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Up until the early 1990s, the outlook for infants with persistent pulmonary hypertension in newborns (PPHN) was bleak. Overall mortality rates were estimated at 40 percent, and as many as 60 percent of survivors suffered some major disability.
Thanks to medical research and improved treatment methods, the prognosis for PPHN sufferers is much brighter today. However, PPHN is still a serious and potentially fatal disorder.
The most common and serious complications of PPHN result from an inadequate supply of oxygen over a prolonged period of time. This lack of oxygen is the primary factor in heart failure and death due to PPHN. Infants who survive may experience long-term complications due to acute medical problems, such as:
One long-term complication of PPHN is bronchopulmonary dysplasia, a chronic lung disease in which scarring causes the lungs to stiffen, resulting in severe breathing problems. Other conditions associated with PPHN include seizure disorders, developmental delays and various neurological problems.
During treatment for PPHN, some infants may be unable to take feedings by mouth. In such cases, a temporary feeding tube may be inserted through the nose or directly into the stomach to deliver the nutrients the baby needs to survive and grow.
Hearing problems are common in survivors of PPHN. Infants with PPHN should be evaluated in early childhood for hearing loss and speech problems.
Without question, medical treatments such as high frequency ventilation, nitric oxide, and ECMO have greatly reduced the number of deaths due to PPHN and improved the prognosis for survivors. Today, the survival rate for infants with PPHN is around 85 percent, and only one in five survivors experience long-term complications.
Still, children with PPHN require close medical supervision by specialists familiar with the disorder. Because PPHN is relatively rare, parents may have to travel long distances to large medical centers in order to obtain the specialized care their child needs.
Parents of children with PPHN must make every effort to keep the child as healthy as possible in order to avoid unnecessary stress on the heart and lungs. A doctor may recommend annual flu vaccinations and prompt treatment of respiratory illnesses and fevers. If symptoms are severe, children with PPHN may need oxygen equipment at home for use in emergencies.
Finally, children with PPHN often have a limited capacity for physical activity. If you have a child with PPHN, you may wish to talk to your doctor about testing to determine appropriate types and levels of activity for your child.