Acquired Heart Diseases

Infective endocarditis: means infection of

endocardium, it may be caused by bacteria, viruses, fungus, others. It is commonly due to complication of congenital & rheumatic heart diseases but also can occur in patients without cardiac anomaly

Infective Endocarditis
50% of cases due to streptococcus viridans. 30% of cases due to staphylococcus aureus. Which is more common in patients who do not have underlying heart diseases. Group D streptococcus ( S.bovis, S.fecalis ). Which is more common in patients exposed to lower bowel & genitourinary procedures. Pseudomonas aeroginosa & serratia marcescens. Common in IV drug users. In 30% of patients following predisposing factors are recognized. 1- Surgical or dental procedures. 2-Poor dental hygiene. 3-Cardiac catheterization.

Infective Endocarditis
Clinical manifestations
It should be suspected in any patients with rheumatic fever & CHD presented with fever. early symptoms & signs usually are mild, the onset either insidious or sudden.

Symptoms
Fever: is main manifestation, it is either prolong fever without other features except wt loss persisting several months. Or the onset is sudden, high, intermittent fever with excessive sweating. In case of strep.Viridance fever not exceeds 39c, while in case of staph.aureus fever it reaches 39-4oc. Head ache, nausea, vomiting. Chest pain, arthralgia, myalgia. Night sweat, wt loss. CNS manifestation ( stroke, convulsion, head ache )

Infective endocarditis
Signs
1. Fever. 2. Tachycardia. 3. Embolic phenomena ( Roth spots, petechaie, splinter hemorrhage, Osler nodes). 4. Jane way lesions ( painless small erthematous, hemorrhagic lesion affecting the palms & soles ). 5. New or changing murmur. 6. Spleenomegaly. 7. Clubbing. 8. Heart failure. 9. Metastatic infections ( arthritis, meningitis, pericarditis, septic pulmonary embolism, mycotic arterial aneurysm )

Infective endocarditis
Investigations
Blood culture is main step in the diagnosis of IE, other investigations are secondary in importance. 3 to 5 separate blood samples should be obtained for culture, in 90% of cases the etiological agent is recovered from first 2 blood cultures. Other sides for culture may includes cutaneous lesion, urine, synovial fluid, abscesses, CSF. CBP &ESR reveals mild anemia, mild to moderate leukocytosis, high ESR. Microscopical hematurea. Due to immune complex glomerulonephritis. Elevated C reactive protein. Hyper gamaglobulinemia. Decrease serum complement. Positive rheumatoid factor.

Infective endocarditis
Other investigations high blood urea, serum creatinine. Echocardiography: is helpful in diagnosis of IE in patients with negative blood culture & in patients with CHD presented with bacteremia. echo. Identify valve vegetation larger than 2 mm, size, shape, locations & mobility of lesion. Prior antibiotics era IE was fatal disease, mortality remain at 20-25%.

Prognosis & complications

Complications occur in 50-60% & includes

1. 2. 3. 4. Heart failure Myocardial abscess Toxic myocarditis Systemic embolizations A-CNS embolism B-Pulmonary embolism

Infective endocarditis
5-Mycotic aneurysm 6- rapture sinus of vulsalva. 7- obstructive valve disease. 8- acquired VSD. 9 heart block.

Treatment Medical treatment

Antibiotics should be administered immediately. Depending on clinical & results of blood culture, antibiotic therapy may require modification. Antibiotics should be given IV & a total of 4-6 weeks of treatment is recommended. For strep. Viridance, strep. Bovis the antibiotic of choice is penicillin G or penicillin G plus gentamycin. if patient allergic to penicillin, the drug of choice is vancomycin plus gentamycin.

Infective endocarditis
Treatment
For staph. Aures drug of choice is nafcillin or cloxacillin plus gentamycin. If patient allergic or resistant to these drugs the alternative is vancomycin paranteraly plus trimetheprim sulfamethaxazole orally. For staph. Epidermidis the drug of choice is vancomycin plus rifampicin. For hemophilus the drug of choice is ampicillin plus gentamycin. For fungal infection the of choice is amphotericin and flucytocin.

Surgical treatment
Indicated in following cases 1. Sever aortic & mitral valve involvement leading to heart failure. 2. Failure to sterilize the blood despite adequate antibiotics. 3. Recurrent emboli. 4. Failure of medical treatment. 5. Rarely mycotic aneurysm.

