Transcriptome Profile of Endothelial Cells in PAH Patients vs Controls

The transcriptome of pulmonary arterial endothelial cells from healthy lungs and from lungs of patients with idiopathic pulmonary arterial hypertension have been analyzed and specific differences in disease-relevant pathways were identified. The genes identified as altered in these patients have direct effects on pulmonary arterial endothelial cell function. This finding may underlie the inability of the pulmonary vasculature to respond to and repair the damage observed in pulmonary hypertension. ]]>
Control donor lung exclusion criteria: Unused donor lungs were not considered for study if the following criteria were met: Lungs from donors ≥ 65 years old (no lower age limit) Lungs from donors with arterial blood gas results showing a PaO2/FiO2 ratio of <300 mmHg (the donor should be mechanically ventilated using a conventional ventilator mode with positive end-expiratory pressure of ≤ 8 mm Hg.) Lungs from donors who are seropositive for HIV, HCV or HBV (Hepatitis B surface antigen). Lungs from donors who are high risk for HIV, HCV or HBV infections (e.g., known history of intravenous drug abuse and/or other high-risk behavior). Lungs from donors with a history of amphetamine/methamphetamine use Lungs noted to have gross aspiration/infiltrates involving both lungs on chest radiographs (unless the infiltrates are due to volume overload and these infiltrates resolved with diuresis.) Lungs from donors who have received massive transfusion (≥10 units of packed red blood cells, 10 units of fresh frozen plasma or 10 units of platelets. Lungs with unexpected pulmonary problems such as significant emphysema or pulmonary fibrosis noted on radiographic studies or significant pulmonary hypertension (right ventricular dilatation and/or estimated systolic pulmonary arterial pressure ≤45 mmHg) noted on echocardiography Lung from donors who have a smoking history of ≥ 50 pack-years. Hospitalization for >7 days. - If lungs from cardiac death donors, further exclusion criteria apply. Infiltrates on the last chest x-ray (this X-ray must be taken within 24 hrs of donor's death.) Fever >38.2°C (100.7°F) within 24 hrs of donor' demise Lungs harvested >60 min after donor's demise Case inclusion criteria Diagnosis of idiopathic or familial pulmonary arterial hypertension Consent for donation Age 18-65 years ]]>