Persistent Pulmonary Hypertension of the Newborn
(PPHN; Persistent Fetal Circulation [PFC]; Syndrome of Pulmonary Hypertension of Newborn [SPHN])
DefinitionOxygen 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|
|Copyright © Nucleus Medical Media, Inc.|
CausesPPHN 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)
- Severe pneumonia
- 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— oligohydramnios
- Abnormal lung development as a result of congenital diaphragmatic hernia or Potter syndrome
- Stress during pregnancy
- Isolated condition with an unknown cause
Risk FactorsFactors that may increase your baby’s chance of PPHN include:
- Stress to the fetus during pregnancy or delivery
- Health conditions of the mother, including diabetes
- Complications during birth or pregnancy
SymptomsPPHN may cause
- Rapid breathing—tachypnea
- Rapid heart rate— tachycardia
- Difficulty breathing—signs may include flared nostrils or grunting
- Blue tint to the skin, even when the baby is receiving oxygen
DiagnosisYour 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
- Echocardiogram —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
TreatmentTalk 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:
VentilatorA 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 OxideNitric 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.
MedicationsThere 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.
Extracorporeal Membrane Oxygenation (ECMO)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.
PreventionMost 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.
Healthy Children—American Academy of Pediatrics
Nemours Kids Health
Canadian Paediatric Society
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: http://www.childrenscolorado.org/wellness-safety/health-library/for-parents/body-basics/lungs-and-respiratory-system.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.
- Reviewer: Kari Kassir, MD
- Review Date: 08/2014
- Update Date: 09/30/2013