Infective endocarditis
Prevention
1. Antibiotics prophylaxis should be administered before & after dental, genitourinary, gastrointestinal, upper respiratory tract procedures. 2. Proper dental care & oral hygiene. 3. Vigorous treatment of sepsis & local infection. recommended antibiotics for prevention of IE as fellow. For dental & URT procedures the drug of choice is amoxicillin orally if patient allergic to penicillin we give oral erythromycin or clindamycin. Gastrointestinal & genitourinary procedures the drug of choice is ampicillin plus gentamycin IV or IM if patient is allergic to penicillin we give vancomycin plus gentamycin. In high risk patient the drug of choice is ampicillin plus gentamycin IV or IM if allergic to penicillin we give vancomycin IV

Acute Myocarditis
Myocarditis refers to inflammation, necrosis of myocardium. It may be caused by many infectious, connective tissues, granulomatous, toxic, or idiopathic processes affecting myocardium. Idiopathic Myocarditis may occur at any age, but is most often seen in first 7 years. Cases have been reported in neonatal period. And peak incidence appears to be between 1-2 years. Most cases are due to infection by group B coxackievirus, adenoviruses, & many other viruses.

Acute Myocarditis
Clinical Manifestations:

s\s depends on the patients age & acute or chronic nature of

infection. A neonate may initially have fever, respiratory distress, cyanosis, distant heart sounds, weak pulses, tachycardia out of proportion of fever, mitral insufficiency caused by dilatation of valve annulus, gallop rhythm, acidosis, sever congestive heart failure, cripitation over lungs, tender hepatomegaly, edema is slight or absent. Older patients commonly presented with gradual onset of congestive heart failure or sudden onset of ventricular arrhythmias.

Acute Myocarditis
CXR shows gross cardiomegaly & pulmonary edema. ECG shows tachycardia, low voltage QRS complex & ST
segment depression& T inversion. (Arrhythmia may be first manifestation &, in presence of fever & a large heart, strongly suggest acute myocarditis).

Echocardiography: demonstrates poor ventricular

function, mitral valve regurgitation & often a pericardial effusion. Other laboratory investigation: ESR & heart enzymes (creatine phosphokinase, lactate dehydrogenase) may be elevated. Serum viral titers may be positive ; PCR can identify specific viral RNA or DNA, rarely myocardial biopsy.

Acute Myocarditis
Treatment: supportive treatment. The patient should be propped up in bed, should supplied with O2. digoxin & diuretics for management of heart failure. dopamine & epinephrine in patients with poor cardiac output & systemic hypotension. Antiarrhythmic drugs like amiodarone to achieve good control of arrhythmia. the use of corticosteroids is controversial. It may effective in reducing myocardial inflammation & improving cardiac function. antibiotics may be indicated in critically ill patients to protect the patient from secondary bacterial infection.

Acute Rheumatic Fever

its caused by group A streptococcus upper respiratory infection. 2/3 of the patients with acute episode of RF have a history of an URTI several weeks before , and peak age & seasonal incidence of acute RF closely parallel those of group A streptococcal infections. The disease is more common in closed community, such as boarding school or military bases, also poverty & overcrowding

Etiology:

increases the incidence of the disease. It is typically develops 2-4 weeks after an acute episode of group A streptococcal pharyngitis. The incidence of both initial attacks & recurrence of acute RF peaks in children aged 5-15 yr, the age of greatest risk for group A pharyngitis.

Acute Rheumatic Fever

Certain serotype of group A streptococcus (M type 1, 3, 5, 6, 18, 24) are more frequently isolated from patients with acute RF

Clinical Features
Depending on Jones criteria the clinical feature divided to major criteria & minor criteria. A-Major Criteria: 1-migratory polyarthritis: it occurs in 75% of patients with acute RF, it involves larger joints, the knee, ankles, wrist, & elbows. While involvement of spine, small joints of hand & feet, or the hips is uncommon. The joint involvement is migratory, severely inflamed joint can become normal within 1-3days without treatment.

ARF

sever arthritis can persist several weeks in untreated patients. If a child is suspected of having acute RF, it is important to not give salicylates & observe for migratory progression. A dramatic response to even small doses of salicylates is another characteristic feature of arthritis, & the absent of response should suggest an alternative diagnosis.

2-Carditis:
it occurs in about 50-60% of all cases of acute RF. Carditis & resultant chronic rheumatic heart disease is the most serious manifestation of acute RF. Rheumatic carditis is characterized by pancarditis (myocardium, pericardium, and endocardium).

