Marfan's syndrome medical therapy
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Editors-In-Chief: William James Gibson, C. Michael Gibson, M.S., M.D.; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [1] ; Assistant Editor-In-Chief: Cassandra Abueg, M.P.H. [2]
Overview
Medical therapy for Marfan syndrome focuses on measures to delay the progression of cardiovascular complications of the disease. Once sufficient progression has occurred, surgical interventions become necessary.
Medical Therapy
Drug therapies for Marfan syndrome focus on decreasing the mechanical forces to which the aorta is exposed. Beta blockers are the standard of care for adults. These drugs decrease myocardial contractility and as an extension, decrease blood pressure. In 1994, a study was published comparing the beta blocker propanol to placebo. Patients receiving propanol experienced a significant four-fold reduction in aortic root dilation and significantly decreased mortality in the middle of the study (around 5 years). However, at the end of the study, there was no significant difference between the groups in mortality. Evidence for use of beta blockers in children is less clear.
Recent studies of Losartan in children have proven promising in preventing cardiovascular complication of the syndrome. A 2008 study of 18 children with severe aortic root dilation who failed treatment with other medical therapy were treated with losartan. The rate of aortic root dilation was dramatically reduced after the initiation of therapy (3.54+/- 2.87 mm per year versus 0.46 +/-0.62 mm per year, p<.001). These findings have not yet been confirmed in a randomized trial. Clinical trials are currently underway comparing standard beta blocker therapy (Atenolol, Nebivolol) to therapy with Angiotensin Receptor Blockers (Losartan) [1], [2].
Even with the advent of new therapies based on angiotensin receptor blockade, patients are still at risk of aortic dissection It is recommended that most patients do not engage in vigorous physical activity. Contact sports such as football, ice hockey are strongly discouraged in addition to surfing and snorkeling. Activities that increase intrathoracic pressure such as weight lifting are also strongly discouraged. Exercise of low to moderate intensity, four to six metabolic equivalents, is permissible.
Clinical trials have been conducted of the drug acetazolamide in the treatment of symptoms of dural ectasia. The treatment has demonstrated significant functional improvements in some sufferers.[3] Other medical treatments, as well as physical therapy, are also available.
Treatment of a spontaneous pneumothorax is dependant on the volume of air in the pleural space and the natural progression of the individual's condition. A small pneumothorax might resolve without active treatment in 1 to 2 weeks. Recurrent pneumothoraxes might require chest surgery. Moderately sized pneumothoraxes might need chest drain management for several days in hospital. Large pneumothoraxes are likely to be medical emergencies requiring emergency decompression.
2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Evaluation and Management of Aortic Dissection (DO NOT EDIT) [4]
Blood Pressure Control (DO NOT EDIT) [4]
| Class I |
| "1. Beta adrenergic–blocking drugs should be administered to all patients with Marfan syndrome and aortic aneurysm to reduce the rate of aortic dilatation unless contraindicated. (Level of Evidence: B)" |
| Class IIa |
| "1. An angiotensin receptor blocker (losartan) is reasonable for patients with Marfan syndrome, to reduce the rate of aortic dilatation unless contraindicated. (Level of Evidence:B)" |
References
- ↑ Gambarin FI, Favalli V, Serio A, Regazzi M, Pasotti M, Klersy C, Dore R, Mannarino S, Viganò M, Odero A, Amato S, Tavazzi L, Arbustini E (2009). "Rationale and design of a trial evaluating the effects of losartan vs. nebivolol vs. the association of both on the progression of aortic root dilation in Marfan syndrome with FBN1 gene mutations". Journal of Cardiovascular Medicine (Hagerstown, Md.). 10 (4): 354–62. doi:10.2459/JCM.0b013e3283232a45. PMID 19430350. Retrieved 2010-12-22. Unknown parameter
|month=ignored (help) - ↑ Lacro RV, Dietz HC, Wruck LM, Bradley TJ, Colan SD, Devereux RB, Klein GL, Li JS, Minich LL, Paridon SM, Pearson GD, Printz BF, Pyeritz RE, Radojewski E, Roman MJ, Saul JP, Stylianou MP, Mahony L (2007). "Rationale and design of a randomized clinical trial of beta-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome". American Heart Journal. 154 (4): 624–31. doi:10.1016/j.ahj.2007.06.024. PMID 17892982. Retrieved 2010-12-22. Unknown parameter
|month=ignored (help) - ↑ "Dural Ectasia in the Marfan Spine: Symptoms and Treatment". Scoliosis Research Society.
- ↑ 4.0 4.1 Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE; et al. (2010). "2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine". Circulation. 121 (13): e266–369. doi:10.1161/CIR.0b013e3181d4739e. PMID 20233780.