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Ipertensione polmonare Eco e diagnosi: vantaggi, limiti, errori evitabili

Ipertensione polmonare Eco e diagnosi: vantaggi, limiti, errori evitabili. Michele D’Alto mic.dalto@tin.it. UOC Cardiologia II Università degli Studi, A.O. “V. Monaldi” - Napoli. Pulmonary hypertension: general definitions. 2009. Pulmonary hypertension: haemodynamic definition. 2009.

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Ipertensione polmonare Eco e diagnosi: vantaggi, limiti, errori evitabili

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  1. Ipertensione polmonare Eco e diagnosi: vantaggi, limiti, errori evitabili Michele D’Alto mic.dalto@tin.it UOC Cardiologia II Università degli Studi, A.O. “V. Monaldi” - Napoli

  2. Pulmonary hypertension: general definitions 2009

  3. Pulmonary hypertension: haemodynamic definition 2009

  4. WHO classification of pulmonary hypertensionVenice 2003revised Dana Point 2008 1. Pulmonary arterial hypertension • Idiopathic PAH • Heritable PAH(BMPR2, ALK1..) • Drugs and toxins • Associated with CTD, HIV, • portal hypertension, congenital heart diseases, chronic hemolytic anemia (SSD) and shistosomiasis • PPHN 1’ PVOD, PHCM 2. PH with left heart disease • Systolic dysfunction • Diastolic dysfunction • Valvular 3. PH with lung diseases/hypoxemia • COPD • Interstitial lung diseases • Sleep-disordered breathing • Altitude exposure • Alveolar hypoventilation • Developmental abnormalities 4. CTEPH No more distinction proximal/distal 5. Miscellaneous Sarcoidosis, histiocytosis X, Gaucher,..

  5. Normal estimated PAPs value at echo? 37 mmHg, but…

  6. TVR Echocardiography for PH diagnosis:pitfalls • Poor Doppler signal • Uncertain TVR peak • Theta angle RV systolic pressure estimation Simplified Bernoulli ΔP = 4 (V)2 TVR (simplified Bernoulli) + RAP estimation • Arbitrary From ICV to… RAP

  7. Echocardiography, age and body size Circulation 2001;104: 2797–802 J Am Coll Cardiol 2009;54:S55–66 • 3790 “normal” subjects (1358 M, 2432 F) from 1 to 89 years. • PASP calculated by modified Bernoulli equation, with RAP assumed to be 10 mmHg. +10

  8. Echocardiography, age and body size Circulation 2001;104: 2797–802

  9. Echocardiography for PH in SSc Arthritis Rheum 2005;52(12):3792-3800 • - 21 SSc expert centers • - 599 SSc patients (-29 known PAH = 570) • Reliability of prospective screening of SSc patients based on: • TVR >2.5 m/s in symptomatic patients • or TVR >3.0 m/s irrespective of symptoms. 33 patients 45% of cases of echocardiographic diagnoses of PH were falsely positive!

  10. Echocardiography for PH in SSc Rheumatology 2004; 43:461-6 137 SSc pts studied false pos echo false neg cath

  11. Estimated right atrial pressure Systolic PAP = RV-RA gradient + RAP ICV < 15mm collasso RAP 0-5 mmHg ICV 15-25mm rid. >50% RAP 5-10 mmHg ICV >25mm rid. <50% RAP 10-15 mmHg ICV >25mm+v.sovr. No rid. RAP 20 mmHg Mod from Otto CM, 2002

  12. Echocardiography for PH in HIV Am J Respir Crit Care Med 2009;179:615–621 65 HIV pts studied Estimated right atrial pressure IVC <20mm Collaps >50% IVC <20mm Collaps <50% IVC >20mm Collaps >50% IVC >20mm Collaps <50%

  13. Echocardiography Am J Respir Crit Care Med 2009;179:615–621 65 HIV pts studied Good quality Doppler Poor quality Doppler 95% limits of agreement: +38.8 and -40.0 mmHg

  14. 2009 • PH possible: • PASP 37-50 mmHg (TVR 2.9-3.4 m/s) • additional echo variables • PH likely: • - PASP >50 (TVR > 3.4 m/s)

  15. 2009 Echocardiography Direct PH signs Indirect PH signs • PASP > 37 (50) mmHg • Increased velocity PV reg (mPAP) • Short acc. time in RVOT (mPAP) • Right heart dilation • Flat IV septum (LV EI <0.8) • Increased RV wall thickness

