Brett F. Curran MD, Fazal Khan MD, Hartzell V. Schaff MD
Mayo Clinic Rochester
August 1, 2024
Abbreviations & Definitions
AF – Atrial fibrillation
LA – Left atrium
MAC – Mitral annular calcification
MR – Mitral regurgitation
MS – Mitral stenosis
MV – Mitral valve
NYHA – New York Health Association
PASP – Pulmonary artery systolic pressure
PMBC – Percutaneous mitral balloon commissurotomy
TMVR – Transcatheter mitral valve replacement
Indications & Guidelines for Management by Grade/Stage of Disease
Mitral stenosis (MS) can be categorized into rheumatic and non-rheumatic, sometimes termed degenerative calcific MS. The incidence of rheumatic fever and rheumatic MS is decreasing globally. Degenerative calcific MS with or without annular calcification (MAC) is encountered increasingly in the developed world due to an aging population Rheumatic MS spares the annulus and causes commissural fusion, leaflet thickening, and chordal shortening.Degenerative MS is the result of calcification of the mitral leaflets that extends onto the annulus. Degenerative MS generally involves the base of the leaflets, whereas rheumatic MS predominantly affects the tips Treatment of rheumatic MS is either percutaneous balloon mitral valvuloplasty (PMBC) or surgery and is based on clinical characteristics, valve anatomy, and available expertise. The Wilkins Score is a scoring system that uses echocardiography to predict the success of PMBC. It encompasses scoring of leaflet mobility, thickening, calcification, and subvalvular thickening, with a score <8 predictive of high procedural success. LA thrombus and moderate or greater mitral valve regurgitation (MR) are relative contraindications to PMBC. Poor anatomic mitral morphology score is also a relative contraindication.3, Surgical treatment is reserved for those who have anatomic characteristics unsuitable for PMBC, patients who have failed PMBC, and those who require concomitant cardiac procedures. Surgical treatment involves prosthetic replacement in most patients or mitral valve (MV) commissurotomy (open or closed) in selected cases. Non-rheumatic MS patients are often elderly with significant medical comorbidities. Treatment options include transcatheter or surgical approaches, which are high-risk secondary to significant calcification and patient factors. The classification of MS is based on patient symptoms, valve anatomy, hemodynamics, and the secondary effects the valve has on the left atrium (LA) and pulmonary circulation
Table 1. Stages of MS.
| Stage | Definition | Valve | Hemodynamics | Result | Symptoms |
|---|---|---|---|---|---|
| A | At risk for MS | Mild doming during diastole | Normal gradient | None | None |
| B | Progressive MS | Rheumatic valve changes, commissural fusion, diastolic doming of MV leaflets MV area >1.5 cm2 |
Increased mitral flow velocity Diastolic pressure half-time <150 ms | Mild to moderate LA enlargement Normal pulmonary pressure at rest |
None |
| C | Asymptomatic severe MS | Rheumatic valve changes, commissural fusion, diastolic doming of MV leaflets MV area ≤1.5 cm2 |
Diastolic pressure half-time ≥150 ms | Severe LA enlargement Elevated PASP >50 mmHg | None |
| D | Symptomatic severe MS | Rheumatic valve changes, commissural fusion, diastolic doming of MV leaflets MV area ≤1.5 cm2 |
Diastolic pressure half-time ≥150 ms | Severe LA enlargement Elevated PASP >50 mmHg | Decreased exercise tolerance Exertional dyspnea |
Rheumatic MS
Table 2. Summarized Indications for Intervention in Rheumatic MS.
