About this trial
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.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
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
Disqualifiers
Diagnosed with pulmonary hypertension after age 18
Refusal to sign informed consent
Trial population
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, HIV, and use of anorexigens.
Trial design
Cohort
Prospective
Treatments tested in this trial
Not listed
Trial groups
Trial outcomes
Primary outcomes
Time to clinical worsening
Time to clinical worsening for death, cardiac transplant, atrial septostomy, or Potts shunt.
Secondary outcomes
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
Right Heart Failure
Elevated Right atrial pressure greater than 10 by right heart catheterization
Sponsors and contacts
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New York Medical College
Lead sponsor
National Heart, Lung, and Blood Institute (NHLBI)
Collaborator
Janssen Pharmaceuticals
Collaborator