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Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team.

ABSTRACT: BACKGROUND:Chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapy (MT). Assessment in a multidisciplinary team of experts (CTEPH team) is currently recommended for treatment decision making. The aim of the present study was to report the effects of such an interdisciplinary concept. METHODS AND RESULTS:A total of 160 patients were consulted by the CTEPH team between December 2015 and September 2018. Patient baseline characteristics, CTEPH team decisions and implementation rates of diagnostic and therapeutic procedures were analysed. Change in World Health Organization (WHO) functional class and survival rates were evaluated by treatment strategy. A total of 51 (32%) patients were assessed as operable and 109 (68%) were deemed inoperable. Thirty-one (61% of operable patients) underwent PEA. Patients treated with PEA, BPA(+MT) and MT alone were 50.9?±?14.7, 62.9?±?15.1 and 68.9?±?12.7 years old, respectively. At the follow-up, PEA patients had the highest WHO functional class improvement. Patients treated with BPA(+MT) had significantly better survival than PEA (p?=?0.04) and MT patients (p?=?0.04; 2-year survival of 92%, 79% and 79%, respectively). CONCLUSIONS:The CTEPH team ensures that necessary diagnostic procedures are performed. A relatively low proportion of patients was assessed by the CTEPH team as operable and underwent surgery, which in survivors resulted in the best functional improvement. Although patients undergoing BPA(+MT) were older than patients treated with PEA, their survival was better than patients subjected to PEA or MT alone. The reviews of this paper are available via the supplemental material section.

SUBMITTER: Siennicka A

PROVIDER: S-EPMC6935880 | biostudies-literature | 2019 Jan-Dec

REPOSITORIES: biostudies-literature

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Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team.

Siennicka Anna A Darocha Szymon S Banaszkiewicz Marta M Kędzierski Piotr P Dobosiewicz Anna A Błaszczak Piotr P Peregud-Pogorzelska Małgorzata M Kasprzak Jarosław Damian JD Tomaszewski Michał M Mroczek Ewa E Zięba Bożena B Karasek Danuta D Ptaszyńska-Kopczyńska Katarzyna K Mizia-Stec Katarzyna K Mularek-Kubzdela Tatiana T Doboszyńska Anna A Lewicka Ewa E Ruchała Marcin M Lewandowski Maciej M Łukasik Sylwia S Chrzanowski Łukasz Ł Zieliński Dariusz D Torbicki Adam A Kurzyna Marcin M

Therapeutic advances in respiratory disease 20190101

<h4>Background</h4>Chronic thromboembolic pulmonary hypertension (CTEPH) may be treated with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapy (MT). Assessment in a multidisciplinary team of experts (CTEPH team) is currently recommended for treatment decision making. The aim of the present study was to report the effects of such an interdisciplinary concept.<h4>Methods and results</h4>A total of 160 patients were consulted by the CTEPH team between December ...[more]

PMID: 31878837

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Project description:Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of severe pulmonary hypertension (PH). The disease is still underdiagnosed, and the true prevalence is unknown. CTEPH is characterized by intraluminal non-resolving thrombus organization and fibrous stenosis, or complete obliteration of pulmonary arteries, promoted by progressive remodeling of the pulmonary vasculature. One consequence of this is an increase in pulmonary vascular resistance and pressure, resulting in PH and progressive right heart failure, leading to death if left untreated. Endovascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for CTEPH patients. PEA surgery is the only technique that can potentially cure CTEPH disease, especially in patients with fresh or organized thrombi of the proximal branches of pulmonary arteries. However, not all patients are eligible for PEA surgery. Recent research has provided evidence suggesting balloon pulmonary angioplasty (BPA) and targeted medical therapy as additional promising available treatments options for inoperable CTEPH and recurrent/persistent PH after PEA surgery. Studies on BPA have shown it to improve pulmonary hemodynamics, symptoms, exercise capacity and RV function in inoperable CTEPH. Subsequently, BPA has developed into an essential component of the modern era of CTEPH treatment. Large randomized controlled trials have demonstrated varying significant improvements with targeted medical therapy in technically inoperable CTEPH patients. Thus, treatment of CTEPH requires a comprehensive multidisciplinary assessment, including an experienced PEA surgeon, PH specialist, BPA interventionist and CTEPH-trained radiologist at expert centers. In this comprehensive review, we address the latest developments in the fast-evolving field of CTEPH. These include advancements in imaging modalities and developments in operative and interventional techniques, which have widened the range of patients who may benefit from these procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed. As the treatment options for CTEPH improve, hybrid management involving multiple treatments in the same patient may become a viable option in the near future.

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Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team.

Publications

Treatment of chronic thromboembolic pulmonary hypertension in a multidisciplinary team.

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