Diagnosis: Plexiform arteriopathy
• Plexiform lesions are the result of vascular wall injury. Lesions of primary pulmonary arterial hypertension reflect the dilatation and organization associated with this injury.
• These lesions can be relatively focal in lung biopsies despite the marked overall effect on pulmonary pressure.
• Familial cases are the result of an autosomal dominant mutation in BMPR2 (bone morphogenic protein receptor 2). The mutation does not result in pulmonary arterial hypertension in all individuals who harbor it.
• Severe pulmonary hypertension is associated with right-sided heart dilatation and failure. Large pulmonary arteries show atherosclerosis in patients with pulmonary hypertension.
• Effective treatments for pulmonary hypertension have significantly improved management of this condition and have resulted in improvements in survival.
• Primary pulmonary arterial hypertension is far more common in women than in men.
