4. PREVALENCE.
In live births (0.5–0.8%)
In premature infants 2% excluding patent [PDA]
In stillborns (3–4%)
In spontaneous abortuses (10–25%)
This does not include mitral valve prolapse, PDA
of preterm infants, and bicuspid aortic valves
(present in 1–2% of adults).
and this incidence increases to 2–6% for a 2nd
pregnancy after birth of a child with CHD or if a
parent is affected.
Recurrence is highly depend on the type of lesion in
the 1st child.
5.
6. …
• Most congenital defects are well tolerated in the fetus
because of the parallel nature of the fetal circulation.
• Even the most severe cardiac defects (hypoplastic left
heart syndrome) can usually be well compensated for
by the fetal circulation.
• One notable exception is the Episten Anomaly
• In utero heart failure often (nonimmune hydrops
fetalis) may occur.
7. Etiology
• Maternal
seizure disorder w/intake of medications
lithium given for depression
uncontrolled IDDM
Lupus
maternal Rubella
• Paternal
• Chromosomal trisomes(chromosome 21,18,13)
,turners syndrome
• For majority cause is unkown
8. Evaluation
by pulse oximetry.
Acyanotic ‘’ Hyperoxia test’’
Cyanotic
By chest radiograph
increased
Normal
decreased pulmonary vascular markings
– byelectrocardiogram can be used to determine
whether :
• right
• left hypertrophy exists.
• biventricular
The final diagnosis is then confirmed by
echocardiography, CT ,MRI or cardiac catheterization.
9. Acyanotic Congenital Heart
Disease(CHD)
1) lesions that cause a volume load
A) left-to-right shunt lesions.
• ASD
• VSD
• PDA
• AVSD
• PAPVR
• Aorticopulmonary Window Defect
• Ruptured Sinus of Valsalva Aneurysm
• Coronary- Arteriovenous Fistula (Coronary-Cameral
Fistula)
11. 2) lesions that cause a pressure load (The
Obstructive Lesions)
• ventricular outflow obstruction
– Pulmonic valve stenosis
– aortic valve stenosis
• narrowing of one of the great vessels
– coarctation of the aorta
Rarely
• MS
• TS
The CXR and ECG are useful tools for
differentiating between volume and pressure
overload lesions.
12. ASD
• can occur in any portion of the atrial septum
– 4 types
• secundum(OS)- the most common type
• primum(OP)-ECD
• sinus venosus(SV)
• Coronary sinus defect
• Less commonly, the atrial septum may be nearly absent, with the
creation of a functional single atrium.
• An isolated valve-incompetent patent foramen ovale (PFO) is a
common echocardiographic finding during infancy. It is usually
of no hemodynamic significance and is not considered as ASD
13.
14. PATHOPHYSIOLOGY.
– left-to-right shunting increased pul. blood flow
At atrial level in Os and SV
Both at atrial and ventricular level in OP
– The degree of left-to-right shunting is dependent on:
the size of the defect
the relative compliance of the right and left ventricles
and the relative vascular resistance in the pulmonary and
systemic circulations.
– The paucity of symptoms in infants with ASDs is related
to the structure of the right ventricle in early life when
its muscular wall is thick and less compliant, thus
limiting the left-to-right shunt.
15. Clinical manifestations
• Usually asymptomatic
• Failure to thrive
• Varying degree of exercise intolerance
• Child tires easily when playing
• Infant tires easily when feeding
• Fatigue
• Sweating
• Tachycardia ,tachypnea
16. The physical findings.
mild left precordial bulge.
A right ventricular systolic lift is generally palpable at
the left sternal border.
A loud 1st heart sound and sometimes a pulmonic
ejection click can be heard.
In most patients, the 2nd heart sound is
characteristically widely split and fixed.
A systolic ejection murmur is best heard at the left
middle and upper sternal border.
A short, rumbling mid-diastolic murmur at the tricuspid
valve is often audible at the lower left sternal border.
Holosystolic murmur at the apex in OP(ECD)
18. VSD
• VSD is the most common cardiac malformation .
• Defects may occur in any portion of the ventricular septum.
