CASE PRESENTATION

CONGENITAL HEART DISEASE IN CHILDREN

SUPERVISOR
dr. Ulynar Marpaung, Sp.A

CREATED BY
Mutammima Rizqiyani
1102014173

Departement of Pediatric

Bhayangkara Tk.I Raden Said Sukanto Hospital

Faculty of Medicine YARSI University

Periode 16 April s.d. 3 Juli 2018

 Table of Contents

Case Report ........................................................................................................... 3

Identity Of Patient ............................................................................................. 3

Personal History ................................................................................................ 3

Physical Examination ........................................................................................ 7

Supporting Examination................................................................................. 11

Working Diagnosis .......................................................................................... 12

Management .................................................................................................... 12

Prognosis .......................................................................................................... 12

Follow Up ......................................................................................................... 12

Literature Review ............................................................................................... 15

Congenital Heart Disease ................................................................................... 15

Atrial Septal Defect ......................................................................................... 17

Ventricular Septal Defect ............................................................................... 19

Patent Ductus Arteriosus ................................................................................ 24

Tetralogy Of Fallot .......................................................................................... 27

References ............................................................................................................ 30

2
 CASE REPORT
CONGENITAL HEART DISEASE

Identity of Patient
Name : Child A
Birth Date : April 18th 2013
Age : 5 years 1 month
Gender : Female
Address : Jln. Kelapa dua asrama gegana
Nationality : Indonesia
Religion : Islam
Date of admission : Mei 13th 2018
Date of examination : Mei 15th 2018

Father Mother

Name Mr. S Mrs. N

Age 38 years old 31 years old

Job Brigadir Housewife

Nationality Javanese Javanese

Religion Islam Islam

Education Bachelor Bachelor

Address Jln. Kelapa dua asrama gegana

Personal History
The anamnesis was taken on Mei 13th 2018 by alloanamnesis from patient’s mother.
 Chief complain : Fever since 2 day before admission to the hospital
 Additional complains : Stomachache, vomitus and diarrhea

3
History Of Present Ilness
A 5 years 1 month child came to Raden Said Sukanto Police Center Hospital
emergency room with fever since 2 day before admission the hospital. Fever is felt
continuously with the highest temperature of 39,9oC. The patient has been admitted
to the clinic and given paracetamol medicine, the fever drops for a while but then
rises again.

The patient also complained of abdominal pain in all parts of the stomach.
Besides the patient vomiting as much as 4 times per day. vomiting occurs after the
patient eats

Patients also complain of diarrhea as much as 5 times per day. Soft stool,
there is mucus, there is dregs and acid odor

When the patient is 4 months old, the patient's mother brought the patient to
a private doctor because the patient often sleep snoring. after examined, the patient
was diagnose Congenital Heart Disease (Ventricle septal defect).

History Of Past Illness

Pharyngitis/Tonsilitis -
Bronchitis -
Pneumonia -
Morbilli -
Pertussis -
Varicella -
Diphteria -
Malaria -
Polio -
Enteritis -
Bacillary Dysentry -
Amoeba Dysentry -
Diarrhea -
Thypoid -

4
 DHF +
Worms -
Surgery -
Brain Concussion -
Fracture -
Drug Reaction -

Allergic History

 The patient didn’t have cow’s milk allergy

 The patient didn’t have asthma, allergic rhinitis, and atopic dermatitis
 The patient didn’t have allergic to medicine
 The patient didn’t have allergic to dust, pollen, etc

Birth History
Mother’s Pregnancy History
The mother routinely checked her pregnancy to the doctor in the hospital. She
denied any problem noted during her pregnancy. She took vitamins routinely given.
Child’s Birth History
 Labor : Hospital
 Birth attendants : Doctor
 Mode of delivery : Pervaginam
 Gestation : 38 weeks
 Infant state : Healthy
 Birth weight : 2700 grams
 Body length : 51 cm
 According to the mother, the baby started to cry and the baby's skin is red.

