rf , carditis, cardiomyopathies

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بسم الله الرحمن الرحيم Acquired Heart Disease Done by: Abdullah Mohammed Al-osaily

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Rheumatic Faver Carditis Cardiomyopathy Definition Incidence Etiology & Risk Factors Prevention Evaluation Management

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Rheumatic Fever

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10 year old girl is brought into the pediatric clinical for first time with sudden onset of difficulty with handwriting and jerky movement. The mother states that the child had a sore throat 1 month earlier with quickly recovered .

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After seeing the child, the doctor suspect that the patient has rheumatic fever. In the addition to what you saw in the video, which of the following will confirm your diagnosis : - Increase antistreptolysin O “ ASO “ titer - Arthralgia - Erythema marginatum “clear centrally and erythema at the margin“in the face - Increase erythrocyte sedimentation rate - Nodule “ small firm painless nodule “ over the triceps tendon In absence of all other findings in this patient, what is the best treatment : - Acetaminophen - Aspirin - Penicillin - Penicillin + Aspirin - Penicillin + Phenobarbital - Penicillin + Phenobarbital + Aspirin - Phenobarbital - Supportive care and monitoring

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Definition rheumatic fever is an inflammatory disease involving mainly the joints and the heart and less frequently the nervous system, skin and subcutaneous tissues.

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Incidence Most common form of acquired heart disease worldwide . Most commonly affected age 5 – 15 years M = F “ F worse prognosis than M “ Rheumatic heart disease is responsible for 99% of mitral valve stenosis in adults in the US . Result from an immune reaction to untreated group A beta- hemolytic sterpt.

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Etiology Pharyngitis with GABH Strept. several weeks later 0.3 – 3 % Rheumatic fever Important predisposing factors include family history of rheumatic fever, low socioeconomic status (poverty, poor hygiene, medical deprivation).

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Clinical Manifestation

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Clinical Manifestation

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Major Criteria 1. Carditis : Manifest as pancarditis (endocarditis, myocarditis and pericarditis), most serious and 2nd most common manifestation occurs in 50% of patients. 1. A heart murmur typically due to valve insfficiency Mitral regurgitation (MR)  pansystolic murmur is most common. Active carditis,relative MS  mid-diastolic murmur “ Carey Coombs” The most commonly affected valve : Mitral 65 – 70 % , Aortic valve 25%, Tricuspid 10% ,pulmonary rare . 2. Pericarditis (friction rub, pericardial effusion, chest pain, and ECG changes) may be present. 3. Signs of CHF (gallop rhythm, distant heart sounds, cardiomegaly).

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Major Criteria 2. Arthritis : It is the most common manifestation (70% of cases) and usually appear early. Characterized by acute, painful, asymmetric, migratory and usually involves large joints These arthritis responds dramatically to salicylate therapy. “ if not, Dx is in doubt “

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Major Criteria 3. Sydenham’s Chorea : It is found in 15% of patients. It occurs more often in prepubertal girls (8 to 12 years). Characterized by :  hyperkinetic  rapid  unsustained irregular  purposeless

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Major Criteria 4. Erythema marginatum: occurs in < 10% of patients. characterized by red macules  which fade in the center but remain red at the edges . Nonpruritic Mainly in the trunk and proximal extremities “never in face” Worsens with application of heat.

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Major Criteria 5. Subcuatenous nodules: occurs in 2% to 10% of patients characterized by small, firm and painless Best felt over extensor surface of bone or tendons Always associated with sever carditis Help to confirm rather than make The diagnosis . Why?? “ > 3 weeks “

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Minor Criteria Arthralgia (It must not be considered a minor manifestation when arthritis is used as a major manifestation). Fever (temperature of at least 38.8°C). In laboratory findings :↑CRP ,↑ESR and ↑ WBC A prolonged PR interval is a weak minor because it’s nonspecific , varying QRS voltage is strong minor “ if measured daily “

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Supportive Evidence A history of sore throat or of scarlet fever is not adequate evidence of recent GABHS. Positive throat cultures or rapid streptococcal antigen tests for GABHS are less reliable because they do not distinguish between recent infection and chronic pharyngeal carriage. Streptococcal antibody tests are the most reliable laboratory evidence of recent streptococcal infection and these include: Antistreptolysin O (ASO) titer Antideoxyribonuclease B titers The Streptozyme test

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Diagnosis * RF is mainly clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA - two major manifestations or - one major and two minor manifestations plus evidence of antecedent streptococcal infection . The presence of Sydenham’s Chorea alone is sufficient for diagnosis

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D.D 1) Juvenile rheumatoid arthritis. 2) Septic arthritis 3)Systemic lupus erythematosus 4)Lyme disease

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Clinical Course Only carditis can cause permanent cardiac damage. Arthritis subsides within a few days to several weeks, even without treatment. Chorea gradually subsides in 6 to 7 months or longer.

