Aterm Neonatus with

Pulmonary Atresia and

Intact Ventricular
septum
Presented by :
Dyah Kusumaningrum

Supervised by :
Dr. dr. Anindita S.,Sp.AK
CASE
Case

Part 2 PATIENT’S IDENTITITY

Name : NA

Date of Birth : 25th of November 2018

Age : 2 days old

Sex : Male
Part 3
Address : Jepara
Part 4 Admitted date : 27th of November 2018
Case Alloanamnesis with parents and

BASIC DATA: Anamnesis information from patient’s medical

record
Part 2
Chief complaint : cyanosis (as referred by Regional General
Hospital of Hadlirin Jepara with suspect congenital heart disease).

At the Emergency Room of Hadlirin Hospital

 The baby inactive, cry inadequately, breath rapidly and cyanotic.

 SpO2↓ 60-70%
 Awareness apathetic
Part 3
 Heart murmur (+)
Part 4  Blood laboratory ↑ leucocyte, hematocrit, and calcium level.
Case Alloanamnesis with parents and

BASIC DATA: Anamnesis information from patient’s medical

record
Part 2
At the Emergency Room of Hadlirin Hospital

 Treatment
• O2 cannula nasal 2 lpm
• D10% 10 drop per minute via umbilical cord vein infused
• Injection bactecyn and gentamycin
Part 3 • Dietary intake via enteral feeding tube.
 Suspected of having a congenital heart disease  referred to
Part 4
Kariadi Hospital after one day hospital care at Hadlirin Hospital.
Case Alloanamnesis with parents and

BASIC DATA: Anamnesis information from patient’s medical

record
Part 2
At the Emergency Room of Kariadi Hospital
 The baby was inactive, still with apathetic consciousness.
 No sign of prematurity
 Body weight 3.200 grams with body height of 50 cm
 HR 143 times per minute
 RR 46 times per minute
 Temp 36.9o C
Part 3  SpO2 ↓ 51-54%
 No chest retraction
Part 4
 Normal S1-S2 regular + continues murmur grade III/6 (punctum maksimum at
the left infraclavicular area)
 Cyanosis extremities
Case Alloanamnesis with parents and

BASIC DATA: Anamnesis information from patient’s medical

record
Part 2
At the Emergency Room of Kariadi Hospital

 Echocardiography : pulmonary atresia with intact septum ventricle (PA-IVS), Patent

Ductus Arteriosus (PDA) and Patent Foramen Ovale (PFO)
 Diagnosed as
• Aterm neonatal (39 month gestational)
• with normal birth weight (3200 gram),
• neonatal infection,
Part 3
• cyanotic congenital heart disease with pulmonary atresia- intact septum
ventricle (PA-IVS), Patent Ductus Arteriosus (PDA) and Patent Foramen Ovale
Part 4
(PFO), without heart failure.
Case Alloanamnesis with parents and

BASIC DATA: Anamnesis information from patient’s medical

record
Part 2
At the Emergency Room of Kariadi Hospital

Treatment
 O2 cannula nasal 2 lpm
 IV D10% 120/5mL/hour + NaCl 3% (2 meq) + KCl otsu (2 meq)
 Ampicillin 160 mg/12 hours and gentamycin 16 mg/24 hours
 Aminosteril 6% 72/3 mL/hour
 Lipid 20% 24/1ml/hour
Part 3
 Prostaglandin E1 (Alprostadil) 60 mcg/kg body weight inside 50mL NaCl0,9% (192 mcg
/50cc NaCl 0.9%) given IV drip 0,5- 5mL/hour (10-60 mg/kg/minutes)
Part 4
 Admitted to level II perinatal unit.
Case Alloanamnesis with parents and

BASIC DATA: History information from patient’s medical

record
Part 2
Perintal History
Prenatal History
 Regular antenatal check-up by the mid-wife and obstetrician
 No history of disease during pregnancy (hypertension, diabetes mellitus and haemorrhage or
injury), Non-prescribed drugs and traditional herbal medicine were not taken, No history of
prematurity rupture of the membrane (PROM), No data that described about the amniotic fluid.

Natal History
Postnatal History
Part 3  Cried immediately
 No history of seizure or jaundice after birth
 Bluish extremities
 Several hours later  rapid breathing, cry
 Apgar score 4-6-8
Part 4 inadequately and more cyanotic
 No record of SpO2 when the baby was born.
 SpO2 70-80%
 Birth weight: 3.200 grams ; height: 50 cm
 Placenta born manually, complete cotyledons,
no infarction or hematoma.
Case Alloanamnesis with parents and

BASIC DATA: History information from patient’s medical

record
Part 2
Family’s History
 No family history of congenital disease including congenital heart disease.

Part 3

Part 4
Picture 1. Pedigree
Case Alloanamnesis with parents and

BASIC DATA: History information from patient’s medical

record
Part 2
Socio- Economic History
• His father graduated from senior high school and works as factory
workers.
• His mother is a housewife.
• Monthly income is ± IDR 1.500,000,-.
• Hospitalization fee was paid with insurance (JKN non PBI).
Part 3
Nutritional history

Part 4 • Breastmilk since birth

• Hospital care : 10 ml/3 hour breastmilk via OGT, intravenous D10%
144/6mL/hour with 2 meq sodium and 2 meq potasium (first day care).
Case Was taken at the first day in the

PHYSICAL EXAMINATION level II perinatal unit

Part 2
General condition
Composmentis consciousness; rapid breath.

