Abstract
BackgroundChronic Thromboembolic Pulmonary Hypertension (CTEPH) results from incomplete resolution of a pulmonary embolus, leading to pulmonary hypertension and progressive right heart failure and death. We aimed to describe the demographics, treatment patterns, health resource utilization and related costs of patients with CTEPH.MethodsIn specialized PH centres across six European countries, medical charts of CTEPH patients on PH medication were retrospectively extracted (chart review between 2006 and 2009). Resource utilization was valued using country-specific unit costs. Descriptive statistical analyses were performed.ResultsTwenty-one hospitals documented 119 consecutive CTEPH patients over an average of 25.4 months. Patients were inoperable (83.9%) or persistent after surgery (16.0%) with mean age 67.5 ± 12.3 years, 61% were female. The average 6-minute walking distance was 298 ± 120 meters, and NYHA class II/III/IV was 27/59/14%. At baseline, 59.7% patients received endothelin receptor antagonist, 34.4% phosphodiesterase-5 inhibitors, and 5.8% prostacyclin. Adding a second PH medication was the most common regimen change. CTEPH patients experienced 1.8 ± 2.2 hospitalizations per year accounting for 14.8 ± 26.1 days in hospital. Patients paid on average 2.8 office visits per year to their general practitioner and 1.3 visits to a specialist. Unadjusted annual mortality rate was 6.0%. Annual cost of PH specific medication was the predominant economic factor averaging € 36,768 per year. Costs for hospitalizations (€ 4,496) and concomitant medications (€ 2,510) were substantially lower. Other health care resource items only accounted for marginal additional costs.ConclusionCTEPH patients are characterised by substantial morbidity and mortality. Health care utilisation, predominantly due to off-label use of PH drugs, is significant.
Highlights
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) results from incomplete resolution of a pulmonary embolus, leading to pulmonary hypertension and progressive right heart failure and death
We aimed to describe the demographics, drug treatment patterns, outcomes and costs of patients with CTEPH treated under everyday practice conditions in six European countries
Patients were eligible for inclusion, if they were at least 18 years old, had a confirmed diagnosis of CTEPH (Group 4 according to Dana Point 2008 criteria), were in New York Heart Association (NYHA) class II to IV, treated with monotherapy or combination therapy with endothelin receptor antagonists (ERA), prostacyclin analogues (PA) or phosphodiesterase-5 (PDE-5) inhibitors
Summary
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) results from incomplete resolution of a pulmonary embolus, leading to pulmonary hypertension and progressive right heart failure and death. An important and cause of PH is chronic thromboembolic PH (CTEPH), which is the result of pulmonary vascular obstruction characterized by recurrent, unresolved pulmonary emboli and/or progressive pulmonary vascular thrombosis and scarring [3]. Roughly 10% of patients who undergo PEA maintain a pulmonary hypertensive state since they obtain limited relief from surgery or experience recurrence [9]. Such inoperable or residual/recurrent patients are frequently treated with PH drugs (off-label) due to the lack of other treatment alternatives [11]. There is a lack of data on costs and resource utilization associated with CTEPH in patients in the real-world setting
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