Multivalvular Disease

Chapter: Multivalvular Disease
McMaster Section Editor(s): P.J. Devereaux
Section Editor(s) in Interna Szczeklika: Andrzej Budaj, Wiktoria Leśniak
McMaster Author(s): Hisham Dokainish
Author(s) in Interna Szczeklika: Marek Konka, Piotr Szymański
Additional Information

Definition, Etiology, Pathogenesis Top

Multivalvular disease affects ≥2 venous or arterial valves; in general order of descending frequency in the contemporary European or North American practice:

1) Mitral regurgitation (MR) and tricuspid regurgitation (TR).

2) Aortic stenosis (AS) and MR.

3) Aortic regurgitation (AR) and MR.

4) AS and TR.

5) Mitral stenosis (MS) and AR.

6) MS and AS.

(Detailed descriptions of individual valvular diseases: Aortic Regurgitation; Aortic Stenosis; Mitral Regurgitation; Mitral Stenosis; Tricuspid Regurgitation.)

Previously, multivalvular disease with secondary effects on the left ventricle and the left atrium used to be mainly caused by rheumatic heart disease (for example, MS and AS); however, with ageing populations (and increased use of antibiotics in childhood and youth), degenerative or functional MR with secondary TR as well as degenerative AS with secondary MR or TR are increasingly common.

Clinical Features and Diagnosis Top

In general, the predominant clinical features are caused by the hemodynamically more severe lesion; however, assuming similar degrees of valvular severity, symptoms are related to the disease of the more proximal (upstream) valvular lesion (eg, AS in combined AS/MR). The primary valve lesion can create secondary (“back pressure”) effects on the secondary valve lesion (eg, chronic severe MR [or AS] leading to right ventricular dilation, secondary functional TR, and pulmonary hypertension). In advanced multivalvular disease, symptoms of heart failure are often present, and are accompanied by secondary hemodynamic effects on the cardiac chambers (eg, chronic severe AR leading to left ventricular and left atrial dilation and secondary functional MR). Features of various types of multivalvular disease: Table. Characteristics of multivalvular disease.

The diagnostic workup of multivalvular heart disease is not significantly different from that of individual valvular lesions.

Treatment Top

To establish indications for surgical treatment, the clinical and hemodynamic significance of the components of multivalvular heart disease must be assessed. Any defect assessed separately may not be severe enough to warrant surgical treatment; however, when combined, these may have significant hemodynamic consequences that require surgery. In some cases, it may not be necessary to replace all the affected valves (eg, surgical treatment of severe AS may, by relieving afterload challenge to the left ventricle, relieve secondary functional MR). Double-valve replacement is generally associated with higher perioperative and long-term risks, owing to a more complex surgical technique and longer cardiopulmonary bypass times, compared to single valve repair or replacement (+/- coronary artery bypass graft); thus, whenever feasible, repair is often preferred (eg, mitral valve repair for severe myxomatous mitral regurgitation with concomitant tricuspid valve annuloplasty for secondary TR). It is important to recognize that management of multivalve disease is essentially an “evidence-free zone,” and thus recommendations in the most current 2014 American College of Cardiology and American Heart Association guidelines are brief and consensus-based.

Tables Top

Table. Characteristics of multivalvular disease

Multivalvular disease

Pathophysiology

Auscultation

Surgical treatment

Comments

MR + TR

Chronic MR causes pulmonary hypertension, leading to RV dilation and secondary TR

Apical systolic murmur caused by MR and lower sternal border murmur of TR

Mitral repair or replacement with tricuspid valve repair or annuloplasty

MR most commonly the dominant lesion; at times relief of MR alone may result in decrease in TR

AS + MR

AS leads to LV afterload challenge or strain and secondary MR; impaired aortic valve outflow can further aggravate MR

Ejection murmur radiating to carotid arteries and murmur caused by MR radiating to axilla

Simultaneous aortic valve replacement and mitral repair or replacement

MR may be reduced after aortic valve replacement (or TAVI) without specific mitral valve intervention

AR + MR

AR leads to LV dilation and secondary MR

Apical systolic murmur caused by MR and diastolic murmur caused by AR

Simultaneous aortic valve repair or replacement and mitral repair or replacement

AR frequently dominant; in such cases it is difficult to distinguish primary from secondary MR due to LV enlargement; at times treatment of AR alone may relieve significant MR due to LV dilation caused by chronic AR

MS + TR

MS leads to pulmonary hypertension and secondary regurgitation of anatomically normal tricuspid valve; in some patients tricuspid valve also affected by rheumatic fever

Typical signs of MS and holosystolic murmur (more prominent during inspiration) caused by TR

Mitral valve replacement (or mitral valvuloplasty) and tricuspid annuloplasty

Hemodynamically significant TR may improve following mitral valve valvuloplasty (or surgery) without specific surgical treatment of the tricuspid valve

MS + AS

MS restricts blood inflow to LV and aggravates the drop of cardiac output caused by AS

Typical signs of MS and murmur caused by AS (less prominent than in isolated AS)

Simultaneous replacement of both valves

Percutaneous balloon mitral valvuloplasty in patients with significant AS may result in pulmonary edema

MS + AR

Impaired LV filling from left atrium is compensated by reverse blood flow through aortic valve

Typical signs of MS and diastolic murmur along left sternal border

Before surgical treatment of aortic valve percutaneous balloon mitral valvuloplasty should be considered

MS reduces LV volume overload and can mask AR

AR, aortic regurgitation; AS, aortic stenosis; LV, left ventricle; MR, mitral regurgitation; MS, mitral stenosis; RV, right ventricle; TAVI, transcatheter aortic valve implantation; TR, tricuspid regurgitation.

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