Active clinical trials for "Familial Primary Pulmonary Hypertension"

In this study, hyperpolarized 129Xe MRI will be used to evaluate treatment efficacy in patients with pulmonary arterial hypertension (PAH). Participants will be imaged at 4 timepoints (baseline, 6 weeks post-therapy initiation, 12 weeks, and 18 weeks). Images will be analyzed to develop new biomarkers and to understand treatment effects.

Group A will be training group and group B will be control group. Deep breathing exercises will be done as baseline treatment in both groups. Both groups will be assessed with Modified Borg scale, 6-PBRT and Fatigue severity scale at the baseline. The control group patients will perform functional active alternating movements for the upper limbs at home involving three sets with 10 repetitions and a rest interval between 1- and 2-minute. Intervention will be for 3 times a week or 6 weeks. The treatment group patients will perform upper extremity aerobic exercises by using an arm ergometer under the supervision of a physiotherapist. Training intensity will adjust according to 50 80 % of max HR or intensity of dyspnea to 4 points on modified Borg scale (MBS) for at least 15 45 min, 3 times/week over 6 weeks.

The purpose of the DelIVery for PAH clinical study is to evaluate the safety of the Medtronic Model 10642 Implantable Intravascular Catheter when used with the Medtronic SynchroMed® II Implantable Infusion System to deliver Remodulin® (treprostinil) Injection. As of June 2021, PMA approval of the Implantable System for Remodulin (ISR) is no longer being pursued and development and commercialization efforts have been halted. The approximately 30 subjects still implanted with the PIVoT system require a pathway for continued support. This protocol is amended and is designed to allow such ongoing support.

The purpose of this study is to test new technology and health coaching aimed to help people with PAH become more physically active in their daily lives.

Establish a pulmonary hypertension registry and biorepository to lead towards a further understanding of the disease.

Prospective registry including incident patients and prevalent patients which pretends to identify clinical characteristics, treatment trends in-hospital and ten years follow-up outcome through major adverse cardiovascular events (MACE) in a China population with well characterized PAH.

This is an observational, ambrispective study of patients in real world clinical practice, who has pulmonary arterial hypertension in whom a prostacyclin IP receptor agonist (Selexipag) is initiated between 2017 and 2021. The aim of this study answer the following questions: Changes in the mortality risk profile of these patients after treatment administration. Baseline characteristics of patients initiating Selexipag. Parameters used for risk stratification prior to treatment escalation. Events during follow-up. No comparison group available

The RECONNECTIVE Registry is an observational single center study, focused on the subgroup of precapillary pulmonary hypertension related to connective tissue diseases. All patients will have hemodynamic confirmation by right heart catheterization and will be follow-up for at least 5 years from admission. All patients diagnosed with Group I Pulmonary Arterial Hypertension (PAH) associated with Connective Tissue Diseases (CTD) and Group IV Pulmonary Hypertension (PH) with CTD will be included. The purpose of the registry is to learn and understand the clinical outcomes and natural history of the pulmonary arterial hypertension in this subgroup of patients to improve the medical care and treatment.

To observe and assess drug administration activities, time spent on drug administration activities, and patient-reported outcomes (PROs) including quality of life, treatment satisfaction, and patient perception of devices related to use of United Therapeutics Corporation (UTC)-developed next-generation infusion pumps to administer Remodulin in patients with Pulmonary Arterial Hypertension (PAH).

This is a long-term open-label safety extension to the Phase 2a study of inhaled QCC374 in adult patients with PAH. This study provides the patients who completed the QCC374X2201 study with the option to continue receiving QCC374. The study will monitor the long-term safety, tolerability and efficacy of QCC374 in patients with PAH.