This document discusses the treatment of pulmonary arterial hypertension (PAH). It notes that PAH is characterized by vascular proliferation in the small pulmonary arteries, resulting in increased pulmonary vascular resistance and eventual right heart failure. While the pathogenesis is complex and diagnosis can be late, understanding of the disease has led to many new drug treatments. These include prostanoids, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and inhaled nitric oxide, which work by different mechanisms. Combination drug therapy may be used for severe, unresponsive cases. Interventional procedures are an option when drug therapies fail. Guidelines recommend starting with calcium channel blockers or diuretics for early, low-risk cases and progressing to approved drugs for severe

1. By
Essam Mahfouz, MD
Professor of cardiology, Mansoura University
Cardio-Egypt 2015

2. Introduction
Drug therapy
Interventional treatment
Guidelines
Emerging therapies
Conclusions
Agenda

3. Pulmonary arterial hypertension is a disease of the
small pulmonary arteries that is characterized by
vascular proliferation and remodeling. It results in a
progressive increase in pulmonary vascular
resistance and, ultimately, right ventricular failure
and death.
The complex pathogenesis of this disease and the late
diagnosis due to the nonspecific presentation make
its treatment to be a difficult task
However, the recent understanding of the
pathogenesis of PHT leads to the addition of many
new drugs for its treatment
Introduction

4. www.escardio.org/guidelines

6. Basic measures in PAH

8. Group Drugs
Prostenoids PG I2
agonist
Epoprostenol IV
Trepostinil IV/SC/Inh./oral
Ilioprost Inh.
Enodthelin antagonist Bosentan
Ambresentan
Macitentan
NO Pathway PDE5 Sildenafil, Tadalafil
NO Inhalation
Riociguat
Investgational drugs Selexipag
Imatinib and nilotinib
Current therapies of PHT

19. Benefit
Increased efficacy
Decrease side effects by use of smaller doses
Simplicity by shifting from IV to oral therapy
The 3 drug groups had been combined as add on
therapy in many clinical trials with good results
though sometimes conflicting
The role of combinations though stressed on in the
guidelines for severe cases need further more data to
confirm its benefit
Combination therapy

20. Lack of survival benefit
Cost of therapy
Treatment failure
Drug interactions
Toxicity and side effects
Limitations of therapy

21. Atrial septostomy
Pulmonary artery denervation
VAD
Stem cell therapy
Lung transplantation
Lung heart transplantation
Interventional therapy

23. www.escardio.org/guidelines

24. Pulmonary HTN is one of the difficult therapeutic
problem in CV medicine
The recent understanding of the complicated
pathophysiology of the disease has added many new
drugs
The starting basic treatment with CCB, Diuretics is
used in early low risk cases
Shift to the FDA approved drugs ( prostenoid, ERA,
NO pathway active) in severe and high risk patients
Combinations therapy may be used in severe , non-
responsive and progressive cases
Interventional therapy is last therapeutic option
Take home message

25. E. Mahfouz 2008