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Investigations

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Management * Treatment of acute attack : 1- Eliminating the group A streptococcal pharyngitis * Oral phenoxymethylpenicillin 250mg 6-hourly for 10 days is drug of choice. or * a single dose of benzathine penicillin 1.2 milion U IM . * if patients is penicillin-allergic, administer erythromycin or a cephalosporin 2-suppressing inflammation  Aspirin in case of arthritis . 60mg/kg/day divided in 6 doses till ESR↓  Steroids like Prednisolone in case of carditis. 1-2mg/kg/day  till ESR↓ 3- providing supportive treatment for congestive heart failure. digoxin, diuretics, supplemental oxygen, bed rest, and sodium and fluid restriction 4- if chorea is only finding  phenobarbital “ drug of choice “ 5- Surgery : valvuloplasty , valvotomy , valve replacement

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Management * Prevention : * a single dose of benzathine penicillin 1.2 milion U IM / month . or * Oral phenoxymethlpenicillin 250mg 12-hourly * if patients is penicillin-allergic, administer sulfadiazine ?? or erythromycin . - Patients with rheumatic heart disease require antibiotic prophylaxis before certain surgical and dental procedures to prevent bacterial endocarditis

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Recommended Duration of Prophylaxis for Rheumatic Fever

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Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if she/he is not on prophylactic medications. Good prognosis : for older age group & if no carditis during the initial attack. Bad prognosis : for younger children & those with carditis + valvar lesions.

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Cardiomyopathies

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Introduction Cardiomyopathies are conditions involving the myocardium. Almost always , the problem is Lt. ventricular dysfunction . - Classification : * in the past _ primary or secondary * now _ dilated cardiomyopathy, hypertrophic cardiomyopathy, and restrictive cardiomyopathy - Genetic cardiomyopathies most progress has been made in the X-linked - The prevalence of cardiomyopathy : * newborn : 10 in 100,000 live births * all children : - 36 in 100,000 for dilated cardiomyopathy - 2 in 100,000 for hypertrophic and restrictive cardiomyopathies.

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Dilated Cardiomyopathy * Pathophysiology : - Massive cardiomegaly as a result of the extensive dilatation of the ventricles, most prominently the left. The cause in the vast majority of pediatric cases is idiopathic . Other common causes , active myocarditis is identified in 2-15% of patients. In 20% of cases, the disease is familial - Histological findings consist mainly of areas of fibrosis and compensatory hypertrophy.

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* Etiology : A B C D E F H I A : Alcoholism B : BeriberiC : Cocaine, Chagas disease, Coxsackie BD : DoxorubicinE : Endocrine “ hypothyroidism “ F : Familial muscular or neuromuscular disorder H : Hypophosphatemia, hypokalemia, hypocalcemia . I : Idiopathic, Ischemic, Infection “ influenza, diphtheria, toxopl. “

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* Clinical presentation : Usually the onset is insidious - Initial nonspecific : respiratory system “ cough and dyspnea “, irritability, abdominal pain “ - Then finding of heart failure : skin is cool and pale, the arterial pulse is decreased, the pulse pressure is narrow, and tachycardia is present. JVP is increased, and hepatomegaly and edema are common. The cardiomegaly , and mitral and tricuspid insufficiency gallop rhythm is usually audible.

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* Diagnosis : ECG : atrial enlargement , Lt. or Rt. Ventricular enlargement and non-specific T-wave change - X-ray : cardiomegaly ,Pulmonary congestion and pleural effusions. - Echo : dilatation of the left atrium + Lt. ventricle + poor contractility ± Rt. ventricle . * Prognosis : usually progressively downhill, some remain stable. temporary remission, but relapses are common, If patients tend to become resistant to therapy , the prognosis for survival beyond a year is poor.

