Ipertensione polmonareIpertensione polmonare

Eco e diagnosi: vantaggi, limiti, errori evitabiliEco e diagnosi: vantaggi, limiti, errori evitabili

Ipertensione polmonareIpertensione polmonare

Eco e diagnosi: vantaggi, limiti, errori evitabiliEco e diagnosi: vantaggi, limiti, errori evitabili

UOC Cardiologia UOC Cardiologia II Università degli Studi, A.O. “V. Monaldi” - NapoliII Università degli Studi, A.O. “V. Monaldi” - Napoli

UOC Cardiologia UOC Cardiologia II Università degli Studi, A.O. “V. Monaldi” - NapoliII Università degli Studi, A.O. “V. Monaldi” - Napoli

Michele D’AltoMichele D’Altomic.dalto@tin.itmic.dalto@tin.it

Michele D’AltoMichele D’Altomic.dalto@tin.itmic.dalto@tin.it

Pulmonary hypertension: general Pulmonary hypertension: general definitionsdefinitions

2009

2009

Pulmonary hypertension: haemodynamic Pulmonary hypertension: haemodynamic definitiondefinition

WHO classification of pulmonary WHO classification of pulmonary hypertensionhypertensionVenice 2003Venice 2003 revised Dana Point 2008revised Dana Point 2008

1. Pulmonary arterial hypertension1. Pulmonary arterial hypertension Idiopathic PAHIdiopathic PAH Heritable PAHHeritable PAH (BMPR2, ALK1..)(BMPR2, ALK1..) Drugs and toxinsDrugs and toxins Associated with Associated with CCTD, TD, HIVHIV,, pportal hypertensionortal hypertension, ,

congenital heart diseases, congenital heart diseases, chronic hemolytic anemia chronic hemolytic anemia (SSD) and shistosomiasis(SSD) and shistosomiasis

PPHNPPHN1’1’ PVOD, PHCM PVOD, PHCM

2. PH with left heart disease2. PH with left heart disease Systolic dysfunctionSystolic dysfunction Diastolic dysfunctionDiastolic dysfunction ValvularValvular

3. PH with lung 3. PH with lung diseases/hypoxemiadiseases/hypoxemia

COPDCOPD Interstitial Interstitial llung ung ddiseasesiseases Sleep-disordered breathingSleep-disordered breathing Altitude exposureAltitude exposure Alveolar hypoventilationAlveolar hypoventilation Developmental abnormalitiesDevelopmental abnormalities

4. 4. CTEPHCTEPHNo more distinction proximal/distalNo more distinction proximal/distal

5. Miscellaneous5. MiscellaneousSarcoiSarcoidosis, histiocytosis X, dosis, histiocytosis X,

Gaucher,..Gaucher,..

Normal estimated PAPs value at Normal estimated PAPs value at echo?echo?

37 mmHg, but…37 mmHg, but…

Echocardiography for PH diagnosis:Echocardiography for PH diagnosis:pitfallspitfalls

RV systolic RV systolic pressure pressure

estimationestimation

TVR (simplified TVR (simplified Bernoulli)Bernoulli)

++RAP RAP estimationestimation

TVTVRR

Simplified BernoulliSimplified Bernoulli

ΔPΔP = 4 (V) = 4 (V)22

From ICV to…From ICV to…

RAPRAP

• Poor Doppler Poor Doppler signalsignal• Uncertain TVR Uncertain TVR peakpeak• Theta angleTheta angle

• ArbitraryArbitrary

Echocardiography, age and body Echocardiography, age and body sizesize

Circulation 2001;104: 2797–802Circulation 2001;104: 2797–802 J Am Coll Cardiol 2009;54:S55–66J 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+10

Echocardiography, age and body Echocardiography, age and body sizesize

Circulation 2001;104: 2797–Circulation 2001;104: 2797–802802

Echocardiography for PH in SScEchocardiography for PH in SSc

- 21 SSc expert centers- 21 SSc expert centers- 599 SSc patients (-29 known PAH = 570)- 599 SSc patients (-29 known PAH = 570)

Reliability of prospective screening of SSc patients based on:Reliability of prospective screening of SSc patients based on:- TVR >2.5 m/s in symptomatic patientsTVR >2.5 m/s in symptomatic patients- or TVR >3.0 m/s irrespective of symptoms.or TVR >3.0 m/s irrespective of symptoms.

Arthritis Rheum 2005;52(12):3792-3800

45% of cases of echocardiographic diagnoses of PH were falsely 45% of cases of echocardiographic diagnoses of PH were falsely positive!positive!

