Study of left and right ventricular function in Romano-Ward syndrome

Area of Science:

• Cardiology
• Genetics
• Nuclear Medicine

Background:

• Romano-Ward syndrome is an inherited disorder associated with prolonged QT intervals and increased risk of sudden cardiac death.
• The underlying cardiac pathology and its contribution to arrhythmia predisposition are not fully understood.

Purpose of the Study:

• To investigate left and right ventricular contraction patterns in individuals with Romano-Ward syndrome.
• To determine if myocardial abnormalities are associated with an increased risk of arrhythmias in these patients.

Main Methods:

• Tomographic radionuclide ventriculography was employed to assess ventricular function.
• Red blood cells were labeled with technetium-99m stannous pyrophosphate for imaging.
• Eight subjects from two families with Romano-Ward syndrome were studied.

Main Results:

• Family 1 showed normal left ventricular contraction, with subtle right ventricular phase abnormalities in two subjects.
• Family 2 exhibited abnormal left ventricular contraction (reduced amplitude and/or abnormal phase) in all three subjects.
• All subjects across both families displayed subtle right ventricular phase abnormalities.

Conclusions:

• Abnormalities in either right or left ventricular myocardium may contribute to electrocardiographic repolarization changes in Romano-Ward syndrome.
• These myocardial abnormalities could serve as an ectopic focus, initiating ventricular tachycardias in susceptible individuals.