Chapter 286 - Multiple and Mixed Valvular Heart Disease

Question 1

Which valves might be involved in rheumatic disease?

Answer 1

Any valve might be involved in rheumatic disease.
In order of frequency, it might involve the mitral valve, aortic valve, tricuspid valve or rarely the pulmonic valve.
“For example, rheumatic heart disease can involve the mitral (mitral stenosei [MS], mitral regurgitation [MR], or MS and MR), aortic (aortic stenosis [AS], aortic regurgitation [AR], or AS and AR), and/or tricuspid (tricuspid stenosis [TS], tricuspid regurgitation [TR], or TS and TR) valve, alone or in combination.”

Question 2

How does one explain the fact that severe mitral calcification can result in either stenosis, regurgitation or both?

Answer 2

“Severe mitral annular calcification can result in regurgitation (due to decreased annular shortening during systole) and mild stenosis (caused by extension of the calcification onto the leaflets resulting in restricted valve opening).”

Question 3

In infetive endocarditis involving the aortic valve should one expect a possible mitral valve involvement?

Answer 3

Yes.
“Aortic valve infective endocarditis may secondarily involve the mitral apparatus either by abcess formation and contiguous spread via the intervalvular fibrosa or by “drop metastases” from the aortic leaflets onto the anterior leaflet of the mitral valve.”

Question 4

Chordal rupture has been described infrequently in patients with severe aortic stenosis.
True or False?

Answer 4

True.

Question 5

Which valves are usually involved in carcinoid heart disease?

Answer 5

Right-sided valves (tricuspid and pulmonic).
(there are two exceptions, which include carcinoid syndrome with a lung neoplasia, and the existent of a previous left-to-right shunting).

Question 6

Name the drugs that have been rarely associated with mixed valve disease involving the aortic and/or mitral valves.

Answer 6

Ergotamines, and the previously used combination of fenfluramine and phentermine.

Question 7

A patient with myxomatous degeneration that causes prolapse of multiple valves (mitral, aortic, tricuspid) might not have an identifiable connective tissue disorder.
True or False?

Answer 7

True.

Question 8

Bicuspid aortic or pulmonic valve disease doesn’t lead to mixed stenosis and regurgitation.
True or False?

Answer 8

False.

Question 9

How does one explain that a more proximally located valve disease might mask a more distal one? Give examples of such a phenomenon.

Answer 9

“In patients with multivalvular heart disease, the pathophysiologic derangements associated with the more proximal valve disease can mask the full expression of the attributes of the more distal valve lesion. For examples, in patients with rheumatic mitral and aortic valve disease, the reduction in cardiac output (CO) imposed by the mitral valve disease will decrease the magnitude of the hemodynamic derrangements related to the severity of the aortic valve lesion (stenotic, regurgitant, or both). Alternatively, the development of atrial fibrilation (AF) during the course of MS can lead to sudden worsening in a patient whose aortic valve disease was not previously felt to be significant. The development of reactive pulmonary vascular disease, sometimes referred to as a “secondary obstructive lesion in series,”, can impose an additional challenge in these settings. As CO falls with progressive tricuspid valve disease, the severity of any associated mitral or aortic disease can be underestimated.”

Question 10

Which valvulopathy is frequently associated with mitral stenosis, due to its natural history course?

Answer 10

“One of the most common examples of multivalve disease is that of functional tricuspid regurgitation (TR) in the setting of significant mitral valve disease. Functional TR occurs as a consequence of right ventricular and annular dilation; pulmonary artery hypertension is often present.”

Question 11

One of the features of tricuspid regurgitation is a large c-v systolic wave. Does the height of this wave have any correlation with other variables? If so, which ones?

Answer 11

“The hight of the c-v wave is dependent on right atrium compliance and the volume of regurgitant flow.”

Question 12

What are the two most common valvulopathies in rheumatic fever?

Answer 12

Mitral stenosis with aortic regurgitation.

Question 13

Explain the hemodynamic pathophysiology of rheumatic mitral valve disease associated with aortic valve disease.

