pulmonary venoocclusive disease 1

A pulmonary venoocclusive disease that has_material_basis_in heterozygous mutation in the BMPR2 gene on chromosome 2q33.

Signs and symptoms

• Centrilobular ground-glass opacification on pulmonary HRCT
• Abnormally loud pulmonic component of the second heart sound
• Interlobular septal thickening
• Pulmonary venous occlusion
• Pulmonary arterial hypertension
• Elevated jugular venous pressure