JERVELL-LANGE-NIELSEN SYNDROME (JLNS) AND PREGNANCY (CLINICAL CASE)
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Abstract
Jervell-Lange-Nielsen syndrome is a rare autosomal recessive disorder characterized by bilateral sensorineural hearing loss and prolongation of the QTc interval. This disorder prolongs ventricular repolarization and increases the risk of ventricular arrhythmias, syncope, and even sudden cardiac death. The risk of death decreases during pregnancy but increases sharply during the nine months postpartum. Women with this disorder are recommended to receive beta-blocker therapy at appropriate doses throughout pregnancy and during the high-risk postpartum period. Because morbidity and mortality in patients with this disorder are high, regular assessment of the patient's cardiovascular needs is essential. This includes adjusting the beta-blocker dosage and evaluating the possibility of implantable cardioverter-defibrillator placement. Regular follow-up with a cardiologist is essential to prevent complications.
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