[Surgical Treatment of Cardiovascular Manifestations of Marfan's Syndrome]

Overview

Journal Herz

Specialty Cardiology & Vascular Diseases

Date 2000 Feb 1

PMID 10652677

Authors

R Lange

E EBERT

Affiliations

Soon will be listed here.

Abstract

Patients with Marfan's syndrome suffer mainly from the cardiovascular manifestations of the disease, in particular the acute dissection or rupture of the dilated aorta. Due to improved diagnostic and early surgical intervention the life expectancy of these patients could be considerably improved. However, rupture is still the most frequent and dissection the second frequent cause of death. Life threatening complications of aortic dissection are pericardial effusion, aortic insufficiency and malperfusion syndrome, due to obstruction of aortic branches. Dissection of the ascending aorta is treated by implantation of a valved conduit with reimplantation of the coronary arteries. Some surgeons favor the complete replacement of the ascending aorta with preservation of the aortic valve, although long-term results show some development of aortic insufficiency after this procedure. Based on the experience of the last years, most surgeons prefer the prophylactic replacement of the aorta in Marfan patients, i.e. before complications have occurred. A special treatment algorithm helps to define the indication for the operative treatment in different manifestations of the disease. The low mortality of the elective replacement of the ascending aorta in contrast to replacement in emergency cases speaks in favor of the early operative treatment. For the long-term prognosis of the patient a closed and continuous cardiologic surveillance is mandatory. The patient should be close to a center with the necessary diagnostic tools and with sufficient experience with the medical and surgical treatment, in order to further improve the life expectancy in the future.

References

Pyeritz R . Marfan syndrome: current and future clinical and genetic management of cardiovascular manifestations. Semin Thorac Cardiovasc Surg. 1993; 5(1):11-6. View

Roman M, Devereux R, Niles N, Hochreiter C, Kligfield P, Sato N . Aortic root dilatation as a cause of isolated, severe aortic regurgitation. Prevalence, clinical and echocardiographic patterns, and relation to left ventricular hypertrophy and function. Ann Intern Med. 1987; 106(6):800-7. DOI: 10.7326/0003-4819-106-6-800. View

Lange R, Thielmann M, Schmidt K, Bauernschmitt R, Jakob H, HASPER B . Spinal cord protection using hypothermic cardiocirculatory arrest in extended repair of recoarctation and persistent hypoplastic aortic arch. Eur J Cardiothorac Surg. 1997; 11(4):697-702. DOI: 10.1016/s1010-7940(96)01114-1. View

Svensson L, Crawford E, Coselli J, Safi H, Hess K . Impact of cardiovascular operation on survival in the Marfan patient. Circulation. 1989; 80(3 Pt 1):I233-42. View

David T . An anatomic and physiologic approach to acquired heart disease. 8th annual meeting of the European Cardio-thoracic Association, The Hague, Netherlands, September 25-28, 1994. Eur J Cardiothorac Surg. 1995; 9(4):175-80. DOI: 10.1016/s1010-7940(05)80141-1. View

Fleischer K, Nousari H, Anhalt G, Stone C, Laschinger J . Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan's syndrome. Ann Thorac Surg. 1997; 63(4):1012-7. DOI: 10.1016/s0003-4975(97)00061-1. View

Fuzellier J, Chauvaud S, Fornes P, Berrebi A, Lajos P, Bruneval P . Surgical management of mitral regurgitation associated with Marfan's syndrome. Ann Thorac Surg. 1998; 66(1):68-72. DOI: 10.1016/s0003-4975(98)00314-2. View

Beighton P, SUJANSKY E, Patzak B, Portele K . Genetic skeletal dysplasias in the Museum of Pathological Anatomy, Vienna. Am J Med Genet. 1993; 47(6):843-7. DOI: 10.1002/ajmg.1320470609. View

David T, Feindel C . An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992; 103(4):617-21; discussion 622. View

10.

Gott V, Laschinger J, CAMERON D, Dietz H, Greene P, Gillinov A . The Marfan syndrome and the cardiovascular surgeon. Eur J Cardiothorac Surg. 1996; 10(3):149-58. DOI: 10.1016/s1010-7940(96)80289-2. View

11.

Crawford E . Marfan's syndrome. Broad spectral surgical treatment cardiovascular manifestations. Ann Surg. 1983; 198(4):487-505. PMC: 1353192. DOI: 10.1097/00000658-198310000-00009. View

12.

Smith J, Fann J, Miller D, Moore K, Deanda Jr A, Mitchell R . Surgical management of aortic dissection in patients with the Marfan syndrome. Circulation. 1994; 90(5 Pt 2):II235-42. View

13.

Crawford E, Crawford J, STOWE C, Safi H . Total aortic replacement for chronic aortic dissection occurring in patients with and without Marfan's syndrome. Ann Surg. 1984; 199(3):358-62. PMC: 1353405. DOI: 10.1097/00000658-198403000-00018. View

14.

Sarsam M, Yacoub M . Remodeling of the aortic valve anulus. J Thorac Cardiovasc Surg. 1993; 105(3):435-8. View

15.

Coady M, Rizzo J, Hammond G, Mandapati D, Darr U, Kopf G . What is the appropriate size criterion for resection of thoracic aortic aneurysms?. J Thorac Cardiovasc Surg. 1997; 113(3):476-91; discussion 489-91. DOI: 10.1016/S0022-5223(97)70360-X. View

16.

Kouchoukos N, Dougenis D . Surgery of the thoracic aorta. N Engl J Med. 1997; 336(26):1876-88. DOI: 10.1056/NEJM199706263362606. View

17.

Gott V, Pyeritz R, CAMERON D, Greene P, MCKUSICK V . Composite graft repair of Marfan aneurysm of the ascending aorta: results in 100 patients. Ann Thorac Surg. 1991; 52(1):38-44; discussion 44-5. DOI: 10.1016/0003-4975(91)91414-q. View

18.

Crawford E, Coselli J . Marfan's syndrome: combined composite valve graft replacement of the aortic root and transaortic mitral valve replacement. Ann Thorac Surg. 1988; 45(3):296-302. DOI: 10.1016/s0003-4975(10)62466-6. View

19.

Shores J, Berger K, Murphy E, Pyeritz R . Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome. N Engl J Med. 1994; 330(19):1335-41. DOI: 10.1056/NEJM199405123301902. View

20.

McDonald G, Schaff H, Pyeritz R, MCKUSICK V, Gott V . Surgical management of patients with the Marfan syndrome and dilatation of the ascending aorta. J Thorac Cardiovasc Surg. 1981; 81(2):180-6. View