Malignant Mitral Valve Prolapse Research Articles

Mitral valve prolapse: an underestimated cause of sudden cardiac death-a current review of the literature.

Mitral valve prolapse (MVP) is a common valve abnormality in general population. Despite the general belief of a benign disorder, several articles since the 1980s report sudden cardiac death (SCD) in MVP patients, with a substantial percentage of asymptomatic young individuals. The problem is to detect those patients at increased risk and implement methods that are suitable to prevent cardiac arrest. This review investigates the correlation between MVP and SCD, the understanding of the pathophysiology, the strategies for detecting those at risk and treatment options. A complete literature survey was performed using PubMed database search to gather available information regarding MVP and SCD. A total of 33 studies met selection criteria for inclusion in the review. MVP is an underrated cause of arrhythmic SCD. The subset of patients with malignant MVP who may be at greater risk for SCD is characterized by young women with bileaflet MVP, biphasic or inverted T waves in the inferior leads, and frequent complex ventricular ectopic activity with documented ventricular bigeminy or ventricular tachycardia (VT) and premature ventricular contractions (PVCs) configurations of outflow tract alternating with fascicular origin or papillary muscle. MVP is a common condition in the general population and is often encountered in asymptomatic individuals. The existing literature continues to generate significant controversy regarding the association of MVP with ventricular arrhythmias and SCD. Early echocardiography and cardiac magnetic resonance (CMR) are essential, as is a greater understanding of the potential electrophysiological processes of primary arrhythmogenesis and the evaluation of the genetic substrate.

Clinical Cases: Valvular heart disease142A sub-aortic valve mass in a rheumatoid arthritis patient: an unconventional mechanism of aortic regurgitation143Symptomatic severe aortic regurgitation with coronary obstruction due to chronic type a dissection144Mitral valve prolapse and ventricular tachycardia: a long-lasting love story

Clinical Cases: Valvular heart disease142A sub-aortic valve mass in a rheumatoid arthritis patient: an unconventional mechanism of aortic regurgitation143Symptomatic severe aortic regurgitation with coronary obstruction due to chronic type a dissection144Mitral valve prolapse and ventricular tachycardia: a long-lasting love story

Abstract 314: Sensory Innervation of the Papillary Muscle: A Neural Link Between Mitral Valve Prolapse and Malignant Ventricular Arrhythmias?

Background: Malignant mitral valve prolapse (MVP) identifies a subset of patients with MVP associated with ventricular arrhythmias (VAs), including sudden cardiac death (SCD). We hypothesized that papillary muscles, which see significant mechanical stress in MVP, are richly innervated with sensory nerves, which induce severe autonomic imbalance on the heart and may trigger malignant VAs. Methods and Results: Longitudinal sections of the anterior and posterior left ventricular papillary muscles, right ventricular outflow tract (RVOT), ventricular septum, and basolateral left ventricular wall (LV) of Yorkshire pigs (n=8) were excised, formalin-fixed, paraffin-embedded, and sectioned. Immuno-staining for protein gene product 9.5 (PGP9.5), a pan-neuronal marker, and calcitonin-gene related peptide (CGRP), a sensory afferent neuron marker, was performed. Areas of immunoreactivity (IR) for CGRP were verified by direct comparison to PGP9.5 IR. The density of CGRP was then compared across anatomical regions. The mean CGRP IR area was 1229.44 ± 116.65 μm 2 /nucleus in papillary muscles, while in the septum, basolateral LV, and RVOT, the CGRP stained areas were 699.36 ± 88.28 μm 2 /nucleus, 681.51 ± 81.90 μm 2 /nucleus, and 381.98 ± 31.14 μm 2 /nucleus, respectively (p<0.001). There was no significant difference between CGRP IR area in the anterior papillary muscle (1091.36 ± 189.82 μm 2 /nucleus) and the posterior papillary muscle (1347.79 ± 140.50 μm 2 /nucleus) (p>0.3). Conclusions: There is a significantly greater amount of afferent innervation in the papillary muscle compared to the septum, basolateral LV, and RVOT. This enrichment of afferent innervation warrants further study to understand how afferent neurotransmission during abnormal mitral valve function may impact ventricular electrophysiology.

Letter by Providencia and Lambiase Regarding Article, “Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death”

Basso and colleagues1 elegantly describe a well-circumscribed entity combining mitral valve prolapse (MVP) with left ventricular fibrosis and ventricular arrhythmia causing 7% of all sudden cardiac deaths (SCDs) in young adults from the Veneto region. Malignant MVP affects a tiny minority of patients with MVP, and it is responsible for a very small absolute number of all SCDs in young adults. Assuming that the annual incidence of SCD in this age group ranges between 1.21 (in young athletes) and 3.76 (general population aged 12–35) per 100 000,2 malignant MVP may be causing …