Basics of Pulmonary Hypertension

1. Pulmonary Hypertension

2.  Pulmonary hypertension is defined as an
mPAP of 25 mm Hg or more
 Pulmonary capillary wedge pressure (PCWP)
of 15 mm Hg or less
 Pulmonary vascular resistance (PVR) of more
than 3Wood units

3. 1) Pulmonary arterial hypertension
 BMPR2 mutation (chromosome 2 (2q31-32) )
 Connective tissue disease
 Human immunodeficiency virus (HIV) infection
 Portal hypertension
 Congenital heart diseases
 Schistosomiasis
 Pulmonary venoocclusive disease and/or pulmonary capillary
hemangiomatosis
 Drugs (Dasatinib)
2) Pulmonary hypertension due to left heart disease
3) Pulmonary hypertension due to lung diseases and/or hypoxia
4) Chronic thromboembolic pulmonary hypertension(CTEPH) and other
pulmonary artery obstructions
5) Pulmonary hypertension with unclear and/or multifactorial mechanisms

4. PRE CAPILLARY
 PCWP < 15 mm Hg
 PVR > 3Wood units
 Group 1, 3, 4, 5
POST CAPILLARY
 PCWP > 15 mm Hg
 PVR - Normal
 PH due to left heart
disease
 Group 2

5.  LIMITED CUTANEOUS SSc is associated with PAH
than DIFFUSE CUTANEOUS SSc
 HIV
 independent of the CD4+ cell count,
 no correlation between the stage of HIV infection and the
development of PAH.
 Eisenmenger syndrome
 Reversal of L to R sunt (ASD,VSD, PDA)

6.  Exertional dyspnea
 Chest pain
 Fatigue
 light-headedness.
 Manifestations of more advanced disease -
attributable to right ventricular failure
 Syncope
 Abdominal distention
 lower extremity edema

7.  Loud S2 (Audible at apex)
 Early systolic click
 Midsystolic ejection murmur(pulmonary area)
 Holosystolic murmur that increases with inspiration (Tricuspid
Area)(Carvallo's sign)
 Left parasternal lift (heave)
 Increased jugular A wave , Increased jugularV waves
 Pulsatile liver
 Pulmonary regurgitation
 Hepatojugular reflux
 Right ventricular S3
 Distention of jugular veins
 Hepatomegaly
 Peripheral edema
 AscitesLow blood pressure, diminished pulse pressure, cool extremities

11.  RHC (Right heart catheterisation)
 RHC with pulmonary vasodilator testing
 inhaled nitric oxide (NO), or inhaled epoprostenol
 A decrease in mPAP by ≥10 mmHg to an absolute
level ≤40 mmHg without a decrease in CO is
defined as a positive pulmonary vasodilator
response, and
 responders are considered for long-term
treatment with calcium channel blockers (CCB)

14.  Prostanoids given through all
routes(IV/SC/inhaled/oral) -Treprostinil
 Binds prostaglandin I₂ (IP) receptor - Activate
adenylate cyclase – ATP to cAMP – decrease Ca2
inside the muscle –Vasodilatation
 Selexipag - oral nonprostanoid - prostaglandin I2
(IP) receptor agonist

15. •Selective ET-A receptor antagonist
•Ambrisentan
•Non-selective receptor antagonists
•bosentan and macitentan

17.  Phosphodiesterase type 5 enzymes
metabolize cGMP
 cGMP phosphodiesterase type 5 (PDE5)
inhibitors prolong the vasodilatory effect of
NO
 Sildenafil (20 mgTID)
 Tadalafil (20 mg OD)

18.  Riociguat (0.5 mgTID)

19.  All the drugs for PAH – oral
 Exception: Prostanoids

20.  Surgery is the treatment of choice for CTEPH
 Pulmonary Endarterectomy