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Pulmonary Hypertension

Due to Left Heart Disease


Diagnosis and Management
Luh Oliva Saraswati Suastika
Cardiologist - Echocardiography Consultant
FK Universitas Udayana/RS Universitas Udayana
Definition, Classification,
Pathophysiology
PULMONARY
HYPERTENSION (PH)
• ESC/ERS 2022: A mean pulmonary artery pressure
(mPAP) > 20 mmHg at rest (by right heart
catheterization).
• Prevalence: 1% of global population.

• PH due to left heart disease (PH-LHD): mPAP > 20


mmHg and pulmonary capillary wedge pressure
(PCWP) > 15 mmHg by RHC.
• Most common types of PH (50-80%).

Humbert et al. European Heart Journal (2022) 43, 3618–3731.


Al-Omary et al. Hypertension (2020), 75(6):1397-1408
CLASSIFICATION OF PH (WHO)

PAH LEFT HEART LUNG DISEASE PULMONARY UNCLEAR /


DISEASE / HYPOXIA ARTERY MULTIFACTORI
Idiopathic
OBSTRUCTION AL
HFpEF
Heritable Hematological
HFrEF Obstructive lung disease CTEPH
Connective tissue disorders
Valvular heart disease Restrictive lung disease Other PA
disease Systemic disorders
Congenital à post Hypoventilation obstructions
Congenital heart Metabolic disorders
capillary PH syndromes
disease

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
MPAP PCWP
Pre-capillary PH PCWP Post-capillary PH
15 mmHg
mPAP >20 mmHg mPAP >20 mmHg
and and
PCWP <15 mmHg PCWP >15 mmHg
and and
PVR > 3 Wood units PVR < 3 Wood units

(2) Left ventricular


disease

(5) Renal failure,


systemic disorder
(1) Vascular remodeling
(4) Thromboembolic occlusion (3) Alveolar- (2) Left sided
(5) Complex congenital heart disease capillary valve disease
destruction
PATHOPHYSIOLOGY
Diagnosis
DIAGNOSTIC
APPROACH TO PH

1. Recognize suspected PH cases à refer


to PH centers (esp. patients with a high
likelihood of PAH, CTEPH, or other
forms of severe PH)
2. Identify underlying disease à proper
management

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
Symptoms of PH

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
Signs of PH

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
Patients with Exertional Dyspnea and/or Suspected PH

What should GPs do?

MEDICAL PHYSICAL BNP / NT PRO-


ECG O2 SATURATION
HISTORY EXAMS BNP

Symptoms of PH Signs of PH P pulmonale Reduced saturation Heart failure


Right axis deviation with stable
RV hypertrophy hemodynamic:
RBBB chronic hypoxia
Other abn. related to
left heart disease
Humbert et al. European Heart
Journal (2022) 43, 3618–3731.
Patients with Exertional Dyspnea
and/or Suspected PH-LHD

MEDICAL PHYSICAL BNP / NT PRO-


ECG O2 SATURATION
HISTORY EXAMS BNP

DOE, PND, Murmur (mitral, LVH Normal, unless: Increased: left or


orthopnea aortic valves) P mitral pulmonary edema, right HF
Palpitation Cardiomegaly A-fib mixed with pre-
Leg swelling Rales LBBB capillary PH
Peripheral congestion Infarction/ischemia

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
CHEST X-RAY REALLY HELPS…

SIGNS OF PH & SIGNS OF LEFT SIGNS OF SIGNS OF LUNG


CONCOMITANT PULMONARY DISEASE
ABNORMALITIES HEART DISEASE CONGESTION

Right heart enlargement Left atrial enlargement Kerley B lines Emphysema: flattening of
PA enlargement (with LVH) diaphragm, hyperlucency
Pleural effusion
Peripheral pruning
CONFIRMATION OF PH: REFER TO…
PH Team
Cardiologist Pulmonologist

OTHER DIAGNOSTIC TESTS:


HEART ASSESSMENT LUNG ASSESSMENT • Ventilation/perfusion scan
• Echocardiography • Pulmonary function test • CT pulmonary angiography
• Arterial blood gas • Routine biochemistry, hematology,
• CPET
• Chest CT immunology, HIV tests
• Abdominal ultrasound
• CPET

