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Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany.

ABSTRACT: BACKGROUND:The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH. METHODS:We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry. The CTEPH incidence was calculated based on German population data, and patient characteristics and treatment patterns were described. RESULTS:A total of 392 patients were newly diagnosed with CTEPH within 2016 in Germany, yielding an incidence of 5.7 new cases per million adults. The (mean?±?standard deviation) age was 63.5?±?15.0 years; males and females were equally affected; 76.3% of the patients had a history of venous thromboembolism. A total of 197 (50.3%) patients underwent pulmonary endarterectomy. Almost all non-operated patients received targeted drug therapy, and 49 patients (25.1% of the non-operated patients) were treated with balloon pulmonary angioplasty. CONCLUSION:The incidence of CTEPH in Germany 2016 was 5.7 per million adults and thus higher than previously reported from other countries. Half of the patients were operated while the remaining patients received medical or interventional therapies. CLINICAL TRIALS REGISTRATION:http://www.clinicaltrials.gov NCT02660463 and NCT01347216.

SUBMITTER: Kramm T

PROVIDER: S-EPMC6002445 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature

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Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany.

Kramm Thorsten T Wilkens Heinrike H Fuge Jan J Schäfers Hans-Joachim HJ Guth Stefan S Wiedenroth Christoph B CB Weingard Bettina B Huscher Doerte D Pittrow David D Cebotari Serghei S Hoeper Marius M MM Mayer Eckhard E Olsson Karen M KM

Clinical research in cardiology : official journal of the German Cardiac Society 20180215 7

<h4>Background</h4>The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH.<h4>Methods</h4>We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a Eur ...[more]

PMID: 29450722

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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.

Dataset Information

Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany.

Publications

Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany.

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