Impacto da não compactação da musculatura do ventrículo esquerdo no prognóstico de pacientes com distrofia muscular de Duchenne
Japão – disfunção cardíaca é uma das principais causas de mortalidade na distrofia muscular de Duchenne; identificá-la e previní-la e uma das melhores maneiras de aumentar a sobrevida. Neste estudo prospectivo utilizando o ecocardiograma os autores constataram que as pessoas com Duchenne que apresentam não compactação do ventriculo esquerdo no ecocardiograma apresentam piora da função cardíaca mais precoce e maior mortalidade. Esta informação é relevante e deveria ser difundida entre neurologistas, cardiologistas e profissionais envolvidos com distrofia muscular de Duchenne.
O resumo em inglês pode ser lido abaixo:
(International Journal of Cardiology, 2013) Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy — Prospective multicenter
Koichi Kimura, Katsu Takenaka, Aya Ebihara, Kansei Uno, Hiroyuki Morita, Takashi Nakajima,Tetsuo Ozawa, Izumi Aida, Yosuke Yonemochi, Shinya Higuchi, Yasufumi Motoyoshi, Takashi Mikata, Idai Uchida, Tadayuki Ishihara, Tetsuo Komori, Ruriko Kitao, Tetsuya Nagata, Shin’ichi Takeda, Yutaka Yatomi, Ryozo Nagai, Issei Komuro – Japan
Background: The reported prevalence of left ventricular noncompaction (LVNC) varies widely and its prognostic impact remains controversial. We sought to clarify the prevalence and prognostic impact of LVNC in patients with Duchenne/Becker muscular dystrophy (DMD/BMD).
Methods:We evaluated the presence of LNVC in patients with DMD/BMD aged 4–64 years old at the study entry (from July 2007 to December 2008) and prospectively followed-up their subsequent courses (n=186). The study endpoint was all-cause death and the presence of LVNC was blinded until the end of the study (median follow-up: 46 months; interquartile range: 41–48 months).
Results: There were no significant differences in baseline characteristics between patients with LVNC (n=35) and control patientswithout LVNC (n=151),with the exception of LV function. Patients with LVNC showed, in comparison with patients without LVNC, a significant negative correlation between age and LVEF (R=−0.7 vs. R=−0.4) at baseline;and showed a significantly greater decrease in absolute LVEF (−8.6±4.6 vs. −4.3±4.5, pb0.001) during the follow-up. A worse prognosis was observed in patients with LVNC (13/35 died) than in patients without LVNC (22/151 died, Log-rank p<0.001).Multivariate Cox analysis revealed that LVNC is an independent prognostic factor (relative hazard 2.67 [95% CI: 1.19–5.96]).
Conclusion: LVNC was prevalent in patients with DMD/BMD.The presence of LVNC is significantly associated with a rapid deterioration in LV function and higher mortality. Neurologists and cardiologists should pay more careful attention to the presence of LVNC.