Answer 13

“In isolated mitral stenosis (MS), left ventricular (LV) preload and diastolic pressure are reduced as a function of the severity of inflow obstruction. With concomitant aortic regurgitation, however, LF filling is enchanced and diastolic pressure may rise depending on the compliance characteristics of the chamber. Because the cardiac output falls with progressive degrees of MS, transaortic valve flows will decline, masking the potential severity of the aortic valve lesion (AR, AS, or its combination).”

Question 14

Atrial fibrilation might be especially deleterious in patients with either mitral stenosis and tricuspid regurgitation or mitral stenosis and aortic regurgitation,
True or False?

Answer 14

True.

Question 15

How does one explain mitral regurgitation (MR) in a patient with severe aortic stenosis (AS)? How should it be treated?

Answer 15

“Functional MR may complicate the course of some patients with severe AS. The mitral valve leaflets and chordae tendinae are usually normal. Incompetence is realted to changes in LV geometry (remodeling) and abnormal systolic tethering of the leaflets in the context of markedly elevated LV systolic pressures. Relief of the excess afterload with surgical or transcatheter aortic valve replacement often, but not always, results in reduction or elimination of the MR. Persistence of significant MR following AVR is associated with impaired functional outcomes and reduced survival. Identification of patients who would benefit from concomitant treatment of their functional MR at time of AVR is quite challenging. Most surgeons advocate for repair of moderate-to-severe or servere functional MR at time of surgical AVR.”

Question 16

If a patient suffers from aortic regurgitation and aortic stenosis, it is expected to find an increased murmur and transaortic flow velocity, which might lead to lower orifice estimations in comparison to planimetry.
True or False?

Answer 16

True: the sentence is only true if the patient has a preserved left ventricle systolic function.
False: if the left ventricle systolic function is decreased, the estimation might be compensated or even decreased to the aortic orifice measurement in planimetry.

Question 17

The Gorlin formula is an accurate way to measure the orifice area in mixed valvular disease.
True or False?

Answer 17

False.

This formula relies on the forward cardiac output (which might be affected by mixed valvular disease).

Question 18

How would you expect to find the left ventricular chamber size in aortic regurgitation (AR) or mitral regurgitation (MR)? And in aortic stenosis (AR) or mitral stenosis (MS)?

Answer 18

“When either AR or MR is the dominant lesion in patients with mixed aortic or mitral valve disease, respectively, the LV is dilated. When AS or MS predominates, LV chamber size will be normal or small.”

Question 19

Paravalvular regurgitation is a significant risk factor for short- to intermediate-term death following transcatheter aortic valve replacement.
True or False?

Answer 19

True.

Question 20

How does one explain the fact that a patient with aortic stenosis might not tolerate as well an acute aortic regurgitation in comparison to a patient without previous valve disease?

Answer 20

“Patients with significant AS, a nondilated LV chamber, and concetric hypertrophy will poorly tolerate the abrupt development of aortic regurgitation, as may occur, for example, with infective endocarditis or after surgical or transcatheter AVR complicated by paravalvular leakage. The noncompliant LV is not prepared to accomodate the sudden volume load, and a result, LV diastolic pressure rises rapidly and severe heart failure develops.”

Question 21

Which conditions might be associated with incapacity for compensatory dilation of the left ventricle in the setting of aortic or mitral valve regurgitation?

Answer 21

“Conditions in which the LV may not be able to dilate in response to chronic AR (or MR) include radiation heart disease and, in some patients, the cardiomyopathy associated with obesity and diabetes.”

Question 22

Angina might occur due to oxygen supply/demand mismatch either to an hypertropied right or left ventricle or due to pressure/volume overload to either of these chambers, either in the presence or not of coronary artery disease.
True or False?

Answer 22

True.

Question 23

A meso-systolic murmur in a patient with suspected aortic regurgitation is always a sign of concomitant aortic stenosis/sclersosis.
True or False?

Answer 23

False.

Question 24

An ejection click is usually present in bicuspid aortic stenosis, especially in the young. If one has a mixed aortic valve disease, do you expect to find this phenomenon?

Answer 24

No.
“An early ejection click, which usually defines bicuspid aortic valve disease in young adults, if often not present in patients with congenital, mixed, AS and AR.”

Question 25

If one suspects mitral valve disease (either stenotic or regurgitant) which pathological pulse might often be present?

Answer 25

Atrial fibrilation.

Question 26

The presence of atrial fibrilation might lead to the suspicion of concomitant mitral valve disease, even if not yet suspected before.
True or False?