Development History
 First dentition: 6 months
 Psychomotor development
 Head Up : 1 month old
 Smile : 1 month old

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  Laughing : 1- 2 month old
 Slant : 5 months old
 Speech Initation : 5 months old
 Prone Position : 6 months old
 Food Self : 6 months old
 Sitting : 8 months old
 Crawling : 9 months old

 Mental Status: Normal

 Conclusion: Good motor development status

Feeding History

- Breast milk : Exclusively 6 months

- Formula milk : None were given
- Baby biscuit : Milna and cerelac
- Fruit and vegetables : Banana and papaya

Immunization History
Immunization Frequency Time
BCG 1 time 1 month old
Hepatitis B 3 times 0, 1, 6 months old
DPT 3 times 2, 4, 6 months old
Polio 4 times 0, 2, 4, 6 months old
Measles 1 times 9 months

Family History
 Patient’s both parents were married when they were 18 years old and 25
years old, and this is their first marriage
 There are not any significant illnesses or chronic illnesses in the family
declared
 Born died : ( - )
 Child dies : ( - )

6
  Miscarriage : ( - )

History of Disease in Other Family Members / Around the House

There is no one living around their home known for having the same condition as
the patient.

Sosial and Economic History

 The patient lived at the house with size 10 m x 8 m together with father and
mother and two brothers.
 There are 1 door at the front side, 1 toilet near the kitchen and 3 rooms, in
which 1 room is the bedroom of three of them and 1 room is for her brothers.
There are 4 windows inside the house. The windows are occasionally
opened during the day.
Home ownership : private
Home condition : clean, good ventilation, good water
condition
Environment : densely populated
 Hygiene:
o The patient’s mother changes her clothes everyday with clean
clothes.
o Bed sheets changed every two weeks.

Physical Examination
A. General Status

- General condition : Mild ill

- Awareness : Apatis
- Pulse : 130x/min, regular, full, strong.
- Breathing rate : 26x/min
- Temperature : 36.7oC (per axilla)
- Saturation O2 : 98%

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 B. Antropometry Status

- Weight : 18 kilograms
- Length : 113 cm

Nutritional Status based NCHS (National Center for Health Statistics) year 2000:
WFA (Weight for Age): 18/18 x 100 % = 100 %
HFA (Height for Age): 113/107 x 100 % = 105 %
WFH (Weight for Height): 18/19,5 x 100 % = 92 %

8
Head to Toe Examination
 Head
Normocephaly, hair (black, rare distribution or almost bald, not easily
removed) sign of trauma (-)
 Eyes
Icteric sclera -/-, pale conjunctiva -/-, hyperaemia conjunctiva -/- ,
lacrimation -/-, sunken eyes -/-, pupils cant be examined/3mm isokor, direct
and indirect light response +/+ and +/+

 Ears
AD: Normal shape, no wound, no bleeding, secretion or serumen
AS: Normal shape, no wound, no bleeding, secretion or serumen
 Nose
Normal shape, midline septum, secretion -/-
 Mouth

 Lips: moist
 Teeth: no caries
 Mucous: moist
 Tongue: no dirty, normal
 Tonsils: T1/T1, no hyperemia
 Pharynx: hyperemia (-)

 Neck
Lymph node enlargement (-), scrofuloderma (-)
 Thorax :
i. Inspection : symmetric when breathing , retraction (-), ictus cordis is not
visible
ii. Palpation : mass (-), tactile fremitus -/-
iii. Percussion : sonor on both of lungs
iv. Auscultation :
1. Cor : regular S1-S2, holosystolic murmur (+), gallop (-)
2. Pulmo : vesicular +/+, Wheezing -/- , Rhonchi -/-

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  Abdomen :
i. Inspection : Convex, epigastric retraction (-), there is no a widening of
the veins, no spider nevi
ii. Palpation : supple, liver and spleen not palpable, fluid wave (-),
abdominal mass (-)
iii. Percussion : The entire field of tympanic abdomen, shifting dullness (-)
iv. Auscultation: normal bowel sound, bruit (-)

 Vertebra : There are no scoliosis, kyphosis, and lordosis, no mass

along the vertebral line
 Extrimities : warm, capillary refill time < 2 seconds, edema(-)
 Skin : Good turgor.