Vital Sign
• Eye icteric (-/-), pale conjunctiva (-),
• HR : 134 x/min, the contain and
pupil Ø 3 mm/3 mm, pupillary light
tension of vein is adequate
reflex is normal
• RR : 72 x/min
• Nose nasal flaring (-)
• Temp : 37,0°C (axilla)
Part 3 • Mouth cyanosis (-)
• SaO2 : 51-54% with nasal O2 1 lpm
• Throat pharynx: hyperemia (-),
• Weight : 3200 grams
Part 4 tonsils : enlargement (-); hyperemia (-)
• Length : 50 cm
Case Was taken at the first day in the

PHYSICAL EXAMINATION level II perinatal unit

Part 2
Thorax
Pulmo
• The chest is symmetrical
• No chest-wall indrawing
• Vesicular sound without any additional sound.
Cor
• Cardiac apex doesn’t shift to lateral
Part 3
• Normal S1-S2 heart sound, regular
• Continues murmur grade III/6 (punctum maksimum: left infraclavicular
Part 4
area)
Case Was taken at the first day in the

PHYSICAL EXAMINATION level II perinatal unit

Part 2
Abdomen
• Flat and smooth
• Umbilical cord vein infuse is installed
• Tender
• Normal bowel sound
• Dullness on side (+), shifting dullness (-)

Part 3 • Liver and spleen were not palpable.

Extremity superior inferior Genitalia

Part 4
Cyanotic +/ + +/+ • Male
Cold acral -/- -/- • Ostium urethrae externum :
Capillary refill <2”/<2” <2”/<2” Hyperemia (-), phimosis (-)
Case
Growth History
Part 2
• Birth weight 3200 gr, birth length 50 cm, birth head circumference 35 cm.
• Lubchenco curve : birth weight 3200 gram (P25P10-90), birth length 49 cm (P25P10-90),
birth head circumference 35 cm (P25P10-90),
• The impression : appropriate for gestational age.

Part 3

Part 4

Figure 1. Lubchencho curve of the patient

Case
SUPPORTING EXAMINATION
Part 2
Table 1. Laboratory finding
Reference value Unit 25/11/2018 27/11/2018
Hemoglobin 13.6- 19.6 gr/dL 17.3 16.2
Hematocrit 44 – 62 % 49.5 46.6
Erythrocyte 3.9- 5.9 10^6/uL - 4.35
MCH 24.0- 34.0 Pg - 37.3
MCV 83 – 110 fL - 107
MCHC 29.0- 36.0 g/dL - 34.8

Part 3 Leukocyte 9 – 30 10^3/uL 20.7 13.8

Platelets 150 – 400 10^3/uL 235 252
RDW 11.6- 14.8 % - 14.3
Part 4
MPV 4.00 - 11.00 fL - 7.39
Table 2. Coagulation profile
Reference value Unit 27/11/18
Plasma Prothrombin Time (PPT) 9.4 - 11.3 Sec 16.1
Activated Partial Thromboplastin Time (APTTK) 27.7 – 40.2 Sec 67.6
Case
SUPPORTING EXAMINATION
Part 2
Table 3. Differential counting of leukocyte and peripheral blood smear

Reference value Unit 27/11/18

Eosinophil 2–4 % 2
Basophil 0–4 % 0
Rod 2–5 % 2
Segment 45 – 75 % 67
Lymphocyte 20 – 40 % 19
Monocyte 3 – 12 % 9
Part 3 Others Myelocyte 1%, Nuclei erythrocyte: 1/100 leucocyte
Pheripheral blood smear

Part 4 Erythrocyte
Mild anisocytosis (macrocytic, normocytic), mild poikilocytosis (ovalocyte, pear shape
cell, eliptocyte), polychromatic +

Platelets Increase estimation number of platelets, normal shape domination, giant shape (+)

Leukocyte Normal estimation number, lymphocyte atypical

Case
SUPPORTING EXAMINATION
Part 2
Table 4. Laboratory finding on Clinical chemistry
Clinical chemistry Reference value Unit 25/11/18 27/11/18
Glucose 80-160 mg/dl 87 246
Ureum 15-39 mg/dL - 16
Creatinin 0,60-1,30 mg/dL - 0.78
Calcium 2,12-2,52 mmol/L 2,7 2.18
Sodium 136-145 mmol/L 137.9 124
Part 3 Potassium 3,5-5,1 mmol/L 4.81 4.2
Chloride 98-107 mmol/L 102.4 88
Part 4
Electrocardiography Finding (27/11/18)
Normosinus rhythm, heart rate 142x/minute, Right Axis Deviation
Case
SUPPORTING EXAMINATION
Part 2
Echocardiography Finding (27/11/2018)

Part 3

Part 4
Case
SUPPORTING EXAMINATION
Part 2
Echocardiography Finding (27/11/2018)

Part 3

Part 4
 Case
SUPPORTING EXAMINATION
Part 2 Echocardiography Finding (27/11/2018)
Findings and measurement :
- Both aorta and inter ventricular septum (IVS) are on the right side of the vertebrae
- AV-VA concordance
- All four pulmonary venous estuaries in left atrium (LA)
- Persistent foramen ovale (+), Right to left (R-L) shunt
- Interventricular septum intact
- Severe tricuspid regurgitation (TR), with pressure gradient 130 mmHg
- No forward flow from RV to PA appears
Part 3 - Mean pulmonary artery (MPA) - Right pulmonary artery (RPA) – Left Pulmonary artery (LPA) konfluens,
normally size, three valves does not open

Part 4
- Pulmonary artery gets blood from the Patent ductus arteriosus (PDA) Ø2,25mm, contious shunt from
aorta to left pulmonary artery
- Left aortic arc, coarctatio aortae (-)

Diagnostic Impression:
Pulmonary atresia with intact septum ventricle (PA-IVS), Patent Ductus Arteriosus (PDA) and Patent Foramen Ovale (PFO).
Case
DIAGNOSIS
Part 2 Level II Perinatal Unit
 Based on the anamnesis, physical and supporting examination, the patient
was diagnosed as an aterm neonate, normal birth weight (3200 grams) with
appropriate for gestational age, neonatal infection, hiponatremia
 Etiological diagnosed as cyanotic congenital heart disease
 Anatomical diagnosed as pulmonary atresia with intact ventricular septum
(PA-IVS), patent ductus arteriosus (PDA), and patent foremen ovale (PFO)
Part 3
 Functionally diagnosed as without heart failure.
Part 4
Case
THERAPY
Part 2 Level II Perinatal Unit
Treatment
 O2 nasal 1 liter per minute
 Ampicillin 160 mg/12 hours and gentamycin 16 mg/24 hours
 Prostaglandin E1 (Alprostadil) 60 mcg/kg body weight inside 50 mLNaCl 0,9% (192 mcg
/50cc NaCl 0.9%) 2 mL/hour
 Partial parenteral nutrition : IV D10% 120/5mL/hour + Nacl3% (3 meq) + KCl otsu (2

