Oxygen is needed in every cell of the body. It first enters the body through the lungs. The oxygen is then picked up by the blood flowing by the lungs. The blood brings oxygen to the rest of the body. In newborns with persistent pulmonary hypertension (PPHN), the blood does not flow by the lungs.
The baby's lungs are not used during pregnancy. Instead, oxygen passes from the mother to the baby through the umbilical cord, bypassing the lungs. Once the baby is born, the baby's lungs should take over. When this does not occur, oxygen bypasses the lungs and cannot move from the lungs to the rest of the body.
PPHN can be a very serious condition. It can cause both immediate and long-term health problems.
Circulatory System of Infant
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PPHN can be caused by a variety of factors, including:
An event or illness during pregnancy or childbirth, such as:
- Meconium aspiration syndrome (the baby inhales meconium—the first stool—prior to or shortly after birth)
- Low blood sugar
- Birth asphyxia (loss of oxygen to the fetus during delivery)
- Respiratory distress syndrome
—caused by lungs that have not fully developed
- Use of nonsteroidal anti-inflammatory drugs (NSAIDs) by the mother during pregnancy
- Late trimester maternal use of antidepressants, especially SSRIs
- Amniotic fluid leak
Low amniotic fluid—
- Abnormal lung development as a result of congenital diaphragmatic hernia or Potter syndrome
- Stress during pregnancy
- Isolated condition with an unknown cause
Factors that may increase your baby’s chance of PPHN include:
- Stress to the fetus during pregnancy or delivery
Health conditions of the mother, including
- Complications during birth or pregnancy
PPHN may cause
- Rapid breathing—tachypnea
Rapid heart rate—
- Difficulty breathing—signs may include 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. Your pregnancy history may also be reviewed. A physical exam will be done.
Tests may include the following:
- Blood tests
- Chest x-rays
—to help diagnose lung disease or enlarged heart
—ultrasound of the heart to show the baby's circulating blood flow
- Head ultrasound
—to look for bleeding in the brain
- Pulse oximetry monitoring—continuous monitoring of oxygen levels in the blood
Talk with your doctor about the best treatment plan for you. Treatment for PPHN is typically administered by a doctor who specializes in newborn illness.
Treatment begins with correcting any related conditions. These conditions can include low blood sugar, low oxygen levels, low blood pressure, and low blood pH. Treatment options include:
A ventilator is a machine that will help your baby breathe. A tube will be placed in the baby's throat. The tube is collected to the ventilation machine. The ventilator will deliver oxygen with gentle pressure. The pressure will help keep the lungs open.
Nitric oxide is a gas. It may be delivered during ventilation. Nitric oxide may relax blood vessels. This will improve the flow of blood in the lungs.
There are a number of new medications that are being reviewed. One example is
sildenafil, which is used to treat
erectile dysfunction. Small studies have shown positive results with sildenafil. However, larger studies are needed to confirm the benefits and safety of the medication.
ECMO is a machine that can take over the job of the lungs. It requires major surgery. ECMO may be done if your child has a severe cases of PPHN that is not responding to other treatments. This procedure is done to take some stress off of your baby's body. It can give your baby some time to heal.
Most cases of PPHN have no clear cause or are caused by uncontrollable events. For these cases there are no clear preventative steps. Some cases of PPHN may be prevented with proper prenatal care and good health of the mother during pregnancy. General tips for a healthy pregnancy include:
- Get good prenatal care. Start as early as possible in pregnancy.
- Eat a healthful diet and take recommended vitamins.
- Avoid smoking, drinking alcohol, and taking drugs.
- Only take medications that your doctor has approved.
- Avoid taking NSAIDs or SSRIs. Talk to your doctor about alternatives that may be safer.
Extracorporeal membrane oxygenation. Cincinnati Children's Hospital website. Available at:
http://www.cincinnatichildrens.org/health/e/ecmo. Updated October 2013. Accessed September 4, 2014.
Kleigman RM, Behrman RE, Jenson HB, Stanton BF, eds.
Nelson Textbook of Pediatrics. 18th ed. Saunders: Philadelphia, PA; 2007.
Lungs and respiratory system. Children's Hospital Colorado website. Available at:
Accessed September 4, 2014.
Persistent pulmonary hypertension of newborn. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated January 30, 2014. Accessed September 4, 2014.
PS Shah, A Ohlsson. Sildenafil for pulmonary hypertension in neonates. Cochrane Database Syst Rev 2007; 3:CD005494.
Last reviewed August 2014 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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