Guideline sources The following summarized guidelines for the evaluation and management of mitral stenosis are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2021), the European Society of Cardiology (ESC/EACTS 2021), the European Society of Cardiology (ESC/EACTS/EHRA 2021), the National Heart Foundation of Australia (NHFA 2018), and the Canadian Cardiovascular Society (CCS 2018). 1 2 3 4 5 6 6 7 8 9 Definition Mitral stenosis is a form of structural heart disease characterized by obstruction to diastolic flow of blood across the mitral valve, from the left atrium to the left ventricle. 6 Epidemiology The etiology of mitral stenosis is rheumatic disease in over 99% of patients. Other rare causes of mitral stenosis include congenital malformed valves, active infective endocarditis, massive annular https://web.pathway.md/diseases/reci4ziz64UyvvxCW 1/8 6/30/23, 1:05 AM Mitral stenosis Pathway calcium, and metabolic or enzymatic abnormalities. 7 Pathophysiology The prevalence of mitral stenosis in the United States is estimated at 100 patients per 100,000 population. 6 Disease course Clinical manifestations of mitral stenosis often occur when there is an increased demand for cardiac output, and thus more transmitral flow, which causes elevation of the resting transmitral gradient. This may occur with pregnancy, fever, arrhythmias, infection, thyrotoxicosis, or during exercise. Mitral stenosis is associated with a significantly increased risk of AF. 8 Prognosis and risk of recurrence In patients with degenerative mitral stenosis, 1- and 5-year survival rates are estimated at 78% and 47%, respectively. Mortality is correlated with echocardiographic severity grades. 9 Calculator Calculator Calculator Cardiac Anesthesia Risk Evaluati NYHA functional classification f Revised C Guidelines 1. Diagnostic investigations Transthoracic echocardiogram: Obtain TTE in patients with signs or symptoms of rheumatic mitral stenosis to establish the diagnosis, quantify hemodynamic severity, assess concomitant valvular lesions, and demonstrate valve morphology (to determine suitability for mitral commissurotomy). B Obtain TTE to assess the presence or absence of LA thrombus and to evaluate the severity of MR in patients considered for percutaneous mitral balloon commissurotomy. B Exercise testing: obtain exercise testing with Doppler or invasive hemodynamic assessment in patients with rheumatic mitral stenosis and a discrepancy between resting echocardiographic findings and clinical symptoms, to evaluate symptomatic response, exercise capacity, and the response of the mean mitral gradient and pulmonary artery pressure. B 2. Diagnostic procedures https://web.pathway.md/diseases/reci4ziz64UyvvxCW 2/8 6/30/23, 1:05 AM Mitral stenosis Pathway Left heart catheterization: obtain invasive hemodynamic assessment or exercise testing with Doppler in patients with rheumatic mitral stenosis and a discrepancy between resting echocardiographic findings and clinical symptoms, to evaluate symptomatic response, exercise capacity, and the response of the mean mitral gradient and pulmonary artery pressure. B 3. Medical management Heart rate control: Consider initiating HR control in patients with rheumatic mitral stenosis and AF with a rapid ventricular response. C Consider initiating HR control to manage symptoms in patients with rheumatic mitral stenosis in normal sinus rhythm with symptomatic resting or exertional sinus tachycardia. B Anticoagulant therapy: initiate anticoagulation with a vitamin K antagonist in patients with rheumatic mitral stenosis and AF, a prior embolic event, or an LA thrombus. B 4. Therapeutic procedures Percutaneous mitral balloon commissurotomy, asymptomatic patients: As per AHA/ACC 2021 guidelines: Consider performing percutaneous mitral balloon commissurotomy at a comprehensive valve center in asymptomatic patients with severe rheumatic mitral stenosis (mitral valve area 1.5 cm , stage C) and favorable valve morphology with < 2+ MR in the absence of LA thrombus and with elevated pulmonary pressures (pulmonary artery systolic pressure > 50 mmHg). C Consider performing percutaneous mitral balloon commissurotomy at a comprehensive valve center in asymptomatic patients with severe rheumatic mitral stenosis (mitral valve area 1.5 cm , stage C) and favorable valve morphology with < 2+ MR in the absence of LA thrombus and with new-onset AF. C As per EACTS/ESC 2021 guidelines, consider performing percutaneous mitral balloon commissurotomy in asymptomatic patients with favorable clinical and anatomical characteristics for percutaneous mitral balloon commissurotomy and: high thromboembolic risk (history of systemic embolism, dense spontaneous contrast in the left atrium, new-onset or paroxysmal AF), and/or high risk of hemodynamic decompensation (systolic pulmonary pressure > 50 mmHg at rest, need for major non-cardiac surgery, desire for pregnancy). C Percutaneous mitral balloon commissurotomy, symptomatic patients: As per AHA/ACC 2021 guidelines, perform percutaneous mitral balloon commissurotomy at a comprehensive valve center in symptomatic patients (NYHA class II-IV) with severe rheumatic mitral stenosis (mitral valve area 1.5 cm , stage D) and favorable valve morphology with less than moderate (2+) MR in the absence of LA thrombus. A Show 3 more https://web.pathway.md/diseases/reci4ziz64UyvvxCW 3/8 6/30/23, 1:05 AM Mitral stenosis Pathway As per EACTS/ESC 2021 guidelines, perform percutaneous mitral balloon commissurotomy in symptomatic patients with favorable characteristics for percutaneous mitral balloon commissurotomy. B Show 2 more 5. Surgical interventions Mitral valve surgery: As per ACC 2021 guidelines, perform mitral valve surgery (repair, commissurotomy, or valve replacement) in severely symptomatic patients (NYHA class III-IV) with severe rheumatic mitral stenosis (mitral valve area 1.5 cm , stage D) not suitable for percutaneous mitral balloon commissurotomy, failed a previous percutaneous mitral balloon commissurotomy, requiring other cardiac procedures, or not having access to percutaneous mitral balloon commissurotomy. B As per ESC 2021 guidelines, perform mitral valve surgery in symptomatic patients not suitable for percutaneous mitral balloon commissurotomy in the absence of futility. B 6. Specific circumstances Pregnant patients (evaluation): obtain clinical evaluation and TTE before pregnancy in female patients with suspected valve disease considering pregnancy. B Show 3 more Pregnant patients (medical therapy): consider initiating -blockers as required for HR control or treatment of arrhythmias in pregnant patients with VHD. C Show 2 more Pregnant patients (interventions): offer intervention before pregnancy on the basis of standard indications in symptomatic patients with severe VHD considering pregnancy. B Show 5 more Patients with atrial fibrillation, anticoagulation: As per AHA/ACC 2021 guidelines, initiate long-term oral vitamin K antagonists in patients with rheumatic mitral stenosis and AF. B As per EACTS/EHRA/ESC 2021 guidelines: Do not use direct OACs in patients with AF and moderate-to-severe mitral stenosis. D Do not use direct OACs in patients with a prosthetic mechanical valve. D As per CCS 2018 guidelines, prefer warfarin over direct OACs in patients with rheumatic mitral stenosis or a mechanical prosthetic valve. B As per NHFA 2018 guidelines: Prefer warfarin in patients with valvular AF (mechanical heart valves or moderate-to-severe mitral stenosis). Do not use direct OACs. B https://web.pathway.md/diseases/reci4ziz64UyvvxCW 4/8 6/30/23, 1:05 AM Mitral stenosis Pathway Obtain point-of-care INR measurement in the primary care management of patients receiving warfarin. B Patients with atrial fibrillation (surgical management): discuss the potential symptomatic benefits and additional procedural risks of adjunctive arrhythmia surgery at the time of cardiac valvular surgery, if surgical intervention is planned, in patients with VHD and AF. B Show 4 more Patients with prosthetic valve stenosis: obtain TTE or TEE in patients with suspected mechanical or