Persistent Pulmonary Hypertension of the Newborn

Persistent Pulmonary Hypertension of the Newborn

Before birth, an unborn baby (fetus) receives oxygen from the placenta and umbilical cord instead of the lungs. During this time, very little blood flows to the baby’s lungs, because the lungs are not yet needed for the baby to get oxygen. When a baby is born and starts breathing normally, the circulation system changes to let more blood flow to the lungs. Persistent pulmonary hypertension of the newborn (PPHN) is when a baby’s circulation system is not able to make this change, resulting in too little blood flowing to the lungs after birth.

PPHN can cause short-term and long-term health problems. It can lead to organ and tissue damage, heart failure, and death. It must be treated immediately.

5 Interesting Facts of Persistent Pulmonary Hypertension of the Newborn

  1. Persistent unsuccessful transition to extrauterine life characterized by sustained suprasystemic pulmonary artery pressures (without the normal drop in pulmonary vascular resistance and increased pulmonary blood flow that normally occurs at birth) and thickened labile pulmonary vasculature (predisposed to vasoconstriction)
  2. Primary type is more common in neonates born at 34 or more weeks of gestation whose mothers took NSAIDs or selective serotonin reuptake inhibitors in the perinatal period. Secondary type can result from a variety of causes, including meconium aspiration syndrome, respiratory distress syndrome, pneumonia and sepsis, other primary pulmonary disorders of infancy, and pulmonary hypoplasia (eg, congenital diaphragmatic hernia, oligohydramnios resulting from renal anomalies or premature rupture of membranes) 
  3. Primary type presents with hypoxemia and respiratory distress shortly after birth in absence of other recognizable parenchymal lung disease 
  4. Typically, infants with this condition have episodic labile periods of hypoxemia; tricuspid regurgitation and a prominent second heart sound may be evident on examination 
  5. Differentiate from meconium aspiration syndrome with history, physical examination, and imaging (a normal chest radiograph and echocardiogram shows right to left extrapulmonary shunting of blood across a patent foramen ovale or patent ductus arteriosus)

What are the causes?

This condition may be caused by:

  • A defect of the heart, arteries, blood, or lungs. Lung problems can be caused by:
    • Meconium aspiration. This is when the baby breathes in his or her first stool (meconium).
    • Pneumonia.
    • Respiratory distress.
  • Blockages or narrowing of structures that affect blood flow.
  • Metabolic conditions, such as having an abnormal blood sugar level or iron level.

Sometimes the cause is not known.

What increases the risk?

Your baby is more likely to develop this condition if he or she:

  • Was delivered by cesarean delivery.
  • Had a late preterm or postterm birth.
  • Is large for his or her age.
  • Has a mother who:
    • Is overweight.
    • Has diabetes.
    • Has asthma.
    • Used certain medicines during pregnancy, such as NSAIDs.

What are the symptoms?

Signs and symptoms include:

  • Severe breathing difficulties.
  • Rapid breathing.
  • Rapid heart rate.
  • Blue or gray skin tone.

How is this diagnosed?

This condition is diagnosed based on:

  • The mother and baby’s medical history and the history of labor and delivery.
  • A physical exam of the baby that is done at birth.
  • Tests, including:
    • A chest X-ray.
    • An ultrasound of the heart, lungs, or head.
    • Blood tests.
    • A test of blood oxygen levels (pulse oximetry).

How is this treated?

Your baby will be treated immediately. The exact treatment will depend on the cause of the PPHN. Your baby may have:

  • Supportive care. This includes keeping your baby warm and calm.
  • Oxygen therapy. This may be given through a mask, hood, throat tube, or ventilation machine.
  • Nutritional support. This may involve giving your baby minerals (electrolytes), controlling blood sugar, or offering other types of support.
  • Medicines. These may be given to relax muscles or blood vessels and improve blood flow and oxygen transport. Some of the medicines may be inhaled. Antibiotic medicines may be given to treat infection.
  • Extracorporeal membrane oxygenation (ECMO). In this treatment, a machine (oxygenator) does the work that the heart and lungs normally do. This may be done if other treatments are not helping.
  • Surgery. This may be done to correct a defect that caused PPHN.

Follow these instructions at home:

  • Provide your baby with a calm environment. This will allow your baby to rest and recover.
  • Feed your baby as directed by your baby’s health care provider.
  • Give your baby over-the-counter and prescription medicines only as told by the health care provider.
  • If your baby was prescribed an antibiotic medicine, give it to him or her as told by the health care provider. Do not stop giving the antibiotic even if your baby starts to feel better.
  • Keep all follow-up visits as told by your baby’s health care provider. This is important.

Contact a health care provider if:

  • Your baby is not feeding well.
  • Your baby has a fever.

Get help right away if:

  • Your baby’s symptoms return.
  • Your baby develops new symptoms.
  • Your baby who is younger than 3 months old has a temperature of 100°F (38°C) or higher.

Summary

  • Persistent pulmonary hypertension of the newborn (PPHN) is when a baby’s blood continues to bypass go around the lungs after birth, instead of flowing to the lungs to get oxygen.
  • PPHN must be treated immediately. Treatment may involve supportive care, nutritional support, oxygen therapy, and medicines. Surgery or extracorporeal membrane oxygenation may also be needed.
  • Give your baby over-the-counter and prescription medicines only as told by your baby’s health care provider.
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