Background The aortic root enlarges progressively in Marfan'ssyndrome, and this enlargement is associated with aortic regurgitationand dissection. Long-term treatment with -adrenergic blockade,by reducing the impulse (i.e., the rate of pressure change inthe aortic root) of left ventricular ejection and the heartrate, may protect the aortic root.
Methods We conducted an open-label, randomized trial of propranololin adolescent and adult patients with classic Marfan's syndrome(32 treated and 38 untreated [control] patients). Aortic-rootdimensions and clinical end points (aortic regurgitation, aorticdissection, cardiovascular surgery, congestive heart failure,and death) were monitored for an average of 9.3 years in thecontrol group and 10.7 years in the treatment group. All 70patients were included in the analysis according to the intention-to-treatprinciple.
Results The dose of propranolol was individualized; the mean(±SE) dose was 212 ±68 mg per day. The mean slopeof the regression line for the aortic-root dimensions, whichreflect the rate of dilatation, was significantly lower in thetreatment group than in the control group (0.023 vs. 0.084 peryear, P<0.001). Clinical end points were reached in fivepatients in the treatment group and nine in the control group.The Kaplan-Meier survival curve for the treatment group differedsignificantly from that for the control group during the middleyears of the trial and remained better for the treatment groupthroughout the study.
Conclusions Prophylactic -adrenergic blockade is effective inslowing the rate of aortic dilatation and reducing the developmentof aortic complications in some patients with Marfan's syndrome.
Source Information
From the Center for Medical Genetics, Johns Hopkins University School of Medicine, Baltimore.
Address reprint requests to Dr. Pyeritz at the Department of Human Genetics, Allegheny-Singer Research Institute, 320 E. North Ave., Pittsburgh, PA 15212-4772.
Brooke, B. S., Habashi, J. P., Judge, D. P., Patel, N., Loeys, B., Dietz, H. C. III
(2008). Angiotensin II Blockade and Aortic-Root Dilation in Marfan's Syndrome. NEJM
358: 2787-2795
[Abstract][Full Text]
Pyeritz, R. E.
(2008). A Small Molecule for a Large Disease. NEJM
358: 2829-2831
[Full Text]
Faivre, L, Collod-Beroud, G, Child, A, Callewaert, B, Loeys, B L, Binquet, C, Gautier, E, Arbustini, E, Mayer, K, Arslan-Kirchner, M, Stheneur, C, Kiotsekoglou, A, Comeglio, P, Marziliano, N, Halliday, D, Beroud, C, Bonithon-Kopp, C, Claustres, M, Plauchu, H, Robinson, P N, Ades, L, De Backer, J, Coucke, P, Francke, U, De Paepe, A, Boileau, C, Jondeau, G
(2008). Contribution of molecular analyses in diagnosing Marfan syndrome and type I fibrillinopathies: an international study of 1009 probands. J. Med. Genet.
45: 384-390
[Abstract][Full Text]
Keane, M. G., Pyeritz, R. E.
(2008). Medical Management of Marfan Syndrome. Circulation
117: 2802-2813
[Full Text]
Williams, B.
(2008). The year in hypertension.. J Am Coll Cardiol
51: 1803-1817
[Full Text]
Chung, A. W.Y., Yang, H. H. C., Radomski, M. W., van Breemen, C.
(2008). Long-Term Doxycycline Is More Effective Than Atenolol to Prevent Thoracic Aortic Aneurysm in Marfan Syndrome Through the Inhibition of Matrix Metalloproteinase-2 and -9. Circ. Res.
102: e73-e85
[Abstract][Full Text]
Williams, A, Davies, S, Stuart, A G, Wilson, D G, Fraser, A G
(2008). Medical treatment of Marfan syndrome: a time for change. Heart
94: 414-421
[Abstract][Full Text]
Matt, P., Habashi, J., Carrel, T., Cameron, D. E., Van Eyk, J. E., Dietz, H. C.
(2008). Recent advances in understanding Marfan syndrome: should we now treat surgical patients with losartan?. J. Thorac. Cardiovasc. Surg.
