Pulmonary arterial hypertension

pulmonary hypertension is

continuous high BP in the pulmonary arteries

Drugs that can cause PAH

cocaine, SSRI use during pregnancy can increase newborn PPHTN, weight loss drugs (phentermine), pethamphetamines

pulmonary hypertension is

continuous high BP in the pulmonary arteries

Drugs that can cause PAH

cocaine, SSRI use during pregnancy can increase newborn PPHTN, weight loss drugs (phentermine), pethamphetamines

Vasoconstrictor substances include

endothelin-1 and thromboxane A2 (TXA2)

Vasodilating substances include

prostacyclins

the high pressure results from

imbalance in vasoconstrictor and vasodilator substances

most common cause of death in PAH

heart failure

True/False: There is no cure for PAH

This is true. a lung-heart transplantation

What should PAH patients restrict their sodium to daily?

<2.4g/day

True/False: A sodium-restricted diet helps PAH patients manage volume status

true

What medications should be avoided in PAH

NSAIDS - manage volume status

Immunizations recommended in PAH

pneumococcal and influenza

To confirm DX of PAH, a patient should get a ____

right heart catheterization

Inhaled nitric oxide may be administered during right heart catheterization to test

vasoreactivity

A responder during right heart catheterization should receive ___ as first line

calcium channel blockers

CCBs used most frequently in first responders are

long-acting nifedipine, diltiazem, amolidipine

True/False Verapamil is NOT recommended due to negative inotropic effects reltive to diltiazem

true

non-responders in heart catheterization should receive

prostacyclin analogues, receptor agonists, endothelin receptor antagonists (ERAs) PDE-5 inhibitors, soluble guanylate cyclase stimulators

This drug has been shown to decrease mortality in non-responders

IV epoprostenol- parenteral prostacyclin analog

supportive therapies in PAH

loop diuretics, digoxin, warfarin (titrated to INR 1.5-2.5)

INR goal of warfarin in PAH

1.5-2.5

Epoprostenol brand name, drug class, warnings

flolan

prostcyclin analogue/prostanoid

warnings: vasodilation reactions (hypotension/flushing)

IV infusions- infections

Epoprostenol side effects

hypotension, flushing, jaw pain, infusion site pain, potent vasodilator, avoid interruptions and sudden large dose reductions

true/false: epoprostenol should be protected from light, and requirees ice packs for stability

true

Enothelin receptor antagonists (ERAs) boxed warning

teratogenic- monthly pregnancy tests

ERAs contraindications and warnings

pregnancy CI

warnings: hepatotox, fluid retention, lower Hgb/Hct, side effects of headache

can decrease effectiveness of hormonal contraception

PDE-5 inhibitors used in PAH

sildenafil (Revatio, Viagra (ED)

Tadalafail (Adcirca) Cialis - ED,BPH)

Contraindications of PDE-5 inhibitors

use with nitrates

warnings with PDE-5 inhibitors

hearing loss, vision loss, Nnonarteritic anterior ischemic optic neuropathy, hypotention, priapism, headache

guanylate cyclase is a receptor for

endoenous nitric oxide

this drug sensitizes sGC to endogenous nitric oxide and directly stimulates the receptor, leading to increased cGMP, and relaxation of pulmonary artery smooth muscle cells

riociguat (adempas)

adempas generic name

riociguat

True/False Riociguat is teratogenic

true

These drugs require a negative pregnancy test prior to starting and monthly thereafter

riociguat, all ERAs

ERAS include-(bosentan, ambrisentan, macitentan)

Contraindications to riociguat

pregnancy, use of PDE-5 inhibitors, nitrates

warnings of adempas

hypotension, can cause headaches

Key drugs that cause pulmonary fibrosis

amiodarone/dronedarone

bleomycin

busulfan

carmustine

lomustine