HEART CONDITIONS IN CHILDREN

noa nyaberi
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Inntroduction
Results in blood flow (hemodynamic) changes, which eventually affect the heart, bld vessels and other organs. FETAL CIRCULATION Fetal lungs are non-functional in utero; Fetal O2 depends on placental circulation. Bld from I.V.C returns to the R.A straight thru the L.A via foramen ovale. Then to the L.V from where it is pumped mainly to the vessels of the head & forelimbs. Bld from the S.V.C, mainly deoxygenated, returns to R.V thru the R.A, then to the pulmonary artery, and eventually to the aorta via the ductus arteriosus. Only 12% of fetal blood circulates thru the lungs Deoxygenated bld from the aorta circulates to the umbilical arteries, where it is eventually oxygenated.
I.V.C = Inferior Vena Cava S.V.C = Superior Vena Cava

CONGENITAL HEART DISEASE (CHD)

Genetic and environmental factors Increased risk in the followings:
Maternal factors 1. diabetes mellitus (DM), phenylketonuria (PKU) 2. medication, alcohol or drug use 3. infection (e.g. rubella, cytomegalovirus (CMV)) Infant factors 1. prematurity (e.g. patent ductus arteriosus (PDA)) 2. chromosomal abnormalities (e.g. Down syndrome AVSD) 3. positive family history (2-4% risk)
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 CHDs are described according to the anatomical abNs

Non closure of the foramen ovale Non closure of the ductus arteriosus Communication btn the ventricles Misplacement of the great vessels

Tissue oxygenation may also get compromised after birth. This is the basis for classification as ACYANOTIC or CYANOTIC. NOTE: Even in acyanotic conditions, there may be cyanosis as one clinical sign.
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A. ACYANOTIC CHD
1.Lft-to-rt shunt lesions ASD,VSD,PDA,AVC 2.Obstructive Lesions Coarctation of aorta Aortic stenosis Pulmonary stenosis
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1. LEFT TO RIGHT SHUNT LESIONS

extra bld is displaced thru a communication from the lft to the rt side of the heart, resulting in sed pulmonary bld flow shunt volume dependent upon three factors: size of defect, pressure gradient btn chambers or vessels, peripheral outflow resistance untreated shunts can result in pulmonary vascular disease, right ventricular hypertension (RVH), & revert to R to L shunts
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Atrial Septal Defect (ASD)

An opening btn the Left and Right atria Of three types ostium primum ; ostium secundum ;sinus venosus often asymptomatic in childhood murmur: often grade 2/6-3/6 pulmonic outflow murmur with widely split and fixed S2 ECG: right axis deviation, mild RVH, Rt bundle branch block. CXR: increased pulmonary vasculature natural Hx: 80-100% spontaneous closure rate if remains patent, CHF & pulm. hypertension can dvp later Mx: elective surgical or catheter closure btn 2-5 years of age
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Atrial Septal Defect

Unn Figure 25-1 Atrial septal defect http://www.youtube.com/watch?v=kPfHD2O9mA go to 2:18

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Ventricular Septal Defect (VSD)

A communication btn the rt and lft ventricular compartments most common congenital heart defect (30-50%) small VSD (majority) asymptomatic, normal growth and development murmur: early systolic to holosystolic, best heard at left lower sternal border (LLSB) ECG and CXR are normal most close spontaneously without surgical intervention moderate to large VSD cause Lft to Rt shunt thus increasing pulmonary circuln When pulmonary hypertension occurs, the shunt reverses to a rt-to-lft shunt(Eisenmenger syndrome) delayed growth and development, decreased exercise tolerance, recurrent URTIs or "asthma" episodes, CHF murmur: holosystolic at LLSB with thrill, mid-diastolic rumble at apex ECG: left ventricular hypertrophy (LVH), left atrial hypertrophy (LAH), RVH CXR: increased pulmonary vasculature, cardiomegaly, CHF natural history: secondary pulmonary hypertension, CHF by 2 months of age management: treatment of CHF; surgical closure

Patent Ductus Arteriosus(PDA)

