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Calcific Atherosclerosis of the Pulmonary Artery in Long-Standing PAH

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Ravinder Kumar

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The pathology of pulmonary arterial hypertension (PAH) is characterized by intimal hyperplasia, medial hypertrophy, adventitial proliferation, thrombosis- in-situ, varying degrees of inflammation and plexiform arteriopathy.

Background: The pathology of pulmonary arterial hypertension (PAH) is characterized by intimal hyperplasia, medial hypertrophy, adventitial proliferation, thrombosis- in-situ, varying degrees of inflammation and plexiform arteriopathy. Calcified atherosclerosis, as seen in the systemic circulation, has not been described as a prominent feature. We present a case of calcific atherosclerosis of the pulmonary artery in a patient with long-standing pulmonary arterial hypertension. 

Case Report: A 46 year old female with 30 year history of PAH associated with connective tissue disease (systemic lupus erythematosus) on treatment with parenteral prostacyclin (epoprostenol) for 15 years in addition to oral sildenafil and bosentan. She had worsening symptoms in spite of maximal medical therapy and underwent lung transplantation.  

Results: During transplantation a large calcified plaque was noted in the left pulmonary artery of the explanted lung. Microscopic examination confirmed the presence of a calcified atherosclerotic plague (figure A). Typical features of long-standing pulmonary hypertension, including muscular hypertrophy, intimal proliferation, concentric laminar intimal fibrosis and plexiform lesions (figure B), were also noted. A contrast enhanced computerized tomographic scan of the chest performed a year before lung transplantation showed calcifications in the left pulmonary artery (figure C) consistent with the pathological findings. 

Conclusion: Calcific atherosclerosis of the pulmonary artery is not one of the typical pathological features described in patients with PAH. With advances in the understanding of the pathophysiology of PAH and the availability of multiple approved therapies for this disease patients are living longer.  With improvement in survival the pulmonary vasculature is exposed to high pressure for longer periods of time and pathological changes, resembling those seen in the systemic circulation,  may be increasingly encountered.