RHEUMATIC FEVER (RHEUMATIC HEART DISEASE)

Rheumatic fever is a disease characterized by the inflammation of many connective tissues throughout the body, particularly in the heart, joint and central nervous system (the brain and spinal cord). The condition is the result of “sore throat” caused by streptococcus a bacteria that has not been treated by antibiotics. Rheumatic fever can cause severe joint pain (e.g. in the knees) and fatigue. Although these symptoms are temporary, rheumatic fever can also damage the heart’s valves, chambers and vessels. Untreated, this damage can be permanent (rheumatic heart disease).

There is also a high likelihood of rheumatic fever developing again, and people will need to take antibiotics for the rest of their lives to avoid additional complications. Rheumatic heart disease may cause deformed heart valves, narrowed or constricted valves and atrial fibrillation – a rapid, abnormal heart rhythm (arrhythmia) caused by abnormal electrical signals from the upper chambers of the heart (atria).

Typically acquired in childhood (age five to 15), dangerous recurrences of rheumatic fever can develop whenever “strep throat” goes untreated. Each subsequent event can further damage the body’s tissues and organs. The heart muscle is at particularly high risk of further damage following each episode of rheumatic fever. Rheumatic fever is the most common form of acquired heart disease in the world, particularly in developing countries, where it can be present in more than one out of every 1000 people.

SIGNS AND SYMPTOMS

The initial “strep throat” infection will cause a fever over 100 degrees and a very sore throat. Untreated, rheumatic fever will eventually develop. The first symptoms of rheumatic fever tend to occur between one and six weeks after the initial infection, but some symptoms may not appear until six months later. Depending on the individual and the severity of the condition, these symptoms may include the following:

1.       Swelling, tenderness and arthritis-like pain in several larger joints (polyarthritis)

2.       Rash

3.       Fatigue

4.       Lack of appetite

5.       Difficulty with speech

                                                                                                                          

Signs of the condition that may be identified by a physician include the following:

1.       Carditis. Approximately 50 percent of patients show this swelling of the heart walls. Damage to a valve and/or chordate, which attach the valve to the heart wall, will mostly affect the mitral valve or aortic valve.

2.       Chorea. Occurring in about 20 percent of cases, chorea is a rheumatic inflammation in the central nervous system. Surprisingly, this condition, which can bring rapid involuntary movements, may not begin until more than 90 days after the onset of the initial streptococcal infection.

3.       Lumps under the skin

4.       Fever

 

Most signs and symptoms are temporary and will disappear without any lasting problems. However, the heart is vulnerable to sustaining permanent damage from rheumatic fever.

TESTS

There are no specific tests to diagnose rheumatic fever. When “strep throat” is suspected, a throat culture will be performed.

Blood tests such as the antistreptolysin-O test (ASO) may be more effective in revealing an underlying infection. Carditis may be detected during a physical examination if the physician hears a valvular murmur while listening to the heart through a stethoscope. Heart damage may also be identified with an echocardiogram (a form of ultrasound imaging that takes pictures of the heart’s structures and functions) or with an electrocardiogram (a test that measures the heart’s electrical activity).

Treatment and prevention

Medical intervention is critical to rid the body of the streptococcal infection. Penicillin therapy is the most commonly used and successful method for killing the infection, though other antibiotics may be used if a patient has an allergic reaction to penicillin. To relieve joint pain, the first step is plenty of bed rest to alleviate some of the pressure on the joints. As patients begin to heal, strenuous activity should be limited until they have fully recovered.

Other medications

1.       Salicylates. Pain relievers of which the most commonly known is aspirin, are used to relieve joint pain.

2.       Corticosteroids. Anti-inflammatory medications that may be prescribed for patients with pericarditis or heart failure.

3.       Heart valve replacement surgery is necessary if serious degeneration of the valve structures occurs.

 

Prevention

Quickly identifying and curing a streptococcal infection with penicillin or other antibiotics can prevent rheumatic fever and rheumatic heart disease. If rheumatic fever has occurred, there is a strong likelihood that the event will recur. Preventive measures must then be taken to stop further damage. Continual antibiotic treatment is usually required. A common preventive program is long-acting penicillin administered every three to four weeks.

RECENT RESEARCH ON VALVULAR HEART DISEASE

Biological heart valves last about 10 years before they start to fail due to tissue disintegration. Mechanical valves, which are made from metal or other man-made (synthetic) materials, are designed to last a lifetime. They are often used if all other factors are equal. However, mechanical valves carry a higher risk of blood clots, so patients with mechanical valves must take anticoagulants for life. Researchers, therefore, are continually exploring possible causes and treatments for heart valve diseases as well as the long term effects of those treatments. Recent findings include:

·         Stem cell research is being applied to congenital heart disease. Found in bone marrow, lymphatic tissue and embryos, immature stem cells can differentiate into specific, specialized body cells. In animal studies, for example, bone marrows isolated from sheep have been successfully cultured to have the properties of smooth muscle cells. These cells in turn could assume the structure and function of normal heart valves.

·         Robotically-assisted surgery is showing benefit of both simple and complex mitral valve repairs. Robotic surgery involves voice-activated robotic “hands” at the operating table, with the cardiac surgeon manipulating the hand controls. The surgeon views the procedure through an endoscope, a slim optical tube with an attached camera positioned inside the chest. Advantages of this and similar procedures are small incisions, less surgical trauma and a shorter operative and recovery period.    

·         Cells from a patient’s own blood vessels can be “grown” over biological valves taken from pigs or human cadavers Scientists remove the cells from the biological valve, leaving only elastic tissue that retains the valve’s shape. The patient’s cultured cells are then grown over the elastic tissue. After about one year, the new valve is implanted into the patient. It has been shown that this procedure resulted in fewer post-operative complications (e.g. fever, hospital stay) compared to conventional valve replacement.

·         Surgeons are exploring heart valve replacement without the need of open heart surgery. Typically, diseased or defective valves are replaced with an artificial valve or a tissue valve (from a pig or cow). The new, less invasive procedure, known as percutaneous transcatheter heart valve implantation, involves the use of balloon catheters and large stents introduced through a puncture in the skin (in the groin area, near the femoral vein). The new heart valve is transported via the stent to the site, where the stent is then expanded to implant the valve. For patients not able to undergo open-heart surgery, due to age and/or physical condition, percutaneous heart valve implantation may impact significantly on survival and quality of life.

·         Studies are evaluating whether medical (drug) therapy can offer improvement in aortic stenosis. Stenosis can develop due to a buildup of calcium, causing decreased mobility in the aortic valve. This calcium buildup is a form of atherosclerosis.

·         Statins, a type of cholesterol-reducing drug, have shown to be effective in reducing calcium deposits in and around the heart. Therefore, there is interest in this class of drugs for the treatment of aortic stenosis. In early studies, researchers found that, while lower cholesterol levels did not impact on aortic stenosis, statins slowed its progression. This could be due to its effect and reducing C-reactive protein and overall inflammation around the heart another cause of atherosclerosis.

·         Treating calcification of the aortic valve with ACE inhibitors is also being explored. These are medications that block the effects of angiotensin-converting enzymes, which normally have a role in blood pressure. It is believed that angiotensin-converting enzyme (ACE) is transported by low-density lipoproteins (LDLs, so-called “bad” cholesterol) into areas damaged by plaque, contributing to calcification.

 

Hope you liked this blog!

This article is written by Dr. Bimal Chhajer (India’s No. 1 heart specialist)