Preview

Eurasian heart journal

Advanced search

CASE REPORT: THE POSSIBILITY OF SPECIFIC THERAPY OPTIMIZATION BY SWITCHING FROM BOSENTAN TO MACITENTAN IN A PATIENT WITH CONGENITAL HEART DISEASE AND EISENMENGER SYNDROME

https://doi.org/10.38109/2225-1685-2018-1-72-81

Abstract

A patient with pulmonary arterial hypertension (PAH) associated with congenital heart disease (ventricular septal defect) and Eisenmenger syndrome was started on an endothelin receptor antagonist bosentan. With treatment, the patient's condition had been stable, however, by the second year, worsening shortness of breath, reduced exercise tolerance and an increase of the right heart were noted. Bosentan was switched to macitentan. After a year of this pathogenetic therapy, an improvement in functional status was registered. Moreover, while on macitentan, the patient has successfully undergone a gynecological surgical procedure under general anesthesia.

About the Authors

S. Ye. Gratsianakaya
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation

Postgraduate student of the department of pulmonary hypertension and heart disease 

121552, Moscow, 3rd Street Cherepkovskaya, 15a

tel. 8-495-414-68-42



O. A. Arkhipova
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation

PhD, Researcher of the department of pulmonary hypertension and heart disease o

121552, Moscow, 3rd Street Cherepkovskaya, 15a.



A. I. Davydov
I.M.Sechenov First Moscow State Medical University
Russian Federation

MD, PhD, DSc, professor of medicine at the chair of obstetrics, gynaecology and perinatology

8/2, ul. Trubetskaya, Moscow, 119991, Russian Federation

phone: 8 (499) 782-3341



T. V. Martynyuk
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation

MD, leading researcher, Head of the department of pulmonary hypertension and heart disease

121552, Moscow, 3rd Street Cherepkovskaya, 15a



N. A. Ananicheva
S.S. Yudin City Clinical Hospital, Moscow Department of Health
Russian Federation

MD, high category cardiologist, head of the cardiological department 

Kolomenskii proezd, 4, Moscow, 115446, Russian Federation

8 (499) 782-30-51



O. S. Belkorey
City Polyclinic No 218
Russian Federation

MD, 1st category physician, cardiologist a

8, pr-d Shokal'skogo, Moscow, 127642, Russian Federation

8 (499) 790-32-68



I. Ye. Chazova
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation

Academician of Russian Academy of Science, MD, Director 

121552, Moscow, 3rd Street Cherepkovskaya, 15a



References

1. Lowe B.S., Therrien J., Ionescu-Ittu R., et al. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J. Am. Coll. Cardiol.2011; 58: 538–546. doi: 10.1016/j.jacc.2011.03.033.

2. Сhazova I.Ye., Martynyuk T.V., Nakonechnikov S.N. According to the results of the European congress of cardiology 2015: new version of guidelines for diagnosis and treatment of pulmonary hypertension. Eurasian heart journal, 2015; 4: 3-10 [in Russian].

3. Сhazova I.Ye., Martynyuk T.V. Pulmonary hypertension. M: Practice, 2015 [in Russian].

4. Beghetti M., Galiè N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Mar 3; 53(9):733-40. doi: 10.1016/j.jacc.2008.11.025.

5. Galiè N., Beghetti M., Gatzoulis M.A. et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, doubleblind, randomized, placebo-controlled study. Circulation. 2006; 114 (1): 48-54.

6. Wagner O.F., Christ G., Wojta J. et al. Polar secretion of endothelin-1 by cultured endothelial cells. J Biol Chem 1992; 267:16066–16068.

7. T.V. Martynyuk, S.N. Nakonechnikov, I.Ye. Chazova. Optimization of specific therapy for pulmonary arterial hypertension: the possibilities of using endothelin receptor antagonists. Eurasian heart journal, 2017; 2: 20-27 [in Russian].

8. Blok I.M., Riel A., A. van Dijk, et al. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement? Intern J Cardiol 2017; 227: 51-52.

9. Herbert, S. et al. Early Experience of Macitentan for Pulmonary Arterial Hypertension in Adult Congenital Heart Disease. Heart, Lung and Circulation, Volume 26, Issue 10, 1113 – 1116.


Review

For citations:


Gratsianakaya S.Ye., Arkhipova O.A., Davydov A.I., Martynyuk T.V., Ananicheva N.A., Belkorey O.S., Chazova I.Ye. CASE REPORT: THE POSSIBILITY OF SPECIFIC THERAPY OPTIMIZATION BY SWITCHING FROM BOSENTAN TO MACITENTAN IN A PATIENT WITH CONGENITAL HEART DISEASE AND EISENMENGER SYNDROME. Eurasian heart journal. 2018;(1):72-81. https://doi.org/10.38109/2225-1685-2018-1-72-81

Views: 373


ISSN 2225-1685 (Print)
ISSN 2305-0748 (Online)