What’s The “Take Home”?® A 78-Year-Old Woman With a Newly Discovered Significant Heart Murmur

Ronald Rubin, MD—Series Editor

A78-year-old woman has moved to your area in order to be closer to her children, and she presents as a new patient to your practice.

History

She has been quite healthy all of her life, without significant major medical diagnoses. Specifically, she has no history of diabetes, hypertension, or chronic obstructive pulmonary disease. She has never smoked. Her most recent interaction with the health system was an uneventful hip replacement 5 years ago. She uses nonsteroidal antiinflammatory drugs sporadically for joint soreness.

Review of systems is negative for chest pain, dyspnea on exertion, paroxysmal nocturnal dyspnea, or edema. She is active and can easily climb 2 flights of steps without symptoms. She does report being told of a heart murmur years ago that “could just be monitored.”

Physical Examination

Physical examination reveals a healthy-appearing woman with a pulse of 84 beats/min and a blood pressure of 114/75 mm Hg. Findings of examination of the head, eyes, ears, nose, and throat are unremarkable. Her chest is clear to percussion and auscultation. A coarse, holosystolic, rasping murmur is loudest at the cardiac base and radiates into the neck. There is delay and blunting of the carotid pulsations. There is no gallop rhythm. Her joints are normal, and she has no pedal edema.

Correct Answer: D

This case is an entry into the clinical presentation, diagnostic evaluation, and management of valvular aortic stenosis (AS), a very important and common condition today.

Valvular Aortic Stenosis

Valvular AS is a progressive disease wherein obstruction of left ventricular outflow eventually causes inadequate cardiac output (syncope), left ventricular hypertrophy with relative ischemia with effort (angina), and congestive heart failure (CHF). Those 3 clinical findings are the cardinal symptoms of advanced AS, but the murmur may be discovered before any symptoms develop, as in the case of the patient presented here. AS is quite common and surely will become more so as the

WHICH ONE OF THE FOLLOWING IS THE CORRECT STATEMENT ABOUT HER MANAGEMENT?

A. Genetic analysis seeking the specific gene abnormality responsible for her heart murmur is an important initial component of her evaluation.

B. If her echocardiogram reveals evidence of severe disease and a preserved, normal ejection fraction (EF), prompt valve replacement is required.

C. Despite her being asymptomatic, her mortality risk will increase by 20% per year.

D. If her echocardiogram reveals evidence of severe disease and a preserved, normal EF, frequent clinical and echocardiographic monitoring is appropriate.

Laboratory Tests

Laboratory evaluation reveals a normal complete blood count and normal levels of blood glucose, hemoglobin A1C, and creatinine. She is scheduled for an echocardiography study.

population ages. The prevalence increases from 0.2% in adults aged 50 to 59 years to 2.8% in adults older than 75, to 9.8% in octogenarians.1

Much basic research is ongoing exploring the complex deranged biochemistry and biophysics interacting at the aortic valve, causing progressive AS. Less-complex is the list of known causative and associated lesions, including (the less common in the United States today) rheumatic heart disease and the presence of a bicuspid aortic valve. Of note, unlike hypertrophic cardiomyopathy, which can cause left ventricular outflow obstruction usually in a younger population, no specific gene cluster or abnormality has been identified for valvular AS.1 Thus, Answer A is incorrect.

As in this case, the initial presentation of AS is the presence of a characteristic heart murmur. The murmur typically is

TAKE-HOME MESSAGE

Valvular aortic stenosis is quite common in the elderly, and its prevalence will increase as the population ages. The natural history is well known, with no increased mortality in asymptomatic patients, but a 50% 2-year mortality rate once the classic symptoms of congestive heart failure, angina, or syncope develop. No effective medical therapy exists, and proper management involves careful, educated monitoring utilizing clinical findings, echocardiography for assessing and monitoring severity and progression, and ensuring appropriate timing of aortic valve replacement.