135: 389-394
[Abstract][Full Text]
Enriquez-Sarano, M., Nkomo, V. T., Michelena, H.
(2008). Principles and Practice of Echocardiography in Cardiac Surgery. Card Surg Adult
3: 315-348
[Full Text]
Brinster, D. R., Rizzo, R. J., Bolman, R. M. III
(2008). Ascending Aortic Aneurysms. Card Surg Adult
3: 1223-1250
[Full Text]
O'Rourke, M. F.
(2007). Arterial aging: pathophysiological principles. Vasc Med
12: 329-341
[Abstract]
Ahimastos, A. A., Aggarwal, A., D'Orsa, K. M., Formosa, M. F., White, A. J., Savarirayan, R., Dart, A. M., Kingwell, B. A.
(2007). Effect of Perindopril on Large Artery Stiffness and Aortic Root Diameter in Patients With Marfan Syndrome: A Randomized Controlled Trial. JAMA
298: 1539-1547
[Abstract][Full Text]
Zierer, A., Voeller, R. K., Hill, K. E., Kouchoukos, N. T., Damiano, R. J. Jr, Moon, M. R.
(2007). Aortic Enlargement and Late Reoperation After Repair of Acute Type A Aortic Dissection. Ann. Thorac. Surg.
84: 479-487
[Abstract][Full Text]
Kalkat, M. S., Rahman, I., Kotidis, K., Davies, B., Bonser, R. S.
(2007). Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm. Eur. J. Cardiothorac. Surg.
32: 250-254
[Abstract][Full Text]
O'Rourke, M. F., Hashimoto, J.
(2007). Mechanical Factors in Arterial Aging: A Clinical Perspective. J Am Coll Cardiol
50: 1-13
[Abstract][Full Text]
von Kodolitsch, Y., Robinson, P. N
(2007). Marfan syndrome: an update of genetics, medical and surgical management. Heart
93: 755-760
[Full Text]
Percheron, G., Fayet, G., Ningler, T., Le Parc, J.-M., Denot-Ledunois, S., Leroy, M., Raffestin, B., Jondeau, G.
(2007). Muscle strength and body composition in adult women with Marfan syndrome. Rheumatology (Oxford)
46: 957-962
[Abstract][Full Text]
Graham Stuart, A., Williams, A.
(2007). Marfan's syndrome and the heart. Arch. Dis. Child.
92: 351-356
[Abstract][Full Text]
Weiss, R. M.
(2007). Lasting Effects of Lost Vascular Elasticity. Circ. Res.
100: 604-606
[Full Text]
Scharfschwerdt, M., Sievers, H.-H., Greggersen, J., Hanke, T., Misfeld, M.
(2007). Prosthetic Replacement of the Ascending Aorta Increases Wall Tension in the Residual Aorta. Ann. Thorac. Surg.
83: 954-957
[Abstract][Full Text]
Svensson, L. G., Blackstone, E. H., Feng, J., de Oliveira, D., Gillinov, A. M., Thamilarasan, M., Grimm, R. A., Griffin, B., Hammer, D., Williams, T., Gladish, D. H., Lytle, B. W.
(2007). Are Marfan Syndrome and Marfanoid Patients Distinguishable on Long-Term Follow-Up?. Ann. Thorac. Surg.
83: 1067-1074
[Abstract][Full Text]
Aburawi, E. H., O'Sullivan, J.
(2007). Relation of aortic root dilatation and age in Marfan's syndrome. Eur Heart J
28: 376-379
[Abstract][Full Text]
Authors/Task Force Members, , Vahanian, A., Baumgartner, H., Bax, J., Butchart, E., Dion, R., Filippatos, G., Flachskampf, F., Hall, R., Iung, B., Kasprzak, J., Nataf, P., Tornos, P., Torracca, L., Wenink, A., ESC Committee for Practice Guidelines (CPG), , Priori, S. G., Blanc, J.-J., Budaj, A., Camm, J., Dean, V., Deckers, J., Dickstein, K., Lekakis, J., McGregor, K., Metra, M., Morais, J., Osterspey, A., Tamargo, J., Zamorano, J. L., Document Reviewers, , Zamorano, J. L., Angelini, A., Antunes, M., Fernandez, M. A. G., Gohlke-Baerwolf, C., Habib, G., McMurray, J., Otto, C., Pierard, L., Pomar, J. L., Prendergast, B., Rosenhek, R., Uva, M. S., Tamargo, J.
