TY - JOUR
T1 - Mechanisms of inherited cardiac conduction disease
AU - Smits, Jeroen P. P.
AU - Veldkamp, Marieke W.
AU - Wilde, Arthur A. M.
PY - 2005
Y1 - 2005
N2 - Cardiac conduction disease (CCD) is a serious disorder of the heart. The pathophysiological mechanisms underlying CCD are diverse. In the last decade the genes responsible for several inherited cardiac diseases associated with CCD have been identified. If CCD is of an inherited nature (ICCD), its underlying mechanism can be either structural, functional or there can be overlap between these two mechanisms. If ICCD is structural in nature, it is often secondary to anatomical or histological abnormalities of the heart. Functional ICCD is frequently found as a "primary electrical disease" of the heart, i.e. resulting from functionally abnormal, or absent proteins encoded by mutated genes, often cardiac ion channel proteins involved in impulse formation. It can thus be hypothesised that patients with inherited structural or functional ICCD suffer from fundamentally different diseases. It is worthwhile to consider this hypothesis, since it could have implications for diagnosis, treatment, prognosis and, possibly, for the patient's relatives. In this review we aim to find evidence for the idea that functional and structural ICCD are fundamentally different diseases and, if so, whether this has diagnostic and clinical consequences. (c) 2005 Published by Elsevier Ltd on behalf of The European Society of Cardiology
AB - Cardiac conduction disease (CCD) is a serious disorder of the heart. The pathophysiological mechanisms underlying CCD are diverse. In the last decade the genes responsible for several inherited cardiac diseases associated with CCD have been identified. If CCD is of an inherited nature (ICCD), its underlying mechanism can be either structural, functional or there can be overlap between these two mechanisms. If ICCD is structural in nature, it is often secondary to anatomical or histological abnormalities of the heart. Functional ICCD is frequently found as a "primary electrical disease" of the heart, i.e. resulting from functionally abnormal, or absent proteins encoded by mutated genes, often cardiac ion channel proteins involved in impulse formation. It can thus be hypothesised that patients with inherited structural or functional ICCD suffer from fundamentally different diseases. It is worthwhile to consider this hypothesis, since it could have implications for diagnosis, treatment, prognosis and, possibly, for the patient's relatives. In this review we aim to find evidence for the idea that functional and structural ICCD are fundamentally different diseases and, if so, whether this has diagnostic and clinical consequences. (c) 2005 Published by Elsevier Ltd on behalf of The European Society of Cardiology
U2 - https://doi.org/10.1016/j.eupc.2004.11.004
DO - https://doi.org/10.1016/j.eupc.2004.11.004
M3 - Review article
C2 - 15763526
SN - 1099-5129
VL - 7
SP - 122
EP - 137
JO - Europace : European pacing, arrhythmias, and cardiac electrophysiology
JF - Europace : European pacing, arrhythmias, and cardiac electrophysiology
IS - 2
ER -