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CONGENITAL HEART DISEASE

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CONGENITAL HEART DISEASE. 1- Zahra Asghari. 2- Samira Baniasadimoghadam. 3-Mahtab Dolatabadi. 4- FahimeGohari. 5-Aida Mirkazemi. 6- AzadeIzadi. 7- SanazkhosraviGhareche – PowerPoint PPT presentation

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Title: CONGENITAL HEART DISEASE


1
CONGENITAL HEART DISEASE
  • 1- Zahra Asghari
  • 2- Samira Baniasadi moghadam
  • 3-Mahtab Dolatabadi
  • 4- Fahime Gohari
  • 5-Aida Mirkazemi
  • 6- Azade Izadi
  • 7- Sanaz khosravi Ghareche

2
  • approximately 0.8 of all live births are
    complicated by congenital cardiac abnormalities.
  • Congenital heart disease is a major cause of
  • infant morbidity and mortality.
  • As a result of advances in pediatric cardiology
    and cardiothoracic surgery, approximately 85 of
    infants born with congenital heart disease can be
    expected to survive into adulthood.
  • Most cases of congenital heart disease occur
    sporadically.

3
Etiology
  • without a known specific cause.
  • Genetic abnormalities are responsible for a
    proportion of cases and may contribute to cases
    occurring sporadically as well
  • Environmental factors.
  • Symptoms can develop shortly after birth when
    transition from fetal to adult circulation
    represents a new dependence on biventricular
    circulation with a pulmonary circuit.?begin of
    symptoms

4
  • The isolated pulmonary and systemic circulations
  • ?D the ductus arteriosus and the foramen ovale.
  • ?L Hypertrophy of the morphologic right
    ventricle in L transposition of the great
    arteries is sufficient to compensate for systemic
    vascular resistance and maintain normal perfusion
    for years with symptoms often developing when the
    systemic ventricle fails.

5
  • Still other lesions may develop in adulthood when
    degenerative changes, such as stenosis of
    apreviously well-functioning bicuspid aortic
    valve, are superimposed on an initial lesion.
  • congenital defects may
  • ?Go undetected throughout life ?small ASDs
  • ?resolve spontaneously ?small muscular VSDs

6
Septal defects
7
ATRIAL SEPTAI DEFECTS(ASD)
  • ASDs are some of the most common congenital
    defects, representing 10to l7
  • a higher prevalence in women (6o)
  • Defects are classified according to their
    location in the interatrial septum
  • 1- primary ostium defect 20, defect in
    atrioventricular junction
  • usually, associated with a cleft mitral valve and
    mitral failure
  •  rarely, great ASD, single VA valve?defect in VA
    septum
  • 2-secundary ostium defect60,defect in fossa
    ovalis

8
  • uncomplicated ASDs ?blood shunts hom the left to
    the right atrium
  • The magnitude of the shunting is determined by
    the size of the defect and the compliance of the
    Ieft and right ventricles.
  • Small ASDs the increased blood flow in the right
    atrium without sequelae and no significant
    hemodynamic compromise of the right heart.
  • Large ASDs the right atrium and right ventricle
    dilate to accommodate the increased volume of
    shunted blood. Pressurein the pulmonary artery
    increases econdary to the increased volume of
    blood.

9
Symptoms
  • Most patients with ASD are asymptomatic until
    adult.
  • when symptoms such as fatigue, dyspnea, and poor
    exercise tolerance develop, secondary to right
    ventricular dysfunction.
  • atrial fibrillation, especially after 50 years of
    age
  • 5Irreversible pulmonary vascular obstruction
    resulting in right-to-left shunting and cyanosis
    (Eisenmenger syndrome)
  • Stroke or transient ischemic attack in order to
    paradoxical emboli

10
Physical exam
  • Parasternal RV impulse
  • Widely and fixed split S2
  • Ejection murmur across pulmonic

11
Diagnosis
  • Two dimensional and color Doppler
    echocardiography
  • Transesophagial echocardiography
  • Electerocardiography
  • MRI
  • Cardiac catheterization
  • Angiography

12
Treatment
  • closure, even in the absence of symptoms,
    pulmonary hypertension,
  • Significant pulmonary hypertension is a
    contraindication to ASD closure.
  • devise Amplatzer
  • Primum and sinus venosus defects should be
    surgically closed.
  • Antibiotic prophylaxis for infective endocarditis
    is not required for small ASDs or patent foramen
    ovale or after ASD closure.

13
Ventricular septal defect (VSD)
  • VSD is a common congenital abnormality in
    newborns.(1 in 500 births)

14
VSDs classification
  • Supracristal vsd
  • Perimembranous vsd(most common80)
  • Atrioventricular canal vsd
  • Muscular vsd

15
  • If the defect is small
  • Right ventricular size function are normal and
    pulmonary vascular resistance dose not increase.
  • If the defect is large
  • The right ventricle dilates to accommodate the
    increased volume pulmonary blood flow
    increases.