(2007). Guidelines on the management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J
0: ehl428v1-39
[Full Text]
Bondy, C. A., for The Turner Syndrome Consensus Study Group,
(2007). Care of Girls and Women with Turner Syndrome: A Guideline of the Turner Syndrome Study Group. J. Clin. Endocrinol. Metab.
92: 10-25
[Abstract][Full Text]
Cheung, Y F, Ou, X, Wong, S J
(2006). Central and peripheral arterial stiffness in patients after surgical repair of tetralogy of Fallot: implications for aortic root dilatation. Heart
92: 1827-1830
[Abstract][Full Text]
Baumgartner, D., Baumgartner, C., Schermer, E., Engl, G., Schweigmann, U., Matyas, G., Steinmann, B., Stein, J. I.
(2006). Different patterns of aortic wall elasticity in patients with Marfan syndrome: A noninvasive follow-up study. J. Thorac. Cardiovasc. Surg.
132: 811-819
[Abstract][Full Text]
Bonow, R. O., Carabello, B. A., Chatterjee, K., de Leon, A. C. Jr, Faxon, D. P., Freed, M. D., Gaasch, W. H., Lytle, B. W., Nishimura, R. A., O'Gara, P. T., O'Rourke, R. A., Otto, C. M., Shah, P. M., Shanewise, J. S., Smith, S. C. Jr, Jacobs, A. K., Adams, C. D., Anderson, J. L., Antman, E. M., Faxon, D. P., Fuster, V., Halperin, J. L., Hiratzka, L. F., Hunt, S. A., Lytle, B. W., Nishimura, R., Page, R. L., Riegel, B.
(2006). ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. J Am Coll Cardiol
48: e1-e148
[Full Text]
Bonow, R. O., Carabello, B. A., Chatterjee, K., de Leon, A. C. Jr, Faxon, D. P., Freed, M. D., Gaasch, W. H., Lytle, B. W., Nishimura, R. A., O'Gara, P. T., O'Rourke, R. A., Otto, C. M., Shah, P. M., Shanewise, J. S., Smith, S. C. Jr, Jacobs, A. K., Adams, C. D., Anderson, J. L., Antman, E. M., Faxon, D. P., Fuster, V., Halperin, J. L., Hiratzka, L. F., Hunt, S. A., Lytle, B. W., Nishimura, R., Page, R. L., Riegel, B.
(2006). ACC/AHA 2006 Practice Guidelines for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. J Am Coll Cardiol
48: 598-675
[Full Text]
Jauch, E. C., Lindsell, C. J., Adeoye, O., Khoury, J., Barsan, W., Broderick, J., Pancioli, A., Brott, T.
(2006). Lack of Evidence for an Association Between Hemodynamic Variables and Hematoma Growth in Spontaneous Intracerebral Hemorrhage. Stroke
37: 2061-2065
[Abstract][Full Text]
Habashi, J. P., Judge, D. P., Holm, T. M., Cohn, R. D., Loeys, B. L., Cooper, T. K., Myers, L., Klein, E. C., Liu, G., Calvi, C., Podowski, M., Neptune, E. R., Halushka, M. K., Bedja, D., Gabrielson, K., Rifkin, D. B., Carta, L., Ramirez, F., Huso, D. L., Dietz, H. C.
(2006). Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome.. Science
312: 117-121
[Abstract][Full Text]
Van de Velde, S., Fillman, R., Yandow, S.
(2006). Protrusio Acetabuli in Marfan Syndrome. History, Diagnosis, and Treatment. JBJS
88: 639-646
[Abstract][Full Text]
Neri, E., Barabesi, L., Buklas, D., Vricella, L. A., Benvenuti, A., Tucci, E., Sassi, C., Massetti, M.
(2005). Limited role of aortic size in the genesis of acute type A aortic dissection. Eur. J. Cardiothorac. Surg.
