Cardiology Test 5- Montepara PAH

Pulmonary Hypertension is a group of conditions relating to elevated blood pressure measured within pulmonary circulation that are classified into ___ groups according to the world health organization (WHO)

5

WHO group 1 = pulmonary ___ hypertension (defined as an increase in mPAP to >20mmHg at rest in the presence of PCWP ≤ 15mmHg)

arterial

PAH Etiology

1) ____ (a parasitic infection)

2) congenital __ ___

schistosomiasis, heart disease

PAH Etiology

3) ____ diseases (scleroderma, SLE, rheumatoid arthritis)

4) ___ infection

rheumatologic, HIV

PAH Etiology

5) ___ cell disease

7) ____ HTN

8) ___ mutations

9) ____

sickle, portal, genetic, pregnancy

Drugs that definitely cause PAH

1) aminorex

2) fenfluramine

3) dexfenfluramine

4) toxic ___ oil

5) benfluorex

6) select __ __ inhibitors

rapeseed, seratonin reuptake

WHO Functional Classification

Class I: __ limitation on physical activity. Ordinary physical activity does not cause symptoms

no

WHO Functional Classification

Class II: __ limitation on physical activity. no discomfort at rest, but ___ physical activity causes symptoms

mild, normal

WHO Functional Classification

Class III: __ limitation on physical activity. no discomfort at rest, but ___ than normal physical activity causes symptoms

marked, less

WHO Functional Classification

Class IV: ___ to perform any physical activity at rest

unable

There is no __ for PAH, our job is to slow the progression

cure

Nonpharm therapy for PAH

-avoidance of __ due to high morbidity and mortality rate

pregnancy

Nonpharm therapy for PAH

-immunization against __ and ___ disease

influenza, pneumococcal

Nonpharm therapy for PAH

-supplemental oxygen (goal saturation > __%)

90

Nonpharm therapy for PAH

-adherence to a low-__ diet to avoid fluid retention

-cardiopulmonary ___

sodium, rehabilitation

Background Drug Therapy (not a focus)

1) ___ -consider in idiopathic PAH, INR 1.5-2.5

2) ___ ___-for symptomatic relief if evidence of fluid overload

3) ____- potentially could be used in PAH+ right HF

warfarin, loop diuretics, digoxin

Acute Vasoreactivity Test

-used to identify patients who may benefit from oral __ __ __

calcium channel blockers

Acute Vasoreactivity Test

-involves assessing hemodynamic response with fast-acting, short-term ___ (inhaled nitric oxide, IV epoprostenol, or IV adenosine)

vasodilators

Acute Vasoreactivity Test

-a positive response is a reduction in mean PAP (pulmonary artery pressure) by at least __mmHg to a value of __mmHg or less

-positive response= we can use a CCB :)

10, 40

Calcium Channel Blocker (CCB)

-___ and ___ (preferred bc DHP)

-____ (non-DHP option)

amlodipine, nifedipine, diltiazem

Calcium Channel Blocker (CCB)

-WHO functional class __-___

I-III

Calcium Channel Blocker (CCB) Adverse Effects

-edema

-fatigue

-palpitations

-vasodilatory effects: flushing, dizziness, headache

-___ hyperplasia

gingival

PDE-5 Inhibitors

-sildenafil

-tadalafil (be aware this drug has __ dose adjustments)

renal

PDE-5 Inhibitors

-WHO functional class __-___ (symptomatic)

II-IV

PDE-5 Inhibitors Adverse Effects

-vasodilatory effects: headache, flushing, dizziness

-GI: dyspepsia, N/V

-hypotension

-___ (nose bleed)

-insomnia

-__ changes

-tinnitus

epistaxis, vision

PDE-5 Inhibitors Contraindications

-don't use with __ or ____

-don't use sildenafil with __ ___

nitrates, riociguat, protease inhibitors

What is the brand name of Riociguat?

Adempas

soluble guanylate cyclase stimulator

-riociguat is WHO functional class __-___

-increase dose of this med if pt is a smoker

-if pt stops therapy for 3+ days, you will need to retitrate up

II-III

soluble guanylate cyclase stimulator adverse effects

-____!!!

