Serial evaluation of bosentan monotherapy in a patient with Eisenmenger syndrome during an 11-year period: A case follow-up report
Year 2022,
Volume: 3 Issue: 3, 117 - 119, 30.09.2022
Gökay Taylan
,
Yekta Gürlertop
,
Kenan Yalta
Abstract
Eisenmenger syndrome is a rare disease primarily characterized by severe degrees of irreversible pulmonary arterial hypertension in the setting of congenital heart disease. In this report, we aim to report, on a yearly basis, the clinical, hemodynamic and echocardiographic parameters of a female patient with Eisenmenger syndrome who had been under bosentan treatment during an 11-year follow-up.
References
- 1. Van Loon RLE, Hoendermis ES, Duffels MG, et al. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?. Am Heart J 2007; 154: 776-82.
- 2. Beghetti M, Galiè N. Eisenmenger syndrome. J Am Coll Cardiol 2009; 53: 733–40.
- 3. Kaya MG, Lam YY, Erer B, et al. Long-term effect of bosentan therapy on cardiac function and symptomatic benefits in adult patients with Eisenmenger syndrome. J Card Fail 2012; 18: 379-84.
- 4. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Pediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37: 67-119.
- 5. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Pediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD). Eur Heart J 2021; 42: 563-645.
- 6. Hascoet S, Fournier E, Jaïs X, et al. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicenter study. Arch Cardiovasc Dis 2017 110: 303-16.
- 7. LI Q, Kuang HY, Wu YH, et al. What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome: A systematic review and meta-analysis. Medicine 2019, 98(20): e15632.
Eisenmenger sendromlu bir hastada 11 yıllık dönemde bosentan monoterapisinin seri değerlendirmesi: Olgu takip raporu
Year 2022,
Volume: 3 Issue: 3, 117 - 119, 30.09.2022
Gökay Taylan
,
Yekta Gürlertop
,
Kenan Yalta
Abstract
Eisenmenger sendromu, konjenital kalp hastalığı ortamında öncelikle geri dönüşümsüz pulmoner arteriyel hipertansiyon ile karakterize nadir bir hastalıktır. Bu raporda, 11 yıllık bir takip sırasında Bosentan tedavisi altında olan Eisenmenger sendromlu bir kadın hastanın yıllık olarak klinik, hemodinamik ve ekokardiyografik parametrelerini bildirmeyi amaçlıyoruz.
References
- 1. Van Loon RLE, Hoendermis ES, Duffels MG, et al. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?. Am Heart J 2007; 154: 776-82.
- 2. Beghetti M, Galiè N. Eisenmenger syndrome. J Am Coll Cardiol 2009; 53: 733–40.
- 3. Kaya MG, Lam YY, Erer B, et al. Long-term effect of bosentan therapy on cardiac function and symptomatic benefits in adult patients with Eisenmenger syndrome. J Card Fail 2012; 18: 379-84.
- 4. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Pediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37: 67-119.
- 5. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Pediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD). Eur Heart J 2021; 42: 563-645.
- 6. Hascoet S, Fournier E, Jaïs X, et al. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicenter study. Arch Cardiovasc Dis 2017 110: 303-16.
- 7. LI Q, Kuang HY, Wu YH, et al. What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome: A systematic review and meta-analysis. Medicine 2019, 98(20): e15632.