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Case Study: Persistent Pulmonary Hypertension

By Santa J. Bartholomew M.D. FAAP, FCCM

See Corresponding Journal Article: Persistent Pulmonary Hypertension of the Neonate (PPHN)

Baby Girl Smith is a  38-week newborn who was born to a primigravida mother via C-Section for failure of mother’s labor to progress.  The baby’s mother had excellent prenatal care and there were no complications with her pregnancy.   The infant was healthy at birth.

Approximately six hours later the baby was noted to have some grunting sounds with each breath, flaring of the nostrils and retraction of the skin between the ribs.  The nursing staff noted the respiratory distress first, and the baby was vigorously suctioned.  Babies born by Cesarian section often have extra fluid in their lungs due to lack of pressure to expel the fluid while traveling through the birth canal.  Baby was transferred to the special care nursery on ½ liter of oxygen for closer observation and the parents were assured that this was “typical” post C-section.  At the time of transfer oxygen saturation was 94-95% on ½ liter of oxygen.  Since the family lived at high altitude, lower oxygen saturation was not abnormal and there was no concern.

The baby was weaned successfully to ¼ liter of oxygen overnight with saturations in the low 90’s.  The new little family was discharged home on day of life 2 which was a Friday, and the mother was instructed to follow up on Saturday with the baby’s pediatrician.

Upon waking Saturday morning, the mother did not feel well due to her surgical incision, extreme exhaustion and lots of visitors so they called the primary care pediatrician’s office. When the pediatrician in clinic looked at the discharge summary, she saw that baby had some mild hypoxia related to altitude and that the baby was on ¼ liter of oxygen.  The pediatrician deduced from the history that the baby could wait until Monday which was day of life #5.

On day of life #4 the parents felt the baby was nursing less but assumed that to be because the mother’s milk had finally come in and baby was just more satisfied.  At around 6 o’clock PM the baby had been asleep for 4 hours, so the parents went to wake her and found her to be unarousable and cyanotic.  They called 9-1-1 and the baby was transported to the local ER in the hospital where she was born.

Her heart rate on arrival was 190 bpm, her BP 50/37, and her oxygen saturation on ¼ liter was 75%.  An urgent echocardiogram was done and the ductus arteriosus was closed but the foramen ovale was wide open with left to right shunting.  The baby was intubated and transported via helicopter to the children’s hospital in the state where the diagnosis of persistent pulmonary hypertension was confirmed.  The assumption was that she had PPHN from birth, but her symptoms were similar to those of babies born post cesarean section and those born at higher altitude, that they went unrecognized.

Over the next weeks, the baby was unable to be weaned from the ventilator, was  placed on a jet ventilator, given nitric oxide and ultimately placed on ECMO and slowly improved finally to go home 8 weeks after admission.   Although the PPHN was likely unavoidable, the baby’s long-term hypoxemia will likely have a neurological effect on her.

Santa J. Bartholomew, MD, FAAP, FCCM

Santa J. Bartholomew, MD, FAAP, FCCM

Award-winning Pediatrician with more than 35 years of clinical experience. She has been performing legal review and court services since 1993.
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