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M-mode echo in a child with dilated cardiomyopathy. A, M-mode echo from a 9-year-old normal child. The left ventricular (LV) diastolic dimension (d) is 36 mm, and the LV systolic dimension (s) is 24 mm, with resulting fractional shortening of 33%. B, M-mode echo from an 8-year-old child with dilated cardiomyopathy with a markedly decreased LV contractile function. The LV diastolic dimension (62 mm) and LV systolic dimension (52 mm) are markedly increased, with a marked decrease in the fractional shortening (16%). IVS, interventricular septum; LVPW, LV posterior wall; RV, right ventricle.

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Fig. 11.4 Survival after diagnosis of idiopathic dilated cardiomyopathy (IDCM) in population-based and referral cohorts. Survival after initial diagnosis of IDCM among residents of Olmsted county in Minnesota, USA, (1975–1984) (population-based cohort) compared with survival after Mayo Clinic diagnosis of IDCM in a referral case series (1960–1973) (referred cohort) and survival of a Minnesota white cohort (1980) (expected

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* Complications : ventricular arrhythmias or emboli syncope and sudden death * Management : Monitor for arrhythmia, if present  antiarrhythmia or ICD . - Systemic anticoagulation with warfarin . - Beta-blocker “metoprolol , carvedilol ” ↓ mortality rate . - If medical therapy fails  heart transplantation

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Hypertrophic Cardiomyopathy * Pathophysiology : Massive ventricular hypertrophy with principal involvement of the ventricular septum . - * secondary to : - obstructive congenital heart disease (ex. critical aortic stenosis ) - to an inborn error of metabolism (ex. glycogen storage disease ) * idiopathic “ called IHSS or asymmetric septal hypertrophy “. - Microscopically, patchy areas of abnormally thick and short muscle fibers  ↓ compliance  ↑resistance  ↓ Lt. ventricular filling . - Mitral insufficiency but systolic pumping function remains intact . - mostly, inherited AD “mutations of the cardiac myosin heavy-chain gene are responsible for 30-40% “ - In childhood, greater tendency for right ventricular outflow obstruction to occur.

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* Clinical presentation : May asymptomatic, and about 50% of cases are first evaluated because of either a heart murmur or an affected family member. - Symptoms : weakness, fatigue, dyspnea on effort, palpitations, angina pectoris, dizziness, and syncope ; risk of sudden death - Cardiovascular examination : The pulse is brisk , left ventricular lift and double apical impulse. No systolic ejection clicks “ differentiates from aortic stenosis” . SEM at the left sternal edge and apex “ ↑after exercise, during Valsalva, or standing “.

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* Diagnosis : ECG : left ventricular hypertrophy ± ST segment depression and T-wave inversion, may intraventricular conduction defects - X-ray : cardiomegaly . - Echo : left ventricular hypertrophy , mostly septal Echocardiography has largely replaced cardiac catheterization for initial diagnosis, * Prognosis : is unpredictable “ stable for years - sudden death caused by arrhythmia " .

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Tracing from a 17-year-old girl with hypertrophic obstructive cardiomyopathy with marked septal hypertrophy. Note prominent Q waves with absent R waves in V5 and V6.

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* Management : - There is no standardized therapy . - No competitive sports and strenuous activity “ sudden death “ - Digitalis or aggressive diuresis is contraindicated . - Infusion of isoproterenol or other inotropes should be avoided . - beta-blocker (propranolol) and CCB (verapamil)  ↓ degree of outflow obstruction and slowing the development of hypertrophy “ no ↓ in sudden death “ - CCB not used in infancy . - Treat arrhythmia vigorously . - Surgery “ ventricular septal myotomy” “ valve replacement “

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* Special Situations : - HYPERTROPHIC CARDIOMYOPATHY IN INFANTS OF DIABETIC MOTHERS. Transient form ± left ventricular outflow tract obstruction. The increased left ventricular mass usually regresses within several months. - CORTICOSTEROIDS IN PREMATURE INFANTS. Transient, which usually resolves rapidly with cessation of steroid therapy. - GLYCOGEN STORAGE DISEASE. Cardiomegaly is massive; murmurs are insignificant. Pulmonary atelectasis with 2ndinfection is common. ECG is characteristic and shows prominent P waves, a short PR interval, massive QRS voltage, signs of isolated left or biventricular hypertrophy, and intraventricular conduction delays. The echocardiogram shows severe ventricular hypertrophy. The prognosis is poor.

