diagnosis and management of pulmonary hypertension

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.