Acquired Heart disease

Dr. Shirsat

Case


   

A 9-year-old Hispanic boy is brought to the 9-yearemergency department because of fever, fever, headache, muscle aches, and abdominal pain for 2 days. He says that he has had no cough, vomiting, diarrhea, constipation, or urinary symptoms. There is no history of skin rash, trauma, or distant traveling. The other family members have been healthy. Two weeks prior to this episode the patient had been evaluated for fever and severe abdominal pain.

Physical examination
 Temperature

-102F 102  HR 146 beats/min,  Pain on palpation of the calves of both legs, and pain in the right ankle with passive movement (but no erythema, heat, or swelling).  Pharynx --no erythema and exudate. --no  no skin lesions.

laboratory data
 WBCWBC-

10,500/mm3;  Hg- 14 g/dL; Hg platelet count, 665,000/mm3;  ESR 100 mm/h;  A rapid streptococcal antigen detection test is negative

Repeat examination in 12 hours



The pulse rate is 160 beats/min and the apical impulse is hyperdynamic, felt best in the sixth intercostal space, on the left anterior axillary line. A holosystolic, high-pitched, blowing murmur is highheard at the apex. The murmur is 3/6 in intensity, transmitted to the left axilla, and loudest when the patient assumes the left decubitus position. The second sound is widely split and fixed, and an accentuated third heart sound is audible.

 Elevated

ESR,  Prolonged PR interval on the ECG,  Presence of arthralgia  A serum antistreptolysin O level was 810 Todd units, providing evidence of preceding group A beta-hemolytic betastreptococcal infection

Rheumatic Fever
 Epidemiology

manifestations  Identify murmurs of MI and AI  Major and minor criteria  Laboratory findings  Importance of Echocardiography  Initial management
 Clinical

Epidemiology
 Incidence

remains low  Age 4 to 14 years  3% of untreated sorethroats Rheumatic fever  1/3rd result from inapperant infection

Clinical Examination
holosystolic murmur that radiates to the left axilla and represents mitral regurgitation .  If the mitral regurgitation is severe, there may be a lowlowfrequency mid- to late diastolic murmur of relative mitral midstenosis, which is referred to as the Carey Coombs murmur.  The clinician also should listen for the early diastolic decrescendo murmur of aortic regurgitation which may be exaggerated when the patient is in the sitting position, leaning forward.  Signs and symptoms of congestive heart failure occur in about 5% of patients who have rheumatic carditis.


 Erythema

nodosum. A, Note the typical red, raised, tender nodules overlying the pretibial surfaces of the legs. B, This 1818monthmonth-old boy developed these tender, indurated, erythematous patches over his chest and abdomen after an upper respiratory infection.

 In

1992, the American Heart Association presented guidelines

The major manifestations

 Carditis  Polyarthritis  Subcutaneous

nodules  Erythema marginatum  Chorea

Minor manifestations
 Arthralgia

(not considered a criterion if polyarthritis is present)  Fever  Increased acute-phase reactants acute(erythrocyte sedimentation rate, CCreactive protein)  Prolonged P-R interval P-

Criteria for diagnosis

 Two

of the major manifestations or one of the major manifestations plus two of the minor manifestations.  Evidence of streptococcal infection such as positive strep culture,antigen detection test, Positive titers ( ASO, AntiDNAs B,antihyaluronidase)

Acute management of rheumatic fever

 Three

primary strategies:  Treat the infection causing the disease,  Control and alleviate the symptoms,  Provide supportive care as indicated.

Antibiotic
 10-day 10-

course of oral penicillin V  Intramuscular or intravenous penicillin  Erythromycin may be used.

Symptomatic
 

  

AntiAnti-inflammatory agents are important for symptomatic relief and to combat the inflammatory process. Salicylates are particularly effective for the migratory arthritis, with improvement often occurring in the first 12 to 24 hours of therapy. In addition, these medications frequently relieve the accompanying fever. Aspirin typically is administered in high doses (80 to 100 mg/kg per day to a serum level of 25 mg/dL) for several weeks before a gradual tapering is begun. For patients who cannot tolerate aspirin, the use of other nonsteroidal anti-inflammatory drugs may be considered. antiSteroids generally are reserved for patients who demonstrate moderate-tomoderate-to-severe carditis with congestive heart failure. Supportive care -- bed rest, especially during the acute phase of the illness.

