1. Role of the Echocardiogram in the Assessment of Pulmonary Hypertension and the Right Ventricle
Antoine Hage, M.D
Director, Solid Organ Transplant Cardiology
Co-Director, Pulmonary Hypertension Program
Cedars Sinai Heart Institute
Clinical Professor of Medicine/ Cardiology
David Geffen School of Medicine at UCLA

2. Role of Echocardiography in Pulmonary Hypertension: Overview
Definition and classification
Role of ECHO in
Diagnosis (allows identification of patients for whom RHC is required)
Screening high risk patient populations
Evaluation of Structure/ Morphology/ Function/ Hemodynamics of RV and PA (CHD)
Determining etiology / PH group (PAH vs PVH) and secondary causes
Risk stratification (Evaluation of functional and hemodynamic impairment)
Formulating therapeutic options, monitoring disease stability and response to therapy (longitutudinal F/U)
Prognostic evaluation

3. Hemodynamic Definition of PH/PAH
Mean PAP ≥25 mm Hg
Mean PAP ≥25 mm Hg plus PCWP/LVEDP ≤15 mm Hg
PAH
Doppler echo is the best noninvasive method to evaluate PAP, and should be used in all patients suspected to have PAH. Cardiac cath is mandatory for the final diagnosis of PAH
The hemodynamic working definition of PAH listed here is derived from the 2009 Proceedings of the 4th World Symposium on PH. In the new recommendations, exercise and PVR criteria have been eliminated.
An accurate PCWP can be difficult to obtain in patients with PH and enlarged pulmonary arteries.
If PCWP is elevated despite multiple attempts, especially if blood obtained in the wedge position is not fully saturated, direct measurement of LVEDP should strongly be considered so as not to misdiagnose patients who have PAH.
In a recent retrospective study of 4300 patients undergoing simultaneous right and left catheterization, 53% meeting criteria for PAH on the basis of a PCWP <15 had a LVEDP >15 (even among patients being evaluated specifically for PH)
Refs: Badesch et al. JACC 2009;126:in press. Halpern SD, Taichman DB. Chest, Mar 24. [Epub ahead of print].
ACCF/AHA CECD includes PVR >3 Wood Units
Badesch D et al. J Am Coll Cardiol. 2009;54:S55-S66.
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:

4. Clinical Classification of Pulmonary Hypertension (Dana Point 2008)
PAH
Idiopathic PAH
Heritable (BMPR2, ALK1, Endoglin)
Drug- and toxin-induced
Persistent PH of newborn
Associated with:
CTD
HIV infection
portal hypertension
CHD
schistosomiasis
chronic hemolytic anemia
1’. PVOD and/or PCH
PH Owing to Left Heart Diseases
Systolic dysfunction
Diastolic dysfunction
Valvular disease
3. PH Owing to Lung Diseases and/or Hypoxia
COPD
ILD
Other pulmonary diseases with mixed restrictive and obstructive pattern
Sleep-disordered breathing
Alveolar hypoventilation disorders
Chronic exposure to high altitude
Developmental abnormalities
4. CTEPH
5. PH With Unclear Multifactorial Mechanisms
Hematologic disorders (MPD, splenectomy,..)
Systemic disorders (sarcoidosis, LAM,..)
Metabolic disorders (e.g Thyroid disorders,.. )
Others (e.g Renal failure/ dialysis, fibrosingmediastinitis,..)
The clinical classification of pulmonary hypertension,updated at the 4th World symposium, is represented here.
PAH is represented in the first subgroup. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are housed within this classification, but in a separate group, distinct from but very close to Group 1 (now called Group 1-prime).
Of note, left heart disease (Category 2) probably represents the most frequent cause of PH. Therefore, it is critically important in the diagnostic work-up to distinguish right heart form left heart disease.
The predominant cause of PH in Category 3 is alveolar hypoxia as a result of lung disease, impaired control of breathing, or residence at high altitude.
Patientswith suspected or confirmed CTEPH (Category 4) should be referred to a center with expertise in the management of this disease.
Group 5 comprises several forms of PH for which the etiology is unclear or multifactorial.
Simmoneau G et al. J Am Coll Cardiol. 2009;54:S43-S54.
Simonneau G et al. J Am Coll Cardiol. 2009;54;S43-S54.

