Case Report
An institutional review board approval and informed consent were waived for this case report.
A 65-year-old male with a history of arrythmogenic right ventricular cardiomyopathy (ARVC) diagnosed 20 years ago.  He was managed with anti-arrhythmics and an AICD placed for primary prevention in 2001.  He presented with two month history of progressive fatigue and nausea.  He had an episode of multiple AICD firings at home secondary to monomorphic ventricular tachycardia (VT).  He underwent an unsuccessful VT ablation but was soon admitted to the hospital again with nausea, hypotension, and worsening right ventricular function.  Patient was initially managed on a telemetry floor but developed worsening end organ perfusion with hypotension, nausea, lethargy, along with a rise in his creatinine and transaminases necessitating transfer to an intensive care unit and initiation of inotropes.  Patient’s heart transplant evaluation had recently started prior to this decompensation and urgent heart team discussions were held to develop a plan about his transplant candidacy.  Given his rising pressor requirement and continued end organ dysfunction, the decision was made to place the patient on peripheral veno-arterial ECMO via his common femoral vessels and complete an expedited workup.  His right ventricular dysfunction did not allow for any approved single ventricular durable mechanical support and a total artificial heart was not feasible given his body habitus. End organ function improved with peripheral VA-ECMO, however the patient’s immobility and deconditioning set him up for aspiration pneumonitis and the decision was made to convert to an alternative approach that would allow for ambulation and physical rehabilitation.
We made a 3 cm skin incision starting at the level of the sternal notch and carried it down to the sternum.  A partial upper hemi-sternotomy was made and then teed off at the second intercostal space.  Particular attention was paid to remain extra-pericardial in order to avoid the risk of pericardial adhesions for his future transplant and potential tamponade on anticoagulation.  The patient was then heparinized to an ACT above 300 seconds and purse-string sutures were placed proximal to the innominate artery. Next, a Seldinger technique was used to cannulate the ascending aorta and the presence of wire into the descending aorta was confirmed with fluoroscopy.  The proximal end of the wire was brought out through a 1 cm stab incision above the right clavicle.  The tract was serially dilated and an 18Fr EOPA cannula was placed (Figure 1 ). For the venous access, a .035” guidewire was inserted in the right internal jugular vein. The position of the wire was confirmed with fluoroscopy and the tract was serially dilated to place a 27-French Avalon catheter.  Both lumens of the Avalon cannula were “Y-ed” together to provide inflow to the ECMO circuit and avoid stasis of either lumen of the cannula.  After proper de-airing, the arterial and venous cannula were connected to the ECMO circuit and flow was established.  The partial sternotomy was closed using two #6 sternal wires and the skin was closed in multiple layers.
Post-operatively, the patient was able to be extubated and ambulated with physical therapy.  He was treated for his combined pneumonitis and pneumonia with a course of antibiotics and pulmonary toilet before being successfully transplanted after three weeks of ECMO support. Intrapericardial adhesions at the time of operation were minimal and the heart transplantation was unremarkable.