CTEPH Case Study 1
58-year-old woman with dyspnea on exertion (DOE), fatigue, and mild intermittent chest pain
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Patient initially referred to local cardiologist
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Mild hypertension, well-controlled Type II diabetes mellitus
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No history of deep vein thrombosis or pulmonary embolism (PE)
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Body mass index (BMI): 28 kg/m2; weight: 168 lbs
Echocardiogram
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Normal left ventricular systolic function; grade 1 diastolic dysfunction
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Mildly dilated right ventricle (RV), with mildly decreased RV function
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Mild biatrial dilation
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No significant valvular disease
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Right ventricular systolic pressure: 64.3 mmHg
Additional studies
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No evidence of acute PE on computed tomographic pulmonary angiogram (CTPA)
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NT-proBNP, 1602 pg/mL
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6-minute walk distance (6MWD): 402 meters
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Assessed as WHO Functional Class II
Right heart catheterization
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Mean pulmonary arterial pressure (mPAP): 44 mmHg
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Right atrial pressure (RAP): 8.5 mmHg
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Pulmonary capillary wedge pressure (PCWP): 9.3 mmHg
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Cardiac output (CO): 3.65 L/min
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Cardiac index (CI): 2.3 L/min/m2
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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.