• 3 types
– membranous - commonest
– Supracristal- less common
– Muscular
• single or
• multiple (Swiss cheese septum).
19.
20. • 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 and shunt magnitude are
determined by the ratio of pulmonary to systemic
vascular resistance
21. 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
right ventricular volume overload and pulmonary arterial
hypertension.
The main pulmonary artery, left atrium, and left ventricle are
enlarged.
When the ratio of pulmonary to systemic
resistance approaches 1 : 1,
the shunt becomes bidirectional
the signs of heart failure abate, and the patient becomes cyanotic
(Eisenmenger physiology)
22.
23. • CLINICAL MANIFESTATIONS
– The clinical findings of patients with a VSD
vary according to the size of the defect and
pulmonary blood flow and pressure.
• Small VSDs with trivial left-to-right shunts and normal
pulmonary arterial pressure are the most common.
– These patients are asymptomatic
– Characteristically, a loud, harsh, or blowing
holosystolic murmur is present and heard best over
the lower left sternal border
– it is frequently accompanied by a thrill.
24. • Large VSDs
– dyspnea
– feeding difficulties
– poor growth
– profuse perspiration
– recurrent pulmonary infections
– cardiac failure in early infancy
Physical finding
– Prominence of the left precordium is common
– palpable parasternal lift
– laterally displaced apical impulse
– apical thrust, and a systolic thrill
– 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
– a mid-diastolic, low-pitched rumble at the apex(shunt ratio >2:1)
– The pulmonic component of the 2nd heart sound may be increased
27. Patent Ductus Arteriosus(PDA)
• Functional closure of the ductus occurs soon after birth
• The aortic end of the ductus is just distal to the origin of the left
subclavian artery, and the ductus enters the pulmonary artery at
its bifurcation.
• Female patients with PDA outnumber males 2 : 1.
• PDA is associated with maternal rubella infection during early
pregnancy.
• It is a common problem in premature infants, where it can cause
severe hemodynamic derangements and several major sequelae
30. Con’d
• CLINICAL MANIFESTATIONS.
– A small patent ductus does not usually have
any symptoms.
– A large PDA will result in:
• heart failure.
• Retardation of physical growth may be a major
manifestation in infants with large shunts
31. physical signs in large PDA
– wide pulse pressure( runnoff blood)
– bounding peripheral arterial pulses.
– The apical impulse is prominent and, with cardiac enlargement, is
heaving.
– 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.
– When pulmonary vascular resistance is increased, the diastolic
component of the murmur may be less prominent or absent.
– a low-pitched mitral mid-diastolic murmur may be audible at the
apex
– Splitting of S2
32. • DIAGNOSIS.
– Clinical
– Chest x-ray
– ECG
– ECHO
– catheterization
• TREATMENT.
– Medical=indomethacin
– Surgical
• Irrespective of age, patients with PDA require surgical or catheter
closure.
• preferably before 1 yr of age
33. • COMPLICATIONS
– Heart failure
– Pulmonary hypertension (Eisenmenger
syndrome)
– Infective endarteritis.
– Pulmonary or systemic emboli may occur
– Rare complications include
• aneurysmal dilatation of the pulmonary artery or the ductus
• calcification of the ductus
• noninfective thrombosis of the ductus with embolization
• paradoxical emboli
35. Coarctation of aorta
• Occur at any site from the arch of aorta to iliac bifurcation
• 98% juxta ductal
Pathogenesis
• In utero arch hypoplasia
Rt heart output passes through the ductus
36.
37.
38. Clinical Manifestation
Hypertension → mechanical obstruction
→ renal
Differential 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
intercostal spaces on the back.
39. Diagnosis
- Clinical
- CXR - cardiomegaly & pulm. congestion
- Notching of ribs
- ECG
- Echocardiography
Prognosis – Untreated cases succumb by 20-40 years
Complications - CVA(cerebrovascular accident)
- I/E
- Aneurysms
Treatment
- Medical - IV PGE1 in neonatal age
- Surgery
40. Pulmonary Vascular Disease (Eisenmenger
syndrome)
- Occur in shunt lesions
VSD - mainly
ASD
PDA
- Reversal of shunt due to pulm. HTN
→ Cyanosis
41. 1. Cyanotic lesions with decreased
pulmonary blood flow
1.1 Tetra logy of Fallot
Consists: 1. Rt ventricular outflow obst.
