CONGENITAL HEART DISEASE

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CONGENITAL HEART DISEASE
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INCIDENCE

• Congenital Heart Disease is one of the most
  common causes of morbidity mortality in the
  newborn.
• It is estimated that the incidence of CHD is in
  the order of 1 of all livebirths.
• One-third of these cases present in the neonatal
  period

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ANTICIPATION
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COMMON PRESENTATIONS

• SYMPTOMS
• SLOW FEEDING
• BREATHLESSNESS
• IRRITABILITY
• PALLOR SWEATING
• FAILURE TO GAIN WEIGHT

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COMMON PRESENTATIONS

• SIGNS
• CYANOSIS
• TACHYCARDIA
• TACHYPNEA
• CARDIAC MURMUR
• CARDIOMEGALY
• SHOCK

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CYANOSIS

• Definition
• Bluish discolouration of the skin mm, due to
  the presence of more than 5gm/dl of reduced Hb.
• Clinically apparent when SaO2 lt 85
• Most significant sign of serious cardiac anomaly.
• Types
• Central
• Peripheral
• Differential

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CYANOSIS

• Central cyanosis
• Resulting from arterial desaturation, affects
  tongue, mm peripheral skin.
• Causes
• R L shunting
• Alveolar hypoventilation
• Ventilation perfusion mismatch (V/Q ratio)
• Diffusion impairment in the lung

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• Peripheral cyanosis
• Results from prolongation of circulation time
  
• increase in tissue oxygen extraction,with
  (N) SaO2.
• Confined to the extremities.
• Differential cyanosis
• Visible colour difference, or discrepant SaO2
  between
• upper lower portions of the body.
• Pink upper Blue lower body occurs when blood
  shunts from R ----gt L ( PA to Ao ) at level of
  ductus arteriosus e.g. In obstructive heart
  lesions.
• Blue upper Pink lower body only d-TGA with PH
  , or Coarctation.

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MURMURS

• Innocent Murmurs
• Pathological Murmurs
• Innocent murmurs
• Occurs in about 75 of neonates
• Examples
• Peripheral pulm. Stenosis (PPS)
• Transient systolic murmur of a closing PDA
• Transient systolic murmur of TR

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Hyperoxia test

• To differentiate cyanotic CHD from pulmonary
  diseases with V/Q mismatch.
• In both situations the patient has low Sao2 in
  room air
• Obtain an ABG from Rt radial A. (preductal)
  umbilical or lower extremity A. (postductal)
  before after exposure to 100 O2 for 10 min.
• Cardiac lesions no or slight change in
  PaO2
• (N) PCO2
• Pulmonary lesions ?? in PaO2 ?? PaCO2

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Hyperoxia Test

• Explanation
• In the infant with alveolar hypoventilation or
  V/Q mismatch, ?O2 conc. in the alveoli will raise
  pulm. venous PO2, therefore
• ? PaO2.
• The infant with R L intracardiac shunt, will ?
  pulm. venous PO2, but because of the intracardiac
  shunting of the hypoxemic blood to the Ao. , the
  PaO2 will not change significantly.

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• If hyperoxia test yields ? pO2
• EXCLUDE
• Pulm valve atresia with intact vent. septum
• Pulm valve atresia with VSD
• TGA
• DO NOT EXCLUDE
• TAPVR
• TOF with R ------gt L shunt
• Hypoplastic left heart

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Chest Radiograph

• Evaluates
• Cardiac SIZE
• Cardiac CONTOUR
• Cardiac POSITION
• Pulmonary Vascular Markings

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Cardiac size

• Cardiac size depend primarily on the volume of
  blood within the cardiac chambers rather than on
  the thickness of the cardiac muscle.
• Cardiothoracic ratio in the neonate should be lt
  0.60
• Cardiac enlargement maybe due to
• L R shunts
• Valve regurgitation
• Ventricular failure
• Factors influencing cardiac size
• Expiratory phase of respiration
• Large thymus

