Multimodal Imaging For Planning Closure Of Ventricular Septal Defect In a Patient With Ebstein’s Anomaly And Post Cardiotomy Shock Assisted With Venoarterial ECMO. The Role Of 3D Printing In Complex Scenarios

doi:10.21203/rs.3.rs-4708232/v1

Download PDF

Case Report

Multimodal Imaging For Planning Closure Of Ventricular Septal Defect In a Patient With Ebstein’s Anomaly And Post Cardiotomy Shock Assisted With Venoarterial ECMO. The Role Of 3D Printing In Complex Scenarios

https://doi.org/10.21203/rs.3.rs-4708232/v1

This work is licensed under a CC BY 4.0 License

Version 1

posted

You are reading this latest preprint version

We present the case of a 19-year-old patient with a history of Ebstein’s anomaly who underwent Da Silva’s cone repair. However, as an immediate complication, she developed post-cardiotomy cardiogenic shock syndrome, requiring venoarterial ECMO circulatory support. She remained completely dependent on circulatory support due to right ventricular (RV) dilation and akinesia, which caused compression of the left ventricle (LV). Therefore, the decision was made to exclude the RV with Starnes surgery in order to reduce dilation and improve LV ventricular function by resolving the ventricular interdependence. Due to RV extreme dilation, an apical ventricular septal defect (VSD) developed as a complication, with a left-to-right shunt perpetuating the RV dilation. However, due to the high surgical risk, percutaneous closure was planned. Different multimodal imaging techniques were used, along with the reconstruction and printing of a 3D model, to successfully plan the percutaneous closure of the VSD.

Multimodality imaging

congenital heart disease

3D printing

In patients with complex heart anatomy, the use of advanced imaging techniques, such as 3D reconstruction and cardiac tomography, can be very useful to assess and plan the interventions.
This case demonstrates the feasibility of percutaneous closure of a challenging apical ventricular septal defect, emphasizing the role of innovative approaches in improving patient outcomes.

Multimodality imaging using multiple noninvasive imaging techniques, allows anatomical, morphological, and functional data to be combined, increasing diagnostic accuracy, and improving the efficacy of interventions and clinical outcomes. Furthermore, novel imaging modalities have been implemented to planning cardiovascular interventions, in this case report we highlight the relevance of the multimodality imaging and the usefulness of the 3-dimensional (3D) as an emergent tool to improve the outcomes in complex

We present the case of a 19-year-old woman with history of Ebstein's anomaly, who was scheduled for a Da Silva's cone repair. The preoperative transthoracic echocardiogram documented Dilated RV. The morphology of the tricuspid valve was observed with elongated anterior leaflet, multiple fenestrations, and multiple regurgitant jets. The posterior leaflet was displaced towards the apex, with a 75% septal leaflet coaptation percentage. The percentage of right ventricle atrialization encompassed nearly the entire cavity. Reduced longitudinal and circumferential systolic function of the right ventricle was documented.

During the first 2 postoperative hours, she developed refractory low cardiac output syndrome after cardiopulmonary bypass, systolic dysfunction, and severe right ventricular dilation with ventricular interdependence phenomenon. It was decided to initiate venoarterial extracorporeal membrane oxygenation (VA-ECMO) through peripheral cannulation.

After 5 days, ECMO removal was attempted but unsuccessful, so the patient underwent a Glenn procedure with a new placement of central cannulation for VA-ECMO support. After 24 hours, she continued to be completely dependent on circulatory assistance with right ventricular dilation and akinesia, leading to the decision to exclude the right ventricle with Starnes surgery. The goal was to decrease right ventricular dilation and improve left ventricular function by solving ventricular interdependence.

During the evaluation with transesophageal echocardiography (TEE), an apical ventricular septal defect with left-to-right shunt was detected, perpetuating the hemodynamic compromise (Fig. 1A). Due to the high surgical risk associated with being the third cardiac surgery and maintaining total dependence on circulatory support due to biventricular dysfunction, the case was discussed with the Heart team to define the best strategy to correct the ventricular septal defect (VSD).

TEE documented an apical VSD measuring 0.68 x 0.67 cm. Using 3D reconstruction (Fig. 1B), the defect area was calculated to be 0.175 cm2. To determine the feasibility of percutaneous closure, cardiac tomography was performed to assess the trajectory and complexity of the VSD (Fig. 2A).

