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Congenital complete atrioventricular block in an infant with long QT syndrome
1Department of Paediatric Cardiology, Ankara University Faculty of Medicine, Turkey.
Insights
A three-month-old infant with congenital complete atrioventricular block and prolonged QT interval syndrome experienced syncope and life-threatening arrhythmias. Treatment included pacemaker implantation and propranolol, resulting in a symptom-free recovery.
Area of Science:
- Pediatric Cardiology
- Electrophysiology
- Congenital Heart Disease
Background:
- Congenital complete atrioventricular block (CCAVB) is a rare condition.
- Prolonged QT interval syndrome (LQTS) increases the risk of malignant arrhythmias.
- CCAVB and LQTS in infants present significant management challenges.
Observation:
- A 3-month-old infant presented with recurrent syncopal episodes.
- The patient developed torsade de pointes (TdP) and ventricular fibrillation (VF) during hospitalization.
- These events occurred in the context of CCAVB and LQTS.
Findings:
- Successful cardiopulmonary resuscitation (CPR) was performed.
- A permanent epicardial ventricular pacemaker was implanted.
- The patient was discharged on propranolol therapy.
Implications:
- This case highlights the successful management of a complex pediatric cardiac condition.
- Combined CCAVB and LQTS requires aggressive arrhythmia management.
- Long-term follow-up is crucial for patients with congenital heart block and channelopathies.
Abstract:
A three months old patient with congenital complete atrioventricular block and prolonged QT interval syndrome is presented. Before admission she had had two syncopal attacks. During hospitalization she had an episode of torsade de pointes which was followed by ventricular fibrillation. After a successful cardiopulmonary resuscitation, a permanent epicardial ventricular pacemaker was implanted and she was discharged on propranolol therapy. She is now free of symptoms and doing well, 16 months after discharge.