3. ATRIAL SEPTAL DEFECT
One third of adult congenital heart disease
Types:
1. Ostium secondum at the region of fossa ovalis
(75%)
2. Ostium primum (15%)
3. Sinus venosus (10%)
4.
5. Magnitude of shunt depends on the size of ASD,
ventricular diastolic properties and relative
impedence of pulmonary and systemic circulations
Increased pulmonary blood flow and dilatation of the
atria, right ventricle, and pulmonary arteries
Develops pulmonary arterial hypertension
Reversal of the shunt
6. Clinical features:
fatigue or dyspnea on exertion.
supraventricular arrhythmias, right heart failure,
paradoxical embolism, or recurrent pulmonary infections
may prompt the patient to seek medical attention
Prominent right ventricular impulse
Palpable pulmonary artery pulsation
Systolic thrill
S1 normal or split
S2 Widely split and fixed
A systolic ejection murmur, audible in the second left
intercostal space, peaks in mid-systole, ends before the
second heart sound (increased flow across pulmonary
valve)
7. Mid diastolic rumbling murmur at 4th left intercostal
space ( flow across tricuspid valve)
As pulmonary hypertension develops..
Pulmonary and tricuspid flow murmurs decreases
P2 loud
Systolic ejection sound at pulmonary area
Diastolic murmur of pulmonary regurgitation
….Cyanosis .. Clubbing– Reversal of shunt
8. ECG:
Right axis deviation, rSr pattern right precordial
leads
Atrial ectopics or first degree heart block- Sinus
venosus ASD
Ostium primum: Left axis deviation, incomplete
RBBB
Atrial arrhythmias, including atrial fibrillation and
supraventricular tachycardia, may appear
9. On chest radiography,
Prominent RA, RV
Pulmonary arteries and a peripheral pulmonary
vascular pattern of “shunt vascularity” (in which the
small pulmonary arteries are especially well
visualized in the periphery of both lungs).
10. ECHO:
Dilated pulmonary artery, RA,RV with paradoxical
ventricular motion
Better visualization with color flow imaging, contrast
echo
Transesophagial echo: sinus venosus defects
Catheterization :to determine the magnitude and
direction of shunting, as well as whether pulmonary
hypertension is present and, if so, its severity
11. treatment
ASD with significant left to right shunting ie
pulmonary to systemic flow ratio > 1.5:1
supportive care
Treatment of arrythmia and infections
Infective endocarditis prophylaxis not indicated
12. VENTRICULAR SEPTAL DEFECT
Most common congenital cardiac abnormality in
infants and children
Types: 1. perimembreanous (most common)
2. Muscular – inlet, tabecular, outlet
13.
14. Natural history
Depending on size and location
Small muscular VSD- spontaneous closure
Large– unlikely
Initial L—R shunt
Increase in pulmonary vascular resistance
Pulmonary hypertension and shunt reversal
15. Clinical features
PSM over 3d or 4th ICS
Mid diastolic flow murmur across mitral valve
Features of pulmonary hypertension develops as
vascular resistance increases
ECG: Biventricular enlargement with or withour
RBBB
X- ray: LV enlargement with pulmonary plethora
16. Treatment
Surgical closure
If pulmonary vascular resistance is 1/3 or less than
systemic, progression of pulmonary HTN is unusual
Operation is indicated when pulmonary to systemic
flow ratio >1.5:1 without high pulmonary resistance (
Pulmonary vascular resistance <2/3 of systemic
resistance)
18. L– R shunt
10% of congenital heart disease
Functional closure occurs soon after birth;
anatomical closure is complete in 2-3 weeks after
birth
More seen in prematurity, perinatal hypoxemia, high
altitude, maternal rubella
19. Clinical features
bounding peripheral arterial pulses
widened pulse pressure
hyperdynamic left ventricular impulse
A continuous “machinery” murmur, audible in the
second left anterior intercostal space, begins shortly
after the first heart sound, peaks in intensity at or
immediately after the second heart sound (thereby
obscuring it), and declines in intensity during
diastole.
