angela Christi Amores Congenital Heart Disease 0508 of live births incidence is higher in stillborns 34 abortuses 1025 and premature infants about 2 diagnosis is established by 1 wk of age in 4050 of patients with congenital heart disease and by 1 mo of age in 50 ID: 750741
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Slide1
Congenital Diseases
Dr. Meg-
angela
Christi
AmoresSlide2
Congenital Heart Disease
0.5-0.8% of live
births
incidence is higher in stillborns (3-4%),
abortuses
(10-25%), and premature infants (about 2
%)
diagnosis is established by 1 wk of age in 40-50% of patients with congenital heart disease and by 1 mo of age in 50-60%Slide3
Relative frequency of Major Congenital lesions
Ventricular
septal
defect25-30
Atrial
septal
defect (
secundum
)6-8
Patent
ductus
arteriosus6-8
Coarctation
of aorta5-7
Tetralogy
of Fallot5-7
Pulmonary
valve stenosis5-7
Aortic
valve stenosis4-7 Slide4
Relative Frequency of Major Congenital lesions
d-Transposition of great arteries3-5
Hypoplastic
left ventricle1-3
Hypoplastic
right ventricle1-3
Truncus
arteriosus1-2
Total anomalous pulmonary venous return1-2
Tricuspid atresia1-2
Single ventricle1-2
Double-outlet right ventricle1-2 Others5-10 Slide5
Fetal circulationSlide6
Congenital Disease
Most congenital defects are well tolerated in the
fetus
because of the parallel nature of the
fetal
circulation
only after birth when the
fetal
pathways (
ductus
arteriosus
and foramen
ovale
) are closed that the full hemodynamic impact of an anatomic abnormality becomes apparent Slide7
Etiology
Cause is unknown
There
is progress
in identifying
genetic basis of many congenital heart
lesions
small percentage - related to chromosomal abnormalities, in particular,
trisomy
21, 13, and 18 and Turner
syndrome
2-4% -associated with known environmental or adverse maternal conditions and
teratogenic
influences, including maternal diabetes mellitus,
phenylketonuria
, or systemic lupus
erythematosusSlide8
diabetic mothers are five times more likely to have congenital cardiovascular malformations
most congenital heart disease is still relegated to a
multifactorial
inheritance
pattern
Fetal
echocardiography improves the rate of detection Slide9
2 major groups
Acyanotic
Congenital heart lesions
Cyanotic Congenital heart lesionsSlide10
Acyanotic
Congenital heart lesions
Increased volume load:
ASD (
atrial
septal
defect)
VSD (ventricular
septal
defect)
AV
septal
defects
PDA (patent
ductus
arteriosus
)
Increased pressure load:
valvular
pulmonic
stenosis
valvular
aortic
stenosis
coarctation
of the aorta Slide11
Cyanotic Congenital heart lesions
Decreased
Pulmonary Blood Flow - obstruction to pulmonary blood flow and a pathway by which systemic venous blood can shunt from right to left and enter the systemic circulation
tricuspid
atresia
T
etralogy
of
Fallot
single ventricle with pulmonary
stenosis
Increased Pulmonary Blood flow
Transposition of the great
vessels
Total
anomalous pulmonary venous
return
Truncus
arteriosusSlide12
PDA (patent ductus
arteriosus
)Slide13
Pathophysiology
blood shunts left to right through the
ductus
from the aorta to the pulmonary
artery
pulmonary artery pressure may be elevated to systemic levels during both systole and
diastole
risk for the development of pulmonary vascular disease if left
unoperatedSlide14
Manifestations
small patent
ductus
does not usually have any symptoms
large PDA will result in heart failure
Cardiac enlargement
Classic continuous murmur (machinery-like)Slide15
Diagnosis
ECG
Left ventricular hypertrophy
Xray
prominent pulmonary artery with increased intrapulmonary vascular
markings
2D echocardiography
left
atrial
and left ventricular dimensions are
increased
Visualization of the patent
ductusSlide16
Treatment
Irrespective of
age, patients with PDA require surgical or catheter
closure
should not be unduly postponed after adequate medical therapy for cardiac failure has been
instituted
thoracoscopic
techniques to minimize scarring and reduce postoperative
discomfortSlide17
Other congenital lesions - acyanoticSlide18
Other congenital lesions - acyanoticSlide19
Other congenital lesions - acyanoticSlide20
Other congenital lesions -
cyanoticSlide21
Other congenital lesions -
cyanotic