AIMS Non-compaction cardiomyopathy (NCCM) is associated with high rates of mortality and morbidity. Knowledge regarding risk stratification, arrhythmogenesis, therapy, and prognosis is limited. The aim of this study was to analyse the outcome of patients suffering from NCCM and ventricular arrhythmias (VAs) focusing on a treatment with implantable cardioverter-defibrillator (ICD) therapy and catheter ablation. METHODS AND RESULTS We conducted a multicentre observational study on 18 patients with NCCM, who underwent ICD implantation for secondary (n = 12) and primary (n = 6) prevention. In patients with multiple symptomatic episodes of VAs catheter ablation was performed. During a follow-up of 62 ± 42 months, 12 patients (67%) presented with appropriate ICD therapies [ventricular tachycardia (VT): n = 8; ventricular fibrillation (VF): n = 4; VT/VF: n = 3]. Ten patients underwent catheter ablation for VT/VF. Solely endocardial ablation was conducted in eight patients, and in two patients endo- and epicardial ablation was performed within the same procedure. Acute procedural success was achieved in 9/10 patients. Ventricular tachycardia recurrence was observed in two patients and the median arrhythmia free interval was 9.5 months (interquartile range 5.3-21 months). One patient underwent reablation, four patients died due to the underlying NCCM, and one patient received a left ventricular assist device. CONCLUSION Ventricular arrhythmias are common in patients suffering from NCCM and ICD therapy may be effective for primary and secondary prevention. In our cohort, consisting of patients with multiple VA episodes and recurrent ICD therapy, catheter ablation offered a safe and effective therapeutically option.

Abstract Background Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by an increased number of LV trabeculations with deep intertrabecular recesses. This abnormality is associated with heart failure, arrhythmias and arterial embolic events (AE). At present, it is unknown if AE is mainly due to blood stasis within the intertrabecular recesses, reduced LV ejection fraction or concomitant atrial fibrillation. LVNC is usually diagnosed by echocardiography but cardiac magnetic resonance imaging (CMRI) has evolved as an alternative method. This study assessed the prognostic value of CMRI for arterial embolic events in patients (pts) with LVNC. Methods 34 consecutive pts (19m, 15f, age 53±16) with LVNC underwent cine and contrast-enhanced CMRI with a 1.5 T scanner. LV diameter, volume, ejection fraction, and ratio of noncompacted to compacted myocardium (NC/C) were determined, and in 32 pts presence and localization of late gadolinium enhancement (LGE) was assessed. Clinical and CMRI findings were compared in pts with and without LV thrombus and/or AE. Results Overall, 20 pts (59%) were in heart failure NYHA III or IV, 14 (41%) had left bundle branch block (LBBB), 7 (21%) paroxysmal atrial fibrillation and 6 (19%) ventricular tachycardia (VT). By CMRI, LV diameter in end-diastole (66±8 mm), end-systole (53±10 mm), end-diastolic (229±69 ml) and end-systolic volume (150±68 ml) were enlarged and ejection fraction (36±14%) was reduced. The NC/C ratio was 3.2±1.4 in end-diastole and 2.6±1.4 in end-systole. One pt had right ventricular involvement with a thrombus. LGE was seen in 9/32 pts (28%) in the compacted myocardial layer (n=6), in the noncompacted trabecular layer (n=6) and within the papillary muscles (n=3). LGE was present in 3 areas in 1 and in 2 areas in 4 pts. In 3 pts (9%) a thrombus was seen within the trabecular layer which resolved under anticoagulation, and 6 additional pts (18%) without detectable thrombus experienced AE (transient ischemic attack n=1, stroke n=5). Thrombus and/or AE were not associated with age, sex, NYHA class, larger left atrial or LV diameter, LV volume, LBBB or documented VT. Atrial fibrillation (2/9 vs 5/25 pts, p=ns), LV ejection fraction (33±13% vs 38±15%, p=ns) and the NC/C ratio in end-diastole (median 3.2 vs 3) or end-systole (both median 2.6, p=ns) were similar. Thrombus and/or AE occurred mainly in pts with LGE (6/9 vs 2/23 pts, p=0.002). Conclusion In LVNC, evaluation by CMRI and demonstration of LGE in the compacted or noncompacted myocardium identifies patients at high risk for thrombus formation and/or arterial embolic events, warranting anticoagulation.

