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The Diagnosis of Pulmonary Hypertension:

Multimodality from Non Invasive to Invasive


Diagnostic

Hasanah Mumpuni
KSM Jantung /Departemen Kardiologi dan Kedokteran Vaskular
RSUP Dr. Sardjito / FK-KMK UGM
Case
• Women, 20 years old, with complaints of fatigue, sometimes
accompanied by shortness of breath since 6 months. The last 2
weeks often experience coughing, without fever .
• Physical exam. Vital sign BP 110/70, P 110 x/mnt, Respiration
22/ mnt
• Cardiac status : Wide fix splitting in upper left sternal border, P2
harden.
• ECG : RVH, complete RBBB
• Assesment → CHF , Susp Atrial septal defect, Pulmonal
hypertension ??
• What supporting investigations are needed ??
1. Electrocardiography
➢ Right ventricular hypertrophy or strain → These include
right-sided axis deviation, an R-to-S wave ratio greater
than 1 in lead V1,
➢ increased P-wave amplitude - RAE
➢ an incomplete or complete right bundle-branch block
pattern.
2. Chest Radiography

• Elevated cardiac apex due


to right ventricular
hypertrophy
• Enlarged right atrium
• Prominent pulmonary
outflow tract
• Enlarged pulmonary
arteries
• Pruning of peripheral
pulmonary vessels
3. Echocardiography in PH
Echocardiography → the first test to detect PH → Estimation of
pulmonary artery systolic pressure to determine if PH is
present.
➢ 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:
➢ RV size and function (eg,TAPSE)
➢ Pericardial effusion
➢ Estimate of CO/CI and RA pressure (hemodynamics)
Echocardiography – 2D
• On 2D echocardiography, signs of chronic right
ventricular pressure overload are present;
• increased thickness of the right ventricle and
paradoxical bulging of the septum into the
left ventricle during systole.
• Right ventricular dilatation occurs, leading to
right ventricular hypokinesis.
• Right atrial dilatation,
• septal flattening, tricuspid regurgitation,
pulmonic insufficiency,
Echocardiography - Doppler
➢ The most reliable noninvasive
method of estimating pulmonary
arterial pressure.

➢ Tricuspid regurgitation →
measurement of pulmonary
arterial pressure with the
modified Bernoulli equation.

➢ Tricuspid regurgitation is
generally detected in more than
90% of patients with severe PH,
and a correlation of greater than
95% by means of catheterization.

➢ Doppler echocardiography is a
useful noninvasive test for long-
term follow-up.
Echocardiography in patients with suspected PH;
The following echo parameters should be used to assess the
probability of PH:
1. Peak Tricuspid Regurgitation (TR)velocity
2. Ventricle
1. Eccentricity index
2. Basal LV/RV diameter ratio
3. Pulmanale Artery (PA)
1. RVOT acceleration time and/or mid systolic notching
2. Early diastolic PR velocity
3. PA diameter
4. Right Atrial (RA) and Inferior Vena Cava (IVC)
1. RA area
2. IVC size and respiratory variability
Probability of PH
Tricuspid Regurgitation Doppler

• TR velocity
• TR gradien
Echocardiographic signs suggesting PH to assess
the Probability of PH in addition to TR Velocity

c
RV – LV basal diameter Rasio:
Qualitative “Eyeball” Estimate

Mild RVE
Normal
Normal

RV Similar to LV/ Shares apex


RV 2/3 size of LV
Severe
Moderate RVE
RVE

RV Larger than LV Very large RV/ Apex forming


D shaped septum
Septal Flattening- Eccentricity Index

D1

D2

Eccentricity Index : D1/D2 > 1


In Diastole= volume overload E.I = 40/25 = 1.6 (D1/D2)
In Systole= volume and pressure
overload

14
c
Pulmonary Acceleration Time
< 105 msec

Mean PA pressure = 79 - (0.45 x AT) PV AccT 72 ms


mPA = 47 mm Hg
Early Diastolic PR Velocity > 2,2 m/s
PA diameter > 25 mm
IVC diameter >21mm w/ <50% collapse

