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Romano Ward Syndrome, a genetic disorder, causes fainting spells due to heart rhythm problems. This family study highlights its inheritance and effective treatment with beta-blockers.
Area of Science:
- Cardiology
- Genetics
- Neurology
Background:
- Syncopal attacks are often linked to cardiac arrhythmias.
- Romano Ward Syndrome is characterized by QT interval prolongation and loss of consciousness.
Observation:
- A three-generation family exhibited characteristic QT interval prolongation and alterations on ECG.
- Affected female members experienced recurrent episodes of syncope, not attributable to epilepsy.
- Ventricular arrhythmias were suspected as the cause of these syncopal episodes.
Findings:
- The observed symptoms align with Romano Ward Syndrome, excluding other congenital abnormalities like deafness.
- The study explored the syndrome's inheritance patterns and underlying pathogenetic mechanisms.
- Beta-blocker therapy (propranolol) successfully eliminated syncopal attacks in a younger patient.
Implications:
- This research underscores the importance of recognizing Romano Ward Syndrome in patients with unexplained syncope and ECG abnormalities.
- Effective management strategies, including beta-blocker medication, can significantly improve patient outcomes.
- Further research into pathogenetic mechanisms may reveal novel therapeutic targets for long QT syndrome variants.
Abstract:
About syncopal attacks caused by cardial arrhythmias had been frequently reported. In one family we found female members of three generations affected by characteristical prolongation and alteration of the QT-interval in ECG. All patients suffered from attacks with loss of consciousness which could not be defined as epileptic fits. Ventricular arrhythmias have to be considered as reason for the attacks. This combination of symptoms without additional malformations (i.e. congenital deafness) is well known in literature as Romano Ward Syndrome. Mode of inheritance, pathogenetic mechanisms and possibilities of therapy are discussed. Therapy witha beta blocking medication (propranolol) made our younger patient free of attacks, the other two patients have no complaints since two years without any therapy.