Left ventricular hypertrabeculation/noncompaction (LVHT) is diagnosed in all ages and is frequently associated with neuromuscular disorders (NMDs). The aim of the study was to compare patients with LVHT depending on age at diagnosis. Included were 232 patients with LVHT (72 women, mean age 52 17 years) diagnosed from 1995 to 2014 at 1 echocardiographic laboratory. In 2014, their survival was assessed. Seventy-six percent of the patients were neurologically investigated, revealing specific NMDs in 18%, unspecific NMDs in 60%, and normal findings in 22%. Forty-five patients (19%) received electronic devices: implantable cardioverter-defibrillators in 26 patients, combined with cardiac resynchronization systems (n = 14) or an antibradycardic pacemaker (n = 1); antibradycardic pacemakers (n = 8); cardiac resynchronization systems (n = 4); implantable loop recorders (n = 4); life vests (n = 2); and a left ventricular assist device as a bridge to transplantation (n = 1). During 72-month follow-up, mortality was 4.9% per year. In younger age groups, more patients were referred for syncope or palpitations, whereas in older age groups, more patients were referred for heart failure. Classic cardiovascular risk factors such as hypertension and diabetes, as well as coronary artery stenosis, were rare in the young age groups but were more prevalent in older age groups. Differences between age groups were found regarding cardiac symptoms, NMDs, electrocardiographic findings, rate of device implantation, and mortality but not in location and extension of LVHT. None of the neurologically investigated patients years of age was neurologically normal. Prevalence of heart failure, electrocardiographic abnormalities, and mortality were highest in the oldest age group. In conclusion, LVHT must be considered as an echocardiographic diagnosis in all age groups. The morphologic pattern of LVHT is similar, whereas clinical manifestations and prognosis are variable among age groups. (C) 2015 Elsevier Inc. All rights reserved.

OBJECTIVES The aim of the study was to assess interobserver agreement (IOA) between 3 observers from 2 Laboratories.BACKGROUND IOA of left ventricular hypertrabeculation/noncompaction (LVHT) in adults has only been studied within single echocardiographic Laboratories. Cross MarkMETHODS Echocardiographic recordings with and without LVHT were selected and anonymized. The "not-LVHT" cases were matched for age and systolic function. Each observer reviewed the recordings, blinded to the initial diagnosis and the other observers' results. Pre-defined criteria for LVHT were: 1) >3 prominent trabeculae at end-diastole, distinct from papillary muscles, false tendons, or aberrant bands; 2) a noncompacted part of a 2-layered myocardial structure formed by these trabeculations; 3) a ratio of >2:1 of noncompacted to compacted layer at end-systole; and 4) perfusion of the intertrabecular spaces from the ventricular cavity. IOA was estimated using the kappa measure of concordance.RESULTS Cine-loops of 100 patients (42 women, ages 16 to 92 years), 50 from each center, and 51 with LVHT as the initial diagnosis, were reviewed. The Left ventricular end-diastolic diameter was 32 to 78 mm, and ejection fraction, 4% to 88%. The observers agreed about presence (n = 29) or absence (n = 36) of LVHT and disagreed in 35 cases. Agreement was higher among the 2 observers from the same Laboratory (kappa 0.793 [95% confidence interval (CD: 0.672 to 0.9151) than from different Laboratories (kappa 0.628 [95% CI: 0.472 to 0.784], kappa 0.669 [95% Cl: 0.521 to 0.818]). The observers agreed with the initial report of LVHT-presence in 53% and of absence in 67%. By reviewing the discordant cases, consensus was achieved about LVHT presence (n = 8) or absence (n = 16); in 11 cases, the diagnosis remained questionable. Discordance was due to poor image quality, lack of views in different apical planes, aberrant bands and chordae tendineae, abnormally sized or inserting papillary muscles, and localized calcifications of the endocardium.CONCLUSIONS IOA was substantial for diagnosing LVHT. However, even the application of pre-defined criteria yielded disagreement in 35% of cases; and after mutual review, there were still 11% questionable cases. (C) 2015 by the American College of Cardiology Foundation.

