This document discusses pulmonary hypertension (PH), defining it as a mean pulmonary artery pressure over 22 mmHg. PH is classified into 5 groups, with Group 1 being pulmonary arterial hypertension (PAH). PAH is defined by a mPAP over 25 mmHg and PCWP under 15 mmHg on right heart catheterization. Symptoms are nonspecific but include dyspnea and fatigue. Diagnosis involves echocardiogram, right heart catheterization, and tests like CT, V/Q scan, and PFTs. Treatments include diuretics, anticoagulants, oxygen, PAH-specific therapies like prostanoids, ERAs, PDE5is, and transplant for severe cases.

1. DR.MD SHAHID IQUBAL
Deptt of Medicine
NMCH,PATNA

2. INTRODUCTION
Definition-
Pulmonary hypertension (PH) is a spectrum of diseases
involving the pulmonary vasculature, and is defined as an
elevation in pulmonary arterial pressures (mean pulmonary
artery pressure >22 mmHg).

3. Classification -
PH is subcategorized into five major groups :
 Group I—Pulmonary arterial hypertension
(PAH)
 Group II—PH due to left heart disease
 Group III—PH due to lung diseases and/or
hypoxemia(COPD, Interstitial lung disease, OSA)
 Group IV—Chronic thromboembolic
pulmonary hypertension (CTEPH)
 Group V—PH with unclear mechanisms

4. Pulmonary Arterial Hypertension
 PAH is defined as a sustained elevation in resting
mPAP ≥25 mmHg, and PCWP or left ventricle end-
diastolic pressure of ≤15 mmHg based on a right heart
catheterization.
 Idiopathic pulmonary arterial hypertension (IPAH) is a
progressive disease that leads to right heart failure and
death
 PAH associated with HIV, connective tissue
disease(systemic sclerosis),and portal hypertension.

5. DIAGNOSIS
Clinical presentation-
Symptoms of PH-
 The symptoms of PH are often nonspecific and variable
 include dyspnea (most common),
 exercise intolerance,
 fatigue,
 palpitations,
 syncope,
 chest pain,
 lower extremity swelling,

6. DIAGNOSIS contd….
On examination-
evidence of right heart failure-
 elevated jugular venous pressure,
 lower extremity edema, and
 ascites.
cardiovascular examination-
 prominent second heart sound (loud S2) with loud P2
componentound, a right-sided S3 or S4, and a
holosystolic tricuspid regurgitant murmur
 clubbing in chronic lung diseases,
 sclerodactyly and telangiectasia in scleroderma, and
 crackles and systemic hypertension in left-sided systolic
or diastolic heart failure

7. Diagnostic Testing-
echocardiogram –(TTE)
 Is screening test
 Shows hypertrophied and dilated right ventricle with
elevated estimated pulmonary artery systolic
pressure
 Identify causes of PH -left ventricular systolic and
diastolic dysfunction, valvular disease
right heart catheterization -
 for definitive diagnosis
 Measure cardiac output and mean right atrial
pressure (RAP) and asses severity condition

8. Diagnostic Testing contd…
Chest x-ray-
 Large central pulmonary arteries with “vascular pruning,”
 Cardiomegaly due to enlarged RA ,RV
 Regional oligemia of pulmonary vasculature (chronic
thromboembolic disease)
 Interstitial infiltrates (ILD)
 Hyperinflated lungs(chronic obstructive lung disease).
HRCT-
 Enlarged pulmonary arteries ,
 peripheral pruning of the small vessels,and
 enlarged right ventricle and atrium
 Centrilobular ground-glass infiltrate
Ventilation–Perfusion (V/Q) lung scan-
 Used for excluding chronic thromboembolic disease

9. Diagnostic Testing contd…
 Pulmonary Function Testing-
Spirometry and lung volumes to assess for COPD or
restrictive- interstitial lung disease [ILD])
Diffusing capacity for carbon monoxide (DLCO)
reduced in parenchymal
 lung diseases
Arterial blood gas (ABG): Elevated PaCO2 is found in
hypoventilation syndrome.
Six-minute walk (6MW) or simple exercise test-
 to evaluate the degree of exertional hypoxemia and
 to monitor progression and response to therapy
Nocturnal oximetry:.
 Nocturnal desaturations is common finding in PH
 Nocturnal desaturations can be find in OSAS