| Clinical and Echocardiogram Manifestations | Class | Intervention | References |
|---|---|---|---|
| Symptomatic patients (NYHA class II, III, or IV) with severe rheumatic MS (MV area ≤1.5 cm2, Stage D) and favorable valve morphology with no LA thrombus and less than moderate MR | I | PMBC is recommended | , |
| Severely symptomatic patients (NYHA class III or IV) with severe rheumatic MS (MV area ≤1.5 cm2, Stage D) who 1) are not candidates for PMBC, 2) have failed a previous PMBC, or 3) require other cardiac procedures | I | Mitral valve surgery (repair, commissurotomy, or valve replacement) | |
| Asymptomatic patients with severe rheumatic MS (MV area ≤1.5 cm2, Stage C) and favorable valve morphology with less than 2+ MR in the absence of LA thrombus who have elevated pulmonary pressures (PASP >50 mmHg) | IIA | PMBC is reasonable | |
| Asymptomatic patients with severe rheumatic MS (MV area ≤1.5 cm2, Stage C) and favorable valve morphology with less than moderate MR in the absence of LA thrombus who have new onset of AF | IIB | PMBC may be considered | |
| Symptomatic patients (NYHA class II, III, or IV) with rheumatic MS and a MV area >1.5 cm2, if there is evidence of hemodynamically significant rheumatic MS based on a pulmonary artery wedge pressure >25 mmHg or a mean MV gradient >15 mmHg during exercise | IIB | PMBC may be considered | |
| Severely symptomatic patients (NYHA class III or IV) with severe rheumatic MS (MV area ≤1.5 cm2, Stage D) who have suboptimal valve anatomy and who are not candidates for surgery or are at high risk for surgery | IIB | PMBC may be considered |
Adapted from 2020 ACC/AHA Guideline for Management of Patients with Valvular Heart Disease7
Figure 1: Algorithm of Interventions for Rheumatic MS.
Adapted from 2020 ACC/AHA Guideline for Management of Patients with Valvular Heart Disease7
Non-rheumatic MS
Valve intervention may be considered in patients with advanced symptoms (NYHA class III or IV) and severe MS with or without extensive MAC (Class IIB). These patients are typically elderly with multiple comorbidities, and any intervention is high risk because of the extensive calcification. Intervention for calcific MS should be performed only in the highly symptomatic patient. No guidelines are available for the management of these patients. Interventions include surgical mitral valve replacement (MVR), repair (MVr), or transcatheter mitral valve replacement (TMVR).
Supporting Evidence for Current Indications and Guidelines
The current guidelines for patients with valvular heart disease were developed in 2020 by the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines and provide a comprehensive review of literature and guidelines for patients with MS. The literature to support the current guidelines is highlighted in the table above in the references section. The European Society of Cardiology (ESC) and the European Association for Cardiothoracic Surgery (EACTS) guidelines are not referenced in this chapter but have only minor discrepancies from those adapted by the ACC/AHA.
Ongoing Trials/Recent Publications
The majority of clinical trials at this time related to MS involve developing TMVR systems. These include the APOLLO trial, which evaluates the safety and efficacy of the Intrepid transcatheter mitral valve. The CEASE-MR compares the Tendyne transcatheter mitral valve system versus standard mitral surgery. Additional clinical trials are ongoing evaluating TMVR in the treatment of MAC.
Expert Commentary
In developing countries, rheumatic MV disease continues to be an important health problem, and the management of young patients with MS by balloon valvotomy and open or closed surgical commissurotomy is relatively common. In North America and Europe, however, the disease is less common, and most patients with rheumatic MS present later in life with calcification and scarring of the valve that precludes successful and durable valve repair; prosthetic MVR is the best procedure for most of these patients.
As the incidence of rheumatic MS has decreased, surgeons are seeing increasing numbers of patients with degenerative calcific MS. Operation in these patients often carries increased risk due to general patient-related factors such as older age, chronic renal disease, and associated calcific aortic valve disease. Further, technical aspects of MVR may be difficult due to calcification of the valve annulus, which prevents secure suture of the prosthesis. If the MAC is not extensive, local debridement with or without patch repair can be performed to facilitate the placement of valve sutures. Some surgeons have approached more extensive calcification with extensive annular debridement and reconstruction. This, however, is associated with relatively high mortality, and the technique is probably best reserved for surgeons with experience with the method. Other techniques for MVR in patients with extensive MAC include sewing a cuff to the sewing ring and securing the valve or portions of the prosthesis to the atrium or placement of an expandable transcatheter valve, essentially suspending the valve in a supra-annular position to avoid placing sutures through a calcific annulus. With the latter procedure, the anterior mitral valve leaflet should be excised or divided to prevent left ventricular outflow tract obstruction. In cases of calcific MS in which orthotopic MVR is considered excessively hazardous, mitral valve bypass with left atrial-to-left ventricle valved conduit has also been used.
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