1. 1. - . . .
2. 2. () () . . ...(PVR) . ...(PVR) P.A.P., () . PVR=TPPG/CO =25/8mmHg =15+/-3mmHg
3. 3. A /PASPNORMAL OR GRADE 1: 27+/-7GRADE 2: 38+/-7GRADE 3-4: 64+/-15 MAYO CLINIC 2008
4. 4. 4,2%GROUP I79%GROUP II 10%GROUP III0,6%GROUP IV 7%GROUP V
5. 5. E.ROMBERG: . , /& . 1951,39 DrD.T.DRESDALE 1967 1973 10 .. . REXFUMARATE, ()
6. 6. Regulation of pulmonary arterial tone
7. 7. PAH The term PAH represents truepulmonary ateriopathy,characterized by: In situ microthrombosis Plexiform lesion formationLeads to progressive increasein pulmonary vascularresistance and culminates inright heart failure and deathThree key pathogeneses: Relative decrease in bioavailability of NO Relative increase in serum endothelin-1 Relative deficieny of PGI2/excess of thromboxane A2The term PH representsincreased PAP but not due tointrinsic vascular disease
8. 8. HistopathologyMain vascular changes ofPAH Vasoconstriction SMC and endothelial-cellproliferation fibrosis thrombosis intimamedia1.Severe concentric laminar intimal fibrosis2. Medial hypertrophy3.in situ thrombosis of the small residual lumen
9. 9. The Progression of PAH
10. 10. Symptoms of PAH Dyspnea60% Fatigue19% Near syncope/syncope 13% Chest pain 7% Palpitations 5% LE edema 3%
11. 11. CXR-PA viewEnlargement of the main pulmonary artery and its major branches,with marked tapering of peripheral arteries .
12. 12. CXR-lateral viewEncroachment of the retrosternal air space -- right ventricularenlargement/hypertrophy
13. 13. CXR-PA viewThe right ventricle and atrium may also be enlarged.
14. 14. CTEPH
15. 15. ECGHighly specific butnot very sensitive. Right axis deviation Right atrial and rightventricularenlargement.Right bundlebranch blockST and T wavechanges in theanterior precordialleads.
16. 16. Echocardiogram FindingsIncreased sPAP / TR jetRight atrial and ventricular hypertrophyFlattening of interventricular septumSmall LV dimensionDilated PAPericardial effusion Poor prognostic sign RA pressure so high it impedes normal drainage from pericardium Do not drain, usually does not induce tamponade since RV under high-pressure and non-collapsible
17. 17. Representation of Echocardiographic findings
18. 18. EchocardiogramRight atrial and ventricularenlargement andflattening of theintraventricular septum --parasternal short axisview (a) and four chamberview (b).
19. 19. A Word about HemodynamicsThe right heart catheterization is crucial.Diagnosis and/or treatment choices shouldalmost never be made based uponechocardiography alone, it is a screening tool.Exercise and volume challenges are helpfulduring RHC to refine your diagnosisUseful calculations:mPAP = 1/3 sPAP + 2/3 dPAPPVR = mPAP PCWP / C.O.