Background: Aortic stenosis (AS) is a common form of valvular heart disease that is graded based on cardiac testing (e.g., echocardiogram). Severe AS is defined by aortic valve area ≤1 cm², mean transaortic pressure gradient ≥ 40 mmHg, and peak aortic jet velocity ≥4 m/s. Despite improvements in access to aortic valve replacement (AVR), limitations to AVR such as difficult anatomy, functional status, and comorbidities remain common (1). This Fast Fact will discuss the illness trajectory of severe AS and the treatment options for severe AS.

Epidemiology: Globally the most common cause of AS is rheumatic fever, however in North America and Europe it is most commonly due to valvular degenerative calcification or a congenital bicuspid valve (2). In individuals aged 75 and older, the prevalence of AS is 12.4% and 3.4% for severe AS (3). Common risk factors for atherosclerosis (male gender, smoking, hypertension, hyperlipidemia) have also been associated with AS (4).

Disease trajectory and prognosis: SevereAS is initially characterized by an asymptomatic course that can last several years. Unless treated with an AVR, a progressive clinical decline ensues after the onset of symptoms. Angina, dyspnea, congestive heart failure, and syncope are commonly reported symptoms. The likelihood of remaining asymptomatic with severe AS in those who do not undergo AVR is 82% at 1 year, 67% at 2 years, and 33% at 5 years (5). The end-of-life illness trajectory of untreated AS resembles that of heart failure (see FF# 326). As such, recurrent hospitalizations, functional decline, and a poor quality of life are common (6,7). The survival rates in those who did not undergo AVR were 62% at 1 year, 32% at 5 years, and 18% at 10 years (8). Mortality risk increases with age, the presence of AS-related symptoms, and concomitant heart failure or renal insufficiency (9,10). Two-year mortality rates of 68% have been reported for symptomatic older patients with severe AS who do not undergo AVR (10).

Treatment options for severe AS:

• AVR: The only treatment option that improves symptoms and survival for severe AS is AVR (11). Indications for AVR are generally determined by symptom status, AS severity, and left ventricular ejection fraction (12). Colloquially, some experts suggest that the ideal timing for an AVR is the day before symptom onset so that the duration of the replaced valve and the patient’s quality of life are maximized. Since this is often impossible to achieve, AVR is usually considered soon after the onset of symptoms in severe AS. AVR options include surgical aortic valve replacement (SAVR) and transcatheter aortic valve replacement (TAVR). The decision of whether to do a SAVR vs TAVR is usually shared between the patient, surrogate, and a multidisciplinary team consisting of a cardiologist and cardiothoracic surgeon. The patient’s functional status, comorbidities, anatomy, life expectancy, stated goals of care, and STS-PROM score (Society of Thoracic Surgeons predicted risk of mortality) are usual factors in this decision (13,9). The role of AVR may be limited for patients with significant medical comorbidities such as lung disease or frailty or for whom valve disease is not the primary driver of symptoms. In fact, reports indicate renal dysfunction and advanced cardiopulmonary disease limit the benefit of TAVR, highlighting the importance of evaluating comorbid conditions when assessing for the benefits of AVR on symptoms and function (14).
• Non-interventional treatment options: The goal of most pharmacotherapy in AS is to treat concurrent medical conditions such as atrial fibrillation, coronary artery disease, volume overload, and hypertension, since there are no known medications which slow down the progression of AS (15). Common palliative-based interventions for AS-related symptom management include diuretics, supplemental oxygen, low dose opioids for dyspnea or angina, benzodiazepines for dyspnea-related anxiety, and behavioral strategies for dizziness, dyspnea, or syncope (10).

Serious illness discussions for patients with severe AS: Generalist clinicians and cardiovascular specialists should introduce discussions about care preferences, quality of life values, prognosis, and advance care planning when severe AS is diagnosed (14). Surrogate decision-makers should be identified via accessible written advance directives. If AVR is planned, a multidisciplinary team should help the patient devise a “valve preparedness plan” that anticipates the patient’s care preferences for common outcomes during and following the procedure. Best Case/Worst Case communication tools and palliative care specialists may be helpful resources for framing discussions on whether to pursue AVR (16). If AVR is not being pursued (due to patient preference or for medical reasons), clinicians should engage patients and caregivers in a serious illness discussion regarding the expected disease course, prognosis, and end-of-life care options (10,17). 

Hospice eligibility: Patients with severe AS should be provided with early education regarding hospice resources (18). While there are no AS-specific hospice admissions guidelines, it would be reasonable to consider hospice for a patient with severe AS who is not pursuing AVR and who is experiencing severe heart failure (refractory volume overload, recurrent hospitalizations, declining functional status, dyspnea at rest, angina, etc.). The presence of comorbidities such as renal failure, liver impairment, and dementia should also lead clinicians to consider initiating hospice support in patients with severe AS.