28: 857-863
[Abstract][Full Text]
Nesta, F., Leyne, M., Yosefy, C., Simpson, C., Dai, D., Marshall, J. E., Hung, J., Slaugenhaupt, S. A., Levine, R. A.
(2005). New Locus for Autosomal Dominant Mitral Valve Prolapse on Chromosome 13: Clinical Insights From Genetic Studies. Circulation
112: 2022-2030
[Abstract][Full Text]
Warnes, C. A.
(2005). The Adult With Congenital Heart Disease: Born To Be Bad?. J Am Coll Cardiol
46: 1-8
[Abstract][Full Text]
Morishita, K., Kawaharada, N., Fukada, J., Kurimoto, Y., Fujisawa, Y., Saito, T., Abe, T.
(2005). Midterm Results of Surgical Treatment of Thoracic Aortic Disease in Dialysis Patients. Ann. Thorac. Surg.
80: 96-100
[Abstract][Full Text]
De Cicco, G., Lorusso, R., Colli, A., Nicolini, F., Fragnito, C., Grimaldi, T., Borrello, B., Budillon, A. M., Gherli, T., Beghi, C.
(2005). Aortic Valve Periprosthetic Leakage: Anatomic Observations and Surgical Results. Ann. Thorac. Surg.
79: 1480-1485
[Abstract][Full Text]
Maron, B. J., Ackerman, M. J., Nishimura, R. A., Pyeritz, R. E., Towbin, J. A., Udelson, J. E.
(2005). Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol
45: 1340-1345
[Full Text]
Kim, S. Y., Martin, N., Hsia, E. C., Pyeritz, R. E., Albert, D. A.
(2005). Management of Aortic Disease in Marfan Syndrome: A Decision Analysis. Arch Intern Med
165: 749-755
[Abstract][Full Text]
O'Rourke, M. F., Nichols, W. W.
(2005). Aortic Diameter, Aortic Stiffness, and Wave Reflection Increase With Age and Isolated Systolic Hypertension. Hypertension
45: 652-658
[Full Text]
Milewicz, D. M., Dietz, H. C., Miller, D. C.
(2005). Treatment of Aortic Disease in Patients With Marfan Syndrome. Circulation
111: e150-e157
[Full Text]
Isselbacher, E. M.
(2005). Thoracic and Abdominal Aortic Aneurysms. Circulation
111: 816-828
[Full Text]
van Dijk, N., Immink, R. V., Mulder, B. J.M., van Lieshout, J. J., Wieling, W.
(2005). Orthostatic blood pressure control in Marfan's syndrome. Europace
7: 25-27
[Abstract][Full Text]
Meijboom, L. J., Westerhof, B. E., Nollen, G. J., Spaan, J. A.E., de Mol, B. A.J.M., Jacobs, M. J.H.M., Mulder, B. J.M.
(2004). Beta-blocking therapy in patients with the Marfan syndrome and entire aortic replacement. Eur. J. Cardiothorac. Surg.
26: 901-906
[Abstract][Full Text]
Oliver, J. M., Gallego, P., Gonzalez, A., Aroca, A., Bret, M., Mesa, J. M.
(2004). Risk factors for aortic complications in adults with coarctation of the aorta. J Am Coll Cardiol
44: 1641-1647
[Abstract][Full Text]
Enriquez-Sarano, M., Tajik, A. J.
(2004). Aortic Regurgitation. NEJM
351: 1539-1546
[Full Text]
Nollen, G. J., Groenink, M., Tijssen, J. G.P., van der Wall, E. E., Mulder, B. J.M.
(2004). Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome. Eur Heart J
25: 1146-1152
[Abstract][Full Text]
Creager, M. A., Jones, D. W., Easton, J. D., Halperin, J. L., Hirsch, A. T., Matsumoto, A. H., O'Gara, P. T., Safian, R. D., Schwartz, G. L., Spittell, J. A.