Clinical diagnosis
 Sore

throat sudden onset  painfull swallowing  Fever

Throat culture
 Both

tonssils and posterior pharynx almost always positive  Does not differentiate carrier from infected

Antigen detection
 High

sensitivity low specificity  Cannot differentiate carrier from infected

Antibody
 Rising

titer only confirms recent infection  Can not differentiate infected vs carrier  ASO persists for several weeks falls first  AntiDNAs B high for several months

Treatment considerations
 Adherance  Cost  Spectrum  No

to recommended regimen

single regomen irradicates GAS from the pharynx

drug of choice  Within 9 days of symptoms  40mg/kg/day 10 days  Amoxicillin has no advantage
 Penicillin

 I.M

Benzathin penicillin G  Non compliance ( less treatment failure)  Family history of rheumatic fever  Overcrowding  600,000 units <27 kg  1200,000U >27kg

 Erythromycin

estolate 20 to 40 mg/kg/day  Erythromycin succinate 40mg/kg/day  No resistance in USA  Azythramycin 5 day course 10mg/kg, 5mg/kg  Cephalosporins ( Cephalexin, Cephadroxil narrow spectrum) 10 day course

Post treatment cultures are recommended

2

to 7 days after completion of treatment  Symptomatic  Recurring symptoms  Rheumatic fever  Hugh risk for recurrence

Repeat treatment
 Not

indicated in asymptomatic carriers

Indications For retreatment

 History

of rheumatic fever  Other members of family with history of rheumatic fever

Antibiotics
 Augmentin  Cephalosporins  Clindamycin  Penicillin

and rifampin

Carriers
cultures without illness or immunologic response  Little risk of Rheumatc fever  Not important in the spread of the disease
 Positive

Prevention of recurrent attacks

 Requires

continuous antibiotic prophylaxis  Family should be treated

Duration of prophylaxis
and residual heart disease 10 years since last episode and at least age 40 sometimes lifelong  Carditis and no residual heart disease 10 years and well in adulthood  No carditis-5 years or until age of 21 carditis Carditis

Prevention
 1200.000

units of Benzathin penicillin q 3 to 4 weeks  Oral for low risk compliant patients  Sulfonamides

You are evaluating an 8-year-old boy for fever, arthritis, and rash. He has 8-yearbeen ill for about 1 week, and he tells you that his knees and elbows have hurt at various times during the previous 5 days. His right knee is swollen, very tender with palpation and motion, and erythematous. The rash (Item Q248A) is irregular, pink, and nonpruritic, with macules that blanch in the center. His lungs are clear on auscultation. His first and second heart sounds are normal. You hear two distinct heart murmurs. One is holosystolic at the cardiac apex with radiation to the left axilla; the other is early in diastole with a decrescendo quality at the left sternal border and is accentuated in the sitting position. Of the following, the MOST likely cause of the murmurs is coronary aneurysms and a patent ductus arteriosus mitral insufficiency and aortic insufficiency mitral insufficiency and aortic stenosis mitral stenosis and aortic stenosis ventricular septal defect and aortic insufficiency

     

A 10-year-old girl has had fever, joint pain, and fatigue for 10 10-yeardays. She also has had symptoms of an upper respiratory tract infection several times during the past 2 months. Findings include normal joints, a grade I/VI high-pitched decrescendo diastolic highmurmur at both the right third intercostal space and left lower sternal border, and a grade II/VI high-pitched holosystolic highmurmur at the apex. The MOST likely explanation for these findings is A. acute rheumatic fever B. bicuspid aortic valve C. complete atrioventricular canal defect D. purulent pericarditis E. viral myocarditis

     

