SclerodermaCALIPSONCT04610788
Cardiac Assessment by PV Loop in IPAH and Scleroderma PAH
An observational study of Scleroderma and Pulmonary Artery Hypertension, run by Johns Hopkins University at 1 site in United States.
Trial 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). Read more about this study
- Who is it for?
- Ages 18 to 100 years
- Conditions the study is for
- Scleroderma, Pulmonary Artery Hypertension
- How many people
- 43 participants
- Where
- 1 siteUnited States
- Study dates
- 15 Apr 2019to 31 Dec 2025
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 to 100 years
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Patients 18 years or older with clinically diagnosed scleroderma or presumed/known idiopathic pulmonary hypertension.
Who may be able to join
- Patients 18 years or older with clinically diagnosed scleroderma or presumed/known idiopathic pulmonary hypertension.
Who cannot join
- Patients found to have secondary pulmonary hypertension (PH due to left heart failure) on clinical RHC.
- Hemodynamically unstable patients (systolic blood pressure < 90mmHg, vasopressor requirement).
- Patients whom are unable to give consent for themselves.
- Patients with RV clot or septal aneurysm will be excluded.
- In order to undergo the clinical right heart catheterization procedures, pregnancy testing (urine or serum) is standard of care.
- Pregnancy
Trial locations and contacts
1 site in 1 country.
About this study
Official title: Understanding Right Ventricular Heart Failure in Scleroderma and Idiopathic Pulmonary Arterial Hypertension
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.
Read the 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 presumed/known 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 (P/V) Loop Assessment and RV biopsy for research purposes. The investigators will also do a standard pathologic assessment of the RV tissue (H\&E, special staining, electron microscopy), microvascular density measurements using immunohistochemistry techniques and isolated skinned myocyte experiments. Additional experiments will include proteomics, genomics/genetics, 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.
- Conditions
- Scleroderma, Pulmonary Artery Hypertension
- Medical subject headings
- Scleroderma, Diffuse, Familial Primary Pulmonary Hypertension
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| SSc-PAH Group | Scleroderma patients referred for a clinically indicated right heart catheterization (RHC). | |
| IPAH Group | Presumed/known IPAH patients referred for a clinically indicated right heart catheterization (RHC). |
What is being measured
The primary outcome is the main question the study is designed to answer.
Right Ventricular Function as assessed by RHC
Assessed on the clinical RHC as normal, moderately reduced, or severely reduced.
Measured: Baseline
Change in pulmonary vascular resistance
Assessed as improved or decreased after 6 months by comparing the change in pulmonary vascular resistance in Wood units on the clinical RHC.
Measured: Baseline and 6months
Change in arterial elastance
Assessed as improved or decreased after 6 months by comparing the change in arterial elastance in pressure volume (PV) loops.
Measured: Baseline and 6 months
Change in myofilament contractility
Assessed as Normal or Abnormal after studying the collected samples in lab. Abnormal can be either reduced or increased; i.e. hyper- or hypo-contractile.
Measured: up to 4 years
Change in calcium sensitivity
Assessed as either increased- or decreased- sensitivity after 6 months, by studying the collected samples in lab.
Measured: up to 4 years
Secondary outcomes (2)
Number of genes expressed
Gene expression as assessed by observing presence of microRNA in the lab.
Measured: up to 4 years
Number of proteins expressed
Protein expression as assessed by observing post-translational modification of candidate proteins in the lab.
Measured: up to 4 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted2 Nov 2020
- Study start15 Apr 2019
- Primary completion31 Dec 2025
- Study completion31 Dec 2025
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for SclerodermaStudies of the same condition, open and under way.
- More trials run by Johns Hopkins UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 43 participants (actual)
Sponsor and investigators
- Lead sponsor
- Johns Hopkins University
- Sponsor type
- University, hospital or other
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI)
- Responsible party
- Sponsor
- Investigators
- Paul Hassoun, MD, Principal investigator, Johns Hopkins University
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04610788
- Sponsor's protocol ID
- NA_00049022
- Other IDs
- R01HL114910-06
- Acronym
- CALIPSO
- Status last verified
- Jan 2026
- First submitted
- 26 Oct 2020
- Last updated
- 9 Jan 2026
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 9 Jan 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Understanding Right Ventricular Heart Failure in Scleroderma and Idiopathic Pulmonary Arterial Hypertension
- ClinicalTrials.gov ID
- NCT04610788
- Acronym / sponsor ID
- CALIPSO / NA_00049022
- Conditions
- Scleroderma, Pulmonary Artery Hypertension
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 43 participants (actual)
- Lead sponsor
- Johns Hopkins UniversityUniversity, hospital or other
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI)
- Study start
- 15 Apr 2019 (actual)
- Primary completion
- 31 Dec 2025 (actual)
- Study completion
- 31 Dec 2025 (actual)
- First posted
- 2 Nov 2020 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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 presumed/known 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 (P/V) Loop Assessment and RV biopsy for research purposes. The investigators will also do a standard pathologic assessment of the RV tissue (H\&E, special staining, electron microscopy), microvascular density measurements using immunohistochemistry techniques and isolated skinned myocyte experiments. Additional experiments will include proteomics, genomics/genetics, 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 design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| SSc-PAH Group | – | –Scleroderma patients referred for a clinically indicated right heart catheterization (RHC). |
| IPAH Group | – | –Presumed/known IPAH patients referred for a clinically indicated right heart catheterization (RHC). |
Who may qualify
- Ages
- 18 to 100 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Patients 18 years or older with clinically diagnosed scleroderma or presumed/known idiopathic pulmonary hypertension.
Key inclusion criteria (1 listed)
- Patients 18 years or older with clinically diagnosed scleroderma or presumed/known idiopathic pulmonary hypertension.
Key exclusion criteria (6 listed)
- Patients found to have secondary pulmonary hypertension (PH due to left heart failure) on clinical RHC.
- Hemodynamically unstable patients (systolic blood pressure < 90mmHg, vasopressor requirement).
- Patients whom are unable to give consent for themselves.
- Patients with RV clot or septal aneurysm will be excluded.
- In order to undergo the clinical right heart catheterization procedures, pregnancy testing (urine or serum) is standard of care.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Right Ventricular Function as assessed by RHC
Time frame: Baseline
Assessed on the clinical RHC as normal, moderately reduced, or severely reduced.
Change in pulmonary vascular resistance
Time frame: Baseline and 6months
Assessed as improved or decreased after 6 months by comparing the change in pulmonary vascular resistance in Wood units on the clinical RHC.
Change in arterial elastance
Time frame: Baseline and 6 months
Assessed as improved or decreased after 6 months by comparing the change in arterial elastance in pressure volume (PV) loops.
Change in myofilament contractility
Time frame: up to 4 years
Assessed as Normal or Abnormal after studying the collected samples in lab. Abnormal can be either reduced or increased; i.e. hyper- or hypo-contractile.
Change in calcium sensitivity
Time frame: up to 4 years
Assessed as either increased- or decreased- sensitivity after 6 months, by studying the collected samples in lab.
2 secondary outcomes
Number of genes expressed
Time frame: up to 4 years
Number of proteins expressed
Time frame: up to 4 years
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 9 Jan 2026.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Paul Hassoun, MD principal investigator
Johns Hopkins University
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Scleroderma and Pulmonary Artery Hypertension, run by Johns Hopkins University at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 1 inclusion and 6 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 9 Jan 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Right Ventricular Function as assessed by RHC (time frame: Baseline). The study lists 5 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 9 Jan 2026.