Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy — Prospective multicenter cohort study

• Published in: International journal of cardiology 2013-10, Vol.168 (3), p.1900-1904
• Main Authors: Kimura, Koichi, Takenaka, Katsu, Ebihara, Aya, Uno, Kansei, Morita, Hiroyuki, Nakajima, Takashi, Ozawa, Tetsuo, Aida, Izumi, Yonemochi, Yosuke, Higuchi, Shinya, Motoyoshi, Yasufumi, Mikata, Takashi, Uchida, Idai, Ishihara, Tadayuki, Komori, Tetsuo, Kitao, Ruriko, Nagata, Tetsuya, Takeda, Shin'ichi, Yatomi, Yutaka, Nagai, Ryozo, Komuro, Issei
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Cardiology. Vascular system; Cardiomyopathy; Cardiovascular; Child; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Diseases of striated muscles. Neuromuscular diseases; Female; Follow-Up Studies; Heart; Heart Ventricles - diagnostic imaging; Humans; Isolated Noncompaction of the Ventricular Myocardium - complications; Isolated Noncompaction of the Ventricular Myocardium - diagnosis; Isolated Noncompaction of the Ventricular Myocardium - epidemiology; Japan - epidemiology; Left ventricular noncompaction; Male; Medical sciences; Middle Aged; Muscular dystrophy; Muscular Dystrophy, Duchenne - complications; Myocarditis. Cardiomyopathies; Neurology; Neuromuscular disease; Prevalence; Prognosis; Prospective Studies; Risk Factors; Survival Rate - trends; Ultrasonography; Young Adult
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2012.12.058

Abstract 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 patients without 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, p < 0.001) 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.