Abstract 14894: A Shocking Deficiency: An Unusual Case of Rapidly Progressive Pulmonary Hypertension

A 48-year-old woman presented with rapidly progressive chest pain and dyspnea. Over the past year she had experienced recurrent pericarditis, joint pain, and rash. Transthoracic echocardiogram showed normal left ventricular function, severe right ventricular (RV) dysfunction, and an estimated RV systolic pressure of 84 mmHg. An echocardiogram six months earlier had been normal. Right heart catheterization revealed a mean pulmonary artery pressure of 41 mmHg, pulmonary capillary wedge pressure of 5 mmHg, cardiac index of 1.9 L/min/m2, and pulmonary vascular resistance (PVR) of 1050 dyn•sec/cm5 (13 Woods units). Vasoreactivity testing with inhaled nitric oxide decreased mean pulmonary arterial pressure (34 mmHg), increased cardiac index (3.6 L/min/m2), and decreased PVR (410 dyn•sec/cm5). She was started on inhaled epoprostenol and a broad rheumatologic, infectious, and nutritional workup was initiated. She continued to decline over the next several days with a persistently elevated PVR and increasing vasop...