engleski

Mechanical circulatory support for life-threatened familial dilated cardiomyopathy in patient with lymphoma

Objective: Familial dilated cardiomyopathy (DCM) is heterogeneous disorder, appears in approximately 30% of cases. Several genes have been identified as cause of DCM, but most cases has unknown etiology. Mostly remain unrecognized for the long time and wide possibilities of treatment could be performed. Methods: Case report. Results: In 36-old patient, Non-Hodgkin’s lymphoma was diagnosed in 2008 (histological type FCC II, clinically II A) with medical history for familial DCM. Patient treated with 6 cycles of R- CHOP. At spring 2014, patient had relapse, received new six cycles of R-CHOP. In November 2014, patient hospitalized because of DCM (LVEF 20-25%, interventricular septum of 6.0 mm, LVIDd 60 mm). Patient admitted in Cardiovascular ICU (NYHA class IV, EuroSCORE I 24.28%) and treated with levosimendan (0.1 mcg/kg/min) and immediate V-A ECMO. Third day, patient intubated and mechanically ventilated, TEE has shown worsening, despite of mechanical and medication procedures (LVEF 10-15%, global left ventricular hypokinesia and dilatation of right ventricle). Patient became hemodynamically unstable on ECMO and norepinephrine. Four days later patient received BiVAD, and removing of ECMO. Procedure passed well, and became hemodynamically stable. Two weeks later, in patient BiVAD replaced with HeartWareIII. Intraoperative TEE showed acute RV failure and inotropic support with levosimendan and norepinephrine administrated for the next 24 hours and temporary RV support implanted. After 24 hours control TEE has shown improvement of RV function and levosimendan discontinued and RVAD removed. Hemodynamic parameters were in normal ranges, and patient transferred to the hospital ward ten days later. Five years later, patient received successful heart transplantation and still alive. Conclusions: Familial DCM can be asymptomatic for decades, but the hematological therapy for lymphomas can accelerate cardiomyopathy and worsen patient’s condition abruptly. As the heart transplantation is not the option during acute phase of disease, urgent implantation of V-A ECMO and VADs is for lifesaving.

mechanical circulatory support ; dilated cardiomyopathy ; lymphoma

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