16
syndrome Eisenmenger
  • Pulmonary vascular obstruction
  • Pulmonary artery HTN
  • Reversal of interventricular shunt
  • Systemic desaturation
  • Cyanosis
  • clubbing

17
Physical examination
  • Hyperdynamic precordium
  • Palpable thrill along the left sternal border
  • Holosystolic murmur
  • Loud p2

18
Diagnosis
  • Tow dimensional echocardiography
  • Doppler echocardiography
  • MRI
  • CT scan

19
Treatment
  • Sutures
  • Prosthetic patch
  • Percutaneous device closure(muscular)

20
Congenital AS bicuspid aortic valve
  • Valvalr stenosis is most often secondary to a
    bicuspid aortiv valve.
  • Congenital left ventricular outflow obstruction
    occurs at valvular,subvalvular or supravalvular.

21
Bicuspid aortic stenosis
  • as with acquired aortic stenosis chest
    pain,syncope,congestive heart failure.
  • Other complications sudden death infective
    endocaditis.

22
Physical examination
  • Ejection quality murmur at the left sternal
    border.
  • Early systolic ejection click
  • Decrescendo diastolic murmur of aortic
    insufficiency

23
Diagnosis
  • Two dimensional echocardiography
  • Doppler echocardiography

24
Treatment
  • Percutaneous valvuloplasty
  • Aortic valve replacement
  • Antibiotic prophylaxis
  • Oral hygiene

25
Sub aortic stenosis
  • Firs diagnosed in adulthood
  • A discrete,fibrous diaghragm

26
Supravalvar aortic stenosis(svsa)
  • Loss-of-function mutations in the extracellular
    matrix protein,elastin,is responsible.
  • It is often part of a syndrome with
    hypercalcemia,multiple skeletal-vascular-developem
    ental abnormalities.

27
PULMONIC VALVE STENOSIS
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  • Pulmonic valve stenosis is the most common cause
    of obstruction to right ventricular outflow
  • usually occurs as an isolated congenital lesion

30
  • Fusion of the pulmonary leaflets creates the
    pressure-overloaded state and results in right
    ventricular hypertrophy
  • Unless the valve is severely stenotic at birth,
    most affected persons live a normal life until
    adolescence or young adulthood
  • Patients with mild-to-moderate stenosis are
    usually asymptomatic and rarely have
    complications associated with the defect
  • Patients with moderate-to-sever obstruction often
    exhibit progressive fatigue and dyspnea
  • If right ventricular dysfunction occurs, then
    symptoms and signs of right-sided ventricular
    heart failure may be present.

31
Physical examination
  • The patient with severe stenosis has a right
    ventricular lift on palpation of the precordium
  • The S I sound is usually normal and is followed
    by an opening click that becomes louder with
    expiration
  • The P2 sound becomes softer and is delayed as the
    severity of the stenosis increase
  • The characteristic murmur of pulmonic stenosis is
    a systolic ejection murmur heard best at the left
    upper sternal border, which increases with
    inspiration
  • A late-peaking murmur indicates more severe
    stenosis
  • A prominent jugular venous a wave and right-sided
    54 sound may also be present in patients with
    severe obstruction to right ventricular outflow

32
treatment
  • For asymptomatic patients with mild pulmonic
    stenosis, therapy is limited to endocarditis
    prophylaxis
  • Patients with moderate stenosis (peak gradient
    gt50mmHg) are likely to develop symptoms and
    require intervention over time and should be
    treated even in the absence of symptoms
  • Patients with severe obstruction (peak gradient
    gt80mmHg) also require intervention
  • In children and adults with isolated pulmonic
    stenosis , percutaneous balloon valvuloplasty is
    a suitable therapeutic option
  • Valve replacement is rarely necessary

33
Ebstein's a nomaly
  • A rare condition (0.50loo f patients with
    congenital heart disease) characterized by apical
    displacement of the tricuspid valve into the
    right ventricle
  • The basal portion of the right ventricle forms
    part of the right atrium and leaves a small
    functional right ventricle
  • The tricuspid leaflets are often dysplastic and
    may partially adhere to the interventricular
    septum or right ventricular free wall
  • The degree of right ventricular dysfunction
    depends on the size of the functioning right
    ventricle and the severity of the tricuspid
    regurgitation
  • Frequently develops in adulthood
  • A patient foramen ovale or ostium secundum ASD is
    present in more than 50 of patients and may
    result in right-to-left shunt flow as right
    atrial pressure increases
  • Supraventricular arrhythmias are common

34
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35
Diseases of Aorta
  • Coarctation of the aorta
  • Coarrctation of the aorta is a firotic narrowing
    of the aortic lumen

36
Diagnosis
  • The diagnosis of coarctation may be made by
    tow-dimensional and Doppler echocardiography.

37
  • Repair in adults is recommended at at the time of
    diagnosis.Surgical repair is standard in many
    center,although primary percutaneous treatment
    with balloon angioplasty.

38
Patent Ductus Arteriosus
  • A persistent communication between the aorta and
    pulmonary artery is the result of the failure of
    the ductus arteriosus to close.

