Shone's syndrome is a rare congenital anomaly defined as the presence of at least two of the following heart obstructions: a mitral supravalvular ring, a "parachute" mitral valve stenosis, subaortic stenosis, and aortic coarctation. A 58-year-old man presented with a mitral ring and a "parachute" mitral valve on two-dimensional transthoracic echocardiography, raising suspicion of Shone's syndrome. Three-dimensional transesophageal echocardiography revealed a subannular mitral ring inserted directly on the mitral leaflets, thus acting as a "valvar ring." This distinction can have therapeutic implications as a "valvar" mitral ring could require valve repair or replacement, instead of simple resection.
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http://dx.doi.org/10.1111/echo.13787 | DOI Listing |
Ann Thorac Surg
October 2023
Department of Thoracic and Cardiothoracic Surgery, Cleveland Clinic, Cleveland, OH; Division of Pediatric Cardiac Surgery and the Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, OH. Electronic address:
Background: Relationships between pulmonary hypertension (PHTN), mitral valve intervention (MVI), and death among patients with Shone's syndrome (SS) are unclear. Our recent data suggested temporal progression of MV disease and early survival benefit with MVI at initial operation. In this expanded cohort, we characterized trajectory and impact of PHTN on survival, and its relationship to MVI(s).
View Article and Find Full Text PDFJ Thorac Cardiovasc Surg
September 2024
Heart Institute, Children's Hospital of Los Angeles, Los Angeles, Calif.
Background: The Ross procedure has excellent outcomes in the pediatric population. Some series have reported age- and anatomy-dependent outcomes, but a comprehensive analysis stratified by these variables has not been reported to date. We sought to describe the landscape of congenital heart disease (CHD) treated with the Ross procedure and identify the patients best served by this operation.
View Article and Find Full Text PDFEchocardiography
September 2024
Department of Ultrasound, Renmin Hospital, Hubei University of Medicine, Shiyan, China.
Handb Clin Neurol
March 2024
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom.
An adaptive immune response in less than 1% of people who develop cancer produces antibodies against neuronal proteins. These antibodies can be associated with paraneoplastic syndromes, and their accurate detection should instigate a search for a specific cancer. Over the years, multiple systems, from indirect immunofluorescence to live cell-based assays, have been developed to identify these antibodies.
View Article and Find Full Text PDFCureus
January 2024
Cardiology, Detroit Medical Center/Sinai Grace Hospital/Wayne State University, Detroit, USA.
A parachute mitral valve (PMV) is a congenital mitral valve anomaly diagnosed in infancy, and it can also be discovered in adults during echocardiography. Surgical management is common in infants to prevent complications from left-heart obstructions. In adults, PMV may be found independently or with other cardiac defects.
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