(2004). Atherosclerotic Vascular Disease Conference: Writing Group V: Medical Decision Making and Therapy. Circulation
109: 2634-2642
[Full Text]
Nienaber, C A, Richartz, B M, Rehders, T, Ince, H, Petzsch, M
(2004). Aortic intramural haematoma: natural history and predictive factors for complications. Heart
90: 372-374
[Full Text]
Boutouyrie, P., Germain, D. P., Fiessinger, J.-N., Laloux, B., Perdu, J., Laurent, S.
(2004). Increased Carotid Wall Stress in Vascular Ehlers-Danlos Syndrome. Circulation
109: 1530-1535
[Abstract][Full Text]
Nollen, G J, Westerhof, B E, Groenink, M, Osnabrugge, A, van der Wall, E E, Mulder, B J M
(2004). Aortic pressure-area relation in Marfan patients with and without {beta} blocking agents: a new non-invasive approach. Heart
90: 314-318
[Abstract][Full Text]
Nienaber, C. A., Eagle, K. A.
(2003). Aortic Dissection: New Frontiers in Diagnosis and Management: Part II: Therapeutic Management and Follow-Up. Circulation
108: 772-778
[Full Text]
Carrel, T., Berdat, P., Pavlovic, M., Sukhanov, S., Englberger, L., Pfammatter, J.-P.
(2003). Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results. Eur. J. Cardiothorac. Surg.
24: 249-254
[Abstract][Full Text]
Steinmetz, E. F., Buckley, C., Thompson, R. W.
(2003). Prospects for the Medical Management of Abdominal Aortic Aneurysms. VASC ENDOVASCULAR SURG
37: 151-163
[Abstract]
von Kodolitsch, Y., Csosz, S. K., Koschyk, D. H., Schalwat, I., Loose, R., Karck, M., Dieckmann, C., Fattori, R., Haverich, A., Berger, J., Meinertz, T., Nienaber, C. A.
(2003). Intramural Hematoma of the Aorta: Predictors of Progression to Dissection and Rupture. Circulation
107: 1158-1163
[Abstract][Full Text]
Yetman, A. T., Bornemeier, R. A., McCrindle, B. W.
(2003). Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death?. J Am Coll Cardiol
41: 329-332
[Abstract][Full Text]
Anderson, C. A., Rizzo, R. J., Cohn, L. H.
(2003). Ascending Aortic Aneurysms. Card Surg Adult
2: 1123-1148
[Full Text]
Niwa, K., Siu, S. C., Webb, G. D., Gatzoulis, M. A.
(2002). Progressive Aortic Root Dilatation in Adults Late After Repair of Tetralogy of Fallot. Circulation
106: 1374-1378
[Abstract][Full Text]
Iung, B., Gohlke-Barwolf, C., Tornos, P., Tribouilloy, C., Hall, R., Butchart, E., Vahanian, A.
(2002). Recommendations on the management of the asymptomatic patient with valvular heart disease. Eur Heart J
23: 1253-1266
Dean, J. C S
(2002). Management of Marfan syndrome. Heart
88: 97-103
[Full Text]
Wityk, R. J., Zanferrari, C., Oppenheimer, S.
(2002). Neurovascular Complications of Marfan Syndrome: A Retrospective, Hospital-Based Study. Stroke
33: 680-684
[Abstract][Full Text]
Svensson, L. G., Khitin, L.
(2002). Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome. J. Thorac. Cardiovasc. Surg.
123: 360-361
[Full Text]
Loeys, B., Nuytinck, L., Delvaux, I., De Bie, S., De Paepe, A.
(2001). Genotype and Phenotype Analysis of 171 Patients Referred for Molecular Study of the Fibrillin-1 Gene FBN1 Because of Suspected Marfan Syndrome. Arch Intern Med
161: 2447-2454
[Abstract][Full Text]
Alexiou, C., Langley, S. M., Charlesworth, P., Haw, M. P., Livesey, S. A., Monro, J. L.
(2001). Aortic root replacement in patients with Marfan's syndrome: the Southampton experience. Ann. Thorac. Surg.
72: 1502-1507
[Abstract][Full Text]
Oosterhof, T., Groenink, M., Hulsmans, F.-J., Mulder, B. J. M., van der Wall, E. E., Smit, R., Hennekam, R. C. M.
