Congenital Heart Disease (CHD)........ppt

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1. Congenital Heart Disease (CHD)
Tadesse Getahun, MD, Pediatrician and Pediatric Cardiologist

2. Congenital Heart Disease (CHD)
Epidemiology of CHD
Incidence - 8/1000 live births
- 3-4/100 still born
- 2/100 premature infants excluding PDA
- 10-25/100 abortuses
Most congenital defects are well tolerated during fetal life.
Etiology - Unknown in most cases
- Genetic factors - single gene defect
- Chromosomal abnormality.
- Environmental factors

3. CHD …
Clues for Evaluation of an Infant with suspected CHD
1. On History and Physical Examination
color)
 Acyanotic
 Cyanotic
2. Chest roentgenogram
 Normal
 Increased/Plethora pulmonary blood flow
 Decreased/Oligemia

4. CHD …
3.Electrocardiogram
- Right
- Left hypertrophy
- Biventricular
Final diagnosis - Precordial examination
- Echocardiography

5. CHD…
Relative Frequency of Congenital Heart Lesions
LesionsLesions % of all Lesions% of all Lesions
- Ventricular septal defect- Ventricular septal defect 25-3025-30
- A trial septal defect (Secundum)- A trial septal defect (Secundum) 6-86-8
- Patent ductus arteriosus- Patent ductus arteriosus 6-86-8
- Coarctation of aorta- Coarctation of aorta 5-75-7
- Tetralogy of Fallot- Tetralogy of Fallot 5-75-7
- Pulomnary Valve Sterosis- Pulomnary Valve Sterosis 5-75-7
- Aortic Valve Stenosis- Aortic Valve Stenosis 4-74-7
- d-Transposition of great arteries- d-Transposition of great arteries 3-53-5

6. I. Acyanotic Congenital Heart Diseases
1.Left to Right Shunt Lesions
1.1 Atrial Septal Defect
 Defect occur in any portion of the
atrium
- Ostium secundum
- Ostium primum (ECD)
- Sinus venosus
Pathophysiology
 Left to right shunt
- Transatrial in OS & SV
- Transatrial & transventricular in OP

7. Acyanotic CHD…
Clinical Manifestations
 Most are asymptomatic
 Right ventricular lift
 Wide & fixed split of 2nd heart sound
 Systolic ejection murmur
 Mid-diostolic murmur at tricuspid area
 Holosystolic murmur at mitral area in OP
(ECD)

8. Acyanotic CHD…
Diagnosis
 Clinical
 CXR - Right. V & A enlargement
- Large pulm. artery
- ↑ed pulm. vascularity
 ECG - volume overload, Rt ventricle & atrium
 Echocardiography
 Catheterization
Prognosis - Well tolerated
Complications - pulm. Hypertension Eismenger syndrome
Treatment
 Surgery-for all symptomatic
 Asymptomatic patients with shunt ratio > 2:1
 Device closure

15. Acyanotic CHD…
1.2 Ventricular Septal Defect
 The most common cardiac malformation
 Defect occur in any portion of the septum
- Majority membranous
- Swiss-cheese defect
Pathophysiology
 Lt to Rt shunt
 Restrictive if defect is small (0.5cm2)
 Non-restrictive - large defect (> 1cm2)
- Right vent. Pressure equalized

16. Acyanotic CHD…
Clinical Manifestation
 Small defects with trivial Lt to Rt Shunt
- Most common
- Asymptomatic
- Loud, harsh holosystolic M at LLSB
 Large defects
- Excessive pulmonary blood flow
- Pulmonary hypertension
- Dyspnea, feeding difficulties, poor growth,
perspiration, recurrent plum. infection, heart failure
- Less harsh but more blowing holosystolic
- Accentuated 2nd heart ground
- Mid-diastolic apical M when shunt ratio > 2:1

17. Acyanotic CHD…
Diagnosis
- Clinical
- CXR - Cardiomegaly
- Plethoric lung
- ECG
- Echocardiography
Prognosis
- 30-50% small defects close
- Rarely moderate to large defects close

18. Acyanotic CHD…
Complications
- Infective endocarditis
- Recurrent lung infection
- Heart failure
- Pulmonary HTN
- Acquired pulmonary stenosis
Treatment
- Small defects - reassurance
- Prophylaxis against IE
- Large defects - medical treatment (control of CHF,
prevent IE, prevent development of p. venous HTN)
- Surgical repair between 6-12m
-Device closure

22. Acyanotic CHD…
1.3 Patent Ductus Arteriosus
 Functional closure soon after birth
 Aortic end of the ductus distal to the
origin of subclavian artery and the other
end of bifurcation of pulmonary artery.
 Male to female ratio 1:2
Pathology - Deficiency mucoid endothelial
layer & muscular or media in term infant.

