pulmonaryhypertension-18082714400005.pdf

Pulmonary hypertension

1. DR.MD SHAHID IQUBAL
Depttof 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
holosystolictricuspid regurgitantmurmur
clubbingin chronic lung diseases,
sclerodactylyand 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, valvulardisease
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 oligemiaof pulmonary vasculature (chronic
thromboembolic disease)
Interstitial infiltrates (ILD)
Hyperinflatedlungs(chronic obstructive lung disease).
HRCT-
Enlarged pulmonary arteries ,
peripheral pruning of the small vessels,and
enlarged right ventricle and atrium
Centrilobularground-glass infiltrate
Ventilation–Perfusion (V/Q) lung scan-
Used for excluding chronic thromboembolic disease

9. Diagnostic Testing contd…
Pulmonary Function Testing-
Spirometryand lung volumes to assess forCOPDor
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 exertionalhypoxemia 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 mPAPby ≥10
mm Hg to an absolute level ≤ 40 mm Hg without a
decresein 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)
Antitopoisomeraseantibodies and anticentromere
antibodies
hepatitis B and C serologies

12. TREATMENT
General measures-
Avoid pregnancy
Immunization for respiratory illness
influenza and pneumovaxvaccination
Minimize valsalvamaneuvers-increase risk of
syncope
cough, constipation,heavylifting
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
Septaldefect 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.
Dobutamineand milrinoneare best for short-term use in
extremely decompensated states.

15. PAH SPECIFIC THERAPY
CCBs
Endothelinreceptor antagonists
-Bosentan,ambrisentan,masitentan
Phosphodiesterase-5 inhibitors
-Sildenafil,tadalafil
Prostanoids
-epoprostenol,treprostinil
guanylylcyclasestimulator-riociguat

16. Treatment contd….
CCBs-
Used only in showing positive vasodilator response
Diltiazemor nifedipinepreferred
Endothelinreceptor antagonists-
Endothelinreceptor antagoniststargetendothelin-1 (ET-
1), a potentendogenousvasoconstrictor
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 prostacyclinehas no risk of infection and infusion
site reaction
guanylylcyclasestimulator-
Stimulator of nitric oxide receptor
Riociguatis an oral soluble guanylylcyclasestimulator-
approved for the treatment of both PAH and CTEPH

18. FOLLOWINGRESPONSE TO THERAPY
Six minute walk test
Echocardiogram
Right heart catheterisation
BNP
Funtionalclass

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.