Care of Child with Cardiovascular Disorders

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Care of Child with Cardiovascular Disorders

• Dr. Manal Kloub

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Outline

• CHD
• CHF
• Hypoxemia
• Acyanotic disorders
• Increased pulmonary flow
• PDA
• ASD
• VSD

• Cyanotic disorders
• Obstructive
• AS
• PS
• COA
• Decreased pulmonary flow
• TOF
• Mixed defects
• TGV or TGA
• Hypoplastic heart

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Heart Anatomy and Physiology
4
Cardiac Conduction
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First Breath

• Pulmonary alveoli open up
• Pressure in pulmonary tissues decreases
• Blood from the right heart rushes to fill the
  alveolar capillaries
• Pressure in right side of heart decreases
• Pressure in left side of heart increases
• Pressure increases in aorta

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Congenital Heart Diseases ?

• Definition
• It includes primarily anatomic abnormalities
  present at birth that results in abnormal cardiac
  functions
• The newborns heart begins to beat at 28 days of
  gestation
• The heart is completely developed on the 9th week
  of intrauterine life

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Incidence CHD

• 4 10/100 live births
• Major cause of death during 1st year of life
  after prematurity
• Affect both sexes differently
• It is more likely to be with other congenital
  anomalies e.g. T.E fistula, Renal agenecies and
  diaphragmatic hernia.

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Etiology CHD

• Factors associated with increased incidence
• Prenatal factors
• Maternal rubella
• Radiation
• Alcoholism
• age gt40 yrs
• Insulin dependent diabetes
• fetal intra uterine cardiac viral disease

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Etiology CHD

• Genetic factors although the influence is
  multifactorial there is high risk of CHD in
  children who have
• a sibling with a heart defect
• a parent with CHD
• chromosomal aberration e.g. Downs syndrome
• Born with other congenital anomalies

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Altered Hemodynamics

1. Blood flows from high pressure to area of lower
   pressure and with lower resistance, in response
   to the pumping action of the heart
2. The higher the pressure gradient the greater the
   rate of flow
3. The higher the resistance the less the rate of
   flow

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1. Normally the pressure in the Rt side is lower
   than the Lt side of the heart
2. The resistance in the pulmonary circulation and
   vessels entering or leaving these chambers have
   corresponding pressure
3. So if there is septal defect, blood will flow
   from Lt to Rt known as left to right shunt, and
   no desaturated blood flows directly into the Lt
   side of the heart so it is acyanotic defect

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Cyanotic defects are due to

• Change in the pressure causing blood to flow Rt -
  to - Lt Shunt.
• Due to increased pulmonary flow through the
  pulmonary vascular resistance
• Or obstruction to the blood flow through the
  pulmonary valve and aorta
• Or due to mixing of the blood between pulmonary
  and systemic circulation e.g. truncus arteriosus
  (the pulmonary artery and the aorta are one
  single vessel that overrides both ventricles

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Classification and clinical consequences of
congenital Heart Diseases

• Depending on the severity of the cardiac defect
  and the altered hemodynamics TWO principal
  clinical consequences can occur
• 1st Consequence is Congestive Heart Failure
• 2nd Consequence is Hypoxemia

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Congestive Heart Failure

• It is the inability of the heart to pump
  adequate blood to the systemic circulation to
  meet the metabolic demands of the body.
• It is a symptom caused by cardiac defect not a
  disease in itself, it is due to increased work
  load on normal myocardium

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Congestive Heart Failure

• Major manifestation of cardiac disease.
• Under 1 year of age due to congenital anomaly.
• Over 1 year with no congenital anomaly may be due
  to acquired heart disease.
• In children failure of one chamber causes change
  in the opposite chamber

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Pathophysiology of CHF

• Two categories
• Rt sided failure the Rt ventricle is unable to
  pump blood to pulmonary artery resulting in
  increase in the pressure in the Rt atrium and
  systemic venous circulations leading to liver and
  spleen enlargement and occasionally edema.
• Lt sided failure, the left ventricle is unable to
  pump blood into the systemic circulation leading
  to increased pressure in the Lt atrium and
  pulmonary veins. The lungs become congested
  leading to increased pulmonary pressure and
  pulmonary edema.

