Defining a Noninvasive Signature for Pulmonary Vascular Remodeling in Group 3 PH
The main goal of this study is to develop a noninvasive signature for pulmonary vascular remodeling in Group 3 PH patients, using hyperpolarized 129Xe magnetic resonance imaging (129Xe MRI). Such a signature may identify Group 3 PH responders to PAH-specific therapies. PAH's unique 129Xe MRI signature has been shown in previous studies. Past studies have lacked a pathologic ground truth correlate of these signatures, which could be provided by comparing them with the pathology of lung explant tissue from patients who have undergone a lung transplant. This signature could be validated in a cohort of patients with Group 3 PH in future studies.
• Age ≥ 18 years' old
• Be on the lung transplant waiting list at Duke University Medical Center.
• PH as defined by RHC - mPAP \> 20 mmHg, PVR \> 3 WU, PCWP \< 15 mmHg
• Groups defined as:
• PAH: Clinical diagnosis of PAH (Group 1 PH) in the absence of severe chronic lung disease, left heart disease, chronic thromboembolism, sarcoidosis, sickle cell disease, or other causes of non-Group 1 PH.
• COPD-noPH: Clinical diagnosis of COPD in the absence of precapillary PH.
• COPD-PH: Clinical diagnosis of COPD with precapillary PH.
• IPF-noPH: Clinical diagnosis of IPF in the absence of precapillary PH.
• IPF-PH: Clinical diagnosis of IPF with precapillary PH.
• Willing and able to give informed consent and adhere to visit/protocol schedules (consent must be given before any study procedures are performed).