Case report
A successful cardiac transplant in a patient with situs inversus totalis and congenitally corrected transposition of the great arteries
A heart transplant is the gold standard therapy for patients with end-stage heart failure. In this case report, situs inversus totalis and congenitally corrected transposition of the great arteries led to a unique and complex preoperative setting. Extended donor organ harvesting, donor graft rotation of 45° to the right and post-operative stenting of the superior vena cava were essential steps in the interdisciplinary management of this case. The patient was transferred to the intensive care unit with moderate inotropic support. He was discharged to rehabilitation on postoperative day 89 and eventually underwent an additional renal transplant 14 months after the cardiac transplant.
Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart condition. Ventriculoarterial and atrioventricular discordance leads to the setting of a morphological right ventricle that functions as the systemic ventricle and an upstream tricuspid atrioventricular valve. Initially, congenital heart surgery is not mandatory. However, patients are at a high lifetime risk of developing severe tricuspid valve regurgitation, atrioventricular blocks and ultimately deterioration of the systemic ventricle [1]. If end-stage heart failure occurs, an orthotropic cardiac transplant remains a promising therapy. A series of successful cardiac transplants using similar techniques were first described by Doty et al. [2]. We demonstrate a case of a cardiac transplant in a 57-year-old male patient with CCTGA and situs inversus totalis (SIT). Essential pre-, intra- and post-operative steps are addressed and visually highlighted in this case report.
This case report describes a 57-year-old male patient with CCTGA and SIT who was admitted to our hospital with cardiac decompensation. His medical history included paroxysmal atrial fibrillation, severe tricuspid valve regurgitation and cardiac resynchronization therapy with a defibrillator (CRT-D) implanted 8 years prior to this hospital admission. At a cardiac index of 1.5 L/min×m2 with recurrent tachycardia and CRT-D shock therapy despite the intravenous administration of amiodarone, the patient was granted high-urgency status on the heart transplant waiting list.
The donor organ was harvested together with the supra-aortic vessels and the innominate vein. The implant was performed using the bicaval technique, and the donor graft was rotated 45° to the right to maintain dextrocardia. The orifices of the left pulmonary vein of the donor were oversewn. The left atrium of the recipient was anastomosed with a surgically enlarged cuff of the right pulmonary veins of the donor graft. Anastomoses of the inferior vena cava, the pulmonary trunk and the aorta were performed consecutively. The donor’s innominate vein was used as a natural extension of the donor’s superior vena cava (SVC) and connected to the left-sided recipient SVC during reperfusion.
The patient was transferred to the intensive care unit with moderate inotropic support. Clinical signs of SVC syndrome required stenting of the innominate vein on POD 1. Re-thoracotomy was necessary due to cardiac tamponade on POD 7, and extubation was successful on POD 8. Acute-on-advanced chronic renal failure required continuous haemodialysis. He was transferred to the intermediate care unit on POD 19 and discharged to rehabilitation on POD 89.
1 - Patient Presentation (0:12)
A 57-year-old male patient with known CCTGA and SIT was admitted to our hospital with cardiac decompensation. Echocardiography showed severe deterioration of the systemic ventricle (cardiac index: 1.5 L/min×m2) and severe tricuspid valve regurgitation (tricuspid regurgitation jet maximal pressure gradient: 43.2 mmHg). Additionally, he suffered from therapy-resistant ventricular tachycardia. The patient was granted high-urgency status on the heart transplant waiting list.
2 - Cannulation strategy and excision of the recipient heart (1:13)
Percutaneous femoral cannulation was performed with point-of-care ultrasound. An additional venous cannula was inserted in the internal jugular vein.
First, the SVC, the inferior vena cava and the aorta were identified, marked and cross-clamped. Subsequently, the right atrium was partially excised, and a remnant of the dorsolateral right atrial wall was left in situ. The atrial wires could be cut and recovered. The aorta and pulmonary artery were incised just above the valvular apparatus. The left atrium was excised thereafter.
3 - Preparation of the donor heart and four consecutive anastomoses (2:50)
The left atrium of the recipient was downsized with a purse-string suture, and the orifices of the donor left pulmonary vein were oversewn. A surgically enlarged cuff of the right pulmonary veins of the donor graft was then anastomosed to the left atrium. The donor graft was rotated 45° to the right. Anastomoses of the inferior vena cava, pulmonary trunk and aorta were performed consecutively.
4 - Arrangement of the innominate vein and the last anastomosis during reperfusion (5:08)
The great arteries passed in front of the innominate vein. The patient’s SVC on the left was then anastomosed with the donor innominate vein during reperfusion. Reperfusion lasted 48 minutes in total; aortic cross-clamping, 83 minutes; extracorporeal circulation, 191 minutes; and total duration of surgery, 440 minutes, respectively.
5 - Postoperative course until discharge (5:33)
The patient was transferred to the intensive care unit with moderate inotropic support. On POD 1, the innominate vein was stented to protect against collapse. A revision was necessary due to a haemothorax without a specific source of bleeding on POD 7. The patient was extubated a day later, transferred to the intermediate care unit on POD 19 and discharged to rehabilitation on POD 89.
A heart transplant in a patient with CCTGA and SIT with dextrocardia is feasible. Similar techniques were first reported by Doty et al. [2]. In addition, the Stanford group published satisfactory long-term results [3,4]. Donor hearts can only be considered if there is no concomitant lung explantation. In addition, the donor innominate vein must be long enough to cross over behind the great arteries to be anastomosed to the recipient’s left-sided SVC. This segment should be carefully monitored postoperatively. Signs of superior or inferior vena cava syndrome must be taken seriously. Despite the possible necessity of SVC stenting, there is no need for excision of the pericardium intraoperatively to maintain dextrocardia and therefore no risk of phrenic nerve injury. In summary, the surgical technique including the harvesting of the donor graft as well as the perioperative management is a challenge that must be carefully discussed prior to the onset of the organ donation process.
1. Kumar TKS. Congenitally corrected transposition of the great arteries. J Thorac Dis, 2020;12:1213–8.
PubMed Abstract | Publisher Full Text
2. Doty DB, Renlund DG, Caputo GR, Burton NA, Jones KW. Cardiac transplantation in situs inversus. J Thorac Cardiovasc Surg 1990;99:493–9.
PubMed Abstract | Publisher Full Text
3. Reinhartz O, Ma M, Hollander SA, Maeda K. Heart Transplantation in Situs Inversus Maintaining Dextrocardia. Operative Techniques in Thoracic and Cardiovascular Surgery 2018;23:34–9.
NA | Publisher Full Text
4. Deuse T, Reitz BA. Heart transplantation in situs inversus totalis. J Thorac Cardiovasc Surg 2010;139:501-3.
Sebastian Johannes Bauer1,2, Hug Aubin1, Raphael Bruno3, Moritz Benjamin Immohr1,2, Yukiharu Sugimura1,2, Arash Mehdiani1,2, Payam Akhyari1,2, Udo Boeken1 & Artur Lichtenberg1
1Department of Cardiac Surgery, Medical Faculty, Heinrich-Heine-University, Duesseldorf, Germany
2Department of Cardiac Surgery, University Hospital RWTH Aachen, Aachen, Germany
3Division of Cardiology, Pulmonology and Vascular Medicine, Medical Faculty, Heinrich-Heine-University, Duesseldorf, Germany
Corresponding author
Payam Akhyari
Department of Cardiac Surgery
University Hospital RWTH Aachen
Pauwelsstraße 30
52074 Aachen
Germany
E-Mail: pakhyari@ukaachen.de
© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