Failure of the ductus arteriosus (btn descending aorta & pulmonary artery) to close within the 1st wks Due to great press in the aorta, blood shunts to the pulmonary arteries thus overloading the LFT atrium. The pulmonary resistance continues to increaese common in premature infants (1/3 of infants < 1750 grams) may be asymptomatic or have apneic or bradycardic spells, poor feeding, accessory muscle use Associated tachycardia, bounding pulses, hyperactive precordium, wide pulse pressure murmur: continuous "machinery" murmur, in left infraclavicular area ECG: may show LVH, RVH CXR: normal to mildly enlarged heart, increased pulmonary vasculature diagnosis by echocardiography (ECHO) natural history: spontaneous closure common in premature infants, less common in term infants management: indomethacin(a pg inhibitor), surgical ligation, or catheter closure high risk of SBE, antibiotic prophylaxis required until 6 months after closure

SBE = subacute bacterial endocarditis

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Patent Ductus Arteriosus

Unn Figure 25-4

Patent ductus arteriosus

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Endocardial Cushion Defect (Atrioventricular (AV) Canal)

Endocardial cushions fails to grow, resulting in abnormalities in the septa i.e. separation btn atria & ventricles. Since the endocardial cushion is where the tricuspid & mitral valves grow, abnormalities in the formation of the atrioventricular valves also present. commonly associated with Down syndrome natural history depends on size of defect and valvular involvement may miimic changes seen in ASD or VSD & may result in enlargement of all cardiac chambers Regurgitation of blood to the atria causing a systolic murmur surgical correction to close septal defects & repair valvular defects before age 1 yr to prevent pulmonary complications
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Atrioventricular Canal Defect

Unn Figure 25-3

Atrioventricular canal defect

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2. OBSTRUCTIVE LESIONS
present with pallor, decreased urine output, cool extremities and poor pulses

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Coarctation of the Aorta

narrowing of aorta; commonly at the level of the ductus arteriosus causes higher pressures above the site or low press below it few have high BP in infancy (160-200 mmHg systolic) but this decreases as collaterals develop if severe, presents with shock in the neonatal period when the ductus closes often asymptomatic with upper extremity systolic pressures of 140145 mm Hg weak pulses, sed bld pressure in lower extremities, radial-femoral delay if associated with other lesions (e.g. PDA, VSD), can cause CHF ECG: RVH early in infancy, LVH later in childhood murmur: absent or systolic with late peak at apex, left axilla, left back management: balloon arterioplasty or surgical correction complications: essential hypertension
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Coarctation of the Aorta*

Unn Figure 25-5

Coarctation of the aorta

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Aortic Stenosis

Narrowing of the aortic valve or aortic outflow tract

valvular (75%), subvalvular (20%), supravalvular and idiopathic hypertrophic subaortic stenosis (IHSS) (5%)

Results in LVH & increaesed left atrial pressure often asymptomatic but may be associated with CHF, exertional chest pain, syncope or sudden death murmur: systolic ejection murmur (SEM) at upper right sternal border (URSB) with aortic ejection click at the apex management: surgical or balloon valvuloplasty, repeated interventions and valve replacement may be necessary SBE prophylaxis and exercise restriction required
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Aortic Stenosis

Unn Figure 25-6

Aortic stenosis

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Pulmonary Stenosis

narrowing of pulmonary outflow tract

Thus rt ventricle may hypertrophy & eventually cause Rt atrium hypertrophy.

usually part of other congenital heart lesions (e.g. Tetralogy of Fallot) or in association with other syndromes (e.g. congenital rubella, Noonan syndrome) critical pulmonic stenosis: inadequate pulmonary blood flow, dependent on ductus for oxygenation, progressive hypoxia and cyanosis presentation varies from asymptomatic to CHF murmur: wide split S2 maximal on expiration, SEM at ULSB, pulmonary ejection click ECG: RVH CXR: dilated post-stenotic pulmonary artery management: balloon valvuloplasty, surgical valvotomy
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Pulmonic Stenosis*

Unn Figure 25-7

Pulmonic stenosis

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B. CYANOTIC CONGENITAL HEART DISEASE systemic venous return re-enters systemic circulation directly most prominent feature is cyanosis (O2 sat < 75%) differentiate between cardiac and other causes of cyanosis with hyperoxic test (if improvement of PaO2, less likely cardiac cause) survival depends on mixing via shunts (e.g. ASD, VSD, PDA)
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1. LESIONS ASSOCIATED WITH DECREASED PULMONARY BLOOD FLOW

