–4.
2. Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med 1972;286:804–8.
3. Kesler KA, Hanosh JJ, O'Donnell J, et al. Heart transplantation in patients with Marfan's syndrome: a survey of attitudes and results. J Heart Lung Transplant 1994;13:899–904.[Medline]
4. Marsalese D, Moodie D, Vaconte M, et al. Marfan's syndrome: natural history and long-term follow-up of cardiovascular involvement. J Am Coll Cardiol 1989;14:422–8.[Abstract]
5. Taniguchi K, Nakano S, Matsuda H, et al. Long-term survival and complications after composite graft replacement for ascending aortic aneurysm associated with aortic regurgitation. Circulation 1991;84(Suppl 3):31–9.
6. Crawford ES. Marfan's syndrome: broad spectrum surgical treatment cardiovascular manifestations. Ann Surg 1983;198:487–505.[Medline]
7. Bull DA, Neumayer LA, Venerus BJ, et al. The effects of improved hemodynamics on aortic dimensions in patients undergoing heart transplantation. J Vasc Surg 1994;20:539–45.[Medline]
8. Swanson RJ, Littooy FN, Hunt TK, Stoney RJ. Laparotomy as a precipitating factor in the rupture of intra-abdominal aneurysms. Arch Surg 1980;115:299–304.[Abstract/Free Full Text]

This article has been cited by other articles:

				A Kiotsekoglou, G R Sutherland, J C Moggridge, D K Nassiri, A J Camm, and A H Child The unravelling of primary myocardial impairment in Marfan syndrome by modern echocardiography Heart, October 1, 2009; 95(19): 1561 - 1566. [Abstract] [Full Text] [PDF]

				C. Knosalla, Y.-g. Weng, R. Hammerschmidt, M. Pasic, I. Schmitt-Knosalla, O. Grauhan, M. Dandel, H. B. Lehmkuhl, and R. Hetzer Orthotopic Heart Transplantation in Patients With Marfan Syndrome Ann. Thorac. Surg., May 1, 2007; 83(5): 1691 - 1695. [Abstract] [Full Text] [PDF]

				V. S. Reddy, H. H. Phan, R. N. Pierson III, D. C. Drinkwater Jr, P. A. Chang, S. F. Davis, and W. H. Merrill Late cardiac reoperation after cardiac transplantation Ann. Thorac. Surg., February 1, 2002; 73(2): 534 - 537. [Abstract] [Full Text] [PDF]

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): John C. Mullen Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mullen, J. C. Articles by Bentley, M. J. Search for Related Content PubMed PubMed Citation Articles by Mullen, J. C. Articles by Bentley, M. J.