Aortic stenosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Mohammed A. Sbeih, M.D. [2]; Lakshmi Gopalakrishnan, M.B.B.S. [3]
Overview
Left untreated, aortic valve stenosis can lead to angina, syncope, congestive heart failure, atrial fibrillation, endocarditis, and sudden cardiac death. Surgical treatment of aortic stenosis also carries risks and potential complications that include vascular complications and mitral valve injury.
Natural History
Degenerative Calcific Aortic Stenosis
Aortic stenosis due to the degeneration of a calcified aortic valve has a prolonged latent period during which symptoms are minimal or even lacking [1]. This form of aortic stenosis presents later in life, usually after the age of 75 [2]. Once moderate aortic stenosis is present and symptomatic, the average rate of progression of the valvular stenosis is a decrease in the valve area of 0.1 cm2 per year [3][1]. In addition, there is an increase in the jet velocity of 0.3 m / second per year and an increase in the mean pressure gradient of 7 mm Hg per year [4] [5][6]. There is a tremendous individual variability in the rate of progression of aortic stenosis. Risk factors for atherosclerosis (such as age, smoking, hypertension, obesity and diabetes, lipid abnormalities, chronic renal failure and dialysis) and atherosclerotic disease itself (such as concomitant coronary artery disease) are associated with more rapid rates of progression of the severity of the aortic stenosis.
Aortic Stenosis Due to Rheumatic Heart Disease
These patients generally become symptomatic after the sixth decade.
Bicuspid Aortic Valve Disease
Bicuspid aortic valve stenosis presents one or two decades earlier than the tricuspid aortic valva. The rate of progression of degenerative aortic stenosis can be faster in patients wit bicuspid aoric valva than in those with congenital or rheumatic disease [7]. Bicuspid aortic valve functions without any significant pressure gradient during childhood. However, the thickening and calcification of the valves may be detectable pathologically and on echocardiography by the second decade[8]. Approximately 75% of bicuspid aortic valves progress into aortic stenosis requiring operative correction.[9][10]
Bicuspid aortic stenosis progressively leads to heart failure, arrythmias, angina and other symptoms. These symptoms generally manifests between 40 to 60 years of age, which is relatively a younger age than that of the manifestation of the symptoms caused by aortic stenosis.[9] However, children who develop early pathological changes in bicuspid aortic valve are more likely to develop aortic insufficiency than stenosis.
Aortic Sclerosis
Aortic sclerosis (defined as aortic valve thickening without obstruction to ventricular outflow) may progress to narrowing of the aortic valve or aortic stenosis. The decrease in pulse pressure or upstroke of the pulse in a patient with aortic sclerosis is a sign of progression to aortic stenosis.
Complications
Degenerative Calcific Aortic Stenosis
If left untreated, aortic stenosis may lead to complications such as angina, syncope, or heart failure. A complete list of complications of aortic stenosis includes the following:
- Angina
- Arrhythmias
- Atrial fibrillation
- Bleeding: Impaired platelet function and coagulation abnormalities, as decreased levels of Von Willebrand factor, can be seen in most patients with severe AS. This resolves after valve replacement procedure. 20% of patients have clinical bleeding, most often epistaxis or ecchymoses [11]Aortic stenosis may result in a form of von Willebrand disease due to an increased turbulence around the stenosed aortic valve which subsequently triggers a break down of coagulation factor VIII-associated antigen, (also called von Willebrand factor) and results in a variant of von Willebrand disease.
- Congestive heart failure, particularly left-sided heart failure or systolic dysfunction
- Endocarditis
- Fainting or syncope: Since the stenosed aortic valve may limit the heart's output, people with aortic stenosis are at risk of syncope and dangerously low blood pressure with the use of some common medications. Ironically, these same medicines are used to treat a variety of cardiovascular diseases, many of which may co-exist with aortic stenosis. Examples include nitroglycerin, nitrates, ACE inhibitors, terazosin (Hytrin), and hydralazine. Note that all of these substances lead to peripheral vasodilation. Normally, however, in the absence of aortic stenosis, the heart is able to increase its output and thereby offset the effect of the dilated blood vessels. However, some cases of aortic stenosis can be associated with outflow blood obstruction which can prevent an increase of the cardiac output. Hence, low blood pressure or syncope may ensue.
