Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with normal segmental cardiac anatomy, severe left ventricular dilatation and dysfunction, aortic atresia, and atrial flutter. ().
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| http://dx.doi.org/10.1016/j.jaccas.2020.11.020 | DOI Listing |
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Prenatally Diagnosed Case of Successful Survival of an Unguarded Mitral Valve Orifice.
JACC Case Rep
September 2024
Department of Obstetrics and Gynecology, Juntendo University Faculty of Medicine, Tokyo, Japan.
Article Synopsis
- An unguarded mitral valve orifice is a rare heart condition seen in fetuses, identified by a weak left ventricle during ultrasound exams.
- This report is the first of its kind to link an unguarded mitral valve orifice with a double-outlet right ventricle while having a normal ventricular septum.
- The diagnosis was made prenatally based on specific ultrasound characteristics.
Fetal Unguarded Mitral Valve Orifice, Aortic Atresia, and Severe Left Heart Enlargement.
JACC Case Rep
February 2021
Division of Cardiology, Department of Pediatrics, University of California, San Francisco, California, USA.
Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with normal segmental cardiac anatomy, severe left ventricular dilatation and dysfunction, aortic atresia, and atrial flutter. ().
View Article and Find Full Text PDFCaught-off guard: Unguarded mitral valve orifice in usual atrial arrangement with discordant atrioventricular connections and pulmonary atresia.
Ann Pediatr Cardiol
June 2019
Institute of Genetic Medicine, Newcastle University, Newcastle Upon Tyne, United Kingdom.
Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of the mitral valvar orifice has previously been reported in the setting of mirror-imaged atrial arrangement. We report a neonate with unguarding of the mitral valvar orifice in the setting of usual atrial arrangement, but with discordant atrioventricular connections and pulmonary atresia.
View Article and Find Full Text PDFUnguarded left atrioventricular orifice: An unusual cause of hypoplastic left ventricle and double-outlet right ventricle with intact ventricular septum.
Ann Pediatr Cardiol
January 2019
Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India.
An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption.
View Article and Find Full Text PDFFatal cardiac anomaly of unguarded mitral orifice with asplenia syndrome.
J Cardiol Cases
January 2017
Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
We report the case of a newborn baby with an unguarded mitral orifice associated with asplenia syndrome, double-outlet right ventricle, dysplastic tricuspid valve, and pulmonary stenosis. This case was accompanied by severe tricuspid regurgitation and severe right ventricular hypertrophy. The patient had a fatal clinical course due to severe hypoxia and congestive heart failure.
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