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CTEPH Case Study 3

38-year-old woman with profound fatigue, chest pain, and lower extremity edema

  • Patient has history of mixed connective tissue disease

  • Patient reports having had difficulty breathing on recent visits to family in Colorado

  • Body mass index (BMI): 22.5 kg/m2; weight: 135 lbs

  • Patient has a history of being overweight and has used drugs to promote weight loss



echocardiogram image

Additional studies

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

  • N-terminal prohormone of brain natriuretic peptide (NT-proBNP): 987 pg/mL

  • Six-minute walk distance (6MWD): 352 meters

  • Assessed as WHO Functional Class II/III


Right heart catheterization

  • Mean pulmonary arterial pressure (mPAP): 46 mmHg

  • Right atrial pressure (RAP): 8.5 mmHg

  • Pulmonary capillary wedge pressure (PCWP): 9.2 mmHg

  • Cardiac output (CO): 3.2 L/min

  • Cardiac index (CI): 2.2 L/min/m2

  • Pulmonary vascular resistance (PVR): 847 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

The V/Q scan has sensitivity >96%, and a normal V/Q scan essentially rules out CTEPH1

With CTEPH ruled out, PH workup should continue to correctly identify the causes of PH and inform treatment


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