Does preliminary Chest Shape Assessment Improve the Prognostic Risk Stratification of Individuals with Mitral Annular Disjunction? A Case Report And Narrative Review

Mitral annular disjunction (MAD), a mitral annular abnormality involving the whole mitral valve annulus circumference, commonly detected in individuals with mitral valve prolapse (MVP), has been recently recognized as a potential risk factor for malignant ventricular arrhythmias (VAs) and sudden cardiac death. Recent evidence indicates that a multimodality imaging assessment comprehensive of echocardiography, cardiac magnetic resonance (CMR) and cardiac computed tomography angiography (CCTA) may improve the MAD detection. To date, no previous author considered the potential influence of chest wall conformation on MAD presence. Considering the strong association between MVP and anterior chest wall deformities and the increased prevalence of MAD among MVP individuals, we have hypothesized that MAD presence might be more frequently detected among MVP individuals with a narrow antero-posterior (A-P) thoracic diameter and/or concave-shaped chest wall conformation, as noninvasively assessed by the modified Haller index (MHI). Herein, we present a case of MVP female with relevant MAD distance and moderate mitral regurgitation (MR), who underwent a diagnostic study comprehensive of transthoracic echocardiography, transesophageal echocardiography, CMR, CCTA and exercise stress echocardiography. The patient was found with a concave-shaped chest wall conformation (MHI >2.5) and narrow A-P thoracic diameter (<13.5 cm), with a moderate and non-hemodynamically significant MR, without areas of LGE on CMR and with low arrhythmic profile. A preliminary chest shape assessment by the MHI might improve the prognostic risk stratification of MVP patients with MAD, potentially identifying a benign phenotype of MVP individuals, i.e. those with a narrow A-P thoracic diameter.