5. When to Suspect and Screen for PAH
Family history
6% - 12% prevalence of positive family history. If BMPR2 present, 20% chance of developing PAH.
Autosomal dominant, incomplete penetrance, genetic anticipation
Connective tissue disease
Limited and diffuse scleroderma: 8%- 30%
CREST: up to 20% - 25%
Systemic lupus erythematosus: 4% - 14%
Rheumatoid arthritis up to 21%
Congenital Heart Disease
Reversal of left-to-right shunt
Ventricular septal defect, patent ductusarteriosus, atrialseptal defect
Portal hypertension
Nearly 10% have elevated right ventricular systolic pressure by echo and RHC.
4% have severe PAH to contraindicate liver transplantation
Deep venous thrombosis/history of pulmonary embolism
Up to 3-4% of survivors
Appetite suppressant or stimulant use
1/20000 of Fen Phen users more than 3 months
Methamphetamine use
HIV
0.5% (1/200) patients.
Sickle cell disease, hemodialysis patients, etc

6. Echocardiography in Pulmonary HTN
Echocardiography is an integral part of the assessment of a patient with PH, Often the first test to detect PH
Evaluates cardiac structure, function and hemodynamics
Rules out congenital heart diseases and shunts*
Provides a reasonably accurate estimate of pulmonary artery pressures
Guides diagnosis and therapy
Helps determine prognosis: Many Echo parameters are prognostic indicators:
RV size and function (eg,TAPSE, S’, FAC, MPI/TEI index)
Pericardial effusion
Estimate of CO/CI and RA pressure (hemodynamics)
* May need TEE

7. Role of Echocardiography in the Screening of Patients at Risk of Developing PH/ PAH
The substantial time delay from symptom onset to definite diagnosis in PAH remains an unresolved issue. This has relevant clinical implications, especially when considering the better prognosis and response to treatment with early detection of the disease (WHO class I or II, 6-min walk distance > 450 m, normal or mildly increased B-type natriuretic peptide, no evidence of right-heart failure). Regular echocardiographic screening of patients at high risk for PAH or with unexplained symptoms of fatigue or dyspnea is essential and provides an overall good sensitivity and specificity.1

8. When to Suspect and Screen for PAH
Family history/ Heritable PAH
Connective tissue disease
Congenital Heart Disease
Portal hypertension
Deep venous thrombosis/ history of pulmonary embolism
HIV patients
Appetite suppressant or stimulant use (Methamphetamine)
Sickle cell disease, hemodialysis patients/fistula, sarcoidosis, post-splenectomy etc.

9. Echocardiographic Criteria Corresponding to Various Levels of Likelihood of Presence of Pulmonary Hypertension
Peak tricuspid jet velocity m/sec
Estimated sPAP mm Hg
Other echo signs of PH present*
PH ( grade of evidence)
< 2.8
<36 No
Unlikely (I-B)
Yes*
Possible (IIa- C)
Possible (IIa-C)#
> 3.4
> 50
Yes/No
Likely (I-B)
* Eg, if RV morphology and function and/or systolic time intervals such as PAAT, or mid systolic deceleration of right ventricular ejection (notching) are suggestive of PH, such diagnosis should
be considered “possible” even if Doppler estimate of sPAP is within normal range .
# TRV > 2.8 m/Sec corresponds to TIPG > 31 mm Hg, suggest PH except in elderly
or very obese patients
Galie N, et al: EurRespir J. 2009; 34;

10. Screening Patient Groups at Risk of Developing PAH
Yearly echocardiography is recommended in patients
At risk for heritable PAH
With CTD, especially patients with scleroderma
Some recommend echo Q 2 years if normal BNP and :
DLCO > 70% and
FVC% /DLCO% < 1.6
With sickle cell disease
Echocardiography should be considered, in patients with PH-suggestive symptoms
After pulmonary embolism
With HIV infection
With portal hypertension
With prior appetite suppressant use
With sarcoidosis
After splenectomy

11. Limitations of Echocardiography in PAH1,2
Experienced technicians and interpreting physicians are essential
Consistency of skilled technicians/readers
Applies to all imaging modalities
Images can be limited in some patient populations
The RV, the chamber of highest concern in PAH, is the least emphasized on the “standard” echocardiography exam
TR jet may be absent in some patients, thus precluding PASP assessment
May overestimate or underestimate actual pulmonary arterial pressure
Can estimate LVEDP (PCWP) or CO/CI, but may prove impractical
CI, cardiac index; CO, cardiac output; LVEDP, left ventricular end diastolic pressure; PASP, pulmonary arterial systolic pressure; PCWP, pulmonary capillary wedge pressure; RV, right ventricle; TR, tricuspid regurgitation.
1. Cheitlin et al. Circulation. 1997;95: McGoon et al. Chest. 2004;126:14S-34S.

