Comments
Unicuspid AVs are rare congenital malformations. However, the true prevalence of this valve morphology is likely underreported. For instance, Noly et al. reported that only 14% of patients with UAV were diagnosed preoperatively 8. Even intraoperatively, UAV may remain under-recognized due to severe calcifications for instance; or inexperience of the surgeon or echocardiographer with identification of complex AV-morphologies.
Unicuspid AVs, without severe calcifications, can be repaired through bicuspidization. The standard technique is a butterfly patch repair4, with symmetrization of the cusps and improvement of cusp mobility, through additional modifications of the functional aortic annulus (e.g. annuloplasty, sinus plication), and thinning of the raphe.
In selected patients, Matsushima et al. have achieved a UAV-repair without patch material, through cusp nadir relocation and root remodeling. This achieved a relative elevation of the newly created commissure, which then more closely matched the height of the true commissure 6. The reason for this approach is to obviate the accelerated degeneration of autologous or xenogenic pericardial patch material, which renders this a palliative repair approach, and thus only allows for deferment of valve replacement later in life. Nonetheless, this is still an advantage in children and adolescents, and we think it is also of benefit when employed in adults, as the better survival of valve repair compared to prosthetic valve replacement is now well documented. Moreover, this may also function as a bridge to a Ross-procedure later in life, in children but also in younger to middle-aged adults.
In this patient however, we performed an AV-repair without a patch, through commissurotomy of a raphe to create a second commissure and two symmetric cusps. We further enhanced symmetry through reimplantation of the commissures at 180ยบ. Cusp mobility was improved through annuloplasty, as well as thinning of the free margins and raphe, and repair of a commissural diastasis. The anterior commissure was elevated through cusp plication and additional resuspension. Since no additional patch material was used, we hope that we were able to minimize the risk of cusp degeneration with overall improvement in valve longevity, similar to our bicuspid AV-repairs 9.