Purpose: Left ventricular noncompaction (LVNC) cardiomyopathy is usually diagnosed by echocardiography (ECHO) but cardiac magnetic resonance imaging (CMRI) has evolved as an alternative method. This study assessed the diagnostic and prognostic value of CMRI in adults with LVNC.Methods: Twenty eight pts (18m, 10f, age 52±16) with ECHO diagnosis of LVNC underwent cine and contrast-enhanced CMRI with a 1.5 T scanner. LV diameter, volume, ejection fraction, degree of mitral regurgitation, ratio of noncompacted to compacted myocardium (NC/C) and the presence and localization of late gadolinium enhancement (LGE) were determined. CMRI findings were correlated to clinical events, ECHO and angiography.Results: Sixteen pts (57%) were in heart failure NYHA III or IV, 11 (39%) had a left bundle branch block (LBBB) and 6 (23%) documented ventricular tachycardia. In 2 pts a thrombus was seen within the trabecular layer which resolved under anticoagulation, and 4 additional pts without detectable thrombus suffered a stroke. By CMRI, LV diameter in end-diastole (65±8 mm), end-systole (52±10 mm), end-diastolic volume (227±74 ml) and end-systolic volume (145±72 ml) were enlarged and ejection fraction (38±15%) was reduced, with similar values determined by ECHO and angio. The NC/C ratio was 3.3±1.5 in end-diastole and 2.6±1.5 in end-systole. No pt had right ventricular involvement.LGE was detected in 6/27 pts (22%). LGE was present in the compacted myocardial layer (n=3), in the noncompacted trabecular layer (n=5) and within the papillary muscles (n=3). LGE was seen in all 3 areas in 1 and in 2 areas in 3 pts. All 3 pts with papillary muscle LGE also had trabecular LGE and high grade mitral regurgitation, and 1 of these pts died while awaiting HTx. Thrombus and stroke occurred mainly in pts with LGE (4/6 vs 2/21 pts, p=0.01). Three of 11 pts with LBBB and 3/6 pts with ventricular tachycardia exhibited LGE. A high NC/C ratio, however, was not associated with heart failure, thrombus and stroke, LBBB, VT or ejection fraction.Conclusions: In LVNC, evaluation by CMRI and demonstration of LGE identifies pts at high risk for clinical events. Extensive LGE may predispose to thrombus formation and stroke, warranting anticoagulation. LGE within the papillary muscles is associated with high grade mitral regurgitation, aggravating heart failure in these patients.

Background: Aim of this study was to assess pathomorphologic findings (PATHO) in patients with echocardiographically (ECHO) diagnosed left ventricular hypertrabeculation/noncompaction.Methods: ECHO-criteria for LVHT were: >3 trabeculations, moving synchronously with the compacted myocardium, and forming the noncompacted part of a two-layered myocardium. At autopsy, the hearts were investigated according to the pathologists' preferences.Results: Twelve patients (2 females, age 27-81 years) were included. Seven suffered from neuromuscular disorders, 5 patients were not investigated neurologically. The specimens were acquired after explantation during heart transplantation (n = 1), death due to heart failure (n = 6), sudden death (n = 2), pneumonia (n = 2) and stroke (n = 1). Eight hearts were investigated without fixation and 4 after formaldehyde fixation. The hearts were opened along the long-axis, in 3 hearts additional short-axis cuts were carried out. At PATHO the trabecular meshwork was better visible in the formaldehyde-fixed hearts than in the fresh hearts. Differentiation from papillary muscles was easier on the long-axis cuts, whereas the two-layered structure was better visible on short-axis cuts. The trabecular pattern was similar in patients with neuromuscular disorders and those who did not undergo neurologic investigation. Subendocardial fibrosis was found in each case. Due to the complex three-dimensional geometry, it was impossible to count the number of trabeculations.Conclusion: Formaldehyde-fixation should be performed when comparing ECHO with PATHO findings in LVHT. Long-axis as well as short-axis cuts should be carried out in order to assess the course of trabeculations and the extent of the two-layered structure. Subendocardial fibrosis in LVHT deserves further research. (C) 2013 Published by Elsevier Ireland Ltd.

Die isolierte Noncompaction Kardiomyopathie (NCCM) ist eine seltene, genetisch determinierte Herzmuskelerkrankung, bei der der Remodellingprozess des linksventrikulären (LV) Myokards während der Embryonalzeit gestört ist, sodass in den betroffenen Abschnitten der LV-Wand eine schmale kompakte und eine dickere nicht kompakte Myokardschicht verbleiben. Die Genetik der NCCM ist dabei durch eine große Heterogenität charakterisiert. Die Diagnostik der NCCM kann durch Echokardiographie oder kardiale Magnetresonanztomographie erfolgen, wobei die Diagnosekriterien immer noch einer Diskussion unterliegen. Die betroffenen Patienten weisen ein breites Spektrum an Befunden und Symptomen auf: Arrhythmien, Herzinsuffizienz und kardioembolische Ereignisse. In der Risikostratifikation erwiesen sich neben der schweren LV-Funktionsstörung ein kompletter Linksschenkelblock und Vorhofflimmern als signifikante Risikomarker für schwere klinische Ereignisse. Die Therapie der NCCM erfolgt individuell und orientiert sich an den Symptomen und Untersuchungsbefunden. Sie umfasst eine medikamentöse Behandlung, im Bedarfsfall auch Ablationsbehandlungen oder gerätebasierte Therapien und bei schwerster Herzinsuffizienz eine Listung zur Herztransplantation. Bei der Betreuung von Patienten mit NCCM sollte neben der regelmäßigen kardiologischen Kontrolle auch ein Familienscreening mit Erhebung der Anamnese, körperlicher Untersuchung, EKG-Registrierung und Echokardiographie durchgeführt werden.

