Rheumatic Fever & Rheumatic Heart Disease QuizOnline version
RF and RHD basics
1
What organism is primarily responsible for rheumatic fever?
2
Which criteria are used for the diagnosis of acute rheumatic fever?
3
What is the primary treatment to prevent recurrent attacks of rheumatic fever?
4
What infection must be treated promptly to prevent rheumatic fever?
5
Name two major Jones criteria.
6
You are seeing a 7-year-old boy in clinic with mitral valve regurgitation from a prior episode of rheumatic fever. How would you ask the patient to be positioned in order to best hear his murmur?
7
A 13-year-old child with polyarthritis has the ECG shown in Figure 6.8. Which of the following may the patient also have?
8
A 12-year-old girl presents with a high-pitched, blowing, holosystolic murmur heard best over the apex of the chest. An echocardiogram confirms moderate mitral valve regurgitation with thickening of the mitral valve. Suspecting rheumatic heart disease (RHD), you send streptococcal antibody titers, which are elevated. Which of the following additional findings would most strongly support the diagnosis of acute RHD?
9
Which of the following is the most common presentation of chronic rheumatic heart disease?
10
A 17-year-old male is referred to pediatric cardiology for evaluation of claudication. His physical examination revealed decreased femoral pulses. A transthoracic echocardiogram showed a diffusely narrowed descending thoracic and abdominal aorta. Which of the following is associated with vasculopathy leading to stenosis of large vessels?
11
An asymptomatic 22-year-old cancer survivor of thoracic lymphoma was previously treated with chemotherapy and radiation therapy. A surveillance echocardiogram showed calcification and thickening of the mitral valve annulus. This pattern of valvular involvement is typical of which of the following?
12
A 7yo male presents with 4d of fever, 3w after being treated for acute pharyngitis with oral amoxicillin. Reports right knee and right elbow swelling, erythema, and pain. Tachypnea & increased WOB is noted. PE reveals a 3/6 holosystolic murmur at the cardiac apex radiating to the left axilla. CXR showed mild cardiomegaly with increased pulmonary vascular markings and hazy lung fields. Laboratory evaluation showed high CRP and serum streptococcal antibody titers. Echo = mod MR. Treatment?
13
A 7yo female whose parents recently emigrated from Mexico is diagnosed with acute rheumatic fever (RF). She complains of mild chest pain, but no shortness of breath. PE reveals normal S1 and S2, with a soft holosystolic murmur at the apex. Neck veins do not appear to be distended. No organomegaly. Echocardiogram shows small pericardial effusion, mild to moderate MR, mild AR regurgitation, mildly dilated LV with EF of 60%. Which of the following treatment regimens should be initiated?
14
A 7yo female who was appropriately treated for her first episode of RF w/mild carditis is followed up at 1 year. Serial follow-up echocardiograms show no effusion, trivial MR, no AR, normal LV size/fx. Maintained on RF antibiotic ppx & is asymptomatic without any recurrence of streptococcal sore throat. If there is no echocardiographic evidence of worsening ventricular or valvular function, which of the following is the best recommendation for ongoing secondary antibiotic prophylaxis?
Feedback
The murmur of rheumatic heart involvement of the mitral valve is typically high pitched, holosystolic, heard best at the apex, and radiating to the left axilla. The murmur will be heard best at the end of expiration while the patient is lying in the left lateral decubitus position
The ECG shows a prolonged PR interval of around 400 ms. ECG evidence of PR interval prolongation is a minor Jones criteria for acute rheumatic fever. To make a diagnosis of acute rheumatic fever, you need two major or one major and two minor criteria. The clinical manifestations of acute rheumatic fever follow the inciting group A streptococcal infection after a period of latency of about 3 weeks. Rheumatic fever is a multisystem disease affecting primarily the heart, the joints, the brain, and the cutaneous and subcutaneous tissues. Carditis associated with acute rheumatic fever is seen in about 50% of the patients. Tachycardia is one of the early signs of myocarditis. Complete heart block is not usually seen in rheumatic carditis. Choreiform movements are rapid jerking movements of the hands, face, and feet and are characteristics of rheumatic fever. Kawasaki disease resulting in coronary artery aneurysms, Marfan syndrome resulting in a dilated ascending aorta, and juvenile rheumatoid arthritis do not typically give a prolonged PR interval. Systemic lupus erythematosus and scleroderma may also be associated with a prolonged PR interval. Glomerulonephritis is not associated with acute rheumatic fever and does not affect the PR interval. A bifid uvula is associated with Loeys–Dietz syndrome, which does not result in PR prolongation.
