PPHN (Persisten

Pulmonary Hipertension
of newborn) and CPAP
Pediatric department of RSD Ryacudu Kotabum
Lampung Utara, Lampung
Febrian Pramana P dr
Ismi citra ismail dr SpA

Main part of different fetal and

newborn Circulation system
Duktus Venosus
Duktus Arteriosus
Foramen Ovale

Differential
Janin : VS + VD moving together
Neonatus : VS moving faster than VD
Janin : VD Push high pressure of sisytemic system(tekanan
sistemik)
VS push lower pressure (plasenta)
Neonatus : VD push high pressure of systemic
system(tekanan paru)
VS push lower pressure(tekanan sistemik)

Anatomi sirkulasi janin dan

neonatus

PPHN
Definisi : Classically, PPHN is defined as a failure of the
normal postnatal fall in pulmonary vascular resistance
which leads to persisting right to left shunts across the
fetal channels and resultant hypoxia

Ethiology of PPHN
PPHN can be:
Idiopathic _ 20%
Associated with a variety of lung diseases:

Meconium aspiration syndrome (50%)

Pneumonia/sepsis (20%)
RDS (5%)
Congenital diaphragmatic hernia (CDH)
Others: Asphyxia, Maternal diabetes, Polycythemia
PPHN

Pathophysiology
PPHN is more common in term & near-term (> 34 wks G/A)
neonates.

The development of smooth muscle around the small pulmonary arterioles

in late gestation (> 28 wks) may predispose term infants to increased
resistance to the pulmonary flow.

PPHN can result from either:

Underdevelopment
Maldevelopment
Functional maladaptation of pulmonary vasculature
PPHN

1- Underdevelopment
Underdevelopment of the pulmonary vasculature is
observed in:

Renal agenesis
Thoracic dystrophy
Alveolar-capillary dysplasia
Pulmonary hypoplasia or dysplasia.

PPHN

2- Maldevelopment
Maldevelopment of the pulmonary vasculature
This vascular muscular hypertrophy encroaches on the vascular
lumen and obstructs blood flow.
Example:
Chronic stress, increased blood flow in utero

PPHN

3- Maladaptation
Maladaptation of pulmonary vasculature:

Acidosis,
Hypoxia,
Hypercarbia,
Aspiration,
Hypothermia,
Hypoglycemia,
Hemorrhage.

PPHN

10

Clinical Presentation
Asphyxia
Tachypnea, respiratory distress
Loud, single second heart sound (S2) or a harsh systolic murmur

(secondary to tricuspid regurgitation)

Low Apgar scores
Meconium staining
Cyanosis; poor cardiac function and perfusion
Systemic hypotension
Symptoms of shock

Lab test and imaging

Arterial blood gas levels (through indwelling line): To assess the pH, partial pressure of carbon
dioxide in arterial gas (PaCO2), and the partial pressure of oxygen (PaO2)

Complete blood count with differential: To evaluate for high hematocrit level (polycythemia and

hyperviscosity syndrome may lead to or exacerbate PPHN); to determine whether an

underlying sepsis or pneumonia is present
Coagulation studies (eg, platelet count, prothrombin time, partial thromboplastin time,
international normalized ratio): To assess for coagulopathy (increased disease severity)
Serum electrolytes (eg, calcium) and glucose levels
Preductal and postductal oxygen saturation measurements via pulse oximetry to assess for
differential cyanosis
Echocardiography with Doppler flow: To assess presence/direction of the intracardiac shunt at
the ductus arteriosus and foramen ovale, as well as estimate the pulmonary arterial
systolic/diastolic pressures

Management Strategies
Oxygen
High enough to act as vasodilator, not just oxygenator

Ventilation
To keep CO2 normal
Studies of Hyperventilation showed immediate improvement but increased lung damage,
decreased hearing

Studies of hypoventilation not ready yet

Jet less PIP to achieve the desired paCO2

BP
High normal To reduce differential pressure between pulmonary and systemic
circulations

Continuous Positive
Airway Pressure

What Does It Do?

Assists in reducing alveolar collapse at end exahalation
Increases the mean airway pressure
Reduces the incidence of obstructive and central apneas

Ways to Deliver CPAP

Mechanical ventilator
High-flow nasal cannula
Nasal prong CPAP device

Airway Devices

Long or short nasal prongs

Tracheostomy tube
Nasopharyngeal tube
Mask
Endotracheal tube

Bubble CPAP

A fluid-filled reservoir is used as a means of maintaining

the desired level of CPAP
Provides a natural pop-off
Oscillations in the circuit have been speculated to aid in
ventilation
Simple, inexpensive, and dependable

Clinical Application

The correct size nasal prongs will be those which

completely fill the lumen of the nares without stretching
them.
Too small of prongs will necessitate the need for an
increased flow setting which leads to internal swelling of
the nasal passages
Too large of prongs will lead to pressure sores and
necrosis

CPAP Component
PIP : Peak inspiratory pressure
PEEP : Positive end expiratory pressure
FiO2 : Oksigen friksion
Air
O2

Assessment of Function

Maintain a fine mist of condensation in the prongs

Maintain the proper fluid level in the reservoir
Use the lowest flow setting which will allow for steady
bubbling in the reservoir

Patient Management

Maintain patency of the nasal passages

Prongs should point posteriorly along the palate
Take steps to reduce pressure sores
The patients head should be in a neutral position
Take steps to reduce gastric insufflation

Daftar Pustaka
Kinsella JP, Abman SH. Recent developments in the
pathophysiology and treatment of persistent pulmonary
hypertension of the newborn. J Pediatr 1995; 126:853-64.
European Respiratory Journal, vol. 15 2000 Effects of
biphasic positive airway pressure in patients with chronic
obstructive lung disease