2. Ventricular septal defect
3. Dextroposition of the aorta
4. Right ventricular hypertrophy
42.
43.
44. Pathophysiology
- Outflow obstruction to right ventricle
- Hypertrophy of subpulmonic muscle
- Normal or small pulmonary valve annulus
- Rarely pulmonary atresia
- VSD - Non-restrictive, located just below
aortic valve
- Aortic arch is right side in 20%
- Right ventricular output shunts to the aorta
When obstruction to right ventricular outflow
A)mild to moderate=pink
B)severe=cyanosis
45. Clinical Manifestation
- Rarely pink TOF - in the absence of
obstruction
- Cyanosis
- Clubbing
- Squatting position in walking children
- Paroxysmal hypercyanotic attacks
occur during 1st 2 years
- Systolic ejection M at pulmonic area
- Delayed growth & development
- Single 2nd heart sound
46. Diagnosis
CXR - Narrow base & uplifted apex
- A boot or wooden shoe
- decreased pulm. vascularity
- Right side aortic arch in 20%
ECG
Echocardiography
Complication
- Cerebral thrombosis - in < 2 years
- Brain abscess
- Infective endocarditis
- Polycythemia
- CHF in pink TOF
47.
48.
49. Treatment
Severe outflow obstruction
- Medical Px - PGE1 infusion
- Prevent dehydration
- Partial exchange transfusion
-iron supplementation
- Oral propranolol for tet spells
- Surgery - Blalock Taussig
- Total correction
50. 1.2Pulmonary Atresia
- With VSD - Extreme form
of TOF
- Without VSD - No egress of
blood from Rt vent.
- Shunt through
foramen ovale to Lt atrium
Leftventricle
systemic circulation
Aorta
pulmonic circulation(PDA)
51. Clinical Manifestation
- Cyanosis at birth
- Respiratory distress
- Single 2nd heart sound
- No murmur
Diagnosis
- CXR
- ECG
- Echocardiography
Treatment - PGE1
- Surgery
52. 1.3 Tricuspid atresia
- No outlet from Right atrium to right vent.
- Systemic venous return
Rt atrium
Lt atrium
Left ventricle
systemic Pulmonic
(VSD, PDA)
54. 1.4 Ebstein Anomaly of the tricuspid valve
- Down ward displacement of the tricuspid
valve
- Right ventricle with two parts
- atrialized
- normal ventricular myocardium
- Abnormal tricuspid valve
- Huge Rt atrium
- Tricuspid regurgitation
- Compromised Rt ventricular function
55. Clinical Manifestations
- Easily fatigability
- Cyanosis
- Dysrhythmia
- Rt to Lt shunt through foramen ovale
- Holosystolic M at tricuspid area
- Heart failure
Diagnosis
- CXR - box shaped heart
- ECG - Right BBB
- Superior axis deviation
Treatment
- PGE1
- Surgery
56.
57. 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 patholology
- Pulm. artery arises from Lt vent.
58. *Systemic & Pulmonary Circulations Consists of
two parallel circuits
*Survival is with associated - patent foramen
ovale or
- VSD or
- PDA
Clinical Manifestations
- Tachypnea & cyanosis at birth
- Rarely congestive heart failure
59.
60. 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
61. Discordant atrio-ventricular relation
(ventricular inversion)
Transposition of great arteries
Clinical Manifestation
Depends on associated malformation
63. 2.2 Truncus arteriosus
- Single arterial trunk for
both pulm. & systemic
circulation
- 4 types depending the
origin of pulmonary
artery
Clinical Manifestation
_ Cyanosis
_CHF at 2-3rd month
_Systolic ejection m .
Treatment - surgery
66. 2.5 Hypoplastic Left Heart Syndrome
- Under development of Lt Side of the heart
- Atretic aortic or mitral orifice
- Non functional Lt ventricle
- Hypoplasia of ascending aorta
Right ventricle supplies both pulm. &
systemic circulation
