What is the mortality and morbidity associated with Turner syndrome?

Updated: Mar 19, 2021
  • Author: Maala S Daniel, MBBS; Chief Editor: Luis O Rohena, MD, MS, FAAP, FACMG  more...
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Answer

Mortality associated with Turner syndrome may be increased in the neonatal period because of hypoplastic left heart [5]  and coarctation of the aorta and in adulthood because of cardiovascular disease, particularly aortic dissection. Obesity, with associated diabetes mellitus and hypertension, can also contribute to early mortality. Limited epidemiologic studies suggest that life expectancy is reduced by about 10 years. Osteoporosis is common.

Although congenital cardiovascular malformations and aortic dilatation are common among patients with Turner syndrome, they are often undiagnosed until later in life, pointing to the need for a more systematic approach to cardiovascular monitoring. [22]

Renal anomalies found in some individuals may cause a predisposition to urinary tract infections or hypertension. Even in the absence of cardiac or renal anomalies, patients are prone to develop hypertension.

Individuals with mitral valve disease or aortic valve disease require subacute bacterial endocarditis (SBE) prophylaxis.

In a Danish study, the overall risk of autoimmune disease among women with Turner syndrome was twice that among Danish women in general. [23]   For individual diseases, associations were strongest for Autoimmune thyroiditis, a condition more common in females, and type 1 diabetes mellitus.


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