Outcomes of cardiothoracic surgery in women with Turner syndrome

Abstract

Background To describe short- and mid-term surgical outcomes of patients with Turner syndrome (TS) after cardiovascular interventions. Methods All individuals >12 years of age at the time of surgical repair for cardiovascular disease (valve or coarctation repairs, aortic disease, aortic dissection) from 2002 to 2022 were eligible. The primary endpoint was complications or death within 30 days of intervention. Secondary outcomes included late complications and reinterventions within six months. Combined data from the University of Texas Health Science Center at Houston and the Turner Syndrome Society of the United States were included in the analysis. Results We identified 22 patients who met the inclusion criterion. The median age was 46 years (range, 21–75 years), with 86% having estrogen replacement therapy. The most common medical condition was hypertension (77%), followed by hypothyroidism (59%). The most frequent indication for surgery was aortic root or ascending aortic aneurysms (68%), followed by symptomatic aortic stenosis in patients with bicuspid aortic valve (64%), coarctation of aorta (45%), and acute aortic dissection (18%). Respiratory complications were the most common (68%). Pleural effusions were the most frequent found sign on imaging studies (68%). Thoracentesis, or chest tube placement, was required in 33% (5/15). Respiratory failure requiring specific support with high flow oxygen and/or thoracentesis occurred in 36% (8/22). Conclusions Patients with TS may be at an increased risk for postoperative complications after aortic surgery. Bicuspid aortic valve (59%) and coarctation of the aorta (45%) were the most common congenital malformations among our study group. Our study showed that respiratory complications were the most common, with pleural effusions being the most common post-surgery complication.

Methodological advances in patient-centered rare disease research: the UTHealth Houston Turner Syndrome Society of the United States research registry

“…Data from these follow up studies were stored in separate REDCap projects and exported to the TSRR after de-identification. Using this approach, TSRR investigators identified health disparities in access to guideline-recommended medical care for Spanish speakers with TS [ 13 ]; characterized the increased prevalence of dermatologic diseases in TS [ 14 ]; and described risk factors for postoperative complications after cardiovascular surgeries in young adults with TS, which occur more frequently than expected [ 15 ].…”

In-Depth Genomic Analysis: The New Challenge in Congenital Heart Disease

“…BAV (15-30%), aortic coarctation (7-20%), and, less commonly, hypoplastic left heart syndrome [93,94] are common in patients with Turner syndrome (45, XO karyotype). Patients with Turner syndrome have an increased risk of left-sided obstruction lesions due to altered gene dosages on chromosome X and copy number polymorphisms at the 12q13.31 locus [95][96][97]. However, it is currently unknown how these genotypes affect the development of left-sided structures.…”

Methodological Advances in Patient-centered Rare Disease Research: the UTHealth Houston Turner Syndrome Society of the United States Research Registry

“…The TSRR has enrolled more than 1,100 participants and successfully deployed two general health update surveys and three project-speci c surveys (Supplementary Data). TSRR data has led to projects on skin concerns across the life spectrum and complications after cardiovascular surgeries [12]. Survey responses from these projects will be available to future researchers who apply for TSRR data.…”