Transcript Cardiovascular Disease

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
• 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.
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
• The isolated pulmonary and systemic
circulations:
•①D: the ductus arteriosus and the foramen
ovale.
•②L :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.
• 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
Septal defects
ATRIAL SEPTAI DEFECTS
(ASD)
• ASDs are some of the most common congenital
defects, representing 10%to 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 defect:60%,defect in fossa ovalis
• 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.
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
• 5%Irreversible pulmonary vascular obstruction
resulting in right-to-left shunting and cyanosis
(Eisenmenger syndrome)
• Stroke or transient ischemic attack in order to
paradoxical emboli
Physical exam
• Parasternal RV impulse
• Widely and fixed split S2
• Ejection murmur across pulmonic
Diagnosis
• Two dimensional and color Doppler
echocardiography
• Transesophagial echocardiography
• Electerocardiography
• MRI
• Cardiac catheterization
• Angiography
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.
Ventricular septal defect (VSD)
• VSD is a common congenital abnormality in
newborns.(1 in 500 births)
VSD’s classification
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Supracristal vsd
Perimembranous vsd(most common:80%)
Atrioventricular canal vsd
Muscular vsd
• 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.
Eisenmenger syndrome
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Pulmonary vascular obstruction
Pulmonary artery HTN
Reversal of interventricular shunt
Systemic desaturation
Cyanosis
clubbing
Physical examination
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Hyperdynamic precordium
Palpable thrill along the left sternal border
Holosystolic murmur
Loud p2
Diagnosis
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Tow dimensional echocardiography
Doppler echocardiography
MRI
CT scan
Treatment:
• Sutures
• Prosthetic patch
• Percutaneous device closure(muscular)
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.
Bicuspid aortic stenosis:
as with acquired aortic stenosis: chest
pain,syncope,congestive heart failure.
Other complications: sudden death & infective
endocaditis.
Physical examination:
• Ejection quality murmur at the left sternal
border.
• Early systolic ejection click
• Decrescendo diastolic murmur of aortic
insufficiency
Diagnosis
• Two –dimensional echocardiography
• Doppler echocardiography
Treatment:
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Percutaneous valvuloplasty
Aortic valve replacement
Antibiotic prophylaxis
Oral hygiene
Sub aortic stenosis:
• Firs diagnosed in adulthood
• A discrete,fibrous diaghragm
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-vasculardevelopemental abnormalities.
PULMONIC VALVE STENOSIS
• Pulmonic valve stenosis is the most common
cause of obstruction to right ventricular
outflow
• usually occurs as an isolated congenital
lesion
• Fusion of the pulmonary leaflets creates the pressureoverloaded 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.
Physical examination
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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
treatment
• For asymptomatic patients with mild pulmonic stenosis,
therapy is limited to endocarditis prophylaxis
• Patients with moderate stenosis (peak gradient
>50mmHg) 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
>80mmHg) also require intervention
• In children and adults with isolated pulmonic stenosis ,
percutaneous balloon valvuloplasty is a suitable
therapeutic option
• Valve replacement is rarely necessary
Ebstein's a nomaly
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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
Diseases of Aorta
• Coarctation of the aorta:
• Coarrctation of the aorta is a firotic narrowing
of the aortic lumen
Diagnosis
The diagnosis of coarctation may be made by •
tow-dimensional and Doppler
echocardiography.
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.
Patent Ductus Arteriosus
A persistent communication between the •
aorta and pulmonary artery is the result of the
failure of the ductus arteriosus to close.
Diagnosis And Treatment
Tow_dimensional and doppller •
Percutaneous device closure •
Surgery for the PDA that is too large •
TETRALOGY OF FALLOT
DEFINITION
• Tetralogy of fallot is the most common
cyanotic congenital heart lesion in adults and
represent 10% of all congenital heart defects
ETIOLOGY
• Malalignment of the aorticopulmonary septum
that divides the truncus arteriosus into the
aorta and pulmonary artery during
development.
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
• 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.
• 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.
TREATMENT
• Surgical correction
• Palliative surgery
Surgical correction
• Usually performed during infancy or
childhood
• Involves relife of right ventricular obstruction
and patch closure of the VSD
After reparative surgery
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Residual polmunary stenosis or regurgitation
Aortic insuffiency
Residual VSD
Aneurysms of the right ventricular outflow
tract
• Sustained arrhythmias
supraventricular
ventricular
• Prolongation of the QRS duration (to > 180 ms) on the
surface electrocardiographic study is a marker for
increased risk for ventricular tachycardia and sudden
death.
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.
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.
Cardiovascular Disease
Congenital Heart Disease
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
Procedures for D-TGA
• Atrial switch procedure (e.g. Mustard or
Senning procedures)
• Arterial switch procedure (Jatene procedure)
D-TGA
Atrial switch procedure
Arterial switch procedure
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 Ebstein’s anomaly
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
The Fontan procedure
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
• 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 .
• 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.
• Patient with Eisenmenger syndrome have
achived a delicat balance and management
of such patients should respect that
balance.prevention of complicationsis the
preferred sterategy.