Mitral Valve Prolapse – ABC CARDIOL

ABCIC

Original Article

The Many Faces of Arrhythmic Mitral Valve Prolapse: Case Series

Yasmim Evelyn Lisboa Barbosa et al. Isadora Wanderley Queiroga de Freitas Evangelista Julyana Maria Ramalho de Sousa Lorena Carvalho Leite Jales … Elisabete Louise de Medeiros Viegas Patrícia Vidal de Negreiros Nóbrega Daniel Moreira Costa Moura Gabriel Pelegrineti Targueta Lilian Amador Resende Renner Augusto Raposo Pereira Guilherme Augusto Teodoro Athayde

Abstract

Background:

Although it is frequently a benign condition, mitral valve prolapse (MVP) may be associated with an increased risk of ventricular arrhythmias (VA), a condition known as arrhythmic mitral valve prolapse (AMVP).

Objectives:

To present diverse manifestations of AMVP by means of clinical cases that illustrate these patients’ symptoms and electrocardiogram (ECG), 24-hour Holter, transthoracic echocardiogram (TTE), and cardiac magnetic resonance imaging (CMRI) findings and to discuss the decisions made in relation to different clinical outcomes.

Methods:

This retrospective, descriptive, and observational study analyzed 5 patients with AMVP treated between 2019 and 2024, investigating markers of elevated risk for severe clinical outcomes, especially sudden cardiac death (SCD).

Results:

Among the 5 patients evaluated, the most common clinical presentation was palpitations (100% of cases), followed by syncope (40%). Three patients (60%) had severe or very severe arrhythmia on 24-hour Holter monitoring, while 4 (80%) had mitral annular disjunction (MAD). Positive delayed enhancement was observed in 2 (40%) patients. In 2 cases (40%), implantable cardioverter-defibrillator (ICD) was indicated. In 1 case, mitral valve replacement was chosen, but the arrhythmias were not resolved; consequently, radiofrequency ablation was indicated. Although SCD has been described in the literature, no cases were observed in our sample.

Conclusion:

AMVP may present distinct clinical manifestations, including severe outcomes. It is essential to identify risk markers for early diagnosis and treatment of this condition, with the goal of reducing SCD-related mortality in these patients.

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Case Report

Marfan Syndrome, Hypertrophic Cardiomyopathy And Long QT A Rare Association Causing Sudden Death

Diana Carvalho et al. Simão Carvalho Adriana Pacheco Carlos Costa … Pedro Carvalho Raquel Ferreira Ana Briosa

Introduction

Marfan syndrome (MS) is a systemic disease of the connective tissue with an autosomal dominant transmission, usually associated with a mutation in the fibrillin 1 gene (FBN1). The estimated prevalence is 6.5/100,000. According to the revised Ghent criteria, mutation in the FBN1 gene, lens ectopy, and aortic root dilation are the key factors for the diagnosis of MS.^, Life expectancy is essentially determined by cardiovascular complications, particularly aortopathy.

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Research Letter

Sudden Death in a Breastfeeding Woman with Arrhythmogenic Mitral Valve Prolapse

Erika Olivier Vilela Bragança et al. Fabio Luis Valério da Silva

Introduction

Mitral valve prolapse (MVP) is the most common valvular heart disease, affecting 2-3% of the general population, and is defined by the systolic displacement of one or both mitral valve leaflets ≥2 mm above the plane of the mitral annulus in the sagittal view of the mitral valve via echocardiography. The outcomes of MVP without regurgitation are generally benign; however, a small, ill-defined subgroup of individuals is at high risk for malignant arrhythmias and sudden death (SD). Autopsy data show a 4% to 7% prevalence of MVP in young people with SD. Due to the low event rate and lack of cohorts with a robust number of patients, accessing the accurate incidence of SD and MVP in the general population and in patient subgroups remains a challenge. The diagnosis of arrhythmogenic MVP is made in the presence of MVP and ventricular arrhythmia, considering a ventricular arrhythmia density ≥5% on 24-hour electrocardiogram (ECG) Holter or the presence of complex arrhythmia – non-sustained ventricular tachycardia (NSVT), ventricular tachycardia (VT) or ventricular fibrillation (VF), as well as in the absence of any other arrhythmogenic substrate.

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ABCIC

Review Article

Mitral Annulus Disjunction: Diagnostic Modalities, Clinical Implications, and Prognostic Progression

Alexandre Costa Souza et al. Marcus Vinicius Silva Freire de Carvalho Marco André Sales Suellen Lacerda Bezerra … Jorge Adinon Torreão Carolina Thé Macêdo

Abstract

Mitral annulus disjunction (MAD) is an abnormal insertion of the flexion line of the mitral annulus into the atrial wall. The annulus presents a separation (disjunction) between the posterior leaflet–atrial wall junction and the left ventricular myocardial crest.¹ MAD was first described more than 30 years ago in an autopsy study and is reportedly related to mitral valve prolapse (MVP) in 92% of cases.² Since then, several studies have been conducted, and reports on the prevalence of MAD in patients with MVP have varied. Ultimately, it may or may not be associated with mitral regurgitation. Transthoracic echocardiography is part of initial MVP assessment, allowing its diagnosis and the assessment of related complications. As new diagnostic methods emerged, cardiac magnetic resonance imaging and transesophageal echocardiography improved the assessment of this pathology in terms of its diagnosis, extension, and location. However, the phenotypic characteristics of MVP that are more closely associated with MAD remain uncertain mainly due to the limited number of patients in classic studies on the subject. Patients with MAD may develop symptoms related to ventricular arrhythmias, configuring the MAD arrhythmic syndrome, which may progress to sudden death. The literature presents conflicting prognostic data among several studies on the subject from clear diagnostic criteria and best imaging method to be used to treatment and prognosis. This review describes MAD characteristics associated (or not) with valve prolapse to improve the diagnosis and management of this important pathology.

