Case Report
A 36-year-old man with d-TGA and Mustard procedure at 15 months after birth and second operation with augmentation plasty for SVC stenosis at the age of 6 years was admitted to the emergency department for heart failure (HF) symptoms. On echocardiography, sRV was dilated (end-diastolic dimension 56mm) with a severely impaired ejection fraction (EF) of 15% and moderate regurgitation at systemic atrioventricular valve. Preoperative computed tomography showed anterior location of the aorta, regular anatomic relation of the Mustard baffle in the systemic atrium in close anatomic relation to the pulmonary valve, moreover the anterior wall of the systemic ventricle grossly adhering to the dorsal aspect of the sternum (Fig.1 A, B). Cardiac index was 1.65 l/min*m2 and initial lactate was 1.6 mmol/l. The patient was admitted to intensive care and high urgency (HU) status was granted. Fifty-two days after HU status, HTX was performed 35 years after Mustard procedure.
The operative strategy included femoral cannulation and cardiopulmonary bypass (CPB) initiation prior to re-sternotomy due to expected adhesions to sRV. After partial dissection of the adhesions, the systemic atrium was vented to avoid pulmonary congestion during further preparation and manipulation. Aortic clamping was performed early to avoid air embolism, and both caval veins were incised for later bicaval implantation of donor heart. Next, systemic (i.e., anatomic right) atrium was opened and Mustard baffle as well as pulmonary vein (PV) ostia were identified from the endocardial side as well as PV location from outside of the atrium (Fig.2). Aorta and pulmonary trunk were transected more distally than in regular HTX to achieve a more regular anatomic relation. The resulting recipient dimension of the aorta was remarkably small. Due to the specific technique of Mustard correction, the inter-PV distance revealed to be relatively small and the anatomic left atrial cuff limited in size when compared to the common anatomy in HF patients. Therefore, when excising recipient heart, the incision line was performed as much as possible distant from the PV ostia. Addition perpendicular incision of the remaining left atrial cuff was performed in between the two left PV ostia as well as caudally and cranially in between the left and right atrial PV ostia in order to enlarge the anastomotic line on the recipient side. As a further modification on the donor side, cardiac graft was harvested with a long segment of the aorta, including most part of the aortic arch. HTX was performed by bicaval method and the anastomosis was performed in the order of left atrium, IVC, SVC, pulmonary artery, ascending aorta. Despite the more liberal excision of recipient great arteries, the distal ascending aorta was yet located anteriorly and slightly left to the normal anatomy. Utilizing longer segments of the donor graft and more distal anastomotic lines, it was possible to perform both anastomoses of great arteries without the use of prosthetic materials. The aortic anastomosis was further complicated by a remarkable size mismatch but proved to be feasible without prosthetic material. Total donor heart ischemia time was 214 minutes. After 131 min of reperfusion, weaning from CPB was performed with moderate doses of catecholamines, inhalative nitric oxide, and intermittent inhalative prostacyclin therapy. The patient was extubated on 1st postoperative day and further postoperative course was unremarkable. There was no particular problem with postoperative echocardiography, and he was discharged on the 33rd postoperative day without any other complications.