PULMONARY HYPERTENSION

DR DANG QUY DUC

ARRHYTHMIA DEPARTMENT
CARDIOLOGY DEPARTMENT
CHORAY HOSPITAL
SOURCES
p ESC 2015 PH
p Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic
definitions and updated clinical classification of pulmonary hypertension.
Eur Respir J 2019; 53: 1801913.
p Pulmonary Arterial Hypertension in 2019: From death sentence to chronic
disease Trushil Shah, M.D.
p Galiè N, McLaughlin VV, Rubin LJ, et al. An overview of the 6th World
Symposium on Pulmonary Hypertension. Eur Respir J 2018.
p 2019 up to date Clinical features and diagnosis of pulmonary hypertension
of unclear etiology in adults.
p 2019 up to date Treatment of pulmonary hypertension in adults.
p 2019 up to date Prognosis of pulmonary hypertension in adults
p 2020 Canadian Cardiovascular Society/Canadian Thoracic Society
Position Statement on Pulmonary Hypertension
Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT
VI. RETURN PATIENT
DEFINE
p 1973: WHO World Symposium On Pulmonary
Hypertension (WSPH) (mPAP) ≥ 25 mm Hg.
p 2009, Kovacs et. al. performed a systematic review
on right heart catheterization (RHC) data on 1187
individuals: normal mPAP 14 ― 3.3 mm Hg →
mPAP rarely > 20 mm Hg (97.5th %).
p 2018 the 6th WHO World Symposium:
¤ PH - mPAP > 20 mm Hg.
 Vascular Pressure in Systemic and
Pulmonary Circulations (mm Hg)
120/80, mean 93 25/8, mean 14
Systemic Pulmonary
Circulation Arteries Arteries Circulation
Right Left
Atrium Atrium
Mean >6 Mean 5

Body Lung

SVR= 17.6
PVR= 1.8 Right Left
Veins Ventricle Ventricle Veins
25/2-5 120/5-10
PH: The Importance of Hemodynamics
Pulmonary venous hypertension
Mixed etiology
PCWP >15 mm Hg

VC RA RV PA PV LA LV Ao
PC

PAH
PH with respiratory disease Other:
High CO
CTEPH
PCWP ≤15 mm Hg PCWP ≤15 mm Hg
PVR >3 Wood units PVR ≤3 Wood units
 HAEMODYNAMIC DEFINITIONS

SOURCES HAEMODYNAMIC DEFINITIONS

Pulmonary Arterial Hypertension (PCWP) ≤ 15 mm Hg and
(PAH) Pulmonary vascular resistance > 3
Pulmonary hypertension due to left (PCWP) > 15 mm Hg
heart disease
Pulmonary hypertension due to lung (PCWP) ≤ 15 mm Hg and
disease and/or hypoxia concomitant lung disease and/or hypoxia
Pulmonary hypertension due to (PCWP) ≤ 15 mm Hg and
pulmonary artery obstructions an entity causing pulmonary artery obstruction
Pulmonary hypertension with unclear multifactorial mechanisms or
and/or multifactorial mechanisms unclear underlying pathophysiological mechanisms
CLASSIFICATION
p pathophysiological mechanisms,
p clinical presentations,
p hemodynamic characteristics and
p therapeutic management

p Pulmonary hypertension:
¤ 5 main subgroups.
PROGNOSIS

GROUP I GROUP II-V

¨ Without therapy: ¨ the underlying disease,
¤ 1 year: 85%. ¨ response to therapy,
¤ 3 year: 68%.
¨ severity of the PH.
¤ 5 year: 57%.

¨ WHO
¤ I-II: 3%
¤ III: 10%

¤ IV: 21%
Cause of death
p rightheart failure with circulatory collapse and
superimposed respiratory failure.
p CPR: 80% dead
¤ 6% survived > 90 days.
 Acute decompensated
pulmonary hypertension
p Sudden worsening of clinical signs of right heart
failure with subsequent systemic circulatory
insufficiency and multisystem organ failure.

p In-hospital mortality: 14% to 100%.

1. Haddad F, Peterson T, Fuh E , et al. Characteristics and outcome after hospitalization for acute right heart failure in patients with pulmonary arterial hypertension. Circ Heart
Fail 2011; 4: 692–699.
2. Jiang R, Ai Z-S, Jiang X, et al. Intravenous fasudil improves in-hospital mortality of patients with right heart failure in severe pulmonary hypertension. Hypertens Res 2015;
38: 539–544.
 Gradual evolution towards
end-stage pulmonary
hypertension.