Part 3 meq), aminosteril 6% 50/2,1mL/hour, lipid 20% 16/0,7ml/hour, and breast milk diet 10
mL/3 hours through orogastric tube.
Part 4  Planned to referred to Cipto Mangunkusumo Hospital with preparation to undergo
radiofrequency perforation and valvuloplasty (RFV) + percutaneous balloon pulmonary
valvuloplasty.
Case
PROGRESS NOTE
Part 2 On the 3rd day of treatment (29/11/18)
The baby’s general condition was improved, • Chest symmetric, chest-wall indrawing (-)
although a fever appears on day 2. • Still found continues murmur grade III/6
Physical Examination : (punctum maksimum: left infraclavicular
• Composmentis consciousness. area)
• HR 134 times per minute • Normal and regular S1-S2 heart sound.
• RR ↓ 60 times per minute • Lung revealed vesicular sound without any

Part 3 • Axillary temperature 37.0°C additional sound

• The contain and tension of vein is • Abdomen flat and smooth, normal bowel
Part 4 adequate sound, liver and spleen were not palpable.
• The SpO2 ↑ 90-94 % (with nasal O2 1 lpm) • Cyanotic (-), cold acral (-) in upper and
• Nasal flaring (-) lower extremities, CRT <2 seconds
• Mouth mucosa cyanosis (-)
Case
PROGRESS NOTE
Part 2 On the 3rd day of treatment (29/11/18)
Treatment
 Nasal O2 1 lpm
 Ampicillin 160 mg/12 hours and Gentamycin 16 mg/24 hours IV
 D10% 120/5mL/hour + Nacl3% (3 meq) + KCl otsu (2 meq), aminosteril 6%
50/2,1mL/hour
 Lipid infussion  stopped

Part 3  Breast milk diet ↑ 20-25 mL/3 hours through orogastric tube
 Alprostadil (Prostaglandin E1) IV drip  tapered, given 60 mcg/kg body weight inside 60
Part 4 cc NaCl 0,9% (192 mcg/50cc NaCl 0.9%) 1,5 mL/hour
Case
PROGRESS NOTE
Part 2 On the 4th day of treatment (30/11/18)
Treatment
 Nasal O2 1 lpm
 Ampicillin 160 mg/12 hours and Gentamycin 16 mg/24 hours IV
 D10% 120/5mL/hour + Nacl3% (3 meq) + KCl otsu (2 meq), aminosteril 6%
50/2,1mL/hour
 Breast milk diet ↑ 30-35mL/3 hours through orogastric tube

Part 3  Alprostadil (Prostaglandin E1) IV drip  tapered, given 60 mcg/kg body weight inside 60
cc NaCl 0,9% (192 mcg/50cc NaCl 0.9%) 1,2mL/hour (24mg/kg/minute).
Part 4
Case
PROGRESS NOTE
Part 2 On the 6th day of care (2/12/18)
 General condition : compos mentis consciousness.
 HR 154 times per minute
 RR 32 times per minute
 Axillary temperature 36,8°C
 The contain and tension of vein is adequate
 SpO2 82% (supine position) to 92% (tilt position), with nasal O2 1 lpm

Part 3  Epigastrial and intercostal retraction (+)

 Stridor while inspiration
Part 4  Suspected as laryngomalacia.
 Previous treatment was continued
 Plan: direct laryngoscopy examination.
Case
PROGRESS NOTE
Part 2 On the 8th day of treatment (4/12/18)
 Laryngoscope flexible examination : M shaped of epiglotis, partially closed the airway
when inspiration, plica vocalis was difficult to examine, diagnostic impression : mild
laryngomalasia.
Treatment
 Nasal O2 1 lpm
 Ampicillin 160 mg/12 hours and Gentamycin 16 mg/24 hours IV

Part 3  D10% 120/5mL/hour + Nacl3% (3 meq) + KCl otsu (2 meq), aminosteril 6%

50/2,1mL/hour
Part 4  Breast milk diet ↑ 40-45mL/3 hours through orogastric tube
 Alprostadil (Prostaglandin E1) IV drip  tapered, given 60 mcg/kg body weight inside 60
cc NaCl 0,9% (192 mcg/50cc NaCl 0.9%) 1mL/hour
 Monitoring: SpO2, positioning tilt to the left, and for airway obstruction
Case
PROGRESS NOTE
Part 2 On the 9th day of care (5/12/18)
 Tachypnea (-), bluish (-)
 Look active, compos mentis consciousness
 HR 152 times per minute
 RR 46 times per minute
 Axillary temperature is 36,8°C
 The contain and tension of vein is adequate

Part 3  SpO2 93-94%, with nasal O2 1 lpm.

 Alprostadil (Prostaglandin E1) IV drip  tapered, given 60 mcg/kg body weight inside 60
Part 4 cc NaCl 0,9% (192 mcg/50cc NaCl 0.9%) 0,5mL/hour  planned to stop
 Programmed to be outpatient on the 10th day while waiting the reference process to
Harapan Kita Hospital.
“Theory”
 Case

Theory The Normal

Heart
Four chambers in the
heart :
• The right and left
atrium which are
Discussion separated by the
atrial septum
• The right and left
Resume
ventricles which are
separated by the
ventricular septum
The right ventricle pumps blood into the pulmonary artery (the blood
vessel that takes blood to the lungs). The left ventricle pumps blood into
the aorta (the blood vessel that takes blood to the rest of the body).

Blood flows from the right side of the heart, through the pulmonary valve
into the pulmonary artery, and then to the lungs where it picks up oxygen.

The oxygen-rich blood flows back into the left side of the heart through
the pulmonary veins.

The left ventricle then pumps the oxygen-rich blood out of the heart
through the aortic valve and into the aorta, and all around the body.

