Persistent pulmonary hypertension (PPHN) of the newborn is a relatively rare, yet potentially very serious condition that can cause both immediate and long-term complications and health concerns. PPHN affects approximately one in every 500-1500 births.
In babies born with PPHN, the circulatory system does not make the adjustment from receiving oxygen through the umbilical cord—as what happens in the womb—to using the lungs to get air. When babies with PPHN are born, the blood that circulates bypasses the lungs, and instead circulates as it did in
utero
. When the lungs don’t receive this blood flow, the oxygen that is normally passed to the lungs through the blood is lacking.
Babies with PPHN do not receive the necessary oxygen to the lungs that is normally supplied by a properly functioning circulatory system.
Symptoms of PPHN typically appear within 12 hours after birth.
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
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 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
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)
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:
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.
Inhaled nitric oxide may relax blood vessels and improve circulation.
Extracorporeal membrane oxygenation requires major surgery; 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.
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.
Last reviewed August 2007 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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