Abstract
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.
Highlights
аритмогенной дисплазии правого желудочка (АДПЖ) — аритмогенная дисплазия правого желудочка, ВСС — внезапная сердечная смерть, ГКС — глюкокортикостероиды, желудочковая экстрасисто‐ лия (ЖЭ) — желудочковая экстрасистолия, имплантирован кардиовертер-дефибриллятор (ИКД) — имплантируемый кардиовертер-дефибриллятор, левого желудочка (ЛЖ) — левый желудочек, ЛОТС — лист ожидания трансплантации сердца, механической поддержк и кровообращения (МПК) — механическая поддержка кровообращения, ПЖ — правый желудочек, ТС — трансплантация, фиб рилляция предсердий (ФП) — фибрилляция предсердий, хронической сердечной недостаточ‐ ности (ХСН) — хроническая сердечная недостаточность, электрокардио граммы (ЭКГ) — электрокардиография, Эндомиокардиальные биопсии (ЭМБ) — эндомиокардиальная биопсия, ЭхоКГ — эхокардиография
arrhythmogenic right ventricular dysplasia (ARVD) is a rare disease in the structure of end-stage heart failure in recipients in HTx waiting list (WL)
По результатам повтор‐ ных ЭМБ до 1 года (n=25): частота случаев R0 — 64% (n=16), R1 — 20% (n=5), R2 — 8% (n=2), AMR1 — 8% (n=2); после 1 года (n=14): R0 — 71,4% (n=10), R1 — 14,3% (n=2), AMR1 и AMR2 — 14,3% (n=2)
Summary
Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list. A. 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). 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 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). Среди пациентов с АДПЖ чаще встречаются мужчины, у которых симптоматика в виде нарушений ритма преобладает над проявлениями правожелудоч‐ ковой сердечной недостаточности (СН) [4]. Высоко сенсибилизирован‐ ными считались пациенты с PRA >10% и/или MFI >5000; Критерии диагностики АДПЖ, выявленные у исследованных пациенток
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