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Restrictive Cardiomyopathy * Pathophysiology : - Poor ventricular compliance is the major abnormality, inadequate filling “ look like constrictive pericarditis “ - Idiopathic or associated with systemic disease “sarcoidosis, amyloidosis, fibosis, hemochromatosis “ , inborn error of metabolism “mucopolysaccharidosis “, malignancy or radiotherapy

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* Clinical presentation : - dyspnea, edema, ascites, hepatomegaly, increased venous pressure, and pulmonary congestion. nonspecific murmurs * Diagnosis : - ECG : prominent P waves, may normal QRS voltage, ST depression, and T-wave inversion. - X-ray : Cardiomegaly “ mild – moderate “ - Echo : ↑ atrial size , small – moderate vnetricles , abnormal filling

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* Prognosis: - Generally poor * Managemet : “ directed toward relief of heart failure” - CCB  ↑ diastolic compliance. - Antiarrhythmic agents “ as needed “ - Anticoagulation “ if risk of mural thrombosis “ - Cardiac transplantation .

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* Special Situations : - LOFFLER'S HYPEREOSINOPHILIC SYNDROME. Severe multisystem dysfunction (skin, lung, nervous system, liver, heart) Predominant cause of death is restrictive cardiomyopathy with endocardial fibrosis of the mitral and tricuspid valves and of the right and left ventricles. Steroids and cytotoxic agents may be beneficial in the hypereosinophilic syndromes. - MUCOPOLYSACCHARIDOSIS. Most commonly Hurler's syndrome, mucopolysaccharides accumulate in many organs, including the heart and great vessels. The prognosis is poor

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* Special Situations : - ISOLATED NONCOMPACTION OF THE LEFT VENTRICLE. Unknown cause results in elements of both left ventricular restriction and dilatation. May present at any age. The echocardiogram is diagnostic and shows a specific pattern of left ventricular hypertrophy with deep muscular crypts. At risk for ventricular arrhythmias and sudden death, mural thromboses and stroke. Cardiac transplantation has been used successfully in this group of patients.

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A 9-year-old boy treated with the chemotherapy agent doxorubicin in the past for a childhood cancer has a dilated left ventricle and congestive heart failure. He is awaiting a heart transplant. Dilated cardiomyopathy A 16-year-old boy presents to the office with dyspnea and chest pain on exertion and reports occasional presyncopal episodes. An echocardiogram reveals a 4-cm septum . Hypertrophic cardiomyopathy

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Athlete with sudden death Hypertrophic cardiomyopathy

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Carditis

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Myocarditis

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A 7 year old girl present to the office with a 3-week history of progressive dyspnea, malaise, and fatigue . She has recently recovered from a viral infection. Physical examination is remarkable for a holosystolic murmur and hepatomegaly .

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Which of the following findings are suggestive for myocarditis : - weak pulse, distant heart sound, gallop - fever, mitral insufficiency, acidosis - fever, respiratory failure, large heart - fever, arrhythmia, large heart The best test for myocarditis is : - CXR - ECG - Echo - heart biopsy which one is incorrect regarding myocarditis : - prognosis is fair - Echo show : poor vent. Function, normal coronary arteries - ↑ CK-MB - Echo show : pericardial effusion is common - heart failure is most common presentation .

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* Introduction : - Inflammation and necrosis of heart muscle . - Coronary pathology is uniformly absent. - Causes : * 2nd to Infection : - most common – viral “ coxsackie B and adenovirus “ “most common cause “ - some nonviral causes : diphtheria, systemic bacterial infection, rickettsial “ Rocky Mountain spotted fever “ * connective tissue disease * granulomatous disease * Toxins * Idiopathic

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* Clinical onset : - In early infancy, viral myocarditis often occurs :acute + fulminant disease; - In toddlers and young children, it occurs : acute but less fulminant myopericarditis; - in older children and adolescents, it is often asymptomatic and comes to clinical attention primarily as a precursor to idiopathic dilatated cardiomyopathy. - The net final result of chronic viral-associated inflammation is often dilatated cardiomyopathy. - Most common manifestation is heart failure, although arrhythmias and sudden death may be the first detectable signs. - Suggested by fever + large heart + arrhythmia

