Sudden Cardiac Arrest in a Youth with Multiple Arrhythmic Substrates

Overview

Journal Case Rep Crit Care

Publisher Wiley

Specialty Critical Care

Date 2024 Apr 16

PMID 38623078

Authors

James Ainsworth

Adrian Ionescu

Affiliations

Soon will be listed here.

Abstract

Background: Mitral valve prolapse (MVP) is a common condition with an estimated prevalence of 1-3%, in which there is systolic displacement of a morphologically redundant mitral valve towards the left atrium. Mitral annular disjunction (MAD) is a separation of the MV attachment with the left ventricle, with hypermobility of the leaflets, and with systolic "curling" of the basal LV (left ventricle) myocardium. It is frequently associated with MVP and may confer an increased arrhythmic risk. . A 28-year-old male had ventricular fibrillation leading to out-of-hospital cardiac arrest, which was successfully resuscitated. His coronary arteries were unobstructed on invasive coronary angiography. Transthoracic echocardiogram (TTE) demonstrated MAD, confirmed by cardiac magnetic resonance (CMR) imaging and transoesophageal echocardiogram (TOE). The LV was severely dilated with reduced EF (ejection fraction), and the QTc interval was also prolonged. His father had died suddenly aged 50 years.

Conclusions: This report describes the clinical dilemma of identifying and treating a patient with multiple potential causes of cardiac arrest. Despite being relatively common, the clinical significance of MAD is still uncertain and the extent to which it may be linked with complications such as ventricular arrhythmias and sudden cardiac death. MAD appears to confer an increased risk of ventricular arrhythmias, particularly when associated with MVP, particularly nonsustained VT.

References

Lancellotti P, Garbi M . Malignant Mitral Valve Prolapse: Substrates to Ventricular Remodeling and Arrhythmias. Circ Cardiovasc Imaging. 2016; 9(8):e005248. DOI: 10.1161/CIRCIMAGING.116.005248. View

Groeneveld S, Kirkels F, Cramer M, Evertz R, Haugaa K, Postema P . Prevalence of Mitral Annulus Disjunction and Mitral Valve Prolapse in Patients With Idiopathic Ventricular Fibrillation. J Am Heart Assoc. 2022; 11(16):e025364. PMC: 9496286. DOI: 10.1161/JAHA.121.025364. View

Wu S, Siegel R . Mitral annular disjunction: A case series and review of the literature. Front Cardiovasc Med. 2022; 9:976066. PMC: 9411994. DOI: 10.3389/fcvm.2022.976066. View

Appignani M, Khanji M, Arbustini E, Stuppia L, Ceriello L, Di Girolamo E . Is Occult Genetic Substrate the Missing Link Between Arrhythmic Mitral Annular Disjunction Syndrome and Sudden Cardiac Death?. Can J Cardiol. 2021; 37(10):1651-1653. DOI: 10.1016/j.cjca.2021.04.014. View

Carmo P, Andrade M, Aguiar C, Rodrigues R, Gouveia R, Silva J . Mitral annular disjunction in myxomatous mitral valve disease: a relevant abnormality recognizable by transthoracic echocardiography. Cardiovasc Ultrasound. 2010; 8:53. PMC: 3014886. DOI: 10.1186/1476-7120-8-53. View

Kukavica D, Guglielmo M, Baggiano A, Muscogiuri G, Fusini L, Muratori M . Arrhythmic Mitral Valve Prolapse: Introducing an Era of Multimodality Imaging-Based Diagnosis and Risk Stratification. Diagnostics (Basel). 2021; 11(3). PMC: 7999774. DOI: 10.3390/diagnostics11030467. View

Hutchins G, Moore G, Skoog D . The association of floppy mitral valve with disjunction of the mitral annulus fibrosus. N Engl J Med. 1986; 314(9):535-40. DOI: 10.1056/NEJM198602273140902. View

Chakrabarti A, Bogun F, Liang J . Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management. J Cardiovasc Dev Dis. 2022; 9(2). PMC: 8879620. DOI: 10.3390/jcdd9020061. View

Tsianaka T, Matziris I, Kobe A, Euler A, Kuzo N, Erhart L . Mitral annular disjunction in patients with severe aortic stenosis: Extent and reproducibility of measurements with computed tomography. Eur J Radiol Open. 2021; 8:100335. PMC: 7960936. DOI: 10.1016/j.ejro.2021.100335. View

10.

Zhou N, Zhao Q, Li R, Cheng R, Wu Q, Cheng J . Mutation in mitral valve prolapse susceptible gene causes familial mitral annular disjunction. J Med Genet. 2023; 61(2):125-131. DOI: 10.1136/jmg-2023-109278. View