Cardiac Transplantation in a Patient With Situs Inversus Totalis and Extracardiac Fontan

Abstract

Objective

This case report describes a successful dextrocardia-preserving orthotopic heart transplant in a patient with situs inversus totalis and an extracardiac Fontan, utilizing a levocardia donor heart.

Key Steps

The donor heart was rotated 90° counterclockwise along its longitudinal axis to optimize inferior venous connection and incorporate existing extracardiac Fontan. Superior venous connections were secured using a harvesting technique preserving the innominate vein. A GORE-TEX graft was wrapped around the innominate vein to prevent its compression as it passed through the aortic arch.

Potential Pitfalls

Counterclockwise rotation requires adequate pulmonary artery length. In the absence of extra aortic tissue to allow mild aortic bowing and prevent superior venous obstruction, a GORE-TEX graft offers an effective alternative to maintain unobstructed superior venous drainage.

Take-Home Messages

Dextrocardia-preserving transplantation is feasible with a 90° counterclockwise rotation. Reusing the extracardiac Fontan reduces ischemic time; reinforcing the innominate vein with GORE-TEX prevents compression, securing venous integrity.

Graphical Abstract

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. Few cases of heart transplant in situs inversus have been previously published,1, 2, 3, 4, 5, 6 and mismatch between donor and recipient extracardiac and intracardiac channels is variable and makes each patient unique. Therefore, these cases highlight the need for meticulous preoperative planning and a highly skilled surgical team to maximize patient outcomes. This report describes a successful dextrocardia preserving orthotopic heart transplant in an adult patient with situs inversus totalis and an extracardiac Fontan conduit.

Take-Home Messages

• •Dextrocardia-maintaining heart transplantation using a levocardia donor heart is feasible with a 90° counterclockwise rotation, ensuring proper anatomical alignment for venous and arterial connections.
• •Reutilizing the existing extracardiac Fontan for the inferior venous connection significantly reduces both cardiopulmonary bypass and ischemic time, while reinforcing the innominate vein with a GORE-TEX graft prevents external compression and provides a secure, innovative solution for maintaining venous integrity.

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 right ventricular (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, some coil embolized years earlier. The cardiac anatomy consisted of a hypoplastic left ventricle (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 optimal therapy, she remained symptomatic with impaired quality of life. As no other therapeutic option, she was ultimately listed for heart transplantation. A suitable donor was eventually found, and she was admitted for transplant (see heart transplant technique section below).

Figure 1.

Preoperative Cardiac Anatomy

Note the systemic dilated RV and the hypoplastic LV, with the extracardiac Fontan draining into the left pulmonary artery. (A) Illustration of the patient's cardiac anatomy before surgery, and pre-transplantation (B) chest X-ray and (C) coronal-view CT scan, respectively, showing situs inversus and dextrocardia. AO = aorta; CAVV = common atrioventricular valve; CT = computed tomography; IVC = inferior vena cava; LA = left atrium; LPA = left pulmonary artery; LV = left ventricle; PA = pulmonary artery; RA = right atrium; RPA = right pulmonary artery; RV = right ventricle; SVC = superior vena cava; VVC = veno-venous collateral.

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 and vasopressors for 10 days. On postoperative day 24, a noncompressive 41-mm pericardial effusion was noted and drained percutaneously without recurrence. Predischarge echocardiography showed normal graft function, with an LV ejection fraction of 65%, normal RV dimensions, central venous pressure of 3 mm Hg, and no signs of compression. Mild tricuspid regurgitation was noted, but no inferior vena cava (IVC) dilation or leakage at the anastomoses. The patient was discharged on immunosuppressive therapy (mycophenolic acid, tacrolimus, and prednisone). At 17 months postoperatively, the patient has no graft rejection, and the echocardiography revealed a normal LV with an ejection fraction of 60% and normal RV dimensions and function. Central venous pressure remained unchanged, and tricuspid valve regurgitation remained stable. The patient remained asymptomatic, with an NYHA functional class of I.

