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Chest radiograph of a 63-year-old, critically ill woman with an elevated jugular venous pressure, hyperdynamic precordium, and grade IV/VI systolic and diastolic murmurs heard best to the left of the sternum at the third and fourth interspaces. Her electrocardiogram showed atrial fibrillation and a pattern of right ventricular hypertrophy.
On hospital day two, right heart catheterization disclosed a pulmonary arterial pressure of 98/35 mm Hg (mean, 60 mm Hg), and angiography demonstrated massive dilatation of the major pulmonary arteries (image below). The next day, the patient died. Autopsy disclosed aneurysmal dilatation of the main pulmonary arteries and a healed dissecting aneurysm extending from the arch of the aorta to the iliac bifurcation. Cystic medial necrosis (CMN) was evident throughout the pulmonary arterial tree as well as the aorta and its major branches.
CMN characteristically affects the aorta. That it may also involve the pulmonary vasculature is not widely appreciated.
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This work is licensed by Herbert L. Fred, MD and Hendrik A. van Dijk under a Creative Commons Attribution License (CC-BY 2.0), and is an Open Educational Resource.
Last edited by Frederick Moody on Dec 4, 2008 3:06 pm -0600.
