ライフサイエンスコーパス: CTEPH

1 CTEPH patients exhibited vessel narrowing, intimal irreg

2 CTEPH pigs replicated the hemodynamics and histological

3 CTEPH results from persistent obstruction of pulmonary a

4 CTEPH-endothelial cells and murine endothelial cells lac

5 Reduced and denatured fibrinogen from 33 CTEPH patients was subjected to liquid chromatography-ma

6 dysfibrinogenemias) were observed in 5 of 33 CTEPH patients: Bbeta P235L/gamma R375W, Bbeta P235L/gam

7 unctional class II 53%, III 46%; PAH n = 98; CTEPH n = 18].

8 brin polymer structure and/or lysis with all CTEPH-associated mutations.

9 s lower in both PAH (2.6 +/- 0.8 mmol/l) and CTEPH (2.7 +/- 0.7 mmol/l) patients when compared to con

10 injury in a cohort of patients with PAH and CTEPH enrolled in 15 randomized clinical trials conducte

11 ces of hepatic injury resulting from PAH and CTEPH have not been well studied.

12 th adverse outcomes in patients with PAH and CTEPH.

13 ning as add-on to medical therapy in PAH and CTEPH.

14 patients with an E<A transmitral pattern and CTEPH who underwent pulmonary thromboendarterectomy (PTE

15 Transcriptome analysis in Sham and CTEPH pigs revealed molecular RV remodeling close to hum

16 we analyzed differences in phenotype between CTEPH thrombus and healthy pulmonary vascular cells.

17 l limitation after intermediate-risk PE, but CTEPH is infrequent.

18 A total of 593 consecutive CTEPH patients undergoing a first diagnostic right and l

19 smooth muscle cells (SMCs), that constitute CTEPH thrombus.

20 tify the multiple cell types that constitute CTEPH thrombusy and to study their dysfunction.

21 protein network analyses of patient derived CTEPH endothelial cells allowed the quantitation of 3258

22 Despite high sensitivity in detecting CTEPH, V/Q scanning is underutilized.

23 riables, with 171 patients (3.3%) developing CTEPH.

24 y and specificity, is integral to diagnosing CTEPH by identifying thrombi and associated pulmonary an

25 CTPA is essential for differentiating CTEPH from other pulmonary vascular conditions.

26 as the imaging modality of choice to exclude CTEPH.

27 5), significantly outperforming the existing CTEPH prediction score (0.57; 0.54-0.61).

28 erectomy remains the treatment of choice for CTEPH and is associated with excellent long-term results

29 clusions: These findings suggest a model for CTEPH similar to atherosclerosis, with chronic inflammat

30 ith CTEPH during follow-up (hazard ratio for CTEPH vs. no CTEPH 393; 95% confidence interval 73-2119)

31 ses were performed on RV cardiomyocytes from CTEPH and Sham-operated pigs.

32 We previously reported that fibrin from CTEPH patients is relatively resistant to fibrinolysis i

33 same cellular phenotype as fibroblasts from CTEPH vascular occlusions.

34 uscle, and myofibroblast cells isolated from CTEPH fibrothrombotic material have distinct phenotypes

35 l therapeutic targets in cells isolated from CTEPH thrombus.

36 Pulmonary endarterectomy specimens from CTEPH patients were analyzed using immunohistochemistry.

37 Furthermore, CTEPH myocytes showed lower [Ca(2+)](i) transients, decr

38 modynamics and histological changes of human CTEPH features.

39 model that replicates the phenotype of human CTEPH.

40 ronic thromboembolic pulmonary hypertension (CTEPH) (RVF, n = 10; no RVF, n = 16).

41 ronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary endarterectomy (PEA).

42 ronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar.

43 ronic thromboembolic pulmonary hypertension (CTEPH) can lead to right ventricular (RV) ischemia and d

44 ronic thromboembolic pulmonary hypertension (CTEPH) cause right ventricular dysfunction, which can im

45 ronic thromboembolic pulmonary hypertension (CTEPH) develops after acute pulmonary thromboembolism is

46 ronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening but curable form of pulmona

47 ronic thromboembolic pulmonary hypertension (CTEPH) is a rare but debilitating and life-threatening c

48 ronic thromboembolic pulmonary hypertension (CTEPH) is a rare, debilitating, and life-threatening dis

49 ronic thromboembolic pulmonary hypertension (CTEPH) is a sequela of acute pulmonary embolism (PE) in

50 ronic thromboembolic pulmonary hypertension (CTEPH) is a vascular disease characterized by the presen

51 ronic thromboembolic pulmonary hypertension (CTEPH) is associated with increased plasma levels of vWF

52 ronic thromboembolic pulmonary hypertension (CTEPH) is characterized by defective thrombus resolution

53 ronic thromboembolic pulmonary hypertension (CTEPH) is characterized by obstruction of major pulmonar

54 ronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with

55 ronic thromboembolic pulmonary hypertension (CTEPH) is the result of pulmonary arterial obstruction b

56 ronic thromboembolic pulmonary hypertension (CTEPH) pig model, which leads to progressive RV hypertro

57 ronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, report

58 ronic thromboembolic pulmonary hypertension (CTEPH) that is not amenable to thromboendarterectomy or

59 ronic thromboembolic pulmonary hypertension (CTEPH) undergoing invasive treatment.

