Congenital Heart

Disease in Neonatus

Putri Sari Wardani 17710010

Ririn Riwayati 17710019
Annisa Fitri Shaza Nabella 17710026
Indra Mukti Pratama 17710036

1
Congenital Heart Disease
Epidemiology of CHD
Incidence:
 8/100 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
 Gender differences in type of CHD

2
3
 Evaluation of the Infant or Child with
Congenital Heart Disease
1. History and Physical Examination
 Acyanotic
 Cyanotic
2. Chest roentgenogram
 Increase
 Normal Pulmonary blood flow/vascular
 Decreased marking

3. Electrocardiogram
 Right, left, biventricular hypertrophy
 Final diagnosis: confirmed by
echocahrdiography, CT or MRI or cardiac
catheterization.
4
I. Acyanotic Congenital Heart Disease
1. Left to Right Shunt Lesions:
a. Atrial Septal Defect
 Defect occur in any portion of the atrium
 Ostium secundum (at fossa ovalis)
 Ostium primum (ECD) (lower atrial septum)
 Sinus venosus (upper atrial septum)
 Pathophysiology:
 Left to right shunt
 Transatrial in OS & SV
 Transatrial & transventricular in OP

5
6
 Clinical  Diagnosis:
Manifestations:  Clinical
 Most are asymptomatic  CXR:
 Right ventricular lift  Right V&A enlargement
 Large pulmonary artery
 Wide & fixed split of 2nd
 ed pulm. vascularity
heart sound
 ECG:
 Systolic ejection  Volume overload
murmur  Right axis deviation
 Mid-diastolic murmur at  Minor right ventricular
tricuspid area conduction delay
 Holosystolic murmur at  Echocardiography
mitral area in OP (ECD)  Catheterization
7
 Echocardiographic findings in
secundum atrial septal defect (ASD)

8
 Complication:
 Pulmonary Hypertension
 Eismenger syndrome
 Treatment:
 Surgery for all symptomatic&asymptomatic ASD
with shunt ratio ≥ 2:1.
 Prognosis:
 Small to moderate sized ASD detected in term
infants may close spontaneously.
 Secundum ASD are well tolerated during
childhood.

9
b. Ventricular septal defect:
 The most common cardiac malformation
 Defect occur in any portion of the septum
- Majority membranous
- Muscular (Single or Swiss-cheese defect
 Pathophysiology:
 Left to right shunt
 Restrictive if detect is small (0.5 cm2)
 Non-restrictive if large defect (>1 cm2)

10
11
Clinical manifestation VSD:
 Small defect:  Large defect:
 Excessive pulmonary blood flow
 Most common
 Pulmonary hypertension
 Asymtomatic  Dyspnea, feeding difficulties,
 Loud, harsh poor growth, perspiration,
holosystolic murmur recurrent plum. infection,
at lower left sternal heart failure
border and frequently  Less harsh but more blowing
accompanied by a holosystolic murmur
thrill.  Accentuated 2nd heart
sound
 Mid-diastolic apical
murmur when shunt ratio
> 2:1

12
 Diagnosis:  Treatment:
 Clinical  Small defect:
 CXR: - Reassurance
 Cardiomegaly - Prophylaxis against IE
 Plethoric lung
 Large defect:
 ECG
- Medical treatment
 Echocardiography (control of CHF,
prevent IE and
development of
pulmonary HTN)
- Surgical repair
between 6-12 months.

13
A. Roentgenogram in patient with B. Three years after surgical
VSD with a large left to right closure of the defect, heart
shunt and pulmonary size is markedly decreased
hypertension, significant
and the pulmonary
cardiomegaly,pulmonary
overcirculation are evident.
vasvulature is normal.

14
 Complication:
 Infective endocarditis
 Recurrent lung infection
 Heart failure
 Pulmonary HTN
 Acquired pulmonary stenosis
 Aortic valve regurgitation
 Prognosis:
 30-50% small defects close by 2 yr of age
 Rarely moderate to large defects close

15
c. Patent ductus arteriosus:
 Functional closure soon after birth
 Aortic end of the ductus distal to the origin of
left subclavian artery and the other end at
bifurcation of pulmonary artery.
 Male to female ratio 1:2
 Pathology:
 Deficiency of mucoid endothelial layer &
muscular media in term infant.

16
 Pathophysiology
:
 Left to the right
shunt:
 Depend on size and
ratio of pulmonary
to systemic vascular
resistence.

17
Clinical manifestation PDA:
 Small PDA:  Large PDA:
 Asymtomatic.  Retardation of physical growth
may be major manifestation.
 Normal peripheral
 Bounding peripheral arterial
pulses.
pulses and wide pulse pressure
 Normal size of the during diastole.
heart.  Moderately or grossly enlarged.
 Continuous or machinery
murmur at 2nd left ICS.

18
 Diagnosis:  Treatment:
 Clinical  Surgical or catheter
 CXR: closure
 ECG  Small defect:
 Echocardiography - To prevent bacterial
endarteritis or other
late complication.
 Large defect:
- To treat heart failure or
prevent the
development of
pulmonary vascular
disease or both.

