Persistent Pulmonary Hypertension Of The Newborn (PPHN): Causes And Treatment

Persistent Pulmonary Hypertension Of The Newborn

Persistent pulmonary hypertension in the newborn(PPHN) happens when their blood continues to circulate in the same manner as it did while they were in the mother’s womb. In babies with PPHN, the blood circulation does not adapt to breathing outside the womb (1). As a result, too much blood bypasses the baby’s lungs.

PPHN leads to high blood pressure in the baby’s lungs. It leads to reduced oxygen levels in the circulating blood even when the baby is provided 100% supplemental oxygen support. All organs in the body need a regular supply of oxygen-rich blood. If the organs do not get the needed oxygen, they may get damaged.

Read this post to know more about the causes, risk factors, symptoms, treatment, and complications of PPHN in neonates.

How Does PPHN Occur?

The following points explain the pathophysiology of PPHN.

  • While in the mother’s womb, the baby’s lungs do not exchange oxygen and carbon dioxide. The fetus receives oxygen through the umbilical cord, and the lungs need less blood supply.
  • After birth, ideally, the circulation changes when the baby begins to inhale and exhale.
  • This change in lung pressure helps in closing the fetal connections between the heart and the blood vessels. It helps in redirecting the blood to the lungs, and this blood flow to the lungs helps in the gaseous exchange.
  • These changes may not occur in newborns who experience low oxygen levels or troubled breathing at birth. The fetus’ circulation sends most blood away from the baby’s lungs through the connections between the heart and large blood vessels.
  • The blood vessels in the lungs remain constricted, reducing the flow of blood from the lungs. It ultimately leads to high blood pressure in the baby’s lungs and PPHN.

Several factors and conditions before and during a baby’s birth may a play role in causing PPHN. The following are the most probable causes (2).

  • Problems with blood vessels in the lungs
  • Abnormality of heart and lungs, such as diaphragmatic hernia, blocked heart valves, or smaller than usual lungs
  • Difficulty in adjusting to the outside atmosphere after birth
  • Meconium aspiration syndrome
  • Lung or chest infections, such as pneumonia
  • Bloodstream infections

Risk Factors Of PPHN

The following factors may increase a baby’s risk of developing PPHN (1).

  • Difficult birth or birth asphyxia
  • Postmature birth (full-term birth after due date)
  • Maternal treatment with certain medications, such as non-steroidal anti-inflammatory medicines
  • Maternal infections, such as strep infection
  • Babies with pneumonia or heart and lung problems at birth
  • Mothers who experience a break in water during pregnancy too early

Signs And Symptoms Of PPHN

The following signs and symptoms may indicate a probability of PPHN (3).

  • Fast breathing
  • Fast heart rate
  • Grunting or moaning while breathing
  • Flaring nostrils
  • Retraction of the skin between and under the ribs while breathing
  • Bluish discoloration of the skin around the lips, hands, and feet (cyanosis)
  • Heart murmur
  • Low blood oxygen saturation, even when on supplemental oxygen
  • Low blood pressure

These symptoms could be similar to other conditions. Therefore, diagnosis is essential to determine the cause.

Diagnosis Of PPHN

The doctor may suspect an anomaly in the baby’s health due to the symptoms. The following diagnostic tests could be conducted to diagnose PPHN (3).

  • Chest X-rays to visualize lung issues
  • Echocardiogram, a heart ultrasound to check if the baby has any heart problems
  • Blood oxygen level assessment through pulse oximetry
  • Blood test to check for arterial blood gas level

The doctor may also order other blood tests (cell count and infection markers) and lumbar puncture (spinal tap). These tests may be conducted to rule out other problems or further confirm the presence of PPHN.

Treatment ForPPHN

The goal of the treatment is to increase oxygen supply to all of the baby’s organ systems. The baby might have long-term health problems if their body does not get enough oxygen.

The treatment plan depends on the baby’s symptoms, age, and overall health. It also depends on the severity of the condition. The treatment of PPHNmay include the following (4) (5).

1. Supplemental oxygen

The baby is administered supplemental oxygeni n the following ways.

  • Endotracheal tube: It is the most efficient way to provide the child with supplemental oxygen. A tube is put in the baby’s trachea (windpipe).
  • CPAP (continuous positive airway pressure): This machine gently pushes oxygen into the baby’s lungs.
  • Mechanical breathing machine (ventilator): The machine breathes for the baby when the baby cannot breathe.
  • High-frequency oscillation ventilation: This special type of ventilator delivers oxygen through rapid, short bursts of air via a breathing tube.

2. Medications

The following medicines are used to treat PPHN and associated disorders.

  • Blood pressure medicines might be prescribed to keep blood pressure in check.
  • Antibiotics are prescribed to treat and prevent any infection.
  • The baby is administered surfactants via breathing tubes to enable their lungs to exchange oxygen and carbon dioxide better.
  • Nitric oxide is given to the baby as it helps in expanding the blood vessels in the lungs.

3. Extracorporeal membrane oxygenation (ECMO)

ECMO therapy is done when the baby does not respond to other modes of treatment. In ECMO, the baby’s blood is pumped through artificial lungs outside the body. This artificial lung oxygenates the blood and removes carbon dioxide. The oxygenated blood is then sent back to the baby’s body. This procedure is done under special care in some NICU facilities.

The doctor may consider one or multiple treatments depending on the baby’s symptoms and response to treatment. The baby’s lungs may take weeks or even months to recover fully after the treatment.

Can A Baby Outgrow Pulmonary Hypertension?

Some babies may respond well to treatment and show recovery, although they may not have optimum lung function. According to the American Academy of Pediatrics, some children may eventually require lung or heart transplant (6). However, with the latest advancements in medical science, new treatments are available to prevent the disease from progressing and the baby’s condition deteriorating.

Babies with PPHN may require long-term care even when their condition improves. It is essential to protect the baby from catching the common cold and seasonal flu. Good hand hygiene and avoiding large crowds can help in avoiding these infections. In addition, it is essential to get the baby’s health checkup done regularly and get them screened for normal developmental milestones.


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Dr Bisny T. Joseph

Dr. Bisny T. Joseph is a Georgian Board-certified physician. She has completed her professional graduate degree as a medical doctor from Tbilisi State Medical University, Georgia. She has 3+ years of experience in various sectors of medical affairs as a physician, medical reviewer, medical writer, health coach, and Q&A expert. Her interest in digital medical education and patient education made... more