1. Ipertensione polmonare
Eco e diagnosi: vantaggi, limiti, errori evitabili
Michele D’Alto
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 hypertension Venice 2003 revised 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. Echocardiography for PH diagnosis: pitfalls
TVR
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):
- 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 mmHg
ICV >25mm+v.sovr. No rid. RAP 20 mmHg
Mod from Otto CM, 2002

12. Estimated right atrial pressure
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 65 HIV pts studied Good quality Doppler
Am J Respir Crit Care Med 2009;179:615–621
65 HIV pts studied
Good quality Doppler
Poor quality Doppler
95% limits of agreement: and mmHg

14. additional echo variables
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
• (AcT)
PAPm =
• 44.3 = = 59
PAPm = 57
Mean PAP

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

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:

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

21. What determines PAPm? PVR = ΔP / Q PVR PVR = (PAPm – PWP) / Q ΔP 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
PAH
High output
LV dysfunction
Argiento, Eur Respir J 2009

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

24. Midsystolic pulmonary artery notching = High PVR
Rats were treated with monocrotaline for:
0 (A),
15 (B),
22 (C),
37 (D) days.
0 d monocrotaline
15 d monocrotaline
Midsystolic pulmonary artery notching. Rats were treated with monocrotaline (MCT) for 0 (A), 15 (B), 22 (C), and 37 (D) days. Pulse-wave Doppler of pulmonary outflow was recorded in the parasternal view at the level of the aortic valve. Sample volume was placed (5 mm) proximal to the pulmonary valve leaflets and aligned to maximize laminar flow. Note the early notching at day 15 (B) and its subsequent progression.
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 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. 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%) %
Portal hypertension %
HIV infection %
Anorexigen drugs %
Unoperated shunt %
*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. PAH 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. PAH PVH 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
PAH
PVH
Group 1 Dana Point
Group 2 Dana Point

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