Rheumatic Fever and Rheumatic heart disease are complications of throat infection caused by a bacterium called ‘Group A beta hemolytic streptococcus‘(GABHS). Though they are rare in developed countries, in INDIA it is still a major health problem among children and young adults affecting 1 to 5 per 1000 school children.
Rheumatic fever (RF):
RF usually affects children of 5-15 yrs of age. Most children develop at least one episode of throat infection every year of which 15-20% are caused by GABHS. Among children who have throat infections due to GABHS, 0.3-3% develop rheumatic fever.
FEATURES OF RHEMATIC FEVER:
Heart involvement in rheumatic fever occurs in 34-40% of patients and in the majority, it occurs within 2 weeks of RF. It has varied clinical presentation including undetectable heart involvement to severe heart failure, leakage of the mitral or aortic valve of varying severity, affection of layers of the heart or heartbeat irregularities. It affects all layers and structures of the heart. It is a serious illness and causes death in 0.3 to 1.6% of patients and some will need emergency valve replacement surgery and most of them will proceed to have long-term problems due to rheumatic heart disease.
Fever occurs in all patients, of about 101 to 1020f and does not have any characteristic pattern and rarely lasts more than several weeks.
Joint pain with out swelling is very common and usually involves large joints, may be mild to incapacitating and may last from days to weeks. Joint involvement with pain and swelling occurs in 67% (2/3rd) of all patients with Rheumatic fever. Especially large joints are involved. In a particular joint, swelling and pain usually lasts for about 2-3 weeks only and the entire bout of joint involvement lasts no longer than a month and then it heels with out permanent damage.
20% of Rheumatic fever patients develop chorea, a disorder that manifests as emotional lability, in coordination and muscle weakness and have involuntary movements of hands and feet and face.
3% of RF patients develop nodules which are round, firm, freely movable painless swellings of 0.5 to 2.0 cm size and skin over then are not red or painful. They occur in crops over bony prominences, persist from days to 1-2 weeks.
2% of patients with RF develop a non-painful, non-itching rash which appears as multiple bright pink patches over trunk, arms or thighs that spreads outwards in a circular or curved pattern and fade with pressure and are only rarely raised.
Bleeding nose can occur in 4-9% of patients and in some may be severe enough to need blood transfusion.
Pain abdomen occurs in about 5% of patients and may occur hours to days before other major manifestations develop.
Most important treatment for rheumatic fever is penicillin. Also penicillin is must to prevent recurrences because recurrence rate of RF is 50% in patients who already had rheumatic fever earlier.
Chronic rheumatic heart disease:
CRHD is the long term sequelae of acute rheumatic fever which manifests mainly as affection of heart valves either as narrowing or leakage or both after a considerable duration after acute rheumatic fever of as less as 3-5 yrs in some patients in developing countries to as much as 20-40 yrs in western countries.
Mitral stenosis is narrowing of the mitral valve (the valve between the two left heart chambers) leading to increased pressure in LA leading to congestion of lungs leading to breathlessness, enlargement of left atrium heart chamber, heartbeat irregularities paralysis, and other problems due to occlusion of blood vessels with clots that form in the heart increased pressures in the blood vessels supplying the lungs and other problems and will need heart valve repair (PTMC) or replacement apart from medication. Once symptomatic, if untreated, only 0 to 15% of patients survive at the end of 10 yrs and if the severe increase in pressures in blood vessels that supply blood to lungs occurs, average survival is less than 3 yrs.
Mitral regurgitation: Mitral regurgitation is leakage of mitral left-sided heart valve leading to back flow of blood from the left lower chamber to left upper chamber during heart contraction. This leads to an increase in the size of left-sided heart chambers and later poor functioning of the left lower heart chamber. In patients with severe leakage of heart valve with normal heart pumping function and without any symptoms, there is a high chance of developing symptoms or weakness of heart or need for open-heart surgery with valve replacement within 6 to 10 yrs. In patients with severe leakage of the heart with symptoms or weakness of heart, 90% of patients are dead or need MVR in 10yrs.
Aortic stenosis is the narrowing of the aortic heart valve present between the left lower heart chamber and aorta, the main blood vessel supplying blood to the entire body. This causes increased pressure load on the heart leading to very high pressures in the heart, increased thickness of the heart as compensation for increased pressure, and eventually may weaken the heart. Once symptoms develop, (chest pain, fainting episodes, or breathlessness) average survival is only 2-3yrs.
Leakage of aortic heart valve leads to backflow of blood from aorta to Left heart during heart relaxation phase leading to increased load on the left heart leading to increase in size and thickness of left lower heart chamber and later weakens the heart. Once symptoms (chest pain, breathlessness) develop, they have death rate of 10-20% per year.
In addition, patients may have involvement of right heart valves also. They may also have weakness of the heart due to heart muscle involvement and various other problems due to increased pressure in blood vessels supplying lungs, formation of blood clots in the heart and their dislodgement to various organs, infection in the heart etc.
Also patients will usually have mixed and multiple valvular lesions with variable and less predictable course of disease.
All patients with need lifelong penicillin prophylaxis and patients with significant valvular involvement will also need valvular interventions (repair /replacement) in addition to medication.