First in Man: Successful Implantation of a Custom-Made Fontan Cannula in a Patient with Failing Fontan Circulation as a Bridge to Transplant

Background: Fontan patients with protein-losing enteropathy and endorgan failure have a poor prognosis and may require at the end stage of the disease heart transplantation. For this purpose a right venticular assist device may be used to improve pulmonary blood flow and lower central venous pressures. To overcome the anatomical difficulties after Fontan palliation, a dedicated cannula to connect the venae cavae to an RVAD and consequently to the main pulmonary artery has been developed.

Method: The 17-year-old-boy with double inlet left ventricle and pulmonary stenosis underwent total-cavopulmonary connection at 23/4 years. Due to an increasing mitral valve insufficency, a mechanical mitral valve was implanted aged 14 years and a DDD pacemaker was implanted due to postoperative AV block III. Subsequently severe and progressive protein loosing enteropathy required a fenestration between the left atrium and Fontan conduit reducing the transpulmonal gradient from 17 to 10 mm Hg. After initial improvement, his clinical condition worsened and he was hospitalized with pleural effusion, ascites, and kidney and liver failure. Listing for heart transplant was impossible due to the poor general condition. With a normal left ventricle function and competent mitral valve, we decided to implant a RVAD as brigde to recovery.

Results: On cardiopulmonary bypass and beating heart, the outflow cannula of the RVAD Berlin Heart was connected with the pulmonary artery, and the SVC and IVC were connected via the new cannula to the inflow part of the RVAD. On the second postoperative day, the patient could be extubated. Central venous pressure decreased from 20 mm Hg directly after surgery to 10 mm Hg a week later. Parenteral nutrition was needed for another 4 weeks. Ascites was present for 3 weeks after surgery and diminished slowly. Eight weeks after the implantation of the RVAD liver and bowel function was recovered. Four months later, he was successfully heart transplanted.

Conclusion: Implantation of the new Fontan cannula enables a simplified technical approach for RVAD in Fontan patients and can be used as a bridge to recovery and to transplant in patients with failing Fontan circulation and preserved left ventricular function.

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Artikel online veröffentlicht:
12. Februar 2022