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Introduction And Clinical Importance: Shone complex (SC) is a rare multilevel congenital heart disease (CHD) characterized by four left-sided heart obstructive lesions: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta (CoA), accounting for 0.6-0.7 % of CHD cases.

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Article Synopsis
  • The study aimed to analyze ultrasound findings, SNP-array results, and pregnancy outcomes in fetuses with 7q11.23 deletions and duplications during the second and third trimesters, gathering comprehensive data from a Chinese medical center over several years.
  • Seven fetuses with 7q11.23 deletions and six with duplications were diagnosed, revealing significant clinical abnormalities such as intrauterine growth restriction and various cardiovascular issues; the deletions were associated with more serious ultrasound findings.
  • Most pregnancies (seven deletions and three duplications) were terminated due to the severe abnormalities discovered, indicating a high risk linked to these genetic conditions.
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Prognostic Effects of Operation Age for Pediatric Patients with Supravalvar Aortic Stenosis.

Rev Cardiovasc Med

October 2024

Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, 100029 Beijing, China.

Background: The appropriate age for surgical repair of asymptomatic congenital supravalvular aortic stenosis (SVAS) is still unknown. The purpose of this research was to assess the safety and effectiveness of various operation ages when managing SVAS.

Methods: Consecutive asymptomatic SVAS pediatric patients in the Beijing Fuwai and Yunnan Fuwai hospitals over a period of 18 years were retrospectively analyzed.

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Background: Supravalvular aortic stenosis (SVAS) is a relatively rare form of left ventricular outflow tract obstruction, often accompanied by other cardiac conditions. However, a standard surgical reparative technique has not been established and repairing SVAS remains challenging.

Case Presentation: We repaired SVAS of a 3-year-old boy accompanied by a bicuspid aortic valve and malpositioned coronary orifices by partial Brom's technique with two glutaraldehyde-treated autologous pericardial patches, using recent advanced preoperative information, including geometric and effective heights.

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Background: The long-term monitoring of biventricular function is essential to identify potential functional decline in patients following the arterial switch operation (ASO). The underlying pathophysiological mechanisms responsible for altered biventricular hemodynamics in ASO patients are not yet well understood. This study sought to: (I) compare the biventricular kinetic energy (KE) and vorticity of ASO patients and age- and sex-matched controls; (II) investigate the associations of four-dimensional (4D) flow biventricular hemodynamics parameters and neo-aortic root dilation, supravalvular pulmonary stenosis, and pulmonary artery transvalvular pressure difference.

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