Flow murmurs through mitral or aortic valves
20. Pulmonary vascular resistance increases …
Features of pulmonary hypertension
Shunt reversal…
Differential cyanosis and clubbing
21.
22. ECG: Left atrial enlargement, Left ventricular
enlargement (volume overload), Right ventricular
enlargement ( pressure overload)
Confirmation by 2D echo and doppler studies
23. Complications:
Calcification and rupture
Infective endocarditis
Pulmonary hypertension and Eisenmenger’s
Treatment: Surgical ligation
24. COARCTATION OF AORTA
Narrowing of aortic lumen
Upper part of body is overperfused
Lower part is underperfused
Types: 1. pre ductal
2. Post ductal (most common)
Extensive collaterals to lower part of body develops
25.
26.
27. Associations: gonadal dysgenesis (e.g., Turner’s
syndrome), bicuspid aortic valve, ventricular septal
defect, patent ductus arteriosus, mitral stenosis or
regurgitation, or aneurysms of the circle of Willis
28. Clinical features:
Upper limb BP> lower limb
Feeble LL pulses
Radio-femoral delay
Systolic thrill in suprasternal notch
Left ventricular enlargement
Systolic ejection click
ESM at left sternal border and back
Continuous murmur over interscapular or
infrascapular areas
29. ECG: LVH
X- Ray: Dilated left subclavian and ascending aorta
“3” Sign: Indentation of aorta at the site of
coarctation and pre and post stenotic dialatation
Notching of 3d to 9th ribs due to inferior rib erosion
by dilated collaterals
Conformation: 2D echo and doppler, TEE, MR or CT
angiography
34. TETRALOGY OF FALLOT
Malaligned VSD
Right ventricular outflow obstruction
Aortic override of VSD
Right ventricular hypertophy due to right ventricular
response to aortic pressure via large VSD
35.
36. R-L shunt
Cyanosis from birth
Severity is proportionate to Right ventricular outflow
obstruction
decrease in systemic vascular resistance increases
right-to-left shunting, whereas an increase in
systemic resistance decreases right-to-left shunting.
37. Clinical features
Cyanosis from birth
hypoxic “spells,” characterized by tachypnea
and hyperpnea, followed by worsening cyanosis
and, in some cases, loss of consciousness,
seizures, cerebrovascular accidents in infancy
dyspnea and limited tolerance of exercise
erythrocytosis, hyperviscosity, abnormalities of
hemostasis, cerebral abscesses or stroke
endocarditis
38. Right ventricular lift or tap
Systolic thrill pulmonary area
Single S1
Aortic ejection click may be heared
Systolic ejection murmur, audible along the left
sternal border, is caused by the obstruction of right
ventricular outflow
Intensity is inversely proportional to severity
39. ECG: RVH, Right axis deviation
X- ray: “bootshaped,” with an upturned right
ventricular apex and a concave main pulmonary
arterial segment
Arterial oxygen desaturation
Compensatory erythrocytosis
Confirmation: 2D echo, Angiography
40.
41. Treatment:
Complete surgical correction: closure of the
ventricular septal defect and relief of right ventricular
outflow obstruction
At present, palliative shunting or balloon pulmonary
valvuloplasty is performed only in severely ill infants
for whom complete repair is unsuitable
42. Complications:
Polycythemia and hyperviscosity
Cerebral abscess
Infective endocarditis
CCF occurs if infective endocarditis, anemia,
pregnancy, systemic hypertension, AR, Stenosis of
aortic valve, Pulmonary atresia
43. Ebstein’s Anomaly
Downward displacement of tricuspid valve into RV
due to anomalous attachment of TV leaflets
Hypoplastic RV
Progressive cyanosis (right to left shunt)
Tricuspid regurgitation and RV dysfunction
Paroxysmal atrial tachyarrythmias
Atrio- ventricular byepass tracts– WPW syndrome