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CTEPH Case Study 1

58-year-old woman with dyspnea on exertion (DOE), fatigue, and mild intermittent chest pain

  • Patient initially referred to local cardiologist

  • Mild hypertension, well-controlled Type II diabetes mellitus

  • No history of deep vein thrombosis or pulmonary embolism (PE)

  • Body mass index (BMI): 28 kg/m2; weight: 168 lbs

 

Echocardiogram

  • Normal left ventricular systolic function; grade 1 diastolic dysfunction

  • Mildly dilated right ventricle (RV), with mildly decreased RV function

  • Mild biatrial dilation

  • No significant valvular disease

  • Right ventricular systolic pressure: 64.3 mmHg

 

Additional studies

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

  • NT-proBNP, 1602 pg/mL

  • 6-minute walk distance (6MWD): 402 meters

  • Assessed as WHO Functional Class II

 

Right heart catheterization

  • Mean pulmonary arterial pressure (mPAP): 44 mmHg

  • Right atrial pressure (RAP): 8.5 mmHg

  • Pulmonary capillary wedge pressure (PCWP): 9.3 mmHg

  • Cardiac output (CO): 3.65 L/min

  • Cardiac index (CI): 2.3 L/min/m2

  • Pulmonary vascular resistance (PVR): 785 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.