Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre
Introduction: Ambrisentan is an oral selective endothelin receptor antagonist licensed for use in pulmonary arterial hypertension (PAH). There are few data on clinical use and long-term tolerability in a wider range of patients with pulmonary hypertension (PH). Methods: All patients treated with amb...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2014-06-01
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| Series: | Therapeutic Advances in Respiratory Disease |
| Online Access: | https://doi.org/10.1177/1753465814532304 |
| _version_ | 1828885792514113536 |
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| author | Robin Condliffe Charlie A. Elliot Judith Hurdman Ian Sabroe Catherine Billings David G. Kiely Neil Hamilton |
| author_facet | Robin Condliffe Charlie A. Elliot Judith Hurdman Ian Sabroe Catherine Billings David G. Kiely Neil Hamilton |
| author_sort | Robin Condliffe |
| collection | DOAJ |
| description | Introduction: Ambrisentan is an oral selective endothelin receptor antagonist licensed for use in pulmonary arterial hypertension (PAH). There are few data on clinical use and long-term tolerability in a wider range of patients with pulmonary hypertension (PH). Methods: All patients treated with ambrisentan over a 4-year period were identified. Baseline characteristics, liver function test (LFT) results and World Health Organization (WHO) functional class were retrieved from hospital databases. Results: 272 patients received ambrisentan between March 2009 and June 2013 (32% idiopathic PAH, 36% connective tissue disease PAH, 11% congenital heart disease PAH, 6% portopulmonary hypertension, 1% HIV PAH, 4% PH in association with lung disease, 8% chronic thromboembolic PH and 2% PH in association with sarcoidosis). 33.5% of patients received ambrisentan as monotherapy and 12% received ambrisentan as their initial PH therapy. 18% stopped treatment due to side effects and 12% stopped due to lack of efficacy. Oedema was the most common side effect leading to cessation of therapy, which occurred in 7% of patients. 57% of patients who discontinued ambrisentan due to side effects also discontinued other PAH therapies due to side effects previously or subsequently. Ambrisentan was discontinued in two (<1%) patients due to abnormal LFTs. The 3-year survival in congenital heart disease PAH, idiopathic PAH and systemic sclerosis-associated PAH was 80%, 62%, and 38%, respectively ( p = 0.003). Survival was superior in patients in whom WHO functional class improved in response to therapy. Conclusion: Ambrisentan is used as an initial therapy and as monotherapy in a minority of patients in a large UK PH referral centre. Discontinuation due to side effects, and especially oedema, was higher than reported in previous studies while discontinuation due to abnormal LFTs was very uncommon. A majority of patients who discontinued therapy due to side effects also previously or subsequently discontinued other PAH therapies. Improvement in WHO functional class was associated with superior survival. |
| first_indexed | 2024-12-13T11:33:05Z |
| format | Article |
| id | doaj.art-e2f4d57975764781989545c580ef6e5b |
| institution | Directory Open Access Journal |
| issn | 1753-4658 1753-4666 |
| language | English |
| last_indexed | 2024-12-13T11:33:05Z |
| publishDate | 2014-06-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Therapeutic Advances in Respiratory Disease |
| spelling | doaj.art-e2f4d57975764781989545c580ef6e5b2022-12-21T23:47:53ZengSAGE PublishingTherapeutic Advances in Respiratory Disease1753-46581753-46662014-06-01810.1177/1753465814532304Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centreRobin CondliffeCharlie A. ElliotJudith HurdmanIan SabroeCatherine BillingsDavid G. KielyNeil HamiltonIntroduction: Ambrisentan is an oral selective endothelin receptor antagonist licensed for use in pulmonary arterial hypertension (PAH). There are few data on clinical use and long-term tolerability in a wider range of patients with pulmonary hypertension (PH). Methods: All patients treated with ambrisentan over a 4-year period were identified. Baseline characteristics, liver function test (LFT) results and World Health Organization (WHO) functional class were retrieved from hospital databases. Results: 272 patients received ambrisentan between March 2009 and June 2013 (32% idiopathic PAH, 36% connective tissue disease PAH, 11% congenital heart disease PAH, 6% portopulmonary hypertension, 1% HIV PAH, 4% PH in association with lung disease, 8% chronic thromboembolic PH and 2% PH in association with sarcoidosis). 33.5% of patients received ambrisentan as monotherapy and 12% received ambrisentan as their initial PH therapy. 18% stopped treatment due to side effects and 12% stopped due to lack of efficacy. Oedema was the most common side effect leading to cessation of therapy, which occurred in 7% of patients. 57% of patients who discontinued ambrisentan due to side effects also discontinued other PAH therapies due to side effects previously or subsequently. Ambrisentan was discontinued in two (<1%) patients due to abnormal LFTs. The 3-year survival in congenital heart disease PAH, idiopathic PAH and systemic sclerosis-associated PAH was 80%, 62%, and 38%, respectively ( p = 0.003). Survival was superior in patients in whom WHO functional class improved in response to therapy. Conclusion: Ambrisentan is used as an initial therapy and as monotherapy in a minority of patients in a large UK PH referral centre. Discontinuation due to side effects, and especially oedema, was higher than reported in previous studies while discontinuation due to abnormal LFTs was very uncommon. A majority of patients who discontinued therapy due to side effects also previously or subsequently discontinued other PAH therapies. Improvement in WHO functional class was associated with superior survival.https://doi.org/10.1177/1753465814532304 |
| spellingShingle | Robin Condliffe Charlie A. Elliot Judith Hurdman Ian Sabroe Catherine Billings David G. Kiely Neil Hamilton Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre Therapeutic Advances in Respiratory Disease |
| title | Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre |
| title_full | Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre |
| title_fullStr | Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre |
| title_full_unstemmed | Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre |
| title_short | Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre |
| title_sort | ambrisentan therapy in pulmonary hypertension clinical use and tolerability in a referral centre |
| url | https://doi.org/10.1177/1753465814532304 |
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