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      Association of Electrocardiographic Abnormalities with Cardiac Findings and Neuromuscular Disorders in Left Ventricular Hypertrabeculation/Non-Compaction


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          Introduction and Objectives: Left ventricular hypertrabeculation/non-compaction (LVHT) is a cardiac abnormality characterized by prominent trabeculations and intertrabecular recesses, and frequently associated with neuromuscular disorders (NMD). The aim of the study was to assess the prevalence of electrocardiographic (ECG) abnormalities in LVHT and its association with clinical symptoms, left ventricular size, wall thickness, systolic function, location and extension of LVHT and presence or absence of NMD. Methods and Results: In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (21 female, 65 male, age: 14–94 years, mean age: 52 ± 14 years). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21 (metabolic myopathy, n = 14; Leber’s hereditary optic neuropathy, n = 3; myotonic dystrophy, n = 2; Becker muscular dystrophy, n = 1; Duchenne muscular dystrophy, n = 1), a NMD of unknown etiology in 32, the neurologic investigation was normal in 13, and 20 patients refused. Only 9 patients (10%) had normal ECGs. Frequent ECG abnormalities were tall QRS complexes (43%); ST/T-wave abnormalities (37%) and left bundle branch block (20%). ECG abnormalities were related with symptoms of heart failure and echocardiographic findings of systolic dysfunction and valvular abnormalities. Only atrial fibrillation (9%) was related to extension of LVHT. ECG abnormalities did not differ between patients with and without NMD. Conclusion: ECG abnormalities are frequent in LVHT. A normal ECG, however, does not exclude LVHT. No ECG pattern is typical for LVHT. ECG abnormalities occur independently of presence or absence of NMD, and thus all patients with LVHT should be referred to the neurologist.

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

          We sought to describe characteristics and outcome in adults with isolated ventricular noncompaction (IVNC). Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Knowledge regarding diagnosis, morbidity and prognosis is limited. Echocardiographic criteria for IVNC include-in the absence of significant heart lesions-segmental thickening of the left ventricular myocardial wall consisting of two layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses. Thirty-four adults (age >16 years, 25 men) fulfilled the diagnostic criteria and were followed prospectively. At diagnosis, mean age was 42 + 17 years, and 12 patients (35%) were in New York Heart Association class III/IV. Left ventricular end-diastolic diameter was 65 + 12 mm and ejection fraction 33 + 13%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in >80% of patients. Follow-up was 44 + 40 months. Major complications were heart failure in 18 patients (53%), thromboembolic events in 8 patients (24%) and ventricular tachycardias in 14 patients (41%). There were 12 deaths: sudden in six, end-stage heart failure in four and other causes in two patients. Four patients underwent heart transplantation. Automated cardioverter/defibrillators were implanted in four patients. Diagnosis of IVNC by echocardiography using strict criteria is feasible. Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias. Risk stratification includes heart failure therapy, oral anticoagulation, heart transplantation and implantation of an automated defibrillator/cardioverter. As IVNC is a distinct entity, its classification as a specific cardiomyopathy seems to be more appropriate.
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            Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders.

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              Left ventricular hypertrabeculation/noncompaction.

              In normal human hearts the left ventricle (LV) has up to 3 prominent trabeculations and is, thus, less trabeculated than the right ventricle. Rarely, more than 3 prominent trabeculations can be found at autopsy and by various imaging techniques in the LV. For this abnormality, different synonyms are used such as spongy myocardium, LV noncompaction, and LV hypertrabeculation (LVHT). In this review it is stated that: (1) LVHT has a higher prevalence than previously thought and the prevalence of LVHT seems to increase with the improvement of cardiac imaging; (2) because LVHT is most frequently diagnosed primarily by echocardiography, echocardiographers should be aware and trained to recognize this abnormality; (3) LVHT is frequently associated with other cardiac and extracardiac, particularly neuromuscular, disorders; (4) there are indications that the cause of LVHT is usually a genetic one and quite heterogeneous; and (5) controversies exist about diagnostic criteria, nomenclature, prognosis, origin, pathogenesis, and the necessity to classify LVHT as a distinct entity and cardiomyopathy by the World Health Organization.

                Author and article information

                S. Karger AG
                May 2007
                05 February 2007
                : 107
                : 4
                : 374-379
                a2nd Medical Department, Krankenanstalt Rudolfstiftung, bInstitute for Demography of the Austrian Academy of Science, cHanusch Krankenhaus, dKrankenanstalt Rudolfstiftung, Wien, Austria
                99055 Cardiology 2007;107:374–379
                © 2007 S. Karger AG, Basel

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                : 06 February 2006
                : 25 August 2006
                Page count
                Tables: 1, References: 21, Pages: 6
                Original Research

                General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
                Left ventricular hypertrabeculation/non-compaction,Myopathy,Echocardiography,Neuromuscular disorder,Cardiomyopathy,Electrocardiogram


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