C. Neurological Examination

Meningeal Sign

Nuchal rigidity (-)

Kernig sign (-)

Lasegue sign (-)

Brudzinski I (-)

Brudzinski II (-)

Autonom Examination

Defecation Normal
Urination Normal ( 3-4 times daily )
Sweating Normal

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 D. Supporting Examination
Routine complete blood count

Mei 13th 2018

Hematology Results Normal Value

Haemoglobin 10,5 g/dL 13-16 g/dL

Leukocytes 5,000 /µL 5,000 – 10,000/µL

Hematocrits 32 % 40 – 48 %

Trombocytes 182,000/ µL 150,000 – 400,000/µL

Rontgen Thorax result (14 Mei 2018)

- Cor tampak membesar

- Sinuses dan diafragma normal
- Dilatasi RA-RV
- Hili normal
- Corakan paru bertambah
- Minimal infiltrate di perihiler dextra
- Skeletal dan soft tissue dalam batas normal

Conclusion : Bronkopneumonia dan suspek kardiomegali

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 E. Working Diagnosis
 CHD (VSD)
 Broncopneumonia

F. Management
- IVFD RL 1400 cc / day
- Paracetamol syr 4x1 cth
- Cetirizin syr 1x5 cc
- Lacto B 3x1 sach
- Zink 1x20 mg

G. Prognosis
 Quo ad vitam : dubia ad bonam
 Quo ad functionam : dubia ad malam
 Quo ad sanationam : dubia ad bonam

A. FOLLOW UP Mei 13th 2018 - Mei 15th 2018

Mei 13th 2018. First day of hospitalization, 3rd day of illness

S Diarrhea (+),Stomachache (+) and Fever (-)

O General condition: Compos mentis.

Heart rate = 104 x/min
Respiratory rate = 24x/min
Temperature = 37.5˚C
Eye: anemic conjunctiva -/-,
Cardio : S1/S2, reguler, holosystolic murmur (+) , gallop (-)
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/-
Abdomen : epigastrium pain (+)
A GEA
CHD

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 P - IVFD RL 1400 cc / day
- Paracetamol syr 4x1 cth
- Cetirizin syr 1x5 cc
- Lacto B 3x1 sach
- Zink 1x20 mg

Mei 14th 2018. Second day of hospitalization, 4 day of illness

S Diarrhea (-), Stomachache (+) and Fever (-)
O General condition: Compos Mentis
Heart rate = 110 x/min
Respiratory rate = 22 x/min
Temperature = 36.5˚C
Eye: anemic conjunctiva -/-
Cardio : S1/S2, reguler, holosystolic murmur (+), gallop (-)
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/-
Abdomen : epigastrium pain (+)
A GEA
CHD
P - IVFD KAEN 3B
- Paracetamol syr 4x1 cth
- Cetirizin syr 1x5 cc
- Lacto B 3x1 sach
- Zink 1x20 mg

Mei 15th 2018. Third day of hospitalization, 5 day of illness

S Diarrhea (-), Stomachache (+) and Fever (-)
O General condition: Compos Mentis
Heart rate = 100 x/min
Respiratory rate = 20 x/min
Temperature = 36.5˚C
Eye: anemic conjunctiva -/-

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 Cardio : S1/S2, reguler, holosystolic murmur (+),gallop (-)
Pulmonary : retraction (+) vesiculer +/+, rhonchi -/-, wheezing -/-
A GEA
CHD
P - IVFD KAEN 3B
- Paracetamol syr 4x1 cth
- Cetirizin syr 1x5 cc
- Lacto B 3x1 sach
- Zink 1x20 mg
- Antasida 3x1 cth

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II. LITERATURE REVIEW

CONGENITAL HEART DISEASE

Formation of Cardiac Septa

The major septa of heart are formed between the 27th and 37th day of
development. Septum formation in the heart begins with the formation of
endocardial cushion tissue in the atrioventricular canal (AV canal) and conotruncal
regions. Initially the primitive AV canal is a rounded aperture situated between the
left atrium and primitive left ventricle. Gradually the atrioventricular canal becomes
transverse and migrate to right, so that it lies beneath both atria and above both
ventricles.