• 3mmHg, IVC diameter <21mm w/ >50% collapse


• 8mmHg, IVC normal in diameter w/ <50% collapse
• 15mmHg, IVC diameter >21mm w/ <50% collapse
Right Atrial Area > 18 cm²
Estimating Pulmonary Artery Systolic Pressure
• Echocardiographic evaluation of pulmonary artery systolic pressure
(PASP),
• PASP approximates right ventricular systolic pressure (RVSP) in the
absence of right ventricular outflow obstruction.
• The most accurate echocardiographic method for estimating (PASP) uses
the simplified Bernoulli equation to obtain a systolic trans-valvular
pressure gradient12. →TR
• DPRV-RA = 4(VTR)2 → VTR : velocity of TR
• PASP » RVSP = 4(VTR)2 + RAP
• The accuracy of this method depends the continuous wave Doppler
beam being parallel to the regurgitant jet. A further limitation of this
method is the false assumption that tricuspid regurgitation is present in
all patients with PH.
• PH is unlikely if VTR < 2.8m/s and PASP < 36mmHg in the absence of
other echocardiographic findings suggestive of PH. If V TR > 3.4m/s and
PASP >50mmHg, the diagnosis of PH is highly likely.
5. Right-Sided Cardiac Catheterization
(RHC)
➢Right-sided heart catheterization is the procedure of choice in
the diagnosis, quantification, and characterization of
pulmonary hypertension. Left-sided heart dysfunction and
intracardiac shunts can be excluded, and the cardiac output
can be measured.
➢The indications for right-sided cardiac catheterization are as
follows:
▪ Making a definitive diagnosis of pulmonary hypertension
▪ Measuring pulmonary pressures accurately (ie, when there is
difficulty in measuring pulmonary pressures with Doppler
echocardiography)
▪ Conducting a vasoreactivity test for assessment of the acute
response to vasodilators.
Right heart catheterisation – the diagnostic gold standard1

25
Right heart catheterisation – the diagnostic gold standard

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Parameter in RHC
1. mPAP ≥ 25 mmHg (PH)
2. PA-WP ≤ 15 mmHg
3. PVR → > 3 Wood unit (WU)
PVR = ( mPAP – mLAP ) / CO or
PVR = ( mPAP –PCWP ) / CO
• mPAP – mLAP = Transgradien pulmonal (TGP),
• mLAP similar PAWP
Recommendation for RHC in PH
Ventilation-Perfusion Lung
Scanning
• Ventilation-perfusion scanning should be
performed to exclude CTEPH. A high- or low-
probability scan result is most useful, whereas
intermediate-probability results should lead to the
performance of pulmonary angiography.
• Diffuse mottled perfusion can be observed in
patients with pulmonary arterial hypertension
(PAH), as opposed to the segmental or
subsegmental mismatched defects observed in
patients with CTEPH (see the image below).
The Diagnose of PH

Suggestive clinical
features

• Assess Cardiac cause of PH


Echocardiography • Assess RV fuction, RVSP, RAE, RVE

• Pulmonary function test


• Sleep study
Workup for other • Ventilation perfusion scan
causes • Serology ; HIV , connective
tissue disease markers

- Functional test
- RHC
- Vasodilator test
Diagnostic Algorithm for PH

• ESC
Notes
33
here 2015
Compare measurements Echocardiography
with RHC

• PVR by echocardiography →
Metode Abbas = 0.618 + 10.006xTRV/TVI RVOT.
• PCWP=1.9 + 1.24 x E/E’ (high>15 mm Hg)
Take home message
• The diagnosis of PH begins with a clinical suspicion of PH
• There are several supporting examinations that can be used to support
the presence of PH
• Echocardiography is an integral part of the assessment of a
patient with PH, the first test to detect PH, and has a high
diagnostic value
• Gold standard PH is RHC, according to the PH definition
• PH is defined as an increase in mean pulmonary arterial pressure
(PAPm) ≥25 mmHg at rest as assessed by right heart
catheterization (RHC)