12. ECHO FEATURES OF PULMONARY ARTERIAL HYPERTENSION
Right atrial enlargement
Right ventricular enlargement/dilatation - D-shaped LV on short axis
Right ventricular hypertrophy
Significant tricuspid regurgitation – (TR jet estimated RVSP is 4V2 + RAP)
Right ventricular dysfunction
Pulmonary regurgitation - PR jet estimated mPA and PAEDP
Reduced RV outflow tract velocity, short acceleration time
Dilated IVC not collapsing with respiration (if patient not ventilated)
Patent foramen ovale (bubble contrast used)
Pericardial effusion
Dilated pulmonary arteries

13. Echocardiographic Findings that Increase Clinical Suspicion of PVH
Absence of right heart chamber enlargement or pericardial effusion
Evidence of left atrial enlargement
Presence of left ventricular hypertrophy
Impaired diastolic relaxation indices
Elevated left ventricular filling pressures as determined by E/e’
ratio >15 (most reliable predictor of LA pressure >15 mmHg)
Extent of functional MR and size of mitral valve regurgitant orifice
at rest or during exercise (predictor of increased PA and pulmonary
edema in ischemic heart disease and probably in HFpEF)
Modest elevation of pulmonary pressures (i.e., 60’s rather than >
80’s)

14. Findings that Increase the Clinical Suspicion of PVH
Age (elderly)
Female gender
Obesity
Systemic Htn (particularly if not optimally controlled) and LVH
Diabetes mellitus
Coronary artery disease
Obstructive sleep apnea
Atrial fibrillation
EKG findings:
Lack of right axis deviation
Lack of right atrial enlargement or RVH
Evidence of left atrial enlargement
Evidence of left ventricular hypertrophy
Chest X-ray findings:
Pulmonary vascular congestion/ Kerley B lines
Pulmonary edema
Pleural effusion

15. Progression of RV Dysfunction in PAH
Champion H C et al. Circulation 2009;120:

16. Role of Echo in Diagnosis of PH and Assessment of PA / RV Hemodynamics

17. Estimating Pulmonary Artery Pressures by EchoTR PR TR TR
Mean
Diast 2*
SPAP
= 4TR Vmax2 + RAP
TR Vmax= Peak TR velocity
Diast PA
= 4PRend Vmax2 + RAP
PRend Vmax= End PR velocity
Mean PA
= TR Vmean+ RAP
Or = 4PR Vmax2 + RAP
PR Vmax= Peak PR velocity
TR Vmean=from VTI
Modified from Garvan Kane

18. Mean Pulmonary Artery pressure
The most reproducible method to estimate mean PA pressure is based on the mean Doppler gradient of the tricuspid regurgitant (TR) signal
MEAN RV-RA SYSTOLIC GRADIENT. Aduen et al recently proposed a novel and simple method to estimate mPAP on the basis of the ad- dition of RAP to the RV-RA mean systolic gradient obtained by tracing the TRv profile. This method was validated in 102 patients, compar- ing it with simultaneous right-heart catheterization; it showed great re-liability (mean difference with invasively obtained pressures, 1.6 mm Hg; median absolute percentage difference, 18%) and accuracy in diagnosing PH (area under the curve, 0.92; 95% confidence inter- val, ). The addition of saline contrast did not improve accuracy. This method appears straightforward and could easily be incorporated into a standard echocardiographic exam, allowing a reliable estimation of mPAP.
Mean PA Mean systolic Estimated
Pressure RV-RA gradient RA pressure = +
Aduen JF, et al. J Am Soc Echocardiogr : 22;

19. Pulmonary Artery Mean and Diastolic Pressures
mPAP
= 79 – 0.45x (PAAT)
= x (130)
=79-59
= 20 mm Hg
=79 – 0.45x (PAAT)
= x (70)
= 79 – 32
= 47 mm Hg
PA Diastolic Pressure
[PADP = 4 x (end-diastolic pulmonary
regurgitant velocity)² + RA pressure]
Mean PA Pressure
mPAP = 1/3(SPAP) + 2/3(PADP)
Or 4 x (early PR velocity)² + estimated RA pressure
Or: 0.61 xsPAP + 2 mm Hg ( Chemla’s Equation)
Or: x (PAAT)
Or: 90 – (0.62 x PAAT)
Or mPAP= 80 – 0.5 x (PAAT)
Or: RAP + VTI of TR jet
Derivation of mPAP by pulsed Doppler echocardiography. A, Positioning of the pulsed Doppler echocardiography sample volume in the main PA. B and C, Case of a patient without (B) and with (C) severe PAH. PAAT is the time in milliseconds from the beginning of the Doppler envelop to the peak of the signal, as shown by the dotted lines (time is in the horizontal axis). On the basis of the formula shown, the mPAP can be estimated, although for heart rates <60 beats/min or >100 beats/min, this has not been validated. In addition to the shortening of the PAAT in severe PAH, the decreased compliance of the PA and the transmission of a reflective wave of blood result in a characteristic midsystolic interruption of flow, as indicated by the arrows. AoV = aortic valve; mPAP = mean pulmonary artery pressure; PA = pulmonary artery; PAAT = pulmonary artery acceleration time; PAH = pulmonary artery hypertension.
CHEST. 2011;139(5):

20. Pulmonary Acceleration Time
PV AccT 72 ms
mPA = 47 mm Hg
Mean PA pressure = 79 - (0.45 x AT)

21. PA in Pulmonary vascular disease
Before and after therapy.
CTEPH- Before PTE
Same pt After PTE

22. Right Atrial Pressure Estimate: IVC and Hepatic Vein
Right atrial (RA) pressure estimate should not not be based on an arbitrary value, but rather based on 2D and Doppler imaging of the IVC and hepatic veins*
Modified from Garvan Kane
*Hepatic veins > 11 mm is abnormal

23. Right Atrial Pressure Estimate: IVC and Hepatic Vein
American Society of Echocardiography Recommends:
3mmHg, IVC diameter <21mm w/ >50% collapse
8mmHg, IVC normal in diameter w/ <50% collapse
15mmHg, IVC diameter >21mm w/ >50% collapse
20mmHg, IVC dilatation with <50% collapse
2D and M-Mode examples of dilated IVC without collapse. On right, a dilated Hepatic Vein (normal value 5-11 mm.)
Some use the following RA estimates:
American Society of Echocardiography Recommends:
3mmHg, IVC diameter <21mm w/ >50% collapse
8mmHg, IVC normal in diameter w/ <50% collapse
15mmHg, IVC diameter >21mm w/ >50% collapse
20mmHg, IVC dilatation with <50% collapse

24. Right Atrial Pressure Estimate: Hepatic Vein Flow in PAH Patients
Systolic filling fraction: Vs/ (Vs + Vd) < 55% sensitive and specific for increased RA pressure
Abnormal: A wave is larger than systolic S wave
Abnormal
The example on the right shows low systolic filling fraction and increased A wave (compared to S wave.) A A A D S S D D
Normal: Systolic predominance in hep. vein flow
Abnormal: Vs/Vd< 1 (eg; High RA pressure)

25. How should you define mild, moderate, and severe PH?
Not by RVSP
Not by the ratio of RVSP to systemic BP
Define PH severity by
the degree of:
RV dilatation
RV dysfunction
RA pressure elevation
Decrease in cardiac index
Modified from Garvan Kane

26. Right Ventricular Afterload
RESISTANCE
PVR: mean PAP – PCWP = TPG
Flow (CO) CO
Reflects the arterial load to steady flow
Doppler correlate:
Peak TR pressure gradient / RVOT TVI
(although does not incorporate LV filling pressure)
Modified from Garvan Kane

27. PVR PVR = [(TRV/TVIRVOT) x 10] + 0.16 (Abbas Formula)*
= (3.9 / 10.2) x ; = 0.38 x ; =
= 3.98 WU
PVRc = (RVSP – E/e’) / VTIRVOT (Corrected Dahiya equation)#
*Abbas, AE et al. JACC :
#Dahiya, A et al . Heart :

