Pulmonary hypertension

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

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

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

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

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

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)

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

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

Pulmonary arterial hypertension

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

PAH - epidemiology prevalence 15-52/1 000 000 inicidence 2,4-7.1/1 000 000 mean age cca 50 females 62.5-80

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

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

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

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

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

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

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

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

Signs and symptoms chronic breathlessness signs and symptoms of RHF

Imaging methods VQ scan

CT angiography

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