Tetralogy of Fallot Common cyanotic heart defect beyond infancy consists of four defects
ventricular septal defect (VSD) right ventrical (RV) outflow tract obstruction (RVOTO) overriding aorta right venticular hypertrophy (RVH)

infants may initially have a left to right shunt and therefore are not cyanotic but the RVOTO is progressive, resulting in increasing right to left shunting with hypoxemia and cyanosis.
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Tetralogy of Fallot

Unn Figure 25-8

Tetralogy of Fallot

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hypoxic tet spells

primary pathophysiology is hypoxia, leading to increased pulmonary vascular resistance (PVR) and decreased systemic resistance, occurring in exertional states (e.g. crying, exercise) paroxysm of rapid and deep breathing, irritability and crying hyperpnea, increasing cyanosis often leading to deep sleep and decreased intensity of murmur peak incidence at 2-4 months of age if severe may lead to seizures, loss of consciousness (LOC), death (rare) management: O2, knee-chest position, fluid bolus, morphine sulfate, propanolol

murmur: single loud S2 due to severe pulmonic stenosis ECG: right axis deviation, RVH CXR: boot shaped heart, decreased pulmonary vasculature, right aortic arch management: surgical repair including closure of VSD and widening of RVOTO
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Knee-chest Position
Nurse puts infant in knee-chest position. Whaley & Wong

Child with a cyanotic heart defect squats (assumes a knee-chest position) to relieve cyanotic spells. Some times called tet spells. Ball & Bindler

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Clubbing of Fingers

Clubbing of Fingers
Whaley & Wong Bowden text
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2. LESIONS ASSOCIATED WITH INCREASED PULMONARY BLOOD FLOW

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Transposition of the Great Arteries (TGA)

Pulmonary artery becomes the outflow tract of Lft ventrilce & aorta for the rt ventricle. Bld from the L.V repeatedly circulates into pulmonary circulation. Oxygenated bld does not reach the systemic circulation. newborn presents with progressive cyanosis unresponsive to O2 therapy as the ductus arteriosus closes and mixing between the two circulations diminishes; severe hypoxemia, acidosis, and death can occur rapidly if VSD present, cyanosis is not prominent, infant presents with CHF after a few weeks of life murmur: none if no VSD ECG: RAD, RVH CXR: egg-shaped heart with narrow mediastinum ("egg on a string") management
prostaglandin E1 (PGE1) infusion to keep ductus open until septotomy or surgery balloon atrial septostomy with catheter surgical correction: arterial switch procedure

infants without VSD must be repaired within 2 weeks to avoid weak LV muscle

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Transposition Of The Great Arteries, Or Transposition Of The Great Vessels*

Unn Figure 25-11

Transposition of great vessels

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Hypoplastic Left Heart Syndrome

a spectrum of hypoplasia of left ventricle, atretic mitral and/or aortic valves, small ascending aorta, coarctation of the aorta with resultant systemic hypoperfusion most common cause of death from congenital heart disease in first month of life presents with circulatory shock and metabolic acidosis on closure of the ductus management
intubate and correct metabolic acidosis IV infusion of PGE1 to keep ductus open surgical correction (overall survival 50% to late childhood) transplantation palliative
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Hypoplastic Left-Sided Heart Syndrome

Unn Figure 25-14

Hypoplastic left-sided heart syndrome

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Nursing management of CHD

Goal of nursing care in uncorrected cardiac abN centre on promoting cardiac output, diminishing respiratory distress, maintaining fluid balance, and promoting growth & development.
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ACQUIRED CARDIOVASCULAR DISORDERS

Infectious and Inflammatory Cardiac Disorders Occur after birth & may result from congenital heart defects or other environmental factors. Include CHF, 10 Hypertension, Rh Heart Disease, & Kawasaki Disease

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CONGESTIVE HEART FAILURE (CHF)

Etiology
congenital heart defects (CHD) ; cardiomyopathy; arrhythmias; pulmonale; myocarditis arteriovenous malformations (AVMs); acute hypertension; anemia; cor