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• Congestive heart failure leads to
• cardiac muscles damage
• decrease the cardiac output
• decrease the flow of blood to the kidneys
• increase the reabsorption of Na and water and
  increase blood volume
• increase systemic congestion
• Distention in neck veins and peripheral veins
• Edema and hepatomegaly
• Forehead sweating due to sympathetic response

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Clinical S S of CHF

• Pulmonary Congestion
• Tachypnea
• Dyspnea
• Cyanosis
• Wheezing
• Grunting
• Retractions with flaring nares
• Cough and hoarseness of voice

• Cardiac Congestion
• Tachycardia
• Cardiomegaly
• Pale cool extremities
• Weak peripheral pulses
• Low blood pressure
• Gallop Rhythm heart beats

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Clinical S S of CHF

• Exercise intolerance
• Orthopnea
• Sweating
• Decreased urine output
• Weakness
• Fatigue
• Restlessness
• Anorexia

• Systemic Venous Congestion
• Weight gain (due to edema)
• Hepatomegaly
• Peripheral edema especially periorbital area
• Ascites
• Neck vein distension

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? Therapeutic management

• Goals
• Improve cardiac function
• Remove accumulated fluid and Na
• Decrease cardiac demands
• Improve tissue oxygenation and decrease O2
  consumption

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Interventions

• Fluid restriction
• Diuretics Lasix (potassium wasting) or
  Aldactone (potassium sparing)
• Bed rest
• Oxygen therapy
• Small frequent feedings soft nipple with
  supplemental NG for adequate calorie intake
• Pulse oximeter
• Sedatives if needed

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Improving Myocardial efficiency

• By giving Digitalis glycosides (Lanoxin
  /Digoxin)
• Increase cardiac output
• Decrease heart size
• Decrease venous pressure
• Decrease edema
• Regulate heart rate
• Digoxin increases the force of the myocardial
  contraction.

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Digoxin Therapy

• Take an apical pulse with a stethoscope for 1
  full minute before every dose of digoxin. If
  bradycardia is detected.
• lt 100 beats / min for infant and toddler
• lt 80 beats in the older child
• lt 60 beats in the adolescent
• Nursing alert
• Call physician before administering the drug

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Signs of Digoxin Toxicity

• Extreme Bradycardia
• Arrhythmia
• Nausea, vomiting, anorexia
• Dizziness, headache
• Weakness and fatigue

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Supplemental Feeding
Infants with cardiac conditions often
require supplemental feeding to provide
sufficient nutrients for growth.
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• Angiotensin-converting enzyme (Captopril/capotin)
  
• Inhibit the normal function of rennin angiotensin
  in the kidney and vasodilatation occur, decrease
  pulmonary and systemic vascular resistance which
  decreases B/P and cardiac after load
• Remove accumulated fluid and Na
• Diuretics. frusamide (Lasix). Thiazide and
  Spironolactone.
• Possible restriction of fluids and decrease Na
• Observe fluid intake and out put and Signs of
  dehydration
• Potassium supplement, because Diuretics increase
  potassium loss

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• Decrease cardiac demand
• Decrease physical activities, by bed rest,
  observe body temp.
• Treat any infections
• Improve breathing semi sitting position and
  sedate irritable children
• Increase tissue oxygenation and decrease O2
  consumption by applying the mentioned measures
  and giving humidified cool O2

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2nd Consequence Hypoxemia

• Referred to arterial O2 tension (or pressure
  PaCo2), that is less than normal can be
  identified by ? SaO2 or ?PaO2
• Hypoxia
• Reduction in tissue oxygenation that results from
  ?oxygen saturation and PaO2 which results in
  impaired cellular process
• Cyanosis
• Blue discoloration of mucus membrane, skin nail
  beds due to reduced O2 saturation, results from
  the presence of deoxygenated hemoglobin in a
  concentration of 5g/dl of blood or more.

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• Cyanosis occurs when O2 saturation is 75 - 85
• May not reflect arterial hypoxemia because both
  O2 saturation and amount of circulating
  hemoglobin are involved
• In severe anemia no cyanosis despite severe
  hypoxemia, because hemoglobin level may be too
  low to produce blue color
• On the contrary, in polycythemia there is
  cyanosis and normal PaO2
• In Heart defects Hypoxemia and Cyanosis result
  from desaturated venous blood entering the
  systemic circulation

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Clinical manifestations of Hypoxemia

• Squatting (rarely seen)
• Polycythemia (increased number of RBC)
• Clubbing of the finger
• Hypercyanotic spells

