Pediatric Pulmonary Hypertension Network (PPHNet) Informatics Registry

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

Official Title
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Pediatric Pulmonary Hypertension Network (PPHNet) Informatics Registry

Conditions
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Pulmonary Vascular Disease, Pulmonary Arterial Hypertension

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 1 Day
Maximum Age: 21 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Pulmonary Arterial Hypertension<br> | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Time to clinical worsening | Death | 36 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Escalation of Pulmonary Hypertension Therapy | The addition of patients baseline medication therapy, this can include going from mono therapy to dual therapy, or dual therapy to triple therapy | 36 months | 
| Right Heart Failure | Elevated Right atrial pressure greater than 10 by right heart catheterization | 36 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary Arterial Hypertension (PAH), Pulmonary Vascular Disease (PVD), Registry, Pediatric