Pulmonary Htn

Question 1

Definition phtn

Normal value

Answer 1

Increase in mean pap> 25mmhg at rest as assessed by rh catheterization
14+_3 upper limit-20

Question 2

What is PAH

Answer 2

subgroup of ph with

Pawp<15 with pvr>3wood units in absence of other precapillary htn su h as lung diseases ,pte

Question 3

Group 1 pah

Answer 3

Idiopathic
Familial - bmpr 2, alk-1
Ass with ctd/hiv/chd/portal htn/ parasites/ drugs
Pulm veno occlusive diaease
Persistent htn of new born

Question 4

Group 2

Answer 4

Phtn due to left heart failure

Question 5

Group 3

Answer 5

Due to limg disease/ hypoxemia

Question 6

Group 4

Answer 6

Cteph 
Other pulmonary artery obstruction- angio sarcoma 
Intravascular tumor
Areteritis
Cong.pa stenosis
Parasitic(hydatidosis)

Question 7

Group 5

Answer 7

Unclear or multifactorial mechansims
Hematological
Systemic disorder- sarcoid , lch, lam , nf
Metabolic- storage disorders.
Other- segmental htn
Crf
Fibrosing mediastinitis

Question 8

Who functional classification

Answer 8

1- no limitation of physical activity
2- slight limitation
3- marked
4@ inability to carryout any physical activity without symptoms

Question 9

Pathogenesis of group 1

Answer 9

Genetic +risk factors- endothelial cell injury and imbalance b/w vd and vc , apoptotic and mitogenic factors, thrombotic and antithromobtic
Causes vc, remodelling , thrombosis
Inc pa resistance ane pressure

Question 10

Pathogenesis of group 2

Answer 10

Valvular disease - inc la pressures - interstitial edema + pa vc- vascular changes- pah

Question 11

Pathogenesis of group 3

Answer 11

Hypoxia- inc circuoatory bdnf (brain derived neurotrophic factor)
Dec no- dec vd
Smooth muscle proliferation
Inc vasoconstriction

Question 12

Pathogenesis group 4

Answer 12

Pe- incomplete resolution/organisation of thrombus- occlusion/ shear stress in non obstructed vessel - inc pa pressure and resistance

Question 13

Diagnosis
Ecg
Cxr
Echo
V/q

Answer 13

Rvh , cor pulmonale
Enlargement of central pulmonary arteries ,rvh
Pasp>40
For embolism

Question 14

Angio
Pft
Oximetry
Usg
6mwt

Answer 14

For embolism
Undrlying lung disease 
Osa
Portal htn
For severity

Question 15

Right heart catheterization

Answer 15

To confirm diagnosis
At rest 25
Exercise 30
Wedge pressure <15
Pvr >240 dines cm sec

Question 16

Vasoreactive test 
Why 
Recommended in whom
Agents
Interpretation 
Use

Answer 16

To detect residual properties of vadodilation of small pulmonary arteries and arterioles
In group 1
Inhaled no , enoprostenol iv. Adenosine
Pos- dec in 10 and value less than 40 with an increased or unchanged co and minimally reduced or unchanged bp
Use- in positive pts ,ccb high dose candidates

Question 17

Group 1 drug therapy

Answer 17

Ccb- nifedipine- 90-180mg /day
Diltiazem 240-720
Amlodepine 20
S/e- Hypotension, edema , syncope , rv failure

Question 18

Endothelial receptor antagonist

Answer 18

Ambrisentan 5-10
Bosentan 125be
Macitentan 3-10 /day

S/e: leg swelling , fluid retention, hepato Toxicity, teratogenic

Question 19

Pde5

Answer 19

Sildenafil 20 tds
Tadalafil
S/e - headache , chanegs in vision, not usedl with nitrates ,hypotensikn

Question 20

Guanylate cyclase inhibitor

Answer 20

Riociguat- 2.5 tds
Syncope , headache, edema , diarrhoea
C/I in pregnancy. Pde5 inh

Question 21

Ip receptor

Answer 21

Selexipag-

Question 22

Prostacyclin analouges

Answer 22

Epoprostenol-iv
Iloprost- inqhled iv
Treprostinil
Beraprsot- oral

Jawpain, diarrhoeaz achesvz

Question 23

Combination therapy

Answer 23

Macitentan plus sildenafil
Riociguat plus bosentan
Selecipag +era/pde5 inhibitors

Question 24

Algorithm

Answer 24

Vr test pos- ccb
Negative- who 2, 3- single or combination
4- initiak combination
Inadequate response- double/ triple-rx

Question 25

Sx

Answer 25

Balloon arterial septostomy
Benefit in fc-4 with rhf refractory to medical therapy
No mortality benefit

Question 26

Ix of lung tx

Answer 26

Nyh 3, 4 desoite 3 minths combination theraoy including prostanoids
Cardiac index less than 2 lit /m2
Mrap >15
6mwt- <35

Question 27

Group 2

Answer 27

Diuretics

Prostanoids. Era, pde5- some benefit

Question 28

3

Answer 28

Ltot

Ccb x

Question 29

Group 4

Answer 29

Life long anti coagulation
Diuretics o2
Riociguat
Ivf filter