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Friday, April 28, 2017
Childhood pulmonary hypertension
My granddaughter was diagnosed with pulmonary hypertension (ranging close to 100) when she was 3. She did not act ill. For a year and a half she had blue feet when she would wake in the morning and she would occaisonally look pale. For most of her life I felt she had apnea. She started with apnea in the hospital at birth. Her mom had preeclampsia for the last 10 weeks of her pregnancy. She did not have real high protein but she did have cramping, decreased fetal movements, RUQ pain, constant uterine cramping, and high blood pressure. At 40 weeks she was in the hospital for 3 days getting really sicker by the minute. Finally a CT of the moms brain was done and a sono for my granddaughter who was breech. There was no labor but they did a C section because of the breech. There were complications and she was stuck fanny out for 3 to 4 minutes. She did ok with her apgar but her eyes were very funny and she stopped breathing when we had her a few hours later. This was just thought to be because she was a C section baby. Within 28 hours she was having seizures and apnea into the 60`s and found to be bleeding all over her brain in all 3 vascular distributions including the pituitary by day 4. We were told she would not make it but she did and has done very, very well. She is very smart. She does not grow well and is only 25 pounds. We weould ask about her heart for a year and a half when she would have the blue feet when she woke up but were not taken very seriously until she passed out at age 3. A two night home oxygen study showed her dipping to the 50`s with her O2 and her heart rate was even as low as 20 and as high as 240. She has a catherter in her chest now and is on Flolan that she wears in a backpack. She is doing well. She did have a spell Memorial weekend when she woke floppy. She tried to lean in and kiss her mom and followed her dads commands for a little neuro exam. She kept her head to the right. One side she could squeeze a finger and wiggle toes but the other she could not. The ambulance gave her O2 and immediately everything was moving again and she started to cry. When we did her cath her pressures were actually improved to the mid 50`s, and when they tried O2 this time her pressures almost went to normal. After a long hospital stay and 2 day EEG and heart cath, and lung x-ray, etc she was sent home on night time O2 and all has been great. My question is: We really want to be sure there is no fixable reason for her pulmonary hypertension. It is idiopathic pulmonary hypertension, but would it not be secondary if it was caused by apnea? Then of course the question would be to find out what is causing the apnea (central versus obstructive or both) I do not understand why she had such great apgars when she was so sick at birth but feel her doing so well all along when her numbers tell people she should be barely able to move just shows me she was having trouble even prior to birth and she adjusted. She does have a diagnosis of HIE but that still does not answer everything for us and it is all so confusing to read about and try to get the best questions for all of her doctors. Thanks. Worried grandma.
I appreciate your question about a very complicated problem.
The simple answer to your question of “is there a fixable reason for her pulmonary hypertension” is that this child needs to have a careful evaluation for lung disease which may be causing secondary pulmonary hypertension. Generally this would require a high resolution chest CT scan and the evaluation by a pediatric pulmonologist which are located in most major medical centers around the country.
If there is a concern about apnea in your granddaughter, a sleep study is generally easy to perform at most pediatric hospitals. One caveat is that some children with primary pulmonary hypertension may in fact have unusual breathing and some central apneas due to cor pulmonale and delayed pulse transit time. A pediatric sleep center should easily be able to differentiate this problem.
Treatment for primary pulmonary hypertension in children may include lung transplantation which is available at a few pediatric centers in the United States.
Mark Splaingard, MD
Clinical Professor of Pediatrics
College of Medicine
The Ohio State University