The primitive AV canal is then divided into two channels by the formation
of a ventral and dorsal endocardial cushion, which grow toward each other through
the centre of AV canal, eventually meeting each other and thus dividing the canal.

These endocardial cushions help in the formation of atrial and ventricular

septa, the AV canal and the aorta and pulmonary channels. The septal surface of
endocardial cushion tissue differentiate further to form the septal leaflets of mitral
and tricuspid valves.

Abnormalities in endocardial cushion formation results in many cardiac

malformations, such as ASD, VSD, TGA and TOF. Since the conotruncal cushions
are rich in neural crest cells, the cardiac malformations can result in craniofacial
defects.

Septum Formation in the Common Atrium

At the end of 4th week of development a thin crescent-shaped septum grows

from the cranial aspect of primitive atrium and it represents the first portion of
septum primum. The opening between the lower rim of the septum primum and
endocardial cushion is called the ostium primum. This ostium primum gradually
gets obliterated when the growing septum primum fuses with endocardial cushion.

15
Before the septum primum fuses with endocardial cushions, several perforations
develop in its midportion that enlarge and coalesce to form a single large opening
the ostium secundum.

Another septum, the septum secundum then develops along the cranial and
posterior wall of right atrium. This septum, which also has a crescent-shaped
leading edge, extends midway along septum primum, and its crescent-shaped lower
margin form the foramen ovale. Usually the septum secundum covers the ostium
secundum. The septum formation is completed in 34 days.

Septum Formation in the Ventricle

The interventricular septum consists of a thick muscular part and a thin

membranous portion. By the end of the 4th week, the two primitive ventricles begin
to expand. The medial wall of the expanding ventricle become apposed and
gradually fuse together, thus forming the muscular interventricular septum. The
membranous portion of interventricular septum is formed by the outgrowth of
tissues from inferior endocardial cushion and the extending ridges of conus system.
This membranous septum occupies the top of muscular interventricular septum.
Thus the ventricular septum is closed between 38 and 45 days of age.

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 LEFT- TO- RIGHT SHUNT LESION

1. ATRIAL SEPTAL DEFECT

a) Prevalence

5 -10 % of all congenital heart disease (CHDs). Female preponderance

(male-to-female ratio of 1:2) [1]

b) Pathology and pathophysiology

There are three types of atrial septal defects (ASDs) [1]

Secundum ASD is in the central portion of the septum and is the most
common type (50-70% of ASDs). Primum ASD (or partial endocardial cushion
defect [ECD]) is in the lower part of the septum (30% of ASDs). Sinus venosus
defect is near the entrance of the superior vena cava (SVC) or inferior vena cava
(IVC) to the right atrium (RA) (about 10% of all ASDs).Partial anomalous
pulmonary venous return (PAPVR) is common with a sinus venous defect.

A left-to-right shunt (L-R Shunt) is present through the defect, with a

volume overload to the RA and right ventricle (RV) and an increase in pulmonary
blood flow.

c) Clinical manifestation
 The patients are usually asymptomatic.
 a widely split and fixed S2 and a grade 2 to 3/6 systolic ejection murmur
at the upper left sternal order (ULSB) are characteristic of moderate-size

17
 ASD. With a large L-R shunt a middiastolic rumble (resulting from
relative tricuspid stenosis [TS]) may be audible at the lower left sternal
border (LLSB). The typical auscultatory findings are usually absent in
infants or toddlers, even in those with a large defect, because the RV is
is poorly compliant.

 The ECG shows right axis deviation (RAD) (+90 to +180 degrees) and
mild right ventricular hypertrophy (RVH) or right bundle branch block
(RBBB) with a rsR’ pattern inV1.
 Chest X-ray (CXR) films show cardiomegaly (with right atrial
enlargement (RAE) and right ventricular enlargement (RVE), increase
pulmonary vascular marking (PVMs), and a prominent mainpulmonary
artery (MPA) segment.
 Two-dimensional echo shows the position and the size of the defect.
Cardiac catheterization is usually not necessary.
 Spontaneous closer of the defect occurs more than 80% of the time in
patients with defect 3-8mm (diagnose by echo) before 1,5 years of age.
An ASD with a diameter greater than 8 mm rarely close spontaneously.
The defect may reduce in size in some patients. If the defect is large and
left untreated, pulmonary hypertension developments in the third and
forth decade of life. Cerebbrovascular accident due to paradoxical
embolization through an ASD is possible.

