Overlapping Phenotypes and Degree of Ventricular Dilatation Are Associated with Severity of Systolic Impairment and Late Gadolinium Enhancement in Non-Ischemic Cardiomyopathies

Abstract

BackgroundDilatation and other infrastructural rearrangements of the left ventricle are connected with poor prognosis. The aim of our study was to analyze the overlapping phenotypes and dilatation of the ventricle on impairment of systolic function and existence of late gadolinium enhancement (LGE).Material/MethodsConsecutive sample of cases with dilated left ventricle due to non-ischemic cardiomyopathy and healthy controls were included from our cardiac magnetic resonance imaging (CMR) database for a period of 3 years (n=1551 exams).ResultsThe study included 127 patients; 30 (23.6%) with dilated cardiomyopathy (DCM); 30 (23.6%) with left ventricular non-compaction (LVNC); 13 (10.2%) with hypertrophic cardiomyopathy (HCM), and 50 (39.4%) controls. Overlapping phenotypes were found in 48 (37.8%) of the studied cases. Odds for impairment of systolic function in connection with overlapping phenotypes were estimated at 7.8 (95%-CI: 3.4–17.6), (p<0.001). There were significant differences in geometric parameters for patients with overlapping phenotypes vs. controls, as follows: left ventricle end-diastolic dimension(LVEDD)=6.6±0.8 vs. 5.6±1.0 cm (p<0.001); left ventricular ejection fraction (LVEF)=39.3±14.0 vs. 52.1±16.1 (p<0.001); and existence of LGE 36 (75.0%) vs. 21 (26.6%), (p<0.001), respectively. Overlapping phenotypes correlated with LVEDD (Spearman’s-Rho-CC)=0.521, p<0.001; LVEF (Rho-CC)=−0.447, p<0.001 and LGE (Rho-CC)=0.472, p<0.001.ConclusionsThis study found there are many patients with overlapping phenotypes among NICMPs with dilated left ventricles. Overlapping phenotype was associated with greater LVEDD, lesser systolic function, and commonly existing LGE, which all impose increased cardiovascular risk. Linear midventricular LGE stripe was the most powerfully connected with loss of systolic function.

Evolution of diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy and their application in clinical practice

“…Для левожелудочковой формы предложено трактовать как большой критерий только субэпикардиальное или интрамиокардиальное LGE в ЛЖ. Тем не менее трактовать LGE в ЛЖ следует осторожно и с учетом клинического контекста, поскольку этот признак недостаточно специфичен и часто встречается не только при АКПЖ, но и при миокардите, дилатационной и гипертрофической кардиомиопатиях, НКМ ЛЖ, саркоидозе и амилоидозе [17][18][19][20]. Разнообразие причин LGE в полной мере показано в работе японских ученых, где данные МРТ сопоставлялись с результатами морфологического исследования миокарда [21].…”

PRDM16 Deletion Is Associated With Sex-dependent Cardiomyopathy and Cardiac Mortality: A Translational, Multi-Institutional Cohort Study

“…7 Of note, NCM can also lead to decreased systolic function and has considerable overlap with DCM. 8 Within the 1p36 region, several candidate cardiomyopathic genes have been suggested, which has made the pathogenesis of 1p36 deletion syndrome-associated cardiomyopathy difficult to evaluate and prognosticate. 1,3,9 PRDM16, which encodes a zinc-finger transcription factor and plays a role in negative TGF-β regulation, has been put forth as a candidate gene underlying the 1p36 deletion syndrome-associated cardiomyopathy.…”

“…7 Of note, NCM can also lead to decreased systolic function and has considerable overlap with DCM. 8 Within the 1p36 region, several candidate cardiomyopathic genes have been suggested, which has made the pathogenesis of 1p36 deletion syndrome-associated cardiomyopathy difficult to evaluate and prognosticate. 1,3,9…”

Ratio of end systolic volume over left atrial area is a respectable yardstick of systolic impairment in non-ischemic cardiomyopathies

“…Background: Impairment of systolic function and late gadolinium enhancement (LGE) are well known negative prognostic markers in non-ischemic cardiomyopathies (NICMPs). 1,2 The aim of our study was to analyze power of connection existing between individual volumetric parameters over left atrial area and systolic dysfunction or existence of LGE in patients with non-ischemic cardiomyopathy and healthy controls.…”