Rare case of mitral annulus disjunction and noncompaction-like myocardium.
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ABSTRACT:
SUBMITTER: Tan BE
PROVIDER: S-EPMC8015991 | biostudies-literature | 2020
REPOSITORIES: biostudies-literature
Publications
Rare case of mitral annulus disjunction and noncompaction-like myocardium.
Tan Bryan E-Xin BE Balmer-Swain Mallory M Rao Mohan M Baibhav Bipul B
Cardiology journal 20200101 3
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| S-EPMC7498626 | biostudies-literature
Project description:AimsWe aimed to assess the prevalence of mitral annulus disjunction (MAD) and to explore the association with aortic disease and mitral valve surgery in patients with Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS).Methods and resultsWe included consecutive MFS patients fulfilling Revised Ghent Criteria and LDS patients fulfilling Loeys-Dietz Revised Nosology. MAD was identified by echocardiography and was quantified as the longitudinal distance from the ventricular myocardium to the hinge point of the posterior mitral leaflet. Aortic events were defined as aortic dissection or prophylactic aortic surgery. We recorded the need of mitral valve surgery including mitral valve repair or replacement. We included 168 patients (103 with MFS and 65 with LDS). The prevalence of MAD was 41%. MAD was present in all age groups. Aortic events occurred in 112 (67%) patients (27 with dissections and 85 with prophylactic surgical interventions). Patients with MAD were younger at aortic event than those without MAD (log rank = 0.02) Patients with aortic events had greater MAD distance in posterolateral wall [8 (7-10) mm vs. 7 (6-8) mm, P = 0.04]. Mitral events occurred more frequently in patients with MAD (P < 0.001).ConclusionMAD was highly prevalent in patients with MFS and LDS. MAD was a marker of severe disease including aortic events at younger age and need of mitral valve surgery. Screening patients with MFS an LDS for MAD may provide prognostic information and may be relevant in planning surgical intervention. Detection of MAD in patients with MFS and LDS may infer closer clinical follow-up from younger age.
| S-EPMC8370565 | biostudies-literature
Project description: Not available
| S-EPMC10895090 | biostudies-literature
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| S-EPMC8370866 | biostudies-literature
Project description:BackgroundThe presence of mitral annulus disjunction (MAD) has been considered a high-risk feature for sudden cardiac death based on selected study populations. We aimed to assess the prevalence of MAD in consecutive patients undergoing clinically indicated cardiovascular magnetic resonance (CMR), its association with ventricular arrhythmias, mitral valve prolapse (MVP), and other CMR features.MethodsThis single-center retrospective study included consecutive patients referred to CMR at our institution between June 2021 and November 2021. MAD was defined as a ≥1 mm displacement between the left atrial wall-mitral valve leaflet junction and the left ventricular wall during end-systole. MAD extent was defined as the maximum longitudinal displacement. Associates of MAD were evaluated at univariable and multivariable regression analysis. The study endpoint, a composite of (aborted) sudden cardiac death, unexplained syncope, and sustained ventricular tachycardia, was evaluated at a 12-month follow-up.ResultsFour hundred and forty-one patients 55 ± 18 years, 267/441 (61%) males) were included, and 29/441 (7%) had MVP. The prevalence of MAD ≥1 mm, 4 mm, and 6 mm was 214/441 (49%), 63/441 (14%), and 15/441 (3%), respectively. Patients with MVP showed a higher prevalence of MAD greater than 1 mm (26/29 (90%) vs 118/412 (46%)); p < 0.001), 4 mm (14/29 (48%) vs 49/412 (12%)); p < 0.001), and 6 mm (3/29 (10%) vs 12/412 (3%)); p = 0.03), and a greater MAD extent (4.2 mm, 3.0-5.7 mm vs 2.8 mm, 1.9-4.0 mm; p < 0.001) compared to patients without MVP. MVP was the only morpho-functional abnormality associated with MAD at multivariable analysis (p < 0.001). A high burden of ventricular ectopic beats at baseline Holter-electrocardiogram was associated with MAD ≥4 mm and MAD extent (p < 0.05). The presence of MAD ≥1 mm (0.9% vs 1.8%; p = 0.46), MAD ≥4 mm (1.6% vs 1.3%; p = 0.87), or MVP (3.5% vs 1.2%; p = 0.32) were not associated with the study endpoint, whereas patients with MAD ≥6 mm showed a trend toward a higher likelihood of the study endpoint (6.7% vs 1.2%; p = 0.07).ConclusionMAD of limited severity was common in consecutive patients undergoing CMR. Patients with MVP showed higher prevalence and greater extent of MAD. Extended MAD was rarer and showed association with ventricular arrhythmias at baseline. The mid-term prognosis of MAD seems benign; however, prospective studies are warranted to search for potential "malignant MAD extents" to improve patients' risk stratification.
