Pulmonary Hypertension

The first step in treatment is usually to identify the underlying cause of PH and try to correct it. Other treatment options may include supplemental oxygen and medications that are taken orally, inhaled or delivered by continuous infusion under the skin or into a vein. The medications we currently use include:

  • Endothelin receptor antagonists (ERAs)— These oral medications block endothelin, a substance in the body that constricts blood vessels. As the vessels relax, blood pressure in the pulmonary artery lowers, heart function improves and ultimately your child can be more active. ERAs may also slow the disease's progression. These medications include bosentan (Tracleer), ambrisentan (Letaris) and macitentan (Opsumit).
  • Phosphodiesterase type 5 inhibitors (PDE 5)— These oral medications are more commonly known as treatments for erectile dysfunction, but we also use them to treat high blood pressure in the lungs. Simply put, they work by increasing a natural substance in the body that makes blood vessels relax and widen. Like endothelin receptor antagonists, they can allow your child to be more active and slow the progression of PH. These medications include sildenafil (Revatio) and tadalafil (Adcirca).

  • Prostacyclin analogues — These synthetic versions of prostacyclin, a substance in the body that dilates blood vessels, can lower blood pressure in the lungs. These medications can allow your child to be more active and reduce symptoms of PH. They include treprostinil, which may be taken orally (Orenitram), inhaled (Tyvaso) or received through a continuous intravenous or subcutaneous infusion (Remodulin). Epoprostenolol (Flolan, Veletri) is also a prostacyclin analogue.
  • Soluble guanylate cyclase stimulators (sGC) —This type of oral medication works by increasing the activity of an enzyme involved in relaxing blood vessels. It can lower blood pressure in the lungs and improve heart function. This category includes riociguat (Adempas).

UCSF's Pediatric Pulmonary Hypertension Program is one of the leading centers for research on pediatric PH. Your child may have the option of participating in a clinical trial testing new treatments.

Reviewed by health care specialists at UCSF Benioff Children's Hospital.

Related Information

UCSF Clinics & Centers

Pulmonary Hypertension Program
1825 Fourth St., Sixth Floor
San Francisco, CA 94158
Phone: (415) 476-3679
Fax: (415) 476-5363
Appointment information