Transplantacija srca u djece u Hrvatskoj – naša prva iskustva [Heart transplantation in children in Croatia – our first experiences]

Malčić, Ivan and Anić, Darko and Bartoniček, Dorotea and Šarić, Dalibor and Kniewald, Hrvoje and Belina, Dražen and Novak, Milivoj and Galić, Slobodan and Cvitković, Miran and Ivančan, Višnja and Jelašić, Dražen and Stoić Brezak, Jasna (2018) Transplantacija srca u djece u Hrvatskoj – naša prva iskustva [Heart transplantation in children in Croatia – our first experiences]. Liječnički vjesnik, 140 (1-2). pp. 7-18. ISSN 0024-3477

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Abstract

Heart transplantation (HT) is an acceptable method of treating severe heart diseases in children when all other options have been exhausted. It is primarily intended for children suffering from cardiomyopathies, less often for children with severe arrhythmias and congenital heart defects. This method of treating children requires a multidisciplinary specialist cooperation and support in the child’s home surroundings. Goal: The main goal is to present the development of Croatian paediatric transplantation cardiology. The secondary goal is to raise awareness of heart transplantations in children in order to motivate organ donations, it being the most limiting factor of the method. Results of study: The first heart transplantation in a child in Croatia was performed in 2011. Transplantation preparations were performed in eleven children. Two of the children died while on the waiting list, one child was removed from the waiting list due to recovery, and in eight of the children a transplantation was performed. Two of the eight died, one during transplantation and one in the early post-transplantation period. All patients met the heart insufficiency criteria, four in stage C – status 2 and seven in stage D – status 1A and 1B. Of the initial eleven who met the HT criteria all had cardiomyopathies, eight dilated and three restrictive. In the dilated cardiomyopathy group of patients there were four with post-myocarditis DCM, one idiopathic, one within the arrhythmogenic CM, one within an entity form called the Carvajal syndrome (ARVCM/LV), while one child had DCM in HLHS, but as an NCCM. As for the two deceased before transplantation, one had post-myocarditis DCM, and one had Carvajal syndrome. An infant who died immediately after transplantation had hypoplastic left heart syndrome with previous Norwood I and Norwood II (Glenn or PCPC) surgeries, and the indication was severe DCM caused by and within NCCM.The death occurred because of hyperacute cellular rejection (PhD). A fifteen-year-old boy with arrhythmogenic DCM died in early postsurgical period due to RV failure (possibly because of acute cellular rejection). He had previously been subjected to a long-term conventional treatment and electrotherapy. Sex distribution (m, f) within the entire group (6:5) and within the HT group (4:4) was even. Mean age in heart transplantations was 84.8 months (22-167 months), and mean donor waiting period was 53 days (7-147 days). Kaplan-Meier curves for a six-year period indicate a 75% survival rate. The study describes numerous post-transplantation complications, immunosuppressive therapy, patient monitoring protocol and quality of life. A significant increase in quality of life is found according to the NYHA criteria (shift from 3-4 to 1-2) for all patients. Conclusion: Our Clinic has been performing heart transplantations since 2011. The success depends on timely diagnosing and identifying the terminal stage of the disease which also meets the transplantation criteria (PVR<6Wj, TPG <15mmHg), on public awareness and on the ability of doctors to recognize potential donors. The six-year period survival rate of 75% testifies to the ability of our team to make such a complex medical step forward.

Abstract in Croatian

Transplantacija srca (TS) prihvatljiva je metoda liječenja teških srčanih bolesti i kod djece kada se iscrpe sve ostale mogućnosti. Namijenjena je primarno djeci s kardiomiopatijama, rjeđe s teškim aritmijama ili prirođenim srčanim grješkama. Liječenje tom metodom nalaže multidisciplinarnu specijalističku suradnju te potporu okoline u kojoj dijete živi. Cilj: Glavni je cilj prikazati razvoj pedijatrijske transplantacijske kardiologije u Hrvatskoj. Sporedni je cilj podizanje svijesti o transplantaciji srca u djece, radi motivacije za donaciju organa jer je to najveći ograničavajući čimbenik ove metode. Rezultati studije: Prva transplantacija srca u djeteta učinjena je u Hrvatskoj 2011. godine. Priprema za transplantaciju napravljena je za 11-ero djece. Dvoje je umrlo na listi čekanja, jedno je skinuto s liste čekanja zbog oporavka, a u osmero je učinjena transplantacija; dvoje od njih je umrlo, jedno dijete neposredno nakon transplantacije, a jedno u ranome posttransplantacijskom razdoblju. Svi su pacijenti zadovoljili kriterije srčane insuficijencije, četvero sa stadijem C – status 2 i sedmero sa stadijem D – status 1A i 1B. Od 11-ero koji su zadovoljili kriterije za TS svi su imali kardiomiopatiju, osmero dilatacijsku i troje restrikcijsku. U skupini bolesnika s dilatacijskom kardiomiopatijom nalaze se četiri s postmiokarditičnim DKM-om, jedan s idiopatskim, jedan u okviru aritmogenog KM-a, jedan u okviru entitetskog oblika koji se zove Carvajalov sindrom (ADVK/LV), dok je jedno dijete imalo DKM kod HLHS-a, ali kao NkKM. Od dvoje umrlih prije transplantacije jedno je dijete imalo postmiokarditični DKM, a jedno Carvajalov sindrom. Neposredno nakon transplantacije umrlo je dojenče sa sindromom hipoplastičnoga lijevog srca koje je prethodno imalo Norwoodove operacije I i II (Glennova operacija ili PCPC), a indikacija je bila teški DKM zbog i u okviru NkKM-a. PHD je pokazao hiperakutno odbacivanje. U ranome postoperativnom razdoblju umro je i 15-godišnji mladić s aritmogenim DKM-om, prethodno dugotrajno liječen konvencionalno i elektroterapijom, zbog zatajenja DV-a (možda zbog akutnoga staničnog odbacivanja). Podjednaka je zastupljenost prema spolu (ž : m) u cijeloj skupini (6 : 5) i u skupini transplantiranih (4 : 4). Srednja dob kod transplantacije srca bila je 84,8 mjeseci (22 – 167 mj.), a srednje vrijeme čekanja donora 53 dana (7 – 147 dana). Kaplan-Meierove krivulje za šestogodišnje razdoblje pokazuju 75%-tno preživljenje. U radu se opisuju brojne posttransplantacijske komplikacije, imunosupresivna terapija, protokol praćenja bolesnika i kvaliteta života. Prema kriterijima NYHA-e, nalazimo izrazito poboljšanje kvalitete života za sve bolesnike (pomak s 3 – 4 na 1 – 2). Zaključak: Transplantacija srca u našoj se Klinici provodi od 2011. godine. Uspješnost ovisi o pravodobnom postavljanju dijagnoze i prepoznavanju terminalnog stadija bolesti s još zadovoljenim uvjetima za transplantaciju (PVR < 6 Wj, TPG < 15 mmHg) te svijesti javnosti i liječnika u prepoznavanju potencijalnih donora. Preživljenje u šestogodišnjem razdoblju (75%) svjedoči o osposobljenosti našeg tima za tako složen medicinski iskorak.

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