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

Abstract

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.

Left ventricular noncompaction in a patient with multiminicore disease

“…Spirometry showed normal expiratory flow rates and lung volumes. Echocardiography revealed a typical pattern of LVNC according to the criteria of Stö llberger et al 3 and Ö echslin et al 4 Apical three-chamber view showed four prominent trabeculations apically to the papillary muscles with similar echogenicity and simultaneous displacement with normal adjacent myocardium (Fig. 2).…”

“…Intertrabecular spaces were perfused from the ventricular cavity. 3 Diastolic ratio of noncompacted to compacted myocardial thickness was 2.3. 4 Additional findings included left ventricular enlargement and left ventricular ejection fraction of 45%.…”

Cardiac involvement in children with neuro-muscular disorders

“…Электрокардиография (ЭКГ) -важный инструмент скрининга для выявления нару-шений ритма и проводимости сердца, гипертрофии или дилатации желудочков [11]. Проведение эхокар-диографии (ЭхоКГ) считается «золотым стандартом» диагностики структурно-функциональных наруше-ний миокарда, способствующим выявлению патоло-гии сердца на доклиническом уровне [12].…”

Prevalence of Barth syndrome in adult left ventricular hypertrabeculation / noncompaction

“…DCMP was diagnosed if there were dilation of the cardiac cavities, reduced systolic function (LV fractional shortening B25%), normal coronary arteries, and absence of a plausible cause for the dilatation. More details about the echocardiographic equipment and parameter settings have been reported earlier (7).…”