33 patients33 patients

Echocardiography for PH in SScEchocardiography for PH in SSc

Rheumatology 2004; 43:461-6Rheumatology 2004; 43:461-6

137 SSc pts studied137 SSc pts studied

cathcath

ech

oech

o

false negfalse neg

false posfalse pos

ICV < 15mmICV < 15mm collassocollasso RAP 0-5 mmHgRAP 0-5 mmHg

ICV 15-25mmICV 15-25mm rid. >50%rid. >50% RAP 5-10 RAP 5-10 mmHgmmHg

ICV >25mmICV >25mm rid. <50%rid. <50% RAP 10-15 RAP 10-15 mmHgmmHg

ICV >25mm+v.sovr.ICV >25mm+v.sovr. No rid.No rid. RAP 20 mmHgRAP 20 mmHg

SystolicSystolic PAP = RV-RA gradient + PAP = RV-RA gradient + RAPRAP

Mod from Otto CM, Mod from Otto CM, 20022002

Estimated right atrial pressureEstimated right atrial pressure

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 HIVEchocardiography for PH in HIV

65 HIV pts studied65 HIV pts studied

EchocardiographyEchocardiography

Am J Respir Crit Care Med 2009;179:615–621Am J Respir Crit Care Med 2009;179:615–621

95% limits of 95% limits of agreement: agreement: +38.8 and -40.0 +38.8 and -40.0 mmHgmmHg

Good quality Good quality DopplerDopplerPoor quality Poor quality DopplerDoppler

65 HIV pts studied65 HIV pts studied

2009

PH possible:PH possible:

- PASP 37-50 mmHg (TVR 2.9-3.4 PASP 37-50 mmHg (TVR 2.9-3.4 m/s)m/s)

- additional echo variablesadditional echo variables

PH likely:PH likely:

- PASP >50 (TVR > 3.4 m/s)- PASP >50 (TVR > 3.4 m/s)

EchocardiographyEchocardiography

Direct PH signsDirect PH signs Indirect PH signsIndirect PH signs

- PASP > 37 (50) PASP > 37 (50) mmHgmmHg

- Increased velocity PV reg Increased velocity PV reg (mPAP) (mPAP)

- Short acc. time in RVOT (mPAP) Short acc. time in RVOT (mPAP)

- Right heart dilationRight heart dilation

- Flat IV septum (LV EI <0.8) Flat IV septum (LV EI <0.8)

- Increased RV wall thicknessIncreased RV wall thickness

2009

Indirect PH signs: PAPmIndirect PH signs: PAPm

• Mean PAPMean PAP

79 - 0.45 • (AcT)79 - 0.45 • (AcT)

PAPm =PAPm =79 - 0.45 • 44.3 =79 - 0.45 • 44.3 =79 - 20 =79 - 20 =5959

PAPm =PAPm =5757

Indirect PH signs:Indirect PH signs:Right heart (and PA) dilationRight heart (and PA) dilation

57 mmAo

PA

Right atrium: and PAHRight atrium: and PAH

Raymond, RJ, J Am Coll Cardiol 2002;39:1214–9Raymond, RJ, J Am Coll Cardiol 2002;39:1214–9

cmcm22/m /m (area/altezza)(area/altezza)

Right atrium sizeRight atrium size

Normal Normal value:value:

<16 cm2<16 cm2

<9 cm2/m<9 cm2/m

<40 ml<40 ml

<20 ml/m2<20 ml/m2

Raymond RJ, J Am Coll Cardiol 2002;39:1214–9Raymond RJ, J Am Coll Cardiol 2002;39:1214–9Wang Y, Chest 1984;86:595-601 Wang Y, Chest 1984;86:595-601

LVLV

RVRV

Left ventricular (LV) eccentricity index (EI): D2/D1 in short axis view (normal value = Left ventricular (LV) eccentricity index (EI): D2/D1 in short axis view (normal value = 1)1)

D2

D1

Indirect PH signs:Indirect PH signs:flat IV septum, hypertrophic RV wallflat IV septum, hypertrophic RV wall

EI = 0.65EI = 0.65

What determines PAPm?What determines PAPm?

PVR = PVR = ΔP / QΔP / Q

PVR = (PVR = (PAPmPAPm – PWP) / Q – PWP) / Q

PVR X Q = PVR X Q = PAPmPAPm – PWP – PWP

PVR X Q + PWP = PVR X Q + PWP = PAPmPAPm

PAPAHH

High High outpuoutpu

tt

LV LV dysfunctiodysfunctio

nn

PVRPVRΔPΔP

QQ

Three different conditions Three different conditions with high estimated PAPmwith high estimated PAPm

(PVR X Q) + PWP = (PVR X Q) + PWP = PAPmPAPm

PAPAHH

LV LV dysfunctiondysfunction

Argiento, Argiento, Eur Respir J Eur Respir J 20092009

High High outpuoutpu

tt

Assessment of LV filling pressuresAssessment of LV filling pressures

Nagueh et al. JACC 1997 & Circulation 2000

Normal LV filling Normal LV filling pressurepressure

Precapillary PH first Precapillary PH first diagnosisdiagnosis

NO PAH or very end-NO PAH or very end-stagestage

PCWP = 1.9 + (1.24 x E/EPCWP = 1.9 + (1.24 x E/Eaa))

9/60 (15%) 9/60 (15%) mistakes mistakes

Midsystolic pulmonary artery notching = High PVRMidsystolic pulmonary artery notching = High PVR

Jones J E, Am J Physiol Heart Circ Physiol 2002;283:364-71Jones J E, Am J Physiol Heart Circ Physiol 2002;283:364-71

Midsystolic pulmonary artery Midsystolic pulmonary artery notching.notching.

