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CTEPH Case Study 2

63-year-old man with progressively worsening dyspnea on exertion (DOE) over last 3 months

  • Moderately active—had walked 30+ mins/day—until recent progression of dyspnea

  • History of acute pulmonary embolism (PE) subsequent to appendectomy 15 years previous

  • History of splenectomy for hereditary spherocytosis

  • Body mass index (BMI): 31 kg/m2; weight: 238 lbs



echocardiogram image


Additional studies

  • No evidence of acute PE on computed tomographic pulmonary angiogram (CTPA)

  • N-terminal prohormone of brain natriuretic peptide (NT-proBNP): 1439 pg/mL

  • 6-minute walk distance (6MWD): 398 meters

  • Assessed as WHO Functional Class II/III


Right heart catheterization

  • Mean pulmonary arterial pressure (mPAP): 48 mmHg

  • Right atrial pressure (RAP): 9.5 mmHg

  • Pulmonary capillary wedge pressure (PCWP): 9.8 mmHg

  • Cardiac output (CO): 3.1 L/min

  • Cardiac index (CI): 1.9 L/min/m2

  • ulmonary vascular resistance (PVR): 792 dyn∙sec∙cm-5


Key takeaways

Patients with confirmed PH should have a V/Q scan to rule out potentially curable (via pulmonary thromboendarterectomy) CTEPH1

A V/Q scan showing perfusion defects should prompt referral to an experienced center for further evaluation and, if CTEPH is confirmed, operability assessment1


1. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl D):D92-D99.