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 d) Management

Medical

 Exercise restriction is not required.

 Nonsurgical closer of the defect using a catheter-delivered closer
device has become a preferred method, provided the indications are
met. These devices are applicable only to secundum ASD. The use of
the closure device may be indicated for a defect measuring ≥ 5 mm in
diameter (but less than 32 mm) with an adequate septal rim (4 mm),
and evidence of RA and RV volume overload. Among several device
available, the Amplatzer Septal Occluder appears to be most popular.
Following the device closure, the patients are placed on Aspirin 81 mg
per day for 6 months. Advantages of nonsurgical closure would include
a hospital stay of less than 24 hours, rapid recovery, and no residual
thoracotomy scar.

2. VENTRICULAR SEPTAL DEFECT

a) Prevalence.

Ventricular septal defect (VSD) is the most common form of CHD, accounting
for 15% to 20% of all CHDs, not including those occurring as part of cyanotic CHD.

b) Pathology and Pathophysiology

The ventricular septum consists of a small membranous septum and a larger

muscular septum. The muscular septum has three components: the inlet,
infundibular, and trabecular (or simply muscular) septa. A membranous VSD often
involves a varying amount of muscular septum adjacent to it (i.e., perimembranous
VSD). The perimembranous defect is more common (70%) than the trabecular (5%
to 20%), infundibular (5% to 7%), or inlet defects (5% to 8%). In Far Eastern
countries the infundibular defects account for about 30%.

The perimembranous VSD is frequently associated with patent ductus

arteriosus (PDA) and coarctation of aorta (COA). The VSD seen with tetralogy of

19
Fallot (TOF) is a large nonrestrictive perimembranous defect with extension into
the subpulmonary region. The inlet VSD is typically seen with endocardial cushion
defects.

 In subarterial infundibular or supracristal VSD the aortic valve may

prolapse through the VSD, with resulting aortic regurgitation (AR) and
reduction of the VSD shunt. The prolapse may occasionally occur with the
perimembranous VSD.
 In VSDs with small to moderate L-R shunts, volume overload is placed on
the left atrium (LA) and left ventricle (LV) (but not on the RV). With larger
defects the RV is also under volume and pressure overload, in addition to a
greater volume overload on the LA and LV. Pulmonary blood flow (PBF)
is increased to a varying degree depending on the size of the defect and the
pulmonary vascular resistance, with a large VSD, pulmonary hypertension
results. With a long-standing large VSD, pulmonary vascular obstructive
disease (PVOD) develops, with severe pulmonary hypertension and
cyanosis resulting from a right-to-left shunt (R-L shunt). At this stage,
surgical correction is nearly impossible.

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a) Clinical Manifestations
 Patients with small VSDs are asymptomatic, with normal growth and
development.
 Patients with large VSDs, delayed growth and development, repeated
pulmonary infections, CHF, and decreased exercise tolerance are relatively
common.
 Patients with PVOD (pulmonary vascular obstructive disease) , cyanosis
and a decreased level of activity may result.
 Patients with a small VSD, a grade 2 to 5/6 regurgitant systolic murmur
(holosystolic or less than holosystolic) maximally audible at the LLSB is
characteristic. A systolic thrill may be present at the LLSB.