bioprosthetic valve stenosis, to diagnose the cause and severity of valve obstruction, assess ventricular function, and estimate pulmonary artery systolic pressure. B Show 5 more Patients with mixed valve disease: Obtain TTE to assess the etiology, severity, and pathophysiological impact in patients with mixed valve disease. B Consider obtaining further assessment of filling pressure by using biomarkers or invasive hemodynamic measurements at rest or with exercise in patients with ambiguous symptoms suspected to be attributable to mixed mitral valve disease. C Patients with coronary artery disease: As per ACC 2021 guidelines, obtain invasive coronary angiography before valve intervention in patients with symptoms of angina, objective evidence of ischemia, decreased LV systolic function, history of coronary artery disease, or coronary risk factors (including males > 40 years of age and postmenopausal females). B Show 2 more As per ESC 2021 guidelines, obtain coronary angiography before valve surgery in patients with severe VHD and any of the following: history of CVD suspected myocardial ischemia LV systolic dysfunction male > 40 years of age postmenopausal female 1 cardiovascular risk factors. B Show 4 more Patients undergoing non-cardiac surgery: obtain preoperative echocardiography in patients with clinically suspected moderate or greater degrees of valvular stenosis undergoing non-cardiac surgery. B Show 2 more 7. Preventative measures https://web.pathway.md/diseases/reci4ziz64UyvvxCW 5/8 6/30/23, 1:05 AM Mitral stenosis Pathway Secondary prevention of rheumatic fever: initiate secondary prevention of rheumatic fever in patients with rheumatic heart disease. B Clinical findings Patient demographics Symptoms Elderly age Chest pain Dizziness Past medical history Fainting Fatigue Acute rheumatic fever Hemoptysis CKD Hoarseness Carcinoid syndrome Palpitations Congenital heart disease Shortness of breath when lying down Coronary artery disease Diabetes mellitus Shortness of breath with exertion Fabry disease Hypertension Infective endocarditis Head and neck exam Pulmonary hypertension Facial erythema Rheumatic heart disease Mitral facies Rheumatoid arthritis SLE Respiratory exam Untreated Streptococcal infection Crackles VHD ECG findings Cardiac exam AF Austin-flint murmur Cardiac arrhythmias Diastolic cardiac murmur Graham Steell murmur Jugular venous distention Loud S1 Opening snap Abdominal exam Ascites Hepatomegaly Imaging findings Hockey stick mitral valve LA enlargement MR https://web.pathway.md/diseases/reci4ziz64UyvvxCW 6/8 6/30/23, 1:05 AM Mitral stenosis Pathway Mitral annular calcification Pulmonary edema Tricuspid regurgitation gradient across the mitral valve Likelihood Ratios Pertinent positives The following findings increase the probability of mitral stenosis in adults. 9 Finding LR+ Value Presence of diastolic cardiac murmur 10.6 Pertinent negatives The following findings decrease the probability of mitral stenosis in adults. 9 Finding LR- Value Absence of diastolic cardiac murmur 0.28 Studies 2018 Zochios In adult patients with pre-existing respiratory disease undergoing elective cardiac surgery, high-flow nasal oxygen was superior to standard oxygen therapy with respect to the total length of hospital stay. V Zochios et al. Anaesthesia. 2018 Dec. 2013 RE-ALIGN In patients who had undergone aortic or mitral valve replacement within the past 7 days and those who had undergone such replacement at least 3 months earlier, dabigatran was inferior to warfarin with respect to death, stroke, systemic embolism, or myocardial infarction. Eikelboom JW et al. N Engl J Med. 2013 Sep 26. Show 1 more References 1. Catherine M Otto, Rick A Nishimura, Robert O Bonow et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart https://web.pathway.md/diseases/reci4ziz64UyvvxCW 7/8 6/30/23, 1:05 AM Mitral stenosis Pathway Association Joint Committee on Clinical Practice Guidelines. J Thorac Cardiovasc Surg. 2021 Aug;162 2):e183-e353. Open 2. 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