39
Diagnosis And Treatment
  • Tow_dimensional and doppller
  • Percutaneous device closure
  • Surgery for the PDA that is too large

40
TETRALOGY OF FALLOT
41
DEFINITION
  • Tetralogy of fallot is the most common cyanotic
    congenital heart lesion in adults and represent
    10 of all congenital heart defects

42
ETIOLOGY
  • Malalignment of the aorticopulmonary septum that
    divides the truncus arteriosus into the aorta and
    pulmonary artery during development.

43
FOUR COMPONENTS OF TETRALOGY
  • Overriding of the aorta in relation to the
    ventricular septum
  • Right ventricular outflow obstruction, which may
    be valvular
  • subvalvular
  • supravalvular
  • or combination of all threes
  • Membranous VSD
  • Right ventricular hypertrophy

44
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46
  • The presence of right ventricular outflow
    obstruction is protective,preventing volume and
    pressure overload of the pulmonary
    circulation,which would result in fixed pulmonary
    hypertension.
  • If pulmonic stenosisi is mild, right - to - left
    shunt flow is minimal,and the patient remains
    acyanotic (Pink tetralogy)
  • The degree of right - to - left shunt flow
    depends on the degree of RV outflow obstruction.

47
  • Tetralogy may also be associated with ASD,
    muscular VSD, right aortic arch, and other
    coronary anomalies.
  • A chromosomal deletion (22q11) is found in 15 of
    cases, particulary in those whit associated
    anomalies.

48
TREATMENT
  • Surgical correction
  • Palliative surgery

49
Surgical correction
  • Usually performed during infancy or childhood
  • Involves relife of right ventricular obstruction
    and patch closure of the VSD

50
After reparative surgery
  • Residual polmunary stenosis or regurgitation
  • Aortic insuffiency
  • Residual VSD
  • Aneurysms of the right ventricular outflow tract
  • Sustained arrhythmias
  • supraventricular
  • ventricular

51
  • Prolongation of the QRS duration (to gt 180 ms) on
    the surface electrocardiographic study is a
    marker for increased risk for ventricular
    tachycardia and sudden death.

52
Palliative surgery
  • Performed in childhood to improve pulmonary blood
    flow
  • Creation of a shunt between the systemic and
    pulmonary circulation (e,g., subclavian artery to
    ipsilateral pulmonary artery blalock taussig
    shunt
  • Palliative surgery caused long-term palliaton of
    hypoxia.

53
Palliative surgery complications
  • Patient may outgrow their shunts, or the shunts
    may spontaneously close and may lead to
    progressive cyanosis.
  • If the shunt is too large, the increased volum of
    blood into the pulmonary circulation and left
    heart may result in pulmonary congestion and
    progress to irreversible pulmonary vascular
    obstruction.

54
Cardiovascular Disease
  • Congenital Heart Disease

55
Complete transposition of the great arteries
  • Known as D-transposition
  • 5-7 of congenital heart disease
  • The most common cyanotic congenital heart disease
    in the newborn
  • The aorta arises from the RV and the pulmonary
    artery arises from the left
  • 90 mortality rate in the first year of life
    without correction
  • Associated defects VSD, left ventricular outflow
    tract stenosis, coarctation of the aorta

56
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Procedures for D-TGA
  • Atrial switch procedure (e.g. Mustard or Senning
    procedures)
  • Arterial switch procedure (Jatene procedure)

58
Atrial switch procedure
Arterial switch procedure
D-TGA
59
Corrected transposition of the great arteries
  • Known as L-transposition
  • The anatomic RV lies on the left and ejects
    oxygenated blood into an anteriorly displaced
    aorta and the anatomic LV lies on the right and
    ejects blood into the posteriorly displaced
    pulmonary artery
  • Not cyanotic
  • Quite uncommon, 0.5 of congenital heart disease
  • Associated anomalies atrioventricular nodal
    block, VSD and Ebsteins anomaly

60
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Single ventricle
  • When a single ventricle supports both the
    pulmonary and systemic circulation. As such,
    tricuspid atresia, double-inlet left ventricle
    with VSD, and large atrioventricular septal
    defect
  • Poor prognosis without repair, median survival of
    14 years of age
  • Cyanosis and functional limitations

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The Fontan procedure
64
Eisenmenger Syndrome
  • In 1958,Paul Wood used the term Eisenmenger
    complex to describe the combination of a large
    VSD with systemic pulmonary pressures and
    reversed or bidirectional shunt

65
  • A VSD , a PDA , or an ASD could all rezult in
    Eisenmenger phisiologic characteristic.
  • The defect size generali exceeds 1.5 diameter for
    VSD , with about half the diameter for PDA
    and twice that diameter for ASD .

66
  • Complicaitions include hyperviscosity syndrome ,
    hemorahge or thrombosis , arrythmias and sudden
    death ,endocarditis and cerebral abscenss
    ,verticular dysfunction , hyperuricemia and goat
    and renal impairment , among others.

67
  • Patient with Eisenmenger syndrome have achived a
    delicat balance and management of such patients
    should respect that balance.prevention of
    complicationsis the preferred sterategy.
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