Coronary calcification in adults with Turner syndrome

Abstract

PurposeAdults with Turner syndrome (TS) have an increased predisposition to ischemic heart disease. The quantitative relationship between coronary atherosclerosis and TS has yet to be established.Methods128 females (62 with TS) participated in this prospective study. Coronary computed tomography angiography (CTA) was performed to measure coronary calcified plaque burden, and prevalent non-calcified plaque burden. Regression analysis was used to study the effects of TS and traditional cardiovascular disease risk factors on coronary plaque burden.ResultsAdults with TS were 63% more likely to have coronary calcifications than controls (odds ratio (OR) 1.63, 95% confidence interval (CI): 1.02, 2.61, P=0.04), with an age cutoff of 51.7 years for a probability of >50% for the presence of coronary calcifications when compared to 55.7 years in female controls. The average age of TS and calcified plaques was significantly lower than controls (51.5±8.9 years vs. 60.5 ±7.0 years, P<0.001). Age increased the likelihood of coronary calcifications by 13% per year (OR 1.13, CI 95%: 1.07–1.19, P<0.001).ConclusionThis study demonstrates a higher prevalence and earlier onset of calcified coronary plaques in TS. These findings have important implications for cardiovascular risk assessment and the management of patients with TS.

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years

“…Almost 50% of the excess mortality is caused by cardiovascular disease including aortic events, aortic valve disease, hypertension, and ischemic heart disease (Schoemaker et al, ; Swerdlow et al, ). Early onset of calcified coronary plaques in Turner syndrome has been demonstrated using coronary computed tomography angiography (Schoepp et al, ) and also coronary artery anomalies (Viuff et al, ). Endocrine disorders such as diabetes also contribute to the increased mortality (Schoemaker et al, ; Stochholm et al, ).…”

“…Coarctation is associated with an increased likelihood of aortic dilation and dissection (Bambul Heck, Pabst von Ohain, Kaemmerer, Ewert, & Hager, ; Cools, Brown, & Gewillig, ; Eckhauser et al, ). Hypertension, a common consequence of aortic coarctation that may manifest in childhood, and altered compliance or distensibility of the aorta, which is prevalent in patients with coarctation, may both contribute to aortic disease (Hjerrild et al, ; Pees et al, ; Schoepp et al, ; Wen et al, ). Re‐interventions due to re‐coarctation are often necessary in adulthood.…”

“…Low estrogen levels due to inefficient estrogen substitution through adolescence and adult life may also contribute to cardiovascular risk in some women with Turner syndrome. Coronary artery disease appears earlier in women with Turner syndrome than in controls and the risk of coronary events and cardiovascular mortality is significantly increased (Schoepp et al, ). Whether the increased rate of cardiovascular events in Turner syndrome is entirely attributable to the burden of risk factors, or may also be related to Turner syndrome‐specific causes, is unknown.…”

“…Turner syndrome is associated with an increased risk of hypertension, diabetes, and atherosclerotic events (Regitz‐Zagrosek et al, ). A recent study revealed an increased likelihood of coronary calcifications at a younger average age in women with Turner syndrome compared with non‐Turner individuals (Schoepp et al, ). At a minimum, cardiovascular evaluation including blood pressure and diabetes management should be mandatory preconception care for women with Turner syndrome.…”

Glucose Metabolism in Turner Syndrome

“…The typical symptoms of TS include short height, webbed neck, low hairline at the back of the neck, low-set ears, markedly elevated levels of follicle stimulating hormone (FSH), chronic otitis media (OM), lymphedema of extremities, small mandible, and multiple pigmented nevi (4). Patients with TS are often affected by many other comorbidities, including autoimmune diseases (AD), hypothyroidism, kidney dysfunction, loss of ovarian function or other reproductive disorders, neurological or ophthalmological abnormalities, osteoporosis, diabetes mellitus (DM), dyslipidemia, hypertension, and heart disease (2, 5–10).…”