Persistent pulmonary hypertension of the newborn (PPHN)

persistent-pulmonary-hypertension-of-the-newborn-2x

Overview

Persistent pulmonary hypertension of the newborn, or PPHN, occurs when a newborn's circulation system doesn't adapt to breathing outside the womb.

While in the womb, the fetus receives oxygen through the umbilical cord, so the lungs need little blood supply. There is high blood pressure in the lungs, so blood in the pulmonary artery is sent away from the lungs to the other organs through a fetal blood vessel, called the ductus arteriosus.

Normally, when a baby's born and begins breathing, the blood pressure in the lungs falls and blood flow to the lungs increases. Oxygen and carbon dioxide are exchanged in the lungs, then the blood is returned to the heart and pumped back out to the body. The ductus arteriosus constricts and permanently closes in the first day of life.

In babies with PPHN, the pressure in the lungs stays high and the ductus arterious remains open, allowing blood to be directed away from the lungs. PPHN is a rare but life-threatening condition that appears most often in full-term or post-term babies who have had a difficult birth or conditions such as infection or birth asphyxia.

Signs & symptoms

The signs and symptoms of persistent pulmonary hypertension of the newborn may include:

  • Rapid breathing, also called tachypnea
  • Rapid heart rate
  • Respiratory distress, including signs such as flaring nostrils and grunting
  • Cyanosis, a condition in which the baby's skin has a bluish tint even while he or she is receiving extra oxygen
  • Heart murmur, where a baby has an extra or abnormal heart sound
  • Low oxygen levels (a baby with PPHN may continue to have low oxygen levels in the blood even while receiving 100 percent oxygen)

Diagnosis

Various imaging and laboratory tests can help determine if a baby has persistent pulmonary hypertension of the newborn. These may include:

  • Chest X-rays to determine if the baby has lung disease or an enlarged heart
  • Echocardiogram — an ultrasound of the heart — to determine if the baby has heart or lung disease and evaluate blood flow in those organs
  • Ultrasound of the head to look for bleeding in the brain
  • Arterial blood gas (ABG) to determine how well oxygen is being delivered to the body
  • Complete blood count (CBC) to measure the number of oxygen-carrying red blood cells, white blood cells and platelets.
  • Serum electrolyte tests to evaluate the balance of minerals in the blood
  • Lumbar puncture, also called a spinal tap, and blood tests to help determine whether the baby has an infection
  • Pulse oximetry, which measures oxygen levels in the blood, to help monitor whether the baby's tissues are receiving enough oxygen

Treatment

In treatment for PPHN, the main goal is to increase oxygen flow to the baby's organs to prevent serious health problems. Treatment may include a wide range of mechanical ventilation and respiratory therapy options such as:

  • Oxygen — 100 percent supplemental oxygen may be given to your baby through a mask or plastic hood.
  • Assisted ventilation — During this procedure, a tube is inserted into your baby's windpipe, a ventilator takes over your baby's breathing and oxygen is given.
  • Nitric oxide — Research has shown that this gas is effective in treating PPHN because it relaxes contracted lung blood vessels and improves blood flow to the lungs. It is given through the ventilator.
  • High frequency oscillatory ventilation — This type of ventilation may improve the oxygen level in the blood if other types of ventilation aren't effective.

Extracorporeal Membrane Oxygenation (ECMO)

In addition, an extracorporeal membrane oxygenation (ECMO) machine may be used for babies who are experiencing serious heart or lung failure. The ECMO delivers oxygen to the brain and body as temporary support while the PPHN resolves. ECMO is similar to a heart-lung bypass machine, which takes over your baby's heart and lung functions with an external pump and oxygenator. Blood is drained from the baby to an artificial lung, where oxygen is added and carbon dioxide is removed, then the blood is pumped back into your child.

At UCSF Benioff Children's Hospital, we treat over 20 patients each year with ECMO and our success outcomes are among the highest in the country. Our team includes a neonatologist, surgeon, respiratory therapists and nurses with special training in the management of ECMO.

UCSF Benioff Children's Hospitals medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your child's doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your child's provider.

Where to get care (2)

life-clinic-320x320-2x

LIFE Clinic

LIFE Clinic

1825 Fourth St., Fifth Floor
San Francisco, CA 94158

neonatal-intensive-care-unit-nicu-320x320-2x 2

Neonatal Intensive Care Nursery

Neonatal Intensive Care Nursery

San Francisco / Oakland

Recommended reading

intensive-care-nursery-glossary-2x

Intensive Care Nursery Glossary

Our glossary explains the terms we use in the intensive care nursery in language that's easy to understand. Learn about chest tubes, respirators and more.

intensive-care-nursery-parents-guide-2x

Intensive Care Nursery Parents' Guide

Is your baby in the intensive care nursery? Our guide helps you take an active role in your child's care, know what to expect and access support services.

Support services

Family Amenities

Family-friendly amenities help you relax and take care of yourself while staying close to your child. We offer lounges, kitchens, showers, breastfeeding rooms and more.

glogau-teddy-bear-rescue-fund-2x

Glogau Teddy Bear Rescue Fund

Families that need additional assistance during their child's hospital stay have access to toys, hotel vouchers and other amenities. Find out more.

interpreters-2x

Interpreter Services & Communication Assistance

Interpreter services in many languages and TDDs are available for families that need help communicating with care teams. Here's how to access them.

patient-relations-2x

Patient Relations

Patient relations reps and nursing supervisors are here to answer questions and address concerns. Learn about your rights, how to reach us and more.

social-services-2x

Social Services

Social workers ease the effect of illness, injury and hospitalization on your family with counseling and assistance to help you navigate the challenges.

spiritual-care-2x

Spiritual Care

Chaplains help UCSF patients, families and staff cope with the spiritual and emotional challenges of childhood illness. Learn more.

Awards & recognition

  • Ranked among the nation's best in 10 specialties

Saving future lives

500+ UCSF investigators are researching cures for hundreds of childhood and adult diseases.

Share