Pulmonary HTN

Question 1

WHO Classification

Answer 1

Group 1: PAH pulmonary artery HTN - mPAP>25mm Hg &amp; PVR>3 W.U. and PCWP<15mm Hg
-Idiopathic PAH
-Heritable PAH (BMP, ALK1)
-Drug and toxin induced
-Associated
--CTD
--HIV
--Portal HTN
--Congential heart disease
--Schistosomiasis
--hemolytic anemia
--persistent pulm HTN of newborn
--pulmonary venoocclusive dz/pulm cap hemangio
Group 2 (post capillary pulmonary HTN): Left heart dz - MS, systolic, diastoic dysfxn, HTN - mPAP>25, PCWP>15, TPG>12mm Hg
Group 3: Lung disease or hypoxia
-COPD
-ILD
-OSA
-high altitude
Group 4: Chronic thromboembolic Pulmonary HTN
Group 5: Other
--Sarcoid, Gauchers, MPd/o

Question 2

PVR

Answer 2

PVR=(mPAP-PCWP) ie transpulmonary gradient/CO
>3.0 = post capillary pulmonary HTN
If transpulmonary gradient >12mm Hg - suggests component of left heart disease - rule out sleep apnea or throboembolic disease (sleep study or V/Q scan)

Question 3

Precapillary pulmonary HTN

Answer 3

mPAP>25
PCWP<15
TPG>12

Question 4

Post capillary pulmonary HTN

Answer 4

mPAP>25
PCWP>15
TPG<12

Question 5

RHC with vasodilator challenge

Answer 5

Postive (iNO, adenosine, epoprosteronol)
dec in mPAP >10mm Hg to absolute mPAP<40mm Hg in setting of unchanged CO (or inc’d) - they can respond to CCB therapy
DO NOT TX Conn Tissue D/o PAH group 1 with CCB even if response to iNOS

Question 6

Diastolic dysfunction

Answer 6

fluid challenge on RHC - check for inc in PCWP

Question 7

Eisenmengers (VSD)

Answer 7

R->L shunt
shunt not repairable if PA/Ao>2/3
more common in shunts distal to TV
differential cyanosis = rev shunt at PDA
risk of systemic embolism (clot or air)
Pulm vasodilators improve Sx not mortality
endocarditis ppx
Bosentan and med management until heart/lung tx

Question 8

Echo features of PAH

Answer 8

RAE, RVE/RVdysfxn, interventricular septal flattening, TR with elevated TR vel, reduced TAPSE,

Question 9

Dx PAH

Answer 9

If no left sided reasons for PH - ie MS, AS, systolic or diastolic dyfxn - RVSP>40 warrants RHC in patients with unexplained dyspnea

Question 10

Group 4

Answer 10

V/Q scan better than CTA

Question 11

Group 3 - lung dz/hypoxia

Answer 11

dx with PFTs (obstructive or restrictive lung dz)

or overnight sleep study to r/o OSA (causes PH with hypoxic pulm vasoconstriction)

Question 12

Group 2 - PH 2/2 left sided dz

Answer 12

MC type of Pulm HTN
TPG normal (<12mm Hg) -> ie elevated PCWP owing to left heart disfunction and relatively low mPAP and PVR near normal (PVR<3 W.U.)

Can eventually develop “out of proportion” pulm HTN with left heart dysfxn group 2 - ie elevated TPG, elevated PVR - then test for concurrent precapillary pulm HTN

TTE to r/o A/mitral valve dz, systolic or diastolic dysfxn
LAE points to chronically elevated left heart filling
pressures
Treat underlying left heart disease with diuresis

Question 13

PHTN Therapy

Answer 13

iNO responsive (fall in mPAP by at least 10 to less than 40mm Hg) without drop in CO in response to iNO, esoprosterol or adenosine
-Tx with Dihydropyradine CCB (nifedipine or verapamil)

If neg vasodilator

1) Prostanoid (IV Esoprosterol) - needs cont IV pump
2) Endothelin antagonist (Bosentan) - hepatic dyfxn
3) PDE-5 Inhib (Sildenafil) - flushign/epistaxis
4) GMP Cycl inhib (riociguat) - hypotension

HIGH RISK patients need dual therapy
IV prostacyclin + endothelin rct ant (ambrisartan or bosentan)

Question 14

PDE inhibitors

Answer 14

improve 6mWT and HD but not time to clinical worsening - need to combine with prostenoid or endothelin rct antag if severe sx

Question 15

Riociguat (GMP)

Answer 15

contraindicated with PDE inhib and nitrates 2/2 hypotension

persistent PH after surgical pulmonary endarectomy or with inoperable CTEPH (not substitute for surgery)

Question 16

Atrial septostomy

Answer 16

only with refractory RHF and severe PAH depsite max med therapy - for palliation and restoration of stability till tx

Question 17

CTEPH (group 4)

Answer 17

Tx: Surgical pulm endarectomy
Surveillence - 6MWT
see reduced PAP, dec PVR, red RH sizes, red TR
TTE annually

Question 18

High risk features Pulm HTN

Answer 18

NYHA IV
Syncope
RAP>15
6MWT<300
pVO2<12
C.I.<2
RVEF<35%
TAPSE<1.5cm

Question 19

High risk therapy

Answer 19

Should include epoprostenol

Question 20

Monitor therapy of PHTN

Answer 20

6MWT >380m
normal or near normal BNP
normal or near normal RV Fxn
WHO fxn class I or II
CI >2.5
VO2 >15ml/min/kg

Question 21

Eisenmenger’s Syndrome

Answer 21

near systolic pulmonary blood pressures
Pulmonary HTN mPAP>25 and R->L shunt - typically shunts below TV (ie VSD, PDA)
PDA eisenmengers have differential cyanosis
unrepaired VSD
blue lips on exertion
avoid preg, exertion
Bosentan - endothelin antagonist
improves 6MWT, symptoms
Do not improve survival

DO NOT USE Vasodilators
-will increase right to left shunt by decreasing SVR

Question 22

de Novo class II pulm htn

Answer 22

tadalafil + ambrisentan

Question 23

Pulm HTN and h/o cancer

Answer 23

r/o CTEPH with v/q scan

Question 24

Group 1 Idiopathic PAH

Answer 24

anticoagulants (warfarin 1.5-2.5), oxygen, diuretics +- CCB if reactive to iNOS testing

Question 25

Prostanoids - Eposprostenol, treprostinil (IV,SQ, inhaled), iloprost (inhaled)

Answer 25

Epoprostanol - improved 6MWT, QOL, survival
-with scleroderma improved 6MWT, HD
Trepostenil - only improved 6MWT, not survuval or clinical worsening
Iloprost - improvement in NYHA class, time to clinical worsening and 6 MWT

Question 26

Endothelin rct antagonist (Bosentan, ambresartan)

Answer 26

Bosentan - improved 6MWT, time to clinical worsening
-needs LFTs

Ambresartan - improved 6MWT, time to clinical worsening
-no LFTs needed

Question 27

PDE inhibitors (Sildenafil, tadalafil)
inhibit hydrolyssi of cGMP

Answer 27

Sildenafil - improved 6mWT and HD NOT time to clinical worsening

Tildenafil - improved 6MWT only

Question 28

Guanylate Cyclase Inhibitor - Riociguat

Answer 28

improved 6MWT, time to clinical worsening
peristent PH after group 4 CTEPH surgery or pt who ware not surgical canddiateas
contraindicated with nitrates