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CTEPH Diagnostic Pathway — EECC MCQ

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ModeratePulmonary Vascular DiseaseCTEPH Diagnostic PathwayEECC

A 50-year-old woman with prior PE 18 months ago presents with progressive exercise intolerance. Echocardiography shows RV dilatation with estimated sPAP 65 mmHg. V/Q scan shows bilateral mismatched perfusion defects. What is the single most important investigation to confirm the diagnosis and guide management?

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Correct answer: C — Right heart catheterisation to confirm pulmonary hypertension haemodynamically and assess PVR, followed by expert assessment of operability

The clinical picture (progressive dyspnoea post-PE with mismatched perfusion defects and echocardiographic signs of PH) is highly suggestive of CTEPH. The diagnostic pathway per the 2022 ESC/ERS PH Guidelines is: (1) V/Q scintigraphy as the screening test of choice (more sensitive than CTPA for CTEPH); (2) CT pulmonary angiography for anatomical assessment; (3) right heart catheterisation to confirm PH (mPAP >20 mmHg, PAWP ≤15 mmHg, PVR >2 WU) and assess severity; (4) referral to an expert CTEPH centre for operability assessment (pulmonary endarterectomy vs BPA vs medical therapy). RHC is the essential confirmatory and prognostic investigation.

Reference: ESC/ERS (2022): Guidelines for Pulmonary Hypertension