Heart Transplant: Procedural Steps

The donor's heart was procured with the innominate vein and aortic arch to facilitate vascular reconstruction. An uncomplicated redo sternotomy was performed. Cardiopulmonary bypass was then established with central aortic and left superior vena cava (SVC) cannulation before the arrival of the cardiac graft. 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 pulmonary artery (PA) and distally cut 3 cm above the diaphragm, as the remaining conduit was used to extend the recipient IVC to the donor's right atrium. The recipient's left SVC was detached from the extracardiac Fontan. The cardectomy was then performed, leaving a left atrium cuff in place. Due to limited space in the left pericardium as a result of a prominent left-lung and strong mediastinal adhesions, we opted to rotate the heart's apex approximately 90° clockwise (to the patient's right), to preserve the dextrocardia. Afterward, the donor's left atrium was anastomosed regularly with the recipient's left atrium cuff. To facilitate venous rerouting, the donor's heart was rotated approximately 90° counterclockwise along its longitudinal axis, positioning the LV anteriorly and the RV posteriorly. Subsequently, the IVC was anastomosed with the remaining Fontan conduit graft. The extra length from the Fontan graft facilitated this anastomosis by bringing both structures together without any kink. Because of the rotation, the main PA was too short to reach the recipient's main PA. To solve this, 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. Subsequently, we performed the anastomosis of the donor's innominate vein 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. Only the innominate vein itself was directly anastomosed to the recipient's left SVC. The graft served purely as a supportive external wrap and did not extend sufficiently to be included in the suture line between the innominate vein and the left SVC. Finally, we completed the end-to-end anastomosis of the donor and recipient aorta, and the heart was reperfused (Figure 2). The total extracorporeal circulation time was 231 minutes, the total ischemic time was 171 minutes, and the estimated blood loss was approximately 1,000 mL. The intraoperative echocardiography confirmed unobstructed flow through all anastomoses. Please refer to Figure 3 for a comparison of the pretransplant and posttransplant electrocardiograms (ECGs).

Figure 2.

Post-Transplantation Cardiac Anatomy

Note the preserved dextrocardia with the counterclockwise heart rotation, the recycled Fontan conduit, and GORE-TEX wrap around the innominate vein. (A) Illustration of the cardiac transplant technique. Post-transplantation (B) chest X-ray and (C) coronal-view CT scan, respectively, showing preserved dextrocardia. D = donor; R = recipient; CT = computed tomography; AO = aorta; IV = innominate vein; IVC = inferior vena cava; LPA = left pulmonary artery; PA = pulmonary artery; PV = pulmonary veins; RA = right atrium; RPA = right pulmonary artery; SVC = superior vena cava; LSVC = left superior vena cava.

Figure 3.

Pretransplant and 17-Month Post-Transplant ECGs

Note the extreme QRS axis deviation in the pretransplant ECG due to standard lead placement instead of reversed lead placement to accommodate dextrocardia. In the post-transplant ECG, with correctly reversed leads, the QRS axis is approximately +120° according to the new lead placement. (A) Pretransplant ECG. (B) Seventeen-month post-transplant ECG. ECG = electrocardiogram.

Discussion and Potential Pitfalls

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 intracardiac¹ and extracardiac² 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 cardiopulmonary bypass and ischemic time. It also extended the recipient's IVC and avoids kinking of the vessel. While not a novel concept, Deng et al³ successfully employed a similar approach, although they utilized a lateral Fontan conduit.

Other authors explored an alternative approach to eliminate the need for artificial or autogenous conduits by rotating the heart counterclockwise to establish the inferior venous connection.^4,5 Our technique for maintaining dextrocardia and for establishing venous connections through counterclockwise rotation of the donor heart aligns with that described by other authors.^4,5

Levocardia was avoided in our case due to the higher risk of compression and tamponade of the RV. In addition, it would have required resection of the left pericardium, posing risks such as phrenic injury.

To the best of our knowledge, we are the first to use a GORE-TEX graft to externally reinforce the donor innominate vein as it travels posterior to the ascending aorta, where it is at risk of compression. 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 if routed anteriorly. Beiras-Fernandez et al⁶ opted for routing the donor SVC anterior to the aorta, this resulted in proximal SVC stenosis also requiring GORE-TEX graft placement on postoperative day 2. This outcome suggests that anterior routing may not entirely eliminate the risk of venous compression. In addition, following counterclockwise rotation of the donor heart to preserve dextrocardia, the anatomical alignment between the donor innominate vein and the recipient's left SVC naturally favored routing the vein posterior to the aorta. This trajectory enabled a more direct and tension-free anastomosis. Moreover, after this rotation, the donor innominate vein lacked sufficient length to safely route anteriorly to the aorta. It should be noted that Deuse and Reitz⁴ used a similar technique to ours but did not use GORE-TEX on the superior venous connection as they used extra tissue on the aorta during heart harvesting, allowing for mild ascending aortic bowing and thus avoiding SVC obstruction. However, our approach proves that extra tissue on the aorta is not an absolute necessity, and the superior venous connection can still be effectively and safely secured with a GORE-TEX graft.

Conclusions

This report documents the successful and secure orthotopic heart transplant in an adult patient with situs inversus totalis, complex congenital heart disease, and an extracardiac Fontan conduit. Our method highlights the safety and practicality of preserving dextrocardia while incorporating the existing extracardiac Fontan conduit into the transplant procedure.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.