Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension

• Published in: European heart journal 2010-08, Vol.31 (15), p.1898-1907
• Main Authors: Montani, David, Savale, Laurent, Natali, Delphine, Jaïs, Xavier, Herve, Philippe, Garcia, Gilles, Humbert, Marc, Simonneau, Gérald, Sitbon, Olivier
• Format: Article
• Language: English
• Subjects: Acute vasodilator testing; Adult; Aged; Antihypertensive Agents - therapeutic use; Appetite Depressants - adverse effects; Biological and medical sciences; Calcium Channel Blockers - pharmacology; Calcium-channel blockers; Cardiology. Vascular system; Female; Haemodynamics; Human immunodeficiency virus; Humans; Hypertension, Pulmonary - chemically induced; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - physiopathology; Long-Term Care; Male; Medical sciences; Middle Aged; Nitric oxide; Pneumology; Pulmonary arterial hypertension; Pulmonary Artery - physiopathology; Pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Vascular Resistance - drug effects; Vasodilation - drug effects; Vasodilator Agents - pharmacology; Young Adult
• ISSN: 0195-668X; 1522-9645
• DOI: 10.1093/eurheartj/ehq170

Aims To assess the acute vasodilator response and long-term response to calcium-channel blockers (CCB) in pulmonary arterial hypertension (PAH) with associated conditions. Methods and results The response to acute vasodilator testing [>20% decrease in mean pulmonary artery pressure (mPAP) and total pulmonary resistance] was assessed in 663 consecutive PAH patients with connective tissue disease (CTD; n = 168), portal hypertension (PoPH; n = 153), anorexigen use (n = 127), human immunodeficiency virus infection (HIV; n = 124), congenital heart disease (CHD; n = 50), and pulmonary veno-occlusive disease or capillary haemangiomatosis (PVOD/PCH; n = 41). An acute vasodilator response was observed in 13.4% of PAH-anorexigen patients, 12.2% of PVOD/PCH, 10.1% of CTD, 1.6% of HIV, 1.3% of PoPH, and was absent in CHD. A long-term response to CCB (marked haemodynamic improvement at 3–4 months and New York Heart Association functional class I or II after 1 year) was reported in 9.4% of PAH-anorexigen patients but was rare in HIV, PoPH, CTD (1.6, 0.7, and 0.6%, respectively) and absent in PVOD/PCH. All patients with a long-term CCB response were alive after 5 years; two deaths not related to PAH occurred after this time. Recent criteria for acute response based on the fall in mPAP to