-vasodilatory effects: dizziness, headache

-GI: dyspepsia, gastritis, GERD, N/V/D

-anemia

-bleeding

-edema

teratogenicity

soluble guanylate cyclase stimulator (riociguat) contrindications

-___

-use with ___ __ or __

pregnancy, PDE-5, nitrates

What are the prostacyclins?

Epoprostenol, Treprostinil, Iloprost

Prostacyclins (epoprostenol, treprostinil, Iloprost) Adverse Effects

-vasodilatory effects: flushing, headache, dizziness

-N/V/D

-hypotension

-rash

-adverse effects involving ___ (jaw and limb pain, myalgia, arthralgia) !!

pain

What are brand names for epoprostenol?

Flolan, Veletri

epoprostenol

-continuous __ infusion

-Flolan is stable at room temp for only 8 hours, so need __ ___ during administration

-Velentri stable at room temp for 24 hours

IV, ice packs

epoprostenol

-WHO functional class __-__ (remember its IV, used in sicker, advanced patients)

III-IV

epoprostenol

half life is __-__ minutes (SHORT)

3-5

What are brand names for treprostinil?

Remodulin, Tyvaso, Orenitram

treprostinil

-half life is __ hours

4

treprostinil

-Remodulin is continuous __ or ___

-Tyvaso is ___

-Oreitram is ___

IV, SC, inhalation, oral

treprostinil

-WHO functional class of Remodulin is __-__ (remember its IV, used in sicker, advanced patients)

II-IV

treprostinil

-WHO functional class of Tyvaso is ___

III

treprostinil

-WHO functional class of Orenitram is ___-___

II-III

What is brand name for iloprost?

Ventavis

iloprost

-Ventavis is ___

-WHO functional class is ___-___

inhalation, III-IV

iloprost

-half life is __-__ minutes

20-30

Prostacyclin Receptor Antagonist is ___

selexipag

What is the brand name of selxipag?

Uptravi

Prostacyclin Receptor Antagonist

-WHO function class __-___

II-III

Prostacyclin Receptor Antagonist Adverse Effects

-vasodilatory effects: headache, flushing dizziness

-N/V/D

-adverse effects involving ___ (jaw and limb pain, myalgia, arthralgia) !!

pain

What are the 3 endothelin receptor antagonists?

Bosentan, Ambrisentan, Macitentan

Brand name of Bosentan?

Tracleer

Brand name of Ambrisentan?

Letairis

Brand name of Macitentan?

Opsumit

ERAs are all ___ medications

oral

Endothelin Receptor Antagonists

WHO FC class of bosentan is __-__

WHO FC class of ambrisentan and macitentan is __-___

II-IV, II-III

Endothelin Receptor Antagonists Adverse Effects

-_____ !!

-_____ !! (for bosentan only)

-edema

-anemia

-vasodilatory effects: headache, flushing dizziness

-upper respiratory tract infection

teratogenicity, hepatotoxicity

Endothelin Receptor Antagonists Contraindications

-pregnancy

-do not use bosentan with ___ or ___

-do not use ambrisentan if pt has idiopathic __ ___

cyclosporine, glyburide, pulmonary fibrosis

What are the 4 drugs with REMS programs?

bosentan, ambrisentan, macitentan, riociguat

-for REMS programs for teratogenicity, need baseline and monthly __ __

-for REMS programs for hepatotoxicity (bosentan), need baseline and monthly ___

pregnancy test, LFTs

REMS program-acceptable contraception

Option 1= IUD, __ sterilization, progesterone ___

tubal, implant

REMS program-acceptable contraception

Option 2= ___ option + ___ method (condom, diaphragm with spermicide, cervical cap with spermicide)

hormonal, barrier

REMS program-acceptable contraception

Option 3= ____ barrier methods (condom, diaphragm with spermicide, cervical cap with spermicide)

2

REMS program-acceptable contraception

Option 4= ____ + either a barrier method or hormonal option

vasectomy

Treatment Algorithm Overview

-FIRST- do an acute vasoreactivity test to see if they can do ___

CCB

Treatment Algorithm Overview

-in general, we start with ___ __ (WHO class II-IV)

PDE-5

We add on 1 drug per ___ for combination therapy

ERAs, prostanoids, and then either PDE-5 or riociguat

pathway

Treatment Algorithm Overview

- for WHO FC IV- we use ___ IV

epoprostenol