| S-EPMC11334631 | biostudies-literature
Project description:Numerous postpneumonectomy complications exist. We present a rare clinical case of postpneumonectomy exertional dyspnea revealing compression of the mitral annulus by the descending aorta. The patient was 42-year-old former smoker with pulmonary emphysema. He has been operated on, in 2012 (i.e., right pneumonectomy). Before the surgery, the patient was a recreational runner. However, after some months, it was difficult for the patient to resume running. Cardiopulmonary exercise testing indicated moderate exercise intolerance with important oxygen desaturation. More interestingly, a decrease of low oxygen pulse was noticed from the first ventilatory threshold with no electrical modification on the electrocardiogram. This decrease was indicative of a decline in stroke volume. The thoracic scan revealed a right pneumonectomy pocket with a liquid abnormal content. Moreover, the mediastinum had shifted toward the pneumonectomy space and the left lung was distended and emphysematous. Echocardiography revealed a major change in the mediastinal anatomy. The mitral annulus was observed to be compressed by the rear wall of the descending aorta. The diagnosis of postpneumonectomy syndrome or platypnea-orthodeoxia syndrome was ruled out in this patient. Mitral annular compression by the descending aorta is rare complication, which must be researched in patients with postpneumonectomy exertional dyspnea.
| S-EPMC5220442 | biostudies-other
Project description:BackgroundNoncompaction of ventricular myocardium(NVM) is a rare kind of cardiomyopathy associated with genetic mutations and nongenetic factors, among which the isolated right ventricular noncompaction (iRVNC) is the most rare type. ACVRL1 is the pathogenic gene of type 2 hereditary hemorrhagic telangiectasia (HHT2), and there's no NVM reported to be associated with ACVRL1 mutation.Case presentationThis is a rare case diagnosed as iRVNC and pulmonary hypertention with ACVRL1 mutation detected.ConclusioniRVNC in this case may be due to ACVRL1 mutation, secondary to pulmonary hypertention and right ventricular failure caused by ACVRL1 mutation, or they happened in the same case coincidently.
| S-EPMC10149031 | biostudies-literature
Project description:BackgroundCaseous calcification of the mitral annulus (CCMA) is a very rare variant of mitral annular calcification, which is typically asymptomatic but can manifest as a cardiac tumour, abscess, or in the form of mitral valve dysfunction.Case summaryWe present a patient who developed shortness of breath and was initially thought to have an intracardiac tumour, but ultimately was recognized as massive calcification of the mitral valve by computed tomography angiogram. This finding was unfortunately made only shortly before the patient's clinical deterioration, and the specific diagnosis of CCMA was made on post-mortem findings, precluding any directed treatment for this entity.DiscussionCaseous calcification of the mitral annulus can lead to significant pathology, including mitral regurgitation, stenosis, or systemic embolization of caseous material. Due to its relative scarcity and tendency to be asymptomatic, a diagnosis of CCMA is often difficult to make or easily overlooked. Early recognition and appropriate treatment is crucial for avoiding these potential complications.
| S-EPMC6426072 | biostudies-literature
Project description:BackgroundCaseous calcification of the mitral annulus is an extremely rare variant of mitral annulus calcification occurring in <1% of cases. The degeneration of caseous masses could act as a source of embolic strokes and a nidus for infective endocarditis (IE).Case summaryA man in his sixties presented with transient left arm weakness. His history was pertinent for bioprosthetic aortic valve replacement secondary to endocarditis and recent pneumococcal pneumonia complicated by empyema and bacteraemia. He was still on intravenous antibiotics when he presented. Evaluation including magnetic resonance imaging of the brain, transoesophageal echocardiography, and computed tomography (CT) of the chest revealed multifocal embolic strokes, degenerative bioprosthetic aortic valve dysfunction, mitral annular calcification with mobile calcific masses, and persistent empyema. 18F-fluorodeoxyglucose-positron emission tomography-CT showed indeterminate activity across a portion of the posterior mitral leaflet and no activity on the bioprosthetic aortic valve. The patient was deemed high risk for surgery and was treated with 6-week course of antibiotics both for the empyema and the possible IE of the native mitral valve. Repeat echocardiography 40 days later showed stable mitral masses. At 4 months of follow up, the patient had no evidence of recurrent clinical strokes.DiscussionCaseous calcification of the mitral annulus is a rare but an increasingly recognized predisposing factor for embolic strokes and IE. Treatment ideally involves surgical resection of the calcified masses in such cases.
| S-EPMC9675592 | biostudies-literature
Project description: Not available
| S-EPMC5881084 | biostudies-literature