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Title: Approach to congenital heart disease


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Approach to chd
  • Dr. Raheel Ahmed Shaikh
  • Paeds unit -1
  • CMCH,larkana

3
Prevalence
  • Acquired
  • Kawasaki disease
  • Rheumatic
  • Tubercular
  • Collagen
  • Congenital
  • Cyanotic 22
  • Acyanotic 68
  • VSD 25
  • ASD 6
  • PDA 6
  • TOF 5
  • PS 5
  • AS 5

Ceylon Med J 2001 Sep 46 (3) 96-8 Indian J
Pediatr. 2001 Aug68 (8)757-7 Nelsons Textbook
of pediatrics 17 ed.
4
Etiology
  • Multifactorial inheritance pattern mostly
  • Chromosomal abnormality (5-10).
  • -Trisomy 21 (50) gt A-V canal,VSD,ASD,
    others.
  • -Trisomy 18 (80)gt VSD,ASD,others.
  • -Trisomy 13 (40)gt VSD,ASD,PDA,others.
  • -Turner syndrome (xo)gtBicuspid aortic valve
    and co-ao
  • -others.
  • Maternal infections gtRubellaPDA,PS
  • Maternal diseasesgt PKU-VSD,ASD
  • DMleft septal
    hypertrophy
  • Drugsgtfetal hydntoin syndrome- VSD
  • Valproate effect-co ao left
    heart hypoplasia
  • Fetal alcohol syndromegt VSD,ASD,CO-AO.
  • Advance maternal age.
  • Majority of cases of the congenital heart
    diseases are unknown

5
History
  • Maternal history of medication, drugs, alcohol
    abuse, excessive smoking.
  • Prenatal history infection
  • Family history of CHD, hereditary, chromosomal

6
Symptoms
Difficult Breathing Chest indrawing Fast
breathing
Difficult Feeding Sweating during feeding
Syncope Exercise intolerence Easy fatigability
Poor growth Poor weight gain
Frequent respiratory infections
Seizure Focal neurological lesion
7
Stridor
Cynosis Bluish spells
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Physical Examination
Appearance Pale, Dusky, Polycythemic,
Syndromic Presence of Cyanosis,
Clubbing,Edema Tachypnea, Respiratory
distress Weight, Height for physical
development Skeletal abnormalities
Polydactyly, others Pulse Tachycardia,
Arrhythmia, Volume, Palpability BP All 4 limb
BP in complex CHD JVP - Elevated in Tricuspid
Atresia, Eisenmenger physiology -
Normal in TOF Abd - Sidedness of liver/spleen
palpation of Apical Impulse
to rule out Dextrocardia -
Hepatomegaly
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Blood pressure
  • Methods sphingnonaometer(different cuffs)
  • -Palpation method
  • -Doppler method
  • Wide pulse pressure
  • -Aortic insufficiency
  • -A-V communication
  • -PDA
  • Low blood pressure(H.F, pericardial
    tamponade,cardiomyopathy).
  • Difference in BP between upper and lower
    extremities Co-ao.

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What is cyanosis?
  • Cyanosis is a bluish discoloration of skin and
    mucus membrane that results when the absolute
    level of reduced hemoglobin in the capillary bed
    exceeds 3 g/dL.

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Cyanosis is it a cardiac cause or lung cause
  • Hyperoxia test
  • Neonates with cyanotic congenital heart disease
    usually do not have significantly raised arterial
    Pao2 during administration of 100 oxygen.

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Physical examination
  • Inspection look for
  • -Prominence of the precordium (cardiomegaly,Rt.he
    art enlargement )
  • -Jugular veins engorgement older children
  • -Any associated defects or findings (down
    syndrome, Digorge syndromeetc)
  • Palpation
  • -Pulses (rate, rythem,volume,peripherial
    pulses ,radio-femoral delay)
  • -Cardiac impulses.
  • -Thrill.
  • -Hyper dynamic precordium/heave.
  • -Hepato-splenomegaly

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  • Auscultation
  • a-First heart sound (A-V valves closure)
  • Best heard at the Lt. lower sternal
    border or apex
  • b-Second heart sound (semilunar valve
    closure)
  • Best heard on the 1st and 2nd I.C.S
    , normally there


    is normal splitting of the 2nd heart
    sound ,
  • -Single Aortic atresia,Pulmonary
    Artesia
  • -Fixed splitting ASD,PS,Rt.B.B.B
  • c-Murmurs

Systolic
Diastolic
Continous
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If we suspect C.H.D Investigation
  • CBC---- polycythemia, anemia.etc
  • CXR----heart size and shape
  • ECG---HR,axis ,rythm
  • LVH,RVH,BVH,BBB.
  • Echocardiography
  • MRI
  • Cardiac catheterization

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Congenital Heart Disease

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Common Acyanotic CHD
  • Left to right lesions
  • Ventricular septal defects
  • Atrial septal defects
  • Atrio-ventricular septal defects
  • Patent ductus arteriosus
  • Aortopulmonary window defect
  • Coronary artery fistula
  • Obstructed lesion
  • Pulmonary stenosis
  • Aortic stenosis
  • Coarctation of aorta
  • Congenital Mitral stenosis
  • Pulmonary venous HTN
  • Regurgitate lesions
  • Congenital Mitral incompetence
  • Mitral valve prolapse
  • Tricuspid regurgitation

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Common Cyanotic Lesions
  • Decreased Pulmonary blood flow
  • Tetralogy of Fallot
  • Tricuspid Atresia
  • Severe Pulmonic Stenosis
  • Pulmonary atresia
  • Ebsteins anamoly
  • Double Outlet Right ventricle
  • Increased Pulmonary blood Flow
  • Transposition of great vessles
  • VSD with pulmonary atresia.
  • Truncus Arteriosus
  • Hypoplastic left heart
  • Single ventricle
  • TAPVR

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Ventricular Defect
  • Subtype
  • Small (lt0.5cm2)
  • Moderate (0.5-1 cm2)
  • Large (gt1cm2)
  • Perimembraneous
  • Muscular
  • Supracristal (superior to crista
    supraventricularis)

80
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Ventricular Septal Defect
  • Small VSD (lt0.5cm2)
  • Asymptomatic
  • A loud, harsh, or blowing holosystolic murmur at
    LSE
  • Large VSD(gt1cm2)
  • dyspnea, feeding difficulties, poor growth,
    profuse perspiration, recurrent pulmonary
    infections, and cardiac failure in early infancy.
  • Apical thrust, systolic thrill at LSE
  • Pansystolic murmur(less harsh more blowing) at
    LSE

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Ventricular Septal Defect (VSD)
Small VSDs, the chest radiograph is usually
normal
Large VSD The presence of right ventricular
hypertrophy, olegeimic lung fields (pulmonary
hypertension or an associated pulmonic stenosis),
gross cardiomegaly with prominence of both
ventricles, the left atrium.
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Ventricular Septal defects management
  • 3050 of small defects close spontaneously, most
    frequently during the 1st 2 yr of life. Vast
    majority will close up to 4yaers.
  • Small muscular VSDs are more likely to close (up
    to 80) than membranous VSDs are (up to 35).
  • Medical management treat heart failure if
    present.
  • Surgical repair prior to development of an
    irreversible increase in pulmonary vascular
    resistance (usually prior to the patient's second
    birthday), chronic volume overload and heart
    failure.

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Indications of surgery
  • Failure to controle CCF
  • Failure to thrive
  • Supracristal VSD
  • Associated Pulmonary stenosis
  • Development of AR
  • 6-12 month child with rising P.HTN
  • gt2years child PBF twice of Systemic BF

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Atrial Septal Defects
  • Subtypes
  • Sinus venosus (high)
  • Ostium secundum (mid portion) most common
  • Ostium primum (low )

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Atrial Septal Defects secundum
  • Most common form of ASD (fossa ovalis)
  • In large defects, a considerable shunt of
    oxygenated blood flows from the left to the right
    atrium.
  • Mostly asymptomatic
  • The 2nd heart sound is characteristically widely
    split and fixed.
  • Soft systolic murmur at upper LSE

Secundum
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Atrial Septal Defectsprimum
  • Situated in the lower portion of the atrial
    septum
  • overlies the mitral and tricuspid valves.
  • In most instances, a cleft in the anterior
    leaflet of the mitral valve is also noted.
  • Combination of a left-to-right shunt across the
    atrial defect and mitral insufficiency
  • Apical pansystolic murmur(AVr)
  • The 2nd heart sound is split and fixed

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Atrial Septal Defect
  • X-ray
  • Enlargement of the right ventricle
  • Enlargement of atrium
  • Large pulmonary artery
  • increased pulmonary vascularity
  • ECG supeior axis in primum, RVH, partial RBBB

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Atrial Septal Defects
  • Secundum ASDs are well tolerated during
    childhood.
  • Antibiotic prophylaxis for isolated secundum ASDs
    is not recommended.(except if MR present)
  • Surgery or transcatheter device closure is
    advised for all symptomatic patients and also for
    asymptomatic patients with a shunt ratio of at
    least 21. or those with RVH
  • Intervention after 1 year before school entery.
  • Ostium primum defects are approached surgically

