4. CHD
Is most common congenital disorder in
newborns.
Prevalence is 0.8%
Most congenital defects are tolerated during
fetal period. This is due to parallel nature of
fetal circulation.
6. ETIOLOGY OF CHD
Most causes of congenital heart disease is
unknown. But it is thought to be multifactorial.
Genetic predisposition
Environmental stimuli
Chromosomal abnormalities includes trisomy
21,13, 18.
8. Evaluation of the Infant or Child with Congenital Heart
Disease
The initial evaluation for suspected congenital heart
disease involves a systematic approach with three
major components.
A. Congenital cardiac defects can be divided into two
major groups based on the presence or absence of
cyanosis, which can be determined by physical
examination aided by pulse oximetry.
Acyanotic
Cyanotic
B. These two groups can be further subdivided
according to whether the chest radiograph shows
evidence of:
increased
normal pulmonary vascular markings
9. Con’d
C. The electrocardiogram can be used to
determine whether :
Right
Left hypertrophy exists.
Biventricular
D. The final diagnosis is then confirmed
by echocardiography, CT or MRI, or
cardiac catheterization
10. I- Acyanotic Congenital Heart Disease(CHD)
Acyanotic congenital heart lesions can be classified
according to the predominant physiologic load that they
place on the heart.
1) lesions that cause a volume load
i) left-to-right shunt lesions.
ASD(atrial septal defect)
VSD(ventricular septal defect)
PDA(patent ductus arterious)
AVSD
Aorticopulmonary Window Defect
Ruptured Sinus of Valsalva Aneurysm
Coronary- Arteriovenous Fistula (Coronary-Cameral
Fistula)
12. 2) lesions that cause a pressure load (The
Obstructive Lesions) most commonly secondary to:
Ventricular outflow obstruction
Pulmonic valve stenosis
aortic valve stenosis
Narrowing of one of the great vessels
coarctation of the aorta
Rarely
MS(mitral stenosis)
TS (tricuspid stenosis)
The chest radiograph and electrocardiogram are useful
tools for differentiating between these major classes of
volume and pressure overload lesions
13. 1.1 Atrial Septal Defect(ASD)
4 types base on the portion of the atrial
septum defect
secundum(OS)- the most common
type
primum(OP)
sinus venosus(SV)
Coronary sinus defect
Less commonly, the atrial septum may be
nearly absent, with the creation of a
functional single atrium.
14. Pathophysiology.
Left-to-right shunting :- increased pul.
Blood flow
The degree of left-to-right shunting is
dependent on:
The size of the defect, compliance of the
right and left ventricles and , vascular
resistance in the pulmonary and systemic
circulations.
15.
16. Results in:
Enlargement of the right atrium and
ventricle
Dilatation of the pulmonary artery.
The left atrium may be enlarged
The left ventricle and aorta normal in
size.
17. CLINICAL MANIFESTATIONS.
In older child most often asymptomatic.
Even an extremely large ASD rarely
produces clinically evident heart failure
in childhood.
In younger children, subtle failure to
thrive may be present.
In older children, varying degrees of
exercise intolerance may be there.
18. Sign
Wide & fixed split of 2nd heart sound
Systolic ejection murmur
Mid-diostolic murmur at tricuspid area(TS)
Holosystolic murmur at mitral area in OP(MR)
20. .
TREATMENT
Surgical or transcatheter device closure is
advised for :
All symptomatic patients
Asymptomatic patients with qp : qs ratio of
at least 2 : 1.
The timing for elective closure is usually
after the 1st yr and before entry into school.
mortality rate is <1% in open surgery.
22. PROGNOSIS
ASDs detected in term infants may close
spontaneously.
Secundum ASDs are well tolerated
during childhood.
The results after surgical or device
closure in children with moderate to
large shunts are excellent.
23. 1.2 Ventricular Septal Defect ( VSD)
VSD is the most common cardiac malformation .
Defects may occur in any of the 3 portion of the
ventricular septum.
