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Published on: February 17, 2023
Association of electrocardiographic abnormalities with cardiac findings and neuromuscular disorders in left
Claudia Stöllberger1, Maria Winkler-Dworak, Gerhard Blazek
12nd Medical Department, Krankenanstalt Rudolfstiftung, Wien, Austria. claudia.stoellberger@chello.at
Insights
Electrocardiogram (ECG) abnormalities are common in left ventricular hypertrabeculation/non-compaction (LVHT), often indicating heart failure. These ECG changes occur regardless of neuromuscular disorders, necessitating neurological referral for all LVHT patients.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Left ventricular hypertrabeculation/non-compaction (LVHT) is a cardiac abnormality with prominent trabeculations and intertrabecular recesses.
- LVHT is frequently associated with neuromuscular disorders (NMD).
Purpose of the Study:
- To assess the prevalence of electrocardiographic (ECG) abnormalities in patients with LVHT.
- To determine the association of ECG abnormalities with clinical symptoms, cardiac structure and function, and the presence of NMD.
Main Methods:
- Echocardiographic diagnosis of LVHT in 86 patients.
- Baseline cardiologic and neurologic investigations were performed.
- ECG analysis and correlation with clinical and echocardiographic findings.
Main Results:
- Only 10% of patients had normal ECGs; frequent abnormalities included tall QRS complexes (43%), ST/T-wave abnormalities (37%), and left bundle branch block (20%).
- ECG abnormalities correlated with heart failure symptoms, systolic dysfunction, and valvular issues.
- Atrial fibrillation was linked to LVHT extension, but ECG patterns did not differ between patients with and without NMD.
Conclusions:
- ECG abnormalities are highly prevalent in LVHT.
- A normal ECG does not rule out LVHT, and no specific ECG pattern is typical.
- ECG abnormalities are independent of NMD, supporting routine neurological referral for all LVHT patients.
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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