Isolated noncompaction cardiomyopathy (NCCM) is a rare genetically determined myocardial disease caused by abnormal fetal development of the myocardium resulting in a thin compacted and a thicker noncompacted layer of the affected left ventricular (LV) wall. The genetic basis of NCCM is heterogenous. Diagnosis can be made using echocardiography or magnetic resonance imaging. The diagnostic criteria for NCCM are still under discussion. Afflicted patients may present with various symptoms caused by arrhythmias, heart failure and cardioembolic events. Severely reduced LV function as well as left bundle branch block and atrial fibrillation were shown to be linked to worse outcomes. Treatment in patients with NCCM should be targeted at individual symptoms and clinical findings. Therapy includes pharmacological treatment, and in individual cases ablation or device therapy, as well as consideration for heart transplantation in selected cases. Aside from regular clinical follow-up of patients with NCCM screening of first degree family members with assessment of medical history, physical examination, ECG recording, and echocardiography are recommended.

ObjectiveThis study evaluated if there are gender differences in the manifestation of tako-tsubo cardiomyopathy (TTC).BackgroundTTC predominantly occurs in elderly females and mimics acute myocardial infarction (AMI) where men and women are known to have a different clinical profile.Methods324 patients from 37 hospitals were prospectively included in a TTC registry. Clinical, electrocardiographic, angiographic and outcome data from male and female patients were compared.ResultsOf 324 patients 296 (91%) were female and 28 (9%) male. Mean age (68±12 vs 66±12years) and prehospital delay were similar. A triggering event preceded TTC onset in 76% of women and 86% of men. Physical stress was more frequent in men (30% vs 57%, p=0.005) whereas more women experienced emotional or no stress. The prevalence of angina and dyspnea did not differ. Fewer females were admitted in cardiogenic shock and/or after out of hospital cardiac arrest (1% vs 14%, p=0.0006), and cardiac troponin was lower (median 7.2 vs 10.7 times the upper limit of normal, p=0.03). The QTc interval was longer in females than in males only on the day of admission (468±52 vs 441±51ms, p=0.047). Overall, complications during the acute course (53% vs 40%) were comparable in both sexes.ConclusionsIn this large TTC registry, males and females showed a similar clinical profile. In males, physical stress as a trigger event and shock or cardiac arrest as presenting symptoms were more frequent. The QTc interval was longer in females only on admission but similar in males and females during the following days.

Background: Left ventricular hypertrabeculation/noncompaction (LVNC) is a cardiac abnormality whose echocardiographic criteria are still controversial. Cooperation between echocardiographic laboratories may contribute to uniformly accepted criteria, as illustrated by the following pilot study.Methods and results: Echocardiograms proposed for inclusion into a registry were reviewed. Three experts with 17-26 years experience with LVNC agreed on a common definition of LVNC: 1. >3 prominent trabeculous formations along the left ventricular endocardial border visible in end-diastole, distinct from papillary muscles, false tendons or aberrant bands; 2. trabeculations move synchronously with the compacted myocardium, 3. trabeculations form the noncompacted part of a two-layered myocardial structure, best visible at end-systole; and 4. perfusion of the intertrabecular spaces from the ventricular cavity is present at end-diastole on color-Doppler echocardiography or contrast echocardiography.During 3 sessions 115 cases (37% females, mean 57 years) were reviewed. Eleven patients(18% females, mean 60 years) were excluded because of <4 trabeculations(n=5), lack of a two-layered myocardial structure(n=1) and poor image quality(n=5). The observers agreed on inclusion or exclusion in all cases. Consensus was achieved that measurements of the thickness of the myocardial layers, and calculation of the noncompacted: compacted ratio is not feasible due to a lack of uniformly accepted standards for measurements.Conclusions: When diagnosing LVNC, end-systolic as well as end-diastolic images have to be considered. The presence of more than three trabeculations as well as a two-layered myocardium are required. Since these criteria are not anatomically controlled, a comparison of echocardiographic images with pathoanatomic findings for assessing sensitivity and specificity is urgently needed. (C) 2011 Elsevier Ireland Ltd. All rights reserved.