1. Pulmonary hypertension

2. PH - definition A clinical syndrome characterized by:
pulmonary artery mean pressure >= 25 mmHg
assesed by (echocardiography) and/or right heart cath

3. PH classification Pulmonary arterial hypertension
PH due to left heart disease
PH due to lung disease
Chronic tromboembolic pulmonary hypertension
PH due to other causes

4. Diagnostic workup of PH
PAH
chronic dyspnea
left heart
echo finding of PH
diagnostic
work-up
lung disease
screening
tromboembolic
right heart failure
other
other reasons

5. appropriate treatment of LHD yes
echo finding of PH
LHD present
PH proportionate?
appropriate treatment
of LHD
yes
CXR, bodypletysmography,
diffusion capacity, HRCT PH
proportionate
to pulmonary
disease?
appropriate treatment
of pulmonary disease
yes
VQ scan
if positive 3 mo OAC
PAH
referral to expert center
right heart cath.
CTEPH

6. Diagnostic work-up of PH II.
rule out pulmonary disease:
spirometry
bodypletysmography
pulmonary diffusion capacity
CXR
lung HRCT
rule out left heart disease

7. Measured values Pulmonary artery pressure: (PASP, PADP, PAMP)
Pulmonary capillary wedge pressure (PCWP)
Right ventricle systolic/filling pressure (RV)
Right artrial pressure (RAP)
Cardiac output (CO)

8. Calculated values Transpulmonary gradient (TPG)
TPG = PAMP-PAWP (mmHg)
Pulmonary arterial resistence
PAR = TPG/CO = (PAMP-PAWP)/CO
Wood units [WU], normally < 3 WU
Cardiac index (CI) = cardiac output/body surface

9. Haemodynamic syndromes
Precapillary pulmonary hypertension
PAMP >= 25 mmHg, PCWP <= 15 mmHg, CI normal/decreased
PAH, PH in lung disease, chronic tromboembolic PH...
Postcapillary pulmonary hypertension
PAMP >= 25 mmHg, PCWP <=15 mmHg, CI normal/decreased
corresponds to left heart disease
Hyperkinetic circulation
PAMP >= 25 mmHg, PCWP <=15 mmHg, CI increased
cardiac shunts, anemia, thyrotoxicosis

10. Pulmonary arterial hypertension

11. PAH - definition
a clinical condition characterized by the presence of precapillary pulmonary hypertension in the absence of other causes of precapillary PH such as PH due to lung disease, chronic thromboembolic PH or other rare diseases
a disease of pulmonary vessels

12. PAH - epidemiology prevalence 15-52/1 000 000
inicidence 2,4-7.1/
mean age cca 50
females

13. Aetiopathogenesis pathological response to pulmonary vascular injury
vasocontriction
angioproliferation
thrombosis in situ
inflammation

14. clinical symptoms chronic progressive dyspnea (very frequent)
ischaemic chest pain
(near) syncope
cough, haemoptysis, dysphonia (rare)

15. objective signs II. sound accentation on pulmonary valve spot
tricuspid/pulmonary murmur
III./IV. heart sound
signs of right heart failure
oedema, hepato(spleno)megaly, icterus, neck veins

16. PAH - treatment General measures diuretics when fluid overload
oxygenotherapy when respiratory failure
pregnancy not recommanded unless disease stable

17. PAH - Treatment Pharmacotherapy
PDE-5 inhibitors (sildenafil, tadalafil)
guanylate-cyclase stimulators (riociguat)
endoteline receptor antagonist (bosentan, ambrisentan)
prostacycline and analogues (epoprostenol, treprostinil)

18. PAH-treatment Non-pharmacological measures
percutaneous baloon septostomy (PBAS)
lung transplant
1 year survival cca 70%, 5yrs <50%

19. Chronic tromboembolic pulmonary hypertension
a disease caused by chronic obstruction of pulmonary artery branches following episodes of pulmonary embolism and incomplete thrombus resolution
combination of:
precapillary pulmonary hypertension
evidence of trombotic occlusion (at least one mismatched segmental defect on VQ scan, evicence of occlusion in CT)
at least 3 months of effective anticoagulation

20. Epidemiology
up to 4% patients after episode of PE
male/female 1:1

21. Signs and symptoms
chronic breathlessness
signs and symptoms of RHF

22. Imaging methods
VQ scan

23. CT angiography

24. Treatment General measures
lifelong anticoagulation (warfarin or LMWH)
No place for NOAC!
diuretics when fluid overload
Surgical treatment – pulmonary artery endarterectomy
Pharmacotherapy – only if inoperable (PAH specific treatment)
Pulmonary artery angioplasty