Pulmonary arterial hypertension

Czech version

Authors: P. Jansa; D. Ambrož; P. Poláček; J. Marešová; M. Aschermann; A. Linhart
Authors‘ workplace: II. interní klinika kardiologie a angiologie, Centrum pro plicní hypertenzi 1. LF UK a VFN Praha
Published in: Kardiol Rev Int Med 2009, 11(4): 166-169
Category: Sympozium

Overview

Pulmonary arterial hypertension (PAH) is a primary disorder of pulmonary arterioles, either of an unknown aetiology (idiopathic and hereditary PAH) or associated with a known cause (systemic connective tissue disorders, liver disease, congenital cardiac shunts, HIV infection, abuse of some anorectic agents). From the haemodynamic perspective, PAH is characterised as a progressive pre‑capillary pulmonary hypertension leading to relatively rapid right ventricular failure and death. The core examination enabling diagnosis of PAH is Doppler echocardiography. Patients with increased risk of PAH must be regularly monitored with echocardiography. Definitive diagnosis must, however, be made during right heart catheterization. Delayed diagnosis of PAH remains a problem. Non‑specific symptomatology and its late manifestation are the main reasons. The acute vasodilatator test determines pharmacotherapy of PAH. Only the patients with the positive test are indicated for treatment with high doses of calcium channel blockers. This therapy is, together with anticoagulation therapy and heart failure treatment, known as conventional therapy. So‑called specific pharmacotherapy (prostanoids, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors) is, in addition to chronic anticoagulation treatment, indicated in test‑negative patients. Balloon atrial septostomy and lung transplantation are indicated in patients in whom all pharmacotherapeutic options had been attempted.

Keywords:
pulmonary arterial hypertension – prostanoids – endothelin receptor antagonists – phosphodiesterase type 5 inhibitors

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Labels

Paediatric cardiology Internal medicine Cardiac surgery Cardiology

Pulmonary embolism

Congenital heart defects with pulmonary hypertension

Pulmonary Hypertension in Patients with Chronic Obstructive Pulmonary Disease

Pleural effusion – aetiology and diagnostics

Treatment of diabetes with respect to cardiovascular risk

The future of dyslipidemia therapy

Who preaches water and drinks wine – Cardiovascular risk in a medical population in a project

Article was published in

Cardiology Review

2009 Issue 4