Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study†

Abstract

Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.

Evaluation of Supravalvular Aortic Gradient Changes Following Inverted Y-Patch Repair

“…For instance, Kramer et al (13) found 90% survival at 10 years in Doty repair patients while 91% at three-patch repair group. Considering all, even Padalino et al (14) were not able to conclude a certain decision about optimal repair method in their multicentre study using data from the European Congenital Heart Surgeons Association with the largest cohort including 301 patients in the literature so far. They stated that outcomes are related to patients' characteristics rather than surgical choice.…”

“…Several risk factors have been defined in prior studies such as diffuse-type SVAS , WBS and infancy (6,9,14) . Compatible with this, only one ,npatient mortality occurred in our cohort as a result of low cardiac output on the first postoperative day.…”

“…However the short duration of follow-up, such as in this study (average of 8 years) may be misguiding when considered that reoperations are needed typically after a decade. However, prior studies have determined pulmonary arterial stenosis as a risk factor for mortality in the long term and catheter interventions, and highlighted the importance of close follow-up (13)(14)(15)(16) . Although some authors observed a decrease of more than 10mmHg or normalization of the gradient over the stenotic site in the majority of patients, generally balloon angioplasty is commonly utilized in order to achieve an intended relief (15,16) .…”

“…Nonetheless, Kramer et al (13) reoperated 6 patients in their cohort and 4 of them reoperated because of recurrent obstruction of the supravalvular aorta at postoperative 5 months and 3.5 years.. Additionally, two cases underwent aortic valve replacement due to severe stenosis. In a study conducted by Padalino et al (14) , the researchers performed 8 redo SVAS and 7 aortic valve replacements. These seven cases were caused by aortic stenosis after a median interval of 5.7 (0.8-22.3) years.…”

“…On the other hand, aortic regurgitation may occur after surgical treatment even in a valve with usual function and morphology. Although the degree of aortic regurgitation improves after surgical correction, aortic valve replacement, for this reason, is not uncommon and has been reported by numerous authors so far (2)(3)(4)(5)(6)(7)(8)(9)(12)(13)(14)(15)(16)(17) .…”