A Pragmatic Score to Unmask Pulmonary Veno-Occlusive Disease and Pulmonary Capillary Hemangiomatosis Among Group 1 Pulmonary Arterial Hypertension

Key Result

A pragmatic score was designed to unmask pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis among patients with Group 1 pulmonary arterial hypertension.

Key Points

• This research aims to develop a pragmatic score to differentiate between pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in patients with pulmonary arterial hypertension.
• Developed a scoring system based on clinical and diagnostic criteria.
• Applied the score in a cohort of patients diagnosed with group 1 pulmonary arterial hypertension.
• Analyzed the score's effectiveness in identifying the two conditions.
• The score accurately identified pulmonary veno-occlusive disease with a sensitivity of 85% and specificity of 90%.
• It distinguished pulmonary capillary hemangiomatosis with an area under the curve (AUC) of 0.92, p<0.001.