Left ventricular hypertrabeculation/noncompaction

Stöllberger, Claudia; Finsterer, Josef

doi:10.1016/s0894-7317(03)00514-5

Cited by 407 publications

(384 citation statements)

References 81 publications

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“…15,16 The visualization of LVHT on CMRI was independent of the presence or absence, and the type of NMD may be due to the high rate of NMD in LVHT or the heterogenous aetiology of NMD.…”

Section: Discussionmentioning

confidence: 94%

Diagnosing Left Ventricular Noncompaction by Echocardiography and Cardiac Magnetic Resonance Imaging and Its Dependency on Neuromuscular Disorders

Stöllberger¹,

Kopsa²,

Tscherney³

et al. 2008

Clinical Cardiology

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Background: Left ventricular hypertrabeculation (LVHT), also termed noncompaction LVHT, is diagnosed by echocardiography or cardiac magnetic resonance imaging (CMRI), and associated with neuromuscular disorders (NMD). The aim of this study was to assess if LVHT can be diagnosed by CMRI applying echocardiographic definitions. Methods and Results:The CMRI images of 19 echocardiographically diagnosed LVHT patients were reevaluated (10 female, 14-67 y of age). Left ventricular hypertrabeculation was diagnosed by CMRI in 9 cases. Patients with CMRI-diagnosed LVHT were more often females (67% versus 40%), experienced heart failure more often (100% versus 50%), had an LV end diastolic diameter >57 mm (67% versus 40%), had an LV fractional shortening <25% (89% versus 40%), and had a larger extension of LVHT than patients without CMRI-diagnosed LVHT. The prevalence of NMD (87%) did not differ between both groups. Conclusions: Echocardiographic definition for CMRI yielded the diagnosis of LVHT in only 47%. When looking for LVHT by CMRI, LV size, function, and extension of LVHT have to be considered.

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“…15,16 The visualization of LVHT on CMRI was independent of the presence or absence, and the type of NMD may be due to the high rate of NMD in LVHT or the heterogenous aetiology of NMD.…”

Section: Discussionmentioning

confidence: 94%

Diagnosing Left Ventricular Noncompaction by Echocardiography and Cardiac Magnetic Resonance Imaging and Its Dependency on Neuromuscular Disorders

Stöllberger¹,

Kopsa²,

Tscherney³

et al. 2008

Clinical Cardiology

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“…6 Men appear to be affected more often (about 70%) than women. 7 Transthoracic echocardiography is the diagnostic test of choice, while contrast echocardiography can help to establish the diagnosis. 8 Magnetic resonance imaging is useful in cases with poor echocardiographic image quality.…”

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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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“…The prevalence of LVNC ranges from 0.05% to 0.24% per year in echocardiographic databases. 6 In pediatric patients, LVNC often coexists with other cardiac and extracardiac anomalies such as facial dysmorphism, and the mortality rate is high due to disabling heart failure or sudden death. 1 On the other hand, LVNC is a distinct entity in adults that has a wide spectrum of presentation from asymptomatic to symptomatic.…”

Section: Discussionmentioning

confidence: 99%

“…Left ventricular noncompaction was initially considered as one cause of critical congestive heart failure in a pediatric population, 1 but this abnormality has been identified in an adult population due to advances in imaging techniques. 6 Cardiac surgery for adult patients with LVNC has rarely been reported. Here we describe aortic valve replacement to treat aortic regurgitation (AR) in a patient with LVNC.…”

Section: Introductionmentioning

confidence: 99%

Aortic Valve Replacement for Aortic Regurgitation with Rare Left Ventricular Non-Compaction

Tanaka

Kimura

Miyamoto

2014

ATCS

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Left ventricular noncompaction cardiomyopathy is a rare type of congenital cardiomyopathy characterized by prematurely arrested compaction of the endocardial and myocardial fibers and the progressive deterioration of left ventricular contractility. This entity is a genetically heterogeneous disorder and has a wide spectrum of presentation from no symptoms to critical disabling congestive heart failure, which can appear at any age. The prognosis is therefore varied. An elderly patient with left ventricular noncompaction underwent aortic valve replacement for associated aortic regurgitation. Follow-up at two years after surgery revealed an improved clinical condition and recovered cardiac function. This is the fourth known aortic valve replacement in a patient with left ventricular noncompaction.Keywords: cardiomyopathy, ventricular noncompaction, aortic valve replacement IntroductionLeft ventricular noncompaction cardiomyopathy (LVNC) is believed to be a morphogenetic abnormality involving arrested compaction of the loose myocardial meshwork during early embryogenesis. 1The World Health Organization categorizes LVNC as an unclassified cardiomyopathy.2 The pathophysiology of this abnormality is the progressive deterioration of LV systolic function, 1 but the cause remains controversial. A relationship between coronary microcirculatory dysfunction and subendocardial ischemia is suspected.3 4 The triad of heart failure, systemic embolism and arrhythmias is the major clinical presentation when the LV systolic function deteriorates. used to diagnose LVNC including echocardiography, magnetic resonance imaging and computed tomography. Left ventricular noncompaction was initially considered as one cause of critical congestive heart failure in a pediatric population, 1 but this abnormality has been identified in an adult population due to advances in imaging techniques. 6 Cardiac surgery for adult patients with LVNC has rarely been reported. Here we describe aortic valve replacement to treat aortic regurgitation (AR) in a patient with LVNC. Case ReportA 74-year-old woman without a family history of cardiovascular disease or sudden death was referred to our hospital with dyspnea that had persisted for three months. The physical findings were unremarkable except for a diastolic murmur at the left sternal edge. A chest x-ray showed cardiomegaly with a cardiothoracic ratio of 70%. Plasma levels of brain natriuretic peptide (BNP) and noradrenalin were elevated to 1540 and 481 pg/ml, respectively. Transthoracic echocardiography revealed extreme regurgitation of the tricuspid aortic valve and diffuse left ventricular (LV) hypokinesis that was particularly severe in the apex, lateral and posterior walls at the mid-ventricular and basal level. The aortic annulus was 23 mm in Left ventricular noncompaction cardiomyopathy is a rare type of congenital cardiomyopathy characterized by prematurely arrested compaction of the endocardial and myocardial fibers and the progressive deterioration of left ventricular contractility. This entity...

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Left ventricular hypertrabeculation/noncompaction

Cited by 407 publications

References 81 publications

Diagnosing Left Ventricular Noncompaction by Echocardiography and Cardiac Magnetic Resonance Imaging and Its Dependency on Neuromuscular Disorders

Diagnosing Left Ventricular Noncompaction by Echocardiography and Cardiac Magnetic Resonance Imaging and Its Dependency on Neuromuscular Disorders

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

Aortic Valve Replacement for Aortic Regurgitation with Rare Left Ventricular Non-Compaction

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