60 ronic thromboembolic pulmonary hypertension (CTEPH) was confirmed in 4 (2.1%) versus 6 (3.2%) cases (

61 ronic thromboembolic pulmonary hypertension (CTEPH) will be accelerated by an animal model that repli

62 ronic thromboembolic pulmonary hypertension (CTEPH), and (ii) PPEI, a combination of persistent or wo

63 ronic thromboembolic pulmonary hypertension (CTEPH), but persistent pulmonary hypertension after PTE,

64 ronic thromboembolic pulmonary hypertension (CTEPH), but precise mechanisms remain unclear.

65 ronic thromboembolic pulmonary hypertension (CTEPH), not all patients have surgically accessible dise

66 ronic thromboembolic pulmonary hypertension (CTEPH), with ventilation-perfusion scanning and echocard

67 ronic thromboembolic pulmonary hypertension (CTEPH).

68 ronic thromboembolic pulmonary hypertension (CTEPH).

69 ronic thromboembolic pulmonary hypertension (CTEPH).

70 ronic thromboembolic pulmonary hypertension (CTEPH).

71 ronic thromboembolic pulmonary hypertension (CTEPH).

72 ronic thromboembolic pulmonary hypertension (CTEPH).

73 sion pressure waveform analysis may identify CTEPH patients at risk for persistent pulmonary hyperten

74 ulmonary vascular resistance, might identify CTEPH patients with significant distal, small-vessel dis

75 graphy being the initial diagnostic tests if CTEPH is a concern.

76 In CTEPH, LV diastolic function often appears abnormal.

77 d 34% in group 5 PH and 81%, 66%, and 42% in CTEPH, respectively.

78 e and RV pathophysiologic characteristics in CTEPH.

79 se, and ischemic heart disease are common in CTEPH patients.

80 he involvement of endothelial dysfunction in CTEPH using patient samples and by network medicine appr

81 y of characteristic radiological features in CTEPH, compare their prevalence with chronic thromboembo

82 ivation, and NET formation were increased in CTEPH patients.

83 (NF)-kappaB2 was significantly increased in CTEPH.

84 likely contribute to chronic inflammation in CTEPH.

85 function of endothelial cells is involved in CTEPH.

86 omal interaction molecule 1 long isoform) in CTEPH myocytes as well as in RV from human patients with

87 left ventricular filling pressures (LVFP) in CTEPH.

88 rsed the prothrombotic phenotype observed in CTEPH-pulmonary artery endothelial cells.

89 arget that links thrombosis to chronic PE in CTEPH, with PAR1 inhibition decreasing SMC and myofibrob

90 eft heart disease, and predicts prognosis in CTEPH.

91 d histone acetylation of the vWF promoter in CTEPH endothelium, facilitating binding of NF-kappaB2 to

92 a levels of vWF, the role of this protein in CTEPH has remained enigmatic.

93 essment of myocardial perfusion, its role in CTEPH remains unclear.

94 andomized comparison with medical therapy in CTEPH patients who are not surgical candidates.

95 Objectives: To identify the role of vWF in CTEPH.

96 then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplas

97 tent after thromboendarterectomy (inoperable CTEPH) include pulmonary vasodilators or balloon pulmona

98 tly improved PVR in patients with inoperable CTEPH and was well tolerated.

99 ng-term outcomes in patients with inoperable CTEPH or persistent or recurrent pulmonary hypertension

100 ing outcomes for >5 patients with inoperable CTEPH were sought.

101 For patients with inoperable CTEPH, various medical and interventional therapies are

102 or therapy alone in patients with inoperable CTEPH.

103 and hemodynamics in patients with inoperable CTEPH.

104 ent of patients enrolled in an international CTEPH registry was investigated.

105 This second international CTEPH registry reveals important improvement in patient

106 ive study analysed 115 patients divided into CTEPH (n = 35), CTED (n = 20), PAH (n = 24), and APE (n

107 At the cellular level, CTEPH myocytes presented reduced L-type Ca(2+) current i

108 Methods: CTEPH-specific patient plasma and pulmonary endarterecto

109 Moreover, CTEPH cardiomyocytes exhibited reduced Ca(2+) spark occu

110 These data indicate that PTE offers most CTEPH patients substantial improvement in survival, func

111 ing follow-up (hazard ratio for CTEPH vs. no CTEPH 393; 95% confidence interval 73-2119).

112 nical presentation, operable and nonoperable CTEPH patients may have distinct associated medical cond

113 ignaling was present in vessel-rich areas of CTEPH specimens.

