Significance of CK-elevation in noncompaction with regard to cardiac and neuromuscular disease

Objectives This retrospective study aimed to find out how often CK is elevated in left-ventricular hypertrabeculation/noncompaction (LVHT), how often CK-elevation is due to neuromuscular disorders (NMDs), other cardiac disease, or causes other than cardiac or NMD, and how often CK-elevation is associated with troponin-T-positivity. Methods Electronic records from all LVHT-patients diagnosed between 1995–2005 were reviewed for CK, CK–MB, and troponin-T-values, frequency of CK-elevation, and causes of CK-elevation. Results Among 100 LVHT-patients, electronic records with laboratory data and final reports were available in 88. The highest CK-values ranged between 9-5547U/l. In 40 patients at least one CK-value was elevated (46%). Only 8 patients were troponin-T-positive. There was no significant difference of the clinical cardiologic, ECG, and echocardiographic parameters between patients with normal and elevated CK. Seventy of the 88 patients were seen by a neurologist. A NMD was diagnosed in 57 patients (81%). CK-elevation was attributed to NMD in 31 patients (79%), to causes other than cardiac or NMD in 6 patients (15%), and to cardiac causes in 4 patients (10%). Conclusions CK-elevation occurs in half of the LVHT-patients. CK-elevation in LVHT is most frequently attributable to NMDs but hardly to cardiac disease. CK-elevation in LVHT suggests NMD and prompts neurological investigations.