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Completed

NCT Number: NCT04610788

Cardiac Assessment by PV Loop in IPAH and Scleroderma PAH

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.

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Key information

Conditions

Scleroderma Connective Tissue Diseases Familial Primary Pulmonary Hypertension Hypertension, Pulmonary Lung Diseases Pulmonary Artery Hypertension Respiratory Tract Diseases Scleroderma, Diffuse Scleroderma, Systemic Skin Diseases Skin and Connective Tissue Diseases

Age range

18 year–100 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Johns Hopkins

Baltimore, Maryland, 21287, United States

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Patients 18 years or older with clinically diagnosed scleroderma or presumed/known idiopathic pulmonary hypertension.

Exclusion criteria

  • 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

Treatment and study plan

Primary outcomes

  1. Right Ventricular Function as assessed by RHC

    Time frame: Baseline

    Assessed on the clinical RHC as normal, moderately reduced, or severely reduced.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

Secondary outcomes

  1. Number of genes expressed

    Time frame: up to 4 years

    Gene expression as assessed by observing presence of microRNA in the lab.

  2. Number of proteins expressed

    Time frame: up to 4 years

    Protein expression as assessed by observing post-translational modification of candidate proteins in the lab.

Sponsors and collaborators

Lead sponsor

Johns Hopkins University

Other

Collaborators

  • National Heart, Lung, and Blood Institute (NHLBI)

Registry information

Official study title

Understanding Right Ventricular Heart Failure in Scleroderma and Idiopathic Pulmonary Arterial Hypertension

Acronym: CALIPSO

Important dates

Study start
2019
Primary completion
2025
Study completion
2025
First posted
Nov 2, 2020
Registry last updated
Jan 9, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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