- Left ventricular hypertrophy
- Myocardial infarction
Bicuspid Aortic Valve Disease
Bicuspid aortic valve disease is associated with the following complications:
- Aortic stenosis in the majority of patients (75%).[9]
- Aortic insufficiency[12][13]
- Endocarditis[14]
- Aortic aneurysm[10]
- Aortic dissection[10]
- Sudden death can occur in children during and immediately after exertion especially among those with pressure gradient > 50 mmHg across the aortic valve.[15]
Prognosis
Asymptomatic Patients
The prognosis of patients with aortic stenosis who do not have symptoms is quite good [16]. The annual mortality rate is < 1% per year in asymptomatic patients. Only 4% of sudden cardiac deaths that occur in patients with aortic stenosis occur in those patients who are asymptomatic.
Symptomatic Patients
Medical treatment of newly diagnosed moderate to severe symptomatic aortic stenosis is associated with a 25% mortality at one year, and a 50% mortality at two years. Half of the deaths are due to sudden cardiac death [17] [18].
When aortic stenosis is left untreated, the average survival is 5 years after the onset of angina, 3 years after the onset of syncope, and 1 year after the onset of congestive heart failure [17][19][20].
Low Flow, Low Gradient, Low Ejection Fraction Aortic Stenosis
If ventricular systolic dysfunction is present, there may be only a moderate transvalvular gradient or low flow aortic stenosis. The presence of fibrosis in the left ventricle may cause an incomplete recovery after aortic valve replacement[21] . This scenario can also occur among patients in whom there is a history of myocardial infarction due to the absence of sufficient contractility to mount an aortic gradient. It may also occur when myocardial fibrosis develops due to longstanding aortic stenosis.
Definition
- An aortic valve areas < 1.0 cm2
- A left ventricular ejection fraction < 40%
- A mean pressure difference or gradient across the aortic valve of < 30 mm Hg
- The aortic valve area should increase to more than 1.2 cm2 with a dobutamine infusion and the mean pressure gradient should rise above 30 mm Hg. While early surgical mortality is 32-33% in patients who fail to to achieve these improvements with dobutamine infusions, it is only 5–7% in patients who demonstrate an increase in their contractility and gradient. Five years survival after surgery was 88% in patients with improved contractility and only 10–25% in patients with unimproved contractility.
References
- ↑ 1.0 1.1 Faggiano P, Aurigemma GP, Rusconi C, Gaasch WH (1996). "Progression of valvular aortic stenosis in adults: literature review and clinical implications". Am Heart J. 132 (2 Pt 1): 408–17. PMID 8701905.
- ↑ Townsend CM, et al. Sabiston Textbook of Surgery. 18th ed. Saunders; 2008:1841-1844.
- ↑ Zoghbi WA, Enriquez-Sarano M, Foster E, Grayburn PA, Kraft CD, Levine RA; et al. (2003). "Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography". J Am Soc Echocardiogr. 16 (7): 777–802. doi:10.1016/S0894-7317(03)00335-3. PMID 12835667.
- ↑ Cheitlin MD, Gertz EW, Brundage BH, Carlson CJ, Quash JA, Bode RS (1979). "Rate of progression of severity of valvular aortic stenosis in the adult". Am Heart J. 98 (6): 689–700. PMID 495418.
- ↑ Jonasson R, Jonsson B, Nordlander R, Orinius E, Szamosi A (1983). "Rate of progression of severity of valvular aortic stenosis". Acta Med Scand. 213 (1): 51–4. PMID 6829320.
- ↑ Peter M, Hoffmann A, Parker C, Lüscher T, Burckhardt D (1993). "Progression of aortic stenosis. Role of age and concomitant coronary artery disease". Chest. 103 (6): 1715–9. PMID 8404089.