 Patients with a large defect, an apical diastolic rumble is audible, which

represents a relative stenosis of the mitral valve due to large pulmonary
venous return to the LA. The S2 may split narrowly, and the intensity of the
P2 increases if pulmonary hypertension is present

 ECG findings: Small VSD, normal; moderate VSD, left ventricular

hypertrophy (LVH) and left atrial hypertrophy (LAH) (±); large VSD,
biventricular hypertrophy (BVH) and LAH (±); PVOD, pure RVH.
 CXR films reveal cardiomegaly of varying degrees with enlargement of the
LA, LV, and possibly the RV. PVMs are increased. The degree of
cardiomegaly and the increase in PVMs are directly related to the magnitude
of the L-R shunt. In PVOD the heart size is no longer enlarged and the MPA

21
 and the hilar pulmonary arteries are notably enlarged, but the peripheral
lung fields are ischemic.
 Two-dimensional echo studies provide accurate diagnosis of the position
and size of the VSD. LA and LV dimensions provide indirect assessment of
the magnitude of the shunt. Figure shows selected 2D echo views of the
ventricular septum, which helps locate the VSD position. The Doppler
studies of the pulmonary artery (PA), tricuspid regurgitation (TR) (if
present), and the VSD itself are useful in indirect assessment of RV and PA
pressures.

Spontaneous closure occurs in 30% to 40% of all VSDs, most often in small
trabecular VSDs, more frequently in small defects than in large defects, and more
often in the first year of life than thereafter. Large defects tend to become smaller
with age. Inlet and infundibular VSDs do not become smaller or close
spontaneously. CHF develops in infants with a large VSD but usually not until 6 or
8 weeks of age, when the PVR drops below a critical level. PVOD may begin to
develop as early as 6 to 12 months of age in patients with a large VSD.

b) Management
 Medical: Treatment of CHF with digitalis and diuretics
 No exercise restriction is required in the absence of pulmonary
hypertension.
 Surgical

22
Surgical Procedure

1. PA banding is rarely performed unless additional lesions make the complete

repair difficult.
2. Direct closure of the defect is performed under cardiopulmonary bypass
and/or deep hypothermia, preferably through an atrial approach rather than
through a right ventriculotomy.

Indications and timing

1. A significant L-R shunt with Qp/Qs of greater than 2:1 is an indication for
surgical closure. Surgery is not indicated for a small VSD with Qp/ Qs less
than 1.5:1.
2. Infants with CHF and growth retardation unresponsive to medical therapy
should be operated on at any age, including early infancy. Infants with a
large VSD and evidence of increasing PVR should be operated on as soon
as possible. Infants who respond to medical therapy may be operated on by
the age of 12 to 18 months. Asymptomatic children may be operated on
between 2 and 4 years of age.

Contraindications

1. PVR/SVR ratio 0.5 or greater or PVOD with a predominant R-L shunt.

Surgical approaches for special situations

1. VSD + large PDA: If PDA is large, the ductus alone may be closed in the
first 6 to 8 weeks, and the VSD may be closed later. If the VSD is large and
nonrestrictive, VSD should be closed early and the PDA ligated at the time
of VSD repair.
2. VSD + COA (Coarctation of the aorta): Controversies exist. One approach
is the repair of COA alone initially and the VSD is closed later if indicated.
Other options include COA repair and PA banding if the VSD appears large
or repair of both defects at the same time using one or two incisions.
3. VSD + AR (Aortic regurgitation) is usually associated with subarterial
infundibular (or supracristal) VSD and occasionally with perimembranous

23
 VSD. When AR is present, a prompt closure of the VSD is recommended,
even if the Qp/ Qs is less than 2:1, to abort progression of or to abolish AR.
Some centers close VSD if aortic prolapse is evident even in the absence of
AR.

Postsurgical follow-up

An office follow-up should be done every 1 to 2 years. The ECG shows

RBBB in 50% to 90% of the patients who had VSD repair through right
ventriculotomy and in up to 40% of patients who had repair through right atrial
approach.

3. PATENT DUCTUS ARTERIOSUS

a) Prevalence

5% to 10% of all CHDs, excluding those in premature infants. PDA in

premature infants is presented under a separate heading.

b) Pathology and Pathophysiology

 There is a persistent postnatal patency of a normal fetal structure
between the PA and the descending aorta.
 The magnitude of the L-R shunt is determined by the diameter and
length of the ductus and the level of PVR. With a long-standing large
ductus, pulmonary hypertension and PVOD may develop with an
eventual R-L shunt and cyanosis.
c) Clinical Manifestations
 Asymptomatic when the ductus is small.
 When the defect is large, signs of CHF may develop.
 A grade 1 to 4/6 continuous (machinery) murmur best audible at the
ULSB or left infraclavicular area is the hallmark of the condition. An
apical diastolic rumble is audible with a large-shunt PDA. Bounding
peripheral pulses with wide pulse pressure are present with a large-shunt
PDA.

