STATE OF THE ART ARTICLE Congenital Cardiovascular Disease in Turner Syndrome
نویسنده
چکیده
Turner syndrome (TS), or monosomy X, occurs in ~1/2000 live born females. Intelligence is normal and short stature is the most obvious and consistent feature of the syndrome. Congenital cardiovascular disease affects ~50% of individuals and is the major cause of premature mortality in adults. Unfortunately, this most important aspect of the syndrome has received little attention outside of pediatric medicine, and adult cardiological follow-up is seriously lacking. This review describes the spectrum of cardiovascular defects with particular attention to identifying risk factors for aortic dissection/rupture. X-chromosome genetic pathways implicated in Turner cardiovascular disease, including premature coronary artery disease, are discussed. Recent guidelines for diagnosis and treatment of girls and women with TS are reviewed.
منابع مشابه
May-Thurner syndrome, an uncommon diagnosis for a common disease: a case report and review of the literature
May-Turner syndrome is a relatively uncommon anatomical variation in which patients develop iliofemoral deep vein thrombosis (DVT) due to venous occlusion. In this syndrome, the left common iliac vein is compressed against the fifth lumbar vertebra by the right common iliac artery. The real incidence/prevalence of May-Turner syndrome is not precisely known, but it is estimated to be between 22 ...
متن کاملDextrocardia and Hiatal Hernia in a Patient with Turner Syndrome
Turner syndrome is a sex-chromosome disorder occurring in one out of 2500 female births and characterized by growth retardation, gonadal dysgenesis and cardiovascular anomalies. The 45, XO karyotype is the most frequent type of this disease. Herein, we report on a 6-year-old girl with Turner syndrome and 45, XO karyotype presenting with short stature. She had dextrocardia and hiatal hernia. To ...
متن کاملسندرم نونان (گزارش یک مورد)
Noonan syndrome is a genetic condition inherited by an autosomally dominant manner, characterised by congenital heart disease, short stature, abnormal facies and the somatic feature of Turner's syndrome, but a normal karyotype. Noonan syndrome affects approximately 1 in 1500 live births. Congenital heart disease occurs in 35-50% of patients diagnosed with noonan syndrome. The most common cardia...
متن کاملDiagnostic and Prognostic Value of Cardiac Biomarkers in Children with Kawasaki Disease: A State-of-the-Art Review
Kawasaki disease (KD) is characterized as the leading cause of acquired cardiac disease in children. Accurate and timely diagnosis of KD is of high importance for preventing its cardiac complications. However, diagnosis merely based on clinical findings has a number of challenges and, limitations. Therefore, researchers are investigating to find more object...
متن کاملCOARCTATION OF THE AORTA Negligence in Diagnosis Four (Case- Reports).
SUMMARY: Coarctation of the aorta is a congenital heart disease consisting 7% of total congenital heart diseases. Its prevalence in men is 2 times more than that in women. This disease is associated with other congenital diseases. including aortic bicuspid valve, the linkage between two ventricles and arteriole channel It is often seen in the Turner syndrome which may lead to complications su...
متن کامل