ARF
cardiac involvement during acute RF varies in severity from fulminant fatal pancarditis to mild, transient cardiac involvement Endocarditis (involvement of heart valves) is a universal finding in rheumatic carditis. Most cases consist of either isolated mitral valve disease or combined aortic & mitral valve disease. Isolated aortic or tricuspid & pulmonary valve involvement is uncommon. Mitral regurgitation or combined mitral & aortic regurgitation occur in early course of disease while mitral or aortic stenosis usually appears several years or even decades after the acute illness.

ARF
Carditis usually presents as tachycardia & cardiac murmurs. Mitral incompetence murmur is high pitch apical pan systolic radiate to axilla. It may be associated with a high pitch diastolic murmur at left sternal border of (aortic incompetence). Moderate to sever rheumatic carditis can result in cardiomegaly & congestive heart failure with hepatomegaly & peripheral & pulmonary edema.

3-Chorea:
sydenham chorea occurs in about 10-15% of patients & usually presents as an isolated, subtle, neurological behavior disorder. Emotional liability, in coordination, poor school performance, involuntary movement & facial grimacing, exacerbated by stress & disappearing with sleep.

ARF
Clinical maneuvers to elicit features of chorea include.

1-demonstration of milkmaids grip (irregular contractions of muscles of the hands while squeezing the examiners finger). 2-Spooning & pronation of the hands when the patients arms are extended. 3-wormian movements of the tongue when protruded.
4-examination of handwriting to evaluate fine motor movements. Chorea may occur as only manifestation of acute RF. It is rarely if ever leads to permanent neurological squeal.

ARF
4-Erythema marginatum:
it is rare; occur in less than 3%.but characteristic rash of RF. It consists of erythematous, serpiginous, macular lesions with pale centers, which is not pruritic. It occurs mainly on trunk & extremities, but not on face & it can exacerbated by warming of skin.
patients & consist of firm nodules, 1 cm in diameter along the extensor surfaces of tendons near bony prominence.

5-subcutaneous Nodules: it is rare occur in less than 1% of

ARF
Minor Manifestations
Consists of 4 criteria:

1- two clinical minor manifestation;

A- Arthralgia in the absence of polyarthritis as major manifestation. B- Fever.

2- two laboratory minor manifestations:

A-elevated acute phase reactant (ESR, C- reactive protein ) B-prolonged PR interval on ECG.

ARF
Diagnosis:
the diagnosis of acute RF can be established by Jones criteria when patients presented with two major or one major & two minor criteria plus evidence of antecedent group A streptococcal infection, which include:
1-positive throat culture or rapid streptococcal antigen test (streptozyme test). 2- Elevated or increasing streptococcal antibody titer. & these are Antistreptolysin O (ASOT), Anti-Dnase B, Antihyaluronidase antibody.

ARF
Treatment:
all patients with acute RF should be placed on bed rest & monitored closely for evidence of carditis. 1-antibiotic therapy: regardless of throat culture results, the patient should receive 10 days of penicillin or erythromycin, or single IM injection of benzathine penicillin to eradicate group A streptococcus from URT. 2-Anti-inflammatory therapy: A- Patients with typical polyarithritis without carditis should be treated with oral salicylates. The usual dose of aspirin is 100 mg\ kg\ day in 4 divided doses for 3-5 days , followed by 75\kg\ day in 4 divided doses for 4 weeks.

ARF
B- Patients with carditis & cardiomegaly or CHF should receive corticosteroids. The usual dose of prednisone is 2 mg\kg\day in 4 divided doses for 2-3 week followed by a tapering of the dose by 5 mg\day every 2-3 days, at the beginning of the tapering of the prednisone; aspirin should be started at 75 mg\kg\day in 4 divided doses for 6 week. Supportive therapy for patients with moderate to sever carditis includes digoxin, fluid & salt restriction, diuretics, & oxygen. Sydenham chorea is best treated with sedatives like Phenobarbital which is drug of choice, if ineffective then haloperidol or chlorpromazine should be initiated.

