Impairment of myocardial perfusion correlates with heart failure severity in patients with non‐compaction cardiomyopathy

Abstract

Aims Non-compaction cardiomyopathy (NCM) is a congenital heart disease characterized by an arrest of the myocardial compaction process. Although NCM patients have impaired formation of microvasculature, the functional impact of these changes remains undefined. We sought to analyse a potential correlation between myocardial ischemia and heart failure severity in NCM patients. Methods and resultsWe enrolled 41 NCM patients (28 male and 13 female), aged 21-70 years. In all patients, we have determined left ventricular end-diastolic volume (LVEDV), left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS) by echocardiography. At the same time, serum levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) have been measured, and myocardial single-photon emission computed tomography at rest and on stress was used to define significant myocardial ischemia defined as summed difference score ≥ 2. Myocardial ischemia has been demonstrated in 11 patients (27%, Group A), and 30 patients showed no significant ischemic changes (73%, Group B). The groups did not differ in sex, age, kidney, or liver function. When compared with Group B, Group A had significantly lower LVEF (35 ± 15% in Group A vs. 53 ± 11% in Group B, P < 0.001), higher LVEDV (188 ± 52 mL vs. 136 ± 52 mL, P = 0.007), lower GLS (À9.9 ± 5.2% vs. À14.5 ± 4.1%, P = 0.001), and higher NT-proBNP levels (1691 ± 1883 pg/mL vs. 422 ± 877 pg/mL, P = 0.006). Overall, higher summed difference score was associated with lower LVEF (r = À0.48, P = 0.001), higher LVEDV (r = 0.39, P = 0.012), lower GLS (r = 0.352, P = 0.024), and higher levels of NT-proBNP (r = 0.66, P < 0.001).Conclusions The presence of myocardial ischemia in patients with NCM is associated with worse left ventricular function, dilation of the left ventricle, and more pronounced neurohumoral activation.

NT‐pro‐BNP in patients with left ventricular hypertrabeculation/non‐compaction

“…It is already known that patients with heart failure and systolic dysfunction show elevated NT‐pro‐BNP levels 9 . For LVHT patients, the association of high NT‐pro‐BNP levels with heart failure, valvular abnormalities, and electrocardiographic abnormalities has already been reported 11,13,14,19 . The present study substantiates these findings by showing a difference between the groups of all three classifications regarding LVEDD and systolic function.…”

“…14 In Slovenian LVHT patients, those with an impairment of myocardial perfusion, assessed by technetium-99m tetrofosmin, had higher NT-pro-BNP than those without perfusion abnormalities. 19 In our study, LVHT patients with one affected segment had lower mean NT-pro-BNP levels than patients with three affected segments. A correlation between high NT-pro-BNP levels and a higher number of trabeculations has been described by Tizón-Marcos et al in apparently healthy patients.…”

“…In Chinese LVHT patients with coronary artery disease, NT‐pro‐BNP levels were higher than in patients without 14 . In Slovenian LVHT patients, those with an impairment of myocardial perfusion, assessed by technetium‐99m tetrofosmin, had higher NT‐pro‐BNP than those without perfusion abnormalities 19 …”

Myocardial Mechanics and Associated Valvular and Vascular Abnormalities in Left Ventricular Noncompaction Cardiomyopathy

“…A total of 33% of patients proved to be respondent, with a reduction in inter-ventricular, longitudinal, and radial intra-ventricular dyssynchrony [ 48 ]. The presence of myocardial ischemia in NCCM patients is associated with LV dilation and worse LV function represented by LV-EF and global LV-LS [ 49 ].…”