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

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Presentation on theme: "Ipertensione polmonare Eco e diagnosi: vantaggi, limiti, errori evitabili Ipertensione polmonare Eco e diagnosi: vantaggi, limiti, errori evitabili UOC."— Presentation transcript:

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

2 Pulmonary hypertension: general definitions 2009

3 Pulmonary hypertension: haemodynamic definition

4 WHO classification of pulmonary hypertension Venice 2003 revised Dana Point Pulmonary arterial hypertension Idiopathic PAH Idiopathic PAH Heritable PAH (BMPR2, ALK1..) Heritable PAH (BMPR2, ALK1..) Drugs and toxins Drugs and toxins Associated with CTD, HIV, Associated with CTD, HIV, portal hypertension, congenital heart diseases, chronic hemolytic anemia (SSD) and shistosomiasis portal hypertension, congenital heart diseases, chronic hemolytic anemia (SSD) and shistosomiasis PPHN PPHN 1’ PVOD, PHCM 2. PH with left heart disease Systolic dysfunction Systolic dysfunction Diastolic dysfunction Diastolic dysfunction Valvular Valvular 3. PH with lung diseases/hypoxemia COPD COPD Interstitial lung diseases Interstitial lung diseases Sleep-disordered breathing Sleep-disordered breathing Altitude exposure Altitude exposure Alveolar hypoventilation Alveolar hypoventilation Developmental abnormalities 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 Echocardiography for PH diagnosis: pitfalls RV systolic pressure estimation TVR (simplified Bernoulli) + RAP estimation TVR Simplified Bernoulli ΔP = 4 (V) 2 From ICV to… RAP Poor Doppler signal Poor Doppler signal Uncertain TVR peak Uncertain TVR peak Theta angle Theta angle Arbitrary Arbitrary

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 - 21 SSc expert centers 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. Arthritis Rheum 2005;52(12): % of cases of echocardiographic diagnoses of PH were falsely positive! 33 patients

10 Echocardiography for PH in SSc Rheumatology 2004; 43: SSc pts studied cath echo false neg false pos

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

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

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

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

15 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 2009

16 Indirect PH signs: PAPm Mean PAP Mean PAP (AcT) PAPm = = = 59 PAPm = 57

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

18 Right atrium: and PAH Raymond, RJ, J Am Coll Cardiol 2002;39:1214–9 cm 2 /m (area/altezza)

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:

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

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

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

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

24 Midsystolic pulmonary artery notching = High PVR Jones J E, Am J Physiol Heart Circ Physiol 2002;283: 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

25 Midsystolic pulmonary artery notching = High PVR

26 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 the marginal probability (or "simple probability") of occurrence of each event 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 the marginal 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 Associated condition for PAH Associated condition for PAH Population at risk for PAH - Connective tissue disease (CREST* 30%, SSc 10%) 10-15% - Portal hypertension1-6% - HIV infection0.5-1% - Anorexigen drugs % - Unoperated shunt 5-10% Relatives of IPAH patients Relatives of IPAH patients *CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) J Am Coll Cardiol 2008;51:1527–38

29 Pre-test probability of precapillary PH 2009

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

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

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

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


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