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**Rosalie McDonough, MD, MSc**

Posted in [**Clinical Education**](https://www.ekohealth.com/blogs/clinical-education)

August 21, 2024

Mitral stenosis causes obstruction to blood flow from the left atrium to the left ventricle. This results in a backlog of blood before the valve, leading to symptoms such as atrial enlargement (which can cause atrial fibrillation and embolism), lung congestion (resulting in cough and dyspnea), and in severe cases, pulmonary edema. It's most commonly caused by rheumatic heart disease. Treatment for severe or symptomatic cases often involves balloon valvuloplasty or transcatheter/surgical valve replacement.

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#### **Etiology**

Rheumatic heart disease (RHD), a continuously progressive lifelong disease, is the most common cause of mitral stenosis and remains a major health concern in resource-limited countries.1,2

Other causes include:

- Mitral annular calcification3,4
- Radiation associated valve disease5,6
- Congenital (rare)4

#### **Pathophysiology**

RHD is an exaggerated immune response to untreated or inadequately treated streptococcal infection. Inflammation and fibrosis of the mitral valve leaflets leads to thickening, calcification, and fusion of the commissures and chordae tendineae.7,8 Over time, the valve opening becomes narrowed, restricting blood flow from the left atrium to the left ventricle.

_95% of mitral stenosis cases are caused by rheumatic fever in developing countries (shown in green).9_

#### **Symptoms/Clinical Presentation**

Duration of the asymptomatic phase varies widely, with more rapid progression in resource-limited countries. In addition, even in cases of severe disease, physical exam findings can be subtle or non-specific.

| Common symptoms include10: | -Exertional dyspnea<br>-Decreased exercise tolerance |
| --- | --- |
| Less common symptoms include: | -Fatigue<br>-Hemoptysis<br>-Chest pain |
| Symptoms may be precipitated by11: | -Physical exertion<br>-Emotional stress<br>-Fever<br>-Atrial fibrillation (AFib)<br>-Pregnancy |
| Complications: | -Right heart failure (ascites, lower extremity edema)<br>-AFib12,13<br>-Thromboembolic events (e.g., stroke)<br>-Hoarseness<br>-Infective endocarditis (rare)14 |

#### **Diagnostics**

Due to the slow progressive nature of mitral stenosis, a careful patient history and physical exam are required. Below is a non-exhaustive list of diagnostic methods:

**1\. Auscultation:**

- Loud first heart sound (S1) due to stiff leaflet motion
- Intensity of second heart sound (S2) is initially normal, but increases with pulmonary hypertension
- When AFib present, possible pre-systolic crescendo
- Low-pitched decrescendo mid-diastolic or pre-systolic murmur (“rumble”), often with opening “snap” (indicative of preserved leaflet mobility)
  - **Tip! → Best heard with stethoscope bell at the apex in left lateral decubitus position**
  - **Tip! → Duration of murmur correlates with severity of disease**

_Exemplary PCG: Mitral stenosis is a low-pitched, mid-diastolic or pre-systolic murmur that begins with an opening snap in early diastole as the mitral valve is forced open._

Sound recorded by [Eko digital stethoscope technology](https://www.ekohealth.com/pages/shop "Shop digital stethoscopes from Eko"):

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**2\. ECG:** There are no pathognomonic ECG changes, but there are common findings from the downstream effects on the heart.

- The physical dilation of the left atrium causes a left atrial abnormality, seen as a prolonged and bifid P wave in lead II (sometimes referred to as “P mitrale”) and a broader and deeper inverted component of the P wave in lead V1.
- However, for the many MS patients who develop [atrial fibrillation](https://www.ekohealth.com/blogs/clinical-education/atrial-fibrillation "Learn more about atrial fibrillation"), no P waves will be seen, and irregularly spaced QRS complexes will be observed instead.

**3\. Echocardiography:** For grading (e.g., trace, mild, moderate, severe), as well as determination of possible concomitant valve disease.

**4\. Chest X-ray:** Enlarged left atrium, signs of pulmonary congestion, right ventricular hypertrophy.

#### **Therapy**

- Asymptomatic patients: Conservative treatment and regular check-ups.
- For patients with heart failure symptoms or atrial fibrillation, medical management (e.g., diuretics, beta blockers, anti-arrhythmics, anticoagulants).
- For patients with advanced/concomitant heart disease: surgical or interventional options (e.g., balloon valvuloplasty, MitraClip, TMVR, surgical commissurotomy).

