Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list
Aim. To determine the clinical features of arrhythmogenic right ventricular dysplasia (ARVD) in recipients on heart transplant waiting list (WL) and after a heart transplantation (HTx).Material and methods. From January 2010 to December 2018, we included 192 recipients in heart transplant waiting li...
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«FIRMA «SILICEA» LLC
2020-08-01
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| Series: | Российский кардиологический журнал |
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| Online Access: | https://russjcardiol.elpub.ru/jour/article/view/3426 |
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| author | M. A. Simonenko P. A. Fedotov A. A. Kostareva Yu. V. Sazonova K. N. Malikov M. A. Bortsova A. P. Polyakova A. V. Berezina I. A. Zemskov T. M. Pervunina L. B. Mitrofanova G. V. Nikolaev M. L. Gordeev M. Yu. Sitnikova M. A. Karpenko |
| author_facet | M. A. Simonenko P. A. Fedotov A. A. Kostareva Yu. V. Sazonova K. N. Malikov M. A. Bortsova A. P. Polyakova A. V. Berezina I. A. Zemskov T. M. Pervunina L. B. Mitrofanova G. V. Nikolaev M. L. Gordeev M. Yu. Sitnikova M. A. Karpenko |
| author_sort | M. A. Simonenko |
| collection | DOAJ |
| description | Aim. To determine the clinical features of arrhythmogenic right ventricular dysplasia (ARVD) in recipients on heart transplant waiting list (WL) and after a heart transplantation (HTx).Material and methods. From January 2010 to December 2018, we included 192 recipients in heart transplant waiting list (HTx WL) on behalf of Almazov National Medical Research Center. ARVD was diagnosed in 4 subjects (F Marcus et al. criteria, 2010). All 4 patients (female, mean age 46,5 years-old (16-54-year-old)) underwent HTx. Prior to HTx, arrhythmias (atrial fibrillation, atrial flutter) were diagnosed in 3 recipients. In patient №2, pacemaker in VVI mode was implanted due to sick sinus syndrome (SSS) and tachycardia-bradycardia syndrome and others underwent ICD implantation.Results. Survival after HTx was 30,9 (3,9-46,2) months. All recipients were treated with triple-drug immunosuppressive therapy (calcineurin inhibitors, mycophenolic acid, steroids) and induction with Basiliximab. All patients experienced high sensitivity to immunosuppressive therapy (agranulocytosis), and therefore a colony-stimulating factor was administered to all of them. After immunosuppression reduction (Tacrolimus plus Methylprednisolone) agranulocytosis did not recur.Conclusion. ARVD is a rare disease in the structure of end-stage heart failure in recipients in HTx WL. An examination of this pathology is necessary to manage patients on-time with surgical treatment (ICD, HTx). According to our results, causal variants in desmosome genes were determined in 1 from 4 patients and simultaneous presence of two unique genetic variants in the RKR2 gene were found in one. A special feature of post-HTx management was the development of agranulocytosis, which once again underlines the need for a personalized approach to the selection of the immunosuppressive therapy. |
| format | Article |
| id | doaj-art-bb8f1258fccc4f14bc9757a5b94df92b |
| institution | DOAJ |
| issn | 1560-4071 2618-7620 |
| language | Russian |
| publishDate | 2020-08-01 |
| publisher | «FIRMA «SILICEA» LLC |
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| series | Российский кардиологический журнал |
| spelling | doaj-art-bb8f1258fccc4f14bc9757a5b94df92b2025-08-20T03:20:55Zrus«FIRMA «SILICEA» LLCРоссийский кардиологический журнал1560-40712618-76202020-08-0125710.15829/1560-4071-2020-34262792Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting listM. A. Simonenko0P. A. Fedotov1A. A. Kostareva2Yu. V. Sazonova3K. N. Malikov4M. A. Bortsova5A. P. Polyakova6A. V. Berezina7I. A. Zemskov8T. M. Pervunina9L. B. Mitrofanova10G. V. Nikolaev11M. L. Gordeev12M. Yu. Sitnikova13M. A. Karpenko14Federal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationFederal State Budgetary Institution «V.A. Almazov National Medical Research Centre» of the Ministry of Health of the Russian Federation, Saint-Petersburg, Russian FederationAim. To determine the clinical features of arrhythmogenic right ventricular dysplasia (ARVD) in recipients on heart transplant waiting list (WL) and after a heart transplantation (HTx).Material and methods. From January 2010 to December 2018, we included 192 recipients in heart transplant waiting list (HTx WL) on behalf of Almazov National Medical Research Center. ARVD was diagnosed in 4 subjects (F Marcus et al. criteria, 2010). All 4 patients (female, mean age 46,5 years-old (16-54-year-old)) underwent HTx. Prior to HTx, arrhythmias (atrial fibrillation, atrial flutter) were diagnosed in 3 recipients. In patient №2, pacemaker in VVI mode was implanted due to sick sinus syndrome (SSS) and tachycardia-bradycardia syndrome and others underwent ICD implantation.Results. Survival after HTx was 30,9 (3,9-46,2) months. All recipients were treated with triple-drug immunosuppressive therapy (calcineurin inhibitors, mycophenolic acid, steroids) and induction with Basiliximab. All patients experienced high sensitivity to immunosuppressive therapy (agranulocytosis), and therefore a colony-stimulating factor was administered to all of them. After immunosuppression reduction (Tacrolimus plus Methylprednisolone) agranulocytosis did not recur.Conclusion. ARVD is a rare disease in the structure of end-stage heart failure in recipients in HTx WL. An examination of this pathology is necessary to manage patients on-time with surgical treatment (ICD, HTx). According to our results, causal variants in desmosome genes were determined in 1 from 4 patients and simultaneous presence of two unique genetic variants in the RKR2 gene were found in one. A special feature of post-HTx management was the development of agranulocytosis, which once again underlines the need for a personalized approach to the selection of the immunosuppressive therapy.https://russjcardiol.elpub.ru/jour/article/view/3426arrhythmogenic right ventricular cardiomyopathyheart failureheart transplantation |
| spellingShingle | M. A. Simonenko P. A. Fedotov A. A. Kostareva Yu. V. Sazonova K. N. Malikov M. A. Bortsova A. P. Polyakova A. V. Berezina I. A. Zemskov T. M. Pervunina L. B. Mitrofanova G. V. Nikolaev M. L. Gordeev M. Yu. Sitnikova M. A. Karpenko Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list Российский кардиологический журнал arrhythmogenic right ventricular cardiomyopathy heart failure heart transplantation |
| title | Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list |
| title_full | Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list |
| title_fullStr | Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list |
| title_full_unstemmed | Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list |
| title_short | Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list |
| title_sort | arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list |
| topic | arrhythmogenic right ventricular cardiomyopathy heart failure heart transplantation |
| url | https://russjcardiol.elpub.ru/jour/article/view/3426 |
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