Abstract
Purpose Noncompaction cardiomyopathy (NCC) results as an arrest of the myocardial compaction process in the embryonic period, leading to the impaired formation of the microvasculature. Myocardial perfusion abnormalities have already been described, however, the functional impact in NCC patients remains undefined. We sought to analyze a potential correlation between myocardial ischemia and heart failure progression in patients with NCC. Methods We enrolled 37 (24 male, 13 female) patients with NCC, average age 47±15 years. Echocardiography determined left ventricular end diastolic diameter and volume (EDD and EDV) and ejection fraction (LVEF). Global longitudinal strain (GLS) postprocessing has been performed. Serum levels of NT-proBNP have been measured. Cardiac MRI has been performed to confirm the diagnosis. Myocardial SPECT was performed at rest and on stress, determining summed difference score (SDS). Significant myocardial ischemia was defined as SDS≥2. Results Myocardial ischemia has been shown in 15 patients (41%, Group A), 22 patients (59%) showed no significant ischemic changes (Group B). The two groups did not differ in sex (male 73% vs. 59% in Group B), age (48±15 years vs. 46±14 years, P=0.65), creatinine (76±14 μmol/L vs. 76±16 μmol/L, P=0.98) or bilirubin (11±6 μmol/L vs. 12±5 μmol/L, P=0.46). When compared to Group B, Group A had significantly lower LVEF (38±15% vs. 55±13% in Group B, P=0.001), higher EDD (6.3±0.9 cm vs. 5.2±0.7 cm, P Conclusion In patients with NCC myocardial ischemia correlates with lower ejection fraction and dilation of the left ventricle, leading to greater neurohumoral activation. As medical heart failure therapy has not been shown to be successful in NCC patients, progressive LV systolic dysfunction due to myocardial ischemia often warrants heart transplantation or left ventricular assist device implantation. Treatment approaches targeting myocardial ischemia, such as CD34+ cell therapy, could slow the progression of disease in NCC patients.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
