Cardiac Assessment by PV Loop in IPAH and Scleroderma PAH

Description

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.

Conditions

Scleroderma, Pulmonary Artery Hypertension

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Study Overview

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Study Details

Study overview

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.

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

Cardiac Assessment by PV Loop in IPAH and Scleroderma PAH

Condition
Scleroderma
Intervention / Treatment

-

Contacts and Locations

Baltimore

Johns Hopkins, Baltimore, Maryland, United States, 21287

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

For general information about clinical research, read Learn About Studies.

Eligibility Criteria

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

Ages Eligible for Study

18 Years to 100 Years

Sexes Eligible for Study

ALL

Accepts Healthy Volunteers

No

Collaborators and Investigators

Johns Hopkins University,

Paul Hassoun, MD, PRINCIPAL_INVESTIGATOR, Johns Hopkins University

Study Record Dates

2024-12-31