Usefulness of stenting in aortic coarctation in patients with the turner syndrome.
Summary of "Usefulness of stenting in aortic coarctation in patients with the turner syndrome."
We report our experience with stent implantation for treatment of aortic coarctation in patients with Turner syndrome. Ten consecutive patients with Turner syndrome and aortic coarctation (median age 12 years, range 9 to 24) underwent coarctation stenting. Of these, 6 patients were treated for isolated coarctation and 4 for recurrent coarctation (3 after balloon dilation and 1 after balloon dilation and surgical repair). Bare metal stents were implanted in 7 patients and covered stents in 3. Immediately after stent implantation, peak systolic gradient decreased from 46.1 ± 24.3 to 1.9 ± 2.1 mm Hg (p <0.001). Aortic diameter at coarctation site increased from 5.1 ± 3.2 to 15.3 ± 2.0 mm after stenting (p <0.001). There were no deaths or procedure-related complications. During a median follow-up of 30.5 months, no patient developed restenosis. Two patients developed late aortic aneurysms at the coarctation site. In conclusion, stent implantation for aortic coarctation in patients with Turner syndrome appears to be a safe and effective alternative to surgical repair. Larger cohorts and longer-term follow-up are required to determine the effects of the procedure on the aortic wall.
Department of Pediatrics, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
This article was published in the following journal.
Name: The American journal of cardiology
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21029833
- DOI: http://dx.doi.org/10.1016/j.amjcard.2010.06.063
Medical and Biotech [MESH] Definitions
A birth defect characterized by the narrowing of the AORTA that can be of varying degree and at any point from the transverse arch to the iliac bifurcation. Aortic coarctation causes arterial HYPERTENSION before the point of narrowing and arterial HYPOTENSION beyond the narrowed portion.
A syndrome of defective gonadal development in phenotypic women with a karyotype of sex chromosome monosomy (45,X or 45,XO), associated with the loss of a sex chromosome X or Y. Patients generally are of short stature with undifferentiated (streak) gonads, sexual infantilism (HYPOGONADISM), webbing of the neck, cubitus valgus, elevated GONADOTROPINS and decreased ESTRADIOL level in blood. Studies of Turner Syndrome and its variants have contributed significantly to the understanding of SEX DIFFERENTIATION. NOONAN SYNDROME bears similarity to this disorder; however, it also occurs in males, has normal karyotype, and is inherited as an autosomal dominant.
Small clusters of chemoreceptive and supporting cells located near the ARCH OF THE AORTA; the PULMONARY ARTERIES; and the coronary arteries. The aortic bodies sense PH; CARBON DIOXIDE; and oxygen concentrations in the BLOOD and participate in the control of RESPIRATION. The aortic bodies should not be confused with the PARA-AORTIC BODIES in the abdomen (which are sometimes also called aortic bodies).
Small masses of chromaffin cells found near the SYMPATHETIC GANGLIA along the ABDOMINAL AORTA, beginning cranial to the superior mesenteric artery (MESENTERIC ARTERY, SUPERIOR) or renal arteries and extending to the level of the aortic bifurcation or just beyond. They are also called the organs of Zuckerkandl and sometimes called aortic bodies (not to be confused with AORTIC BODIES in the THORAX). The para-aortic bodies are the dominant source of CATECHOLAMINES in the FETUS and normally regress after BIRTH.
A multifaceted disorder characterized by short stature, webbed neck, ptosis, skeletal malformations, hypertelorism, hormonal imbalance, CRYPTORCHIDISM, multiple cardiac abnormalities (most commonly including PULMONARY VALVE STENOSIS), and some degree of MENTAL RETARDATION. The phenotype bears similarities to that of TURNER SYNDROME that occurs only in females and has its basis in a 45, X karyotype abnormality. However, Noonan syndrome occurs in both males and females with a normal sex chromosome constitution (46,XX and 46,XY). NS1 is due to mutations at chromosome location 12q24.1, in PTPN11, a gene encoding PROTEIN TYROSINE PHOSPHATASE, NON-RECEPTOR TYPE 11. LEOPARD SYNDROME, a disorder that has clinical features overlapping those of Noonan Syndrome, is also due to mutations in PTPN11. In addition, there is a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).
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