Frequency and Outcomes of Cardiac Operations and Catheter Interventions in Turner Syndrome

Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome

“…Atrial and ventricular septal (1%–2%) defects are usually small and not frequent in patients with TS, and if present, they do not usually require surgical repair . There are other less commonly described types of CHD lesions in this population, most of them in case reports, such as hypertrophic cardiomyopathy, tetralogy of Fallot, AVSD, patent arterial duct, and aortic or pulmonary artery valve stenosis, among others …”

Moderate Aortic Enlargement and Bicuspid Aortic Valve Are Associated With Aortic Dissection in Turner Syndrome

“…However, a recent review of outcomes in a large multi-institutional study found no deaths among 29 coarctation angioplasty/stenting procedures in TS. 17 One subject in the ITSAD registry experience an aortic dissection during pregnancy and 2 others had pregnancies before dissection. Approximately 0.5% of TS individuals become pregnant spontaneously.…”

A population-based analysis of mortality in patients with Turner syndrome and hypoplastic left heart syndrome using the Texas Birth Defects Registry

“…Two recent studies from Madriago et al and Patel et al analyze surgical outcomes in this population . Patel et al utilized data from the Society of Thoracic Surgeons Database from 2002 to 2006 and the Congenital Heart Surgeons' Society Database from 1994 to 2001 and found a decreased 10‐y survival of 25% in patients with chromosomal defects (including but not limited to TS) and HLHS compared to 54% in patients with HLHS and normal chromosomes .…”

“…There were isolated early reports in the 1960s and 1970s of an association between TS and hypoplastic left heart syndrome (HLHS), the most severe form of left‐sided CHD, but it was not until the mid‐1980s that it became widely accepted that a small but significant percentage of individuals with TS also have HLHS . Estimates of the prevalence of TS in live‐born infants with HLHS range from 1 to 7% . The prevalence is likely even higher in fetal life, given high fetal loss and termination rates for fetuses with TS and left‐sided obstructive lesions .…”