Rheumatic Heart Disease

Original Editor - Lucinda hampton

Top Contributors -  

Key Facts[edit | edit source]

  • Rheumatic Fever.png
    Rheumatic heart disease is the most commonly acquired heart disease in people under age 25.
  • Rheumatic heart disease and claims over 291,000 lives each year - the large majority in low- or middle-income countries[1]. Over the last few decades an uncontrolled epidemic of rheumatic heart disease has been uncovered in developing countries and in Indigenous populations[2]
  • The disease results from damage to heart valves caused by one or several episodes of rheumatic fever, an autoimmune inflammatory reaction to throat infection with group A streptococci (streptococcal pharyngitis or strep throat). It most commonly occurs in childhood, and can lead to death or life-long disability (it seems far-fetched to think a sore throat or skin sore could take a lasting toll on your health, leading to heart failure and premature death. But this is the reality for many Indigenous children and those in low and middle income countries).[2]
  • Rheumatic heart disease can be prevented by preventing streptococcal infections, or treating them with antibiotics when they do occur[1].
  • Rheumatic heart disease causes at least 200000 to 250000 premature deaths every year. It is also the major cause of cardiovascular death in children and young adults in countries that have poor medical attention. If the valvular disease is not serially monitored, the patient may not present until severe heart failure is present resulting in surgical contraindication[3]

Etiology[edit | edit source]

Heart Valves.jpg

Rheumatic heart disease results from either single or repeated attacks of rheumatic fever that results in rigidity and deformity and progressive fibrosis of heart valves. Over 2 to 3 decades, valvular stenosis and/or regurgitation results.

In chronic rheumatic heart disease

  • The mitral valve alone is the most commonly affected valve in an estimated 50 to 60% of cases.
  • Combined lesions of both the aortic and mitral valves occur in 20% of cases.
  • Involvement of the tricuspid valve occurs in about 10% of cases but only in association with mitral or aortic disease (tricuspid valve cases are thought to be more common when recurrent infections have occurred).
  • The pulmonary valve is rarely affected[3].

Epidemiology[edit | edit source]

Rheumatic fever mostly affects children and adolescents in low and middle-income countries, especially where poverty is widespread and access to health services is limited. People who live in overcrowded and poor conditions are at greatest risk of developing the disease.

Where rheumatic fever and rheumatic heart disease are endemic, rheumatic heart disease is the principal heart disease seen in pregnant women, causing significant maternal and perinatal morbidity and mortality. Pregnant women with rheumatic heart disease are at risk of adverse outcomes, including heart arrythmias and heart failure due to increased blood volume putting more pressure on the heart valves. It is not uncommon for women to be unaware that they have rheumatic heart disease until pregnancy.

  • Despite it being eradicated in many parts of the world, the disease remains prevalent in sub-Saharan Africa, the Middle East, Central and South Asia, the South Pacific, and among immigrants and older adults in high-income countries, especially in indigenous peoples.[1]
  • Rheumatic heart disease was common until the 1960s in wealthy populations. Since then, improved living standards in high-income countries have reduced the transmission of the bacterial infection. A combination of less crowded housing, improved sanitation and better access to health services also resulted in a dramatic decrease in the incidence of acute rheumatic fever and resultant heart disease[2].

Symptoms[edit | edit source]

Rheumatic fever is the primary cause of acquired heart disease in children and young adults worldwide. Rheumatic fever occurs 2 to 3 weeks after a group A beta-hemolytic streptococcal pharyngeal infection.

Symptoms of rheumatic heart disease depend on the degree of valve damage and may include:

Rheumatic heart disease predominantly affects the left-sided cardiac valves. The tricuspid valve and rarely pulmonary valve can be affected, but very unlikely without mitral valve involvement.[3]

As noted above: pregnant women with rheumatic heart disease are at risk of adverse outcomes, including heart arrythmias and heart failure due to increased blood volume putting more pressure on the heart valves. It is not uncommon for women to be unaware that they have rheumatic heart disease until pregnancy.

