Congenital mitral valve stenosis is a rare and severe disease, usually associated with other heart defects. The appropriate intervention depends on the site and mechanism of valvular obstruction and the aim is to avoid or delay valve replacement since it is associated with significant morbidity and mortality. Early single-stage complete repair is associated with better prognosis. We report the case of a 20-month-old child with a supravalvar mitral ring combined with a ventricular septal defect; pulmonary arterial systolic pressure before the surgery was 79 mmHg. The patient underwent a successful surgical repair with good clinical resolution.
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http://dx.doi.org/10.21470/1678-9741-2018-0403 | DOI Listing |
Semin Cardiothorac Vasc Anesth
March 2024
Division of Paediatric Cardiac Anesthesiology, Department of Anesthesiology, Seattle Children's Hospital, Seattle, WA, USA.
Shone's complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. Though not all components are required for a diagnosis, the end result is both left ventricular inflow and outflow obstruction, which typically present in patients as congestive heart failure. The complex pathology requires careful management and surgical decision-making to ensure an optimal outcome.
View Article and Find Full Text PDFSemin Cardiothorac Vasc Anesth
December 2023
Congenital Cardiac Surgery, Seattle Children's Hospital, Seattle, WA, USA.
Shone complex is defined by 4 anomalies: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and coarctation of the aorta. Establishing a clear definition is one of the principal challenges in the study of Shone complex as not all patients have all lesions. The essential feature of Shone complex is multilevel left-sided obstruction involving both the left ventricular inflow and outflow.
View Article and Find Full Text PDFEchocardiography
February 2022
Department of Pediatrics, Division of Pediatric Cardiology, Morgan Stanley Children's Hospital of NewYork Presbyterian/Columbia University Irving Medical Center, New Haven, Connecticut, USA.
Background: The ratio of early diastolic mitral inflow velocity (E) to early diastolic mitral annular tissue velocity (e'), or E/e', is an echocardiographic measure of left ventricular filling pressure. Peri-operative changes in E/e' and association with outcomes have been demonstrated in adults undergoing surgery for aortic stenosis (AS). We sought to explore changes in E/e' and other diastolic indices in the setting of congenital AS surgery and to assess for association with post-operative outcomes among children and young adults.
View Article and Find Full Text PDFJ Card Surg
April 2021
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.
Aim: To evaluate the imaging features and associations in patients with supravalvar aortic stenosis on multidetector computed tomography (CT) angiography.
Materials And Methods: We retrospectively reviewed all CT angiography studies performed for evaluation of congenital heart diseases at our institution through the period from January 2014 to June 2020. Cases with supravalvar aortic stenosis were identified and classified as syndromic and nonsyndromic based on history, physical examination, and relevant investigations.
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