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Image

Incremental Value Of 3D Transesophageal Echocardiography for The Assessment of Mitral Valve Prolapse

Viviane Tiemi Hotta et al. Mirian Magalhães Pardi Valdir Ambrósio Moisés

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Artigo Original

Cardiovascular magnetic resonance imaging-derived mitral valve geometry in determining mitral regurgitation severity

Andre Mauricio Fernandes et al. Vikas Rathi Robert W. Biederman Mark Doyle … June A. Yamrozik Ronald B. Willians Vinayak Hedge Saundra Graunt Roque Aras Jr.

BACKGROUND: Mitral regurgitation is the most common valvular heart disease worldwide. Magnetic resonance may be a useful tool to analyze mitral valve parameters. OBJECTIVE: To distinguish mitral valve geometric patterns in patients with different severities of mitral regurgitation (MR) based on cardiovascular magnetic resonance imaging. METHODS: Sixty-three patients underwent cardiovascular magnetic resonance imaging. Mitral valve parameters analyzed were: tenting area (mm2) and angle (degrees), ventricle height (mm), tenting height (mm), anterior leaflet, posterior leaflet length and annulus diameter (mm). Patients were divided into two groups, one including patients who required mitral valve surgery and another which did not. RESULTS: Thirty-six patients had trace to mild (1-2+) MR and 27 had moderate to severe MR (3-4+). Ten (15.9%) out of 63 patients underwent surgery. Patients with more severe MR had a larger left ventricle end systolic diameter (38.6 ± 10.2 vs 45.4 ± 16.8, p<0.05) and left end diastolic diameter (52.9 ± 6.8 vs 60.1 ± 12.3, p= 0.005). On multivariate analysis, the tenting area was the strongest determinant of MR severity (r= 0.62, p=0.035). Annulus length (36.1 ± 4.7 vs 41 ± 6.7, p< 0.001), tenting area (190.7 ± 149.7 vs 130 ± 71.3, p= 0.048) and posterior leaflet length (15.1 ± 4.1 vs 12.2 ± 3.5, p= 0.023) were larger on patients requiring mitral valve surgery. CONCLUSIONS: Tenting area, annulus and posterior leaflet length are possible determinants of MR severity. These geometric parameters could be used to determine severity and could, in the future, direct specific patient care based on individual mitral apparatus anatomy.

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Review Article

Joint hypermobility in patients with mitral valve prolapse

Simone Cavenaghi et al. Lais Helena Carvalho Marino Patrícia Pantaleão Oliveira Neuseli Marino Lamari

Studies on hypermobility have aroused great interest in the last decades, as they are associated to musculoskeletal disorders, as well as abnormalities in several organic systems, such as the mitral valve prolapse. Therefore, in this study, data on the association between joint hypermobility and the mitral valve prolapse were investigated and reviewed. Studies in the literature have shown that genetic alterations in the collagen composition seem to be the main cause of this association.

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Artigo Original

Serial clinical and echocardiographic evaluation in children with Marfan syndrome

Victor Manuel Oporto Lopez et al. Ana Beatriz Alvarez Perez Valdir Ambrósio Moisés Lourdes Gomes … Patricia da Silveira Pedreira Célia C. Silva Orlando Campos Filho Antônio Carlos C. Carvalho

OBJECTIVE: To describe the clinical cardiac manifestations and temporal evolution of Marfan syndrome in children; to estimate the incidence of annuloaortic ectasia and mitral valve prolapse; and to evaluate tolerability and efficacy of beta-blockers in these patients. METHODS: During one year, 21children with Marfan syndrome underwent serial clinical and echocardiographic examinations. Echocardiograms assessed: the presence of mitral valve prolapse, aortic root diameter, mitral and aortic valves regurgitation, and aortic enlargement during beta-blocker therapy. Eleven patients had two measurements of the aortic root taken one year apart. RESULTS: The children were asymptomatic throughout the study. Mitral prolapse was found in 11 (52%) children. Annuloaortic ectasia occurred in 16 (76%) patients and found to be mild in 42.8%, moderate in 9.5%, and severe in 23.8%. One of these patients underwent aortic valve replacement and repair of the ascending aorta by the Bentall-De Bono technique, with good results. Heart rate decreased by 13.6% (from 85 to 73 bpm; p < 0.009) with the use of beta-blockers; however, aortic root diameter increased by 1.4 mm/year (p<0.02). One child could not be given beta-blockers due to bronchial asthma, and no significant side effects were observed in the remaining children, including one who also had bronchial asthma. CONCLUSION: The children remained asymptomatic throughout the study, the use of beta-blockers led to a significant decrease in heart rate, and no significant adverse effects were observed. Contrary to the literature, incidence of annuloaortic ectasia was high among the study population, greater than that of mitral valve prolapse, even during beta-blocker therapy.

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