The cardiac tomography showed severe right ventricular dilation, leftward displacement of the ventricular septum with a "D-shaped" configuration of the left ventricle, and an apical VSD measuring 6.6 x 7.7 mm. Due to the complexity of percutaneous closure due to the inability to advance a guidewire through the tricuspid valve, 3D reconstruction of the tomographic images was performed (Fig. 2B), along with a 3D-printed model (Fig. 3B) to plan the approach and passage of the occluder device through the left ventricle.

After evaluating the images and 3D reconstructions, successful closure was achieved by placing a GORE cardioform 25 device through the VSD via the left ventricle cannulation (Fig. 3A).

Advances in 3D printing over the past 10 years have converted this technology from a specialized industrial process into a widely used tool with applications in many areas, including cardiology[1]. The potential of this technology has been demonstrated in diverse industrial applications and in biomedicine. The cardiological applications of these models range from teaching anatomy to the development of new procedures, and support for structural interventions[2].

In the field of congenital heart disease, as in this case after the Starnes procedure, planning the interventional approach was very difficult because of the complex heart anatomy and because of the high surgical risk in a patient with a third cardiac surgery and the circulatory support with VA ECMO, the heart team decided to perform a percutaneous transcatheter VSD closure.

The Starnes procedure is a palliative surgery for patients with Ebstein’s anomaly, This approach involves the exclusion of the malformed right ventricle with an autologous pericardial patch sewn at the “anatomic” level of the tricuspid valve annulus while maintaining the Pulmonary blood flow by a systemic to pulmonary artery shunt[3]. The goals of this procedure are to exclude and decompress the malformed right ventricle; to assure an uninterrupted atrial communication; to perform a reduction atrioplasty; and to provide pulmonary blood flow via a systemic to pulmonary shunt[4].

Due to the impossibility to access into the right ventricle through the tricuspid valve to create an arteriovenous circuit, the 3D model was used to plan the access through the VSD into the LV.

3D printed patient-specific models can be created for a number of different applications, in this specific case we created a model for complex procedural planning, even if we had different multimodality studies, the cardiac 3D printing allowed the medical team to provide a tangible model to make decisions about the correct device and the best therapeutic approach to perform the cardiovascular intervention.

Patient consent statement

Written informed consent for patient information and images to be published were provided by the patient or a legally authorized representative.

Conflict of interest

The Author(s) declare(s) that there is no conflict of interest

Celi, S., Martini, N., Pastormerlo, L., Positano, V., & Berti, S. (2017). Multimodality imaging for Interventional Cardiology. Current Pharmaceutical Design, 23(22). doi:10.2174/1381612823666170704171702
Vukicevic, Marija, Bobak Mosadegh, James K. Min, and Stephen H. Little. “Cardiac 3D Printing and Its Future Directions.” JACC: Cardiovascular Imaging 10, no. 2 (2017): 171–84. https://doi.org/10.1016/j.jcmg.2016.12.001.
Kron, Irving L., and Mark Elliot Roeser. “Management of Ebstein’s Anomaly.” Annals of Cardiothoracic Surgery 6, no. 3 (2017): 266–69. https://doi.org/10.21037/acs.2017.05.03.
Da Silva, Jose Pedro, Melita Viegas, Mario Castro-Medina, and Luciana Da Fonseca Da Silva. “The Da Silva Cone Operation after the Starnes Procedure for Ebstein’s Anomaly: New Surgical Strategy and Initial Results.” JTCVS Techniques 3 (2020): 281–83. https://doi.org/10.1016/j.xjtc.2020.05.011.

No competing interests reported.

Download PDF

Version 1

posted

You are reading this latest preprint version

Multimodal Imaging For Planning Closure Of Ventricular Septal Defect In a Patient With Ebstein’s Anomaly And Post Cardiotomy Shock Assisted With Venoarterial ECMO. The Role Of 3D Printing In Complex Scenarios

Status:

Version 1

Abstract

Figures

Learning objective

Introduction

Case presentation

Discussion

Conclusion

Declarations

References

Additional Declarations

Status:

Version 1