holosystolic and has a crescendo-decrescendo, “diamondshaped” character. The murmur usually is loudest at the base and radiates into the neck. Refined auscultation skills may detect an absence of S2 sounds. More easily detected is a delay and blunting of the carotid pulsations. Having said all this, the refined technology of echocardiography is required (1) to confirm that the murmur indeed is AS rather than another lesion; (2) to assess whether the AS is the sole abnormality or whether other lesions also are present, and which one is the hemodynamically significant one (eg, in rheumatic multiple valvular situations); and (3) to assess the severity of the AS using long established anatomic and hemodynamic parameters.2

Echocardiography

The specifics of a detailed echocardiographic study include quantitation of calcification and leaflet motion of the aortic valve, maximum flow velocity (Vmax), Vmax through the valve (mild, < 2.0 m/s; moderate, 2.0-3.9 m/s; severe, ≥ 4.0 m/s), and mean transaortic pressure gradient (mild to moderate, 20-39 mm Hg; severe, > 49 mm Hg). The echocardiogram also will establish the functional capacity of the left ventricle with an EF measurement, normal being ≥ 50%.3

The above measurements then must be coupled with clinical findings—symptoms or no symptoms, comorbid diseases, and general prognosis. This is because regardless of echo findings (there are complex exceptions beyond the scope of this vignette), mortality essentially is not increased when AS is asymptomatic. And conversely, when symptoms are present, pathophysiologic changes can alter echocardiogram numbers, but the AS must be addressed, because as a broad statement, symptomatic AS has a 50% 2-year mortality rate unless the valve is replaced.1-4 These facts make Answer C incorrect. She is asymptomatic, and the stated mortality risk in this setting is too high.

Similarly, Answer B is incorrect in that even severe disease as demonstrated by echocardiography does not require urgent valve replacement so long as the patient is asymptomatic. This is the case for now, in part because the mortality of a surgical procedure to correct AS still exceeds the mortality curve of an asymptomatic AS population. 1,4 This patient can be safely and carefully monitored, clinically for symptom changes and hemodynamically for echocardiographic deterioration, with the most specific finding being a decrement in EF to < 50%, which would prompt aortic valve replacement to preserve left ventricular function. 1,3,5 Thus, Answer D is the best choice here.

Outcome Of The Case

The clinical findings strongly suggested hemodynamically significant AS. This was confirmed by echocardiography, which revealed severe calcification with reduced leaflet motion, Vmax of 4.2 m/s, a mean transvalvular pressure of 46 mm Hg, and an EF of 55%.

The history was reviewed again with the patient, and she truly was asymptomatic, confirming the diagnosis of stage C1 severe AS.1 The plan was to monitor the patient clinically in the office at 3-month intervals and perform echocardiogram monitoring at 6-month intervals. She understands that aortic valve surgery is very likely in the future. She knows to promptly report any symptoms of angina, CHF, or syncope. n

Ronald Rubin, MD, is a professor of medicine at Temple University School of Medicine and chief of clinical hematology in the Department of Medicine at Temple University Hospital, both in Philadelphia, PA.

REFERENCES:

1. Otto CM, Prendergast B. Aortic-valve stenosis—from patients at risk to severe valve obstruction. N Engl J Med. 2014;371(8):744-756.

2. Rosenhek R, Binder T, Porenta G, et al. Predictors of outcome in severe, asymptomatic aortic stenosis. N Engl J Med. 2000;343(9):611-617.

3. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline 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. J Am Coll Cardiol. 2014;63(22):2438-2488.

4. Iung B, Vahanian A. Degenerative calcific aortic stenosis: a natural history. Heart. 2012;98(suppl 4):IV7-IV13.

5. Kodali SK, Williams MR, Smith CR, et al; PARTNER Trial Investigators. Twoyear outcomes after transcatheter or surgical aortic-valve replacement. N Engl J Med. 2012;366(18):1686-1695.