2016-01-19
Elevated Plasma Cardiac Troponin T Levels Caused by Skeletal Muscle Damage in Pompe Disease
Publication
Publication
Circulation: Cardiovascular Genetics , Volume 9 - Issue 1 p. 6- 13
Background—Elevated plasma cardiac troponin T (cTnT) levels in patients with neuromuscular disorders may erroneously
lead to the diagnosis of acute myocardial infarction or myocardial injury.
Methods and Results—In 122 patients with Pompe disease, the relationship between cTnT, cardiac troponin I, creatine
kinase (CK), CK-myocardial band levels, and skeletal muscle damage was assessed. ECG and echocardiography were
used to evaluate possible cardiac disease. Patients were divided into classic infantile, childhood-onset, and adult-onset
patients. cTnT levels were elevated in 82% of patients (median 27 ng/L, normal values <14 ng/L). Cardiac troponin I
levels were normal in all patients, whereas CK-myocardial band levels were increased in 59% of patients. cTnT levels
correlated with CK levels in all 3 subgroups (P<0.001). None of the abnormal ECGs recorded in 21 patients were
indicative of acute myocardial infarction, and there were no differences in cTnT levels between patients with and without
(n=90) abnormalities on ECG (median 28 ng/L in both groups). The median left ventricular mass index measured with
echocardiography was normal in all the 3 subgroups. cTnT mRNA expression in skeletal muscle was not detectable in
controls but was strongly induced in patients with Pompe disease. cTnT protein was identified by mass spectrometry in
patient-derived skeletal muscle tissue.
Conclusions—Elevated plasma cTnT levels in patients with Pompe disease are associated with skeletal muscle damage,
rather than acute myocardial injury. Increased cTnT levels in Pompe disease and likely other neuromuscular disorders
should be interpreted with caution to avoid unnecessary cardiac interventions.
Additional Metadata | |
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doi.org/10.1161/CIRCGENETICS.115.001322, hdl.handle.net/1765/91636 | |
Circulation: Cardiovascular Genetics | |
Organisation | Proteomics Center |
Wens, S., Schaaf, G. J., Michels, M., Kruijshaar, M., Van Gestel, T. J. M., in 't Groen, S., … Pijnappel, P. (2016). Elevated Plasma Cardiac Troponin T Levels Caused by Skeletal Muscle Damage in Pompe Disease. Circulation: Cardiovascular Genetics, 9(1), 6–13. doi:10.1161/CIRCGENETICS.115.001322 |