Answer 26

True.

Question 27

What are the findings characteristic of involvement of the pulmonary vasculature, including pulmonary artery (PA) hypertension?

Answer 27

“The latter could inlude enlargement of the main and proximal pulmonary arteries with PA hypertension and pulmonary venous redistribution/engorgement or Kerley B lines with increasing degrees of left atrium hypertension.”

Question 28

What is the meaning of an engorged azygos vein in the frontal projection of the chest x-ray?

Answer 28

Right atrium hypertension.

Question 29

Name two situations where transesophageal echocardiography might be advantageous.

Answer 29

“Transesophageal echocardiography (TEE) may sometimes be required for more accurate assessment of valve anatomy (specifically, the mitral vale) and when infective endocarditis (IE) is considered responsible for the clinical presentation.”

Question 30

What is the usefulness of exercise testing regarding valvulopathy?

Answer 30

“Exercise testing (with or without echocardiography) can be useful when the degree of functional limitation reported by the patient is not adequately explained by the findings on TTE performed at rest.”

Question 31

Name the usefulness of cardiac magnetis resonance and its limitations, as well as CT scan for cardiac valvular disease.

Answer 31

“Cardiac magnetic resonance (CMR) can be used to provide additional anatomic and physiologic information when echocardiography proves suboptimal, but is less well suited to the evaluation of valve morphology. Cardiac computed tomography (CT) has been used to assess intracardiac structures in patients with complicated IE. Coronary CT angiography provides a noninvasive alternative for the assessment of coronary artery anatomy prior to surgery.”

Question 32

When should one use invasive hemodynamic evaluation?

Answer 32

“Invasive hemodynamic evaluation with right and left heart catheterization may be required to characterize more completely the individual contributions of each lesion in patients with either multiple or mixed valvular heart disease. Measurement of PA pressures and calculation of pulmonary vascular resistance can help inform clinical decision-making in certain patient subsets, such as those with advanced mitral and tricuspid valve disease. Attention to the accurate assessment of cardiac output is essential.”

Question 33

The novel oral anticoagulants are approved for significant valvular heart disease.
True or False?

Answer 33

False.

Question 34

Pulmonary vasodilators to lower pulmonary vascular resistance are generally effective in valvulopathic disease.
True or False?

Answer 34

False.

Question 35

Regarding concomitant aortic and mitral valve disease, when should one replace both the diseased valves? Why is it so?

Answer 35

“Concomitant aortic and mitral valve replacement surgery is associated with a significantly higher perioperative mortality risk than replacement of either valve alone, and operation should be carefully considered. Double valve replacement surgery is usually performed for treatment of severe (unrepairable) valve disease at both locations and for the combination of severe disease at one location with moderate disease at the other, so as to avoid the hazards of reoperation in the intermediate to late term for progressive disease of the unoperated valve. In addition, the presence of a prosthesis in the aortic position significantly restricts surgical exposure of the native mitral valve.”

Question 36

Tricuspid valve repair for moderate or severe functional tricuspid regurgitation is now common if left-sided valve surgery is also preformed. When is it indicated and what are the advantages of this intervention?

Answer 36

“Tricuspid valve repair for moderate or severe functional TR at the time of left-sided valve surgery is now commonplace, particularly if there is dilation of the tricuspid annulus (>40mm). The addition of tricuspid valve repair, consisting usually of insertion of an annuloplasty ring, adds little time or complexity to the procedure and is well tolerated. Reoperation for repair (or replacement) of progressive TR years after initial sugery for left-sided valve disease, on the other hand, is associated with a relatively high perioperative mortality risk.”

Question 37

Repair of moderate or severe functional mitral regurgitation at time of aortic valve replacement for aortic stenosis can usually be undertaken with acceptable risk for perioperative death or major complication.
True or False?

Answer 37

True.

Question 38

Name a contraindication for percutaneous balloon valvotomy of either the mitral or aortic valve.

Answer 38

“The presence of moderate or severe MR in patients with rheumatic mitral stenosis is a contraindication to percuteaneous mitral balloon valvotomy (PMBV). Likewise, the presence of significant AR in patients with AS disqualifies them from percutaneous aortic balloon valvotomy (PABV).