10. Diagnostic Testing contd…
ECG-
 RVH,
 right atrial enlargement,
 right bundle branch block, and
 right ventricular strain pattern
Acute vasodilator testing.-
 Performed with a short-acting vasodilator, such as
intravenous adenosine, intravenous epoprostenol, or
inhaled nitric oxide
 Definition of a responder is decrease in mPAP by ≥10
mm Hg to an absolute level ≤ 40 mm Hg without a
decrese in CO.
 responders are considered for long-term treatment with
CCBs.

11. Diagnostic Testing contd…
Laboratories
 Complete blood counts (CBCs)
 Blood urea nitrogen, serum creatinine
 Hepatic function tests
 B-type natriuretic peptide (BNP)
 Human immunodeficiency virus (HIV) serology
 Thyroid-stimulating hormone (TSH)
 Antinuclear antibody (ANA)
 Antitopoisomerase antibodies and anticentromere
antibodies
 hepatitis B and C serologies

12. TREATMENT
General measures-
 Avoid pregnancy
 Immunization for respiratory illness
influenza and pneumovax vaccination
 Minimize valsalva maneuvers-increase risk of
syncope
cough, constipation,heavy lifting
 Should stop cigarette smoking

13. Classes of therapy
 MEDICAL-
 Diuretics
 Anticoagulants
 Inotropic agents
 Oxygen
 PAH specific therapy
 SURGICAL THERAPY-
 Lung transplantation or heart–lung transplantation
 Atrial septostomy
 Septal defect closure

14. Treatment contd…
DIURETICS-
 Principally to treat edema from right heart failure
 May need to combine classes(loop diuretic +aldosterone
antagonist+ thiazides)
 Careful to avoid too much preload reduction
ANTICOAGULANTS-
 Warfarin is dosed to target international normalized ratio
(INR) of 1.5 to 2.5
INOTROPIC AGENT
 improves right heart function, cardiac output, and
symptoms.
 Dobutamine and milrinone are best for short-term use in
extremely decompensated states.

15. PAH SPECIFIC THERAPY
CCBs
Endothelin receptor antagonists
-Bosentan,ambrisentan,masitentan
Phosphodiesterase-5 inhibitors
-Sildenafil,tadalafil
Prostanoids
-epoprostenol,treprostinil
guanylyl cyclase stimulator-riociguat

16. Treatment contd….
CCBs-
 Used only in showing positive vasodilator response
 Diltiazem or nifedipine preferred
Endothelin receptor antagonists-
 Endothelin receptor antagonists target endothelin-1 (ET-
1), a potent endogenous vasoconstrictor
 improve symptoms, 6MWD,and WHO functional class
Phosphodiesterase-5 inhibitors-
 block the enzyme that shuts down nitric oxide–mediated
vasodilation and platelet inhibition.

17. Treatment contd….
Prostanoids-
 epoprostenol,treprostinil
 Epoprostenol - continuous intravenous infusion improves
functional capacity and survival in PAH.
 Inhaled prostacycline has no risk of infection and infusion
site reaction
guanylyl cyclase stimulator-
 Stimulator of nitric oxide receptor
 Riociguat is an oral soluble guanylyl cyclase stimulator-
approved for the treatment of both PAH and CTEPH

18. FOLLOWING RESPONSE TO THERAPY
 Six minute walk test
 Echocardiogram
 Right heart catheterisation
 BNP
 Funtional class

19. Take home message-
 Idiopathic pulmonary arterial hypertension is a
progressive disease
 Transthoracic Echocardiography is screening test
 Definitive diagnosis by right heart catheterization
 there is no cure for PAH, current pharmacologic therapies
improve morbidity and, in some cases mortality
 Improved awareness among clinicians and patients could
lead to more timely diagnosis that will affect the response
to therapy and survival.