28. PA – W Sign

29. Right Ventricular Afterload
Compliance / Capacitance
Can be estimated by SV / Pulse Pressure (by cath or ECHO)
May be as (more) important in PH as resistance
Pulsatile component of pressure and flow is 30-50% of power transferred from RV to pulmonary bed
Less than 1.0 is abnormal and < 0.8 mL/mm Hg predicts mortality in PAH patients
PVCAP = Stroke Volume = LVOT Area x TVI
PA Pulse Pressure* (TR Vmax2 – PRendV2)
PVCAP = Pulmonary vascular capacitance
* PA systolic – PA diastolic
Mahapatra, S et al. J. Am Soc Echocardiogr, 2006, 19:

30. Indirect Echocardiographic Findings in PH
Mean PA pressure :
= 79 - (0.45x PAAT)
Or = 90 – (0.62 x PAAT)
Or =80 – 0.5 x (PAAT)
PV AccT 72 ms
= 47 mm Hg
RVOT Acceleration time < 90 msec
“Flying W” sign by M-Mode (mid-systolic notching)
Dilated Coronary Sinus
Decreased PV acceleration time in patient with PH. Dilated coronary sinus in a patient with Carcinoid Heart Disease.
Reynolds, BS, RDCS, Terry. The Echocardiographer’s Pocket Reference. Arizona: Print.

31. Echocardiographic Features of PAH
Left:RVE/RAE . Apex forming RV
Upper right LPAX
Lower right : Septal flattening. Short axis, parasternal

32. RV Apical 4 Chamber view: Obtain apical long axis views optimized to visualize RV
RV centric
LV centric
Can be obtained by sliding the transducer to a more lateral position than the standard Apical-4.

33. RA / RV Morphology in PAH
RV Dilatation / LV Compression
Flattening / D-shaped Septum
RAE / IAS Displacement
Apex-forming RV

34. RV size: Qualitative “Eyeball” Estimate
Mild RVE
Normal
Normal
RV Similar to LV/ Shares apex
RV 2/3 size of LV
Severe
RVE
Moderate
RVE
Very large RV/ Apex forming
D shaped septum
RV Larger than LV

35. Quantitative Estimate of RV Size
Length (> 86 mm*)
Mid diameter (> 35 mm*)
Basal diameter (>42 mm*)
RV area > 28 cm2*
* Measures indicate dilatation
RV end-diastolic diameter has been identified
as a predictor of survival in patients with
chronic pulmonary disease
Tips
Measure at end diastole from an RV
focused apical 4-chamber view
Optimize image to have maximum
diameter without foreshortening the
ventricle
Rudski,LG et al. J. Am Soc Echocardiogr :
RVH: RV thickness > 0.5 cm
LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.
Voekel et al. Circulation. 2006;114:
Images courtesy of Kirk Spencer, MD, and Lissa Sugeng, MD, MPH, University of Chicago.

36. RV measurements in 2D
RV end-diastolic diameter has been identified as a predictor of survival in patients with chronic pulmonary disease
RV Enlargement:
RVD1 > 42 mm or
RVD2 > 35 mm or
RV Length > 79 mm
RV Area > 28 cm2
RV dimesnsions mesaured in the Apical-4 view. The RV wall dimension measured in the subcostal view.
RVH: RV thickness > 0.5 cm
J Am Soc Echocardiogr 2010;23:

37. Septal Flattening- Eccentricity Index
Eccentricity Index : D1/D2 > 1
In Diastole= volume overload
In Systole= volume and pressure overload
E.I = 40/25 = 1.6 (D1/D2)

38. Pericardial Effusion: A bad Sign in PH
Associated with greater
disease severity
Increases mortality risk
Likely reflects high venous
pressure and poor lymphatics drainage rather than a risk of cardiac tamponade

39. Echo and RV Function 3D ECHO might become gold standard
No other valid quantitative assessment of RV function
Subjective evaluation
Tricuspid annular plane systolic excursion (TAPSE)
Tricuspid annulus TDI velocities (S’)
RIMP (Tei Index)
RV area fractional shortening
Dp/dt
RV longitudinal strain measurement
Measurement of cardiac output

40. Echo measures of RV Function: TAPSE
Simple, reproducible
Represents longitudinal function
Correlates well with radionuclide angiography in determining RV systolic function. Relatively load dependent.
Normal > 20 mm.
TAPSE < 18 mm has negative prognostic implications
Angle and load dependent
To execute:
In Apical-4 chamber view, place M-Mode cursor through the lateral tricuspid annulus
Measure excursion from end-diastole to
end-systole
Average over 3 beats
Off-axis views tend to overestimate TAPSE (as with the apex-forming RV)
J Am Soc Echocardiogr 2010;23: , Heart 2006;92:i19-i26 doi: /hrt

41. Echo Measures of RV Function: TV annular velocity (S’) by TDI
MPI=(TCO-ET)/ET
Simple, sensitive, reproducible
Good indicator of basal free wall function
By TDI, several indices of RV function can be obtained from a single cardiac cycle
Peak velocities
Isovolumic parameters
Tei index
Angle dependant
Relatively independent of loading conditions
Correlated with RVEF by first pass radionuclide ventriculography
Normal > 10 cm/s
Normal MPI by TDI < 0.55
Our lab prefers this method of obtaining MPI since it is usually very simple to obtain a quality TDI, and multiple other measurements can also be made at the same time.

42. The RV Index of Myocardial Performance (RIMP)
Global Indicator of Systolic and Diastolic Function.
Needs the measurements
of 2 different cardiac cycles
(tricuspid inflow and RV
outflow by PW Doppler)
Normal values below 0.4
(mean 0.28)
Relatively independent of HR
and from loading conditions
Prognostic in PH
May get pseudonormalized
with high RVDP/ RAP
Figure 6. The RV myocardial performance index (MPI). E indicates the rapid filling velocity; A, atrial filling velocity; IVCT, isovolumic contraction time; IVRT, isovolumic relaxation time; and ET, ejection time.
Haddad F et al. Circulation 2008;117:
Tei C, et al: J Am Soc Echocardiogr. 1996; 9:

43. RV FAC (Fractional Area Change) / Apex-forming RV
RV FAC%= 100x RVarea diastole – RVareasystole / Rvareadiastole
/ 27.2 = 20%

44. Dp/dt Rate of rise of LV or RV pressure
Normal dp/dt> 400 mm Hg/sec

45. Role of ECHO in Therapy, Longitudinal F/U and Prognosis

46. Prognostic Parameters and Determinants of Risk in PAH
LOWER RISK
DETERMINANTS OF RISK
HIGHER RISK No
Clinical evidence of RV failure
Yes
Gradual
Progression of symptoms
Rapid
II, III
WHO class IV
Longer (>400 m)
6MWD
Shorter (<300 m)
Peak VO2>10.4 mL/kg/min
CPET
Peak VO2<10.4 mL/kg/min
Minimal RV dysfunction
Echocardiography
Pericardial effusion, significant RV enlargement/dysfunction; RA enlargement
(TAPSE in ESC guidelines)
RAP <10 mm Hg; CI >2.5 L/min/m2
Hemodynamics
RAP >20 mm Hg; CI <2.0 L/min/m2
Minimally elevated
BNP
Significantly elevated
Syncope is poor prognostic sign added in the ESC guidelines
McLaughlin VV et al. J Am CollCardiol. 2009;53:

47. Prognostic Value of Echo Parameters of RV Function
47 pts with PAH
2-year survival = 88 % if TAPSE > 18 mm
2-year survival = 50 % if TAPSE < 18 mm
Forfia PR – Am J RespirCrit Care Med 2006; 174:
Prognostic Value of MPI
Meluzin J – Eur J Echocardiogr 2003; 4:
Prognostic Value of MPI
YeoTc – Am J Cardiol 1998; 81:
Tei, C- JASE 1996; 9:
Van Wolferen, SA et al. Eur Heart J (2007) 28, 1250–1257

48. Kaplan-Meier Survival Curves for Echocardiographic Predictors of Outcomes
81 pts prostacyclinevs placebo
F/U 36 months, 20 deaths, 21 transplantations
Right atrial area > 20 cm2, abnormal; > 27 cm2 associated with poor prognosis
Eccentricity index > 1 abnormal; > 1.7 carries poor prognosis
Raymond, R. J. et al. J Am CollCardiol 2002;39:

49. Suggested Assessments and Timing for Follow-ups in patients with PAH: ESC Guidelines

50. Role of Echo in Determination of PH Group

51. Determining PH Type on Basis of ECHO
Caution is needed in distinguishing PAH from PH related to diastolic abnormalities solely on the basis of ECHO
Features of “diastolic dysfunction”, e.g., delayed relaxation pattern and reduced e’ (mitral annular tissue velocities), may occur in PAH ( secondary to bad RV)
Pulmonary vascular resistance (PVR) calculations by ECHO do not take into account left atrial pressure- they do not distinguish PAH from PVH.

52. Left-sided vs. Right-sided Origin of Pulmonary Hypertension
Left-Sided Origin of PH
Right-Sided Origin of PH
2-D Echocardiographic Findings:
LVH, LAE
Normal LV size, normal LA size
Variable LV function
Normal LV function
Normal RV size
RV dilation (ratio of RV:LV size >1)
No interventricular septal bowing
Right to left interventricularseptal bowing
Atrial septum neutral or bowed to right
Atrial septum bowed to left
Normal or mildly reduced RV function
Mild to severe RV dysfunction
No pericardial effusion
Mild to moderate pericardial effusion
Doppler Findings:
≥2+ mitral valve disease (MR or MS)
Minimal or no MR or MS
Grade II or III diastolic dysfunction
Normal diastolic function or grade I diastolic
dysfunction (E:A reversal)
Variable TR
Variable TR (TR severity > MR severity)
Absence of notched pattern in Doppler signal obtained from RVOT
Notched Doppler signal in RVOT
Variable PASP (typically <70 mm Hg)
Variable PASP (typically ≥70 mm Hg)

53. EchocardiographicParameters for the Assessment of Pulmonary Hypertension/ Right Ventricle
Size and surface areas of both atria.
Bi-ventricular size and systolic /diastolic function (RV), presence of RVH, CMY, any valvular abnormality (MS, MR, AS etc..), pericardial effusion or intracardiacshunt:
Subjective “eyeball” assessment of RV function ( good vs mild, moderate or severe RV dysfunction)
Percent Fractional Area Change (% FAC)
Tricuspid Annular Plane Systolic Excursion (TAPSE)
Eccentricity Index / D-shaping of the IVS
RV Myocardial Performance Index (MPI) or Tei index
TDI systolic velocity of the RV lateral annulus (S’), (and short IVRT on RV TDI)
Pulmonary artery pressure estimation / Hemodynamics:
Pulmonary Artery Acceleration Time (PAAT) and presence/ timing of Notching
Pulmonary artery pressures (Systolic, Diastolic, Mean) , Resistance and Capacitance
RA pressure ( IVC size and collapse)
Assessment of C.O ( LVOTdiameter and time-velocity integral of aortic flow by PW Doppler)
Bubble study (CHD, PFO)
3D echocardiography, myocardial deformation techniques (strain imaging or speckle-tracking techniques derived from tissue Doppler ultrasonography) if available
BP should be reported on echo , as well as systolic and diastolic functiona and any valvular abnormality.
ECCENTRICITY INDEX ( NORMAL =1) : SYSTOLIC >1= RV pressure overload
: DIASTOLIC>1= RV volume overload
Tei index relatively independent from loading conditions. Represents global estimate of RV function, independent of geometric assumtions. Normal 0.28 by PULSED DOPPLER MPI Abnormal >0.4 Prolonged in PAH
Limited by mode of calculation based on 2 different cardiac cycle measurement.
Can be obtained by tissue doppler imaging (TDI) which has advantage of obtaining both measurement during same cardiac cycle. Tissue Doppler MPI Abnormal > 0.55

54. Role of ECHO in PH: Summary
ECHO is an essential tool and plays a key role the initial and subsequent evaluation of a patient with PH. Despite its limitations, it remains the most clinically useful nonivasive test for the assessment of the pulmonary circulation
ECHO Provides a reasonably accurate estimate of the RV / PA pressures and hemodynamics .
The prognostic value of the echocardiographic parameters discussed here are well established, and the regular assessment of these, as part of a goal-oriented therapy, is critical to monitor the progression of PH and the response of patients to PAH specific therapy, independent of clinical and RHC data

55. Acknowledgements Lauren Skinner, RDCS: Sonographers RDCS: Partners
Preparation and acquisition of many of the slides
Sonographers RDCS:
Yvonne Golomb
Liliana (Lily) Miranda
Carol Mortier
Yousef Kohen
Partners
Jon Kobashigawa, M.D
Jaime Moriguchi, M.D
Michele Hamilton, M.D
Jignesh Patel, M.D
BabakAzarbal, M.D
Michelle Kittleson, M.D
David Chang, M.D

56. THANK YOU
Any questions?