Publication Date:
2012
abstract:
Pulmonary endarterectomy (PEA) is the treatment of choice for patients with symptomatic CTEPH1. However, a substantial proportion of these patients are considered inoperable due to a distal pulmonary vascular obstruction, or to attendant hypertensive pulmonary arteriopathy1-3.
Patients with Inop-CTEPH treated with conventional therapy (oxygen, anticoagulants, digitalis, diuretics and calcium-channel blockers) have a poor survival particularly those with a pulmonary artery mean pressure>30 mmHg 4,5.
More recently, drugs such as prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have produced functional and even long-term (3 years) survival benefits in patients of Group 1 of the new pulmonary artery hypertension (PAH) classification.
Several off-label studies with these drugs have been performed in Inop-CTEPH patients suggesting hemodynamic, clinical and survival improvement 6-10.
Beneficial effects of these drugs in Inop-CTEPH patients may be further defined through randomized controlled trials
The aim of this study was to present data on the 3-year survival and on the clinical and functional courses of Inop-CTEPH patients treated with conventional therapy versus those treated with conventional and combined novel drug therapy.
Conclusions: Novel vasodilator drugs to treat PAH and oxygen supplementation improve hemodynamic, O2 handling and acid-base balance in patients with inoperable CTEPH, delaying progression of right heart failure and increasing 3-year survival.
1. Hoeper MM, Circulation 113:2011- 2020
2. Dartevelle P, Eur Respir J 23:637-648
3. Galie` N, Proc Am Thorac Soc 3:571-576
4. Bresser P, Proc Am Thorac Soc 3:594-600
5. Lewczuk J, Chest 119:818-823
6. Reichenberger F. Eur Respr J30:922-927
7. Condliffe R., Am J Respir Crit Care Med 177:1122-1127
8. Hughes RJ, Eur Respir J28:138-143
9. Ja?¨s X, J Am Coll Cardio l52:2127-2134
10. Seyfarth H-J, Clin Res Cardiol 99:553-556
Iris type:
04.02 Abstract in Atti di convegno
Keywords:
Radiografia del torace; ipertensione polmonare
List of contributors: