Bosentan Therapy for Inoperable Chronic Thromboembolic Pulmonary Hypertension

Hoeper, Marius M.; Kramm, Thorsten; Wilkens, Heinrike; Schulze, Christine; Schäfers, Hans Joachim; Welte, Tobias; Mayer, Eckhard

doi:10.1378/chest.128.4.2363

Cited by 154 publications

(81 citation statements)

References 17 publications

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“…Hoeper recommended that an estimated reduction in PVR of >50% could be indicated for surgery. 20 In the present study, when patients were divided into 2 groups (ie, sufficiently improved group and modestly improved group) according to percentage decrease in PVR >50%, the sufficiently improved group showed significantly better QOL scores in physical functioning (p=0.01), role function (physically related) (p=0.045), general health perceptions (p=0.019), social functioning (p=0.017), role function (emotion-related) (p<0.01), mental health (0.03), and baseline dyspnea index (p=0.03) than those in the modestly improved group at follow up. Furthermore, the number of patients with a percentage decrease in PVR was significantly smaller in patients with relatively peripheral type compared with those with central type.…”

Section: Discussionmentioning

confidence: 99%

“…16 Recently, there have been some reports about improved 6-min walk distance and pulmonary hemodynamics after epoprostenol, 17,18 sildenafil 19 and bosentan [20][21][22] in patients with CTEPH. These new drugs might improve vascular remodeling, and may offer improved survival in patients with relatively peripheral type CTEPH, in whom we predicted a poor reduction in PVR after surgery.…”

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confidence: 99%

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Survival and Quality of Life for Patients With Peripheral Type Chronic Thromboembolic Pulmonary Hypertension

Yoshimi

Tanabe

Masuda

et al. 2008

Circ J

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hronic thromboembolic pulmonary hypertension (CTEPH) is a relatively rare disease. Its natural history and etiology remain unclear. [1][2][3][4][5] There have been a number of reports that pulmonary thromboendarterectomy (PTE) is an effective modality for treatment in selected patients with CTEPH. [1][2][3][6][7][8][9][10][11] However, the hemodynamic benefit varies according to the location and extent of the thromboembolic occlusion, and Bergin and colleagues reported that the computed tomography (CT) angiographic extent of central disease (ie, CD score) is related to a low pulmonary vascular resistance (PVR) after surgery. 12 Our preliminary data also showed high operative mortality with CD scores of 0 (25%) and 1 (20%), compared with those with a CD score of ≥2 (7.7%), so we classified those patients with a CD score of ≤1 as having relatively peripheral type CTEPH. The cause of operative death was related to residual pulmonary hypertension as a result of failure to remove a distal obstruction. New guidelines have recommended a post-surgical estimated reduction in PVR of >50%. 10 The validity of PTE might be limited to central-type patients, especially in institutions in which the operation is performed infrequently.The prognosis of CTEPH in the medically treated group had been thought to be poor, 13-15 but Ono et al reported that oral beraprost sodium improved their survival. 16 Recently, there have been some reports about improved 6-min walk distance and pulmonary hemodynamics after epoprostenol, 17,18 sildenafil 19 and bosentan [20][21][22] in patients with CTEPH. These new drugs might improve vascular remodeling, and may offer improved survival in patients with relatively peripheral type CTEPH, in whom we predicted a poor reduction in PVR after surgery. We retrospectively tested the validity of PTE from the aspects of survival and quality of life (QOL) at long-term follow up in patients with relatively peripheral type CTEPH. Methods PatientsBetween April 1999 and March 2006, a total of 83 patients admitted consecutively to Chiba University Hospital were diagnosed as having CTEPH and evaluated for surgical indication. CTEPH was defined as having a mean pulmonary arterial pressure (PPA) of ≥25 mmHg with normal wedge pressure in patients who had dyspnea on exertion during a period of more than 6 months. In addition, lung

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Survival and Quality of Life for Patients With Peripheral Type Chronic Thromboembolic Pulmonary Hypertension

