Persistent Pulmonary Hypertension of the Newborn: Key Signs


Also known as persistent fetal circulation syndrome, persistent pulmonary hypertension of the newborn (PPHN) is a severe breathing condition where the lung vessels (pulmonary vasculature) are not open enough. As a result, the baby's blood and oxygen flow is restricted. Possible long-term PPHN complications include kidney failure, hearing deficits, brain hemorrhage, and pulmonary (lung) disease.

If you suspect that your newborn has PPHN, contact your child's medical team right away. You should also contact a birth injury lawyer at The Moore Law Firm to learn more about your legal options.

Read on to learn more about PPHN, what causes it, how it's diagnosed, and the potential long-term effects and complications of severe PPHN. You will also learn how to receive trusted legal advice if your newborn is diagnosed with it.

What Is Persistent Pulmonary Hypertension of the Newborn and What Causes It?

While in the womb, the baby is given maternal oxygen through the placenta. The infant's lungs are still developing and unused, so the vessels that let blood flow to the lungs are closed. After birth, the blood vessels should open when the baby takes their first breath, allowing blood to circulate into the lungs so the baby can start breathing independently.

PPHN occurs when a baby's lung blood vessels do not open fully after birth to allow for normal blood circulation. Blood vessels that are closed can cause blood to bypass the lungs, depriving the body and brain of oxygen. This is called shunting or abnormal blood movement. 

Closed blood vessels in the lungs can also cause too much pressure to accumulate in the blood vessels to the lungs, which can hurt the baby's lungs and heart.

The cause of PPHN is unknown, but certain factors increase the risk of PPHN. These include:

  • Alveolar capillary dysplasia (ACD) with misalignment of lung vessels
  • Chronic lung disease is a disorder that affects the lungs and other parts of the respiratory system
  • Exposure to selective serotonin reuptake inhibitors (SSRIs) during late gestation — one study has shown that SSRI exposure is associated with a six-fold increase in PPHN
  • Diaphragmatic hernia is a hole in the diaphragm, the muscle that separates the abdomen from the chest. A hernia is a gap in the diaphragm that allows the organs from the abdomen to spill into the chest
  • Infection
  • Lack of oxygen during or before birth
  • Low birth weight — premature infants are particularly susceptible to PPHN
  • Meconium aspiration syndrome is when the baby breathes in feces mixed with amniotic fluid while still in the womb
  • Pre- and post-birth medical malpractice
  • Respiratory distress syndrome (RDS) is a breathing problem that happens in babies who do not have fully developed lungs

PPHN usually happens to full-term babies or babies who were born at 34 weeks or more. It is not a common birth injury as it only affects 2 out of every 1,000 births.

Diagnosing PPHN: Signs and Symptoms

PPHN symptoms can resemble those of other conditions. If you believe that your child has PPHN, contact the child's health care team for an accurate diagnosis. 

Doctors will use echocardiograms (heart ultrasounds), chest x-rays, pulse oximeters, and other tools to perform a PPHN diagnosis. They will look for common PPHN symptoms, such as cyanosis, rapid breathing and heart rate, severe hypoxemia, and low blood pressure throughout the body.

Cyanosis (Blue-Looking Skin and Lips at Birth)

Cyanosis refers to a purplish-blueish hue to the skin. It indicates that there may be decreased oxygen attached to red blood cells in the baby's blood, suggesting a problem with the heart and lungs.

Rapid Breathing and Heart Rate

Infants with PPHN have rapid breathing and heart rates due to difficulties breathing and decreased oxygen in the blood.

Severe Hypoxemia (Low Blood Oxygen Levels)

Another sign of PPHN is severe hypoxemia or low blood oxygen levels. Severe hypoxemia causes symptoms like breathing difficulties, headaches, bluish skin, and a rapid heart rate. Doctors will use a pulse oximeter to measure your child's oxygen saturation levels.

Low Blood Pressure Throughout Body

Infants with PPHN can have low systemic blood pressure. Doctors will use a leg cuff to measure your child's blood pressure.

Potential Long-Term Effects and Complications of Severe PPHN

Severe PPHN can cause several long-term effects and complications. PPHN long-term effects may include:

  • Brain hemorrhage
  • Cerebral palsy
  • Hearing deficits
  • Heart failure
  • Kidney failure
  • Oxygen dependence
  • Pulmonary disease
  • Seizures
  • Stroke

Even with the appropriate treatments, 5 to 10% of infants with PPHN die. Additionally, 25% of infants with moderate or severe PPHN will experience significant neurodevelopment impairment at 12 to 24 months.

Receive Trusted Legal Advice if Your Newborn Infant Is Diagnosed With PPHN

Treatments for PPHN include:

  • Blood pressure support, such as intravenous medicine
  • Endothelin receptors blockers like Bosentan
  • Hydrocortisone treatment to increase cGMP levels
  • Medicine for treating PPHN-related infections and relaxing your baby's muscles, which makes your baby respond better to the breathing machine
  • Supplemental oxygen through a mask, plastic hood, or prongs in the nose
  • An endotracheal (ET) tube to help your baby breathe
  • A mechanical breathing or ventilation machine
  • Extracorporeal membrane oxygenation (ECMO)

These treatments can be prohibitively expensive. This is especially true if your PPHN newborn develops life-long complications. For example, if your child develops hearing deficits as a result of PPHN, they may need hearing aids and speech therapy. Similarly, if they developed cerebral palsy due to PPHN, they may need a wheelchair and physical therapy. You may also need to remodel your home for accessibility.

That's where The Moore Law Firm's knowledgeable Cincinnati pre- and post-birth medical malpractice lawyers come in. Tenacious, compassionate, and savvy, we will thoroughly analyze your case to see if you have a valid birth injury lawsuit

If we determine that preventable accidents caused your child to develop PPHN, we can help you file a lawsuit against the responsible parties. We will fight to recover the maximum financial compensation for your child's injuries and pain and suffering. 

Interested in learning more about how we can help? Request a free consultation today by calling 513-232-2000. We represent medical negligence victims on a contingency fee basis, so you don't have to pay unless we win your case.

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If you have been injured or have lost a loved one as a result of another person's negligence, you deserve to be fully compensated for your losses. The simple fact is that you should not be forced to pay the price for another person's careless or reckless actions.