Current management of

pulmonary arterial
hypertension
NURAMALINA BINTI REMAN
112016183
DR. RETNA DEWAYANI, SP. JP
Definition

Mean pulmonary artery pressure of above 25mmHg in the

setting of a normal or reduced cardiac output, with a
normal pulmonary capillary wedge pressure (PCWP) and
elevated pulmonary vascular resistance (PVR)
Classification

Classification of pulmonary hypertension, WHO 2008 (modified and not exhaustive

Epidemiology

1. PAH is not recognised until late and most patients

present in WHO functional class III or IV
2. National Institute of Health (NIH)
 Median survival : 2,8 years untreated
 Collagen vascular disease and HIV- related : worst prognosis
 Congenital heart disease : best prognosis
Clinical presentation

 Non-specific (lethargy, malaise and exercise

intolerance)
 Dyspnoe
 Physical examination
1. Increased intensity of second heart sound
2. Parasternal heave (right chamber hypertrophy)
3. Ventricle fails (peripheral oedema,ascites, elevated JVP)
4. Tricuspid regurgitation
5. Pulsatile hepatomegaly
WHO Functional classification
Treatment

1. Calcium channel blocker (CCB)

 High dose nifedipine/diltiazem
 Patients who respond favourably to vasoreactivity test
 Side effect : peripheral oedema and systemic hypotension
 Close follow up 3-6 months
Treatment
2. Prostanoids (Prostacyclin analogues, PG2)

Epoprostenol
• Short acting sodium salt, intravenous
• Improve haemodynamic, six-minute walk, survival
• Side effect : route of administration (infection,
thrombosis,embolism,leakage and pneumothorax)

Iloprost
• Longer half life (120 minute)
• Nebulizer (pulmonary selectivity and avoid systemic hypotension)
• Adjunctive therapy
Treatment
3. Endothelin-receptor antagonist

Bosentan
• Orally active dual receptor blocker (ET4 and ET8)
• Initiation of treatment at an early stage
• Hepatic dysfunction and teratogenic

Ambrisentan

• Orally selective receptor blocker (ET4)

• Once daily
• Lower hepatotoxic effect

Macitentan

• Orally active dual receptor blocker (ET4 and ET8)

Treatment

4. Phophodiesterase inhibitor
 Sildenafil : specific phosphodiesterase type-5
 Side effect : flushing, epistaxis, heartburn, headache, interaction with
other drugs (bosentan and nitrates)

5. Soluble guanylate cyclase stimulators

 Riociguat : stimulates guanylate cyclase and synergic action with
endogenous nitric oxide
 Side effect : bleeding,dyspepsis, AKI, elevation of liver enzymes,
hypotension
Treatment

6. Prostacyclin-receptor agonist
 Selexipag : target the same receptor as PGI2, but structurally
different
 Side effect : headache, jaw pain, GI upset
Conclusion

 The diagnosis and management of PAH has undergone a dra-

matic transformation over the last two decades. Newer thera- pies
developed on the background of a greater understanding of the
molecular pathogenesis of the condition have entered routine
clinical practice and have improved patient survival and wellbeing.
Moreover, a large number of experimental studies are continuing,
and there is great hope that it will not be long before more
treatments are made available that potentially offer a cure for this
disease.
THANK YOU