19
Transcatheter closure of small patent ductus
arteriosus using a coil.

20
 Complication:
 Infective endocarditis/endarteritis
 CHF
 Embolization
 Pulmonary HTN
 Prognosis:
 Small PDA (normal life)
 Large PDA (CHF).

21
II. Cyanotic Congenital Heart Disease
1. Cyantoic lesions with decreased
pulmonary blood flow
a. Tetralogy of Fallot:
 Obstruction to right ventricular outflow (pulmonary
stenosis).
 Malalignment type of ventricular septal defect
(VSD).
 Dextroposition of the aorta so it overrides the
ventricular septum, and
 Right ventricular hypertrophy.

22
 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

23
24
 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 murmur
 Delayed growth & development
 Single 2nd heart sound

25
 Diagnosis:  Treatment:
 CXR :  Severe outflow
 Narrow base & obstruction:
uplifted apex  Medical:
 A boot or wooden  PGE1 infusion
shoe  Prevent dehydration
 decreased pulmonary  Partial exchange
vascularity transfusion
 Right side aortic arch  Oral propanolol for tet
in 20% spells.
 ECG  Surgery:
 Echocardiography  Blalock-Taussig shunt
 Total correction

26
CXR 8 yr boy with tetrallogy of Echocardiogram in patient with tetrallogy
Fallot of Fallot
27
 Complication:
 Cerebral thrombosis (<2 years)
 Brain abscess
 Infective endocarditis
 Polycythemia
 CHF in pink TOF

28
b. Tricuspid atresia:
Pathophysiology:
 No outlet the right atrium to the right ventricel is
present
 Systemic venous return leaves the right atrium
and enters the left side of the heart, trough an
antrial septal defect.
 The physiology depend on the presence of other
congenital heart defect;

29
30
 Clinical  Diagnosis:
Manifestations:  EXR
 Cyanosis at borth  ECG
 Polycythemia  Echocardiography
 Easily fatiguability
 Exertional dyspnea.

31
 Treatment:  Complication:
 PGE1  Complication after
 Surgery: Fontan procedure:
 Aortico-pulmonary
  systemic venous
shunt pressure
 Bidirectional Glenn
 Fluid retention
shunt  Pleural or pericardial
 Modified Fontan effusion.
operation  Late complication:
 Superior or inferior
vena cava syndrome
 Hepatic cirrhosis, etc.

32
2. Cyantoic lesions with increased
pulmonary blood flow
a. Transposition of GA:
 D-TGA (uncorrected)
 Systemic venous return to right atriumNormal
 Pulmonary venous return to left atrium
 Aorta arises from right ventricle
 Pulmonary artery arises from left ventrikel
 D-TGA is more common in infants of diabetic
mothers and in males (3:1).
 D-TGA is accompanied by other cardiac defect and
associated with deletion of chromosome 22q11.

33
 Clinical  Diagnosis:
manifestation:  ECG:
 Cyanosis and tachypnea.  Right sided dominant
 Hypoxemia (depending on pattern.
the degree of atrial shunt)
 CXR:
 Precordial impulse normal
and parasternal heave
 Mild cardiomegaly,
may be present. narrow mediastinum
(egg-shaped heart),
 2nd heart sound is usually
normal to increased
single and loud, although
pulmonary blood flow.
it may be split.
 Soft systolic ejection  Echocardiography:
murmur may be noted at
midleft sternal border.

34
 Treatment:
 Infusion PGE1
 Rashkind ballon atrial septostomy (for hypoxic or
acidotic despite prostaglanding infusion in infant).
 Arterial switch (Jatene) procedures

35
 L-Transposition of GA:
 Right atrium is connected to the left ventricle and
the left atrium to the right ventricle.
 The great arteries are also transposed, with aorta
arising from the right ventricle and the pulmonary
artery from the left.
 Desaturated systemic venous return via vena cava
to a normal right atrium, from which is passes
through a bicuspid atrioventricular (mitral) valve
into right sided ventricle.

36
 Clinical  Diagnosis:
manifestation:  Clinical
 Depend on associated  CXR:
malformation.  Narrow mediastinum
 Increased pulmonary
blood flow
 ECG
 Echocardiography
 Treatment:
 PGE1 emergency
 Surgery

37
b. Truncus arteriosus:
 Single arterial trunk for both pulmonary &
systemic circulation.
 4 types depending the origin of pulmonary
artery.

38
39
 Clinical manifestation:
 In newborn: sign of heart  Diagnosis:
failure are usually absent,
murmur and minimal
 ECG
cyanosis.  CXR
 In 1-2 month: pulmonary  Echocardiography
blood flow begins to
become torrential and the
clinical picture is dominant  Treatment:
by heart failure, mild
cyanosis. Systolic ejection
 1st week of life:
murmur accompanied by anticongestive
thrill in left sternal border. medication, surgery
within 1st few month if
heart failure
symptoms worsen.
40
Echocardiography of truncus arteriosus

41
 Thankyou

42