Discussion
Over the past 20 years, the number of HLTx has been decreasing from 145
in 1999 to 45 in 2019 and gradually replaced with bilateral lung
transplant (BLTx).4 It has been an ongoing debate on
whether to perform HLTs or BLTx for patients with PH.5HLTx is recommended to patients with congenital heart disease and
Eisenminger syndrome, severe right ventricular (RV) dysfunction, and
severe left ventricular dysfunction. On the other hand, BLTx provides
comparable outcomes, moreover, it offers the advantage of better organ
sharing. Given the long waiting time, patients with PH and simple
cardiac anomalies such as atrial septal defect, patent ductus arteriosus
or perimembranous ventricular septal defect can be successfully managed
with a combination of BLTx and cardiac repair. The cut-off values for
BLTx can be approximated at 10-25% RVEF and 32-55% LVEF. For values
less than this cut-off, HLTx is recommended.5
We are reporting a successful HLTx in a patient with cc-TGA and
dextrocardia. Our patient was presented with advanced cardiac problems
including severe PH, severe systemic and sub-pulmonary atrioventricular
valves regurgitation, complete heart block and biventricular failure. In
our patient, the RV was the systemic ventricle and its failure
attributed to the severely incompetent systemic valve and the afterload
systemic pressure. Hence, BLTx will not improve the condition of the
failing systemic RV and the HLTx was deemed the suitable option. Based
on the aforementioned findings, the multidisciplinary team recommended
HLTx procedure. HLTx is technically a well-established surgical
procedure, however, our case demonstrated unique surgical challenges due
to the complexity of the congenital cardiac anomaly (cc-TGA and
dextrocardia) of the recipient. The combination of the cc-TGA and
dextrocardia is extremely rare. To the best of our knowledge, only one
case of cc-TGA and dextrocardia has been reported by Takemoto et al. in
the literature which was managed medically (i.e. no
HLTx).6 This combination posed challenges for both the
surgeon and anesthesiologist during the preoperative preparation and
during the transplant procedure. The strategy of the approach,
cannulation, and the technique was quite different from the standard
HLTx. Giving the fact that both the superior and inferior vena cava were
presented posteriorly, venous cannulation was initiated via dual stage
venous cannula placed in the right atrium transitioned to bi-caval
configuration after decompressing the heart.
According to the 2016 ISHLT report included the data of patients
underwent HLTx in 2004–2014, the survival rates at 1, 2, 5, and 10
years are 63%, 52%, 45%, and 32%, respectively, and a median
survival of 5.8 years.7 As of today, 15 months
postoperatively, the patient is doing well.