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Hypercyanotic spells

• Hypercyanotic spells
• Cyanosis
• Hyperapneia (increased depth of breathing)
• ?Rt -to-Lt Shunt.
• Rare lt 2 months of age
• More common in gt1year of age increase in the
  morning and during feeding, crying and defecation
• Possible consequences
• CVA
• Brain abscess
• ?Development

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Congenital Heart Diseases

• TYPES OF
  DEFECTS and
• 
  Classification
• 1st Acyanotic ?
  2nd Cyanotic ?
• ?Pulmonary Obstruction to
  ?Pulmonary Mixed
• blood flow blood flow
  flow flow
• ? ?
  ?
  ?
• ASD
  Tetralogy TPOGA
• VSD Coarctation of
  of Fallots Truncus
• the aorta
  
  arteriosis.
• PDA
  Tricuspid Hypoplastic
• Pulmonic stenosis
  Artesia Lt Heart
• 
  
  syndrome

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Right to Left Shunts

• Occurs when pressure in the right side of the
  heart is greater than the left side of the heart.
  
• Resistance of the lungs in abnormally high
• Pulmonary artery is restricted
• Deoxygenated blood from the right side shunts to
  the left side

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Right to Left Shunt

• Hole in septum obstructive lesion
• Deoxygenated blood from the right side of the
  heart shunts to the left side of the heart and
  out into the body.

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Clinical Manifestations

• Hypoxemia the result of decreased tissue
  oxygenation.
• Polycythemia increased red blood cell
  production due to the bodys attempt to
  compensate for the hypoxemia.
• Increase viscosity of the blood heart has to
  pump harder.

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Potential Complications

• Thrombus formation due to sluggish circulation.
• Brain abscess or stroke due to the un-oxygenated
  blood bypassing the filtering system of the lungs.

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Left to Right Shunt

• Pressures on the left side of the heart are
  normally higher than the pressures in the right
  side of the heart. If there is an abnormal
  opening in the septum between the right and left
  sides, blood flows from left to the right.

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Clinical Manifestations

• The infant is not cyanotic.
• Tachycardia due to pushing increased blood
  volume.
• Cardiomegaly due to increased workload of the
  heart.

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Clinical Manifestations

• Dyspnea and pulmonary edema due to the lungs
  receiving blood under high pressure from the
  right ventricle.
• Increased number of respiratory infections due to
  blood pooling in the the lungs promoting
  bacterial growth.

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? Acyanotic Defects

• Lt to Rt shunting through an abnormal opening
  or obstructive lesions leading to decrease blood
  flow to various parts of the body.
• The most common clinical manifestation is heart
  failure. Some of them may be asymptomatic (ASD,
  VSD, PDA)

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Patent Ductus Arteriosus
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Patent Ductus Arteriosus (PDA)

• The fetal ductus artery connecting the aorta and
  the pulmonary artery fails to close within few
  weeks after birth, so the blood flows from the
  high pressure of the aorta to the lower pressure
  of the pulmonary artery (Lt to Rt shunt
• Ductus normally closes within hours of birth
• Connection between the pulmonary artery (low
  pressure) and aorta (high pressure)

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Patent Ductus Arteriosus

• Incidence
• Incidence 10
• It is most common cardiac anomaly
• One of the most common benign defects
• The ratio is 2 ? 1?
• High risk for pulmonary hypertension

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Clinical Manifestations PDA

• Might be asymptomatic
• Show signs of CHF
• Machinery - like murmur
• Widened pulse pressure
• Bounding pulses resulting from runoff of blood
  from aorta to pulmonary artery
• Risk of bacterial endocarditis and pulmonary
  vascular obstructive disease in later life from
  chronic excessive pulmonary blood flow

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Diagnosis tests

• Diagnosis by
• Chest x-ray enlarged heart and dilated
  pulmonary artery
• Echo-cardiogram show the opening between
  pulmonary artery and aorta

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Treatment PDA

• Medical management by using Endomethacin
  (Prostaglandin inhibitor) which constricts the
  muscle in the wall of the PDA and promotes
  closure
• Nonsurgical treatment where coil is placed in the
  open duct and acts like a plug used during
  catheterization procedure
• Surgical Management via small incision made
  between ribs on left hand side and PDA is ligated
  or tied and cut off
• Prognosis is good with less than 1 mortality

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Atrial Septal Defect (ASD)
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Atrial Septal Defect (ASD)

• An abnormal opening between atria with a Lt-to-Rt
  Shunt. Blood in left atrium flows into right
  atrium.
• 10 of defects
• Reduced blood volume in systemic circulation

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Clinical Manifestations (ASD)

• May be asymptomatic
• May develop CHF
• Characteristic murmur
• Pt is at risk for atrial dysrythmia ( may be due
  to atrial enlargement and stretching of
  conduction fibers)
• At risk for pulmonary vascular obstructive
  disease, and emboli formation later in life due
  to chronic increased pulmonary blood flow
• If left untreated may lead to pulmonary
  hypertension, congestive heart failure or stroke
  as an adult.