Symptoms infant: feeding difficulties, easy fatiguability, exertional dyspnea, diaphoresis when sleeping or eating, respiratory distress, vomiting, lethargy, cyanosis child: decreased exercise tolerance, fatigue, decreased appetite, failure to thrive, respiratory distress, syncope, frequent URTIs or "asthma" episodes orthopnea, paroxysmal nocturnal dyspnea, edema are all uncommon in children
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 Physical Findings 4 key features: tachycardia, tachypnea, cardiomegaly, hepatomegaly (2 tachys, 2 megalys) failure to thrive (FTT) respiratory distress, gallop rhythm, wheezing, crackles, cyanosis, clubbing (with CHD) alterations in peripheral pulses, four limb blood pressures dysmorphic features associated with congenital syndromes Management correction of underlying cause general: sitting up, O2, sodium and water restriction, increased caloric intake pharmacologic: diuretics, inotropic agents, afterload reduction
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Acute rheumatic fever

Rheumatic fever is the most common cause of acquired heart disease in children in developing countries. A complication of pharyngitis (a throat infection) caused by Streptococcus bacteria; Group A beta haemolytic Streptococcus

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clinical features of acute rheumatic fever

Develops 23 weeks after a Streptococcal pharyngitis. Classical features Fever A flitting polyarthritisusually large jts Carditis Eythema marginatum --- A short-lived erythematous (pink) rash which forms irregular patterns on the trunk. Subcutaneous nodulesover the elbows,knuckles, wrists, knees and spine Chorea
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signs of carditis
Carditis is an inflammation of the heart. The heart muscle, valves and pericardium are involved. A heart murmur Tachycardia An enlarged heart A rubbing noise (friction rub) heard on auscultation
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clinical diagnosis of acute rheumatic fever

A Streptococcal infection plus 2 major or 1 major and 2 minor criteria. The major criteria are:
Flitting polyarthritis Carditis Erythema marginatum Nodules Chorea Fever Arthralgia Blood tests indicating inflammation An abnormal ECG esp a prolonged PR interval
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The minor criteria are:

Treatment
Bed rest Amoxycillin 10 mg/kg QID P.O X 10/7 Or a single dose of benzathine penicillin 1.2 MU IM Aspirin for symptomatic relief of fever and joint pain. Observe closely for signs of heart failure.
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Preventing repeated attacks of acute rheumatic fever

Benzathine penicillin 1.2 million units intramuscularly every 4 weeks (600 000 units if the child weighs less than 30 kg). This must be continued until adulthood when it should be reviewed

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INFECTIVE ENDOCARDITIS
Serial +ve cultures are needed for definitive diagnosis, but rely on clinical suspicion and other investigations. 5% of cases are culture ve ---- a risk factor for poor prognosis Osler's nodes, Janeway's lesions, splinter hemorrhages are late findings in children antibiotic prophylaxis for prevention is necessary for all patients with
congenital heart disease (except for isolated secundum ASD) rheumatic valve lesions prosthetic heart valves surgical shunts previous endocarditis pacemaker leads
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DYSRHYTHMIAS Can be transient or permanent, congenital (structurally normal or abnormal) or acquired (toxin, infection)
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 Sinus Arrhythmia phasic variations with respiration in almost all normal children Premature Atrial Contractions (PACs) may be normal variant or can be caused by electrolyte disturbances, hyperthyroidism, cardiac surgery, digitalis toxicity Premature Ventricular Contractions (PVCs) common in adolescents benign if single, uniform, disappear with exercise, no associated structural lesions if not benign, may degenerate into more severe dysrhythmias
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 Supraventricular Tachycardia (SVT) most frequent sustained dysrhythmia in children not life-threatening but can lead to symptoms caused by re-entry via accessory connection (atrioventricular (AV) node most common site) characterized by a rate of greater than 210 bpm treatment: vagal maneuver, adenosine, digoxin (except in Wolfe-Parkinson-White (WPW)) or B-blockers Complete Heart Block congenital heart block can be caused by maternal Rho antibody formed in mothers with CVD clinical symptoms related to level of block the lower the block, the greater the symptoms of inadequate cardiac output (CO) symptomatic patients need a pacemaker
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