Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect

D′Alto, Michele; Vizza, Carmine Dario; Romeo, Emanuele; Badagliacca, Roberto; Santoro, Giuseppe; Poscia, Roberto; Sarubbi, Berardo; Mancone, Massimo; Argiento, Paola; Ferrante, Fabio; Russo, Maria Giovanna; Fedele, Francesco; Calabrò, Raffaele

doi:10.1136/hrt.2006.097360

Cited by 78 publications

(45 citation statements)

References 30 publications

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“…26 Although not significant, a trend towards improvement was seen in patients with Eisenmenger syndrome. This treatment effect in both studies is different from the observation by D'Alto et al and Diller et al 32,33 The discrepancy between the first two studies and the other two CHD-PAH trials may be due to a higher cardiac output with lower pulmonary vascular resistance of their population, possibly indicating less advanced disease stage or natural progression.…”

contrasting

confidence: 55%

“…Bosentan was reduced from 125 mg twice a day to 62.5 mg twice a day with a complete normalization of the aminotransferase level. 32 In the other two studies with a long-term follow-up no significant rises in liver transaminases were reported. 33,34 Furthermore, the induction of hepatic enzymes can make hormonal contraception unreliable.…”

Section: Liver Function Test Disturbancesmentioning

confidence: 99%

“…D' Alto et al described a 12 months safety and efficacy study in ES patients and showed a significant reduction of the pulmonary vascular resistance index (PVRi) and systemic vascular resistance index (SVRi) ratio. 32 This …”

Section: Long-term Follow-up and Survivalmentioning

confidence: 99%

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Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

Schuuring

Vis²,

Duffels³

et al. 2010

TCRM

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Pulmonary arterial hypertension (PAH) is a progressive disease with poor survival outcome. PAH is classified by the 2009 updated clinical classification of pulmonary hypertension and a major subgroup is PAH due to congenital heart disease (CHD) with systemic-to-pulmonary shunt. CHD-PAH is a result of systemic-to-pulmonary shunting and chronic increased flow that ultimately results in adaptations of pulmonary vasculature and endothelial dysfunction. The advanced stage is called Eisenmenger syndrome which forms a small percentage (1%) of all CHD patients. Therapies targeted on PAH symptoms are called primary therapy for PAH, but most CHD-PAH patients progress to advanced therapy which is directed at the PAH itself. In CHD-PAH, advanced therapies are extensively investigated for all three major pathways: endothelin-1 receptor antagonists such as bosentan, prostanoids such as epoprostenol and phosphodiesterase 5 inhibitors such as sildenafil. Endpoints in most trials were catheterization hemodynamics, World Health Organization functional class, six-minute walking distance and patient-focused outcomes, based on quality of life questionnaires and Borg dyspnea index. The BREATHE-5 and EARLY study were two important randomized controlled trials showing efficacy of bosentan at short follow-up. Moreover in patients with Eisenmenger syndrome, one recent survival retrospective study with majority of patients on bosentan showed strong survival benefit over conservative therapy. A diversity of prospective cohort and retrospective studies were performed but all with limited data, due to small numbers and heterogeneity of underlying CHD diagnoses. Further larger studies are needed to determine optimal treatment for adults with CHD-PAH. This review focuses on bosentan in CHD-PAH. In particular, we discuss outcome of various clinical trials and compare efficacy and safety of bosentan to other advanced therapies.

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contrasting

confidence: 55%

Section: Liver Function Test Disturbancesmentioning

confidence: 99%

See 1 more Smart Citation

Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

Schuuring

Vis²,

Duffels³

et al. 2010

TCRM

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“…[47][48][49][50] Long-term data suggest that improvements are maintained for as long as two years of treatment, without safety or tolerability issues 51,52 but other have shown that the effect may decrease with time. 53 The findings from these studies challenge the dogma that pulmonary vascular disease in patients with ES is not amenable to treatment.…”

Section: Prostacyclin and Prostacyclin Analoguesmentioning

confidence: 99%

Pulmonary Hypertension in Congenital Shunts

Beghetti¹,

Tissot

2010

Revista Española de Cardiología (English Edition)

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“…110 Long-term data have shown that the improvement on bosentan can be maintained without safety or tolerability issues. [111][112][113][114][115][116] A more recent randomized controlled trial of bosentan in patients with NYHA functional class II PAH included patients with PAH-CHD (n 5 32 of 185 individuals). At 6 months, PVR significantly decreased from baseline but 6MWT distance change did not reach statistical significance (mean treatment effect 19.1 m, P 5 .076).…”

Section: Specific Ph Therapeutic Optionsmentioning

confidence: 99%