ARF
Prevention: prevention of both initial & recurrent attacks of
acute RF depends on controlling group A streptococcal infections of URT. & include.
A-Primary prevention: means prevention of initial attacks depends on identification & eradication of the group A streptococcus that produces episodes of acute pharyngitis. Appropriate antibiotics therapy with oral penicillin or erythromycin 50 mg\kg\day in 4 divided doses, or single IM injection of benzathine penicillin in a dose of 1.200.000 in child more than 25 kg weight or 600.000 in child less than 25 kg, is highly effective in preventing first attack of acute RF.

ARF
B-Secondary prevention: patients who have attack of acute RF are particularly susceptible to recurrence of RF with any subsequent group A streptococcal URTI. These patients should receive continuous antibiotic prophylaxis to prevent recurrences (secondary prevention). The regimen of choice is a single IM of benzathine penicillin 1.2 millions every 4 weeks. Or continuous oral penicillin V in 250 mg twice daily or sulfadiazine 500-1000 mg once daily or erythromycin in 250 mg twice daily in patient who is allergic to both penicillin & sulfonamides. Antibiotics prophylaxis may be discontinued when patients reach their early 20s year or after at least 5 years after the initial attacks of acute RF, while in patients with carditis, should receive prophylaxis for life.

Congestive Heart Failure

Heart failure : is state in which heart cannot produces the
cardiac output required to sustain metabolic needs of body without evoking certain compensatory mechanisms. It considered pediatrics emergency.

Etiology -Fetal 1. Severe anemia ( hemolysis, aplastic anemia, fetal-maternal transfusion) 2. Supraventricular tachycardia. 3. Ventricular tachycardia. 4. Complete heart block.

Congestive Heart Failure

Etiology
-premature neonate Fluid over load PDA. VSD. bronchopulmonary dysphasia ( corpulmonale ). Hypertension. -Full term neonates Cardiomyopathy due to asphyxia. Arteriovenous malformation. Left sided obstructive lesions ( Coarctation of aorta, Hypoplastic LF heart syndrome ). Single ventricle. Truncus Arteriosus. Viral myocarditis.

1. 2. 3. 4. 5. 6.

CHF
Etiology -Infants & toddlers 1. VSD. 2. Arteriovenous malformation. 3. Anomalous LF coronary artery. 4. Metabolic cardiomyopathy. 5. Acute hypertension ( hemolytic uremic syndrome) 6. Supraventricular tachycardia ( SVT ). 7. Viral myocarditis.

CHF
Etiology -Children & adolescent 1. Rh. Fever. 2. Acute hypertension ( glomerulonephritis ). 3. Viral Myocarditis. 4. Thyrotoxicosis. 5. Hemochromatosis & hemosiderosis. 6. Infective endocarditis. 7. Sickle cell anemia. 8. Cystic fibrosis ( corpulmonale). 9. Cardiomyopathy.

CHF
Clinical manifestations
cardinal manifestations of heart failure are tachycardia, tachypnea, tender hepatomegaly, cardiomegaly, anxious & restless. Other prominent manifestation in infants includes. 1. Feeding difficulty. 2. Excessive sweating. 3. Poor wt. gain & FTT. 4. Weak cry. 5. Signs of respiratory distress. 6. Gallop rhythm. 7. Pulmonary congestion with wheeze & cripitation undistinguished from acute bronchiolitis. 8. Edema is late sign in infants & frequently not detected clinically. 9. JVP assessment is difficult due to short neck. 10. Auscultation findings of other cardiac lesions causing heart failure.

CHF
Clinical manifestations -In children the signs & symptoms similar to those in adult & includes 1. Fatigue. 2. Effort intolerance. 3. Anorexia. 4. Abdominal pain. 5. Cough. 6. Elevated JVP. 7. Orthopnea & basal cripitation. 8. Edema either generalize or in dependant part of body. 9. Gallop rhythm.

CHF
Investigations
CXR shows cardiomegaly, increase pulmonary vascularity, while pulmonary edema rarely seen in pediatrics age group. ECG shows LF or RT ventricle ischemic changes or hypertrophy. low voltage QRS with ST-T wave abnormalities indicates myocardial inflammation. also show us rhythm abnormalities. Echocardiography confirms the diagnosis. Arterial blood gas analysis may shows metabolic acidosis in cases of sever CHF. Hyponatremia due to water retention.

CHF
Treatment
1-General supportive measures In critical patients Admission to intensive care unite O2 supply & careful use of IV fluid Keeping patient in semi-upright position If patient not critically ill Strict bed rest is rarely necessary except in sever cases. For infant with HF infant chair may be advisable. Most older patients feels better in semi-upright position.