The blood then returns to the right side of the heart through two main
veins; superior vena cava and inferior vena cava
Physiological Change of The Heart
Fetal Circulation
• Four shunt : placenta, ductus venosus, foramen ovale and ductus arteriosus
• Oxygen saturation in IVC (70%) is higher than that in SVC (40%).

Superior Vena Cava Right Ventricle (RV)

About one third of the IVC blood with higher

oxigen saturation is directed by the crista dividens
Inferior Vena Cava  the left atrium (LA)  the foramen ovale

The remaining two thrids enters the RV and

pulmonary artery (PA).
• Less oxygenated blood in the PA flows through the widely open ductus
arteriosus to the descending aorta and then to the placenta for oxygenation
Physiological Change of The Heart
Physiological Change of The Heart After Birth
The primary change : shift of
blood flow or gas exchange
from the placenta to the lungs
The placental circulation
disappear and the pulmonary
circulation is established

Increase in systemic
Removal of the placenta
vascular ressistance (SVR)

Reduction of the pulmonary vascular

Lung expansion ressistance (PVR), an increase in pulmonary
blood flow and a fall in PA pressure, functional
clossure of foramen ovale
Physiological Change of The Heart
Physiological Change of The Heart After Birth

Functional clossure of the

ductus arteriosus occurs
within 10 to 25 hours after
birth by constriction of the
medial smooth muscle in the
ductus
Anatomic clossure is completed by
2-3 weeks of age by permanent
changes in the endothelium and
subintimal layers of the ductus.

• Oxygen, prostaglandi E2 ( PGE2) levels, and maturity of the

newborn are impotant factors in clossure of the ductus.
Acetylcholine and bradykinin also constrict the ductus.
Physiological Change of The Heart
Physiological Change of The Heart After Birth
Postnatal increase in oxygen saturation of the systemic
circulation (from PO2 of 25 mmHg in utero to 50 mmHg
after lung expansion)

Strong stimulus for constriction of the ductal

smooth muscle

Related to the gestational age;

the ductal tissues of premature infant response less
intensely to oxygen than that of fullterm infant
Physiological Change of The Heart
Physiological Change of The Heart After Birth

Removal of the Indomethacin or

Constricting effect of
placental source of PGE2 ibuprofen,
indomethacin or
production at birth and Decrease in PGE2 level cyclooxygenase
ibuprofen and dilator of
from the marked of after birth result in the inhibitor (or “PG
PGE2 and PGI2 are
increase pulmonary constriction of the synthetase inhibitor”)
greater in the ductal
blood flow, which allow ductus can be use to close a
tssue of immature fetus
removal of circulating significant PDA in
than a near-term
PGE2 by the lungs premature infants
 Pulmonary Atresia
Pulmonary atresia is a birth defect of the pulmonary valve, which is the valve
that controls blood flow from the right ventricle to the main pulmonary artery

• Blood cannot directly flow from the right ventricle of the heart out to the
pulmonary artery, blood must use other routes to bypass the unformed
pulmonary valve.
• The foramen ovale, a natural opening between the right and left upper chambers
of the heart during pregnancy that usually closes after the infant is born, often
remains open to allow blood flow to the lungs.
• Another way to bypass the unformed pulmonary valve by keep the infant’s patent
ductus arteriosus open after the infant’s birth  allows blood to move around the
infant’s lungs before the infant is born and it also usually closes after birth.
 Type of Pulmonary Atresia
Pulmonary atresia with an intact ventricular septum

• Septum between the

ventricles remains complete
and intact.
• During pregnancy when the
heart is developing, very
little blood flows into or out
of the right ventricle (RV),
and therefore the RV
doesn’t fully develop and
remains very small
 Type of Pulmonary Atresia
Pulmonary atresia with a ventricular septal defect

• Ventricular septal defect (VSD) allows blood to flow into and

out of the right ventricle (RV).
• Blood flowing into the RV can help the ventricle develop
during pregnancy, so it is typically not as small as in
pulmonary atresia with an intact ventricular septum.
• Pulmonary atresia with a VSD is like a very severe form of
tetralogy of Fallot
 About 1 in every 7,700 infants born
in the United States each year are
Prevalence
born with pulmonary atresia

In a 2012 study using data from birth defects tracking systems across the United States,
estimated that each year about 520 infants in the United States are born with pulmonary atresia

Most studies report ~1.5 cases per 1,000 live births for severe congenital heart disease and a
similar number for moderately severe congenital heart disease

Only +15% of cases of congenital heart
disease can be traced to a known cause.
Some infants have heart defects because
of changes in their genes or
chromosomes.
Causes and Risk Factors
Several well-recognized chromosomal
aneuploidies cause the malformation
syndromes that comprise congenital heart
disease, for example, Down syndrome, trisomy
13, trisomy 18, Turner syndrome and DiGeorge
syndrome

The etiology of
congenital heart disease
is largely unknown

The etiology of the remaining cases of
nonsyndromal congenital heart disease is
considered to be multifactorial,
suggesting that several genetic and
environmental factors interact
•Maternal diabetes mellitus and phenylketonuria,
maternal obesity, alcohol use, rubella infection, febrile
illnesses, use of certain drugs, such as thalidomide
and retinoic acid, exposure to organic solvents,
maternal exposure to herbicides, maternal age >40
years and paternal age >35 years
 Pulmonary Atresia with Intact Ventricular Septum
Prevalence
Approximately 1-3% of all
congenital heart disease and
2.5% of all critically ill infants
with congenital heart disease
4 to 8 per 100,000 live births
and is the third most common
form of cyanotic congenital In the UK/Eire study critical
heart disease pulmonary stenosis was
diagnosed in six fetuses at a mean
There were 183 live births with gestational age of 22 weeks, with
an overall incidence of 4.5 per subsequent progression to atresia
100 000 live births by 31 weeks
Pulmonary Atresia with Intact Ventricular Septum
Embriology PA-IVS is acquired later in gestation when compared with
pulmonary atresia with a ventricular septal defect.

The pulmonary valve becomes atretic later in development following closure

of the interventricular communication, a finding suggested by what is
generally a well-developed pulmonary trunk

Membranous atresia of the pulmonary valve (imperforate valve)  not

generally associated with coronary abnormalities and may allow for a less
invasive approach such as percutaneous pulmonary valve perforation and
balloon angioplasty

Development of PA-IVS varies, thought to occur between 6-10 weeks gestation.