  16. Indirect PH signs: PAPm 79 - 0.45 • (AcT) PAPm = 79 - 0.45 • 44.3 = 79 - 20 = 59 PAPm = 57 • Mean PAP

  17. PA 57 mm Ao Indirect PH signs:Right heart (and PA) dilation

  18. Right atrium: and PAH cm2/m (area/altezza) Raymond, RJ, J Am Coll Cardiol 2002;39:1214–9

  19. Right atrium size Normal value: <16 cm2 <9 cm2/m <40 ml <20 ml/m2 Raymond RJ, J Am Coll Cardiol 2002;39:1214–9 Wang Y, Chest 1984;86:595-601

  20. RV LV D1 D2 Indirect PH signs:flat IV septum, hypertrophic RV wall Left ventricular (LV) eccentricity index (EI): D2/D1 in short axis view (normal value = 1) EI = 0.65

  21. What determines PAPm? PVR = ΔP / Q PVR ΔP PVR = (PAPm – PWP) / Q Q PVR X Q = PAPm – PWP PVR X Q + PWP = PAPm High output LV dysfunction PAH

  22. Three different conditions with high estimated PAPm (PVR X Q) + PWP = PAPm High output PAH LV dysfunction Argiento, Eur Respir J 2009

  23. Normal LV filling pressure Precapillary PH first diagnosis NO PAH or very end-stage Assessment of LV filling pressures PCWP = 1.9 + (1.24 x E/Ea) 9/60 (15%) mistakes Nagueh et al. JACC 1997 & Circulation 2000

  24. Midsystolic pulmonary artery notching = High PVR • Midsystolic pulmonary artery notching. • Rats were treated with monocrotaline for: • 0 (A), • 15 (B), • 22 (C), • 37 (D) days. 0 d monocrotaline 15 d monocrotaline 22 d monocrotaline 37 d monocrotaline Jones J E, Am J Physiol Heart Circ Physiol 2002;283:364-71

  25. Midsystolic pulmonary artery notching = High PVR Normal High PVR Very high PVR

  26. Midsystolic pulmonary artery notching = High PVR Why? = reverse wave for high PVR

  27. Pre-test probability: the Bayes’ theory The probability of an event A given an event B (e.g., the probability of CAD given a positive stress test) depends not only on the relationship between events A and B (i.e., the accuracy of stress test) but also on themarginal probability (or "simple probability") of occurrence of each event in a specific population. Rev. Thomas Bayes, 1763 Stress test for CAD detection: - CAD prevalence in group A = 50%; test + = 82% CAD - CAD prevalence in group B = 3%; test + = 13% CAD

  28. Population at risk for PAH J Am Coll Cardiol 2008;51:1527–38 • Relatives of IPAH patients • Associated condition for PAH • Connective tissue disease (CREST* 30%, SSc 10%) 10-15% • Portal hypertension 1-6% • HIV infection 0.5-1% • Anorexigen drugs 0.006-0.01% • Unoperated shunt 5-10% *CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia)

  29. Pre-test probability of precapillary PH 2009

  30. Pre-test probability of pre-capillary PH high RA > LA RV > LV D-shaped LV

  31. Pre-test probability of pre-capillary PH low RA < LA RV < LV Normal shaped LV

  32. RV adaptation to pressure overload PAH RV hypertrophy and progressive dilatation Tricuspid regurgitation and RA dilatation Paradoxical septal motion and altered LV filling Diastolic and systolic RV dysfunction Pericardial effusion in the more severe cases LV dysfunction Haddad et al. Circulation 2008

  33. Pulmonary arterial or venous hypertension? RV dilation/hypertrophy LV dilation/hypertrophy RA enlargement LA enlargement E/A <1 (mild diastolic dysf) E/A >1 (pseudonorm/restr) PAH predisposing condition Left heart disease D-shape LV Normal LV shape PA notch No PA notch PVH PAH Group 2 Dana Point Group 1 Dana Point

  34. ECHO Take-at-home message • The gold standard for PAH diagnosis remains right heart catheterization! • It is strongly encouraged a deep knowledge of PAH pathophysiology (echo as part of clinic evaluation!). • Echo plays a key-role in screening, differential diagnosis and follow-up. • Echo does not provide “magic numbers”: multi-parametric evaluation! • It is mandatory to evaluate the PAH “pre-test probability”.

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