Pediatric Pulmonary Hypertension Network (PPHNet) Informatics Registry

Purpose

Patients are being asked to be in this research study because medical researchers hope that by gathering information about a large number of children with pulmonary hypertension over time, their understanding of the disease process will increase and lead to better treatment. Investigators believe that pulmonary hypertension in children is different than pulmonary hypertension in adults and this study will help us understand those differences.

Conditions

  • Pulmonary Vascular Disease
  • Pulmonary Arterial Hypertension

Eligibility

Eligible Ages
Between 1 Day and 21 Years
Eligible Genders
All
Accepts Healthy Volunteers
No

Inclusion Criteria

  • The subject's age of onset of pulmonary hypertension must be prior to age 18 years - The person providing consent must be able to read either Spanish or English. - The subject (and/or parent/legal guardian) must be able to provide informed consent

Exclusion Criteria

  • Diagnosed with pulmonary hypertension after age 18 - Refusal to sign informed consent

Study Design

Phase
Study Type
Observational [Patient Registry]
Observational Model
Cohort
Time Perspective
Prospective

Arm Groups

ArmDescriptionAssigned Intervention
Pulmonary Arterial Hypertension

Recruiting Locations

Vanderbilt University Medical Center
Nashville, Tennessee
Contact:
Eric Austin, MD
eric.austin@Vanderbilt.edu

More Details

Status
Recruiting
Sponsor
University of Colorado, Denver

Study Contact

Steven Abman, MD
303-881-9765
steven.abman@uanschutz.org

Detailed Description

Pulmonary Hypertension (PH) is a syndrome characterized by vasoconstriction and abnormal growth and function of endothelial and smooth muscle cells and other components within the pulmonary vessels, which leads to elevation of the pulmonary artery pressure. PH may be idiopathic (primary) without any known cause. Some cases of PH are familial. PH may also be secondary to a specific disease process such as portal hypertension, congenital heart disease, chronic lung disease, thromboembolic disease, connective tissue disease, human immunodeficiency virus (HIV), and use of anorexigens. Left untreated, PH is often progressive and fatal. There is no cure for PH. Therapy focuses upon treatment of secondary causes if present, and reduction of the pulmonary artery pressure through medical therapy. There have been many new developments within the past few years in the management of patients with PH. While there is no cure for PH early detection and treatment are important for survival of patients. Limited data is available that describes the etiologies, clinical course and prognosis of pediatric pulmonary hypertension. Objectives Aim 1: Clinical Research 1. To provide a mechanism to store information about newborns, infants and children with PH; 2. To determine the incidence and natural history of the various etiologies of pediatric PH; 3. To define the investigator current diagnostic and therapeutic approaches to the diverse conditions associated with pediatric PH; 4. To determine the response of children with PH to chronic therapies. Aim 2: Research Infrastructure To create a robust scalable data architecture, to combine traditional registry data, electronic Health Record (EHR), and PRO (Patient Reported Outcome) data in a single resource. Aim 3: Informatics Address three classes of unanswered questions crucial for the characterization and management of PH, comparing the information value of registry vs. EHR vs. fused data across registry/EHR/PROs, in the domains of spectrum of PH comorbidities, PH indicators and endpoints of morbidity and mortality, and response to therapies in PH.