Specific mechanisms of pulmonary valve (PV) malformation unknown, it could be a
pulmonary valve leaflet malformations or failure in separation of valve leaflets
Pulmonary Atresia with Intact Ventricular Septum
Hemodynamics and Pathophysiology
In utero, the fetus with PA-IVS with severe tricuspid
regurgitation can present with significant hydrops and
intrauterine demise secondary to a low- pressure, severely
hypoplastic, and failing RV

Obstruction to right vetricular outflow is complete +

infundibular atresia (atresia of the bulbar ostium) or
atresia of the main pulmonary artery  Blood passes in
and out of the ventricle through the incompetent
tricuspid valve

The ductus arteriosus is usually patent, but tends to

close in the first few weeks of life. After birth, the
arterial duct remains the sole source of pulmonary
blood flow
Pulmonary Atresia with Intact Ventricular Septum
Morphology of PA-IVS
Pulmonary atresia with intact ventricular septum is
defined by a membranous or muscular atresia of the right
ventricular outflow tract and an intact ventricular septum

The greatest morphofunctional changes in

pulmonary atresia with intact septum are
related to the tricuspid valve, trabecular
portion, and right ventricular outlet

In almost all cases, the ductus arteriosus

begins the closure process right after birth,
and, occasionally, it may remain functional
after the 30th day of extrauterine life.
Pulmonary Atresia with Intact Ventricular Septum
Morphology of PA-IVS

Because in pulmonary atresia with Possibility of a

intact septum no communication patient surviving
between the right ventricle and the after birth is very
pulmonary trunk exists small

Therefore, pulmonary blood is supplied through the ductus arteriosus,

and, occasionally, through systemic pulmonary collaterals
Pulmonary Atresia with Intact Ventricular Septum
Morphology of PA-IVS

Classification of RV in PA-IVS based on presence or

absence of inlet, trabecular and infundibular
portions
Pulmonary Atresia with Intact Ventricular Septum
Morphology of PA-IVS
RV morphology can be categorized into three major types
based on the presence of number of portions of RV cavity:
1. Muscular obliteration of the apical trabecular
cavity, and in some cases its infundibulum,
resulted in “bipartite” right ventricle (RV) in
33.6%
2. “Unipartite” chamber in 7.7%
3. The remaining 58.7% had “tripartite”
morphology
 Pulmonary Atresia with Intact Ventricular Septum
Morphology of PA-IVS
Tripartite RV
• Consisting of inlet, trabecular and infundibular portions of the RV
are nearly normal in size (Panel A)

Bipartite RV with absent or severely Hypoplastic RV

• The inlet and infundibular portions are present and the trabecular
portion is obliterated (Panel B)

Monopartite RV with only inlet portion

• Only the inlet portion is present. The RV is severely hypoplastic

with high pressure
Pulmonary Atresia with Intact Ventricular Septum
Clinical Features
Pulmonary atresia with intact ventricular
septum is a very serious type of congenital
heart condition.

Two main abnormalities:

• The pulmonary valve (the valve which allows blood to
flow from the heart to the lungs) is completely blocked.
• The right ventricle has often not developed normally
Pulmonary Atresia with Intact Ventricular Septum
Infants born with pulmonary atresia will show
symptoms at birth or very soon afterwards

Cyanosis
Clinical
Features Tachypnea and dyspnea associated cyanosis

The second heart sound (single S2) due to absent P2

Hepatomegaly are common physical findings

Poor feeding

Extreme sleepiness
Pulmonary Atresia with Intact Ventricular Septum
Diagnosis

During Pregnancy
•- Cyanosis skin or
problems breathing
Pulmonary atresia •- Murmur/ second
might be seen heart sound

After the Infant is Born

during an
- Screening using
ultrasound and pulse oximetry
then
fetal echocardio- - Electrocardiogram
gram to confirm - The chest X-ray may
the diagnosis show a normal sized
heart
- Echocardiogram
Pulmonary Atresia with Intact Ventricular Septum
Management: Pre-cathetherization
• To maintain adequate pulmonary blood flow via the
PDA
• The starting dose of prostaglandin E is 0,05 to 0,1
ug/kg per minutes, when desired effect is achieved,
Intravenous
the dosage is gradually reduce to 0,01 ug/kg per
infusion of minute
prostaglandin • Initial dose of 20 ng/kg/minute of PGE1 is started
E1 (PGE-1 • After 1 day  gradually tapered to around 10
ng/kg/minute as maintenance to keep the PDA open
• Low initial dose is only 25- 50% of the conventional
dose
Pulmonary Atresia with Intact Ventricular Septum
Management: Pre-cathetherization
Decompress the right • If ASD is inadequate, atrial septostomy may be
atrium should be indicated to stabilize the baby.
ascertained at the time • However, ASD or PFO shunt usually is adequate and
echocardiography the baby usually is stabilized

• Corrected prior to going to cardiac catheterization

Metabolic derangements
laboratory

• Correct metabolic as well as respiratory acidosis

• Treat any suspected infections
Maintain a stable airway
• Maintain a hematocrit above 40 to insure adequate
tissue oxygenation

Imaging of the abdomen

• Required to rule associated birth defects
and brain
Pulmonary Atresia with Intact Ventricular Septum
Management: Transcatheter management and
technique
Promote Thoracotomy or by the percutaneous route
clearance of the
right ventricular Stimulating the intracavitary flow
outflow tract
Reducing the degree of hypertrophy

Improving ventricular compliance

Enabling enlargement of the ventricular cavity

to make biventricular correction feasible

These procedures are not indicated for all patients

Pulmonary Atresia with Intact Ventricular Septum
Management: Transcatheter management and
technique
Surgical RF valvotomy and balloon valvuloplasty more
valvotomy with effective compared to surgical valvotomy and
BT shunt BT shunt