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* Clinical manifestation :“depends on age and the nature of the infection” - In neonatal, present with : * fever, severe heart failure, respiratory distress, cyanosis, distant heart sounds, weak pulses, tachycardia out of proportion to the fever, mitral insufficiency , gallop, acidosis, and shock. “may present viral hepatitis, aseptic meningitis, rash” - In older, present with : * Congestive heart failure “ graual > acute “ or Sudden onset of ventricular arrhythmias. Dilatated cardiomyopathy is present

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* Diagnosis : - Labe : * ↑ ESR , ↑cardiac enzymes “ CK-MB , LDH “ * +ve serum viral “ -ve not exclude “ / PCR * Heart biopsy “ best but risk “ - X-ray : cardiomegaly, pulmonary edema - ECG : sinus tachycardis, ↓QRS voltage, ST/T-wave abnormalities - Echo : poor ventricular function , pericardial effusion, normal coronary arteries * D.D. : - The predominant diseases mimicking acute myocarditis include : * carnitine deficiency * hereditary mitochondrial defects * idiopathic dilatated cardiomyopathy * pericarditis * anomalous origin of the Lt.coronary arter * endocardial fibroelastosis

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* Treatment : - supportive measures for severe congestive heart failure . - If poor COP with ↓ B.P  Dopamine or epinephrine . - Caution with inotropes  arrhythmia  antiarrhythmia - If cardiac tamponade  Pericardiocentesis - If cardiogenic shock,  extracorporeal membrane oxygenation - Heart transplantation - Acute viral myocarditis  corticosteroids “controversial “ * Prognosis : - symptomatic neonate remains poor “mortality as high as 75% “ - lesser symptoms better prognosis - 50% of untreated older patients die within 2 yr and 80% within 8 yr without heart transplantation

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Endocardial Fibroelastosis

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- Also called fetal endocarditis, endocardial fibrosis, prenatal fibroelastosis, elastic tissue hyperplasia, and endocardial sclerosis. - In primary EFE  dilatated left ventricular chamber - In secondary EFE ventricular cavity is often contracted * Pathology : - white, opaque fibroelastic thickening of the endocardium, usually in the left ventricle , may involve the valves * Clinical presentation: - Variable , congestive heart failure, die within 1 – 6 months

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* Diagnosis : - X-ray : cardiomegaly - ECG : Lt. atrial , Lt. ventricle hypertrophy with strain - Echo : bright-appearing endocardial surface and poorly functioning Lt. ventricle * Treatment : - Goal : treat congestive heart failure and prevention of intercurrent infections - Heart transplantation .

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Pericarditis

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- Pericardial inflammation fluid accumulation in the pericardial space  CT “serous, fibrinous, purulent, or hemorrhagic “ * Clinical manifestations: - Pericordial pain “1st symptom “exaggerated by lying supine and relieved by sittin and leaning forward … referred pain from pleual , diaphragm - The major complaint is a sharp, stabbing sensation over the precordium - Cough, dyspnea, abdominal pain, vomiting, and fever may also occur - If small amount of fluid  friction rub “ diagnostic “ , - If large amount  muffled heart sounds , ↓ B.P, narrow pulses, tachycardia, neck vein distention, and an increased pulsus paradoxus - pulsus paradoxus > 20 mm Hg in a child with pericarditis is an indicator of the presence of cardiac tamponade

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* Etiology : - Viral , most common “ coxsackie B , adenovirus, influenza, echovirus “ - Purulent, bacterial infection .. treatment > open pericardial drainage + antibiotic - RF  part of pancarditis - JRA … common ,,, treatment > steroid or ASA - SLE … should be considered in any patient with pericarditis . - Uremia … prolonged , sever renal failure  chemical irritation - Neoplasm … Hodgkin, leukemia - Constrictive …mostly without preceding illness ,, clinical manifestations occur as a result of impairment of diastolic ventricular filling … treatment > pericardiectomy

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* Diagnosis : - ECG : Low voltage of the QRS, ST segment and T-wave changes with pericarditis are more generalized , and ST segment elevations tend to precede the T-wave changes. Electrical alternans “ demonstrated by a variable QRS “ - X-ray : * if large fluid  water bottle appearance . * if constrictive  small heart, may calcification - Echo : most sensitive, clear, echo-free space between epicardium and pericardium

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Pediatric Cardiology for Practitioners 4th edition (February 15, 2002) by Myung K. Park, R. George, Md, Mph Troxler By Mosby Nelson textbook of pediatrics.-17th ed by Richard E. Behrman, Robert M., Kliegman, Hal B. Jenson. Reference