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Patent Ductus Arteriosus
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Patent Ductus Arteriosus
  • Connects pulmonary artery and descending aorta.
  • Normally closed shortly after birth.
  • Common in VLBW with pulmonary diseases and in
    congenital rubella.
  • FM 21
  • Spontaneously close in premature
  • PDA persisting in term beyond 1st few weeks will
    rarely close spontaneously

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Patent Ductus Arteriosus
  • Small defect
  • no symptoms.
  • Pulses are normal
  • Large defect
  • Breathlessness while feeding
  • Slow growth
  • Repeated Lower RTI
  • Wide pulse pressure
  • Enlarged heart
  • Apical heaving
  • Thrill in L second IS
  • Continuous murmur (machinary)in 2nd LICS
  • X-ray prominent pulmonary artery with increased
    vascular markings, Left heart enlarge

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Patent Ductus Arteriosus
  • Medical Management
  • Medical closure in preterm tried with
    indomethacin (0.2mg/kg/dose iv for 3 doses 8-12hr
    apart) or brufen (ibuprofen).
  • Start therapy after echo only.
  • Surgical Management
  • Ligation and division of ductus is treatment of
    choice
  • In asymptomic patiants before 1year
  • P.HTN is not contraindication.

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Coarctation of the Aorta 6
  • In boys more than in girls
  • Almos always juxtaductal in position.
  • Weak femoral pulses, Radio femoral delay,
    hypotention in lower parts of body.
  • High BP in upper body part in the arm 20mmhg more
    than leg, headache, vertigo, epistaxis.
  • Murmur at left interscapular area in back.
  • Treatment
  • Digoxin diuretic PGE1 infusion.
  • Surgical repair at the age of 2yrs

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pulmonary stenosis 10
  • Narrowing in pulmonary valve
  • RT side heart failure
  • Ejection Systolic murmur at Left 2nd ICS rediate
    to back, S2 widely split
  • ECG Echo RT side hypertrophy
  • Treatment
  • Balloon valvuloplasty
  • Surgical repair

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Aortic stenosis
  • 7 of total cases of CHD
  • Increased pressure in the LF side of the heart
    (LV hypertrophy)
  • Easy fatigability, exertional chest pain, syncope
  • Ejection systolic murmur at right _at_nd ICS rediate
    to neck, high HR.
  • Treatment..
  • Beta-blocker or ca channel blocker to decreased
    hypertrophy
  • Balloon valvoplasty
  • Surgical repair

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Cyanotic Congenital Heart Disease
- Cardinal Clinical features -
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ONSET OF CYANOSIS
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CLINICAL DIFFERENCES BETWEEN CYANOTIC HD WITH ?
AND ? PBF
DECREASED PBF (eg. TOF) INCREASED PBF (eg. TGA, TAPVC)
Presentation at Any age Neonate / Infant
Appearance Comfortable Sick, Lethargic, Irritable
Cyanosis Mild - Severe Mild (except TGA with intact IVS)
Squatting /Cyanotic spells Common Uncommon
Feeding difficulty / ?Sweating Absent Present
Failure to thrive Absent Present
Weight Gain Normal Suboptimal
Recurrent LRI No Yes
Tachypnea Absent Present
Heart size Normal Cardiomegaly
CHF, Tachycardia, S3, S4 Absent Present
Thrill Maybe Present Absent
Murmur Systolic Soft, Diastolic
CXR Ischemic Lungs, No Cardiomegaly Plethoric Lungs, Cardiomegaly
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Tetralogy of Fallot
  • Most common cynotic
  • Ventricular septal defect
  • Pulmonic stenosis
  • Overriding aorta
  • Right ventricular hypertrophy

Cyanotic
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Risk factors
  • Environmental factors
  • maternal diabetes threefold increased risk,
    retinoic acids, maternal phenylketonuria (PKU),
    and trimethadione
  • Genetic cause heterogeneous

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Clinical Presentation
  • Clinical presentation is directly related to the
    degree of pulmonary stenosis.
  • Severe stenosis results in immediate cyanosis
    following birth.
  • Mild stenosis will not present until later.
  • Growth is retarded insufficient oxygen and
    nutrients
  • SOB on exertion ? rest
  • Digital clubbing
  • Paroxysmal hypercynotic attacks (tet spells)
  • Left parasternal heave
  • Systolic thrill (50) at left PSE 3 and 4 ICS
  • S2 is often single
  • Harsh ejection systolic murmur at left PSE 3rd
    ICS

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Tet Spell
  • Tet spells at 2-3yo, child becomes cyanotic,
    restless, gasping respiration,may experience
    syncope
  • More frequently in morning upon awakening or
    after vigorous cry.
  • Children assume squatting position
  • During spell, dec in intensity or disappearance
    of systolic murmur

