Key result
Conventional HF therapy and NIPPV stabilized DCM in a nemaline myopathy patient for 10 months.
Why the study?
The complication of dilated cardiomyopathy in nemaline myopathy has rarely been reported, and its myocardial pathology is not well characterized.
Case Report (n=1)
No
This case report highlights the rare complication of dilated cardiomyopathy in nemaline myopathy and demonstrates successful management with conventional heart failure therapy and noninvasive ventilation.
Nemaline myopathy may involve cardiac muscle and cause dilated cardiomyopathy; this case leaves open whether screening is warranted in congenital myopathies.
Nemaline myopathy is a representative form of congenital myopathy, and is characterized by nemaline bodies in muscle fibers. Here we report a 47-year-old man with congenital nemaline myopathy complicated with dilated cardiomyopathy-related heart failure, and restrictive respiratory failure. The complication of dilated cardiomyopathy in nemaline myopathy has rarely been reported. In this case, nemaline bodies were detected in the cardiac muscle fibers, demonstrating the presence of underlying disease-related myocardial degeneration. The patient responded to the combination of conventional therapy for heart failure including β-blocker and noninvasive continuous positive-pressure ventilation for respiratory failure. His general condition has been stable during a 10-month follow up period.
No takes yet. Share an insight, caveat, or question.
Nagata et al. (2011) conducted a case report in Nemaline myopathy with dilated cardiomyopathy and respiratory failure (n=1). Conventional heart failure therapy and noninvasive continuous positive-pressure ventilation (NIPPV) was evaluated on Clinical stabilization and normalization of heart failure parameters. The patient with nemaline myopathy complicated by dilated cardiomyopathy and respiratory failure responded well to conventional heart failure therapy and noninvasive positive-pressure ventilation, remaining stable over a 10-month follow-up.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: