An Electrical Storm9th May 2014

U Buckley, D Eaton, J Galvin, T KeelanMater Misericordiae Hospital, Dublin

• 55 year old female

• Atrial septal defect & mitral valve repair in 1990’s

• Out of hospital cardiac arrest 2011 on clarithromycin• AED torsade de pointe

• LV dysfunction – 40%

• Long QT on ECG at 490ms

• Fhx sudden cardiac death – 2 first degree relatives

• ICD – single chamber device implanted

• Left sided invasive ductal carcinoma requiring radiotherapy • Post operatively cardiac arrest requiring 6 shocks to defibrillate (ICD off for surgery)• Device repositioned & tunnelled lead to right

• Inappropriate therapy from the device Nov 2013• Reprogrammed• Increased meds• Rising threshold and drop in R wave

• Beta-blockade

• Appropriate therapy from the device Jan 2014

•Further 5 shocks from device

• First looked inappropriate• 4 others were appropriate

•Check Coronary Angiogram

•Medications optimized• Switched to propranolol• Mexilitene after loading with intravenous lignocaine

•Further fine ventricular fibrillation undetected by the device

•Atrial lead inserted & new RV lead

•Further Appropriate therapies

Video Assisted Transaxillary Transthoracic Left Cervical

Sympathectomy

• LCSD • lengthening repolarization• Prolong refractory periods• Increase VF threshold

• Defibrillation threshold testing was performed

• Genetic testing sent

• No Horner's Syndrome

• 8 weeks on and no further ventricular arrhythmias

• Natural history of electrical storm or success?

Management of Ventricular Arrhythmic Storms

1. Beta blockers2. Antiarrhythmics3. Reprogramme Device4. Manage reversible proarrhythmic causes5. Ablation6. Deep sedation/skeletal muscle relaxants7. Stellate ganglion blocking8. Cervical Sympathectomy

Neuromodulation

Presynaptic

Postsynaptic

Central inhibition

Sympathetic Activation

Previous Surgical Options

• Supraclavicular

• Open thoracotomy

• Posterior approach

• Chemical ablation

• Resection

How much of the chain is enough?

• T1-4

• C8

• Can the nerves grow back?

• Lessons from orthotopic heart transplantation

• Left versus bilateral sympathectomy

Surgical Failure

• Multiple shocks prior to surgery

• Decompensated end stage intractable heart failure

• Lack of sympathetic triggers

• Circulating catecholamines

• Failure to dissect the nerve of Kuntz