Bosentan for patients with chronic thromboembolic pulmonary hypertension

Vassallo, F; Kodric, Metka; Scarduelli, Cleante; Harari, Sergio; Potena, A; Scarda, Angelo; Piattella, Marco; Cassandro, Roberto; Confalonieri, Marco

doi:10.1016/j.ejim.2008.03.008

Cited by 23 publications

(8 citation statements)

References 29 publications

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“…Vassallo et al also studied patients with inoperable chronic thromboembolic pulmonary hypertension, but compared bosentan with conventional therapy. 54 The results confirmed a significant improvement in exercise capacity, functional class, and Borg dyspnea index in the group that received bosentan.…”

Section: Trial In Chronic Thromboembolic Pulmonary Hypertensionmentioning

confidence: 56%

Dual receptor blockade by bosentan: clinical experience in treatment of pulmonary hypertension

Hoette

O’Callaghan²,

Jardim³

et al. 2010

JRLCR

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Pulmonary hypertension is characterized by an increase in mean pulmonary arterial pressure and right ventricular overload. Endothelin-1 (ET-1) is a potent vasoconstrictor with an important role in the pathogenesis of pulmonary hypertension. In addition to its vasoconstrictive action, ET-1 also stimulates cell proliferation, fibrosis, and inflammation. The blockade of both receptors involved in the action of ET-1 is beneficial in the treatment of pulmonary hypertension. The use of endothelin dual receptor blockade leads to improvements in quality of life, functional capacity, and pulmonary hemodynamics in affected patients. This article reviews the importance of ET-1 in the pathogenesis of pulmonary hypertension and demonstrates the benefits of blockage of the action of ET-1 in this disease.

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Section: Trial In Chronic Thromboembolic Pulmonary Hypertensionmentioning

confidence: 56%

Dual receptor blockade by bosentan: clinical experience in treatment of pulmonary hypertension

Hoette

O’Callaghan²,

Jardim³

et al. 2010

JRLCR

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“…Bosentan has previously shown promise for CTEPH patients, including those considered unsuitable for surgery and cases that have residual PH after surgery [13][14][15][16]. The response of the current patient also suggests that bosentan may be of use for PH that persists despite surgery.…”

Section: Discussionmentioning

confidence: 71%

Chronic thromboembolic pulmonary hypertension as a cause of dyspnoea in an older patient with a complex history

Kähler

2009

European Respiratory Review

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A full diagnostic work-up for patients with pulmonary hypertension (PH) is vital. Classification and diagnosis of the underlying cause is important to ensure optimal management, but may be complicated by overlapping signs and symptoms. This case study describes how a full work-up identified chronic thromboembolic PH (CTEPH) as the cause of dyspnoea in a 68-yr-old male with a history of pulmonary embolism and an original diagnosis of chronic obstructive pulmonary disease. Key indicators included decreased tricuspid annular plane systolic excursion, increased Tei index and elevated systolic pulmonary artery pressure. Multi-slice spiral chest computed tomography and pulmonary angiography showed severe chronic thromboembolic pulmonary disease, both centrally and distally. Diffusing capacity of the lung for carbon monoxide was reduced and blood gas analysis revealed a wide alveolar-arterial oxygen pressure difference, which is typical of CTEPH. The patient was eligible for pulmonary endarterectomy according to established criteria. Residual PH after surgery was successfully managed with bosentan.KEYWORDS: Chronic thromboembolic pulmonary hypertension, dyspnoea, pulmonary hypertension A 64-yr-old male first presented in 2003 at a local chest hospital with exertional dyspnoea, following a pulmonary embolism in 2002 and a history of smoking. At that time, a computed tomography (CT) scan revealed pulmonary embolism mainly in the left lower lobe; transthoracic echocardiography showed a slightly reduced left ventricular ejection fraction (EF) and elevated pulmonary arterial pressure (Ppa) (table 1). He was hypoxic and hyperventilating, with low oxygen saturation. The patient was diagnosed with chronic obstructive pulmonary disease with hypoxaemia, and discharged; congestive heart failure was attributed to the low EF and the prior history of pulmonary embolism. Acenocoumarol, ramipril, carvedilol, oxygen and salbutamol were started. In 2007, he presented again at another local hospital with worsening dyspnoea; investigations showed increased Ppa, with similar EF and lung function to those at the first assessment (table 1).The patient was subsequently reinvestigated by a specialist pneumology unit. Transthoracic echocardiography revealed decreased tricuspid annular plane systolic excursion (TAPSE; 1.4 cm) with increased Tei index (1.61) and increased right atrial and ventricular dimensions, with estimated systolic Ppa of 70 mmHg ( fig. 1). At this stage, forced vital capacity was 90% predicted, forced expiratory volume in 1 s was 76% pred (a ratio of 69%) and peak expiratory flow was 70% pred. Lung function tests and CT excluded emphysema, with normal total lung capacity and residual volume, and no hyperinflation. The patient's 6-min walk distance (6MWD) was 384 m. However, the diffusing capacity of the lung for carbon monoxide was reduced (59%), as is the case in ,20% of patients with idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension (CTEPH) [1,2]. Multi-slice spiral chest...

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“…The results of the open label trials are mainly positive, with only one obvious outlier [ 9 ]. Besides this outlier, RTM-adjusted treatment effects vary from 9.9 m to 138.7 m with an overall mean of 44.6 m (95% CI: [25.4; 63.8]).…”

Section: Resultsmentioning

confidence: 99%

“…Studies assessing the walking distance only once (e.g., at the beginning of the study) were excluded. Finally, a total of 18 randomized controlled trials and 24 open label studies were included in the quantitative analysis [ 9 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 ].…”

Section: Methodsmentioning

confidence: 99%

No Placebo Effect beyond Regression to the Mean on the Six Minute Walk Test in Pulmonary Arterial Hypertension Trials

Anheyer

Bugaj

Lüdtke

et al. 2023

IJMS

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In drug studies, patients are often included when the disease activity is high. This will make any treatment appear to lessen disease activity, although the improvement is biased by selection. This effect is known as regression towards the mean (RTM). We aimed at investigating drug trials in Pulmonary Arterial Hypertension (PAH) using the 6-minute walking distance test (6MWD) as a primary outcome for the phenomenon of RTM. An existing registry of 43 open label studies and 23 randomized controlled trials conducted between 1990 and 2009 was used as the data source. Data analysis was carried out for 18 randomized controlled trials (RCTs) and 24 open label studies out of this registry. Data were analyzed for verum and placebo arms of the RCTs separately, as well as for the open label arms. In the verum arms, the overall effect given as 33.2 m (95% CI: 25.7; 40.6]); 6MWD was slightly lower than the effect in the observational studies, with 44.6 m (95% CI: [25.4; 63.8]). After studying and interpreting the data, we found that regression towards the mean plays only a minor role in PAH studies. In particular, placebo effects in the RCTs were negligibly small, with a mean 6MWD of −2.5 m (95% CI: [−9.8; 4.7]) in the placebo arm. Therefore, our analysis indicates that results of non-randomized observational studies can be regarded as valid tools for gaining valid clinical effects in patients with PAH.

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Bosentan for patients with chronic thromboembolic pulmonary hypertension

Cited by 23 publications

References 29 publications

Dual receptor blockade by bosentan: clinical experience in treatment of pulmonary hypertension

Dual receptor blockade by bosentan: clinical experience in treatment of pulmonary hypertension

Chronic thromboembolic pulmonary hypertension as a cause of dyspnoea in an older patient with a complex history

No Placebo Effect beyond Regression to the Mean on the Six Minute Walk Test in Pulmonary Arterial Hypertension Trials

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