14 year old girl found to have a secundum ASD. Was posted for surgery. On induction patient developed a hemodynamically unstable SVT not responding to cardioversion/drugs and required emergent sternotomy and CPB for hemodynamic collapse. SVT did not terminate with cardioversion on CPB and with drug loading on CPB (cordorone). On opening the chest the RA appendage was found to be absent, fatty infiltration was seen over the RA and the RA wall was found to be thickened (approx 9 mm) as seen on a biopsy specimen. The heart rate was around 230 and not getting terminated with adenosine or cardioversion on CPB so I presumed it was some sort of flutter but was unable to make out the location on the monitor ECG . The left atrial wall was also thick so I arrested the heart , did a biatrial emaze with specific attention to the Isthmic and coronary sinus burn to eliminate a major flutter cycle.ASD was closed with a Goretex patch.There was a small rudimentary appendage "nubbin" seen within the RA which did not manifest as an appendage externally.
Patient came out with sinus rhythm and has persisted to be in sinus rhythm with no ectopics on 24 hr analysis.
The plan is to withdraw cordorone at 3 months and do a Holter sos EP study then. My electrophysiologist feel that since the burns are good nothing else may be required.
The pctures clearly show the fatty infiltration and actually the fatty extension from the sinus node was extensive and well seen .
Histopath shows fatty infiltration and otherwise nothing else remarkable. (Click on the image to see a larger version)