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Cardiac Position

• The heart maybe on the Right, Left, Midline.
• The normal position of the heart is in the Left
  hemithorax
• Malposition of the heart maybe
• Primary Developmental Dextrocardia
• isolated
• situs inversus
• Secondary Pneumothorax
• Diaphragmatic hernia
• Hypoplasia of the lung

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Cardiac Contour

• Some intracardiac structural defects give rise to
  characteristic shapes
• e.g. TGA egg-on-side shape
• TOF couer-en-sabot
• TAPVR 8-shaped figure

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Pulmonary Vascularity

• In NORMAL infant, the anteropost. film shows the
  following
• The Rt PA its primary branches are visisble in
  the Rt hilum.
• The Lt PA its primary branches are hidden
  behind the heart.
• The vessels taper gradually towards the periphery
  are too small to be seen in the distal 1/3 of
  the lung fields.

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Four abnormal patterns can be distinguished

• Pulmonary blood flow is decreased
• Lungs appear dark
• Pulm. Vessels in the hilum are ? in size
• No vessels are seen in the middle 1/3 of lung
  fields
• Pulmonary blood flow is increased
• Hilar vessels are enlarged
• Middle 1/3 of lung fields has vessels larger than
• normal
• Vessels are seen upto the outer 1/3 of lung

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• Pulmonary venous hypertension
• Lung fields are hazy.
• Hila are prominent ( due to dilatation of PVs )
• Peripheral PAs are indistinct ( elevated pulm.
  venous pressure causes ? in interstitial fluid,
  diminishing the contrast between the air-filled
  alveoli fluid filled arteries )
• e.g. TAPVR, hypoplastic lt heart syndrome
• Pulmonary plethora in peripheral lung fields
• ? central hilar vessels
• e.g. TOF PA ( central PAs are atretic or
  hypoplastic pulm. Blood flow is supplied by
  large collateral vessels from Ao that enter the
  lung distally anastomose to the peripheral PAs
  )

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Serious Congenital Heart Disease

• Transposition of Great Vessels
• Tetralogy of Fallot (severe)
• Pulmonary atresia
• Ebstein anomaly
• Truncus arteriosus
• Total Anomalous Pulmonary Venous Return
• Double Outlet Right Ventricle
• Hypoplastic Left Heart Syndrome
• Aortic arch anomalies

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CLASSIFICATION

• CYANOTIC ACYANOTIC
• TGA
  VSD
• PA
  ASD
• TOF
  PDA
• DORV
  Coarctation of Ao.
• TA
  Common AV canal
• Truncus Arteriosus PS intact
  VSD
• TAPVR Hypoplastic Lt heart
  syndrome
• Ebstein Anomaly

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Cyanotic CHD

• ?? PBF
  ?? PBF
• TOF
  TGA
• PAVSD
  TAPVR
• PAintact VS Hypoplastic Lt Heart
  Syndrome
• PS severe
  DORV VSD
• TGA PS
  Truncus Arteriosus
• DORV
• Ebstein anomaly
• TA
• Truncus Arteriosus PS

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DIAGNOSIS

• Clinical presentation
• BP (4 limbs)
• Hyperoxia test
• Chest radiograph
• ECG axis, vent. hypertrophy
• Echocardiography (M-mode, 2D-mode) Doppler
  studies
• Cardiac catheterization
• Angiography

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MANAGEMENT

• Secure cardiorespiratory support
• Mechanical Ventilation if necessary
• I.V fluids Flow chart
• Correct electrolyte metabolic disturbances
• Diuretics if CHF
• Inotropes
• Digitalize if CHF !!! Obstructive lesions
• Supportive Prostaglandine E2
• Palliative Corrective surgery

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TGA

• Anomaly
• The pulm. systemic circulations are two
  parellel circuits.
• Aorta arises anteriorly from RV PA arises
  posteriorly from LV
• Systemic veins return to RA, desaturated blood
  flows from RA to RV to Ao. to the body again,
  whereas oxygenated blood from pulm. veins return
  to LA to LV to PA again to the lungs.
• Survival depends on the presence of a shunt
  between the two circuits, ASD , VSD, PDA
• D (dexro) Ao arises to the Rt
• L (Levo) Ao arises to the Lt

