Subject(s)
Humans , Hermanski-Pudlak Syndrome/genetics , Pulmonary Fibrosis , Hemorrhagic Disorders , Albinism , Transplants , TherapeuticsABSTRACT
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Subject(s)
Humans , Male , Female , Aged , Aged, 80 and over , Pulmonary Fibrosis/etiology , Lung Neoplasms/complications , Risk Factors , Lung Diseases, Interstitial/pathology , Pulmonary Emphysema/complications , Lung Neoplasms/drug therapy , Lung Neoplasms/radiotherapy , Thorax/pathology , Tomography, Emission-ComputedABSTRACT
BACKGROUND: Macitentan is the latest endothelin-receptor antagonist (ERA) approved for the treatment of pulmonary arterial hypertension (PAH), presenting enhanced properties over previous ERAs. OBJECTIVE: We describe the clinical and echocardiographic evolution of patients with PAH who started macitentan after discontinuing bosentan/ambrisentan. METHODS: This was a retrospective series of patients with different etiologies who started macitentan after the suspension of other ERAs under routine clinical practice at five Spanish hospitals. World Health Organization functional class (WHO-FC), 6-min walk distance (6MWD), levels of N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and cardiac imaging data were collected and described at baseline (before macitentan initiation) and after 3, 6, and 12 months, when available. RESULTS: In total, 12 patients (ten women; mean age 65.63 ± 13.27 years) were observed. At baseline, most patients were receiving concomitant PAH medications, and five patients were classed as WHO-FC III. After 3 months of macitentan treatment, WHO-FC had improved in four patients, 6MWD increased in eight patients, and NT-proBNP levels and right atrial area were lowered in seven and eight patients, respectively. Similar results were observed after 6 and 12 months. Macitentan was well-tolerated, with no PAH hospitalizations, septostomies, transplants, or deaths registered. CONCLUSIONS: Our results suggest that switching to macitentan in patients with PAH who discontinued bosentan/ambrisentan was well-tolerated and effective. Further studies are needed to confirm these observations.
Subject(s)
Endothelin Receptor Antagonists , Pulmonary Arterial Hypertension , Pyrimidines , Sulfonamides , Aged , Echocardiography/methods , Endothelin Receptor Antagonists/administration & dosage , Endothelin Receptor Antagonists/adverse effects , Female , Humans , Male , Natriuretic Peptide, Brain/blood , Peptide Fragments/blood , Pulmonary Arterial Hypertension/blood , Pulmonary Arterial Hypertension/diagnosis , Pulmonary Arterial Hypertension/drug therapy , Pulmonary Arterial Hypertension/physiopathology , Pyrimidines/administration & dosage , Pyrimidines/adverse effects , Retrospective Studies , Spain/epidemiology , Sulfonamides/administration & dosage , Sulfonamides/adverse effects , Treatment Outcome , Walk Test/methods , Walk Test/statistics & numerical dataABSTRACT
No disponible
