An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management

Abstract

Abstract:We conducted an international study to evaluate practices in the diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) globally across different regions. Between August and October 2012, CTEPH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 2-5 most recent patients with CTEPH. Overall, 496 physicians (Europe: 260; United States: 152; Argentina: 52; Japan: 32) completed the questionnaire and provided patient record data for 1,748 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension (PH) center ranged from 38% in France and Italy to 83% in the United States. A large proportion of patients did not undergo ventilation/perfusion scanning (46%-67%) or right heart catheterization (24%-57%) for the diagnosis of CTEPH. Referral rates for pulmonary endarterectomy evaluation ranged from 25% in Japan to 44% in Europe, with higher referral rates in PH centers; the main reasons for lack of referral were that surgery was not considered unless medical treatment was failing and patient refusal. Other variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of combination treatment in the United States than in Europe. Physicians' perceptions of their treatment strategy were generally consistent with patient record data. Results from this study, which includes a global aspect of CTEPH care, demonstrate not only regional differences in CTEPH management but, more importantly, considerable nonadherence to the diagnosis and treatment guidelines for CTEPH, even in PH centers.Keywords: chronic thromboembolic pulmonary hypertension, pulmonary arterial hypertension-specific therapies, diagnosis, clinical practice, guidelines. Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of obstructive pulmonary artery remodeling as a consequence of major vessel thromboembolism. 1 A ventilation/perfusion (V/Q) scan is recommended in the workup of all patients with pulmonary hypertension (PH) to screen for CTEPH. 1 Diagnosis of CTEPH is challenging for several reasons, including nonspecific presenting symptoms, which may occur late in the progression of the disease, and a lack of prior pulmonary embolism in a high percentage of patients. 2 Once CTEPH is diagnosed, pulmonary endarterectomy (PEA) is the gold-standard treatment for these patients and is potentially curative. 1,3 However, between 20% and 40% of these patients are considered to have inoperable CTEPH, 4 and approximately 30% may have residual PH after PEA (defined as mean pulmonary artery pressure of ≥30 mmHg at 3-month follow-up after surgery). 5 Balloon pulmonary angioplasty (BPA) is an emerging treatment option for patients with inoperable or persistent CTEPH. 3 However, the role of BPA in CTEPH has not been investigated in randomized trials, and further studies are warranted; current guidelines state ...

An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan

“…GALL et al [41] recently reported the findings of an international physician survey of CTEPH management practice, in which it was found that a relatively higher proportion of patients in Europe than the USA (43% versus 12%) were diagnosed with CTEPH in pulmonary hypertension expert centres. In addition, the use of V′/Q′ scanning in the diagnosis of CTEPH was very low (40% and 45% in pulmonary hypertension centres in Europe and the USA, respectively), contrary to guideline recommendations.…”

“…There appeared to be regional variations in the numbers of patients in NYHA FC III/IV at diagnosis in this study, particularly between Japan and Europe. An international physician survey of CTEPH diagnosis and management practice showed that the majority of patients were in WHO FC III/IV at diagnosis, with some variation in WHO FC between the regions assessed (which included Europe, the USA and Japan) [41]. The reasons for these regional differences are unclear, but may relate to levels of awareness of CTEPH in different regions, which could then impact on likelihood of earlier diagnosis.…”

“…However, one study indicated that many patients diagnosed with CTEPH are not assessed by a surgeon for PEA operability [41]. Furthermore, among patients who are assessed and have been deemed to be eligible for PEA, only ∼85% undergo the procedure.…”

“…Further data included in the analysis were obtained from the Pulmonary Arterial Hypertension (PAH)/CTEPH Treatment Tracking Study, Ipsos Healthcare (2012) [41,42], unpublished internal audit data (Papworth Hospital (Cambridge, UK) internal audit, 2013), market research and data from the International CTEPH Association.…”

“…PEA operability assessment, treatment rate and outcomes One study showed that suitability for PEA was assessed by a surgeon in 18-47% of patients diagnosed with CTEPH in Europe, the USA and Japan [41].…”