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Background Ebstein anomaly (EA) is characterized by anomalous tricuspid valve resulting in the whole right heart's distorted anatomy. Heart failure is the most frequent complication in adults with EA. Recently more attention is also paid to the left ventricle. Some limited echocardiographic data suggest a markedly increased occurrence of the left ventricular hypertrabeculation, by some authors called a non-compacted myocardium. Currently, cardiac magnetic resonance (CMR) is a gold standard in the evaluation of hypertrabeculation. However, different diagnostic criteria and discrepancies in the study outcomes make the clinical interpretation of this phenomenon questionable and challenging. Purpose To determine the frequency of the left ventricular hypertrabeculation among adults with EA; to assess its association with the ventricular size and function and the markers of heart failure. Methods Study group consisted of 35 unoperated adults (mean age 41.9±14.8 years) with Ebstein anomaly. The study protocol included: a) cardiac magnetic resonance (CMR) with the assessment of the left ventricle (LV) and functional right ventricle (fRV), including ejection fraction (EF), end-diastolic, end-systolic, and stroke volumes indexed by body surface area (respectively: EDVind, ESVind, SVind), and presence of hypertrabeculation defined according to Jacquier (1) as trabeculation mass >20% of the LV mass; b) cardiopulmonary tests with the assessment of peak oxygen consumption (peak VO2, % of predicted peak VO2), ventilation/carbon dioxide slope (VE/VCO2 slope) c) brain natriuretic peptide (BNP). Results Left ventricular hypertrabeculation was present in 13 (37.1%) patients. We demonstrated no difference between patients with and without LV hypertrabeculation regarding: s Conclusions Left ventricular hypertrabeculation demonstrated by cardiac magnetic resonance is a phenomenon frequently occurring among adults with Ebstein anomaly. However, its presence is not associated with both ventricles' worse function nor with reduced exercise capacity. Therefore, the clinical relevance of left ventricular hypertrabeculation in this population remains questionable and requires further prospective studies. Funding Acknowledgement Type of funding sources: None.

Purpose: Sudden cardiac death (SCD) in adults with congenital heart diseases (CHD) is usually caused by ventricular arrhythmia (VA). Microvolt T-wave alternans (MTWA) proved its role in SCD risk stratification. Aim of study: Evaluation of spectral MTWA incidence in patients (P) with CHD and determination its association with VA and potential risk factors of SCD. Methods: Study group: 204P (93men), aged 33,8±11,4years with CHD (TGA, UVH, Ebstein's anomaly, ccTGA, Eisenmenger syndrome, DORV, CAT, unoperated and operated ToF) underwent MTWA, ECG, 24h Holter ECG, BNP assessment and cardiopulmonary exercise test. Control group: 45 adults age- and sex-matched. MTWA classification: normal: negative (-) and abnormal: positive (+) and indeterminate (ind). Due to similar prognostic significance tests classified as MTWA(+) and MTWA(ind) were combined into non-negative(non-) group. VA classification: malignant- sustained ventricular tachycardia (sVT), potentially malignant- non-sustained ventricular tachycardia (nsVT) and >10 premature ventricular complexes per hour (PVC/h). Results: 31P were MTWA(+), 53P MTWA(ind) and 120P MTWA(-). Due to excessive noise 25P were excluded. MTWA(non-) was more frequent in study group vs. controls (p=0,000001). The highest ratio of MTWA(non-) was in Eisenmenger syndrome (87,5%). Among patients with MTWA(non-) malignant and potentially malignant VA was more frequent compared to MTWA(-) (p=0,003; p=0,016, respectively). Male gender and pulmonary hypertension (PH) were more often in MTWA(non-) vs. MTWA(-) (p=0,0049; p=0,00007, respectively). MTWA(non-) compared to MTWA(-) had lower: blood saturation (p=0,000295), maximal oxygen consumption (peakVO2) (p=0,002) and VE/VCO2slope (p=0,012). MTWA(non-) vs. MTWA(-) had higher: BNP values (p=0,0306) and NYHA class (p=0,007). Factors increasing MTWA(non-) incidence are: in univariate logistic regression: PH (OR=24,29), sVT (OR=7,31), VA (OR=3,01), male gender (OR=2,61), NYHA>I (OR=2,06), worse VE/VCO2slope (OR=1,07) and peakVO2 fall (OR=0,92); in multivariate logistic regression: PH (OR=14,16), male gender (OR=10,2), worse VE/VCO2slope (OR=1,06) and peakVO2 fall (OR=0,89). Conclusions: In adults with CHD abnormal MTWA occurs more often than in healthy population. Probability of this phenomenon increases in patients with malignant ventricular arrhythmia and clinical findings possibly related to lethal arrhythmia including advanced heart failure and pulmonary hypertension. Incidence of abnormal MTWA is higher in men. MTWA might be of potential role in SCD risk stratification in adults with congenital heart diseases.