Yoshimi

Tanabe

Masuda

et al. 2008

Circ J

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“…The short-term efficacy of bosentan in this group of patients has been described in a few, non-placebo controlled and mainly retrospective studies. [8][9][10][11] Bonderman et al described the short-term efficacy and safety of bosentan in 16 inoperable CTEPH patients with a mean age of 70 years, a mean PAP of 52 mmHg, and an NYHA functional class of II in two, III in nine, and IV in the remaining five patients. After a followup of six months, they found a significant improvement in the NYHA functional class, an increase in the mean 6-MWT distance from 299 m to 391 m, and a significant decrease in the serum levels of NT-pro BNP.…”

Section: Patients Characteristicsmentioning

confidence: 99%

“…A right-sided heart catheterisation at three-month follow-up was performed and showed a significant decrease in the mean pulmonary artery pressure to 42 mmHg and pulmonary vascular resistance, and an increase in the cardiac index. 9 In an observational study by Hughes et al, 20 patients with inoperable CTEPH could be included. The NYHA classification was II in five, III in 14, and IV in one patient, and the mean PAP was 48 mmHg.…”

Section: Patients Characteristicsmentioning

confidence: 99%

“…7 In recent studies, bosentan has been demonstrated to improve function during short-term follow-up and may offer a therapeutic option for patients with inoperable CTEPH. [8][9][10] The beneficial effect during long-term follow-up is largely unknown. The aim of the present study was to evaluate the efficacy of bosentan during mid-and long-term follow-up in inoperable CTEPH.…”

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Long-term efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension

Post¹,

Plokker²,

Kelder³

et al. 2009

NHJL

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Background. Inoperable chronic thromboembolic pulmonary hypertension (CTEPH) is associated with a poor survival. Objectives. To evaluate the long-term response to a dual endothelin receptor antagonist in patients with inoperable CTEPH. Methods. All consecutive 18 patients (mean age 63±14 years) treated with bosentan for symptomatic inoperable CTEPH were included. Efficacy was evaluated by the log value of serum levels of N-terminal-pro brain natriuretic peptide (log NTpro BNP), New York Heart Association functional class (NYHA), and the six-minute walk test (6-MWT). All follow-up data (median 31 months) were compared with baseline and divided into: short-term (<12 months), mid-term (between 12 and 24 months), and long-term follow-up (>24 months). Results. At baseline, 15 patients were in NYHA class III and three in NYHA class IV, mean log NT-pro BNP level was 7.2±1.4 log pg/ml, and mean 6-MWT distance was 404±125 m. During short-term follow-up (n=18), the NYHA class improved (p=0.001), 6-MWT distance increased by 33 m (p=0.03), and log NT-pro BNP decreased to 6.9±1.4 log pg/ml (p=0.007). During mid-term follow-up (n=17), the NYHA class improved (p<0.001), the mean 6-MWT distance increased by 41 m (p=0.01), and log NT-pro BNP was 6.9±1.4 log pg/ml (p=0.31). During late followup (n=14) the NYHA class was still improved (p=0.03), the 6-MWT distance decreased by 9 m (p=0.73), and log NT-pro BNP was 7.1±1.5 log pg/ml (p=0.91). The overall four year survival rate was 88%. Conclusion: Bosentan seems to be effective during long-term treatment in patients with inoperable CTEPH. (Neth Heart J 2009;17:329-33).

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Classification of Pulmonary Hypertension: History and Perspectives

Badesch

2010

Textbook of Pulmonary Vascular Disease

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Bosentan Therapy for Inoperable Chronic Thromboembolic Pulmonary Hypertension

Cited by 154 publications

References 17 publications

Survival and Quality of Life for Patients With Peripheral Type Chronic Thromboembolic Pulmonary Hypertension

Survival and Quality of Life for Patients With Peripheral Type Chronic Thromboembolic Pulmonary Hypertension

Long-term efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension

Classification of Pulmonary Hypertension: History and Perspectives

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