Z index Z = measured diameter – mean normal

value/standard deviation of the mean normal
diameter

Diameter of the tricuspid valve < 70% of the

normal or 70% of the diameter of the mitral
ring represents a risk factor or failure factor for
biventricular correction
 Pulmonary Atresia with Intact Ventricular Septum
Management: Transcatheter management and
technique
Femoral venous - Measurement RV Placing a catheter

Diagnostic part
Vascular access

Transcatheter perforation

Hybrid approach
Serial balloon
access is systolic pressure– in the RVOT in
usually
valvuloplasty : correct orientation
preferred over from 3 mm to 6-
suprasystemic if to the RVOT and
umbilical venous the tricuspid valve 8 mm balloons pulmonary valve.
access is reasonably
competent
- RV angiography–
to demonstrate RV
cavity and RVOT
(right ventricular
outflow tract)
- Evaluation for RV-
DCC
Pulmonary Atresia with Intact Ventricular Septum
Management: Post-procedure care
Continuation of prostaglandin infusion + intervenous
esmolol or oral propranolol

BT shunt or stenting of patent ductus arteriosus if

pulmonary valve annulus or the valve orifice is
inadequate

Baby observed as long as 4-6 weeks for the oxygen

saturation to improve back to satisfactory levels
Pulmonary Atresia with Intact Ventricular Septum
Prognosis
Study of 183 patients born with of Pulmonary Atresia with
Intact Ventricular Septum in The UK and Ireland from 1991
through 1995
 One- and 5-year survival was 70.8% and 63.8%,
respectively
 9 years of follow-up :
29% have achieved a biventricular repair
3% a so-called one-and-a-half ventricular repair
10.5% a univentricular repair
16.5% still having a mixed circulation (41% died)
Critical Congenital Heart Disease
 a term that refers to a group of serious
heart defects that are present from birth

Defined Structural malformations of the heart

that are present at birth and require
as intervention in the 1st year of life

Pulmonary atresia is one of a critical congenital

heart disease (CCHD)
Critical Congenital Heart Disease
Prevalence
• Nearly 30% of infant deaths due to birth defects

In the United States, about 7,200 (or

18 per 10,000) babies born every
year have critical congenital heart
defects (CCHDs)

Babies with CCHDs usually require

surgery or catheter intervention in
the first year of life
Critical Congenital Heart Disease
Type of CCHD
The primary targets of CCHD screening are seven
specific lesions :

1.Total anomalous
1.Hypoplastic left 1.Pulmonary 1.Tetralogy of
pulmonary venous
heart syndrome atresia Fallot
return

1.Transposition of 1.Truncus
1.Tricuspid atresia
the great arteries arteriosus
Critical Congenital Heart Disease
Diagnosis
• During pregnancy using a special type of ultrasound called
a fetal echocardiogram
• Possible physical symptoms after birth:
– problems breathing (rapid breathing or tachypnea)
– pounding heart
– weak pulse
– low blood pressure (hypotension)
– abnormal heart sound during a heartbeat (heart
murmur)
– very pale or blue skin color caused by a shortage of
oxygen (cyanosis)
– poor feeding
– very sleepy
Critical Congenital Heart Disease
Diagnosis
Complication
Organs and tissues
within the first few
throughout the
days or weeks of
body do not
life and often
If untreated, CCHD receive enough
require emergency
can lead to shock, oxygen, which can
care  require
coma, and death lead to organ
surgery or catheter
damage and life-
intervention
threatening
during the first
complications
year of life
Critical Congenital Heart Disease
Diagnosis
Newborn screening using pulse oximetry  can
identify some infants with a CCHD before they show
signs of a CCHD.

The presence of low blood oxygen saturation

Performed 24–48 h (hypoxemia), or a difference between pre-
ductal and post-ductal (proximal and distal to
after birth the aortic opening of the ductus arteriosus,
respectively) saturation

Once identified, babies with a CCHD can be Treatment can include

seen by cardiologists and can receive
specialized care and treatment that could medications and
prevent death or disability early in life. surgery soon after birth
Critical Congenital Heart Disease
Pulse Oximetry Screening
Most likely to detect seven of the critical
CHDs :
1. hypoplastic left heart 5. transposition of the
syndrome great arteries
2. pulmonary atresia 6. tricuspid atresia, and
3. tetralogy of Fallot 7. truncus arteriosus
4. total anomalous
pulmonary venous
return
Critical Congenital Heart Disease
Pulse Oximetry Screening Easy, safe,
sensitive, for diagnosis of
and highly CCHD
specific

Determine the
amount of
Low saturation = a oxygen in a
sign of a CCHD baby’s blood and
the baby’s pulse
rate

Done when
a baby is 24 before he or she is
to 48 hours 24 hours of age
of age
Critical Congenital Heart Disease
Pulse Oximetry Screening
A prospective cross-sectional study conducted in the Saudi
Arabia, between February 2016 and February 2017 :
 The sensitivity was 77%
 The specificity was very high at 97%,
 With a positive predictive value of 18%
and a negative predictive value of 99.8%
(95% confidence interval 13.78-19.18,
p=0.0001)
Critical Congenital Heart Disease
Effectiveness of critical congenital heart disease
(CCHD) screening program

Neonatal infection is a
significant cause of mortality and
morbidity in newborn babies
can lead to life-threatening
sepsis and accounts for 10% of
all neonatal mortality
Neonatal Infection
 Early-onset (infection arising within
72 hours of birth)
 Late-onset (infection arising more
than 72 hours after birth).
 Present in 8 of every 1000 live
births and 71 of every 1000
neonatal admissions.
 82% occur in premature babies
(less than 37 weeks) and 81% in
low birthweight babies (below
2500 grams).
 Neonatal Infection
invasive group B streptococcal infection in a previous baby

