What is PPHN?

PPHN stands for persistent pulmonary hypertension in the newborn. This is one issue that many CDH children must overcome and it is serious.

When your child is in the womb, your body acts as an ECMO machine. The circulation of blood through the baby’s system bypasses the lungs. There is an open valve in every baby’s heart called the ductus arterosis.

Blood flows from the umbilical cord to the heart and back. When a child is born, the ductus usually closes on its own and blood flows from the heart to the lungs and back. In CDH kids, compromised lung function oftentimes allows the ductus arterosis to remain open affecting the oxygenation and removal of carbon dioxide from the blood.

PPHN can be overcome with time, ECMO and/or drug therapy. It might resolve quickly or it may take months. The ductus can close and then reopen due to various reasons including stress. The ductus can also remain open but the blood can flow correctly but that ductus will eventually need to close off.

Two drugs used to help treat PPHN are Viagra and Flolan. Viagra was initially developed to treat pulmonary hypertension in both adults and children; the help with erectile dysfunction was a side effect. Viagra was fast tracked for FDA approval and is now marketed under another name to treat adult pulmonary hypertension. For children, it is crushed and placed into their mouth or ventilator tube. There are medical professionals who doubt its effectiveness as the method of delivery can be compromised if the baby spits up or has any residuals.

Flolan is an IV administered drug usually used in adults. I believe its usage is still considered experimental on infants. It can cause redness at and around the IV site.

Whatever measures taken to treat PPHN, it is often a large obstacle to overcome.