Pulmonary Hypertension Research

• The Patient Registry for Primary Pulmonary Hypertension (PPH Registry) was the first registry of patients diagnosed with a specific type of pulmonary hypertension. Patients were enrolled from 32 medical centers across the United States and were followed from 1995 to 1999. Before the NHLBI PPH Registry was established, little was known about the disease. Now researchers know more about the causes, natural history, and outcomes of patients diagnosed with pulmonary hypertension.
• The NHLBI supported the creation of the Centers for Advanced Diagnostics and Experimental Therapeutics in Lung Diseases (CADET) program. The CADET program includes research projects that developed new medicines to treat lung diseases, including pulmonary hypertension.

RESEARCH FEATURE

Learn how antibodies could help reverse harmful changes caused by pulmonary hypertension: NHLBI Supports CADET Researchers to Produce New Pulmonary Disease Drugs (Part 3)

Our Division of Lung Diseases and its Lung Biology and Disease Branch oversee much of the research on pulmonary hypertension that we fund.

Current research on treatment of pulmonary hypertension

Research funded by the NHLBI is working to discover new drugs to treat pulmonary hypertension.

• Enzymes called proteases are activated and contribute to inflammation and vascular damage in people with pulmonary hypertension. Researchers are studying a drug called elafin that blocks these enzymes in the body. The Food and Drug Administration (FDA) has granted orphan drug status to elafin for use in patients with pulmonary hypertension.
• Despite the condition’s high morbidity and mortality rates, very few studies investigate pulmonary hypertension in children. To address this, the NHLBI funds researchers who collaborate with the Pediatric Pulmonary Hypertension Network (PPHNet), an association of medical professionals and centers focused on all aspects of pediatric pulmonary hypertension. As a part of this collaboration, scientists are conducting research to develop an effective treatments for pulmonary hypertension in children.

Find more NHLBI-funded studies on pulmonary hypertension treatment at NIH RePORTER.

Current research on pulmonary hypertension in women

Pulmonary hypertension affects women more than it affects men. In fact, being female is the strongest risk factor for pulmonary hypertension that is genetic (runs in families) and idiopathic (has no known cause).

• Researchers are currently investigating certain types of estrogen metabolites and the role they play in increasing the risk of pulmonary hypertension.
• A clinical trial for pulmonary hypertension is currently underway to test the safety and effectiveness of anastrozole, an FDA-approved drug currently used to treat breast cancer.

Find more NHLBI-funded studies on pulmonary hypertension in women at the NIH RePORTER.

Current research on the causes of pulmonary hypertension

Improving our understanding of the causes of pulmonary hypertension will help scientists develop tailored treatments for patients.

• Vascular remodeling causes the blood vessels in the lungs to narrow. This leads to increased pressure in the blood vessels of the lungs, which is a hallmark sign of pulmonary hypertension. The NHLBI funds research that explores different causes of vascular remodeling, including genetic mutations and the roles of ion channels and cell membrane receptors.
• Other NHLBI-funded research is actively investigating the hormonal and metabolic causes of vascular dysfunction in pulmonary hypertension.

Find more NHLBI-funded studies on the causes of pulmonary hypertension at the NIH RePORTER.

Our Division of Intramural Research is actively engaged in the study of pulmonary hypertension.

Read more about these projects and ongoing clinical trials.

• The NHLBI established the Pulmonary Trials Cooperative (PTC) to facilitate research into chronic lung disease. The PTC brings together patients, researchers, and health care professionals from more than 50 institutions, with a common goal of developing new treatments and testing current treatments for lung diseases, including pulmonary hypertension.
• The Vascular Interventions/Innovations and Therapeutic Advances (VITA) program is a translational program that supports and accelerates early-stage development of promising diagnostics and treatments. VITA seeks to address unmet clinical needs for vascular diseases, particularly in underserved medical communities.
• The NHLBI sponsors the Trans-Omics for Precision Medicine (TOPMed) program, which aims to provide treatments tailored to an individual’s unique genes and environment.
• Precision medicine may help address individual differences in patients with pulmonary hypertension. The Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics (PVDOMICS) program aims to better understand the causes and risk factors of pulmonary hypertension to increase personalized approaches for intervention and prevention.
• The PAH Biobank and Pulmonary Hypertension Breakthrough Initiative (PHBI) are two NHLBI-funded resources of biological samples, genetic data, and clinical data for researchers to use to study pulmonary hypertension.
• The NHLBI hosted a Blood Diagnostic Working Group, which explored new technologies to diagnose and predict diseases and complications, such as pulmonary hypertension in sickle cell disease.