(2001). Quantitative Assessment of Dural Ectasia as a Marker for Marfan Syndrome. Radiology
220: 514-518
[Abstract][Full Text]
Genoni, M., Paul, M., Jenni, R., Graves, K., Seifert, B., Turina, M.
(2001). Chronic {beta}-blocker therapy improves outcome and reduces treatment costs in chronic type B aortic dissection. Eur. J. Cardiothorac. Surg.
19: 606-610
[Abstract][Full Text]
van Karnebeek, C D M, Naeff, M S J, Mulder, B J M, Hennekam, R C M, Offringa, M
(2001). Natural history of cardiovascular manifestations in Marfan syndrome. Arch. Dis. Child.
84: 129-137
[Abstract][Full Text]
Robinson, P. N, Godfrey, M.
(2000). The molecular genetics of Marfan syndrome and related microfibrillopathies. J. Med. Genet.
37: 9-25
[Abstract][Full Text]
Birks, E. J., Webb, C., Child, A., Radley-Smith, R., Yacoub, M. H.
(1999). Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome. Circulation
100: II-29-35
[Abstract][Full Text]
Groenink, M, Lohuis, T A J, Tijssen, J G P, Naeff, M S J, Hennekam, R C M, van der Wall, E E, Mulder, B J M
(1999). Survival and complication free survival in Marfan's syndrome: implications of current guidelines. Heart
82: 499-504
[Abstract][Full Text]
Walton, L. J., Franklin, I. J., Bayston, T., Brown, L. C., Greenhalgh, R. M., Taylor, G. W., Powell, J. T.
(1999). Inhibition of Prostaglandin E2 Synthesis in Abdominal Aortic Aneurysms : Implications for Smooth Muscle Cell Viability, Inflammatory Processes, and the Expansion of Abdominal Aortic Aneurysms. Circulation
100: 48-54
[Abstract][Full Text]
Nistri, S, Sorbo, M D, Marin, M, Palisi, M, Scognamiglio, R, Thiene, G
(1999). Aortic root dilatation in young men with normally functioning bicuspid aortic valves. Heart
82: 19-22
[Abstract][Full Text]
Westaby, S.
(1999). Aortic dissection in Marfan's syndrome. Ann. Thorac. Surg.
67: 1861-1863
[Abstract][Full Text]
Coady, M. A., Rizzo, J. A., Hammond, G. L., Kopf, G. S., Elefteriades, J. A.
(1999). Surgical intervention criteria for thoracic aortic aneurysms: a study of growth rates and complications. Ann. Thorac. Surg.
67: 1922-1926
[Abstract][Full Text]
Jondeau, G., Boutouyrie, P., Lacolley, P., Laloux, B., Dubourg, O., Bourdarias, J.-P., Laurent, S.
(1999). Central Pulse Pressure Is a Major Determinant of Ascending Aorta Dilation in Marfan Syndrome. Circulation
99: 2677-2681
[Abstract][Full Text]
Gott, V. L., Greene, P. S., Alejo, D. E., Cameron, D. E., Naftel, D. C., Miller, D. C., Gillinov, A. M., Laschinger, J. C., Pyeritz, R. E.
(1999). Replacement of the Aortic Root in Patients with Marfan's Syndrome. NEJM
340: 1307-1313
[Abstract][Full Text]
Devereux, R. B., Roman, M. J.
(1999). Aortic Disease in Marfan's Syndrome. NEJM
340: 1358-1359
[Full Text]
Wilson, D. G., Bellamy, M. F., Ramsey, M. W., Goodfellow, J., Brownlee, M., Davies, S., Wilson, J. F., Lewis, M. J., Stuart, A. G.
(1999). Endothelial Function in Marfan Syndrome : Selective Impairment of Flow-Mediated Vasodilation. Circulation
99: 909-915
[Abstract][Full Text]
Clouse, W. D., Hallett, J. W. Jr, Schaff, H. V., Gayari, M. M., Ilstrup, D. M., Melton, L. J. III
(1998). Improved Prognosis of Thoracic Aortic Aneurysms: A Population-Based Study. JAMA
280: 1926-1929
[Abstract][Full Text]
Maron, B. J., Moller, J. H., Seidman, C. E., Vincent, G. M., Dietz, H. C., Moss, A. J., Towbin, J. A., Sondheimer, H. M., Pyeritz, R. E., McGee, G., Epstein, A. E.
