Postoperative transesophageal echocardiographic images on mitral valve regurgitation.

Due to advances in the medical and surgical treatment of congenital heart disease (CHD), up to 90% of children born with CHD now reach adulthood. Unfortunately, many ACHD who underwent previous repair, still experience residual intracardiac lesions or, less frequently, develop acquired disease (eg, coronary, valvular, or aortic disease).

Repaired ToF is more frequently associated with the development of pulmonary regurgitation during follow-up. However, given the high risk of a redo surgery associated with the increasing expertise with the use of transcatheter valves, pulmonary regurgitation is currently preferentially managed by means of endoprosthesis implantation. Acquired degenerative diseases in this special population are rarely reported, but their management might be a challenge when surgery is recommended. Microinvasive surgery includes procedures that mimic conventional operations but are performed off-pump on a beating heart (eg, transapical neochordae implantation). Thus far, as with transcatheter aortic valve replacement and edge-to-edge mitral valve repair, microinvasive procedures are still not widely performed in ACHD.

To the best of our knowledge, this case is the first of transapical neochordae implantation in an ACHD patient. Redo surgeries in this subset of patients are associated with a high risk of intraoperative and postoperative complications (sternal re-entry, bleedings, unexpected cardiac and pulmonary lesions, long cardiopulmonary bypass and cardioplegic arrest, postoperative cardiogenic shock, neurologic problems, arrhythmias, infections, and wound issues) and, consequently, lower overall postoperative survival. To prevent these problems, congenital interventional cardiologists have already prioritized and standardized a few procedures to avoid (unless mandatory) conventional surgical reoperation (eg, pulmonary endoprosthesis or stent implantation). Unfortunately, current knowledge of acquired disease in ACHD remains limited, but it is going to become a serious issue in the very near future. 

Degenerative mitral regurgitation is not a frequent finding in ToF; however, it might require surgical treatment (according to current guidelines) when symptomatic and severe. In patients such as the current individual (with multiple risk factors such as OSAS, obesity, previous major cardiac intervention, and recent acute heart decompensation), a traditional surgical strategy might have been high risk. The absence of standardized surgical scores in this special population to predict postoperative mortality emphasizes the importance of an informed discussion with patients to offer them the best care. In fact, the more frequently used EuroSCORE (European System for Cardiac Operative Risk Evaluation) II for predicting in-hospital mortality after major cardiac surgery is not appliable to ACHD patients. In addition, patients with ToF who underwent late surgical repair (as in this case) frequently exhibit abnormal hypertrophy and biventricular fibrosis, which might negatively affect heart recovery after a redo cardiopulmonary bypass and cardioplegic arrest. Even if no studies are available to compare transapical neochordae implantation vs transcatheter mitral valve repair (eg, MitraClip, Abbott), we were more confident with the first procedure, and we believe that neochordae implantation might be more physiological in restoring mitral competence at long-term follow-up.

Given our experience with indications, technical aspects, and results of the transapical neochordae implantation, we decided to direct the patient toward a microinvasive surgical strategy. In fact, according to our experience, in favorable mitral anatomies (isolated P2 prolapse), a transapical neochordae repair yields 5-year results similar to those of traditional open surgery in terms of freedom from death and reoperation. Indeed, at 5 years of follow-up, incidence of severe mitral regurgitation in favorable anatomies after neochordae repair remains low (<15%). Due to the novelty of this microinvasive procedure, the patient will require a strict follow-up. However, should long-term failure of this procedure occur, conventional redo mitral valve repair will be feasible. 

With this case we want to highlight that microinvasive surgery can be successfully applied to complex cases, with the advantage of lowering procedural times, anesthesia risks, and, finally, hospital complications and length of stay.

The patient did not experience any major or minor postoperative complications according to MVARC (Mitral Valve Academic Research Consortium) criteria  and was discharged home 9 days after the procedure in good clinical condition. The patient will undergo strict clinical and echocardiographic follow-up during the first year (with outpatient visits every 3 months). These visits will then be reduced to one clinical and echocardiographic check-up every 6 months.

Transapical neochordae implantation off-pump on a beating heart in high-risk ACHD might be safe and feasible. This case highlights the importance of a multidisciplinary preoperative evaluation and proper planning to tailor the procedure according to each patient’s characteristics. Indeed, microinvasive surgery might be considered as a good therapeutic alternative in high-risk ACHD patients with acquired cardiac disease. Furthermore, a microinvasive mitral valve repair will enable a more physiological approach to restoring normal mitral anatomy and function.