##### **References**

1. Olson, Lyle J., et al. “Surgical pathology of the mitral valve: A study of 712 cases spanning 21 years.” _Mayo Clinic Proceedings_, vol. 62, no. 1, Jan. 1987, pp. 22–34.

2\. Horstkotte, D., et al. “Pathomorphological aspects, Aetiology and natural history of acquired mitral valve stenosis.” _European Heart Journal_, vol. 12, no. suppl B, 2 July 1991, pp. 55–60.

3\. Kato, Nahoko, et al. “The natural history of severe calcific mitral stenosis.” _Journal of the American College of Cardiology_, vol. 75, no. 24, June 2020, pp. 3048–3057.

4\. Otto, Catherine M., 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 Association joint committee on clinical practice guidelines.” _Circulation_, vol. 143, no. 5, 2 Feb. 2021.

5\. Adabag, A. Selcuk, et al. “Critical stenosis of aortic and mitral valves after mediastinal irradiation.” _Catheterization and Cardiovascular Interventions_, vol. 63, no. 2, 23 Sept. 2004, pp. 247–250.

6\. Hull, Matthew C. “Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy.” _JAMA_, vol. 290, no. 21, 3 Dec. 2003, p. 2831.

7\. Carapetis, Jonathan R, et al. “Acute rheumatic fever.” _The Lancet_, vol. 366, no. 9480, July 2005, pp. 155–168.

8\. Rajamannan, Nalini M., et al. “Calcified rheumatic valve Neoangiogenesis is associated with vascular endothelial growth factor expression and osteoblast-like bone formation.” _Circulation_, vol. 111, no. 24, 21 June 2005, pp. 3296–3301.

9\. Chang, Christopher. “Cutting edge issues in rheumatic fever.” _Clinical Reviews in Allergy & Immunology_, vol. 42, no. 2, 20 May 2011, pp. 213–237.

10\. Chandrashekhar, Y, et al. “Mitral stenosis.” _The Lancet_, vol. 374, no. 9697, Oct. 2009, pp. 1271–1283.

11\. Van Hagen, Iris M., et al. “Pregnancy outcomes in women with rheumatic mitral valve disease.” _Circulation_, vol. 137, no. 8, 20 Feb. 2018, pp. 806–816.

12\. Diker, Erdem, et al. “Prevalence and predictors of atrial fibrillation in rheumatic valvular heart disease.” _The American Journal of Cardiology_, vol. 77, no. 1, Jan. 1996, pp. 96–98.

13\. Karthikeyan, Ganesan. “Stroke risk in rheumatic heart disease.” _Heart_, vol. 107, no. 9, 18 Jan. 2021, pp. 694–696.

14\. Zühlke, Liesl, et al. “Characteristics, complications, and gaps in evidence-based interventions in rheumatic heart disease: The Global Rheumatic Heart Disease Registry (the remedy study).” _European Heart Journal_, vol. 36, no. 18, 26 Nov. 2014, pp. 1115–1122.

##### **Medical Advice Disclaimer**

DISCLAIMER: THE CONTENT SET FORTH HEREIN DOES NOT PROVIDE MEDICAL ADVICE NOR IS AN ATTEMPT TO PRACTICE MEDICINE

The information, including but not limited to, text, graphics, images, and other material contained on this website are for informational purposes only. No material on this website or document are intended to be a substitute for professional medical education, advice, diagnosis, or treatment.

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#### About the Author

Rosalie McDonough, MD, MSc \- Clinician Scientist

Rosalie McDonough, MD, MSc, is a clinician scientist with a diverse background in medical research and clinical practice. She earned her Master of Science degree from McGill University in Montreal, Canada, later completing her medical degree at the University Medical Center Hamburg-Eppendorf in Hamburg, Germany. Dr. McDonough’s career has spanned various fields within medicine, focusing on clinical research and health technology. Her interests have encompassed cardiopulmonary disease, lung cancer, molecular cardiology, occupational medicine, and acute ischemic stroke imaging. She is currently the Senior Manager of Medical Affairs at Eko Health.

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