Pulmonary hypertension is defined by specific hemodynamic criteria and can be categorized into 5 groups. Group 1 includes pulmonary arterial hypertension which can be caused by various genetic, medical, and environmental factors. Groups 2-5 involve pulmonary hypertension secondary to other conditions such as left heart disease, lung disease, chronic thromboembolic pulmonary hypertension, or unclear mechanisms. Diagnosis involves right heart catheterization and testing for vasodilator response. Symptoms range from exertional dyspnea to right heart failure. Treatment depends on the type and severity of pulmonary hypertension and may include vasodilator medications, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostanoids, or surgery for chronic thromboembolic pulmonary

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