Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis

Oechslin, Erwin; Jost, Christine H. Attenhofer; Rojas, J; Kaufmann, Philipp A.; Jenni, Rolf

doi:10.1016/s0735-1097(00)00755-5

Cited by 1,107 publications

(1,323 citation statements)

References 22 publications

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“…An arrhythmogenic trait associated with increased risk of sudden cardiac death (SCD) is well known in cardiomyopathies such as arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) and left‐dominant arrhythmogenic cardiomyopathy,1, 2 as well as in hypertrophic cardiomyopathy3 and LV noncompaction,4, 5, 6, 7 and is used for the purpose of risk stratification. However, the specific criteria for “arrhythmogenic” DCM (AR‐DCM) are lacking.…”

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confidence: 99%

Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life‐Threatening Arrhythmias

Spezzacatene

Sinagra

Merlo

et al. 2015

JAHA

111

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BackgroundPatients with dilated cardiomyopathy (DCM) may present with ventricular arrhythmias early in the disease course, unrelated to the severity of left ventricular dysfunction. These patients may be classified as having an arrhythmogenic DCM (AR‐DCM). We investigated the phenotype and natural history of patients with AR‐DCM.Methods and ResultsTwo hundred eighty‐five patients with a recent diagnosis of DCM (median duration of the disease 1 month, range 0 to 7 months) and who had Holter monitoring at baseline were comprehensively evaluated and followed for 107 months (range 29 to 170 months). AR‐DCM was defined by the presence of ≥1 of the following: unexplained syncope, rapid nonsustained ventricular tachycardia (≥5 beats, ≥150 bpm), ≥1000 premature ventricular contractions/24 hours, and ≥50 ventricular couplets/24 hours, in the absence of overt heart failure. The primary end points were sudden cardiac death (SCD), sustained ventricular tachycardia (SVT), or ventricular fibrillation (VF). The secondary end points were death from congestive heart failure or heart transplantation. Of the 285 patients, 109 (38.2%) met criteria for AR‐DCM phenotype. AR‐DCM subjects had a higher incidence of SCD/SVT/VF compared with non–AR‐DCM patients (30.3% vs 17.6%, P=0.022), with no difference in the secondary end points. A family history of SCD/SVT/VF and the AR‐DCM phenotype were statistically significant and cumulative predictors of SCD/SVT/VF.ConclusionsOne‐third of DCM patients may have an arrhythmogenic phenotype associated with increased risk of arrhythmias during follow‐up. A family history of ventricular arrhythmias in DCM predicts a poor prognosis and increased risk of SCD.

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confidence: 99%

Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life‐Threatening Arrhythmias

Spezzacatene

Sinagra

Merlo

et al. 2015

JAHA

111

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“…12 High-risk features for death include higher left ventricular end-diastolic diameter on presentation, New York Heart Association class III-IV, permanent or persistent atrial fibrillation, and bundle branch block. 13 Because of the supposed familial association of isolated noncompaction, screening of first-degree relatives by echocardiography is recommended. 14 Conclusion In the described case, the diagnosis of isolated left ventricular noncompaction was made by chance, during hospitalisation for a subacute myocardial infarction.…”

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confidence: 99%

Isolated left ventricular noncompaction in a patient presenting with a subacute myocardial infarction

Swinkels¹,

Boersma²,

Rensing³

et al. 2007

NHJL

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Isolated left ventricular noncompaction in a patient presenting with a subacute myocardial infarctionIsolated left ventricular noncompaction is a rare cardiomyopathy that is often not recognised. So far, it is not well established how best to manage this abnormality. We describe a patient in whom the diagnosis of isolated left ventricular noncompaction was made after presentation with a subacute myocardial infarction. Because of nonsustained ventricular tachycardias during hospitalisation, which were inducible and deteriorated into ventricular fibrillation on electrophysiological examination after coronary artery bypass grafting, he received an implantable defibrillator. Whether the ventricular tachycardias were due to the myocardial infarction or to the noncompacted myocardium remains uncertain. (Neth Heart J 2007; 15:109-11.) Keywords: noncompaction (isolated), cardiomyopathy, echocardiographyA 73-year-old man without a cardiac history presented to the Emergency Department because of chest pain radiating to the throat for two days. On physical examination he measured 1.70 m and weighed 58 kg. His blood pressure was 110/60 mmHg and the pulse regular at a rate of 70 beats/min. The jugular venous pressure appeared normal. Cardiac auscultation revealed normal heart sounds without murmurs. The lungs were clear to auscultation. The electrocardiogram showed sinus rhythm with right bundle branch block, 1 mm ST-segment elevation in leads I, II, aVL and V 3 to V 6 , and slightly terminally negative T waves in leads V 3 to V 6 . The chest X-ray was normal with a cardiothoracic ratio of 43%. Creatine kinase (CK) and CK-MB were increased at presentation (2263 and 356 U/l, respectively). The diagnosis of subacute anterolateral myocardial infarction was made and the patient received aspirin, clopidogrel, lowmolecular-weight heparins, nitrates, β-blockers and a statin. The next day, the CK and CK-MB had fallen and transthoracic echocardiography revealed akinetic anterolateral wall segments, a left ventricular ejection fraction of 35 to 40%, no significant valve disease, and prominent trabeculation of the apex of the left ventricular myocardium consistent with noncompaction. The presence of noncompaction was confirmed by contrast echocardiography (1.5 ml SonoVue intravenously), showing direct communication between the left ventricular cavity and the deep intertrabecular recesses (figure 1). The patient's convalescence was complicated by the occurrence of recurrent nonsustained ventricular tachycardia from the fourth day after admission, and ST depression (without chest pain) during bicycle ergometry on the fifth day after admission. Coronary angiography showed significant two-vessel disease of the left anterior descending (LAD) and left circumflex (LCX) coronary artery. Three weeks after admission, the patient underwent coronary artery bypass grafting (CABG). The left internal mammary artery was anastomosed with the LAD, and a venous jump graft was anastomosed with the obtuse marginal branch of the LCX via the anterolateral br...

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“…LVNC has been associated with high incidence of complications such as cardiac failure (%53), ventricular tachycardia (41%), thromboembolism (24%), and sudden death (16%) [13]. Cardiovascular complications such as decompensated heart failure, embolic event, ventricular arrhythmia and NYHA class III or greater at presentation were the strongest predictors of adverse outcomes [14].…”

Section: Discussionmentioning

confidence: 99%

Peripartum noncompaction cardiomyopaty:new diagnosed after pregnancy

Asoğlu¹,

Türkmen²,

Aktürk³

et al. 2018

Med-Science

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Left-ventricular non-compaction (LVNC) cardiomyopathy is an arrest in the normal process of myocardial compaction and few cases have been reported just after pregnancy. In this case report, we present a patient who had heart failure symptoms at first month of after 38 weeks pregnancy. Echocardiography revealed prominent trabeculation and deep intertrabecular recesses at the left ventricular with an impaired ejection fraction. There is no specific treatment for LVNC just after pregnancy besides the usual management of dilated cardiomyopathy.

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Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis

Cited by 1,107 publications

References 22 publications

Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life‐Threatening Arrhythmias

Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life‐Threatening Arrhythmias

Isolated left ventricular noncompaction in a patient presenting with a subacute myocardial infarction

Peripartum noncompaction cardiomyopaty:new diagnosed after pregnancy

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