Laurent Savale et al. Eur Respir Rev 2017;26:170092

©2017 by European Respiratory Society

Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT
VI. RETURN PATIENT
Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT
VI. RETURN PATIENT
 CLINICAL PRESENTATION
20% patients severe-serious symptoms : 2 years.

p Dyspnea and fatigue.

p Symptoms of right ventricular (RV) :
•Exertional chest pain.
•Exertional syncope.
•Weight gain from edema.
•Anorexia and/or abdominal pain and swelling

Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest 2011; 140:19.
 Functional classification
New York Heart Association functional classification
Class 1 No symptoms with ordinary physical activity.
Class 2 Symptoms with ordinary activity. Slight limitation of activity.
Class 3 Symptoms with less than ordinary activity. Marked limitation of activity.
Class 4 Symptoms with any activity or even at rest.
World Health Organization functional assessment classification
Class I Patients with PH but without resulting limitation of physical activity. Ordinary
physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.
Class II Patients with PH resulting in slight limitation of physical activity. They are
comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest
pain, or near syncope.
Class III Patients with PH resulting in marked limitation of physical activity. They are
comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest
pain, or near syncope.
Class IV Patients with PH with inability to carry out any physical activity without symptoms.
These patients manifest signs of right-heart failure. Dyspnea and/or fatigue may even
be present at rest. Discomfort is increased by any physical activity.
Signs on examination
p Jugular venous pressure (JVP) abnormalities.
p Right-sided auscultatory:
-A right-sided third or fourth heart sound (ie, a gallop) in
association with a left parasternal heave or a downward
subxiphoid thrust.
-Wide splitting of the second heart sound.
-A holosystolic murmur of tricuspid regurgitation,
diastolic pulmonic systolic ejection murmur, diastolic pulmonic
regurgitation murmur.
p Hepatomegaly, a pulsatile or tender liver, peripheral edema,
ascites, and pleural effusion, splenomegaly
Imaging
Chest x-ray:
Imaging
Chest CT scan:
Imaging

Chest CT scan:
Imaging
ECG
Imaging
Imaging: ECHOCARIDIOGRAPHY

Ø TRV > 2.8 m/s or

Ø sPAP > 35 mm Hg

Possible PH
Risk assessment
pulmonary arterial hypertension
Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT
VI. RETURN PATIENT
Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT
VI. RETURN PATIENT
Diagnostic TRV > 2.8 m/s or
sPAP > 35 mmHg
algorithm
DIAGNOSIS

1. PH
2. Classification 5 groups PH
3. Risk assessment pulmonary arterial
hypertension
 DIAGNOSIS PH
RIGHT HEART
ECHOCARDIGRAPHY
CATHETERIZATION

¨ mPAP (supine and at Accuracy of Doppler

rest) >20mmHg. Echocardiographic
SOURCES
¨ HAEMODYNAMIC DEFINITIONS Estimates of Pulmonary
Pulmonary Arterial
Hypertension (PAH)
(PCWP) ≤ 15 mm Hg and
Pulmonary vascular resistance > 3 Artery Pressures in
Pulmonary hypertension
due to left heart disease
(PCWP) > 15 mm Hg
Patients With
Pulmonary hypertension
due to lung disease and/or
(PCWP) ≤ 15 mm Hg and
concomitant lung disease and/or hypoxia Pulmonary
hypoxia
Pulmonary hypertension (PCWP) ≤ 15 mm Hg and Hypertension ???
due to pulmonary artery an entity causing pulmonary artery
obstructions obstruction
Pulmonary hypertension multifactorial mechanisms or
with unclear and/or unclear underlying pathophysiological
multifactorial mechanisms mechanisms
 DIAGNOSIS PH

Conclusions: DE estimates of PASP are inaccurate in

patients with PH and should not be relied on to make the
diagnosis of PH or to follow the efficacy of therapy.
CHEST 2011; 139(5):988–993
DIAGNOSIS PH
Classification 5 groups PH

NUMBER GROUP
1 PAP
2 PH due to left heart disease
3 PH due to chronic lung disease and/or hypoxemia
4 PH due to pulmonary artery obstructions
5 PH due to multifactorial mechanisms
Risk assessment
pulmonary arterial hypertension
Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT PH1
VI. RETURN PATIENT
Objectives

I. PROFILE PATIENT
II. DEFINE
III. CLINICAL PRESENTATION
IV. DIAGNOSIS
V. TREATMENT PH1
VI. RETURN PATIENT
GENERAL TREAMENT
p Oxygen
p Diuretic
p Anticoagulant
p Digoxin therapy
p Exercise
p Vaccinations
p Supportive therapy
Oxygen
p 1-4l/min.
p SpO2 > 90%.
 Diuretic
p fluid retention from PH-related RV failure .

p Oral
p IV: bolus-continuous infusion
p Ultrafiltratration.

p The use of angiotensin-converting enzyme inhibitors,

angiotensin-2 receptor antagonists, beta-blockers and
ivabradine is not recommended in patients with PAH.
 Anticoagulation
p MECHANISM: intrapulmonary vascular
thrombosis and venous thromboembolism and early
studies that suggested a mortality benefit.

p INDICATIONS: group 1 PAH

We recommend against systemic anticoagulation in patients
with PAH associated with:
1.connective tissue disease,
2.congenital heart disease,
3.portal hypertension, and
4.HIV
Digoxin
p COPD and biventricular failure.
p Control AF.
 Exercise training
Mechanism: Conditions:
oxidative stress,
¤
¤ Rehabilitations center.
¤ inflammation,
¤ PH experts.
¤ vasoconstriction,
¤ vascular remodelling, and ¤ Cardiologist,
¤ thrombosis. Pulmonologist.
¤ Physiotherapist.

