1. Ventricular noncompaction and long QT syndrome – A deadly double hit for the foetus
- Author
-
John Roshan, Jesu Krupa, Pramitha Lankala, and Anne George Cherian
- Subjects
AV, Atrioventricular ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Double hit ,Heart block ,Long QT syndrome ,Ventricular non compaction ,Case Report ,030204 cardiovascular system & hematology ,Ventricular tachycardia ,Low resource setting ,QT interval ,03 medical and health sciences ,0302 clinical medicine ,Channelopathy ,Physiology (medical) ,Internal medicine ,medicine ,Diseases of the circulatory (Cardiovascular) system ,ICD, Implantable Cardioverter Defibrillator ,cardiovascular diseases ,030212 general & internal medicine ,Fetus ,VT, Ventricular tachycardia ,business.industry ,LQTS, Long QT Syndrome ,Fetal arrhythmias ,medicine.disease ,VNC, Ventricular Non compaction ,Electrophysiology ,Congenital long QT syndrome ,RC666-701 ,Cardiology ,Cardiology and Cardiovascular Medicine ,business - Abstract
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.
- Published
- 2021