Management and outcomes in chronic thromboembolic pulmonary hypertension: From expert centers to a nationwide perspective

Escribano, Pilar; Pozo, Roberto Del; Román-Broto, Antonio; Morera, J.A. Domingo; Lara-Padrón, Antonio; Hernández, Teresa Elías; Molina-Ferragut, L.; Blanco, Isabel; Cortina, José M.; Barberà, Joan Albert

doi:10.1016/j.ijcard.2015.11.039

Cited by 48 publications

(37 citation statements)

References 27 publications

(31 reference statements)

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“…CTEPH can be cured by surgical removal of these chronic thrombi by pulmonary endarterectomy (PEA) . However, when PEA is not feasible, owing to advanced distal pulmonary artery remodeling or the patient's performance status, the prognosis is poor . Therefore, early CTEPH diagnosis and referral to an expert center are both crucial for optimal treatment .…”

Section: Introductionmentioning

confidence: 99%

Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

Ende-Verhaar

Hout

Bogaard

et al. 2018

Journal of Thrombosis and Haemostasis

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Essentials Diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) is long. We explored healthcare utilisation of patients diagnosed with CTEPH after pulmonary embolism. A large number of physicians were consulted and test results were not always interpreted correctly. Better education and higher awareness of CTEPH may lead to faster diagnosis. SUMMARY: Background The median diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) is 14 months, which may affect prognosis. We aimed to explore the healthcare utilization of patients diagnosed with CTEPH after acute pulmonary embolism (PE), and to identify the causes of diagnostic delay. Methods We collected all data on patient symptoms, medical specialist referrals and ordered diagnostic tests to reconstruct the clinical pathways of 40 patients referred to the VU University Medical Center Amsterdam (VUMC, the Netherlands) for CTEPH treatment. Diagnostic delay was defined as the time between first symptom onset and referral to the VUMC. Correlations of patient-specific characteristics and diagnostic delay were evaluated. Results Patients consulted four (median) different physicians for a median of 13 (interquartile range [IQR] 10-18) consultations before the correct diagnosis was made. The median diagnostic delay was 21 months (IQR 12-49 months). Echocardiographic results suggestive of CTEPH were not always followed by an adequate work-up; most patients were not subjected to ventilation/perfusion scanning. Prior cardiopulmonary comorbidity and recurrent venous thromboembolism were predictors of a longer delay. Conclusion Healthcare utilization in patients before their final CTEPH diagnosis was far from optimal, contributing to a considerable diagnostic delay. Better education and higher awareness of CTEPH among PE caretakers may lead to faster diagnosis.

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Section: Introductionmentioning

confidence: 99%

Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

Ende-Verhaar

Hout

Bogaard

et al. 2018

Journal of Thrombosis and Haemostasis

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“…Patients not being referred for PEA assessment were older and had a worse functional capacity. Older age was the most deterrent factor for non-operability [8].…”

Section: Surgical Treatmentmentioning

confidence: 95%

Prognosis Evaluation of Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension

S¹,

Mezghani²

2016

J Pulm Respir Med

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“…Single centres and registries have described the management of CTEPH: examples are summarised in table 3 [10,[43][44][45][46][47][48]. The patients reported may be postoperative, technically inoperable, those who refuse surgery and those with operable disease but who are not suitable for PEA because of comorbid conditions.…”

Section: Real-world Datamentioning

confidence: 99%

Medical management of chronic thromboembolic pulmonary hypertension

2017

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Chronic thromboembolic pulmonary hypertension (CTEPH) results from incomplete resolution of acute pulmonary emboli, organised into fibrotic material that obstructs large pulmonary arteries, and distal small-vessel arteriopathy. Pulmonary endarterectomy (PEA) is the treatment of choice for eligible patients with CTEPH; in expert centres, PEA has low in-hospital mortality rates and excellent long-term survival. Supportive medical therapy consists of lifelong anticoagulation plus diuretics and oxygen, as needed.An important recent advance in medical therapy for CTEPH is the arrival of medical therapies for patients with inoperable disease or persistent/recurrent pulmonary hypertension after PEA. The soluble guanylate cyclase stimulator riociguat is licensed for the treatment of CTEPH in patients with inoperable disease or with recurrent/persistent pulmonary hypertension after PEA. Clinical trials of this agent have shown improvements in patients' haemodynamics and exercise capacity. Phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostanoids have been used in the treatment of CTEPH, but evidence of benefit is limited. Challenges in the future development of medical therapy for CTEPH include better understanding of the underlying pathology, end-points to monitor the condition's progress, and the optimisation of pulmonary arterial hypertension therapies in relation to diverse patient characteristics and emerging options such as balloon pulmonary angioplasty.

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Management and outcomes in chronic thromboembolic pulmonary hypertension: From expert centers to a nationwide perspective

Abstract: Despite the increase in diagnosis and expertise in PEA-specialized centers, an important percentage of patients do not benefit of PEA in a decentralized organization model of CTEPH management.

Cited by 48 publications

References 27 publications

Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

Healthcare utilization in chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

Prognosis Evaluation of Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension

Medical management of chronic thromboembolic pulmonary hypertension

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