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[Prolonged Q-T syndrome (Romano-Ward syndrome). Description of a case diagnosed in infancy]
Insights
Romano-Ward syndrome, a type of prolonged Q-T interval without deafness, can cause syncopal events. Propranolol effectively treated a 2-month-old infant experiencing these symptoms.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Prolonged Q-T interval syndrome (LQTS) is a disorder of cardiac repolarization.
- Romano-Ward syndrome is an inherited LQTS subtype characterized by cardiac arrhythmias and syncope, but without congenital deafness.
- Early diagnosis and management are crucial for preventing life-threatening events.
Observation:
- A 2-month-old female infant presented with a syncopal attack, a near-miss event.
- Electrocardiogram (EKG) revealed a significantly prolonged Q-T interval, indicative of LQTS.
- No auditory deficits were noted, consistent with Romano-Ward syndrome.
Findings:
- The infant was diagnosed with Romano-Ward syndrome.
- Treatment with propranolol was initiated.
- The therapy successfully resolved the syncopal episodes and corrected the prolonged Q-T interval.
Implications:
- This case highlights the importance of recognizing LQTS in infants presenting with syncope.
- Propranolol demonstrates efficacy in managing Romano-Ward syndrome in early infancy.
- Prompt therapeutic intervention can prevent adverse cardiac events and improve outcomes in affected infants.
Abstract:
A case of prolonged Q-T interval syndrome without deafness (Romano-Ward syndrome) is reported. A 2-month-old female was seen in consultation because of a near-miss event (syncopal attack). An EKG showed a long Q-T interval. Successful therapy was achieved with propanolol.