Rats were treated with Rats were treated with monocrotaline for:monocrotaline for:

- 0 (0 (AA), ), - 15 (15 (BB), ), - 22 (22 (CC),),- 37 (37 (DD) days.) days.

0 d monocrotaline

15 d monocrotaline

22 d monocrotaline

37 d monocrotaline

Midsystolic pulmonary artery notching = High PVRMidsystolic pulmonary artery notching = High PVR

Midsystolic pulmonary artery notching = High PVRMidsystolic pulmonary artery notching = High PVR

Why?Why?

= reverse wave for high PVR

Pre-test probability: the Bayes’ theoryPre-test probability: the Bayes’ theory

The The probability of an event A given an event Bprobability of an event A given an event B (e.g., the (e.g., the probability of CAD given a positive stress test) depends not probability of CAD given a positive stress test) depends not only on the relationship between events A and B (i.e., the only on the relationship between events A and B (i.e., the accuracyaccuracy of stress test) but also on the of stress test) but also on the marginal probabilitymarginal probability (or "simple probability") of occurrence of each event(or "simple probability") of occurrence of each event in a specific population.

Rev. Thomas Bayes, 1763Rev. Thomas Bayes, 1763

Stress test for CAD detection:Stress test for CAD detection:

- CAD prevalence in group A = 50%; test + = 82% CAD- CAD prevalence in group A = 50%; test + = 82% CAD

- CAD prevalence in group B = 3%; test + = 13% CAD- CAD prevalence in group B = 3%; test + = 13% CAD

• Associated condition for PAHAssociated condition for PAH

Population at risk for PAHPopulation at risk for PAH

- Connective tissue disease (CREST* 30%, SSc 10%) Connective tissue disease (CREST* 30%, SSc 10%) 10-10-15%15%- Portal hypertensionPortal hypertension 1-6%1-6%- HIV infectionHIV infection 0.5-1%0.5-1%- Anorexigen drugsAnorexigen drugs 0.006-0.01%0.006-0.01%- Unoperated shuntUnoperated shunt 5-10%5-10%

• Relatives of IPAH patientsRelatives of IPAH patients

*CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, *CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia)sclerodactyly, telangiectasia)

J Am Coll Cardiol 2008;51:1527–38

Pre-test probability of precapillary PHPre-test probability of precapillary PH

2009

Pre-test probability of pre-capillary Pre-test probability of pre-capillary PHPH

RARA > LA > LA

RVRV > LV > LV

D-shaped LV D-shaped LV

RARA < LA < LA

RVRV < LV < LV

Normal shaped Normal shaped LVLV

Pre-test probability of pre-capillary Pre-test probability of pre-capillary PHPH

RV adaptation to pressure overloadRV adaptation to pressure overload

RV hypertrophy and progressive dilatationRV hypertrophy and progressive dilatation

Tricuspid regurgitation and RA dilatationTricuspid regurgitation and RA dilatation

Paradoxical septal motion and altered LV fillingParadoxical septal motion and altered LV filling

Diastolic and systolic RV dysfunctionDiastolic and systolic RV dysfunction

Pericardial effusion in the more severe casesPericardial effusion in the more severe cases

LV dysfunctionLV dysfunction Haddad et al. Circulation 2008Haddad et al. Circulation 2008

LV LV dilation/hypertrophydilation/hypertrophy

LA enlargementLA enlargement

E/A >1 E/A >1 (pseudonorm/rest(pseudonorm/rest

r)r)Normal LV shapeNormal LV shape

No PA notchNo PA notch

RV RV dilation/hypertrophydilation/hypertrophy

RA enlargementRA enlargement

E/A <1 (mild E/A <1 (mild diastolic dysf)diastolic dysf)

D-shape LVD-shape LV

PA notchPA notch

Pulmonary Pulmonary arterialarterial or or venousvenous hypertension?hypertension?

Group 1 Dana Group 1 Dana PointPoint

Group 2 Dana Group 2 Dana PointPoint

PAH PAH predisposing predisposing

conditioncondition

Left heart Left heart diseasedisease

Take-at-home messageTake-at-home message

It is strongly encouraged a deep knowledge of PAH It is strongly encouraged a deep knowledge of PAH pathophysiology (pathophysiology (echo as part of clinic echo as part of clinic evaluationevaluation!).!).

The The gold standardgold standard for PAH diagnosis remains for PAH diagnosis remains right right heart catheterizationheart catheterization!!

Echo plays a key-role in Echo plays a key-role in screeningscreening, , differential differential diagnosisdiagnosis and and follow-upfollow-up..

Echo does not provide “magic numbers”: Echo does not provide “magic numbers”: multi-multi-parametric evaluationparametric evaluation! !

It is mandatory to evaluate the PAH “It is mandatory to evaluate the PAH “pre-test pre-test probabilityprobability”.”.