Risk maternal group B streptococcal colonisation

Factors bacteriuria or infection in the current pregnancy

prelabour rupture of membranes

preterm birth following spontaneous labour (before 37 weeks' gestation)

suspected or confirmed rupture of membranes for more than 18 hours in a preterm birth

intrapartum fever higher than 38°C

confirmed or suspected chorioamnionitis

parenteral antibiotic treatment given to mother for confirmed or suspected invasive bacterial infection
(such as septicaemia) at any time during labour
suspected or confirmed infection in another baby in the case of a multiple pregnancy (in the 24-hour
periods before and after the birth)
 Neonatal Infection
Sign and symptoms
• Nonspecific (eg, vomiting or poor feeding, increased
sleepiness or lethargy, fever or hypothermia,
tachypnea, rashes, diarrhea, abdominal distention).
• Acquired before birth  various symptoms or
abnormalities (eg, growth restriction, deafness,
microcephaly, anomalies, failure to thrive,
hepatosplenomegaly, neurologic abnormalities).
 Neonatal Infection
Clinical indicators
 Altered behaviour or responsiveness, altered muscle tone (for example,
floppiness)
 Feeding difficulties (for example, feed refusal), feed intolerance, including
vomiting, excessive gastric aspirates and abdominal distension
 Abnormal heart rate (bradycardia or tachycardia)
 Signs of respiratory distress, respiratory distress starting more than
4 hours after birth, hypoxia (for example, central cyanosis or reduced
oxygen saturation level)
 Jaundice within 24 hours of birth
 Apnoea
 Signs of neonatal encephalopathy, seizures
 Neonatal Infection
Clinical indicators
 Need for cardio–pulmonary resuscitation, need for mechanical ventilation in a
preterm baby, need for mechanical ventilation in a term baby
 Persistent fetal circulation (persistent pulmonary hypertension),
 Temperature abnormality (lower than 36°C or higher than 38°C) unexplained by
environmental factors
 Signs of shock
 Unexplained excessive bleeding, thrombocytopenia, or abnormal coagulation
(International Normalised Ratio greater than 2.0)
 Oliguria persisting beyond 24 hours after birth
 Altered glucose homeostasis (hypoglycaemia or hyperglycaemia)
 Metabolic acidosis (base deficit of 10 mmol/litre or greater)
 Local signs of infection (for example, affecting the skin or eye)
 Neonatal Infection
When starting antibiotic treatment in babies with risk factors
for infection or clinical indicators of possible infection
• Perform a blood culture before administering the first dose of antibiotic
1

• Measure the C-reactive protein concentration at presentation and again 18–24 hours later
2

• Perform a lumbar puncture to obtain a cerebrospinal fluid sample before starting antibiotics
3

• Urine microscopy or culture not routinely perform

4

• In babies with clinical signs of umbilical infection, such as a purulent discharge or signs of
5 periumbilical cellulitis perform a blood culture, take a swab sample for microscopy and culture
 Neonatal Infection
Sensitivity, specificity and cut-off values of biomarkers in reviewed studies
 Neonatal Infection
Management
Antibiotics
increase the risk of eczema and asthma in later life
widespread antibiotic use associated with a risk of
antimicrobial resistance
babies should have minimal exposure to antibiotics
 Neonatal Infection
Red Red
Management
flags (+) Perform investigations flags (-)
Consider : whether it is safe to withhold
and start antibiotic antibiotics, and whether it is necessary
treatment to monitor the baby's vital signs and
clinical condition

Do not delay starting If clinical concern increases, consider

antibiotics pending the performing necessary investigations and
test results starting antibiotic treatment

Give antibiotic as soon as possible and

always within 1 hour of the decision
 Neonatal Infection
The first-choice antibiotic regimen for empirical treatment :
benzylpenicillin with gentamicin
until local bacterial resistance patterns indicating a different antibiotic

Benzylpenicillin in a dosage Gentamicin in a starting

of 25 mg/kg every 12 hours dosage of 5 mg/kg,
the dose interval can be recommends a dosage of 4–
shortening to 8-hourly 7 mg/kg/day administered
based on clinical judgement in a single dose.
 Neonatal Infection
• Newborn babies should have their antibiotic treatment reassessed 36
hours after starting treatment
• NICE’s guideline on neonatal infection recommends:

Perform blood culture before Consider performing a lumbar puncture

administering the first dose, and in a baby who did not have a lumbar
measuring the CRP concentration at puncture at presentation who is receiving
presentation and again 19–24 hours later antibiotics

Stop the antibiotics at 36 hours if: the blood culture is negative, and the
initial clinical suspicion of infection was not strong, and the baby's
clinical condition is reassuring with no clinical indicators of possible
infection, and the levels and trends of CRP concentration are reassuring
 Laryngomalacia
Defined as collapse of supraglottic structures during inspiration

The most common laryngeal disease of infancy

Presents in the form of stridor, a high-pitched, musical, vibrating,

multiphase inspiratory noise appearing within the first 10 days of life

Not all neonates have the same laryngeal anatomy, but all neonates
potentially present supraglottic structures that can invaginate during
inspiration
 Laryngomalacia
Prevalence
The real Presents within
incidence of 2 weeks after
laryngomalacia birth
is unknown

The most common

cause of stridor in
newborns, affecting
45–75% of all infants
with congenital
stridor
Laryngomalacia
Pathophysiology
 Laryngomalacia
Diagnosis
 Present during the first 10 days of life
 Characterized by stridor which is a high-pitched, musical, vibrating,
multiphase inspiratory noise
 Stridor worsened by agitation, crying, feeding, and flexion of the
cervical spine, and is often improved by extension of the cervical
spine, the prone position and quiet breathing
 Intensity of stridor is variable during sleep
 Laryngomalacia
Laryngomalacia Signs of severity
with no signs of of laryngomalacia Diagnosis
severity
 Laryngomalacia
Flexible laryngoscopy must be performed to the positive diagnosis of
laryngomalacia requires dynamic examination of the larynx in a conscious infant

Holinger’s classification:
Type 1: anterior prolapse of the arytenoid and corniculate cartilages
Type 2: tubular epiglottis which curls on itself, often associated with type 1
Type 3: anteromedial collapse of the arytenoids;
Type 4: posterior prolapse of the epiglottis;
Type 5: short aryepiglottic folds
 Laryngomalacia
Management

Conservative treatment is the Surgical treatment is only

rule. indicated in 10% of cases.