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• Incidence 17 of all CHD 14500 livebirths
• Predisposing factors
• IDM
• Male sex (21)
• Presentation
• Cyanosis within 1st 12-24hrs or days of life
• Diagnostic features
• Clinically
• Cyanosis
• Marked hypoxaemia
• Absent heart murmur ( maybe short systolic murmur
  over LSB )

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• CXR Mild cardiomegaly
• ( N ) or ? PBF (later)
• egg-on-side appearance, ( narrow
  superior mediastinum due to unexplained absence
  of thymic shadow, relative anteroposterior
  position of the great vessels)
• ECG Rt Axis
• Diagnosis
• Echocardiography
• Cardiac catheterization
• Management
• Supportive PGE2 infusion
• Palliative surgery Rashkind Balloon atrial
  septostomy
• !!! Corrective surgery

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TOF

• Anomaly
• Pulmonary stenosis ventricular seotal defect
  Right ventricular hypertrophy- Aorta overriding
  the ventricular septum.
• The pulmonic obstruction maybe infundibular
  (subvalvular) or at pulmonary valve or peripheral
  pulmonary arteries.
• The pulmonic obstruction ranges from mild
  stenosis to complete atresia ( accounts for the
  severity of the clinical presentation )
• The VSD is located high up in the ventricular
  septum

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• Incidence 9 of all CHD during 1st week of life,
• 1/5000 livebirths.
• Clinical presentation depends on the severity of
  the pulmonary stenosis.
• In severe PS the resistance to the blood flow
  out of the RV is very high, desaturated blood
  preferentially passes through the VSD into the
  Ao, resulting in arterial hypoxemia cyanosis.
• In mild PS there is little resistance to the
  blood passing out of the PA, there is no
  hypoxemia cyanosis initially, but as the
  pulmonary vascular resistance falls over the
• 1st few weeks of life, there is increasing
  L-R shunt across the VSD these infants enter
  into heart failure.
• In moderate PS (occasional) there is equal
  resistance between pulmonary systemic
  circulation, causing bidirectional shunt , which
  becomes predominantly R-L as the infant cries.

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• Signs
• S2 ?
• murmur ejection systolic at LSB (PS)
• Tetralogy spells unusual in neonates
• CXR
• heart size (N)
• pulmonary blood flow?
• ch.ch. coeur-en-sabot ( main PA segment is ?)
• Rt sided Aortic arch in ¼ of cases
• ECG
• Rt axis
• RV hypertrophy

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• 2D echo diagnostic
• Cardiac catheterization angiography
• Diagnosis cyanotic infant (N) heart size
  ?pulm. blood flow on CXR Rt axis deviation RV
  hypertrophy on ECG systolic ejection murmur
• Treatment
• In profound cyanosis with arterial hypoxemia
• Propranolol 2-6mg/kg/day
• Blalock-taussig shunt surgery ???
• One-stage repair surgery !!! (close VSD, relief
  RV outflow tract obstruction)

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TOF
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TOF
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TOF - ECG
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Pulmonary atresia/stenosis

• PA IVS
• PS IVS
• Anatomy
• PA IVS the pulm. valve is an imperforate
  membrane, the RV is hypoplastic, the tricuspid
  valve annulus is hypoplastic, RA is enlarged
  hypertrophied, PA are (N).
• All venous blood returning to the RA must pass
  through the FO, to LA, LV, Asc. Ao causing
  dilatation of all these chambers, RV is
  hypoplastic. The pulm. Blood flow is derived from
  the Ao via a small tortuous DA. As the latter
  closes, during the 1st hours or days after birth,
  this minimal pulm. blood flow diminishes further,
  causing severe hypoxemia acidosis.