References

1. Lauck S, Garland E, Achtem L, et al. Integrating a palliative approach in a transcatheter heart valve program: bridging innovations in the management of severe aortic stenosis and best end-of-life practice. Eur J Cardiovasc Nurs. 2014;13(2):177-184. doi:10.1177/1474515114520770
2. Pujari SH, Agasthi P. Aortic Stenosis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; October 1, 2022.
3. Pinto SC, Ancona R. Epidemiology of Aortic Valve Stenosis (as) and of aortic valve incompetence (AI): Is the prevalence of AS/AI similar in different parts of the world? E-Journal of Cardiology Practice. Published February 12, 2020. Accessed March 15, 2023. 
4. Faggiano P, Antonini-Canterin F, Baldessin F, Lorusso R, D’Aloia A, Cas LD. Epidemiology and cardiovascular risk factors of aortic stenosis. Cardiovascular Ultrasound. 2006;4(1). doi:10.1186/1476-7120-4-27 
5. Pellikka PA, Sarano ME, Nishimura RA, et al. Outcome of 622 adults with asymptomatic, hemodynamically significant aortic stenosis during prolonged follow-up. Circulation. 2005;111(24):3290-3295. doi:10.1161/CIRCULATIONAHA.104.495903
6. Deutsch MA, Bleiziffer S, Elhmidi Y, et al. Beyond adding years to life: health-related quality-of-life and functional outcomes in patients with severe aortic valve stenosis at high surgical risk undergoing transcatheter aortic valve replacement. Curr Cardiol Rev. 2013;9(4):281-294. doi:10.2174/1573403×09666131202121750
7. Tribouilloy C, Rusinaru D, Bohbot Y, Maréchaux S, Vanoverschelde JL, Enriquez-Sarano M. How Should Very Severe Aortic Stenosis Be Defined in Asymptomatic Individuals?. J Am Heart Assoc. 2019;8(3):e011724. doi:10.1161/JAHA.118.011724
8. Varadarajan P, Kapoor N, Bansal R, Pai R. Clinical profile and natural history of 453 nonsurgically managed patients with severe aortic stenosis. The Annals of Thoracic Surgery. https://pubmed.ncbi.nlm.nih.gov/17126120/. Accessed February 12, 2023. 
9. Brecker S, Aldea G. Choice of intervention for severe calcific aortic stenosis. UpToDate. https://www.uptodate.com/contents/choice-of-intervention-for-severe-calcific-aortic-stenosis?topicRef=8130&source=see_link#H22201191. Published June 15, 2022. Accessed March 2, 2023. 
10. Grimard BH, Safford RE, Burns EL. Aortic Stenosis: Diagnosis and Treatment. Am Fam Physician. 2016;93(5):371-378.
11. Tang L, Gossl M, Garberich R, Bradley S. Contemporary reasons and clinical outcomes for patients with severe. Contemporary Reasons and Clinical Outcomes for Patients With Severe, Symptomatic Aortic Stenosis Not Undergoing Aortic Valve Replacement. Circulation: Cardiovascular Interventions. American Heart Association. https://www.ahajournals.org/doi/10.1161/circinterventions.118.007220. Accessed February 12, 2023. 
12. Vahanian A, Beyersdorf F, Praz F, et al. 2021 ESC/EACTS Guidelines for the management of valvular heart disease [published correction appears in Eur Heart J. 2022 Feb 18;:]. Eur Heart J. 2022;43(7):561-632. doi:10.1093/eurheartj/ehab395
13. Online STS Adult Cardiac Surgery Risk Calculator. Society of Thoracic Surgeons. https://riskcalc.sts.org/stswebriskcalc/. Accessed March 7, 2023. 
14. Steiner J, Kirkpatrick J. Chapter 22: Palliative Care and TAVR. https://www.acc.org/~/media/Non-Clinical/Files-PDFs-Excel-MS-Word-etc/Membership/TAVR-Handbook/Chapter-21-Palliative-Care-and-TAVR-March-2-2018.pdf. Accessed February 13, 2023. 
15. Kanwar A, Thaden JJ, Nkomo VT. Management of Patients With Aortic Valve Stenosis. Mayo Clin Proc. 2018;93(4):488-508.doi:10.1016/j.mayocp.2018.01.020
16. Taylor LJ, Nabozny MJ, Steffens NM, et al. A Framework to Improve Surgeon Communication in High-Stakes Surgical Decisions: Best Case/Worst Case. JAMA Surg. 2017;152(6):531-538. doi:10.1001/jamasurg.2016.5674.
17. Steiner JM, Cooper S, Kirkpatrick JN. Palliative care in end-stage valvular heart disease. Heart (British Cardiac Society). https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5657597/. Published August 2017. Accessed February 12, 2023. 
18. Lindman BR, Alexander KP, O’Gara PT, Afilalo J. Futility, benefit, and transcatheter aortic valve replacement. JACC Cardiovasc Interventions. 2014;7(7):707-716. doi:10.1016/j.jcin.2014.01.167

Authors’ Affiliations: University of Colorado Anschutz Medical Campus, Aurora, CO; Saint Joseph Hospital, Denver, CO; Rocky Mountain Region VA Medical Center, Aurora, CO
Conflicts of Interest:  None
Version History:  Originally edited by Sean Marks MD; first electronically published in June 2023