A 14-year-old boy complaines of dull chest pain over the 14-yearleft precordium. It began 4 days ago and occurs intermittently. It is not associated with activity, but it does increase when he is in the supine position and decreases when he is leaning forward. The frequency, duration, and intensity of the pain have been increasing. Among the following, the MOST likely explanation for these findings is A. acute rheumatic fever B. arrhythmia C. costochondritis D. myocardial ischemia E. pericarditis

     

An 8-year-old boy who had an upper respiratory tract 8-yearinfection 2 weeks ago now complains of joint pain. Physical examination reveals tenderness, redness, and swelling of the right ankle and left knee and a grade II/VI holosystolic murmur at the apex. Among the following, the laboratory study that is MOST likely to establish a diagnosis in this patient is a(n) A. antistreptococcal antibody titer B. C-reactive protein level C. erythrocyte sedimentation rate D. platelet count E. rheumatoid factor test

     

Two weeks after having a nonspecific upper respiratory tract infection, a previously healthy 3-year-old boy is 3-yearnoted to have a respiratory rate of 40 breaths/min, a heart rate of 140 beats/min, hepatomegaly, and a gallop rhythm. No heart murmurs are detected. Of the following, the MOST likely diagnosis is A. acute rheumatic fever B. infective endocarditis C. myocarditis D. paroxysmal atrial tachycardia E. pericarditis

     

A 3-year-old girl with a history of recent upper 3-yearrespiratory tract infection is noted to have mild peripheral edema, hepatomegaly, jugular venous distention, gallop rhythm, and cardiomegaly. She is afebrile. These clinical findings are MOST likely caused by A. aortic stenosis B. bacterial endocarditis C. congestive heart failure D. pneumonia E. rheumatic fever

     

An 11-year-old boy with a recent history of sore throat 11-yearcomplains of abdominal, leg, and arm pain. Physical examination reveals erythema and swelling of the right ankle and left knee; a grade I-II/VI high-pitched Ihighholosystolic murmur is heard at the apex. Of the following, the MOST likely diagnosis is A. bacterial endocarditis B. juvenile rheumatoid arthritis C. mitral valve prolapse D. polyarteritis nodosa E. rheumatic fever

     

Two weeks after having a nonspecific upper respiratory tract infection, a previously healthy 3-year-old boy is 3-yearnoted to have a respiratory rate of 40 breaths/min, a heart rate of 140 beats/min, hepatomegaly, and a gallop rhythm. No heart murmurs are detected. Of the following, the MOST likely diagnosis is A. acute rheumatic fever B. infective endocarditis C. myocarditis D. paroxysmal atrial tachycardia E. pericarditis

            

Which one ofthe following statements regarding coronary artery aneurysms due to Kawasaki syndrome is true? A. Aneurysmal rupture and hemorrhage is the most common mechanism of sudden death. B. Coronary artery aneurysms typically appear 3 months after the onset of Kawasaki syndrome. C. Risk ofcoronary artery aneurysm is decreased by intravenous gamma globulin therapy. D. Serial echoc&diographic evaluation at 3-month intervals over a 32 year period is necessary for all patients. E. There is no correlation between the duration of fever and the development of giant coronary aneurysm.

            

Which one ofthe following statements regarding coronary artery aneurysms due to Kawasaki syndrome is true? A. Aneurysmal rupture and hemorrhage is the most common mechanism of sudden death. B. Coronary artery aneurysms typically appear 3 months after the onset of Kawasaki syndrome. C. Risk ofcoronary artery aneurysm is decreased by intravenous gamma globulin therapy. D. Serial echoc&diographic evaluation at 3-month intervals over a 32 year period is necessary for all patients. E. There is no correlation between the duration of fever and the development of giant coronary aneurysm.

 The

most important factor in planning followfollow-up care for a child who has Kawasaki syndrome is:  A. Age of the patient.  B. Duration of the febrile illness.  C. Presence or absence of arthritis.  D. Presence or absence of coronary artery abnormalities.  E. Severity of the original liver disease.