- ↑ Rosenhek R, Binder T, Porenta G, Lang I, Christ G, Schemper M; et al. (2000). "Predictors of outcome in severe, asymptomatic aortic stenosis". N Engl J Med. 343 (9): 611–7. doi:10.1056/NEJM200008313430903. PMID 10965007.
- ↑ Beppu S, Suzuki S, Matsuda H, Ohmori F, Nagata S, Miyatake K (1993). "Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves". The American Journal of Cardiology. 71 (4): 322–7. PMID 8427176. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ 9.0 9.1 9.2 Fenoglio JJ, McAllister HA, DeCastro CM, Davia JE, Cheitlin MD (1977). "Congenital bicuspid aortic valve after age 20". The American Journal of Cardiology. 39 (2): 164–9. PMID 835475. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ 10.0 10.1 10.2 Lewin MB, Otto CM (2005). "The bicuspid aortic valve: adverse outcomes from infancy to old age". Circulation. 111 (7): 832–4. doi:10.1161/01.CIR.0000157137.59691.0B. PMID 15723989. Retrieved 2012-04-10. Unknown parameter
|month=ignored (help) - ↑ Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F; et al. (2003). "Acquired von Willebrand syndrome in aortic stenosis". N Engl J Med. 349 (4): 343–9. doi:10.1056/NEJMoa022831. PMID 12878741.
- ↑ Keane MG, Wiegers SE, Plappert T, Pochettino A, Bavaria JE, Sutton MG (2000). "Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions". Circulation. 102 (19 Suppl 3): III35–9. PMID 11082359. Retrieved 2012-04-10. Unknown parameter
|month=ignored (help) - ↑ Roberts WC, Morrow AG, McIntosh CL, Jones M, Epstein SE (1981). "Congenitally bicuspid aortic valve causing severe, pure aortic regurgitation without superimposed infective endocarditis. Analysis of 13 patients requiring aortic valve replacement". The American Journal of Cardiology. 47 (2): 206–9. PMID 7468467. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Gersony WM, Hayes CJ, Driscoll DJ, Keane JF, Kidd L, O'Fallon WM, Pieroni DR, Wolfe RR, Weidman WH (1993). "Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect". Circulation. 87 (2 Suppl): I121–6. PMID 8425318. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Keane JF, Driscoll DJ, Gersony WM, Hayes CJ, Kidd L, O'Fallon WM, Pieroni DR, Wolfe RR, Weidman WH (1993). "Second natural history study of congenital heart defects. Results of treatment of patients with aortic valvar stenosis". Circulation. 87 (2 Suppl): I16–27. PMID 8425319. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Lancellotti P, Magne J, Donal E, et al. Clinical outcome in asymptomatic severe aortic stenosis insights from the new proposed aortic stenosis grading classification. J Am Coll Cardiol. Jan 17 2012;59(3):235-43.
- ↑ 17.0 17.1 Ross J, Braunwald E (1968). "Aortic stenosis". Circulation. 38 (1 Suppl): 61–7. PMID 4894151.
- ↑ Chizner MA, Pearle DL, deLeon AC (1980). "The natural history of aortic stenosis in adults". Am Heart J. 99 (4): 419–24. PMID 7189084.
- ↑ Kelly TA, Rothbart RM, Cooper CM, Kaiser DL, Smucker ML, Gibson RS (1988). "Comparison of outcome of asymptomatic to symptomatic patients older than 20 years of age with valvular aortic stenosis". Am J Cardiol. 61 (1): 123–30. PMID 3337000.
- ↑ Iivanainen AM, Lindroos M, Tilvis R, Heikkilä J, Kupari M (1996). "Natural history of aortic valve stenosis of varying severity in the elderly". Am J Cardiol. 78 (1): 97–101. PMID 8712130.
- ↑ Carabello BA, Green LH, Grossman W, Cohn LH, Koster JK, Collins JJ (1980). [[]]: 42–8 http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=7379284. Retrieved 2012-04-10. Unknown parameter
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