Diagnosis[edit | edit source]

Apical-4-chamber-view-normal-transthoracic-echocardiography.jpeg

Rheumatic heart disease (RHD) is diagnosed using an echocardiogram (ultrasound) machine. In 2012 the World Heart Federation published an evidence-based guideline for diagnosing RHD on echocardiogram, including classifications for mild, moderate and severe RHD. There is also a borderline RHD classification for people aged under 20 years.4

  • Before echocardiography was widely available, RHD was commonly diagnosed using a stethoscope to identify abnormal heart sounds (murmurs). Diagnosis of RHD with echocardiography has been found to be more accurate than using a stethoscope alone.
  • Screening for RHD in populations at high risk can identify people with RHD who do not have symptoms. Screening is also performed using echocardiogram machines.[4]

Image 3: Echocardiogram, Abbreviations: LA (left atrium), RA (right atrium), IAS (interatrial septum), MV (mitral valve), TV (tricuspid valve), MVAL (mitral valve anterior leaflet), MVPL (mitral valve, posterior leaflet), LV (left ventricle), IVS (interventricular septum).

Treatment[edit | edit source]

Mechanical heart valves.jpeg

There is no cure for rheumatic heart disease and the damage to the heart valves are permanent. Patients with severe rheumatic heart disease will often require surgery to replace or repair the damages valve or valves. Depending on the severity of disease, medication may also be needed to treat symptoms of heart failure or heart rhythm abnormalities. Medications which thin the blood to reduce the risk of blood clots may also be needed. In the case of serious disease surgery may be required to repair or replace the heart valves. This is often not available in low-income settings, or when it is available the costs may be too high if not covered as part of national health plans, putting families under increased financial strain[1].

  • Percutaneous mitral balloon valvuloplasty is the standard first-line therapy for cases of rheumatic mitral stenosis in the absence of regurgitation, arrhythmias and left atrial thrombus. Surgical intervention is commonly the mainstay of treatment in severe cases of valvular disease. Surgical intervention can either be valve replacement or repair.
  • If a patient develops heart failure due to valve disease, the patient should be placed on medical therapy as tolerated for heart failure including ACE inhibitors, diuretics, and beta blockers.
  • At this time, there is no cure of rheumatic heart disease[3].

Image 4: Different types of mechanical heart valves.

Complications[edit | edit source]

Rheumatic heart disease is generally latent or silent until cardiac complications develop in late adulthood. Infective endocarditis, an embolic event, heart failure, pulmonary hypertension, and atrial fibrillation from untreated severe valvular disease are the most common complications.[3]

Physiotherapy[edit | edit source]

see Cardiac Rehabilitation

Palliative Care[edit | edit source]

Unfortunately, most children and young adults in the world with RHD do not have access to life-saving cardiac surgical intervention. Prognostication is difficult, and young patients often decompensate gradually over the course of weeks, months, or years as the result of congestive cardiac failure, pulmonary hypertension, and atrial arrhythmias. It is the terrible reality that patients from remote and resource-poor settings often receive no formal counseling or palliative care support. Diuretic drugs and morphine are proven to reduce respiratory distress and improve the quality of end-of-life care, yet these are not currently available to the majority of the world’s population. While awaiting socioeconomic and political changes to rid the world of RHD, children and young adults should have the right access to basic and culturally appropriate palliative care, which has been shown to reduce depression and anxiety and to improve quality of life scores[5].

See also Physiotherapy in Palliative Care

References[edit | edit source]

  1. 1.0 1.1 1.2 1.3 WHO RHD Available from: https://www.who.int/news-room/fact-sheets/detail/rheumatic-heart-disease(accessed 20.4.2021)
  2. 2.0 2.1 2.2 The conversation Why are Aboriginal children still dying from rheumatic heart disease? Available from: https://theconversation.com/why-are-aboriginal-children-still-dying-from-rheumatic-heart-disease-63814(accessed 20.4.2021)
  3. 3.0 3.1 3.2 3.3 3.4 Dass C, Kanmanthareddy A. Rheumatic Heart Disease. [Updated 2020 Nov 21]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-.Available from:https://www.ncbi.nlm.nih.gov/books/NBK538286/#_NBK538286_pubdet_ (accessed 20.4.2021)
  4. RHD Australia RHD Available from:https://www.rhdaustralia.org.au/how-are-acute-rheumatic-fever-and-rheumatic-heart-disease-diagnosed-and-managed (accessed 20.4.2021)
  5. Kumar, R.K., Antunes, M.J., Beaton, A., Mirabel, M., Nkomo, V.T., Okello, E., Regmi, P.R., Reményi, B., Sliwa-Hähnle, K., Zühlke, L.J. and Sable, C., 2020. Contemporary diagnosis and management of rheumatic heart disease: implications for closing the gap: a scientific statement from the American heart associationCirculation142(20), pp.e337-e357.Available: https://www.ahajournals.org/doi/10.1161/CIR.0000000000000921(accessed 21.4.2021)