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Thoracics > PAH > Flashcards

PAH Flashcards

1
Q

5 groups PAH list-

A 
1- Idiopathic PAH
2- Left heart disease 
3. Pulmonary disease and sleep disordered breathing 
4. Thromboembolic disease
5. Mixed aetiology
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2
Q

Tell more about group 1 PAH-

A

Idiopathic PAH- sporadic with no FH and no risk factors
Heritable -6% eg major assoc is BMPR2
Drugs and toxins- SSRIs!!!
CT disease espec scleroderma and SLE
HIV- related to duration of infection NOT CD4 count
Portal hypertension
Schistosomiasis
Chronic haemolytic anaemia
Congenital heart disease
Persistent pulm hypertension of the newborn

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3
Q

What’s in group 5?

A

Haem disorders eg splenectomy and MPD
Systemic- LAM, vasculitis, neurofibromatosis, sarcoid
Metabolic eg thyroid
CKD on dialysis

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4
Q

ECG findings in PAH?

A

RV strain pattern (ST dep/TWI in inferior leads and V1-4)
RAD
R atrial enlargement (2.5mm in inferior leads)
RVH (R/S >1 in V1,

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5
Q 
Increase or decrease in:
thromboxane
nitric oxide
prostacyclin
endothelin
A

Thromboxane and endothelin increase

NO and prostacyclin decrease

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6
Q

What should initial therapy be?

A

Treat underlying cause
anti coagulate target INR 1.5-2 if group 1 or 4
supp oxygen if resting or exercise induced hypoxaemia
Pulmonary rehab
Pneumococal and flu vax
Avoid pregnancy and strenuous activity (supervised exercise training ok)
Assess need for diuretics for management of fluid overload

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7
Q

bosentan MOA

A

endothelin receptor antagonist- receptors A and B

endothelin blocking prevents vasoconstriction and proliferation

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8
Q

macitentan MOA

Also two side effects

A

endothelin receptor antagonist- A and B receptors

Causes nasopharyngitis and anaemia
Improves SURVIVAL as well as functional class and 6MWT
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9
Q

ambrisentan MOA

A

endothelin receptor antagonist- receptor A only

teratogenic

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10
Q

sildenafil MOA

A

oral phosphodiesterase 5 inhib

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11
Q

tadalafil MOA

A

oral phosphodiesterase 5 inhin

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12
Q

riociguat

A

oral guanylate cyclase activator –>increases sensitivity of sGC to endogenous NO AND directly stimulates the NO receptor

Also benefit in class 4!

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13
Q

Epoprostenol MOA

A

PGI2 prostacyclin
Produces vasodilation and antiproliferation

THE ONLY THERAPY SHOWN TO PROLONG SURVIVAL

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14
Q

iloprost MOA

A

PGI2 prostacyclin analogue

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15
Q

teprostinil

A

PGI2 prostacyclin analogue

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16
Q

Who do you actually treat with advanced therapy in terms of WHO functional classification?

A

I- onserve
II, III-(meaning SOB on activity but not rest) endothelin receptor antagonist, oral phosphodiesterase 5 inhibitor, oral guanylate cyclase activator
IV- IV (means evidence of RHF and may have SOB at rest) epoprostenol over alternative agents- improves survival and exercise capacity

17
Q

What are the poor prognostic factors in PAH?

A 
underlying scleroderma
Increase BNP
Increase RA pressure
RHF signs
6 min walk test poor
high WHO status
RV dysfunction 
Men
Older people

usually if any of these then straight to parenteral therapy rather than trying phosphodiesterase 5 inhib or endothelin receptor antagonist first

18
Q

What monitoring whilst on Bosentan?

A

LFT monthly

Need barrier contraception

19
Q

Mechanism of action phosphodiesterase 5 inhibitors?

A

NO–>activate guanylate cyclase–>increase cyclic GMP production–>vasorelaxation.

Phosphodiesterase usually breaks down cGMP.
So inhibitors increase concentration of cGMP and cAMP

20
Q

Indications for surgical intervention for MR-

A

LVEF under 60%
LVESP over 40 mmHg
SEVERE Pulmonary hypertension at rest (over 50) or during exercise (over 60)
New onset AF

21
Q

How does BMPR work?

A

inhibits SM proliferation and induces apoptosis
If abnormal–>vascular remodelling
Only 1/4 patients develop
Mutations found in 75% of familial cases and 40% of sporadic

22
Q

Where are the endothelin A and B receptors

A

On vascular smooth muscle –.vasoconstriction
ET-B also on endothelium–>NO release and vasodilation

levels correlate with disease activity and mortality

23
Q

Which advanced therapy can you give for group 4?

A

Riociguat

24
Q

Which ones are those where advanced therapies may be life threatening?

A

Class 2 PAH- those with increase PCWP

25
Q

Which Ca ch bl do you avoid in vasoreactive patients?

A

Not verapamil- neg inotrope
use nifedipine or diltiazem

NO EVIDENCE IN CTD ASSOCIATED PULMONARY HYPERTENSION
Improves survival IF you are a responder.

26
Q

When do you NOT use warfarin in PAH?

A

COPD
systemic sclerosis as there is increased risk of bleeding

Used in IPAH, familial, chronic thromboembolic

27
Q

Prostacyclin/Epoprostenol MOA

A
  1. Potent IV vasodilator- increases intracellular cAMP
  2. Inhibits platelet aggregation

Improves exercise tolerance, survival and haemodynamics

28
Q

Side effects of prostacyclin/epoprostenol

A

Jaw pain
Diarrhoea
Arthralgia
CVC related

29
Q

What is the problem with treprostinil?

A

Painful subcut infusion

30
Q

What’s the problem with inhaled illoprost?

A

Short half life so have to use 6-12 times a day

Improves QOL, dyspnoea, 6mwt but not yet evidence mortality

31
Q

What is selexipag?

A

The only orally available selective prostacycline receptor agonist
Add on therapy- trials ongoing

32
Q

What is the limiting factor with Bosentan use?

A

hepatotoxicity

33
Q

What do you risk if you transplant one of these patients?

A

Higher incidence of bronchiolitis obliterans.

34
Q

Scleroderma- what evidence for what agents?

A

If vasoractive positive- ca ch bl can achieve prolonged survival, sustained functional improvement, and haemodynamic improvement

Epoprostenol - improves haemodynamics, functional capacity, and survival
Trepostinil- improve haemodynamics, symptoms, exercise capacity, possibly survival

Bosentan-mproved symptoms, the six-minute walking distance, and the WHO functional class

Sildenafil- significant improvement in hemodynamics and six-minute walk distances

Riociguat- increase time to clinical worsening, increase 6mwt

35
Q

Survival scleroderma PAH?

A

Worse than idiopathic but better than SSc-associated ILD-PH

Thoracics (16 decks)

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