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Echocardiographic Approaches and Protocols for Comprehensive Phenotypic Characterization of Valvular Heart Disease in Mice
Published on: February 14, 2017
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Echocardiographic characteristics of annulo-leaflet mitral ring
David N Schidlow1, Abbas Zaidi2, Kimberlee Gauvreau1
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Summary
Annulo-leaflet mitral ring (ALMR) is a rare congenital heart defect. Echocardiography reveals diverse ALMR morphologies, with intraleaflet types linked to severe mitral stenosis and younger age/short chordae associated with surgical resection.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Annulo-leaflet mitral ring (ALMR) is a rare congenital cardiac anomaly.
- It results from fibrous tissue on the atrial surface of the mitral valve.
- ALMR can progress to cause severe mitral stenosis.
Purpose of the Study:
- To detail the echocardiographic features of ALMR.
- To investigate associations between echocardiographic findings and surgical resection.
- To classify ALMR morphology based on echocardiographic evaluation.
Main Methods:
- Retrospective single-center study.
- Analysis of clinical and echocardiographic data from 57 patients (2004-2012).
- Data analyzed for associations with surgical resection.
Main Results:
- Median age at diagnosis was 1.8 years; 63% were male.
- 51 patients (89%) had associated cardiac lesions.
- Intraleaflet ALMR morphology showed higher mitral stenosis gradients (8.4 vs 5.8 mmHg).
- Younger age at diagnosis and short chordae were associated with ALMR resection.
Conclusions:
- ALMR morphology is heterogeneous, classifiable by location and leaflet involvement.
- Intraleaflet morphology is linked to significant mitral stenosis.
- Younger age at diagnosis and short chordae predict ALMR resection.
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