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Persistent Pulmonary Hypertension of the Newborn

(PPHN; Persistent Fetal Circulation [PFC]; Syndrome of Pulmonary Hypertension of Newborn [SPHN])

Definition

Oxygen passes from a mother to a baby through the umbilical cord before birth. A baby's lungs should take over after birth. Persistent pulmonary hypertension of the newborn (PPHN) is when this does not happen and blood flows away from the lungs because of high blood pressure in the arteries that go to the lungs. The baby will not get enough oxygen. This is a serious problem that can cause long-term health problems.

Circulatory System of Infant
AL00079-B_97870_1_circulatory_infant

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Causes

The exact cause is not always known. It may be caused by

  • Meconium aspiration syndrome—the baby inhales the first stool prior to or shortly after birth
  • Respiratory distress syndrome —caused by lungs that have not fully developed
  • Lack of oxygen during delivery
  • Severe infection, such as pneumonia
  • Low blood sugar
  • Birth defects of the heart and lungs

Risk Factors

This problem is more common in full term babies. It is also more common in premature babies who have health problems that affect breathing. Other things that may raise a baby's risk are:

  • Stress to the fetus during pregnancy or delivery
  • Health conditions of the mother, such as diabetes

Symptoms

PPHN may cause:

  • Problems breathing, such as flared nostrils or grunting
  • Fast breathing
  • Fast heart rate
  • Blue skin color

Diagnosis

A physical exam will be done. Blood tests will also be done. The baby's oxygen levels will be checked. This can be done with pulse oximetry monitoring. This is often enough to make the diagnosis.

Images will be taken of the heart to confirm the diagnosis. This can be done with an echocardiogram.

Treatment

The goal of treatment is to increase oxygen to the baby. This can be done with:

  • Extra oxygen to relax blood vessels to the lungs and improve blood flow to them
  • Breathing support in babies who cannot breathe on their own

Other methods may be:

Medicine

Medicine may be given to relax blood vessels and improve blood flow.

Extracorporeal Membrane Oxygenation (ECMO)

ECMO is a machine that can take over the job of the lungs. It requires major surgery. It may be done in babies with severe PPHN who do not respond to other treatments.

Prevention

There are no known ways to prevent PPHN.

RESOURCES:

Healthy Children—American Academy of Pediatrics
http://www.healthychildren.org

Kids Health—Nemours Foundation
http://www.kidshealth.org

CANADIAN RESOURCES:

Canadian Paediatric Society
http://www.cps.ca

Health Canada
https://www.canada.ca

REFERENCES:

Nair J, Lakshminrusimha S. Update on PPHN: mechanisms and treatment. Semin Perinatol. 2014 Mar;38(2):78-91.

Persistent pulmonary hypertension of the newborn (PPHN). EBSCO DynaMed website. Available at:https://www.dynamed.com/condition/persistent-pulmonary-hypertension-of-the-newborn-pphn. Updated January 23, 2019. Accessed January 3, 2020.

Last reviewed September 2019 by EBSCO Medical Review Board Kari Kassir, MD  Last Updated: 1/3/2020