CTEPH Case Study 3
38-year-old woman with profound fatigue, chest pain, and lower extremity edema
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Patient has history of mixed connective tissue disease
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Patient reports having had difficulty breathing on recent visits to family in Colorado
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Body mass index (BMI): 22.5 kg/m2; weight: 135 lbs
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Patient has a history of being overweight and has used drugs to promote weight loss
Echocardiogram
Additional studies
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No evidence of acute pulmonary embolism (PE) on computed tomographic pulmonary angiogram (CTPA)
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N-terminal prohormone of brain natriuretic peptide (NT-proBNP): 987 pg/mL
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Six-minute walk distance (6MWD): 352 meters
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Assessed as WHO Functional Class II/III
Right heart catheterization
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Mean pulmonary arterial pressure (mPAP): 46 mmHg
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Right atrial pressure (RAP): 8.5 mmHg
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Pulmonary capillary wedge pressure (PCWP): 9.2 mmHg
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Cardiac output (CO): 3.2 L/min
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Cardiac index (CI): 2.2 L/min/m2
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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
References:
1. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl D):D92-D99.
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