Assessing the safety and efficacy of Macitentan in patients with portopulmonary hypertension
Several drugs (blood vessel dilators), including macitentan, are currently approved for the treatment of pulmonary arterial hypertension (PAH) in many parts of the world. These drugs have significantly improved the outcome of the condition. However, none of these treatments have been evaluated in portopulmonary hypertension, a form of PAH caused by liver disease. The purpose of this study is to assess the safety and effectiveness of macitentan in portopulmonary hypertension by looking at several clinical indicators, including the pressures within the arteries of the lungs and a person’s capacity for exercise.
Recruiting | High Blood Pressure / Hypertension | Not Multisite
Pulmonary Arterial Hypertension (PAH) Quality Enhancement Research Initiative (QuERI) Extension Program
This is a prospective, U.S.-based, multi-center, knowledge translation program tracking patient management until approximately early 2018. To improve the management of PAH patients through an evidence-based approach aimed at achieving optimal WHO functional class (FC). The goal of the optimal functional class is to improve WHO functional class III and IV patients to functional class I or II, and to improve all functional class II patients to functional class I, or at least to maintain functional class I/II in patients presenting in that functional class. Endpoints:1. Proportion of patients achieving guideline-recommended treatment at each documented visit.2. Proportion of patients achieving optimal functional class using an evidence-based reatment algorithm.3. Proportion of patients on various therapies in relation to their WHO functional class.4. PAH management (use of therapies) by physicians across patient risk strata based on FC.5. Association between treatment and physician and patient adherence with recommended treatment.6. Utilization of Knowledge Translation Program system reminders, and response by the physicians when asked for reasons evidence based therapy was not utilized.7. Survival by FC (baseline and change), etiology, prevalent vs incident, academic vs community, adherence and reminders.Descriptive analysis of demographic variables, physical exam, medical history and treatment profile will be performed as appropriate. Confidence Intervals (95%) will be calculated for descriptive and comparative purposes.
Recruiting | High Blood Pressure / Hypertension | Multisite
Registry of Sarcoidosis Associated Pulmonary Hypertension (RESAPH)
This is a multi center registry of sarcoidosis associated pulmonary hypertension (SAPH). With
this registry, the investigators will characterize the demographics, clinical course,
hemodynamics, pulmonary physiology, and disease management of sarcoidosis associated
pulmonary hypertension on the United States. The investigators will also compare these
features to non-US sites.
Unknown status | High Blood Pressure / Hypertension | Not Multisite