Why the study?
Congenital long QT syndrome presents clinical challenges and carries a high risk of intrauterine fetal demise from secondary life-threatening arrhythmias.
Early recognition of fetal long QT syndrome via echocardiography is crucial for prompt medical and surgical management to optimize survival.
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May aid fetal LQTS suspicion via echo in bradycardia/VT cases; leaves open prospective validation before clinical adoption.
presents many challenges for clinicians, and there is high risk for intrauterine fetal demise as life-threatening arrhythmias develop secondary to QT prolongation. We describe a challenging case of a fetus presenting with sinus bradycardia and second-degree atrioventricular block with episodes of ventricular tachycardia. A prenatal diagnosis of LQTS was suspected given the fetal echocardiographic findings of a short ventricular relaxation time, due to extremely prolonged refractory period. The patient was delivered emergently due to Torsade's with hydrops, with ongoing arrhythmia despite medical management requiring implantation of pacemaker and sympathectomy. Early recognition of LQTS is important to optimize fetal survival with prompt medical management.
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Santi et al. (2022) studied this question.
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