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• Incidence
• 7 of CHD in 1st week of life
• 1/14,000 livebirths.
• Clinical presentation
• Intense cyanosis within 1st 24hrs after birth.
• S2 single
• Murmur
• continuous from PDA or
• systolic along LSB from TR
• Liver enlargement
• CXR
• cardiomegaly ( RA LV dilatation )
• ? pulm. vascular markings
• Ao arch to left of trachea

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• ECG ch.ch !!! Left axis
• LV hypertrophy in precordial leads
• ( dominant S in V1)
• Tall peaked T- wave ( RA hypertrophy )
• 2D-echo to identify small RV
• Colour flow doppler to differentiate stenosis
  from atresia
• Cardiac catheterization
• DD Cyanotic neonate TGA
• S2 split ? pulm. Blood flow on CXR
• RV hypertrophy in ECG ch.ch. Echo
• Treatment
• Supportive PGE2 corrective surgery
  (pulmonary vulvotomy atrial septostomy RV
  outflow tract reconstruction)

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Pulmonary atresia with VSD

• Mimics a severe form of TOF.
• Pulmonary valve the pulm. Trunk is atretic. The
  entire RV output is ejected into the Ao. Pulm.
  Blood flow is dependent on a PDA.
• Clinically Cyanosis during 1st few days of life
  (after closure of ductus)
• S2 single loud
• murmur contiuous PDA murmur
• CXR enlarged cardiac shadow
• ECG RV hypertrophy
• Treatment PGE2 Aortopulm. shunt

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Ebstein Anomaly

• Anatomy redundency dysplasia of the tricuspid
  valve, causing its downward displacement into the
  RV, with adherence of the posterior leaflets to
  the RV wall. Thus the Rt side is divided into 3
  segments a normal RA ( a portion of the Rt
  atrium above), the displaced part , that is
  partly the ventricular myocardium, the true RV.
• The tricuspid valve is stenotic regurgitant,
  causing a hugely enlarged RA, the RV is small, an
  ASD is always present.
• Venous blood from RA passes through the FO, into
  LA LV causing ? flow to left side of the heart.

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• There is ? blood flow to RV, pulmonary A.
  lungs. The added ? PVR, immediately after birth
  will further ? the pulm. blood flow leading to
  severe hypoxemia. Pulm. Blood flow depends mainly
  on the blood shunting through the PDA, but as the
  ductus closes the hypoxemia becomes more intense.
  As the FO becomes restictive, RA decompensates
  leading to Rt sided heart failure hepatomegaly.

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• Incidence Rare !!! 0.5 of CHD,
• 1/80,000 livebirths.
• Clinically Cyanosis
• Murmur pansystolic (TR)
• Rt sided CHF
• CXR enlarged cardiac shadow (RA dilatation)
• ECG P waves Tall, peaked
• P-R intervals short delta waves
• ( prolonged QRS complexes)
• 2Decho diagnostic
• Diagnosis cyanosis murmur over LSB huge
  cardiomegaly on CXR LV hypertrophy on ECG
• DD TGA ? Pulm. Vascular markings on CXR
• TOF no cardiac enlargement

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• Treatment
• Two approaches !!!
• Many infants improve markedly over 1st few weeks
  of life as PVR ?
• Supportive anticongestive measures
• PGE2 to maintain DA patency till PVR ?
• Critically ill neonates need a surgical
  intervention ( surgical patch closure of the
  tricuspid valve, aortopulm. shunt, atrial
  septectomy.

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TRUNCUS ARTERIOSUS

• Anatomy A single arterial trunk arises from the
  base of the heart supplies the coronary,
  pulmonary systemic arteries. A VSD is always
  present, with the truncus overlying the defect
  receiving blood from both RV LV.
• Three types
• (1) Type 1 the main PA arise from the posterior
  left side of the TA then divide into Rt PA Lt
  PA.
• (2) Type 2 No main PA, the Rt Lt PA arise
  directly from separate orfices from post. Aspect
  of TA.
• (3) Type 3 No main PA, the Rt Lt PA arise from
  the lateral aspect of the TA.

