Epidemiology and management of chronic thromboembolic pulmonary hypertension: experience from two expert centers

Bazmpani, Maria Anna; Arvanitaki, Alexandra; Toumpourleka, Maria; Pitsiou, Georgia; Panagiotidou, Evangelia; Mouratoglou, Sophia Anastasia; Sianos, Georgios; Hadjimiltiades, Stavros; Pitsis, Antonis A.; Mayer, Eckhard; Stanopoulos, Ioannis; Karvounis, Haralambos; Giannakoulas, George

doi:10.1016/j.hjc.2017.05.005

Cited by 13 publications

(10 citation statements)

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“…The group of CTEPH patients was well characterized in a large prospective international registry which enrolled patients between February 2007 and January 2009. 7 As the phenotype of CTEPH patients may be population dependent, 8 several national reports as summarized in Table 1 7 , 9 – 29 were of added value. Most of them, however, recruited patients before the marketing authorization of riociguat by the European Medicines Agency through the European Union (27/03/2014), and before the first experience with balloon pulmonary angioplasty (BPA) outside of Japan.…”

Section: Introductionmentioning

confidence: 99%

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Kopeć

Dzikowska-Diduch

Mroczek

et al. 2021

Therapeutic Advances in Chronic Disease

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Background: Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients’ management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies. Methods: We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry. Results: We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants ( n = 301; 58.3%) were preferred over vitamin K antagonists ( n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment. Conclusions: The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH. Clinical Trial Registration: clinicaltrials.gov/ct2/show/NCT03959748

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Section: Introductionmentioning

confidence: 99%

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Kopeć

Dzikowska-Diduch

Mroczek

et al. 2021

Therapeutic Advances in Chronic Disease

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“…Shear stress and hypoxia trigger pulmonary endothelial dysfunction and in ammation which play a vital role in the pathophysiology of pulmonary arterial hypertension (PAH), and lead to the remodeling of small and medium sized pulmonary arterioles, and thus to a progressive obstructive pulmonary vasculopathy [2]. Chronic thromboembolic pulmonary hypertension (CTEPH), on the other hand, develops from the occlusion of the pulmonary vascular bed with nonresolving thromboemboli, leading also to pulmonary vascular remodeling and increased PVR [3].…”

Section: Introductionmentioning

confidence: 99%

Peripheral Microangiopathy in Pre-capillary Pulmonary Hypertension: A Nailfold Video Capillaroscopy Prospective Study

Arvanitaki

Giannakoulas

Triantafyllidou

et al. 2020

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Background: Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of this study was to investigate the presence of peripheral microangiopathy in patients with precapillary pulmonary hypertension by exploring nailfold video-capillaroscopic (NVC) structural changes among various subgroups with precapillary PH and to identify possible associations of NVC characteristics with markers of disease severity. Methods: Α cross-sectional study was performed in 46 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH), 18 with PAH associated with connective tissue diseases (CTD-PAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated. Results: Patients with precapillary PH (71.7% were women, mean age 60.8 ± 13.4 years) presented reduced capillary density compared to healthy controls (7.5 ± 1.6 loops/mm vs. 9.7 ± 0.81 loops/mm, p<0.001) and increased capillary loop width (18.8 ± 6.7 μm vs. 11.5 ± 2.3 μm, p<0.001) detected mainly in PAH-CTD. Half of patients presented microhaemorrhages on capillary nailfold, while morphological capillary abnormalities where also detected compared to healthy controls. Log10 (NT-proBNP) was independently associated with capillary density in patients with precapillary PH [r= -0.68, B= -1.9, 95% CI (-3,3, -0.4) p=0.014].Conclusion: Significant NVC microvascular changes were detected in patients with various types of precapillary PH, suggesting an impaired peripheral microcirculation associated with right ventricular remodeling parallel to pulmonary vasculopathy.

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“…Shear stress and hypoxia trigger pulmonary endothelial dysfunction and inflammation, which in turn promote the remodeling of small and medium sized pulmonary arterioles culminating in progressive obstructive pulmonary vasculopathy [2]. Chronic thromboembolic pulmonary hypertension (CTEPH), develops from the occlusion of the pulmonary vascular bed with non-resolving thromboemboli, leading also to pulmonary vascular remodeling and increased PVR [3].…”

mentioning

confidence: 99%

Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study

et al. 2021

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Background Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of the current study was to investigate the presence of nailfold video-capillaroscopic (NVC) structural changes in patients with precapillary PH and to identify possible associations of NVC measurements with markers of disease severity. Methods Α prospective case–control study was performed in 28 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability capillaroscopic images were reviewed by two independent investigators. For multiple comparisons among continuous variables, one-way ANOVA or the Kruskal–Wallis test were used. Differences between the groups were tested with post-hoc analysis with adjustment for multiple comparisons (Bonferroni test). Results Both IPAH (71.4% were women, mean age 53.1 ± 13.4 years) and CTEPH (64.3% women, mean age 60.9 ± 14.4 years) groups presented reduced capillary density compared to healthy controls (8.4 ± 1.2 loops/mm and 8.0 ± 1.2 loops/mm vs. 9.7 ± 0.81 loops/mm, p < 0.001) and increased loop width (15.7 ± 3.9 μm and 15.8 ± 1.9 μm vs. 11.5 ± 2.3 μm, p < 0.001). More than half of patients with IPAH presented microhaemorrhages on capillary nailfold, while increased shape abnormalities in capillary morphology and more capillary thrombi per linear mm were detected in patients with CTEPH compared to patients with IPAH and healthy controls. All PH patients presented a non-specific NVC pattern compared to controls (p < 0.001). Conclusion The findings of the study reveal a degree of significant peripheral microvascular alterations in patients with IPAH and CTEPH, suggesting a generalized impairment of peripheral microvasculature in pulmonary vascular disease.

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Epidemiology and management of chronic thromboembolic pulmonary hypertension: experience from two expert centers

Cited by 13 publications

References 20 publications

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Peripheral Microangiopathy in Pre-capillary Pulmonary Hypertension: A Nailfold Video Capillaroscopy Prospective Study

Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study

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