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Diagnosis (ASD)

• Heart murmur may be heard in the pulmonary valve
  area because the heart is forcing an unusually
  large amount of blood through a normal sized
  valve.
• Echocardiogram is the primary method used to
  diagnose the defect it can show the hole and
  its size and any enlargement of the right atrium
  and ventricle in response to the extra work they
  are doing.

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Treatment (ASD)

• Nonsurgical treatment might use techniques and
  devices during cardiac catheterization to close
  the opening
• Surgical treatment using surgical dacron patch
  closure of moderate to large defects.
• After closure in childhood the heart size will
  return to normal over a period of four to six
  months.
• No restrictions to physical activity post closure
• Prognosis very low operative mortality, lt1

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Ventricular Septal Defect (VSD)
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Ventricular Septal Defect (VSD)

• It is an abnormal opening between right and left
  ventricles, may vary in size small pinhole to
  the absence of the septum, resulting in common
  ventricle. Frequently associated with other
  defects
• Lt to Rt Shunt
• 30 of defects
• May be classified according to defect location

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Ventricular Septal Defect (VSD)

• Small holes generally are asymptomatic
• Medium to moderate holes will cause problems when
  the pressure in the right side of the heart
  decreases and blood will start to flow to the
  path of least resistance (from the left ventricle
  through the VSD to the right ventricle and into
  the lungs)
• May be associated with other defects such as (PS,
  TGV,PDA)
• Many VSD closes spontaneously 1st year of life

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Clinical Manifestations VSD

• May develop CHF
• Characteristic murmur
• Right ventricular hypertrophy
• Deficient systemic blood flow
• High risk of bacterial endocarditis and pulmonary
  vascular obstruction disease
• May develop Eisenmenger syndrome
• refers to the combination of systemic-to-pulmonary
  communication, pulmonary vascular disease and
  cyanosis

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Diagnostic tests VSD

• Diagnosis heart murmur clinical pearl a
  louder murmur may indicate a smaller hole due to
  the force that is needed for the blood to get
  through the hole.
• Electrocardiogram to see if there is a strain
  on the heart
• Chest x-ray size of heart
• Echocardiogram shows size of the hole and size
  of heart chambers

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Management VSD

• Palliative placing band on pulmonary artery to
  decrease pulmonary blood flow
• Complete repair small defects are reparied with
  purse-string. Large defect require knitted Dacron
  patch sewn over the opening.
• Post operative complication include conduction
  system disturbances
• Nonsurgical treatment closure during cardiac
  catheterization are still under study
• Prognosis risk depend on location of the defect,
  number of defects and other cardiac factors.
  Mortality rate ranges from 5 to 20

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Surgical Repair

• Over a period of years the vessels in the lungs
  will develop thicker walls the pressure in the
  lungs will increase and pulmonary vascular
  disease
• If pressure in the lungs becomes too high the
  un-oxygenated blood will cross over to the left
  side of the heart and un-oxygenated blood will
  enter the circulatory system.
• If the large VSD is repaired these changes will
  not occur.

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Medical Treatment VSD

• CHF diuretics of help get rid of extra fluid in
  the lungs
• Digoxin if additional force needed to squeeze the
  heart
• FTT or failure to grow may need higher calorie
  concentration
• Will need prophylactic antibiotics before dental
  procedures if defect is not repaired

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? Major Cyanotic Defects

• Cyanotic defects result from
• Obstructive defects (PS, AS, COA)
• And Mixing of desaturated blue venus blood with
  fully saturated red arterial blood within the
  chambers of the heart (TOF, TGV / TGA,
  Hypoplastic heart)

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Pulmonary stenosis
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Pulmonary Stenosis (PS)

