Aortic valve reimplantation in patients with Marfan and other connective tissue syndromes: a success story for these patients!

After the introduction of aortic valve (AV) and root replacement (Bentall–De Bono procedure), survival changed dramatically for patients with AV and/or root pathologies, including patients with Marfan syndrome or other genetic connective tissue diseases (CTD) [1, 2]. Since the latter mentioned patients are presented for surgery at a very young age, mechanical prosthesis is generally chosen over the biological. The consequent need for life-long anticoagulation exposes the patients to the potential, sometimes severe complications accounting up to 5% per year [3]. Due to this fact, AV-sparing techniques have been evolving over the last 30 years. Especially, AV reimplantation, as described by David and Feindel in 1992, became the standard technique for valve-sparing aortic root replacement with excellent short- and long-term results, even in patients with Marfan syndrome or other CTD [2, 4, 5]. In this issue of the European Journal of Cardiothoracic Surgery, Gil et al. describe their experience with AV reimplantation (David operation) in 157 patients with Marfan syndrome (143 patients) and other CTD [6]. The presented cohort is one of the largest published yet. The early- and long-term outcomes (median of 7.3 years) are impressively good. Especially, survival at 10 and 15 years with 98% is excellent and not different to the general Spanish population for the same age and gender. Furthermore, reported freedom from greater than moderate aortic regurgitation and reintervention were 90% and 96% at 10 years, respectively. Importantly, all these patients were operated by the same cardiosurgical team. The clear message that can be derived from this article is that David operation is an excellent option for the surgical treatment of these patients. The authors also conclude these patients should be centralized in referral centres and operated by very experienced surgeons. Finally, based on these results, authors postulate the threshold for prophylactic surgery in CTD should be reviewed and lowered. One very important point that should be discussed is the use of different AV reimplantation techniques and different grafts. The authors applied the ‘so-called’ David I technique in the first 8 patients using a manufactured Valsalva graft designed by De Paulis et al. [7] and later they switched to the ‘so-called’ David V technique as described by Miller [8] for the following 149 patients. David himself reported he has returned to the original reimplantation technique using a cylindrical graft since 2015, while supposing that creating neo-sinuses or using manufactured Valsalva graft adversely affects the durability of AV reimplantation [4]. In my opinion, this fact and currently ongoing debate are a bit overestimated since until now no clinical studies were able to prove it. Additionally, many centres with great experience in AV repair reported almost but not so excellent long-term results as David et al. [7, 9]. The most likely explanation for the extraordinary and almost not reproducible results of David’s cohort is his extreme selection of patients, while the patients with severe aortic regurgitation and huge ascending aortic aneurysm were only scarcely accepted for AV reimplantation and rather received Bentall–De Bono operation in the increasing course of time. Despite the excellent results of AV-sparing operations in patients with Marfan syndrome and other CTD published in different studies, even with the direct comparison between AV-sparing and Bentall–De Bono procedure, no single randomized controlled study has been published yet enabling us to derive a definite conclusion. Recently, Soto et al. published a systematic review and meta-analysis of AV-sparing surgery versus replacement surgery in ascending aortic aneurysms and dissection in patients with Marfan syndrome and other genetic CTDs [10]. This very well-done study could conclude that the choice of the best surgical technique (AV- sparing versus Bentall–De Bono) is dependent on the type of CTD as well as the accompanying aortic and cardiovascular damage. The results of this meta-analysis showed that AV-sparing operations were associated with a reduced risk of endocarditis and thromboembolism; however, the Bentall–De Bono operations favoured freedom from AV and/or root reoperation. Given these facts and until no randomized trial will be existent, the surgeon can only make the best decision during the operation.