This site is intended for a US audience Non-US Residents For Patients and Caregivers

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

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

References:

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