The primary objective of this study is to evaluate the safety and tolerability of
treprostinil palmitil inhalation powder (TPIP) compared with placebo
Pulmonary arterial hypertension (PAH) is a type of high blood pressure in the arteries that
carry blood from the heart to the lungs. PAH occurs when the openings in the blood vessels of
the lungs get smaller and smaller. These smaller openings can be caused by the following:
- The walls of the arteries tightening
- The walls of the arteries becoming stiff and narrow from an overgrowth of cells The
increased pressure in the pulmonary arteries strains the right side of the heart and it
begins to fail, causing difficulty breathing and other symptoms. As PAH progresses,
symptoms get worse.
There is no...
Congenital diaphragmatic hernia (CDH) occurs in approximately 1 in 3000 US live births,
similar to the incidence seen within the Utah Birth Defects cohort. The diaphragmatic defect
compromises lung growth and alters pulmonary vascular development. This is reflected
postnatally as respiratory failure, pulmonary hypertension (PH) and overall cardiopulmonary
dysfunction, particularly post-repair. Currently, optimal management of post-repair PH
remains poorly investigated. Sildenafil citrate is a highly selective phosphodiesterase-5
inhibitor that increases cGMP levels, leading to smooth muscle relaxation and an
The purpose of this study is to determine whether a regimen of high-dose immunoablative
therapy will demonstrate safety that is consistent or improved with other published regimens
in SSc patients, while maintaining a treatment effect.
Balloon pulmonary angioplasty (BPA) emerged as a potential therapeutic option for
non-operable patients with chronic thromboembolic pulmonary hypertension (CTEPH).
The aim of this study was to evaluate the safety and efficacy of BPA in patients disqualified
from surgery or suffered from persistent CTEPH despite pulmonary endarterectomy.
This is a interventional study analyzing the benefits and the risk of BPA. Clinical
evaluation, including: functional capacity, 6-minutes walking test, haemodynamics,
biomarkers, cardiopulmonary exercise test, echocardiography, electrocardiography and QoL
assessment with Short Form 36 (SF36)...
Nowadays, biomarkers are broadly used in clinical practice. Blood-derived biomarkers fulfil
an important role in the field of cardiology. However, most biomarkers have been investigated
for adult left ventricular disease. In congenital heart diseases (CHD) and pulmonary arterial
hypertension (PAH), which involves children and mostly the right ventricle, less is known
about the clinical and predictive value of blood-derived biomarkers. Since the group of
survivors of CHD and PAH is growing because of the improved techniques nowadays, development
of better tools to maintain the quality of life for the longer term in these patients is
Pulmonary hypertension (PHT) patients often receive long term oral anticoagulants. If the
indication is strong, in the secondary chronic thrombo-embolism pulmonary hypertension
(CTE-PHT) prevention, the frequent prescription (50 to 90% of patients) contrasts with their
low level of proof in the PHT. Last but not least, anticoagulants are known to be the
principal cause of iatrogenic hospitalization (major bleeding).
In this study, patients are all followed during one year, to determine the annual frequency
of major bleedings (according to the International Society on Thrombosis and Haemostasis
(ISTH) international definition). Each...
To study the effect of acute normobaric hypoxia during exercise in patients with pulmonary
hypertension on blood markers (serum markers of iron and red blood cell homeostasis and
micro-RNAs known to be associated with PH).
This observational study is being done to understand why people with scleroderma can develop
pulmonary arterial hypertension (high blood pressure in the lungs, abbreviated PAH) and a
weak heart muscle (heart failure). The study will also help the investigators understand why
people with PAH from an unknown cause (called idiopathic PAH, or IPAH) can also develop a
weakened heart muscle. The response of the right side of the heart or right ventricle (RV) to
standard PAH therapy in scleroderma-associated PAH and in IPAH will be assessed. Blood and
tissue samples will be collected from research participants during participants' normal
Pulmonary Arterial Hypertension is characterized by a progressive increase in pulmonary
vascular resistance inducing shortness of breath and exercise intolerance. We aim to
correlate cardiac function (evaluated at rest by right heart catheterism and RMN) to exercise
capacity (evaluated by endurance time at 75% of maximal workout), in prevalent patients with
pulmonary arterial hypertension, and their evolution at three and twelve months.