Pulmonary HTN

Question 1

epidemiology for PAH
sex
age
when does dx happen?

Answer 1

young females - 3rd or 4th decade
mean age at dx - 36 (any age possible)
diagnosis often delayed - non specific presenting symptoms

Question 2

what is the median survival after diagnosis of PAH

Answer 2

3-5 years

not good

Question 3

what is the definition of PAH? in terms of numbers

Answer 3

Mean PAP > 25 mm Hg at rest
PAP > 30 mm Hg w/ exercise
Mean PCWP and LVEDP less than 15 mm Hg

Question 4

why do we include a mean PCWP and LVEDP of less than 15 mm Hg in our definition of PAH?

Answer 4

to rule out L heart failure

these are the gold standard measurements for diagnosing L heart failure, i.e. seeing an increase in LV and LA pressure

Question 5

what is the definition of Primary Pulmonary Hypertension or IPAH? (idiopathic)

Answer 5

unclear etiology
mean PAP > 25 at rest, > 30 w/ exercise
absence of a demonstrable cause

Question 6

what are 6 causes of Secondary Pulmonary HTN?

Answer 6

1) parenchymal lung dz
2) chronic thromboembolic dz
3) left sided valvular disease
4) myocardial disease
5) congenital heart disease
6) systemic connective tissue disease

Question 7

what are the 5 groups of PAH? which ones do we need to know?

Answer 7

1) PAH (IPAH, heritable, BMPR2, PortoPH, CTDz, Schisto)
2) PAH due to Heart disease
3) PAH due to Lung Disease
4) CTEPH (Chronic thromboembolic PH)
5) PAH due to multifactorial etiology

need to know 1 and 4

Question 8

why are the disease process of Group 1 grouped together? what is their common feature?

Answer 8

plexiform lesions

Question 9

in general, what is the pathogenesis of PAH? what are two associated factors?

Answer 9

PAH is an imablance of vascular effectors (constrictors and dilators) in favor of the vasoconstrictors
Associated environmental factors
associated genetic abnormalities

Question 10

which of the collagen vascular diseases is most often associated with PAH?

Answer 10

systemic sclerosis

clicker ?

Question 11

In addition to systemic sclerosis, what are 2 other associated conditions for PAH? include percent

Answer 11

1) HIV - 0.5%

2) Sickle Cell - 10% (hemogloinopathy in general)

Question 12

Familial PPH - gene mutation leading to defective function of what protein?
(THIS IS MUST KNOW)

Answer 12

BMPR2

bone morphogenic protein receptor type II

Question 13

history of which class of medications is important to obtain in a pt w/ suspected PAH?

Answer 13

stimulants such as Fenfluramine/Phentermine
(appetite suppressors)
have to get this hx if you suspect PAH
(especially obese pts)

Question 14

PPH pathogenesis w/ regards to nitric oxide - what do you see in the pts w/ PAH in their endothelium?

Answer 14

endothelium showed negligible immunohistochemical staining for nitric oxide synthase
they do not have enough NO locally

Question 15

pathogenesis of PAH - what is endothelin 1 and what does it do in PAH

Answer 15

ET-1 is a potent vasoconstrictor and mitogen for SM cells

in PAH, it localizes to muscular pulmonary arteries - causes constriction

Question 16

what factor do you see overexpressed in plexiform lesions in PAH?

Answer 16

VEGF

Question 17

PPH pathogenesis and serotonin
where is serotonin stored?
what does it do to pulmonary vasculature?
what is its role in pathogenesis of PAH?

Answer 17

stored mainly in platelets
associated with pulmonary vasoconstriction and proliferation of SM cells
plasma serotonin concentrations are higher than normal in PPH

Question 18

what is the pathogenesis of anorexiants in PPH? (This is impt to know)

Answer 18

appetite suppressant drugs may generate PAH by blocking volt-gated potassium channels
these volt gate K channels control the cell-membrane potential and release of calcium
blocking them leads to an increase in intracellular calcium, more contractility, and thus constriction

Question 19

what are 5 presenting symptoms of PPH?

Answer 19

Fatigue 
Exertional Dyspnea 
Exertional chest pain 
Exertional syncope 
edema

Question 20

what are 5 findings on physical exam of PPH

Answer 20

1) dyspnea - variable severity
2) apical impulse shifted outwards (R heart, it shifts towards mid axillary line)
3) parasternal heave (b/c of RV enlargement)
4) palpable P2, and accentuated
5) widely split S2

Question 21

if you suspect PAH, what is the most appropriate diagnostic test to perform next (first after HnP and shit) *** know for boards (and exam)

Answer 21

Echo

good screening test

Question 22

what would be signs seen on echo that would indicated PPH?

Answer 22

dilated RV, RVSP increased drastically (normal around 15-20)

Question 23

if you see indicative signs of PPH on echo, what is the next step?

Answer 23

Right heart catheterization

this will confirm presence of PAH

Question 24

in a R heart cath report, the parameter that distinguishes PAH (arterial HTN) from PVH (venous HTN) is…
(This is must know concept)

Answer 24

the PCWP - pulmonary capillary wedge pressure
this is a surrogate measure for L Atrial pressure
(Swan Ganz catheter)
if the PCWP is less than 15, pt does not have L heart failure

Question 25

just b/c it was a clicker question - define vasoreactivity and compare prognosis of pts w/ PAH who are and are not vasoreactive

Answer 25

vasoreactivity - if we give vasodilators, will the vasculature respond to them?
pts who are vasoreative have better prognosis

Question 26

what agent do we use in the cath lab to see if pts are vasoreactive or not?

Answer 26

nitric oxide inhaled

Question 27

after admin of nitric oxide what parameters for MPAP do we expect to see if a pt is vasoreactive

Answer 27

> 10 mm Hg drop in MPAP

and MPAP should drop to less than 40 mm Hg

Question 28

what measurement is used to measure the pulmonary artery pressure (he told us to remember this)

Answer 28

tricuspid regurgitant jet velocity

Question 29

what are the 2 conditions that can cause exercise desaturation even at mild stage?

Answer 29

PAH

Pulmonary Fibrosis

Question 30

what are the PFT findings in PAH?

USMLE

Answer 30

isolated reduction in DLCO

no change in FEV1, FVC, and TLC

Question 31

which agent should be selected for a pt w/ PAH who is short of breath at rest? (USMLE)

Answer 31

Epoprostenol
a prostacyclin analog
(“prost”)

Question 32

what is the anticoagulant of choice in treating PAH? and why?

Answer 32

warfarin

use of anticoagulants has been shown to improve survival of PPH pts

Question 33

when comparing the functional classes (I - IV) who has better prognosis?

Answer 33

Functional classes I and II tend to live for longer than those who fall into classes III and IV

Question 34

CPR after CV collapse in PAH is usually successful or unsuccessful? (good to know)

Answer 34

usually unsuccessful

Question 35

name the ET-1 receptor antagonists

Answer 35

Bosentan, Ambrisentan, Macitentan