Key result
A foetus presenting with ventricular tachycardia and complete heart block was diagnosed with long QT syndrome and ventricular noncompaction, highlighting the need to avoid QT-prolonging drugs.
Why the study?
Defects in ion channels in congenital long QT syndrome may cause myocardial architectural disruption leading to ventricular noncompaction.
Case Report (n=1)
The presence of ventricular tachycardia and complete heart block in utero suggests long QT syndrome, which can co-occur with ventricular noncompaction and requires avoidance of QT-prolonging antiarrhythmics.
Answers are grounded in this paper and cite their sources.
May raise suspicion for long QT syndrome in fetal VT with block; hypothesis-generating for noncompaction association and drug selection.
Congenital long QT syndrome [LQTS] is a channelopathy characterized by QT prolongation and polymorphic VT. LQTS however need not be a purely electrical disease. Defects in ion channels may cause myocardial architectural disruption leading to ventricular non compaction [VNC]. It is defined as the presence of prominent ventricular trabeculations and deep intertrabecular recesses within the endomyocardium. We describe the in-utero management of a foetus who was later found to have LQTS with VNC. The detection of ventricular tachycardia and complete heart block in utero should arouse the suspicion of LQTS. It would be wise to avoid QT prolonging antiarrhythmics in this subset of patients.
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Cherian et al. (2021) conducted a case report in Long QT syndrome and ventricular noncompaction (n=1). In-utero management was evaluated. A foetus presenting with ventricular tachycardia and complete heart block was diagnosed with long QT syndrome and ventricular noncompaction, highlighting the need to avoid QT-prolonging drugs.
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