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• Both ventricles have the same systemic pressure,
  eject blood to the truncus
• As the PVR is initially high, the pulm. blood
  flow is normal, but as PVR ? over 1st few weeks
  of life, the pulm. blood flow ?, causing CHF.
• Initially cyanosis is minimal, BUT as the pulm.
  Blood flow ?, PVR ? leading to ? in pulm.blood
  flow cyanosis ensues.
• Incidence 2 of CHD 1/ 33,000 livebirths.
• Clinically
• Manifestations of CHF, as early as the 1st week
  of life.
• Hyperactive precordium, full bounding pulses.
• S2 single

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• Clinically
• murmur ejection systolic over LSB
• CXR normal
• ECG RV,LV combined hypertrophy
• 2Decho large truncal artery overriding the VSD
• Catheterization to show the shunting at the
  ventricular level
• Diagnosis Mild cyanosis CHF bounding pulses
• Common association DiGeorge syndrome
• Treatment
• Antifailure measures
• At 4-8 weeks reconstructive surgery

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TRUNCUS ARTERIOSUS
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Total Anomalous Pulmonary VenousReturn (TAPVR)

• Anatomy
• The pulm.veins have no connection with the LA,
  they drain directly or indirectly into the RA.
  There is total mixing of the systemic venous
  blood pulm. Venous blood flow within the heart.
  The pulm. Veins may drain
• above ( supradiaphragmatic) or
• below (infradiaphragmatic)

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• (a) Supradiaphragmatic into
• RA directly
• coronary sinus
• SVC
• (b) Infradiaphragmatic into
• IVC or
• one of its main tributaries via ductus
  venosus
• Oxygenated deoxygenated blood mix before or at
  the level of RA, passing into RV PA, or through
  an ASD into LA. RA, RV, PA are enlarged while
  LA, LV are (N) or small

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• Clinically depends on the presence or absence of
  obstruction to the pulm. venous return.
• Three patterns
• 1- Severe obstruction to PVR in NNperiod, in
  infracardiac group severe pulm. congestion
  pulm. hypertension severe cyanosis ,tachypnea,
  hypoxemia, severely ill fail to respond to MV.
• 2- Moderate to mild obstruction to PVR in early
  infancy, with large L-R shunt, causing pulm.
  hypertension, cyanosis is mild, systolic murmur
  over LSB.
• 3- No obstruction to PVR total mixing of
  systemic pulm venous blood, large L-R shunt, no
  PH, mildly cyanosed.

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• Clinically
• (1) Cyanosis, tachypnea, hypoxemia
• S2 single accentuated
• CXR perihilar pattern of pulm. edema, small
  heart
• ECG RV hypertrophy ( qR pattern in V3R, V1)
• P waves tall, peaked
• (2) Cyanosis mild, signs of CHF hyperdynamic
  RV, ejection systolic murmur over LSB
• CXR pathognomonic !!! Snowman appearance
• large supracardiac shadow normal cardiac
  shadow anomalous pulm. Veins enter the SVC)

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TAPVR
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• Doppler flow study pathognomonic
• Cardiac catheterization SaO2 in both atria,
  ventricles Ao are similar.
• Treatment
• Supportive PGE2
• Corrective surgery

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DOUBLE OUTLET RIGHT VENTRICLE

• Anatomy
• Both Ao. PA arise from the RV.
• The only outlet from LV is through the VSD.
• Mild systemic desaturation due to mixing of
  saturated desaturated blood in RV.
• Clinically mild hypoxemia
• murmur pansystolic over LSB
• ECG biventricular hypertrophy
• 2Decho diagnostic
• Treatment surgical correction (PA banding
  creating an intracardiac tunnel for blood from LV
  through VSD into the Ao.

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DORV
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DD OF CYANOTIC CHD

• Cyanosis

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Hypoplastic Left Heart Syndrome

• Anatomy

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• Incidence
• Clinically
• CXR
• ECG
• 2Decho
• Catheterization

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Interrupted Aortic Arch

• Anatomy

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