Eisenmenger syndrome presenting with chronic thromboembolic disease

Yıl 2024, Cilt: 10 Sayı: 4, 426 - 429, 04.07.2024

Ahmet Cemal Pazarlı , Kayıhan Karaman , Tuğba Yıldırım

https://doi.org/10.18621/eurj.1440680

Öz

Eisenmenger syndrome is characterized by the reversal of blood flow due to increased pulmonary vascular resistance. It can be prevented with early diagnosis and surgical treatment. Thromboembolism is a leading cause of death in patients with Eisenmenger syndrome. Pulmonary endarterectomy is the primary treatment, but medical treatments may be considered in inoperable cases. Regular follow-up and a multidisciplinary approach are important for diagnosis and treatment. Lifestyle changes and medical therapy can improve patient's quality of life and prevent complications. Our case is presented because of the chronic thromboembolic disease in addition to the pulmonary hypertension due to the partial atrioventricular septal defect and the management of the treatment.

Anahtar Kelimeler

Eisenmenger syndrome, pulmonary hypertension, chronic thromboembolic disease

Kaynakça

• 1. Basit H, Wallen TJ, Sergent BN. Eisenmenger Syndrome. [Updated 2023 Feb 13]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507800/
• 2. Diller GP, Lammers AE, Oechslin E. Treatment of adults with Eisenmenger syndrome-state of the art in the 21st century: a short overview. Cardiovasc Diagn Ther. 2021;11(4):1190-1199. doi: 10.21037/cdt-21-135.
• 3. Otani N, Watanabe R, Tomoe T, Toyoda S, Yasu T, Nakamoto T. Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension. Int J Mol Sci. 2023;24(4):3979. doi: 10.3390/ijms24043979.
• 4. Diller GP, Kempny A, Alonso-Gonzalez R, et al. Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre. Circulation. 2015;132(22):2118-2125. doi: 10.1161/CIRCULATIONAHA.115.017202.
• 5. Engelfriet P, Boersma E, Oechslin E, et al.The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease. Eur Heart J. 2005;26(21):2325-2333. doi: 10.1093/eurheartj/ehi396.
• 6. Broberg CS, Ujita M, Prasad S, et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007;50(7):634-642. doi: 10.1016/j.jacc.2007.04.056.
• 7. Silversides CK, Granton JT, Konen E, Hart MA, Webb GD, Therrien J. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol. 2003;42(11):1982-1987. doi: 10.1016/j.jacc.2003.07.022.
• 8. Hjortshøj CMS, Kempny A, Jensen AS, et al. Past and current cause-specific mortality in Eisenmenger syndrome. Eur Heart J. 2017;38(26):2060-2067. doi: 10.1093/eurheartj/ehx201.