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A 57 Year Old Woman with Pulmonary Hypertension Suffering Worsening Dyspnea on Endothelin Receptor Antagonist Therapy

Case Editor - Judd Flesch

Reviewed By Pulmonary Circulation Assembly

Submitted by

Dylan J Wirtz, MD


Pulmonary and Critical Care Medicine

Ohio State University College of Medicine

Columbus, Ohio 

J Shaun Smith, DO 

Assistant Professor of Medicine

Division of Pulmonary, Critical Care, and Sleep Medicine

University of Louisville School of Medicine

Louisville, Kentucky

Submit your comments to the author(s).


The patient is a 57-year-old woman with a history of pulmonary hypertension who presented to the outpatient pulmonary clinic for a second opinion concerning her worsening dyspnea on exertion. She had been hospitalized two years prior with shortness of breath. At that time echocardiogram was preformed suggesting pulmonary hypertension with a right ventricular systolic pressure (RVSP) of 45 mmHg. A radionuclide ventilation-perfusion (V/Q) scan revealed one mismatched defect and was deemed intermediate probability for pulmonary embolism. Subsequent computed tomography pulmonary angiography (CT-PA) did not reveal a pulmonary embolism and showed no underlying parenchymal lung abnormalities.  Right heart catheterization demonstrated a mean pulmonary artery pressure (MPAP) of 58 mmHg with a pulmonary capillary wedge pressure (PCWP) of 7 mmHg, thereby supporting a diagnosis of pulmonary arterial hypertension. Bosentan, diuretics, and warfarin were initiated and the patient was discharged home.

She now presents with worsening dyspnea on exertion and fatigue for the last two years. Recently she has developed palpitations and dizziness with exertion, but did not endorse syncope or chest pain.

Physical Exam

The patient is 63 inches tall and weighs 130 pounds. Her blood pressure is 105/60 and her heart rate is 78. She is in no acute distress with an oxygen saturation is 94% on room air. Examination of the heart reveals regular rate and rhythm. There is an accentuated pulmonic component of the second heart sound as well as a significant right ventricular heave. Examination of the lung fields reveals audible bilateral flow murmurs posteriorly.  She has no hepatomegaly, ascites, or peripheral edema.

Question 1

What is the most likely underlying etiology of this patient’s pulmonary arterial hypertension?


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