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Persistent Pulmonary Hypertension of the Newborn
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Persistent Pulmonary Hypertension of the Newborn

(PPHN)

En Español (Spanish Version)

Definition

Persistent pulmonary hypertension (PPHN) of the newborn is a relatively rare, yet potentially very serious condition. It can cause both immediate and long-term complications and health concerns. PPHN affects approximately one in every 500-1500 births.

When a baby is in the womb, the oxygen is supplied through the umbilical cord. After birth, the baby's system should switch to receive oxygen from the lungs. In babies born with PPHN, the heart, blood vessels, and lungs (circulatory system) do not make the adjustment. When babies with PPHN are born, the blood does not interact with the lungs, and instead circulates as it did in the womb.

Babies with PPHN do not receive the necessary oxygen from the lungs that is normally supplied.

Symptoms of PPHN typically appear within 12 hours after birth.

Circulatory System of Infant

AL00079-B_97870_1_ciruclatory_infant

© 2008 Nucleus Medical Art, Inc.

Causes

PPHN can be caused by a variety of factors, including:

  • An event or illness during pregnancy or childbirth
    • Meconium aspiration syndrome (the baby inhales the meconium—its first stool—prior to or shortly after birth)
    • Severe pneumonia
    • Infection
    • Low blood sugar
    • Birth asphyxia (loss of oxygen to the fetus during delivery)
    • Respiratory distress syndrome
    • Maternal use of nonsteroidal anti-inflammatory medications
    • Late trimester maternal use of selective serotonin reuptake inhibitors
    • Amniotic fluid leak
    • Low amniotic fluid (oligohydramnios)
    • Abnormal lung development as a result of congenital diaphragmatic hernia
  • Stress during pregnancy
  • Isolated condition with an unknown cause

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.

The following factors may increase your baby’s chance of developing PPHN:

  • Stress to the fetus during pregnancy or delivery
  • Health conditions of the mother, including diabetes
  • Complications during birth or pregnancy

Symptoms

Symptoms include:

  • Tachypnea (rapid breathing)
  • Tachycardia (rapid heart rate)
  • Difficulty breathing or other symptoms of respiratory difficulties, including flared nostrils or grunting
  • Blue tint to the skin, even when the baby is receiving oxygen

Diagnosis

Your doctor will ask about your baby’s symptoms and medical history, and perform a physical exam.

Tests may include the following:

  • Chest x-rays to diagnose lung disease or enlarged heart
  • Echocardiogram (ultrasound of the heart) to show the baby's circulating blood flow
  • Head ultrasound to diagnose bleeding in the brain
  • Laboratory tests
    • Arterial blood gas (ABG)
    • Complete blood count (CBC)
    • Serum electrolyte tests
    • Pulse oximetry (monitoring the percentage of hemoglobin saturated with oxygen)

Treatment

Talk with your doctor about the best treatment plan for you. Treatment for PPHN is typically administered by a neonatologist, a doctor who specializes in newborn illnesses. Treatment begins with correcting any predisposing condition such as low blood sugar, administering oxygen, preventing low blood pressure, and correcting low blood pH. Treatment options include:

Ventilator

To increase the amount of oxygen to the baby's lungs, a tube may be placed directly into the trachea. A ventilator administers the oxygen into the tube, and breathes for the baby.

Nitric Oxide

Inhaled nitric oxide may relax blood vessels and improve circulation.

Medications

There are a number of novel medication strategies that are currently under investigation. For example, sildenafil (eg Viagra®) has been studied in small numbers of patients with overall positive results. However, studies with larger numbers are needed to confirm the drug’s effectiveness and safety.

Extracorporeal Membrane Oxygenation (ECMO)

Extracorporeal membrane oxygenation requires major surgery. It is typically only performed on the most serious cases of PPHN when the patient has not responded to other treatments. In ECMO, a machine acts as an artificial heart and lungs to the baby so that the natural organs can heal.

Prevention

Because in many cases the cause of PPHN is unknown or uncontrollable, there is no completely effective method of preventing PPHN. However, proper prenatal care and good health of the mother during pregnancy can reduce the risk of some causes of PPHN.

RESOURCES:

Lucile Packard Children's Hospital
http://www.lpch.org

University of California, San Francisco
http://www.ucsfhealth.org

CANADIAN RESOURCES:

British Columbia Ministry of Health
http://bchealthguide.org

Canadian Medical Association Journal
http://www.cmaj.ca

References:

Kleigman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Saunders: Philadelphia, PA; 2007.

Mayock D. Persistent pulmonary hypertension of the newborn. University of Washington Academic Medical Center website. Available at: http://depts.washington.edu/nicuweb/NICU-WEB/pphn.stm#Author. Accessed May 30, 2007.

Persistent pulmonary hypertension of the newborn (PPHN). KidsHealth for Parents: Medical Problems. The Nemours Foundation website. Available at: http://www.kidshealth.org/parent/medical/lungs/pphn.html. Accessed May 30, 2007.

Persistent pulmonary hypertension research. Neonatology (Neonatal intensive care unit). Children's Memorial Hospital website. Available at: http://www.childrensmemorial.org/depts/neonatology/research.asp. Accessed May 30, 2007.

PS Shah, A Ohlsson. Sildenafil for pulmonary hypertension in neonates. Cochrane Database Syst Rev 2007; 3:CD005494.



Last reviewed April 2008 by Kari Kassir, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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