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  ECG findings are similar to those of VSD:
- Normal or LVH in a small to moderate PDA
- BVH (Biventricular hypertrophy) in a large PDA
- RVH if PVOD develops.
 CXR findings are also similar to those of VSD:
- normal with a small shunt PDA.
- cardiomegaly (with LA and LV enlargement) with a large-shunt
PDA, and increased PVM are present.
- PDA with PVOD (Pulmonary vascular obstructive disease) the heart
size is normal, with a marked prominence of the MPA and hilar
vessels.
- The PDA can be directly imaged and its hemodynamic significance
determined by 2D echo and color flow Doppler examination.
Cardiac catheterization is usually not indicated in isolated PDA.
- CHF or recurrent pneumonia or both develop if the shunt is large.
Spontaneous closure of PDA usually does not occur in term infants

d) Differential Diagnosis.

The following conditions require differentiation from PDA because they

may present with a heart murmur similar to that of PDA and/or with bounding
pulses.

1. Coronary atrioventricular (AV) fistula (the murmur is audible over the

precordium, not at the ULSB)

25
 2. Systemic AV fistula (a wide pulse pressure with bounding pulse, CHF,
and a continuous murmur over the fistula [head or liver] are
characteristic)
3. Pulmonary AV fistula (a continuous murmur over the back, cyanosis,
and clubbing in the absence of cardiomegaly)
4. Venous hum (an innocent condition that disappears when the patient is
supine)
5. Murmurs of collaterals in patients with COA or TOF (audible in the
intercostal spaces)
e) Management

Medical

 No exercise restriction is required in the absence of pulmonary

hypertension.
 Indomethacin is ineffective in term infants with PDA.
 Catheter closure of the ductus may be employed. Small ductus 4 mm in
diameter are closed by Gianturco stainless coils and larger ones by
Amplatzer PDA device. An optimal candidate for the coil occlusion has
the ductus 2.5 mm or less in size, but the use of multiple coils can close
a ductus up to 5 mm. Amplatzer device may be used for PDAs ranging
in size from 4 to 10 mm (with 100% closure rate). Complications may
include residual leaks, pulmonary artery coil embolization, hemolysis,
left PA stenosis, aortic occlusion with the Amplatzer device, and
femoral vessel occlusion.

Surgical

Surgical closure is reserved for those patients in whom nonsurgical closure

technique is not considered applicable. Ligation and division through left
posterolateral thoracotomy without cardiopulmonary bypass is indicated for all
significant PDAs. Surgical mortality is near 0%. PVOD is a contraindication to
surgery. Repair through a smaller incision with video assisted thoracoscopy is
becoming popular.

26
Postsurgical Follow-up

No restriction of activity is indicated unless pulmonary hypertension

persists.

4. TETRALOGY OF FALLOT

With an incidence of 10% of all congenital heart defects, tetralogy of Fallot

is by far the most common form of cyanotic CHD a paediatrician comes across in

clinical practice. The four components of this malformation are:


1. Ventricular septal defect

2. Obstruction to the right ventricular outflow

3. Overriding of the aorta

4. Right ventricular hypertrophy.

The basic anomaly is the result of an anterior and cephalad deviation of the
septal insertion of the infundibular ventricular septum.

The degree of obstruction to the right ventricular outflow tract (RVOT) is

the major determinant of the clinical presentation. Lung blood flow is reduced, and
as a compensatory mechanism, bronchial and other collateral arteries establish an
alternative pulmonary circulation.

