Pulmonary Arterial Hypertension Overview

Pulmonary Arterial Hypertension (PAH) Overview

• Pulmonary hypertension is defined as a mean pulmonary artery pressure (PAP) of 25 mmHg or greater.

Drug Risks During Pregnancy

• Selective serotonin reuptake inhibitors (SSRIs) can increase the risk of pulmonary hypertension in newborns when taken during pregnancy.

Agents Associated with PAH

• Cocaine, Dasatinib (Sprycel), Diazoxide (Proglycem), methamphetamines, certain weight loss agents, and SSRIs used during pregnancy can cause pulmonary arterial hypertension (PAH).

Bosentan Information

• Bosentan (Tracleer) is an endothelin receptor antagonist with a boxed warning for hepatotoxicity and embryo-fetal toxicity (not safe in pregnancy).
• Common side effects include headache and anemia; it is a substrate and inducer of CYP 3A4 and 2C9.

Mechanisms of Action for Pulmonary Vasodilators

• Tadalafil increases cGMP levels and is classified as a PDE-5 inhibitor.
• Epoprostenol is a prostacyclin analogue that acts to stimulate vasodilation.
• Other medications, like riociguat, also work by increasing cGMP levels.

Warnings Associated with Adcirca (Tadalafil)

• Adcirca has warnings for hearing loss, vision loss, priapism, and hypotension; hair loss is not associated.
• It is commonly taken once daily compared to sildenafil, which is taken three times a day.

Side Effects of Epoprostenol

• Common side effects include jaw pain and flushing, attributed to its vasodilating properties.
• Other reactions include hypotension, headache, and gastrointestinal side effects (nausea/vomiting/diarrhea).

Acute Vasoreactivity Testing

• Positive responders to acute vasoreactivity testing can be treated with calcium channel blockers, specifically diltiazem and amlodipine.
• Non-responders are unlikely to benefit from calcium channel blockers.

Treprostinil Administration

• Subcutaneous administration of treprostinil can cause significant pain at the infusion site, affecting patient comfort.
• It has an administration schedule that varies based on the formulation, particularly for inhaled treprostinil.

Generic Names of Medications

• Revatio is the brand name for sildenafil.