MITRAL AND TRICUSPID VALVULOPLASTY IN A PATIENT WITH MYEL-ODYSPLASTIC SYNDROME

Autori:

Delfa Radić-Krišto, Inga Mandac Rogulj, Biljana Jelić-Puškarić, Helena Jerkić, Gordana Kaić, Anita Škrtić, Viktor Zatezalo

Sažetak
Mijelodisplastični sindrom (MDS) klonska je bolest matične hematopoetske stanice, koja se očituje poremećenom proliferacijom, diferencijacijom i sazrijevanjem hematopoeze te displastičnim promjenama u koštanoj srži. U kliničkoj slici i laboratorijskim nalazima bolesnika s MDS-om dominira anemija, nešto rjeđe neutropenija ili trombocitopenija različitih stupnjeva težine. Kardiokirurški zahvat u bolesnika s MDS-om velik je izazov jer su neutropenija i trombocitopenija vodeći uzroci infekcija i krvarenja tijekom operacije. Malen je broj izvješća u literaturi koja opisuju kardiokirurški zahvat u bolesnika s MDS-om. Prikazujemo 66-godišnju bolesnicu s MDS-om kod koje je uspješno izvedena rekonstrukcija mitralnog i trikuspidalnoga srčanog zalistka. Bolesnica se javila u hitnu službu zbog otežanog disanja, opće slabosti i znakova srčanog popuštanja. Radiološkom obradom verificirani su obostrani pleuralni izljev, izraženije lijevo, i povećana sjena srca s naglašenim vaskularnim hilusima. Daljnjom obradom dokazane su mitralna i trikuspidalna insuficijencija teškog stupnja pa je bolesnica operirana, a pet godina nakon zahvata ima dobru kvalitetu života. Multidisciplinarnom suradnjom kardiologa, hematologa, anesteziologa i kardiokirurga u bolesnice je uspješno izveden kirurški zahvat rekonstrukcije mitralne i trikuspidalne valvule. Ovaj prikaz bolesnice upućuje na važnost multidisciplinarnog pristupa specifičnoj i ranjivoj grupi bolesnika s MDS-om i kardiovaskularnim komorbiditetima.
Summary

Myelodysplastic syndrome (MDS) is a clonal disease of mutated hematopoietic stem cells characterized by abnormal hematopoietic differentiation and dysplastic bone marrow changes. MDS usually tends to present with anemia, less often with neutropenia and thrombocytopenia of varying degrees. Cardiac surgery in MDS patients is a major challenge because neutropenia and thrombocytopenia are the leading causes of infection and bleeding during the procedure. There are only a few reports of cardiovascular surgery in patients with MDS. We are presenting a 66-year-old MDS patient with a successful reconstruction of the mitral and tricuspid heart valve. The patient presented in the Emergency department with weakness and dyspnea. Radiological workup verified 3rd stage heart failure. Severe mitral and tricuspid insufficiency was verified, after which the patient underwent cardiac surgery, and five years after the procedure has good quality of life. Through the multidisciplinary collaboration of cardiologists, hematologists and cardiac surgeons an operative reconstruction of mitral and tricuspid valve was successfully performed. This case indicates the need for multidisciplinary care for the specific and vulnerable group of MDS patients with cardiovascular comorbidities.