A rare case of mitral valve dysplasia and left ventricular noncompaction: surgical management and genetic investigation.
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ABSTRACT:
SUBMITTER: Liu L
PROVIDER: S-EPMC10895090 | biostudies-literature | 2024 Feb
REPOSITORIES: biostudies-literature
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A rare case of mitral valve dysplasia and left ventricular noncompaction: surgical management and genetic investigation.
Liu Lei L Fang Aijuan A Chen Hui H Shao Yongfeng Y Ni Buqing B Yao Jing J
Quantitative imaging in medicine and surgery 20240123 2
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Project description:BackgroundAccessory mitral valve tissue rarely causes left ventricular outflow tract obstruction in adults. It is often associated with other cardiac and vascular congenital malformations. Here, we report the rarest presentation of accessory mitral valve tissue (AMVT) causing left ventricular outflow tract obstruction.Case summaryA 22-year-old female patient presented with a history of shortness of breath and chest pain for more than 5 years. A diagnosis of AMVT with parachute mitral valve, ventricular septal defect (VSD), bicuspid aortic valve, unruptured aneurysm of aortic sinus, and left ventricular outflow tract obstruction was made. Successful closure of VSD with mitral valve replacement, excision of AMVT, and repair of the aortic sinus were performed. The post-operative course was uneventful, and an echocardiogram showed complete resection of the accessory mitral valve, no residual shunt and no left ventricular outflow gradient. Additionally, the peak gradient of rapid filling phase and atrial systolic phase across the prosthetic mitral valve were 16 mmHg and 4 mmHg, respectively. The peak velocity across left ventricular outflow tract was 1.4 m/s.DiscussionAccessory mitral valve tissue is associated with other cardiac abnormalities and is usually diagnosed in the first or second decade of life. It is responsible for left ventricular outflow tract obstruction. The obstruction can occur in the early period of life due to continued deposition of fibrous tissues within left ventricular outflow tract. Accessory mitral valve tissue should be considered a rare but important cause of left ventricular outflow tract obstruction.
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Project description:The major clinical features of myocardial noncompaction are heart failure, arrhythmias, and thromboembolic events. Prominent myocardial trabeculae and deep recesses characteristic of myocardial noncompaction can cause stagnant blood flow and the formation of left ventricular clots. We describe the case of a 62-year-old woman who presented with symptoms of heart failure secondary to left ventricular noncompaction. Transthoracic and transesophageal echocardiography revealed multiple left ventricular thrombi, which had formed despite the patient's long-term therapy with aspirin. Anticoagulative therapy should be considered for patients with myocardial noncompaction who also have risk factors for thromboembolism, such as atrial fibrillation, a history of systemic embolism, or severe left ventricular systolic dysfunction. However, chronic antiplatelet therapy may not sufficiently prevent clot formation in patients who have myocardial noncompaction and severe left ventricular systolic dysfunction.
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Project description:This study sought to identify the short- and long-term changes in left ventricular ejection fraction (LVEF) after mitral valve repair (MVr) in patients with chronic primary mitral regurgitation according to preoperative LVEF (pre-LVEF) and preoperative left ventricular end-systolic diameter (pre-LVESD). This study evaluated 461 patients. Restricted cubic spline regression models were constructed to demonstrate the long-term changes in postoperative LVEF (post-LVEF). The patients were divided into four groups according to pre-LVEF (<50%, 50-60%, 60-70%, and ≥70%). The higher the pre-LVEF was, the greater was the decrease in LVEF immediately after MVr. In the same pre-LVEF range, immediate post-LVEF was lower in patients with pre-LVESD ≥ 40 mm than in those with pre-LVESD < 40 mm. The patterns of long-term changes in post-LVEF differed according to pre-LVEF (p for interaction < 0.001). The long-term post-LVEF reached a plateau of approximately 60% when the pre-LVEF was ≥50%, but it seemed to show a downward trend after reaching a peak at approximately 3-4 years after MVr when the pre-LVEF was ≥70%. The patterns of short- and long-term changes in post-LVEF differed according to pre-LVEF and pre-LVESD values in patients with chronic primary mitral regurgitation after MVr.
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Project description:OBJECTIVES:Ischemic mitral regurgitation (IMR) results from ischemic left ventricular (LV) distortion and remodeling, which displaces the papillary muscles and tethers the mitral valve leaflets apically. The aim of this experimental study was to examine efficacy of an adjustable novel polymer filled mesh (poly-mesh) device to reverse LV remodeling and reduce IMR. METHODS:Acute (N = 8) and chronic (8 weeks; N = 5) sheep models of IMR were studied. IMR was produced by ligation of circumflex branches to create myocardial infarction. An adjustable poly-mesh device was attached to infarcted myocardium in acute and chronic IMR models and compared with untreated sham sheep. Two- and 3-dimensional echocardiography and hemodynamic measurements were performed at baseline, post IMR, and post poly-mesh (humanely killed). RESULTS:In acute models, moderate IMR developed in all sheep and decreased to trace/mild (vena contracta: 0.50 ± 0.09 cm to 0.26 ± 0.12 cm; P < .01) after poly-mesh. In chronic models, IMR decreased in all sheep after poly-mesh, and this reduction persisted over 8 weeks (vena contracta: 0.42 ± 0.09 cm to 0.08 ± 0.12 cm; P < .01) with significant increase in the slope of end-systolic pressure-volume relationship (1.1 ± 0.5 mm Hg/mL to 2.9 ± 0.7 mm Hg/mL; P < .05). There was a significant reduction in LV volumes from chronic IMR to euthanasia stage with poly-mesh compared with sham group (%end-diastolic volume change -20 ± 11 vs 15% ± 16%, P < .01; %end-systolic volume change -14% ± 19% vs 22% ± 22%, P < .05; poly-mesh vs sham group) consistent with reverse remodeling. CONCLUSIONS:An adjustable polymer filled mesh device reduces IMR and prevents continued LV remodeling during chronic follow-up.
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