Abstract

A 68-year-old woman with a 3-year history of breathlessness (World Health Organization functional class III) was diagnosed with idiopathic pulmonary arterial hypertension. Investigations included a 12-lead ECG, chest radiograph, and computed tomography scan of the thorax (Figure 1, A-C). At cardiac catheterization, mean pulmonary arterial pressure and pulmonary vascular resistance were elevated (73 mm Hg and 1545 dyne · s · cm−5, respectively), and cardiac index was reduced (1.9 L/min/m2). A solitary 60% left circumflex coronary artery stenosis was present. Cardiovascular magnetic resonance identified right ventricular (RV) hypertrophy (indexed mass of 64 g/m2; normal=16 to 36 g/m2), right ventricular systolic dysfunction (RV ejection fraction 31%; normal >57%), and paradoxical interventricular septal (IVS) motion. Late gadolinium-enhanced (LGE) images taken 10 minutes after injection of 0.1 mmol/kg gadolinium–diethylenetriamine pentaacetic acid showed enhancement of both insertion regions (Figure 2A, 2B, and 2D). Figure 1. A, Twelve-lead ECG: …

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