Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders

Stöllberger, Claudia; Finsterer, Josef; Blazek, Gerhard

doi:10.1016/s0002-9149(02)02723-6

Cited by 523 publications

(456 citation statements)

References 20 publications

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“…3 Hypertrabeculation of the left ventricle, not fulfilling the criteria of isolated left ventricular noncompaction, is sometimes seen in patients with other congenital cardiac anomalies or in patients with neuromuscular disorders. 4 Isolated left ventricular noncompaction has been described in children as well as adults, including the elderly. 5 The prevalence is low, probably 0.05%, but may actually be higher because of underdiagnosing or misdiagnosing as hypertrophic cardiomyopathy.…”

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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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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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“…32 • Presence of more than three trabeculations in the left ventricular wall, with the papillary muscles located at the apex, visible in one image plane.…”

Section: Criteria Proposed By Chin Et Almentioning

confidence: 99%

“…The prevalence of neuromuscular disorders was of 82% in 49 patients with NCC who were neurologically investigated 32 . In some of these cases, the neuromuscular disorder had not yet manifested itself clinically.…”

Section: Treatmentmentioning

confidence: 99%

Miocardiopatia não compactada: uma visão atual

Rosa¹,

Salemi²,

Alexandre³

et al. 2011

Arq. Bras. Cardiol.

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Isolated non-compaction cardiomyopathy is a rare disease that is likely to develop in the embryonic period. It is caused by the intrauterine arrest of the myocardial compaction process in the beginning of the fetal development. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses, as well as the thickening of the myocardium into two distinct layers (compacted and not compacted). Even though this disease is said to be prevalent in the pediatric population or together with congenital heart disease, one can understand that this disease occurs in isolation, because the diagnosis is becoming more common in adult patients that have no other heart disease. The clinical manifestations vary greatly, because they range from absence of symptoms to congestive heart failure, arrhythmias and systemic thromboembolism. Echocardiography is the most widely used diagnostic procedure, but the little knowledge about this disease, its similarity to other myocardial diseases and the limitation of the echocardiographic technique used delay the diagnosis. The purpose of this review is to show that that other imaging techniques, such as MRI, CT and left ventriculography have emerged as diagnostic alternatives.

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“…A recently published study identified gene variants encoding proteins of the sarcomere (MYH7, ACTC and TNNT2) in affected adult individuals. These same genes are associated with hypertrophic and dilated cardiomyopathy 6 .…”

Section: Introductionmentioning

confidence: 96%