Balkan Journal of Medical Genetics

CARDIOVASCULAR DISORDERS OF TURNER’S SYNDROME: A REVIEW
Yuan S-M, Jing H
*Corresponding Author: Hua Jing, Department of Cardiothoracic Surgery, Jinling Hospital, Clinical School of Medicine, Nanjing University, Nanjing 210002, Jiangsu Province, People’s Republic of China; Tel.: +86-25-8480-1332; Fax: +86-25-8482-4051; E-mail: shiminyuan@126.com
page: 3

TREATMENT

Growth hormone therapy is recommended for TS patients to promote their secondary sexual development and to improve quality of life [47]. As a result, estrogen deficiency as well as estrogen deficiency-induced memory and motor coordination problems, can be ameliorated after treatment with growth hormones [48]. Systematic clinical assessments including gynecological examinations should be regularly performed on TS patients who receive growth factor treatment due to the potential risk of developing neoplasms in these subjects [49]. The severity of aortic dilation seemed to be related to the dose of growth hormone treatment, with a beneficial effect of a larger growth hormone treatment dose [50]. Aortic root dilatation is a significant risk in women with TS and is closely dependent on blood pressure. Aortic root dilatation does not appear to be related to atherosclerosis and is more likely to be due to a mesenchymal defect [5]. Women with TS are often hypertensive as a result of aortic abnormality or renal vascular disorder. In all individuals with hypertension, with or without aortic dilation or aortic dissections, medications should be given to control the blood pressure in order to prevent coronary heart disease, heart attack or other potentially fatal complications. Beta blockers or rennin-angiotensin system antagonists may benefit TS patients in retarding the progression of aortic dilation [51]. Coarctation of the aorta and bicuspid aortic valve are commonly associated with TS, leading to an increased risk to infective endocarditis. Therefore, prophylactic antibiotics are strongly recommended for TS patients with a susceptibility of bacteremia [5,8]. In addition, diabetes type II can be well-controlled through medication with careful monitoring of blood glucose levels, healthy meal planning and exercise, etc. Thyroid hormone supplements can help boost underactive thyroid function in patients with hypothyroidism. Prophylactice aortic replacement may minimize the risk of potentially fatal aortic dissection of the TS patients [51]. A patch plasty using felt reinforcement [52], tube graft insertion [53], or composite aortic valve replacement [54] with satisfactory postoperative courses have been documented in the literature. However, the situation would be more complicated and may lead to a critical condition if the TS patient had a history of previous coarctation repair [53]. In recent years, stent graft deployment was well-developed as an alternative choice to open surgery in proper cases [55].

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