¤ Psychologist.

¤ Stable in 2 months/medical
therapy
 Supportive therapy
p Vaccinations:
¤ all age.
¤ influenza and pneumococcal pneumonia vaccines.
p Contraception:
¤ progesterone-only preparations such as medroxyprogesterone
acetate and etonogestrel (bosentan may reduce the efficacy of
oral contraceptive).
¤ levonorgestrel-releasing intrauterine coil

¤ A combination of two methods may also be utilised.

p Avoid pregnant. Surgical (patient or partner) methods.

p Travel: WHO-FC III and IV and those with arterial blood O2 <
60mmHg with supplemental O2.
p In elective surgery: epidural rather than general anaesthesia.
 PH SPECIFIC THERAPY

pPH-targeted therapy > the underlying

cause of the PH.
pPH with WHO functional class II, III, or
IV despite treatment of the underlying
cause → PH-specific therapy centers.
1. improved exercise capacity,
2. reduced disease progression, and
3. improved survival.
 PH1 SPECIFIC THERAPY

30 mg per day. Increase to the

Nifedipine LA Oral maximum tolerated dose over days
to weeks.
120 mg per day. Increase to the
Diltiazem Oral maximum tolerated dose over days
extended-release to weeks.
2.5 mg per day. Increase to the
Amlodipine Oral maximum tolerated dose over days
to weeks.
 Vasoreactivity test
p Testing:
¤ the administration of a short-acting vasodilator
¤ followed by measurement of the hemodynamic response using an RHC. Agents commonly
used for vasoreactivity testing include inhaled nitric oxide, epoprostenol, adenosine, and
inhaled iloprost :
●Inhaled nitric oxide 10 to 20 ppm. It is selective for the pulmonary vasculature with minimal
systemic effects and is therefore better tolerated than the intravenous agents listed below.
●Epoprostenol 1 to 2 ng/kg per min and increased by 2 ng/kg per min every 5 to 10 minutes until
a clinically significant fall in blood pressure, an increase in heart rate, or adverse symptoms (eg,
nausea, vomiting, headache).
●Adenosine 50 mcg/kg per min and increased every two minutes until uncomfortable symptoms
develop or a maximal dose of 200 to 350 mcg/kg per min is reached.
p The test (+):
¤ meanPAP decreases at least 10 mmHg and

¤ a minimally reduced or unchanged systemic blood pressure.

 Vasoreactivity test

NOT:
1. use of oral or intravenous CCBs in acute
vasoreactivity testing
2. PH groups 2, 3, 4 and 5.
Mechanism of PH1 target therapy
PH (group 1) therapy
WHO functional class
Initial drug combination
PH (group 1) WHO functional class

¨ Low risk: initial oral

monotherapy .
¨ Intermediate risk: initial dual
oral combination therapy
¨ High risk: + I.V. epoprostenol;
I.V./subcutaneous treprostinil.
THE LAST THERAPY
Ø RIGHT TO LEFT SHUNT
Ø atrial septostomy and placement of a Potts shunt via a
transcatheter.
Ø Severely high pulmonary vascular resistance (from
obstructive shock)→ reduction in left ventricular preload,
systemic pressure→ elevating systemic blood flow and
maintaining tissue perfusion, albeit with less oxygenated
blood.
Ø ­ cardiac output and ­ systemic oxygen: 27%.

Ø refractory severe PAH and right heart failure, despite

aggressive advanced therapy and maximal diuretic therapy.
THE LAST THERAPY
TRANSPLANTATION
p Bilateral lung or heart-lung transplantation
p 3 year survival patients: 50%
Correction
congenital heart disease with shunts
 Treatment
congenital heart disease with shunts
p Bosentan: WHO-FC III patients with Eisenmenger
syndrome.
p Other ERAs, PDE-5’s and prostanoids: Eisenmenger
syndrome
p oral anticoagulant treatment: thrombosis or signs of heart
failure.
p Hyperviscosity (Hct > 65%): isovolumic replacement
p Supplemental iron treatment
p The use of CCBs is not recommended in patients with
Eisenmenger syndrome.
 PREGNENCY

p Endothelin receptor antagonists (bosentan)

and guanylate cyclase stimulants (riociguat):
FDA category X.
p Prostanoid (epoprostenol).
p Warrantting: fetal and maternal
complications.
KẾT LUẬN

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