(1998). Impact of Laboratory Molecular Diagnosis on Contemporary Diagnostic Criteria for Genetically Transmitted Cardiovascular Diseases: Hypertrophic Cardiomyopathy, Long-QT Syndrome, and Marfan Syndrome : A Statement for Healthcare Professionals From the Councils on Clinical Cardiology, Cardiovascular Disease in the Young, and Basic Science, American Heart Association. Circulation
98: 1460-1471
[Full Text]
Groenink, M, Rozendaal, L, Naeff, M S J, Hennekam, R C M, Hart, A A M, van der Wall, E E, Mulder, B J M
(1998). Marfan syndrome in children and adolescents: predictive and prognostic value of aortic root growth for screening for aortic complications. Heart
80: 163-169
[Abstract][Full Text]
Huang, W., Gelas, F. A., Osborne-Pellegrin, M. J.
(1998). Protection of the Arterial Internal Elastic Lamina by Inhibition of the Renin-Angiotensin System in the Rat. Circ. Res.
82: 879-890
[Abstract][Full Text]
SM, S. C. S., Sermer, M., Harrison, D. A., Grigoriadis, E., Liu, G., Sorensen, S., BS, J. F. S., Farine, D., Amankwah, K. S., Spears, J. C., Colman, J. M.
(1997). Risk and Predictors for Pregnancy-Related Complications in Women With Heart Disease. Circulation
96: 2789-2794
[Abstract][Full Text]
Gillinov, A. M., Zehr, K. J., Redmond, J. M., Gott, V. L., Deitz, H. C., Reitz, B. A., Laschinger, J. C., Cameron, D. E.
(1997). Cardiac Operations in Children With Marfan's Syndrome: Indications and Results. Ann. Thorac. Surg.
64: 1140-1144
[Abstract][Full Text]
Fleischer, K. J., Nousari, H. C., Anhalt, G. J., Stone, C. D., Laschinger, J. C.
(1997). Immunohistochemical Abnormalities of Fibrillin in Cardiovascular Tissues in Marfan's Syndrome. Ann. Thorac. Surg.
63: 1012-1017
[Abstract][Full Text]
Xu, Q., Fawcett, T. W., Udelsman, R., Holbrook, N. J.
(1996). Activation of Heat Shock Transcription Factor 1 in Rat Aorta in Response to High Blood Pressure. Hypertension
28: 53-57
[Abstract][Full Text]
Cusimano, R. J.
(1996). Repeat Cardiac Operation in a Patient With Osteogenesis Imperfecta. Ann. Thorac. Surg.
61: 1294-1294
[Full Text]
Xu, Q., Li, D.-g., Holbrook, N. J., Udelsman, R.
(1995). Acute Hypertension Induces Heat-Shock Protein 70 Gene Expression in Rat Aorta. Circulation
92: 1223-1229
[Abstract][Full Text]
Milewicz, D. M.
(1995). Ultrasonic Characterization of the Aortic Architecture in Marfan Patients. Circulation
91: 1272-1274
[Full Text]
Pereira, L., Levran, O., Ramirez, F., Lynch, J. R., Sykes, B., Pyeritz, R. E., Dietz, H. C.
(1994). A Molecular Approach to the Stratification of Cardiovascular Risk in Families with Marfan's Syndrome. NEJM
331: 148-153
[Abstract][Full Text]
(1994). PROPHYLACTIC PROPRANOLOL IN MARFAN'S SYNDROME. JWatch General
1994: 4-4
[Full Text]
Francke, U., Furthmayr, H.
(1994). Marfan's Syndrome and Other Disorders of Fibrillin. NEJM
330: 1384-1385
[Full Text]
-adrenergic blockade, by reducing the impulse (i.e., the rate of pressure change in the aortic root) of left ventricular ejection and the heart rate, may protect the aortic root. 
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