114 Diagnostic confirmation of CTEPH is provided by digital subtraction pulmonary angio

115 brin may be implicated in the development of CTEPH after acute thromboembolism.

116 multiple mechanisms, with equal diagnoses of CTEPH and group 5 PH.

117 d in plasma and the pulmonary endothelium of CTEPH patients.

118 iography usually reveals typical features of CTEPH, including mosaic perfusion, part or complete occl

119 The exact incidence of CTEPH is unknown but appears to approximate 2.3% among s

120 ve study, the cumulative 2-year incidence of CTEPH was 2.3%, but PPEI diagnosed by standardized crite

121 cations for the prevention and management of CTEPH.

122 d to investigate the underlying mechanism of CTEPH.

123 gen) might contribute to the pathogenesis of CTEPH.

124 he role of TGFbeta in the pathophysiology of CTEPH is unknown.

125 to reproduce much of the known phenotype of CTEPH, including the pivotal pathophysiological role of

126 i, or endarterectomy specimens and plasma of CTEPH patients, and endothelin-1 overexpression was prev

127 ombosis in the development or progression of CTEPH.

128 ls to identify patients at increased risk of CTEPH and post-PE syndrome.

129 At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1

130 Objectives: Our current understanding of CTEPH pathobiology is primarily derived from cell-based

131 The diagnostic work-up to detect or rule out CTEPH should include ventilation-perfusion scintigraphy,

132 For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 cen

133 Chronic thromboembolic PH (CTEPH) is an important complication and contributor to P

134 re diagnosed with chronic thromboembolic PH (CTEPH); the other half were considered to have group 5 P

135 e learning models show promise in predicting CTEPH but are less effective for post-PE syndrome.

136 agnetic navigation in patients with residual CTEPH after PEA.

137 Fifty patients with residual CTEPH despite medical therapy at least 6 months after PE

138 PADN in patients with residual CTEPH resulted in substantial reduction of PVR at 12 mon

139 Patients with suspected CTEPH should be referred to a specialist centre for righ

140 in the evaluation of patients with suspected CTEPH, the presence of mismatched segmental defects bein

141 The CTEPH model demonstrated good performance with an AUC of

142 The CTEPH was diagnosed in 16 (1.6%) patients, after a media

143 In the CTEPH group, LDL-C increased (from 2.6[2.1-3.2] to 4.0[2

144 The performance of the CTEPH prediction model was benchmarked against an existi

145 helial dysfunction in the development of the CTEPH.

146 on resulted in elevated platelet adhesion to CTEPH endothelium.

147 whereas PV and ET were generally related to CTEPH.

148 gical understanding of how PE transitions to CTEPH in human treatments.

149 Bbeta P235L was found in 3 unrelated CTEPH patients.

150 dentify 15 of the 16 patients diagnosed with CTEPH during follow-up (hazard ratio for CTEPH vs. no CT

151 y 2018 and December 2020 in 51 patients with CTEPH (mean age, 47 years +/- 17 [SD]; 27 women) were re

152 e mouth to the mitochondria in patients with CTEPH (n=20) as compared with healthy participants (n=10

153 rial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable.

154 /A is consistently abnormal in patients with CTEPH and increases post-PTE.

155 lmonary artery hypertension in patients with CTEPH and is associated with long-term improvement in Ne

156 Patients with CTEPH and other symptomatic patients with extensive resi

157 Patients with CTEPH and Rup value <60% appear to be at highest risk.

158 erfusion CMR findings from two patients with CTEPH before and after pulmonary thromboendarterectomy (

159 s of functional limitations in patients with CTEPH before and after pulmonary vascular intervention.

160 ation of hypoperfused areas in patients with CTEPH can be performed from clinical multienergy CT exam

161 ntricular pressure overload in patients with CTEPH causes abnormal LV diastolic filling.

162 Global HLV correctly separated patients with CTEPH from controls (area under the receiver operating c

163 We demonstrated that patients with CTEPH have significant impairment of all steps in the O(

164 relaxation pattern observed in patients with CTEPH is not solely the result of geometric effects of R

165 dynamic data in 39 consecutive patients with CTEPH over the age of 30 (55 +/- 11 years) with mean pul

166 ) was significantly reduced in patients with CTEPH relative to controls (56 17 versus 112 20% of pred

167 y endarterectomy material from patients with CTEPH were used to study the relationship between inflam

168 The LV of patients with CTEPH with RVF also exhibited ERP prolongation (306 +/-

169 udies in humans, we focused on patients with CTEPH, a severe type of deep venous and pulmonary artery

170 osed (</=6 months) consecutive patients with CTEPH, from February 2007 until January 2009.

171 were diagnosed in half of the patients with CTEPH.

172 ) on the long-term survival of patients with CTEPH.

173 iguat may be used long term in patients with CTEPH.

174 Not all patients presenting with CTEPH have a history of clinically overt pulmonary embol

175 age, 51.8 years; range, 14 to 75 years) with CTEPH underwent BPA; they averaged 2.6 procedures (range