23. Acyanotic CHD…
Pathophysiology
Lt to Rt shunt - size
- ratio of pulm. to systemic resistance
Reversal of shunt
Clinical Manifestation
Asymptomatic in small ductus
Wide pulse pressure
Bounding pulse Large
Continuous or machinery
M at 2nd Left ICS

24. Acyanotic CHD…
Diagnosis
- Clinical
- Chest X-ray
- ECG
- Echocardiography
Prognosis
- Small PDA - normal life
- Large PDA - CHF
Complications
- Infective Endocarditis/Endarteritis
- CHF
- Embolization
- Pulmonary HTN
Treatment - Medical
- Device closure
- Surgical closure

27. Acyanotic CHD…
2.Obstructive Lesions
2.1 Pulmonic Stenosis - 4 types
- Valvular
- Infundibular
- Supra valvular
- Peripheral
Pathophysiology
- Rt outlet obstruction → Pressure work
↓
Rt vent. hyperthropy

28. Acyanotic CHD…
Clinical Manifestation
- Mild to moderate - asymptomatic
- Critical stenosis
- Systolic ejection murmur
- Heart failure in neonates & infants
- Rarely cyanosis

29. Acyanotic CHD…
Diagnosis
- Clinical
- CXR - Rt vent. hypertrophy
- reduced pulm. blood flow
- ECG
- Echocardiography
Prognosis - good in mild to moderate
Complications - CHF in severe Ps
- rarely IE
Treatment - vavular PS - ballon valvoplasty
- surgery

31. Acyanotic CHD…
2.2 Aortic Stenosis
-Valvular - the commonest
-Supra valvular
-Subvalvular (subaortic)

32. Acyanotic CHD…
Clinical Manifestation
 Mild stenosis
- Normal pulse & apical impulse
- Systolic ejection M
- Normal to enlarged heart size
 Critical stenosis
- Left ventricular failure
- pulm. edema, cardiomegaly
- Weak peripheral pulses
- Weak systolic M
- Paradoxical split M 2nd heart sound

33. Acyanotic CHD…
Diagnosis
- Clinical
- CXR
- ECG
- Echocardiography
- Graded exercise testing
Prognosis is good for mild to moderate
Treatment
- Balloon valvoplasty
- Surgical

37. Acyanotic CHD…
2.3Coarctation of the 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

38. Acyanotic CHD…
Clinical Manifestation
 Hypertension → mechanical obstruction
→ renal
 Differential cyanosis → pale upper extr.
→ cyanosed lower extr.
 Classic signs
- Disparty in pulse & BP
- Radio-femoral delay
- Systolic M at LMSB & inter-scapular area

39. Acyanotic CHD…
Diagnosis
- Clinical
- CXR - cardiomegaly & pulm. congestion
- Notching of ribs
- ECG
- Echocardiography
Prognosis – Untreated cases succumb by 20-40 years
Complications - CVA
- I/E
- Aneurysms
Treatment
- Medical - IV PGE
1 in neonatal age
- Surgery

44. II. Cyanotic Congenital Heart Disease
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

45. Cyanotic CHD…
Pathophysiology
- Outflow obstruction
- Hypertrophy of subpulmonic muslce
- 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

46. Cyanotic CHD…
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
- Delayed growth & development
- Single 2nd heart sound

47. Cyanotic CHD…
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

48. Cyanotic CHD…
Treatment
Severe outflow obstruction
- Medical Px - PGE
1
infusion
- Prevent dehydration
- Partial exchange transfusion
- Oral propranolol for tet spells
- Surgery - Blalock Taussig
- Total correction

52. Cyanotic CHD…
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
Left ventricle
systemic circulation
Aorta
pulmonic circulation

- Hypoplastic right ventricle (PDA)

53. Cyanotic CHD…
Clinical Manifestation
- Cyanosis at birth
- Respiratory distress
- Single 2nd heart sound
- No murmur
Diagnosis
- CXR
- ECG
- Echocardiography
Treatment - PGE
1
- Surgery

55. Cyanotic CHD…
1.3Tricuspid atresia
- No outlet from Right atrium to Left vent.
- Systemic venous return

Rt atrium
Lt atrium

Left ventricule
systemic Pulmonic
(VSD, PDA)

56. Cyanotic CHD…
Clinical Manifestation
- Cyanosis at birth
- Polycythemia
- Easily fatiquability
- Exertional dyspnea
Diagnosis
- EXR -Pulm. Under circulation
- ECG -Lt axis deviation & Lt vent. hpertrophy
- Echocardiography

57. Cyanotic CHD…
Treatment
- PGE
1
- Surgery - Aortico - pulmonary Shunt
- Bidirectional Glenn shunt
- Modified Fontan operation

59. Cyanotic CHD…
1.4Ebstein 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

60. Cyanotic CHD…
Clinical Manifestations
- Easly fatiguability
- Cyanosis
- Dysrhythmia
- Rt to Lt shunt through formen ovale
- Holosystolic M at tricuspid area
- Heart failure
Diagnosis
- CXR - box shaped heart
- ECG - Right BBB
- Superior axis deviation
Treatment
- PGE
1
- Surgery

64. Cyanotic CHD…
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

65. Cyanotic CHD…
*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

68. Cyanotic CHD…
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

69. Cyanotic CHD…
 Discordant atrio-ventricular relation (ventricular
inversion)
 Transposition of great arteries
Clinical Manifestation
Depends on associated malformation

70. Cyanotic CHD…
Diagnosis
- Clinical
- CXR - Cardiomegaly
- Narrow mediastinum
- Increased pulmonary blood flow
- ECG
- Echocardiography
Treatment
- PGE
1
- emergency
- Surgery

71. Cyanotic CHD…
2.2 Truncus arteriosus
- Single arterial trunk for both pulm. &
systemic circ.
- 4 types depending the origin of pulmonary artery
Clinical Manifestation
-Cyanosis
-CHF at 2-3rd m
-Systolic ejection m
Treatment - surgery

74. Cyanotic CHD…
2.3 Total Anomalous Pulm. Venous return
- Pulm. drainage into systemic vein

77. Cyanotic CHD…
2.4Single Ventricle
- No interventricular septum

78. Cyanotic CHD…
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

81. THANK YOU