The natural history in most patients is

Tracheostomy has been
for spontaneous recovery, however
the standard treatment
because of frank airway obstruction or
for upper airway
other complications 10-15% of babies
obstruction for over a
require surgical intervention to improve
century.
their airway.
 High Risk Baby
According to the American Academy of Pediatrics, high-risk
babies are defined broadly as including
1. the preterm infant
2. the infant with special health care needs or dependence
on technology (the main types of technological support
needed are nutritional support and respiratory support,
including supplemental oxygen
3. the infant at risk because of family issues
4. the infant with anticipated early death
 High Risk Baby
Complications :
Risk of severe
• Perinatal asphyxia hyperbilirubinemia :
• Hypothermia • Primiparity
• Hypoglycemia, hyperglycemia • ABO incompatibility
• Hypocalcemia • Rhesus hemolytic disease
• Polycythemia • G6PD deficiency
• Jaundice • Low gestational age
• Feeding difficulties • Weight loss
• Necrotizing enterocolitis • Sepsis
• Sepsis • High transcutaneus/total serum
• Pulmonary hemorrhage bilirubin levels
 High Risk Baby
Long-term complications  growth, developmental, neurological,
hearing and visionary problems

Systematic review study  prevalence of each impairment per

insult and overall 82% of the survivors were followed up and
assessed for 6-60 months

Impairments: learning difficulties, cognition, or developmental

delay 59%; cerebral palsy 21%; hearing impairment 20%; and
visual impairment 18%. Behavioral problems were reported 11%.
 High Risk Baby
Discharge conditions:
good nutritional intake
no cyanosis with “full feed” without
problem that might stabilize
clinically stable episode for a interfere nutritional intake temperature
week such as apnea, cyanosis,
vomiting

parents already receive appropriate

adequate increase of body
weight for 5-7 days before
discharge and body weight
or social problem
>1800 gram when
discharge
education and confident
no any medical immunizations
to take care the baby and
parent agree to visit have been
doctor a week after
administered
discharge and afterwards

hearing
safe
evaluation has
transportation
been completed
“Discussion”
 Part 1

Part 2
DISCUSSION
 In this case, the patient was in high risk because he had respiratory
problem that needed some support, had congenital heart defect, low
maternal education level and low social economy level.

 Screening of vision and hearing function were already programmed. In

this case eye and hearing function examinations had been carried out.
The results of both were normal and further evaluation was
Disscussion
programmed at the age of 3-6 months.
Part 4  Discharge criteria for this patient had yet to be fulfilled, because of
respiratory distress and adequate feeding competence had not
established.
Primigravida O
High risk baby Short term outcome:
Anorexia Nutritional intervention (breast milk) - Without any procedur P
Hyperemesis Gravidarum Vitamin K Injection T
all case with
Neonatus aterm Growth & development monitoring
pulmonary atresia with I
Appropriate for gestational Immunization
intact ventricular M
39 weeks gestational age age
Birth weight septum will died  A
important information L
Clinical
Cyanosis, Tachypnea, for counseling in fetal
echocardiography
Desaturation life and for surgical G
Oxygenation
Continues murmur at the left strategy after birth
PA-IVS, Prostaglandin E1 injection [LoE R
infraclavicular
PDA, II] O
ECG : Right Axis Deviation Long term outcome:
Echo Pulmonary atresia with PFO Radiofrequency perforation and W
With catheter procedure one-
intact septum ventricle (PA- valvuloplasty (RFV) + T
and 5-year survival was
IVS), Patent Ductus Arteriosus percutaneous balloon pulmonary H
70.8%
(PDA) and Patent Foramen valvuloplasty [LoEII]
With surgical one- and 5- &
Ovale (PFO)
year survival was 70.8% D
Stridor inspiration
Mild laryngomalasia Conventional therapy and 63.8%, respectively. E
Chest retraction
Laringoscopy : M shaped V
Prognosis
epiglotis, partially closed the E
airway when inspiration, plica Empirical antibiotic [LoE I] Quo ad vitam: dubia ad malam
Neonatal infection Quo ad functionam: dubia ad L
vocalis difficult to examine
malam O
clinical indicators of possible Quo ad sanam: dubia ad P
early-onset neonatal infection malam M
E
Stimulating – Loving – Caring – Integrated Psychosocial Support N
T
Risk factors & findings Diagnosis & complication Management Prognosis
“Resume”
 Part 1

Part 2  A 2-days-old boy was suspected of having a congenital heart disease  referred to Dr

Kariadi Hospital.

 He had history asphyxia neonatorum (Apgar score 4-6-8) and bluish.

 There was no family history of congenital disease including congenital heart disease.

 The baby was born from a mother with good obstetric history  birth weight 3200 gr

 Cardiovascular system physical examination: normal S1-S2 regular, pan systolic

murmur at spatium inter costal IV linea parasternal dextra grade 3/6 and cyanosis
Part 3
extremities.

 The echocardiography finding was pulmonary atresia with septum ventricle intake
Resume
(PA-SVI), Patent Ductus Arteriosus (PDA), and Patent Foramen Ovale (PFO).
 Part 1
 Diagnosed as cyanotic congenital heart disease
Part 2
 Anatomical diagnosed as pulmonary atresia with intact ventricular
septum (PA/IVS), patent ductus arteriosus (PDA), and patent foremen
ovale (PFO)
 Functionally diagnosed as without heart failure
 Management of congenital heart disease was surgery
 Patient received prostadil (Prostaglandin E1) 60 mcg/kg body weight

Part 3 inside 60 cc NaCl 0,9% (192 mcg /50cc NaCl 0.9%) 0,25 - 3 mL/hour
 Patient planned to referred to Cipto Mangunkusumo Hospital with
Resume preparation to undergo radiofrequency perforation and valvuloplasty
(RFV) + percutaneous balloon pulmonary valvuloplasty.
 Part 1

Part 2

 During 7th day of care (4/12/18)  laryngoscope : mild laryngomalacia.

 Alprostadil flow velocity ↓ to 1 mL/hour
 After 9th day of care (5/12/18) the patient is no longer tachypnea and
bluish
 Patient suggested to be outpatient while waiting the reference process to
Part 3
Harapan Kita Hospital.

Resume
“Thank You”