Ignite Creation Date:
2024-05-06 @ 3:22 PM
Last Modification Date:
2024-10-26 @ 1:48 PM
Study NCT ID:
NCT04610788
Status:
RECRUITING
Last Update Posted:
2024-01-19
First Post:
2020-10-26
Brief Title:
Cardiac Assessment by PV Loop in IPAH and Scleroderma PAH
Sponsor:
Johns Hopkins University
Organization:
Johns Hopkins University
Study Overview
Official Title:
Understanding Right Ventricular Heart Failure in Scleroderma and Idiopathic Pulmonary Arterial Hypertension
Status:
RECRUITING
Status Verified Date:
2024-01
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
CALIPSO
Brief Summary:
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 standard of care procedures People with scleroderma-associated PAH or idiopathic cause IPAH who need a right heart catheterization may join this study
Detailed Description:
Patients with scleroderma associated pulmonary hypertension with or without interstitial lung disease have a worse prognosis compared to patients with idiopathic pulmonary arterial hypertension IPAH The investigators have discovered through a previous protocol that patients with scleroderma associated pulmonary hypertension SSc-PAH have intrinsic right ventricular RV contractile dysfunction compared with patients with idiopathic pulmonary hypertension IPAH despite similar afterload imposed by the pulmonary vasculature Patients with scleroderma or presumedknown IPAH who are clinically referred for right heart catheterization RHC will undergo in addition to a clinically indicated RHC state-of-the-art Pressure-Volume PV Loop Assessment and RV biopsy for research purposes The investigators will also do a standard pathologic assessment of the RV tissue HE special staining electron microscopy microvascular density measurements using immunohistochemistry techniques and isolated skinned myocyte experiments Additional experiments will include proteomics genomicsgenetics and RV protein and microRNA expression The investigators will compare these findings in both groups IPAH and SSc-PAH before and after standard treatment for 6 months in order to fully understand the differences in how the RV adapts to pressure overload and reasons for impaired RV function in SSc-PAH as well as identifying potential therapeutic targets
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None
Secondary IDs
| Secondary ID | Type | Domain | Link |
|---|---|---|---|
| R01HL114910-06 | NIH | None | httpsreporternihgovquickSearchR01HL114910-06 |