• It is narrowing at the entrance of the pulmonary
  artery (pulmonary valve) leads to narrowing and
  obstruction between the right ventricle and the
  pulmonary artery.
• Resistance to blood flow cause Hypertrophy of
  right ventricle
• Thickened tissue become less pliable and
  increases the obstruction
• Right ventricle must work harder to eject blood
  into the pulmonary artery.
• 7 of defect

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Clinical Manifestations (PS)

• Some might be asymptomatic
• Some might have mild cyanosis or CHF
• Newborns with severe narrowing will be cyanotic
• Characteristic murmur
• Cardiomegally is evident in chest X-ray
• ? Risk of Bacterial Endocarditis with progressive
  narrowing lead to increase symptoms

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Diagnostics PS

• Diagnosis heart murmur is heard clicking sound
  when the thickened valve snaps to an open
  position.
• Chest x-ray, enlarged heart
• Electrocardiogram would be normal
• Echocardiogram most important non-invasive test
  to detect and evaluate pulmonary stenosis
• Cardiac Catheterization to measure pressures
  and measure the stenosis

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Management PS

• Surgical correction
• Infants Transventricular valvotomy Bock
  procedure
• Children Valvotomy with cardiopulmonary bypass
• Nonsurgical treatment
• Cardiac catheterization to dilate the valve and
  open up the obstruction by using a balloon
  angioplasty
• Prognosis less than 2 mortality

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Aortic stenosis
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Aortic Stenosis (AS)

• Narrowing of aortic valve causing resistance to
  blood flow in the Lt ventricle, decrease cardiac
  output, Lt ventricular hypertrophy and pulmonary
  vascular congestion.
• Causes obstruction to blood flow between the left
  ventricle and aorta.
• Most common form is obstruction of the valve
  itself

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Aortic Stenosis

• 6 of defects, 30 incidence of sudden death
• Aortic valve has two rather than three leaflets.
  Leaflets are thickened or fused.
• When the aortic valve does not open properly the
  left ventricle must work harder to eject blood
  into the aorta.
• Left ventricular muscle becomes hypertrophied.

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Clinical Manifestations (AS)

• Infants with severe defects
• Signs of decreased cardiac output and faint
  pulses
• Hypotension and tachycardia
• Poor feeding
• Exercise intolerance
• Chest pain and dizziness
• Characteristic murmur
• Risk for endocarditis, ventricular dysfunction,
  and coronary insufficiency

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Diagnostics AS

• Heart murmur of turbulent like noise caused by
  ejection of blood through the obstructed valve.
• Electrocardiogram is usually normal
• Echocardiogram will show the obstruction and rule
  out other heart anomalies
• Exercise stress test provides information on
  impact of the stenosis on heart function

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Management AS

• Surgical correction
• - valvotomy if the closed procedure does not
  work often done when patient is older when
  severe calcium deposits further obstruct the
  valve.
• Nonsurgical correction
• - Dilating narrowed valve with balloon
  angioplasty in the cath lab
• Prognosis
• Newborn critical conditions mortality 10 - 20
• Older children elective valvotomy has lower risk
• Complication Recurrent valve obstruction is a
  complication and if valve replacement is done too
  early the child may outgrow the valve.
  Prophylactic antibiotic needed

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Coarctation of the Aorta (COA)
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Coarctation of Aorta (COA)

• There is localized narrowing near the insertion
  of ductus arteriosus resulting in
• Increased pressure in proximal structures to the
  defect (Head and upper extremities)
• Decreased pressure distal to obstruction
• (body and lower extremities)
• Congenital narrowing of the descending aorta
• 7 of defects
• 80 have aortic-valve anomalies
• Difference in BP in arms and legs (severe
  obstruction)

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Clinical manifestations (COA)

• High B/P and bounding pulses in arms
• Weak or absent femoral pulses
• Cool lower extremities with low B/P
• Signs of CHF in infants
• Older children may experience dizziness,
  headaches, fainting and epistaxis due to
  hypertension
• Risk of hypertension, ruptured aorta, aortic
  aneurism or stroke

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Diagnostics COA

• In 50 the narrowing is not severe enough to
  cause symptoms in the first days of life.
• When the PDA closes a higher resistance develops
  and heart failure can develop.
• Pulses in the groin and leg will be diminished
• Echocardiogram will show the defect in the aorta

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Management COA

• Surgical correction Resection of narrowed
  portion and end - to - end anastomosis or graft
  replacement via thoracotomy incision
• Nonsurgical treatment balloon angioplasty may be
  successful in some cases but risk of aneurysm
  formation is present
• Prognosis less than 5 mortality rate in
  isolated coarctation
• - high risk in infants with other complex
  cardiac defects

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• Medical Treatment
• Prostaglandin may given to keep the PDA open to
  reduce the pressure changes
• Antibiotic prophylactic need due to possible
  aortic valve abnormalities.
• Complications
• Surgical complications kidney damage due to
  clamping off of blood flow during surgery
• High blood pressure post surgery may need to be
  on antihypertensives

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Tetralogy of Fallot (TOF)
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Tetralogy of Fallot (TOF)

• 6 of defects
• Most common cardiac malformation responsible for
  cyanosis in a child over 1 year

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TOF

• Four Components
• Ventricular Septal defect - VSD
• Pulmonary stenosis narrowing of pulmonary valve
• Overriding of the aorta aortic valve is
  enlarged and appears to arise from both the left
  and right ventricles instead of the left
  ventricle
• Hypertrophy of right ventricle thickening of
  the muscular walls because of the right ventricle
  pumping at high pressure

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Clinical Manifestations TOF

• Dependent on degree of right ventricular outflow
  obstruction.
• Some infants are acutely cyanosed at birth,
  others have mild cyanosis that progresses over
  the 1st year as the pulmonary stenosis worsen
• Children are at risk of developing emboli, C.V
  disease, brain abscess, Seizures and loss of
  consciousness, or sudden death following an
  anoxic spell.

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Clinical Manifestations TOF

• Acute episodes of cyanosis and hypoxia (blue
  spells) usually during crying or after feeding
• With increased cyanosis increased clubbing of
  fingers, Squatting, Poor growth
• Severe irritability due to low oxygen levels
• tet spells - treated by flexing knees forward
  and upward

84
Knee-chest Position
Nurse puts infant in knee-chest position.
Child with a cyanotic heart defect squats
(assumes a knee-chest position) to
relieve cyanotic spells. Some times called
tet spells.
85
Diagnostic tests TOF

• Cyanosis
• Oxygen will have little effect on the cyanosis
• Loud heart murmur
• Echocardiogram demonstrates the four defects
  characteristic of tetralogy

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Management (TOF)

• Palliative to increase blood flow back to the
  pulmonary artery from right or left subclavian
  artery by doing modified blalock taussig shunt
• If oxygen levels are extremely low prostaglandins
  may be administered IV to keep the PDA open
• Prognosis less than 5 total operative mortality
  rate

87
Surgical Treatment

• Corrective Elective reparit in 1st year of life
  based on increased symptoms.
• Correction includes
• Closure of the VSD with dacron patch
• The narrowed pulmonary valve is dilated
• Coronary arteries will be repaired
• Hypertrophy of right heart should remodel within
  a few months when pressure in right side is
  reduced

88
Long Term Outcomes

• Leaky pulmonary valve that can lead to pulmonary
  insufficiency
• Arrhythmias after surgery
• Heart block occasionally a pacemaker is
  necessary
• Periodic echocardiogram and exercise stress test
  or Holter evaluation

89
Transposition of Great Vessels
90
Transposition of Great Arteries
91
Transposition of Great Arteries or Vessels (TGA
or TGV)

• Pulmonary artery leaves the Lt ventricle
• Aorta exits from the Rt ventricle
• With no communication between systemic and
  pulmonary circulation
• Males are affected more than females
• Associated defects such as Septal defects or
  patent ductus arteriosus permits blood to enter
  the systemic circulation and or pulmonary
  circulation for mixing of saturated and
  nonsaturated blood

92
Clinical manifestations TGV/TGA

• It depends on the type and size of the associated
  defects.
• If minimum communication present, then children
  are severely cyanosed
• If PDA or septal defect is present, less cyanosis
  symptoms present but might show signs of CHF.
• Heart sounds vary according to defects
• Cardiomegally occur after few weeks of life

93
Management TGV/TGA

• Surgical Palliative treatment (To provide
  intracardiac mixing)
• Administration of IV prostaglandin E1 to keep the
  ductus arteriosus open to temporary increase in
  blood mixing and provide O2 saturation of 75 or
  to maintain cardiac output.
• Rashkin procedure Enlarge septal defect
• Complete repair
• Switching the great vessels to their correct
  anatomic position.
• Prognosis Operative mortality about 5 10

94
ACUTE RHEUMATIC FEVER

• Acute rheumatic fever (ARF) is a systemic disease
  characterized by inflammatory lesions of
  connective tissue and endothelial tissue.
• It is a primary type of acquired heart disease.

