Internal Medicine Resident Université de Montréal Montreal, Quebec, Canada
Case background: Introduction : Managing advanced heart failure in adults with complex congenital heart disease and failing Fontan circulation presents increasing challenges. These patients often have a history of multiple surgeries and face technical hurdles at transplant, including vascular reconstruction, collateral management, and coagulopathy. Situs inversus totalis—a rare condition with complete organ reversal—further complicates implantation due to dextrocardia and reversed chamber orientation, making standard donor placement difficult. This clinical case describes a successful dextrocardia preserving orthotopic heart transplant in an adult patient with situs inversus totalis and an extracardiac Fontan conduit.
Case summary : A 27-year-old woman with complex congenital heart defects was admitted because of heart failure due to a failing Fontan circulation from systemic RV dysfunction. Her history includes situs inversus totalis, an unrepaired right-dominant complete atrioventricular septal defect, severe atrioventricular valve regurgitation, pulmonary stenosis, levo-transposition of the great arteries, and small venous and aortopulmonary collaterals. The cardiac anatomy consisted of a hypoplastic LV and a systemic RV. During infancy, the patient underwent pulmonary valvulotomy and a Blalock-Taussig shunt, followed by a hemi-Fontan procedure at age 2. At age 4, she received a fenestrated extracardiac Fontan, later occluded with an Amplatz device. The patient’s cardiac anatomy is illustrated in Figure 1. Over time, she developed refractory RV failure and Fontan-associated hepatopathy without cirrhosis or indication for liver transplantation. She had multiple admissions for decompensated heart failure. Clinical signs included jugular venous distention, peripheral edema, and rales. Despite GDMT, she remained symptomatic with impaired quality of life. As no other therapeutic option, she was ultimately listed for heart transplantation.
Heart Transplant Technique : The donor's heart was harvested with the innominate vein and aortic arch to facilitate vascular reconstruction. Redo sternotomy was performed. CBP was then established with central aortic and left SVC cannulation. The lower body was drained with peripheral venous cannulation advanced to the IVC through the left femoral vein. The lateral tunnel Fontan was proximally detached from the left PA and distally cut 3 cm above the diaphragm. The recipient’s left SVC was detached from the extracardiac Fontan. Cardectomy was performed, leaving a left atrium cuff in place. The heart was rotated approximately 90 degrees clockwise (to the patient's right), to preserve the dextrocardia. The donor left atrium was anastomosed with the recipient’s left atrium cuff. To facilitate venous rerouting, the donor's heart was rotated approximately 90 degrees counterclockwise along its longitudinal axis, positioning the LV anteriorly and the RV posteriorly. The donor IVC was anastomosed with the remaining recipient Fontan conduit graft. Because of the rotation, the main PA was too short to reach the recipient’s main PA so a 3 cm piece of the donor's aorta was used to extend the donor's main PA that is sewn to the recipient's main PA. The donor's innominate vein was anastomosed to the recipient's left SVC. Due to the counterclockwise rotation of the donor heart, the neo-vena cava passed between the PA and the ascending aorta, placing it at risk of external compression. To address this, the donor's innominate vein was externally wrapped with a 22 mm reinforced GORE-TEX® graft to provide structural support and minimize compression risk. Finally, an end-to-end anastomosis of the donor and recipient aorta was done, and the heart was reperfused (Figure 2).
Outcome and Follow-up post-transplant: The patient was hospitalized for 36 days postoperatively due to complications, including acute tubular necrosis requiring prolonged intermittent dialysis, Klebsiella pneumonia, and moderate transient graft dysfunction requiring inotropes for 6 days. Pre-discharge echocardiography showed normal graft function, with a LVEF of 65%, normal RV dimensions and a CVP of 3mmHg. The patient was discharged on immunosuppressive therapy (mycophenolic acid, tacrolimus, and prednisone). At 17 months post-operatively the patient has no graft rejection, and the echocardiography revealed a normal LV with an ejection fraction of 60%, normal RV dimensions and function. CVP remained unchanged. The patient remained asymptomatic, with a NYHA class of I.
Management Challenges: This case report demonstrates that patients with situs inversus, dextrocardia, and extracardiac Fontan can still successfully undergo heart transplant surgery with a normal donor heart.
The most challenging aspect of the surgery involves reconstructing the pathways for blood flow, both arterial and venous, to align with the patient's unique anatomy. Both intracardiac and extracardiac channels for rerouting systemic venous return into the donor right atrium have been previously described.
We chose to utilize the pre-existing Fontan conduit to minimize additional foreign body introduction, infection risk, and potential complications associated with additional anastomoses, including stenosis and bleeding. This strategy not only saved time but also reduced both CBP and ischemic time. It also extended the recipient's IVC and avoids kinking of the vessel.
Levocardia was avoided in our case due to the higher risk of compression and tamponade of the RV. Additionally, it would have required resection of the left pericardium, posing risks such as phrenic injury. To maintain dextrocardia and facilitate inferior venous connection, a counterclockwise rotation can be done, placing the LV anteriorly and the RV posteriorly.
This is the first clinical case in litterature utilizing a Gore-Tex® graft to externally reinforce the donor innominate vein as it travels between the Aorta and the main PA, where it is at risk of compression. Previous described techniques in the litterature described routing the the innominate vein anterior to the Aorta, but this proved to cause venous stenosis and obstruction also requiring Gore-tex® grafts. In this patient, routing of the innominate vein anterior to the aorta was avoided due to limited anterior mediastinal space, a small body habitus, and the proximity of the aorta to the sternum, all of which increase the risk of external compression of the innominate vein.
While prior reports used extra aortic tissue during heart harvesting to allow mild aortic bowing and avoid innominate vein obstruction, our case demonstrates that this is not mandatory: safe and effective superior venous reconstruction can still be achieved with a reinforced Gore-Tex® graft.
