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Abstract 15737: Impairment of Myocardial Perfusion Correlates With Heart Failure Progression in Patients With Noncompaction Cardiomyopathy

IntroductionNoncompaction 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.HypothesisWe sought to analyze a p...

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Published in:Circulation (New York, N.Y.) N.Y.), 2018-11, Vol.138 (Suppl_1 Suppl 1), p.A15737-A15737
Main Authors: Cerar, Andraz, Jaklic, Martina, Frljak, Sabina, Dolenc Novak, Maja, Guzic Salobir, Barbara, Zbacnik, Rok, Kozelj, Mirta, Vrtovec, Bojan
Format: Article
Language:English
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Summary:IntroductionNoncompaction 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.HypothesisWe sought to analyze a potential correlation between myocardial ischemia and heart failure progression in patients with NCC.MethodsWe 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.ResultsOf 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
ISSN:0009-7322
1524-4539