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Exams and Tests
  • CBC
  • - ? hematocrit
  • ECG
  • -RVH, RAD
  • CXR
  • -boot shaped heart, right sided aortic arch
  • Echocardiogram
  • -VSD, PS, RVH

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Tetralogy of Fallot
Apex is lifted, concavity in pumonary segment,
oligemic lung field. Boot shaped Heart
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Treatment
  • Severe TOF with worsening cynosis in early
    neonatal period require prostaglandins E
    infusion and surgery (modified Blalock-taussing
    shunt)
  • Corrective surgery carried out from 3 months to
    2year depending upon expertise availablity

Blalock-Taussig shunt
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POTTS SHUNT WATERSTON
SHUNT
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Treatment of the cyanotic spells
  • Try to calm the patient .
  • Knee chest position,
  • O2
  • Propranolol(0.1-0.2mg/kg slow IV).
  • Morphine s.c
  • NaHCO3 iv
  • Increase IV fluid.
  • Prevented by oral Propranolol (1mg/kg every 4hr)

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Transposition Of great arteries
  • Most serious cynotic lesion,
  • Seen in neoborn period (5)
  • More common in infants of diabetic mothers and in
    males.
  • Survive when ASD, PDA, or VSD

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Clinical Features
  • TGA with intact ventricular septum
  • Cynosis and tachypnea within 1st hours of life.
  • Single S2, no murmur
  • PGE1 (o.o5-o.2ug/kg/min infusion)is immediately
    started to maintain patency of DA.
  • CCF is less common
  • TGA with VSD
  • Mild cynosis recognised 1st month of life.
  • Murmur is pansystolic
  • Many Neonates are large 4kg at birth then growth
    retardation occurs.
  • They need anti-CCF measures

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Transposition Of great arteries
  • Slight cardiomegaly, narrow base
  • Egg shaped Heart

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Management
  • Corrective surgery by age of 2 weeks
  • Procedures
  • Rashkind or ballon atrial septoplasty
  • Mastured procedure
  • Total repair Arterial swich technique.

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Tricuspid Atresia
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Clinical Features
  • Progressive Cynosis
  • Poor feeding
  • Tachypnea over the first 2 weeks
  • Holosystolic murmur due to VSD
  • Single S2
  • Left axis Deviation and left VH on ECG
    characteristics.
  • Normal heart size

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Clinical Findings
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Treatment
  • Medical management
  • Anti-CCF measure
  • Surgical Repair
  • VSD closure and placement of conduit between
    right ventricle and Pulmonary artries.

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Total Pulmonary venous return
  • 2
  • Pulmonary vein return to the right atrium or the
    superior vena cava instead of the left atrium
  • Atrial level communication is required.
  • Without obstruction Hyperactive RV impulse with
    wide split S2
  • Systolic ejection murmur at Left USB
  • Mid diastolic murmur at left LSB
  • With obstruction signs of right sided heart
    failure, no murmur, no change in S2
  • Treatment
  • Give PGE, cath, and surgical treatment

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Hypoplastic left Heart Syndrome
  • Most common cause of death from cardiac side in
    1st month
  • Failure of development of MV,Av,or arch.
  • Infants may appear healthy at birth, but signs
    of HLHS soon become apparent after the ductus
    arteriosus closes.
  • Cyanosis minimal, weak pulses, Cold extremities,
    greyish colour(low cardiac output)
  • S2 single and loud no heart murmur
  • Right ventricle Hypertrophy
  • Treatment
  • Give PGE,and surgical treatment

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Treatment of C.H.D
  • This is depend on the type of the C.H.D.
  • No treatment (observationreassurance)
  • Medical treatment(antifailure,antiarythmaic..etc).
  • Surgical treatment (palliative or curative).
  • Cardiac transplant or lung heart transplant.

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1-General measures
  • Special positions. (semisiting ,knee chest
    position (
  • O2 (most patients need O2 and other need little
    O2).
  • IVF(again depend on type of CHD ).
  • Salt restriction.
  • Exercise restriction.
  • Rx of anemia.
  • Rx of polycythemia. PCVgt65
  • Avoidances of dehydration mainly polycythemic
    patients.
  • Avoidances of high altitude.
  • Avoidance of contraceptive thrombosishypertensio
    n.
  • Correction of acidosis.
  • Correction of electrolyte disturbances .
  • Careful monitoring during surgery.

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2-Rx of congestive heart failure
  • Digoxin Digitalization 0.04mg/kg
  • Maintenance 0.01mg/kg
  • Loop diuretics frusemide 1-2 mg/kg/day.
  • Potassium sparing diuretics spironlactone
  • After load reducing agents
  • eg. Captopril 0.5-6mg /kg/24 hours.
  • Positive intropic agents .dopamine and
    dobutamine

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