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.