Rheumatic fever is diagnosed based on the Jones criteria. The probability is high if there is group A streptococcal infection as well as two major criteria or one major and two minor criteria. The five major criteria are migratory arthritis, carditis and valvulitis, central nervous system involvement/Sydenham chorea, erythema marginatum, and subcutaneous nodules. The four minor criteria are arthralgia, fever, elevated ESR or CRP, and prolonged PR interval. The patient has one major criterion—valvulitis. To confirm the diagnosis, one more major criterion would need to be present. Otherwise, two minor criteria would be required to make the diagnosis.
Mitral valve stenosis in isolation and in combination with aortic valve disease is the most common manifestation of chronic rheumatic heart disease.
HIV disease can cause vasculitis of the aorta and pulmonary arteries that can lead to diffuse stenosis. Late syphilis causes occlusion of vasa vasorum of large vessels resulting in aneurysmal dilation, not stenosis, of aorta. Other options are not associated with large vessel vasculopathy.
Thoracic radiation exposure can lead to a degenerative process of the mitral valve which typically starts from the annulus and progresses toward the leaflet tips. Anthracycline exposure is more commonly associated with cardiomyopathy. Infective and nonbacterial thrombotic endocarditis typically do not involve the valve annulus primarily.
The patient has acute rheumatic fever with carditis. The echocardiogram shows severe mitral valve regurgitation, which is the most common manifestation of acute rheumatic carditis. Left heart enlargement and a small amount of pericardial effusion along the right atrium and right ventricle can also be noted. The treatment includes (1) antibiotics against group A streptococcal infection, (2) anti-inflammatory therapy with NSAIDs or high-dose aspirin, and (3) treatment of congestive heart failure. Penicillin G benzathine is a long-acting intramuscular injection that serves as prevention of group A streptococcal infection. Naproxen has favorable side effect profile compared to high-dose aspirin and is a preferred choice as an anti-inflammatory agent. Furosemide is needed in this child as there is evidence of respiratory distress and pulmonary edema from mitral regurgitation. IVIG and high-dose aspirin are first-line treatment for Kawasaki disease. IVIG is typically not used in acute rheumatic fever unless there is severe Sydenham chorea which is not responding to other therapy.
The patient has mild to moderate carditis that needs therapy with high-dose aspirin (80 to 100 mg/kg/d in four divided doses in children). Oral prednisone is indicated for more severe carditis associated with a sicker patient in the setting of heart failure, severe valvular regurgitation, significant pericarditis/myocarditis, or reduced cardiac function. There is no recommendation for combining oral steroids with aspirin for treatment of acute RF. IVIG + aspirin is used in the treatment of KD. There is no indication for a β-blocker in pericarditis.
The patient had mild carditis during RF but is free of residual heart disease now. Per guidelines, she will need RF antibiotic prophylaxis for at least 10 years or until 21 years of age, whichever is longer.
As per the current guidelines,
RF patients with carditis and residual heart disease (persistent valvular disease) should receive treatment for a duration of 10 years or until 40 years of age (whichever is longer, sometimes lifelong) after the last attack of RF.
RF patients with carditis but without residual heart disease (no valvular disease) should receive treatment for a duration of 10 years or until 21 years of age (whichever is longer) after the last attack of RF.
RF patients without carditis should receive treatment for a duration of 5 years or until 21 years of age (whichever is longer) after the last attack of RF.
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Spanish alphabet, listening-based assessment