Few children with TOF remain asymptomatic or acyanotic. Most are

cyanotic from birth or develop cyanosis before age 1 year. Dyspnoea on exertion
presenting as feeding difficulty, sweating and tachypnoea, clubbing and
polycythemia is common. When resting after exertion, children typically adopt a
squatting posture. Hypercyanotic ‘spells’ begin to occur between 2 and 9 months
of age and can be fatal. They are characterized by hyperpnoea, increasing cyanosis
and can terminate in loss of consciousness, syncope and convulsions, even rarely
death.

On examination the child is underdeveloped and cyanotic. Clubbing is

prominent after the first year of life. The precordium is typically quiet and the heart

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is not hyperactive or enlarged. An early systolic ejection sound that is aortic in
origin may be heard at the lower left sternal border. The second heart sound is single
and the pulmonic component is not audible. A systolic ejection murmur is produced
by flow across the narrow RVOT. In severe RVOT narrowing or pulmonary atresia,
collateral channels may produce a continuous murmur. A loud continuous murmur
of a co-existing PDA may also be heard.

The ECG ordinarily shows RV hypertrophy. Chest X-ray reveals a boot-

shaped heart (couer en sabot) with prominence of the RV and a concavity in the
region of the under- developed RVOT. With a right aortic arch, the cardiac
silhouette is golf-shaped. Pulmonary vascular markings are diminished. Twenty-
five percent of patients have a right aortic arch. Echocardiography reveals aortic
enlargement and overriding of the interventricular septum. If the override is more
than 50%, it becomes a double outlet right ventricle (DORV). Pulmonary artery
size and site and severity of RVOT narrowing can be determined. Any co-existing
cardiac defects can also be identified.

Cardiac catheterization and angiography are necessary to confirm the

diagnosis, provide details of any additional VSDs, evaluate the architecture of the
RVOT, pulmonary valve, annulus and the morphology and caliber of the pulmonary
artery branches. If large collateral arteries are present, these can be defined by
injecting dye into the descending aorta. They can also be occluded by trans-catheter
coil embolisation or device closure prior to, or even after, definitive surgical repair.

Management of TOF is a combination of medical and surgical elements.

Paroxysmal hypercyanotic ‘spells’ may respond quickly to oxygen, placing the
child in knee-elbow position, morphine, sodium bicarbonate and occasionally beta-
blockade (IV propranolol). Surgical creation of a systemic-pulmonary shunt like
the Blalock-Taussig shunt between the subclavian artery and pulmonary artery may
be necessary to relieve recurrent spells.

Surgical repair for TOF is best performed between 9 and 18 months of age,
when the child weighs around 10 kg. Some centers, however, perform this operation
in the early newborn period. Intracardiac repair involves closure of the VSD using

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a patch which also redirects blood from the left ventricle into the overriding aorta,
along with relief of the RVOT obstruction by excising muscle bundles in the
outflow or using a trans-annular patch. When the pulmonary artery annulus is very
small or atretic, or when anomalous coronary artery branches cross the RVOT, a
homograft may be implanted as a conduit between the RV and PA.

Results of intracardiac repair for TOF are very good with relief of cyanosis
and improve- ment in growth and exercise tolerance. Complications include
pulmonary regurgitation, RV failure and late ventricular arrhythmias.

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III. REFERENCES

1. Myung KP, Mehrdad S. The Pediatric Cardiology Handbook 4th edition.

MOSBY Elsevier. Philadelphia: 2010.
2. Kulkarni ML. Pediatric Cardiology 2nd edition. JAYPEE RPTHERS Medical
Publishers.New Delhi: 2003
3. Walter J, James M. Pediatric Cardiology The Essential Pocket Guide 3rd edition.
WILEY Blackwell: 2014
4. Park MK. Park’s Pediatric Cardiology for Practitioners 6th edition. ELSEVIER
Saunders. Philadelphia: 2014.
5. Suree L. Pathophysiology of Heart Disease. Chiangmai. Thailand: 2011
6. Bambang M, Sri ER, Ruiana. Penanganan Penyakit Jantung Pada bayi dan anak.
FKUI: 2005
7. Sudigdi S, Bambang MK. Buku Ajar Kardiologi Anak. IDAI: 2009

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