Management of a young patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome with venous-venous extracorporeal membrane oxygenation and heart-lung transplantation

Abstract

Dextrocardia is a rare congenital condition which presents important challenges for surgical management. We discuss a patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome, which ultimately led to decompensated end-stage lung disease and heart-lung transplant. Venous-venous extracorporeal membrane oxygenation was an important strategy to bridge the patient until donor organs became available. Transplantation of a heart-lung block allowed for the treatment of the patient’s underlying congenital heart defect, anatomic reversal of dextrocardia with appropriate venous and arterial connections, and management of pulmonary damage from pulmonary hypertension.

1 ∣. INTRODUCTION

Dextrocardia is a rare congenital condition which presents important challenges for surgical management. End-stage lung disease in the setting of dextrocardia is particularly challenging to manage. On the one hand, it is often refractory to medical treatment and, on the other, conventional surgical techniques are not applicable.^1-4 While an appropriate management strategy entails heart-lung transplantation, the scarcity of such organs compels additional ways of managing the patient until the transplant. In the following, we report the case of a patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome, which resulted in decompensated end-stage lung disease, requiring venous-venous extracorporeal membrane oxygenation (V-V ECMO), and heart-lung transplant.

2 ∣. CLINICAL DESCRIPTION

A 29-year-old female with dextrocardia and an unrepaired secundum atrial septal defect presented with progressive dyspnea from severe pulmonary hypertension that was resistant to maximal medical management. Echocardiography showed severe right ventricular (RV) dilation with moderate impairment, and right-to-left shunting through the atrial septal defect. Right heart catheterization fixed systemic pulmonary artery (PA) pressures. Due to dextrocardia, left to right shunting across her autism spectrum disorder (ASD) with progressively worsening systemic hypoxia, and failure of medical management, heart-lung transplantation was deemed to be the most appropriate, definitive management. In addition, she had a left-sided aortic arch with venae cavae in the usual position (Figure 1). Once all medical options were exhausted, she was listed for a heart-lung transplant. Before a donor organ became available, she was admitted with worsening hypoxemia that was resistant to O₂ therapy due to right-to-left shunting. Therefore, she was placed on V-V ECMO via a right internal jugular Avalon cannula as bridge to transplantation. During this time, the patient was anticoagulated with heparin while on ECMO to a partial thrombin time (PTT) goal of 60 to 80 seconds. Donor organs became available 23 days later and she was taken for heart-lung transplantation (Figure 2). Since the patient did not tolerate weaning ECMO, she was anticoagulated to activated clotting time (ACT) greater than 400 seconds and transitioned to cardiopulmonary bypass with an aortic cannula and a temporary right atrial cannula before ECMO was stopped. Next, the Avalon cannula in the right internal jugular was removed. Then the superior vena cava was directly cannulated with a 24 Fr Pacifico cannula. The right atrial cannula was removed, and the inferior vena cava was directly cannulated with a 28 Fr Pacifico cannula to complete bicaval cardiopulmonary bypass. Following explantation of the heart-lung block (Figure 2), the heart-lung transplant was performed in the usual technique.⁵ The ascending aorta was deaired using an aortic root vent in the native aorta.⁶ Warm ischemic time was 20 minutes, and total ischemic time 222minutes. The patient was weaned from cardiopulmonary bypass without difficulty on moderate inotropic support. However, after further reperfusion, the patient became hypoxic despite maximal respiratory support with bronchoscopy revealing significant pulmonary edema. Therefore, we elected to use the cannulas that were already in place to support the patient with V-V ECMO. Postoperatively, the patient was not anticoagulated while on ECMO. Following aggressive diuresis, ECMO could be weaned on postoperative day 2 and the chest was closed.⁷ The rest of the postoperative course was relatively uncomplicated, and she was discharged to rehabilitation on postoperative day 16.

FIGURE 1.

Chest x-rays show dextrocardia (left) and that the aortic arch and venae cavae were in their usual position (right)

FIGURE 2.

Dextrocardia as seen from the head of the operating table before and after removal of the recipient heart-lung block

3 ∣. DISCUSSION

This case describes the management of a young patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome. Transplantation of a heart-lung block served to treat her underlying congenital heart, manage her pulmonary failure from pulmonary hypertension, and reverse the patient’s dextrocardia with appropriate venous and arterial connections.

Cardiac surgery in patients with dextrocardia of any type is scarcely reported. Dextrocardia is a rare condition which may lead to surgical challenges due to mirror image anatomy of associated vasculatures especially if associated with situs inversus. For instance, in the case of coronary artery bypass, surgical intervention is notably challenging due to anatomic reversal of coronary arteries.⁸ Previous reports of managing isolated dextrocardia and dextrocardia situs inversus with pulmonary failure have focused on heart-lung or double-lung transplantation.^1-4 Notably, the literature discusses associated anatomical challenges and appropriate surgical approaches. For instance, heart-lung transplantation poses challenges of the right atrial anastomosis among others.^2,3 This approach avoided the risk of mechanical complications from rotation or compression that may be associated with conventional lung transplantation.

Timely initiation and appropriate weaning of V-V ECMO were critical to the patient’s outcome. Given the significant right to left shunting that made her hypoxia-resistant to conventional therapy, V-V ECMO served the important function pre-operatively of bridging to transplantation. It was also significant postoperatively, as it was used to manage the ensuing pulmonary edema post-transplant. V-V ECMO functioned to limit the development of hypoxemia and improve clinical outcomes of pulmonary edema. This supports the use of V-V ECMO as an effective way to manage pulmonary edema, whether secondary to cardiogenic shock or inflammatory.⁹ In this manner, previous reports have supported the beneficial, and often critical, the role of V-V ECMO in transplantation of heart, lung, and liver.^10,11 This case illustrated how managing the complex pathology inherent in multiple congenital heart diseases could significantly benefit from this technology to improve clinical outcomes.

In summary, a desirable outcome was achieved following a combined heart-lung transplantation in a patient with the V-V ECMO despite a difficult clinical course, demonstrating the promise of such management approaches.