First-line, non-invasive diagnostic


tool for PH assessment

PH-LHD

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
F/44: RHEUMATIC MIXED MITRAL VALVE DISEASE

Severe rheumatic MS and MR


M/56: CORONARY ARTERY DISEASE (HFMREF)

o All chambers dilatation with


Eccentric LVH
o Mildly reduced EF, decreased
RV systolic function
o Severe TR with intermediate
probability of PH
INDICATIONS FOR RHC IN PH-LHD PATIENTS

o RHC is not indicated in patients with LHD as the


only cause of PH or with established underlying
LHD and mild PH (echo)

o Indication of RHC in LHD:


o Suspected PAH/CTEPH
o Suspected CpcPH with severe precapillary
component
o Advanced HF and evaluation for heart
transplantation

Humbert et al. European Heart


Journal (2022) 43, 3618–3731.
Management
PH-LHD Treatment

Heart Failure Patients Survival by PA pressure and RV function

Ghio et al. J Am Coll Cardiol 2001;37:183–8.


Guazzi et al. Am J Physiol Heart Circ Physiol 2013;305:H1373–81.
TREATMENT FOR PH-LHD

Optimal therapy for underlying LHD (I/A)


Diuretics for fluid retention
Drugs approved for PAH are NOT
recommended in patients with PH-LHD (III/A)
PH IN HFREF & HFMREF

FOUR PILLARS PAH DRUGS

• ARNI/ACEi/ARB • Sildenafil:
• Beta Blocker • Improve hemodynamic and exercise
• MRA capacity in small studies, no RCTs.
• SGLT2i • SilHF study: sildenafil did not improve
symptoms, QoL, exercise capacity in
patients with HFrEF and PH.
• May be added for patients with CpcPH
(mixed pre-post capillary PH)
• Bosentan: no efficacy
• Increase in adverse events vs placebo
(fluid retention)

Desai et al. Children (Basel). 2023 Jan 31;10(2):270.


Cooper et al. https://doi.org/10.1002/ejhf.2527
PH IN HFPEF
SGLT2I PAH DRUGS
• Treat the etiology of • Sildenafil:
HFpEF • In IpcPH: no improvement in
• No specific treatment mPAP, clinical or other
for PH in HFpEF hemodynamic parameters
• In CpcPH: improve
hemodynamics, RV function, QoL
at 6 and 12 months vs placebo
INTERATRIAL SHUNT • No recommendation for or
DEVICES against the use of PDE5i in
• No HF events reduction in HFpEF with Cpc-PH
HFpEF • Not recommended for HFpEF
• May trigger vascular with IpcPH
remodeling in pts with PH • Bosentan & Macitentan: no efficacy
due to sustained increase in • Increase in adverse events vs
PA blood flow placebo (fluid retention)
Humbert et al. European Heart
Journal (2022) 43, 3618–3731.
PH IN VALVULAR HEART DISEASE
MITRAL & AORTIC VALVE DISEASE

• Treat significant mitral and/or aortic valve disease as recommended


by guidelines.
• Sildenafil before MV surgery: decreases sPAP intra- and post-op,
NO effect on mPAP, mortality and other outcomes.1
• Sildenafil in patients with persistent PH after successfully
corrected VHD is associated to worse clinical outcomes than
placebo.2

SEVERE TRICUSPID REGURGITATION


• Intervention on severe functional TR à help reduces TR and RV
volume overload
• NOT for patients with TR in PAH
1Villanueva et al. Cardiol Res. 2019 Dec;10(6):369-377.
2Bermejo et al. Eur Heart J. 2018 Apr 14;39(15):1255-1264.
PH-LHD

SUMMARY
Patients with PH-LHD (PH Group 2) have clinical symptoms and
signs of HF and PH.
The diagnosis needs to be confirmed with echocardiography (and
RHC in selected cases).
Treat the underlying LHD.
PAH therapy has conflicting evidence for its impact on PH in LHD.
PDE5i is only recommended in patients with mixed pre- & post-
capillary PH.
“ PULMONARY HYPERTENSION
IS COMPLEX, AND WE ALWAYS


NEED TO LEARN MORE TO
UNDERSTAND
TERIMA
KASIH

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