Course: Progressive Dyspnea on Exertion in an 82-Year-Old

CME Credits: 1.00

Released: 2023-12-18

An 82-year-old with hereditary hemorrhagic telangiectasia and history of past cigarette smoking presented to pulmonary clinic for evaluation of dyspnea that had worsened over the previous 5 years. Two years prior, he was diagnosed with a right lower lobe segmental pulmonary embolism on a computed tomography (CT) pulmonary angiogram after evaluation of shortness of breath. Warfarin was initiated but discontinued after 2 months due to recurrent epistaxis.
On presentation to the pulmonary clinic, the patient reported dyspnea with minimal activity, fatigue, lightheadedness, and leg swelling. His temperature was 96.8 °F (36 °C); blood pressure, 120/71 mm Hg; heart rate, 73/min; and oxygen saturation, 98% on room air. He appeared frail and had multiple telangiectasias on his lips, tongue, and hands. He had jugular venous distension, a cardiac systolic murmur, right ventricular heave, and bilateral lower extremity edema to the knees. Laboratory testing revealed brain-type natriuretic peptide level of 1333 pg/mL (reference, <100 pg/mL) and hemoglobin level of 12.9 g/dL (reference, 13.9-16.3 g/dL). During a 6-minute walk test on room air, his oxygen saturation decreased to 87%. With oxygen delivered by nasal cannula at 4 L/min, his oxygen saturation was 98%, and he walked 90 m (15% of predicted). An echocardiogram showed a normal left ventricular ejection fraction, moderate-severe right ventricular dilatation, and right ventricular systolic pressure of 60 to 70 mm Hg (reference, 35-40 mm Hg). Findings on an echocardiogram performed 10 years prior were normal.

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

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