CHF
2-Digoxin is main drug in treatment of HF. The dose vary with age Premature 0.2 mg\kg. Full term neonate 0.25-0.3 mg\kg. Infant & children 0.25-0.4 mg\kg. Adolescent & adult 0,5-1 mg\kg. Rapid digitalization of infant & children carried out IV. By giving half of total digitalizing dose immediately & the remaining two quarter doses given at 12 hr interval later. The dose of digoxin given IV should be 75% of oral dose. Monitor the patient by ECG to detect any rhythm disturbance like prolong PR interval, ST depression, T inversion, extra systole, junctional tachycardia.

CHF
Following conditions increases digoxin toxicity, hypokalemia, hypomagnesaemia, hypercalcemia, prematurity & myocarditis. Side effects of digoxin include arrhythmia, hypokalemia, while nausea & vomiting are rare in children. The maintenance digoxin therapy started 12 hr after full digitalization. The daily dose is divided in two & given at 12 hr interval. The maintenance dose of digoxin is 1\4 of total digitalizing dose. Patient who are not critically ill, can start digitalization by oral route, then after 24 hr start maintenance digoxin.

CHF
3-Diuretics: these drugs inhibit reabsorption of water & sodium. It is
commonly used with digoxin in patients with HF. A-frusemide ( lasix ) is most commonly used diuretics. It inhibit reabsorption of sodium & chloride in the distal tubules & loop of henle. In acute & sever CHF frusemide given IV or IM in a dose of 1-2 mg\kg\dose. In chronic CHF given orally 1-4mg\kg\day either 6 hr, 8 hr, 12 hr. we should monitor serum K because frusemide causes hypokalemia.

B- spironolactone ( aldactone ) it is inhibitor of aldesterone, also

called K sparing diuretics. It may be used alone or with frusemide in a dose of 2-3 mg\kg\day either 8 hr or 12 hr.

C- chlorothiazide: it is occasionally used in less patients with less

sever chronic CHF in a dose of 20-50 mg\kg\day either 6 hr or 12 hr.

CHF
4-afterload reducing agents & ACE inhibitors This group of drugs reduces ventricular after load by decreasing peripheral vascular resistance & there by improving myocardial performance. Some of these drugs also decreases systemic venous tone, which significantly reduces ventricular preload. These drugs useful in patients with HF secondary to cardiomyopathy & in patient with sever mitral or aortic regurgitation but they not useful in patient with aortic stenosis. These drugs most often used in conjunction with digoxin & diuretics. A- Nitroprusside: given IV in intensive care unite, it causes hypotension

CHF
B-Captopril ( capoten ): given orally, it is ACE inhibitor producing arterial dilatation by blocking the production of angiotensin II, resulting in significant after load reduction also causes venodilatation resulting in preload reduction. In addition it interfere with aldesterone production, so it helps in control salt & water retention. The oral dose is 0.3-6 mg\kg\day given in 2 or 3 divided doses. Side effects includes hypotension & its sequel's (syncope, weakness & dizziness ) , maculopapular rash, neutropenia, renal toxicity & chronic cough. C-Enalapril : is a long acting ACE inhibitor, can be given once or twice daily.

CHF
5-Alpha& B-Adrenergic Agonist: these drugs are usually administered in ICU with continuous determination of BP & HR. It is useful in patients with low cardiac output A-Dopamine: its predominantly B-adrenergic receptor agonist but in high doses it has alpha adrenergic effect at higher doses. B-Dobutamine. C-Isoproterenol: its pure B-adrenergic agonist, most effective in patient with low HR. D-Epinephrine ( Adrenaline ) is mixed alpha & Badrenergic agonist, its usually effective in patient with cardiogenic shock & low BP.

CHF
6-Phosphodiesterase inhibitors: useful to treat patient with low cardiac output who are refractory to stander therapy. A-Milrinone: it has both positive inotropic effects on heart & significant peripheral vasodilatation effect. B-Amrinone.

7-Diet: infant with HF may FTT due to increased metabolic

requirement & decrease caloric intake. Increasing daily caloric intake is an important aspect of management. Chronic treatment with B-Blocker: B-Blocker are used for chronic treatment of patient with HF & should not be used when patient are still in acute phase of HF. A-Metoprolol: its selective B1-adrenergic receptor antagonist. B-Carvedilol: its alpha & B-adrenergic receptor antagonist.