title: [Pulmonary arterial hypertension. Part II: Medical and surgical treatment].
abstract: Treatment of pulmonary arterial hypertension (group 1 of clinical classification) has been recently characterized by important progresses, particularly in pharmacological therapy. Only until few years ago, patients with pulmonary arterial hypertension were treated with non-specific drugs, such as diuretics and digoxin for right heart failure and calcium-channel blockers in the minority of cases, responders to the acute vasoreactivity test. In addition, use of oral anticoagulant treatment was supported by uncontrolled studies. In the last 15 years (in particular in the last 8 years) different randomized controlled trials assessing the functional, clinical and hemodynamic efficacy of three classes of targeted drugs (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) with pulmonary vascular dilating and antiproliferative effects have been performed. This information has allowed the proposal of an evidence-based treatment algorithm. Treatment starts with general measures (physical activity, fertility control, respiratory tract infection, etc.) and supportive therapy (anticoagulant therapy, diuretics, oxygen, digoxin). Patients who respond to the acute vasoreactivity test (10% of idiopathic form) are treated with high doses of calcium-channel blockers, non-responders with targeted therapies either on monotherapy or combination. Usually an oral active drug is initiated and a second compound of a different class is combined in case of non-satisfactory response to the first treatment. Combination therapy should be performed only in specialized centers with large experience on use of targeted therapies and their relevant side effects. In case of failure of medical therapy, possible options are balloon atrial septostomy and/or listing for lung or heart-lung transplantation. As available treatments do not constitute a cure for pulmonary arterial hypertension, further progresses are expected in the near future.
authors: Centro di Diagnosi e Terapia dell'Ipertensione Polmonare, Istituto di Cardiologia, Azienda Ospedaliero-Universitaria, Bologna.
authors: Alessandra
authors: A
authors: Manes
authors: Alessandro
authors: A
authors: Marinelli
authors: Massimiliano
authors: M
authors: Palazzini
authors: Luca
authors: L
authors: Negro
authors: Enri
authors: E
authors: Leci
authors: Simona
authors: S
authors: Gambetti
authors: Cristina
authors: C
authors: Bachetti
authors: Elena
authors: E
authors: Beciani
authors: Elisa
authors: E
authors: Conficoni
authors: Angelo
authors: A
authors: Branzi
authors: Nazzareno
authors: N
authors: Galiè
mesh_terms: D000465:Algorithms; D000800:Angioplasty, Balloon / Q000379:methods; D000925:Anticoagulants / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002316:Cardiotonic Agents / Q000627:therapeutic use; D004077:Digoxin / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists; D019317:Evidence-Based Medicine; D016041:Heart-Lung Transplantation / Q000379:methods; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*; D010100:Oxygen / Q000627:therapeutic use; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011453:Prostaglandins / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D002121:Calcium Channel Blockers; D002316:Cardiotonic Agents; D004232:Diuretics; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins; D004077:Digoxin; D010100:Oxygen