PERSISTENT PULMONARY HYPERTENSION SLIDES WILL BE USEFUL FOR NURSING STAFF WHO WORKS IN NICU

1. PERSISTENT PULMONARY
HYPERTENSION(PPHN)
PRESENTED BY:GILY JACOB
CRN NICU

2. PERSISTENT PULMONARY HYPERTENSION OF THE
NEWBORN (PPHN) OCCURS WHEN PULMONARY
VASCULAR RESISTANCE (PVR) REMAINS
ABNORMALLY ELEVATED AFTER BIRTH, RESULTING
IN RIGHT-TO-LEFT SHUNTING OF BLOOD THROUGH
FETAL CIRCULATORY PATHWAYS. THIS IN TURN
LEADS TO SEVERE HYPOXEMIA THAT MAY NOT
RESPOND TO CONVENTIONAL RESPIRATORY
SUPPORT.
INTRODUCTION

3. FETAL CIRCULATION

4. TRANSITION AFTER FETAL PERIOD

5. PATHOGENESIS
EPIDEMIOLOGICAL ASSOCIATIONS
PPHN OCCURS AT A RATE OF 1 TO 2 1,000 LIVE BIRTHS
AND IS MOST COMMON IN FULL TERM AND POST TERM INFANTS .
THREE TYPES OF PULMONARY VASCULATURE UNDERLIE THE DISORDER
THAT ARE
UNDERDEVELOPMENT
MALDEVELOPMENT
AND
MALADAPTATION

6. UNDERDEVELOPMENT
UNDERDEVELOPMENT OCCURS WITH PULMONARY HYPOPLASIA
IN DISEASE CONDITIONS SUCH AS
CONGENITAL DIAPHRAMATIC HERNIA
CONGENITAL PULMONARY MALFORMATION
FETAL GROWTH RETARDATION

7. PPHN CAUSED BY VASCULAR MALDEVELOPMENT INCLUDE
POST TERM DELIVERY ,
MECONIUM STAINING ,AND
MECONIUM ASPIRATION SYNDROME
IN THESE DISORDER PULMONARY VASCULATURE RESPONDS POORLY TO
STIMULI THAT NORMALLY DECREASE IN PVR ,SUCH AS INCREASED
ALVEOLAR OXYGEN TENSION AND THE ESTABLISHMENT OF EFFECTIVE
VENTILATION.
MALDEVELOPMENT

8. MALADAPTATION
IN MALADAPTATION ,THE PULMONARY VASCULAR BED IS NORMALLY
DEVELOPED .
HOWEVER ,ADVERSE PERINATAL CONDITIONS
CAUSE ACTIVE VASOCONSTRICTION AND
INTERFERE WITH THE NORMAL POSTNATAL FALL IN PVR
SUCH AS
PERINATAL ASPHYXIA
PULMONARY PARENCHYMAL DISEASE
BACTERIAL INFECTION (GBS)

9. POSSIBLE RISK FACTORS
• HYPOTHERMIA
• POLYCYTHEMIA
• RDS (RESPIRATORY DISTRESS SYNDROME)
• TRANSIENT TACHYPNOEA OF NEWBORN

10. CLINICAL MANIFESTATIONS
•NEONATAL FINDINGS
• WITHIN 24 HRS OF LIFE NEONATE START TO DEVELOP
RESPIRATORY DISTRESS SUCH AS
TACHYPNOEA,RETRACTION,GRUNTING AND CYANOSIS AND
SEVERE DESATURATION
CHEST EXAMINATION
• AUDIBLE MURMER
• IF MECONIUM STAINING EXISTS CRACKLES OR WHEEZING MAY
BE PRESENT
• IN SEVERE CASE MYOCARDIAL DYSFUNCTION MAY MANIFEST
AS SYSTEMIC HYPOTENSION

11. DIAGNOSIS
• PHYSICAL EXAMINATION
• BABYS WITH PPHN WILL DEVELOP RESPIRATORY SYMPTOMS SUCH AS
TACHYPNOEA,RETRACTION,GRUNTING AND CYANOSIS .
• PULSE OXYMETRY ASSESSMENT –IT GENERALLY DEMONSTRATE
10% DIFFERENCE IN PRE AND POST DUCTAL SATURATION
• ARTERIAL BLOOD GAS EXAMINATION-ARTERIAL BLOOD GAS WILL
SHOW LOW PARTIAL PRESSURE OF OXYGEN LESS THAN 100 EVEN WITH 100%OF INSPIRED
OXYGEN CONCENTRATION

12. OXYGEN INDEX
• WHAT IS OXYGEN INDEX –IT IS USED TO ASSESS THE
HYPOXEMIA IN PPHN
• OI =(MEAN AIRWAY PRESSURE X FIO2 ÷ PAO2 ) × 100
• OXYGEN INDEX ≥ 15-25 CONSIDERED AS SEVERE
HYOXEMIC RESPIRATORY FAILURE

13. ECHO CARDIOGRAPHY
• ECHO CARDIOGRAPHY - THE DEFINITIVE DIAGNOSIS
IS MADE BY ECHO CARDIGRAPHY.
IT CAN PROVIDE ESTIMATION OF SEVERITY OF PULMONARY
HYPERTENSION
• Estimation of right ventricle pressure (RVp)
TR jet and/or changes in septal position, is compared with systemic
blood pressure (BP),
and the degree of atrial and/or patent ductus arteriosus shunting is
determined.
Estimations of severity are as follows:
• •Mild to moderate PPHN – Estimated RVp is between one-half to
three-quarters systemic BP
• •Moderate to severe PPHN – Estimated RVp is greater than three-
quarters systemic BP but less than systemic BP
• •Severe PPHN – Estimated RVp greater than systemic BP

14. MANAGEMENT
TREAT UNDERLYING CAUSE
 SURFACTANT THERAPY-FOR PRIMARY LUNG PARANCHYMAL
DISEASE SUSPECTED SURFACTANT DEFICIENCY OR MECONIUM
ASPIRATION SYNDROME
ANTIBIOTIC ADMINISTRATION –TO TREAT GBS

15. GENERAL SUPPORTIVE CARE
MECHANICAL VENTILATOR SUPPORT-HYPERCARBIA AND
ACIDOSIS WILL INCREASE PVR .
FLUID THERAPY AND INOTROPIC AGENTS FOR CIRCULATORY
SUPPORT-IN PPHN RIGHT TO LEFT SHUNT WILL INCREASE AS
CARDIAC OUTPUT AND BLOOD PRESSURE DECREASE ,SO
ADQUATE FLUID VOLUME MUST BE MAINTAINED .SYSTEMIC
BLOOD PRESSURE MUST BE MAINTAINED AND SUPPORTED
WITH INOTROPES SUCH AS DOPAMINE ,DOPUTAMINE AND
ADRENALINE

16. SEDATION-PAIN AND AGITATION WILL RELEASE
CATACHOLAMINE WHICH WILL INCREASE PVR AND
RIGHT TO LEFT SHUNTING .THEARAPEUTIC CHOICE
INCLUDE MORPHINE AND FETANYL INFUSION

17. VASODILATORS
• INHALED NITRIC OXIDE THERAPY
• EFFICACY — INHALED NITRIC OXIDE (INO) IMPROVES OXYGENATION BY CAUSING
RELAXATION OF VASCULAR SMOOTH MUSCLE AND REDUCES THE NEED FOR
ECMO IN TERM AND LATE PRETERM INFANTS WITH SEVERE PPHN (DEFINED AS
AN OI ≥15 -25 )
• THE RECOMMENDED INITIAL DOSE IS 20 PPM .

18. SILDENAFIL — Sildenafil, a phosphodiesterase
inhibitor type 5, is an agent that has been shown to
selectively reduce pulmonary vascular resistance
MILIRINONE-it has both inotropic and vasodilative
action

19. ECMO –EXTRACORPOREALMEMBRANE
OXYGENATION
• patients who fail to respond to iNO, ECMO therapy
should be considered. The goal of this treatment is
to maintain adequate tissue oxygen delivery and
avoid irreversible lung injury from mechanical
ventilation while PVR decreases and pulmonary
hypertension resolves.

20. PPHN NURSING CARE
• MINIMIZE STIMULATION
• MAINTAIN ADEQUATE RESPIRATORY STATUS
• MAINTAIN ADEQUATE NUTRITION AND HYDRATION STATUS
• CLOSE MONITORING OF VITAL SIGNS

21. OUTCOME
• The estimated mortality rate in developed countries is
between 7 and 10 percent. All survivors of PPHN are at risk for
postdischarge mortality and morbidity. All infants with severe
PPHN who have been treated with inhaled nitric oxide (iNO)
and/or extracorporeal membrane oxygenation (ECMO) should
have neurodevelopmental follow-up .

22. REFERENCE
• UPTODATE
• MANUAL OF NEONATAL CARE -SIXTH EDITION