#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Pulmonary arterial hypertension


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


Sources

1. Simonneau G, Robbins IM, Beghetti M et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43–S54.

2. Galie N, Hoeper MM, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. The task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Respir J 2009 Sep 12 [Epub ahead of print].

3. Humbert M, Sitbon O, Chaouat A et al. Pulmonary arterial hypertension in France. Results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023–1030.

4. D’Alonzo GE, Barst RJ, Ayres SM et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med 1991; 115: 343–349.

5. Nichols WC, Koller DL, Slovis B et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31–32. Nat Genet 1997; 15: 277–80.

6. Morse JH, Jones AC, Barst RJ et al. Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31–q32. Circulation 1997; 95: 2603–2606.

7. Trembath RC, Thomson JR, Machado RD et al. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 2001; 345: 325–334.

8. Daniels LB, Krummen DE, Blanchard DG. Echocardiography in pulmonary vascular disease. Cardiol Clin 2004; 22: 383–399.

9. McGoon MD, Garvan CK. Pulmonary hypertension: diagnosis and management. Mayo Clin Proc 2009; 84 (2): 191–207.

10. Sitbon O, Humbert M, Jais X et al. Long‑term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105–3111.

11. Barst RJ, Gibbs JS, Ghofrani HA et al. Updated evidence‑based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54: S78–S84.

12. Keogh AM, Mayer E, Benza RL et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009; 54: S67–S77.

Labels
Paediatric cardiology Internal medicine Cardiac surgery Cardiology
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#