Membranous - commonest
Supracristal- less common
Muscular
single or
Multiple (Swiss cheese septum).
24. PATHOPHYSIOLOGY
Left-to-right shunt :- increased pul. Blood flow.
Restrictive VSDs(usually <0.5 cm2)
Right ventricular pressure is normal
The size of the defect limits the magnitude of the
shunt
Nonrestrictive VSDs (usually >1.0 cm2)
Right and left ventricular pressure is
equalized
The direction of shunting is determined by the
25.
26. The magnitude of intracardiac shunts is usually
described by the qp : qs ratio.
If the left-to-right shunt is small (qp : qs <1.75 : 1),
The cardiac chambers are not appreciably
enlarged and the pulmonary vascular bed
is probably normal.
If the shunt is large (qp : qs >2 : 1),
Left atrial and ventricular volume overload
occurs
The main pulmonary artery, left atrium,
27. CLINICAL MANIFESTATIONS
The clinical findings of patients with a VSD vary
according to the size of the defect and
pulmonary blood flow and pressure.
Most patients are asymptomatic
Characteristically, a loud, harsh, or
blowing holosystolic murmur is
present and heard best over the lower left
sternal border(TR)
It is frequently accompanied by a thrill.
28. • Large VSDs
– Dyspnea
– Feeding difficulties
– Poor growth
– Profuse perspiration
– Recurrent pulmonary infections
– Heart failure in early infancy
Physical finding
– Prominence of the left precordium is common
– Laterally displaced apical impulse
– The holosystolic murmur of a large VSD is generally less harsh
than that of a small VSD and more blowing .
– Early systolic murmur at pulmonic area(PS)
– A mid-diastolic, low-pitched rumble at the apex(shunt ratio
>2:1)(MS)
– The pulmonic component of the 2nd heart sound may be
increased
30. TREATMENT
Medical management
To control heart failure and
Prevent the development of pulmonary vascular disease.
Prophylaxsis for infective endocarditis
Surgical management
Indications for surgical closure :
Patients at any age with large defects in whom clinical
symptoms and failure to thrive cannot be controlled
medically
Infants between 6 and 12 mo of age with large defects
associated with pulmonary hypertension, even if the
symptoms are controlled by medication
Patients older than 24 mo with a qp : qs ratio greater than 2
: 1.
32. PROGNOSIS.
The natural course of a VSD depends to a large
degree on the size of the defect.
A significant number (30–50%) of small defects
close spontaneously, most frequently during the 1st
2 yr of life.
Small muscular VSDs are more likely to close (up to
80%) than membranous VSDs are (up to 35%).
The vast majority of defects that close do so before
the age of 4 yr, although spontaneous closure has
been reported in adults
The results of primary surgical repair are excellent
33. 1.3 Patient Ductus Arteriosus
During fetal life most of pulmonary arterial blood
is shunted through ductus arteriosus into the aorta.
Functional closure soon after birth
Male to female ratio 1:2
34.
35. Pathophysiology
Lt to Rt shunt
Reversal of shunt
Clinical manifestation
Asymptomatic in small ductus,
36. Physical signs in large PDA
– Wide pulse pressure( runnoff blood)
– Bounding peripheral arterial pulses.
– The apical impulse is prominent and, with cardiac
enlargement
– A thrill, maximal in the 2nd left interspace
– The classic continuous murmur is localized to the 2nd left
intercostal space or radiate down the left sternal border , to the left
clavicle or toward the apex
– The murmur radiates along the pulmonary arteries and is often well
heard over the left back.
– A low-pitched mitral mid-diastolic murmur may be audible at the
38. Prognosis
Small PDA lead normal life
Large PDA presentes with CHF
Treatment
Medical Rx
Surgical closure
39. COMPLICATIONS
Heart failure
Pulmonary hypertension (eisenmenger
syndrome)
Infective endarteritis.
Pulmonary or systemic emboli may occur
40. Occur at any site from the arch of aorta to iliac
bifurcation
98% juxta ductal
Pathogenesis
can occur as discrete juxtaductal obstruction or as
tubular hypoplasia of transverse arch.
