Assessment for residual disease after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension

Abstract

ObjectivesPulmonary endarterectomy (PEA) is recommended for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH) and is potentially curative. However, persistent/recurrent CTEPH post-PEA can occur. Here we describe symptom and diagnostic assessment rates for residual disease post-PEA and longitudinal diagnostic patterns before and after riociguat approval for persistent/recurrent CTEPH after PEA.MethodsThis US retrospective cohort study analysed MarketScan data (1 January 2002–30 September 2018) from patients who underwent PEA following a CTEPH/pulmonary hypertension (PH) claim with at least 730 days of continuous enrolment post-PEA. Data on pre-specified PH symptoms and the types and timings of diagnostic assessments were collected.ResultsOf 103 patients (pre-riociguat approval, n=55; post-riociguat approval, n=48), residual PH symptoms more than 3 months after PEA were reported in 89% of patients. Overall, 89% of patients underwent 1 or more diagnostic tests (mean 4.6 tests/patient), most commonly echocardiography (84%), with only 5% of patients undergoing right heart catheterisation (RHC). In the post- versus pre-riociguat approval subgroup, assessments were more specific for CTEPH with an approximate 2-fold increase in 6-minute walking distance and N-terminal prohormone of brain natriuretic protein measurements and ventilation/perfusion scans, and a 4-fold increase in RHCs.ConclusionsLow RHC rates suggest that many patients with PH symptoms post-PEA are not being referred for full diagnostic work-up. Changes to longitudinal diagnostic patterns may indicate increased recognition of persistent/recurrent CTEPH post-PEA; however, there remains a need for greater awareness around the importance of continued follow-up for patients with residual PH symptoms post-PEA.

Lessons learned in developing a chronic thromboembolic pulmonary hypertension program

“…We perform this follow up protocol to ensure well tolerated and effective outcomes, particularly as a CTEPH team develops early experience. Moreover, evidence suggest that residual pulmonary hypertension after PTE is rather common even in expert centers [22], and routine RHC 3–6 months after PTE is recommended to detect it by the guidelines [16 ▪▪ ], but rarely performed [23]. If the patient is unable to return to the CTEPH center, the referring team is contacted and instructed on follow up care.…”