95
Etiology/Incidence

• The pathogenesis is thought to be an autoimmune
  response to group A beta-hemolytic
  Streptococcus.
• Most attacks of ARF are preceded by an untreated
  streptococcal infection of the throat or upper
  respiratory tract at an interval of 2 to 6 weeks.
  
• ARF is not caused by direct infection of the
  organism.
• ARF is commonly seen in children 5 to 15 years of
  age, during winter months, and in poorer living
  conditions.
• Incidence is greater in underdeveloped countries,
  although it is on the rise in the United States.

96
Altered Physiology

• There is cross-reactivity between cardiac tissue
  antigens and streptococcal cell wall components.
• The Streptococcus may no longer be present, but
  auto antibodies attack one's heart (myocardium,
  pericardium, or valves)
• The unique pathologic lesion of rheumatic fever
  is the Aschoff body, a collection of
  reticuloendothelial cells surrounding a necrotic
  center on some structure of the heart.
• The inflammatory process involves the heart,
  joints, skin, and central nervous system.

97

• The inflammation may involve the leaflets or
  chordae tendinae of the heart valves, most
  frequently THE MITRAL or aortic valves, resulting
  in sclerosis and fusion of valve margins
• Valvular incompetence results
• There is a high recurrence rate.
• Of those with ARF, 75 progress to rheumatic
  heart disease in adulthood.
• ARF is a preventable condition with penicillin
  treatment of the primary infection. Erythromycin
  is treatment for those with penicillin
  sensitivities.

98
Complications

• ? Significant chronic heart failure
• ? Pericarditis, pericardial effusions
• ? Aortic/Mitral valve regurgitation
• ? Permanent cardiac damage

99
Major Manifestations

• CARDITIS manifested by significant murmurs,
  signs of Pericarditis, cardiac enlargement, or
  CHF
• POLYARTHRITIS almost always migratory and is
  manifested by
• swelling,
• heat,
• redness and tenderness
• or by pain and limitation of motion of two or
  more joints.
• (The synovial fluid is sterile (

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Cont. Major Manifestations

1. Chorea, a CNS disorder that lasts 1 to 3 months
   purposeless, involuntary, rapid movements often
   are associated with muscle weakness, involuntary
   facial grimaces, speech disturbances, and
   emotional liability

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Cont. Major Manifestations

• Erythema marginatum are temporary
  nonpruritic,pink rash.
• The erythematus areas have pale centers
• and round or wavy margins, vary greatly in size,
  And occur mainly on the trunk and extremities.
• Erythema is transient, migrates from place to
  place, and may be brought out by the application
  of heat.
• (Erythema marginatum)

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Cont. Major Manifestations

• 5. Subcutaneous nodules are firm, painless
  nodules seen or felt over the extensor surface of
  certain joints, particularly elbows, knees, and
  wrists, in the occipital region, or over the
  spinous processes of the thoracic and lumbar
  vertebrae the skin overlying them moves freely
  and is not inflamed.
• (Subcutaneous nodule)

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Minor Manifestations

• History of previous rheumatic fever or evidence
  of preexisting rheumatic heart disease
• Arthralgiapain in one or more joints without
  evidence of inflammation, tenderness to touch, or
  limitation of motion
• Fevertemperature in excess of 38C
• Elevated erythrocyte sedimentation rate (ESR)
• Positive C-reactive protein (CRP)
• ECG changesmainly PR interval prolongation

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• Supporting Evidence of Streptococcal
  Infection
• Increased titer of streptococcal antibodies
  (Antistreptolysin O or ASO titer)
• Positive throat culture for group A
  beta-hemolytic streptococci or recent scarlet
  fever

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Treatment

• Treatment of streptococcal infectiongenerally
  intramuscular (IM) penicillin G (Penicillin L-A)
  erythromycin for patients with penicillin allergy
  
• Prevention of permanent cardiac damage
  corticosteroids for patients with Carditis
• Palliative management of other
  symptomsSalicylates prescribed for patients with
  arthritis (but not while on high-dose
  corticosteroids due to risk of gastrointestinal
  bleeding) antipyretics after diagnosis has been
  established
• Prevention of recurrences of ARF

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Thank you for listening

• Have a wonderful day ?