By

Essam Mahfouz, MDProfessor of cardiology, Mansoura University

Cardio-Egypt 2015

Introduction

Drug therapy

Interventional treatment

Guidelines

Emerging therapies

Conclusions

Agenda

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

www.escardio.org/guidelines

Basic measures in PAH

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

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

Lack of survival benefit

Cost of therapy

Treatment failure

Drug interactions

Toxicity and side effects

Limitations of therapy

Atrial septostomy

Pulmonary artery denervation

VAD

Stem cell therapy

Lung transplantation

Lung heart transplantation

Interventional therapy

www.escardio.org/guidelines

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

E. Mahfouz 2008