Aneurysmal protrusion of the posterior leaflet of the mitral valve: An auscultatory-electrocardiographic syndrome

doi:10.1016/0002-8703(66)90179-7

American Heart Journal

Volume 71, Issue 2, February 1966, Pages 166-178

https://doi.org/10.1016/0002-8703(66)90179-7 Get rights and content

Abstract

In previous communications, evidence has been produced that apical late systolic murmurs are invariably due to mitral regurgitation, and that the commonly associated nonejection systolic clicks result from abnormalities of the mitral chordae tendineae. The present paper describes 5 patients with both of these auscultatory findings who had, in addition, electrocardiographic signs compatible with posteroinferior myocardial ischemia or infarction. Left ventricular cineangiocardiography was performed in 4 of the 5 patients, and an aneurysmal dilatation of the posterior leaflet of the mitral valve was demonstrated.

It is postulated that distortion of the circumflex coronary artery by the abnormal mitral valve may be the cause of the electrocardiographic features. The probability is briefly discussed that in some instances the mitral valve anomaly results from a genetically determined defect, or weakness, of the posterior leaflet.

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Genetic mechanisms underlying arrhythmogenic mitral valve prolapse: Current and future perspectives
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Mitral valve prolapse (MVP) is a heart valve disease that is often familial, affecting 2%–3% of the general population. MVP with or without mitral regurgitation can be associated with an increased risk of ventricular arrhythmias and sudden cardiac death (SCD). Research on familial MVP has specifically focused on genetic factors, which may explain the heritable component of the disease estimated to be present in 20%–35%. Furthermore, the structural and electrophysiological substrates underlying SCD/ventricular arrhythmia risk in MVP have been studied postmortem and in the electrophysiology laboratory, respectively. Understanding how familial MVP and rhythm disorders are related may help patients with MVP by individualizing risk and working to develop effective management strategies. This contemporary, state-of-the-art, expert review focuses on genetic factors and familial components that underlie MVP and arrhythmia and encapsulates clinical, genetic, and electrophysiological issues that should be the objectives of future research.
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Mitral valve prolapse (MVP) affects about 2% to 3% of the general population, mostly women, and is the most common cause of primary chronic mitral regurgitation (MR) in western countries. The natural history is heterogeneous and widely determined by the severity of MR. Although most patients remain asymptomatic with a near-normal life expectancy, approximately 5% to 10 % progress to severe MR. As largely recognized, left ventricular (LV) dysfunction due to chronic volume overload per se identifies a subgroup at risk of cardiac death. However, there is rising evidence of a link between MVP and life threating ventricular arrhythmias (VAs)/sudden cardiac death (SCD) in a small subset of middle-aged patients without significant MR, heart failure and remodeled hearts. The present review focuses on the underlying mechanism of electric instability and unexpected cardiac death in this subset of young patients, from the myocardial scarring of the LV infero-lateral wall due to mechanical stretch exerted by the prolapsing leaflets and mitral annular disjunction, to the inflammation's impact on fibrosis pathways along with a constitutional hyperadrenergic state. The heterogeneity of clinical course reveals a necessity of risk stratification, preferably through noninvasive multimodality imaging, that will help to identify and prevent adverse scenarios in young MVP patients.
An autopsy case of sudden unexpected death with Barlow's disease
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Citation Excerpt :
Carpentier and colleagues then characterized the surgical lesions seen in Barlow's disease as a separate category in MVP and differentiated them from other MVP cases without billowing or excess tissue, whose cases were categorized as fibroelastic deficiency (FED) [3]. They defined Barlow's disease as a bileaflet prolapse and billowing valve with excessively thickened leaflet [3]. The histopathological hallmark of Barlow's disease is myxoid degeneration, which is the accumulation of proteoglycan with destruction of the 3-layer structure and collagen alteration [4].
A 45-year-old man was clinically diagnosed with mitral valve regurgitation 2 years before death. The autopsy showed left ventricular hypertrophy and mitral valve prolapse of the bileaflet with billowing valve and excessively thickened leaflet, the findings of which were consistent with Barlow's disease. Microscopically, destruction of the 3-layer structure of the mitral valve and advanced interstitial fibrosis of the left ventricular wall were evident. Additionally, a marked but limited reduction in conduction fibers was found in the branching point of the left and right branches, as seen in cases of idiopathic complete atrioventricular block. Genetic investigation using whole-exome sequencing showed some genetic variants with uncertain significance. In patients with Barlow's disease, a marked reduction of conduction fibers might be a subtype of sudden cardiac death. The overlap of some arrhythmogenic substrate in the heart may increase the risk of sudden cardiac death with asymptomatic Barlow's disease.
Commentary: A novel cross-species model of Barlow's disease to biomechanically analyze repair techniques in an ex vivo left heart simulator
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A novel cross-species model of Barlow's disease to biomechanically analyze repair techniques in an ex vivo left heart simulator
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Barlow's disease remains challenging to repair, given the complex valvular morphology and lack of quantitative data to compare techniques. Although there have been recent strides in ex vivo evaluation of cardiac mechanics, to our knowledge, there is no disease model that accurately simulates the morphology and pathophysiology of Barlow's disease. The purpose of this study was to design such a model.
To simulate Barlow's disease, a cross-species ex vivo model was developed. Bovine mitral valves (n = 4) were sewn into a porcine annulus mount to create excess leaflet tissue and elongated chordae. A heart simulator generated physiologic conditions while hemodynamic data, high-speed videography, and chordal force measurements were collected. The regurgitant valves were repaired using nonresectional repair techniques such as neochord placement.
The model successfully imitated the complexities of Barlow's disease, including redundant, billowing bileaflet tissues with notable regurgitation. After repair, hemodynamic data confirmed reduction of mitral leakage volume (25.9 ± 2.9 vs 2.1 ± 1.8 mL, P < .001) and strain gauge analysis revealed lower primary chordae forces (0.51 ± 0.17 vs 0.10 ± 0.05 N, P < .001). In addition, the maximum rate of change of force was significantly lower postrepair for both primary (30.80 ± 11.38 vs 8.59 ± 4.83 N/s, P < .001) and secondary chordae (33.52 ± 10.59 vs 19.07 ± 7.00 N/s, P = .006).
This study provides insight into the biomechanics of Barlow's disease, including sharply fluctuating force profiles experienced by elongated chordae prerepair, as well as restoration of primary chordae forces postrepair. Our disease model facilitates further in-depth analyses to optimize the repair of Barlow's disease.
Professor John Barlow - The Legend
2020, Revista Portuguesa de Cardiologia

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^∗: Address: Department of Medicine, University of the Witwatersrand School of Medicine, Johannesburg, South Africa.

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Clinical communicationAneurysmal protrusion of the posterior leaflet of the mitral valve: An auscultatory-electrocardiographic syndrome

Abstract

Am. Heart J.

J. Chron. Dis.

Prog. Cardiovas. Dis.

Prog. Cardiovas. Dis.

Am. Heart J.

Dis. Chest

Prog. Cardiovas. Dis.

Dis. Chest

Am. Heart J.

Am. Heart J.

Circulation

Lancet

Am. Heart J.

Am. Heart J.

The significance of late systolic murmurs and mid-late systolic clicks

Maryland M. J.

The atrial sound in hypertension and ischaemic heart disease

Brit. Heart J.

The systolic murmur. Its clinical significance

J.A.M.A.

Left ventricular aneurysm of uncertain etiology

Brit. Heart J.

Clinical communication
Aneurysmal protrusion of the posterior leaflet of the mitral valve: An auscultatory-electrocardiographic syndrome