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3 years ago
Inherited arrhythmias comprise a group of disorders with inherited susceptibility to arrhythmias and conduction disturbances due to mutations in genes mainly encoding the Na+, and K+ channels, and other arrhythmogenic mechanisms such as those linked to Ca++ transport (Table 1).1 The majority of heritable cardiomyopathies and channelopathies are associated with disease-susceptibility genes…
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3 years ago
Atrial fibrillation (AF) is a highly prevalent arrhythmia that represents an important burden on healthcare systems. The presence of AF is associated with an increased risk of conditions such stroke, heart failure and dementia. Further, AF is associated with increased mortality. Over the past half century, significant advances have been made in understanding the pathobiology of AF. Important…
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3 years ago
Early repolarisation pattern is defined electrocardiographically by a distinct J wave or J-point elevation that is either a notch or a slur of the terminal part of the QRS entirely above the baseline, with or without ST-segment elevation. The peak of the notch or slur (Jp) should be ≥0.1 mV in two or more contiguous leads, excluding leads V1 to V2 (see Figure 1).1,2 Early repolarisation syndromes…
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3 years ago
HCN4 (hyperpolarisation-activated, cyclic nucleotide gated 4) channels, the pore-forming α–subunits of ‘funny’ channels originally described in pacemaker cells of the sinoatrial node (SAN),1 are responsible for the early phase of diastolic depolarisation in these cells and are key determinants of pacemaker generation and control of heart rate.2–5 HCN4 channels are selectively expressed in the SAN…
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3 years ago
The Risk of Atrial Arrhythmias in Inherited Primary Arrhythmia Syndromes
The inherited primary arrhythmia syndromes (IPAS) are a heterogeneous group of diseases caused by mutations in genes encoding for cardiac ion channels. People affected by one of these inherited diseases have no overt structural cardiac abnormalities but are at higher risk of sudden cardiac death due to the occurrence of…
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3 years ago
Sudden cardiac death (SCD) is usually defined as death due to cardiac causes occurring within 1 hour of the onset of symptoms. Unexplained sudden death occurring in an individual older than 1 year of age is known as 'sudden unexplained death syndrome'. Unexplained sudden death occurring in an individual younger than 1 year of age is known as 'sudden unexplained death in infancy'. SCD with…
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2 years ago
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3 years ago
Since its initial description by Jervell and Lange-Nielsen in 1957,1 congenital long QT syndrome (LQTS) has been the most investigated cardiac ion channelopathy. A prolonged QT interval on the surface ECG is a surrogate measure of prolonged ventricular action potential duration (APD).
Congenital as well as acquired alterations in certain cardiac ion channels can affect their currents in such a…
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3 years ago
Catheter ablation (CA) is a widely used first-line treatment for AF. Randomised controlled trials (RCTs) have shown its superiority to pharmacological treatment in terms of symptom control, rhythm control and mortality in selected patients; observational studies have also suggested a decreased risk of stroke.1,2 Pulmonary vein isolation (PVI) is the standard endpoint, but studies have reported…
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