In the presence of arch hypoplasia right heart out put
passes through the ductus into descending aorta resulting
in differential cyanosis.
Blood pressure is elevated in vessels proximal to the
1.4 Coarctation of the Aorta
41.
42. Clinical manifestation
Hypertension → mechanical obstruction
→ renal
Different cyanosis → pale upper extr.
→ Cyanosed lower extr.
Classic signs
- Disparity in pulse & BP
- Radio-femoral delay
- Systolic M at LMSB & inter-scapular area
-Systolic or continuous murmurs may be heard
over the left and right sides of the chest laterally and
posteriorly.
- A palpable thrill can occasionally be
appreciated in the back.
LMSB:-left middle sternal border
43. Diagnosis
- Clinical
- CXR - cardiomegaly & pulm. congestion
- Notching of ribs
- ECG
- Echocardiography
Prognosis
Untreated cases may loss his life by 20-40 years
Complications
- I/E
- Aneurysms
-hypertensive encephalopathy
Treatment
- Medical - IV PGE1 in neonatal age
- Surgery
DDx of cardiomegally -VSD
- PDA
- Coarctation of aorta
s- Transposition of great artery
44. 1.5 Pulmonary Vascular Disease
(Eisenmenger syndrome)
The triad of
i. systemic-to-pulmonary communication,
ii. pulmonary arterial disease, and
iii. cyanosis is called Eisenmenger syndrome.
Occur in shunt lesions
VSD - mainly
ASD
PDA
Reversal of shunt due to pulm. HTN
→ Cyanosis
Heart and lung transplantation, or lung transplantation with repair
of the cardiac defect, is appropriate in some patients with severe
disease.
45. II. Cyanotic Congenital Heart Disease
1.Cyanotic lesions with decreased
pulmonary blood flow
2.Cyanotic CHD With increased pulmonary
blood flow
46. 1. Cyanotic lesions with decreased
pulmonary blood flow
1.1 Tetralogy of Fallot
Consists: 1. Rt ventricular outflow obst.
2. Ventricular septal defect
3. Dextroposition of the aorta
4. Right ventricular hypertrophy
47. Pathophysiology
- Outflow obstruction;
- Hypertrophy of subpulmonic muscle
- Normal or small pulmonary valve annulus
- Rarely pulmonary atresia
- VSD - Non-restrictive, located just below
aortic valve
- Aortic arch is on right side in 20%
- Right ventricular output shunts to
the aorta
48. Clinical Manifestation
Exertional dyspnea
Squatting position in walking children
Paroxysmal hyper cyanotic attacks
Occur during 1st 2 years
Delayed growth & development
50. Diagnosis
CXR - Narrow base & uplifted apex
- A boot or wooden shoe
- decreased pulm. vascularity
- Right side aortic
ECG
Echocardiography
Complication
- Cerebral thrombosis in < 2 years
- Brain abscess
- Infective endocarditis
- Polycythemia
- CHF
51. Treatment
Severe outflow obstruction
- Medical px - PGE1 infusion
- Prevent dehydration
- Partial exchange transfusion
- Surgery
- Total correction
52.
53. 2.Cyanotic CHD With increased pulmonary
blood flow
2.1 Transposition of GA
a. D -TGA (uncorrected)
- Systemic venous return to Rt atrium
Normal
- Pulmonary venous return to Lt atrium
- Aorta arises from Right ventricle
- Pulm. artery arises from Lt vent.
Pathology
54. Survival is with associated - patent foramen ovale
or
- VSD or
- PDA
Clinical Manifestations
- Tachypnea & cyanosis at birth
- Rarely congestive heart failure
55.
56. b. L. TGA (corrected transposition)
Systemic VR to normally positioned Rt atrium
Through bicuspid (Mitral) valve
Right sided left ventricle
Pulmo. artery pulm. venous return
Normally positioned Lt atrium
Through tricuspid valve
Left sided Right ventricle Aorta
