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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 Results: We 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 (-9.9 ± 5.2% vs. -14.5 ± 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 = -0.48, 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., (© 2020 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Noncompaction cardiomyopathy is a rare congenital heart disorder characterized by an arrest of the myocardial compaction process. This results in the altered formation of coronary microvessels with a resulting decrease in myocardial perfusion. Transendocardial CD34 + cell transplantation has been shown to increase myocardial perfusion and function in patients with non-ischemic heart failure. In our first-in-man case study, we investigated the feasibility, safety and clinical effect of transendocardial CD34 + cell therapy in a patient with noncompaction cardiomyopathy.

Introduction: End-diastolic volume (EDV) between 200 and 370 mL has been associated with favorable clinical response to cell therapy in ischemic heart failure. Hypothesis: We sought to investigate the effects of EDV, a marker of disease severity, on the response to cell therapy in patients with nonischemic dilated cardiomyopathy (DCM). Methods: Five-year registry data from 134 consecutive patients with DCM (LVEF<40%, NHYA III) who underwent CD34+ cell transplantation was analyzed. All patients received granulocyte-colony stimulating factor (G-CSF; 10 mcg/kg, 5 days); CD34+ cells were collected by apheresis and delivered by transendocardial injection in areas of electromechanical missmatch. Patients with baseline EDV<200 mL were included in Group A (N=72), patients with EDV 200-370 mL were included in Group B (N=54); patients with EDV>370 mL were excluded (N=7). Favorable clinical response was defined by improved LVEF≥5% at 1 year post-cell therapy. Results: At baseline, Groups A and B did not differ in age (52±11 years in Group A vs. 53±10 years in Group B, P=0.95), sex (male: 79% vs. 83%, P=0.55), creatinine (1.07±0.28 mg/dL vs. 1.03±0.21 mg/dL, P=0.21), bilirubin (0.98±0.55 mg/dL vs. 1.00±0.51 mg/dL, P=0.58), or NTproBNP levels (1454±1658 pg/mL vs. 1589±1338 pg/mL, P=0.80). Baseline LVEF was higher in Group A (32.8±8.7%) than in Group B (30.2±8.7%, P=0.03). During follow-up, there were 4 deaths in Group A (6%), and 2 in Group B (4%, P=0.63); heart transplantation was performed in 4 patients from Group A (6%) and in 5 patients in Group B (9%, P=0.42). Favorable clinical response was found in 31/72 patients (43%) in Group A vs. 35/54 (65%) in Group B (P=0.016). Furthermore, EDV significantly decreased at 1 year in Group B (-56±30 mL, P=0.003), but not in Group A (+12±97 mL, P=0.13). On multivariate analysis, baseline EDV was an independent correlate of 1-year clinical outcome (P=0.02). Conclusions: EDV predicted clinical outcome following transendocardial CD34+ cell therapy in a nonischemic DCM patient population, informing target individuals with the highest likelihood of regenerative response. [ABSTRACT FROM AUTHOR]

Introduction: Noncompaction cardiomyopathy (NCC) is a congenital heart disease characterized by an arrest of the myocardial compaction process. Although NCC patients have impaired formation of microvasculature, the functional impact of these changes remains undefined. Hypothesis: We sought to analyze a potential correlation between myocardial ischemia and heart failure progression in patients with NCC. Methods: We enrolled 20 (12 male, 8 female) patients with NCC, diagnosed by cardiac MRI. The average age was 46±15.3 years. Echocardiography was performed to determine left ventricular end diastolic diameter and volume (EDD and EDV), ejection fraction (LVEF), with global longitudinal strain (GLS) postprocessing. At patient inclusion we also measured serum levels of NT-proBNP. Myocardial SPECT was performed at rest and on stress, determining summed difference score (SDS). Significant myocardial ischemia was defined as SDS≥2. Results: Of 20 patients enrolled, 9 patients (45%) had evidence of myocardial ischemia (Group A), 11 patients (55%) showed no significant ischemic changes (Group B). The two groups did not differ in sex (male 67% in Group A vs. 55% in Group B, P=0.58), age (45±14,6 years vs. 46±14,6 years, P=0.92), creatinine (79±14 μmol/L vs. 74±12 μmol/L, P=0.48) or bilirubin (12±7 μmol/L vs. 11±3 μmol/L, P=0.51). When compared to Group B, Group A had significantly higher EDD (6.3±0.9 cm vs. 5.3±0.6 cm, P=0.018), lower NTproBNP levels (1950±2004 pg/mL vs. 294±429 pg/mL in Group B, P=0.02) and a tendency toward lower LVEF (36.6±16.7% vs. 51.8±16.8% in Group B, P=0.06), higher EDV (179.2±53.9 mL vs. 138.7±47.4 mL, P=0.09), and lower GLS (-11.4±7% vs. -15.4±4%, P=0.12). Overall, higher SDS was associated with lower LVEF (r=-0.46, P=0.04), higher EDV (r=0.53, P=0.02), higher levels of NT-proBNP (r=0,82, P<0.001), and a tendency toward lower GLS (r=0.40, P=0.06). Conclusions: In patients with NCC, the presence of myocardial ischemia is associated with worse left ventricular function, dilation of left ventricle and more pronounced neurohumoral activation. Further studies are needed to investigate whether treatment approaches targeting myocardial ischemia, such as CD34+ cell therapy, may halt the progression of disease in this patient cohort. [ABSTRACT FROM AUTHOR]