Pediatric Pulmonary Hypertension Network (PPHNet) Informatics Registry
Last updated on July 2021Recruitment
- Recruitment Status
- Recruiting
- Estimated Enrollment
- Same as current
Summary
- Conditions
- Pulmonary Arterial Hypertension
- Pulmonary Vascular Disease
- Design
- Observational Model: CohortTime Perspective: Prospective
Participation Requirements
- Age
- Younger than 21 years
- Gender
- Both males and females
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...
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 To provide a mechanism to store information about newborns, infants and children with PH; To determine the incidence and natural history of the various etiologies of pediatric PH; To define the investigator current diagnostic and therapeutic approaches to the diverse conditions associated with pediatric PH; 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.
Tracking Information
- NCT #
- NCT02249923
- Collaborators
- National Heart, Lung, and Blood Institute (NHLBI)
- Investigators
- Principal Investigator: Steven H Abman, MD Children's Hospital Colorado Principal Investigator: David D Ivy, MD Children's Hospital Colorado Principal Investigator: Kenneth D Mandl, MD Boston Children's Hospital, Harvard School of Medicine Principal Investigator: Jeffrey Fineman, MD University California San Francisco Principal Investigator: Jeffrey Feinstein, MD Stanford University Principal Investigator: Ian Adatia, MD University of Alberta Edmonton Principal Investigator: Catherine Avitabile, MD Children's Hospital of Philadelphia Principal Investigator: Mary Mullen, MD Boston Children's Hospital Principal Investigator: Eric Austin, MD Vanderbilt University Medical Center Principal Investigator: Marc Natter, MD Boston Children's Hospital Principal Investigator: Erika Rosenzweig, MD Columbia University
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