Turner syndrome, unicuspid aortopathy, and pregnancy: Difficult decisions for complex scenarios

Abstract

Correspondence: *María Martín E-mail: [email protected] *Antonio Adeba E-mail: [email protected] Available online: 12-04-2021 Arch Cardiol Mex (Eng). 2021;91(2):252-254 www.archivoscardiologia.com Date of reception: 22-04-2020 Date of acceptance: 25-05-2020 DOI: 10.24875/ACM.20000176 1405-9940 / © 2020 Instituto Nacional de Cardiología Ignacio Chávez. Published by Permanyer. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). A 31-year-old woman with previous diagnosis of Turner syndrome and desire of pregnancy was submitted for further evaluation for aortic valvulopathy. She was finally diagnosed of unicuspid aortic valve with severe regurgitation and an aortic ascending aneurysm. Given these findings, surgical intervention was decided, with a replacement of the aortic root and ascending aorta by a 34 mm tube with reimplantation of the aortic valve using the DAVID technique and reconstruction of the unicuspid valve in bicuspid. We review both the unicuspid aortic valve, Turner syndrome, and the recently published European Society of Cardiology guidelines about pregnancy and heart disease. They include a more detailed risk classification that includes different aspects not only maternal and fetal risk but also others such as the periodicity of follow-ups and the right place for delivery, specifically in aortic pathology. An update of the guidelines of the European Society of Cardiology (ESC) on heart disease and pregnancy was published in 20181,2. They include, in relation to the previous ones, several novelties: the World Health Organization (WHO) classification on maternal risk is reinforced; the “heart team” of pregnancy is introduced just like in other pathologies; and greater attention is paid to assisted reproduction therapies that are key in certain pathologies such as Turner syndrome.