Materials and Methods
A 53-year-old male patient presented to our institution with the clinical picture of biventricular failure. The echocardiogram revealed cc-TGA, dextrocardia with situs solitus, atrioventricular discordance and ventriculoatrial discordance (figure 1A&B), severe systemic (morphologic tricuspid valve) and sub-pulmonary atrioventricular (morphologic mitral valve) valves regurgitation, and severe pulmonary hypertension (PH) (mean pulmonary artery pressure: 51 mmHg). His past medical history was remarkable for complete heart block mandating pacemaker implantation, and atrial fibrillation. The patient was evaluated and was deemed a candidate for HLTx. A 25-year-old male donor who died of hemorrhagic stroke became available and the en-bloc heart-lung was recovered by an experienced team (figure 2A).
The patient underwent HLTx via a clamshell incision. Because of hemodynamic instability, we commenced cardiopulmonary bypass (CPB) with an aortic cannula and a dual-stage venous cannula placed in the right atrium. After the heart was decompressed, the venous cannulation was converted from atrial into bi-caval configuration.
Cardiectomy was performed, including complete removal of right and left atria, leaving a stump of atrial tissue on the inferior vena cava. The recipient trachea was resected one tracheal ring above the carina. The left recurrent laryngeal and the vagus nerves were identified and preserved during dissection. The two phrenic nerves were identified and protected by leaving pericardial flaps on each side. Tracheal anastomosis was performed first followed by the IVC, aorta and then the superior vena cava. The aortic cross clamp was removed and the patient was safely weaned from the CPB. Inotropic support was initiated using Dobutamine at 5 mcg/kg/min and epinephrine at 0.05 mcg/kg/min. At the end of the procedure, the patient was taken to the intensive care unit in a stable condition.
The ischemic time was 296 minutes The CPB time was 280 minutes.
The standard immunosuppression protocol was initiated:
1- Methylprednisone 1 g IV intraoperatively, then 125 mg bid for the first day, then 1mg/kg/day in divided doses for 3 days then prednisone 20 mg daily for 4 weeks then 10 mg daily.
2- Mycophenolate 1g IV intraoperatively, followed by 500 mg bid. Dose was temporarily increased to 1000 mg bid on POD 18 with suspicion of humoral rejection.
3- Single dose induction with Alemtuzumab 30 mg IV administered on POD 1.
4- Tacrolimus 0.15mg/kg/day dose titrated to achieve level of 6-10 mcg/L.
On postoperative day 2 the patient was extubated and ambulation was initiated. The patient progressed steadily and ready for discharge, however on POD 18, he developed pleural effusion and lung infiltrates. Transbronchial lung biopsy revealed focal vasculitis. The diagnosis of possible humoral rejection was considered and the patient received a single dose of 500 mg, methylprednisone and 5 daily sessions of plasma exchange. Also he developed gastroparesis which completely resolved before discharge. Postoperative chest radiograph showed the heart and lungs in the proper position (figure 2B).