The Experience of Pediatric Heart Transplantation on North-West of Russian Federation

Simonenko, Maria; Первунина, Т. М.; Федотов, П. А.; Сазонова, Ю. В.; Васичкина, Е. С.; Рубинчик, В. Е.; Березина, А. В.; Zverev, Dmitry; Баутин, А. Е.; Kostareva, Anna; Полякова, А. П.; Mitrofanova, Lubov; Ситникова, М. Ю.; Николаев, Г. В.; Гордеев, М Л; Карпенко, М. А.

doi:10.15825/1995-1191-2018-2-37-46

Cited by 2 publications

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“…Группа пациентов с АДПЖ характеризовалась меньшей частотой применения МПК, более низким диастолическим давлением в легочной артерии и давлением заклинивания легочных капилляров по сравнению с пациентами (p<0,001) [5]. Согласно опыту нашего Центра, одной из четырех пациенток до проведения ТС потребовалась МПК [13].…”

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“…Декомпенсация ХСН, потребовавшая инотропной поддержки и перевода в отделение реанимации, была у пациентки № 1; далее в течение 3 нед. ей проводили экстракорпоральную мембранную оксигенацию с последующей имплантацией бивентрикулярной системы экстракорпоральной механической поддерж ки кровообращения (МПК) Berlin Heart "EXCOR" [13], которая продолжалась 250 сут., вплоть до ТС. Инфекционные осложнения (пневмония и гайморит) развились только у этой больной.…”

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Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list

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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.

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Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list

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Features of the clinical picture and outcomes in patients from a heart transplantation waiting list with different causes of heart failure

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Aim To identify clinical differences between patients on the heart transplant waiting list (HTWL) in the origin of chronic heart failure (CHF).Materials and methods From January 2010 through September 2019, 235 patients (age, 47+13 years (from 10 to 67 years); men, 79% (n=186)) were included in the HTWL. The patients were divided into two groups; group 1 (n=104, 44 %) consisted of patients with ischemic heart disease (IHD); group 2 (n=131, 56 %) included patients with noncoronarogenic CHF. Clinical and instrumental data and frequency of the mechanical circulatory support (MCS) as a “bridge” to heart transplantation (HT) were retrospectively evaluated.Results Group 1 included more male patients than group 2 [97 % (n=101) and 82 % (n=85), р<0.0001]; patients were older (54±8 and 42±14 years, р=0.0001). On inclusion into the HTWL, the CHF functional class was comparable in the groups, III [III;IV]; there were more patients of the UNOS 2 class in group 1 than in group 2 [75 % (n=78) and 57 % (n=75), р=0.005]. Patient distribution in UNOS 1B and 1A classes was comparable in the groups: 21% (n=22) and 3% (n=4) in group 1 and 33 % (n=43) and 10 % (n=13) in group 2. According to echocardiography patients of group 1 compared to group 2 showed a tendency towards higher values of left ventricular ejection fraction (Simpson method) [22 [18;26] % and 19 [15;24] %, р=0.37] and stroke volume [59 [44;72] % and 50 [36;67] %, р=0.07]. Numbers of patients with a cardioverter defibrillator or a cardiac resynchronization device with a defibrillator function were comparable in the groups [35 % (n=36) and 34 % (n=45)]. Comparison of comorbidities in groups 1 and 2 showed higher incidences of pulmonary hypertension [55 % (n=57) and 36 % (n=47), р=0.005], obesity [20 % (n=21) and 10 % (n=13), р=0.03], and type 2 diabetes mellitus [29 % (n=30) and 10 % (n=13), р=0.0004]. Rates of chronic obstructive lung disease, stroke, chronic kidney disease and other diseases were comparable. Duration of staying on the HTWL was comparable (104 [34; 179] and 108 [37; 229] days). During staying on the HTWL, patients of group 1 less frequently required MCS implantation [3 % (n=3) and 28 % (n=21), р=0.0009]. HT was performed for 59 % patients (n=61) in group 2 and 52 % (n=69) patients in group 2. Death rate in the HTWL was lower in group 1 [13 % (n=14) and 27 % (n=35), р<0.01].Conclusion On inclusion into the HTWL, patients with noncoronarogenic CHF had more pronounced CHF manifestations and a more severe UNOS class but fewer comorbidities than patients with CHF of ischemic origin. With a comparable duration of waiting for HT, patients with noncoronarogenic CHD more frequently required MCS implantation and had a higher death rate.

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The Experience of Pediatric Heart Transplantation on North-West of Russian Federation

Cited by 2 publications

References 16 publications

Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list

Arrhythmogenic right ventricular cardiomyopathy in recipients on heart transplant waiting list

Features of the clinical picture and outcomes in patients from a heart transplantation waiting list with different causes of heart failure

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