Increased remote extracellular volume measured by CMR T1 mapping allows early identification of left atrial dysfunction in hypertrophic cardiomyopathy

Abstract

Abstract Background Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) detects replacement fibrosis (RF) through late gadolinium enhancement (LGE) and interstitial fibrosis (IF) in apparently unscarred myocardium by T1 mapping-derived increased extracellular volume (ECV). Differently from LGE, to date only few small studies have explored the clinical significance of IF in HCM and a correlation between IF and diastolic dysfunction (DD) has been proposed. However, DD detection is challenging in this population since the accuracy of standard echocardiographic parameters is controversial, especially in presence of left ventricular outflow tract obstruction (LVOTO). Left atrial (LA) dysfunction is associated with high left ventricular (LV) filling pressures and may represent an early marker of DD in HCM. Purpose To explore the correlation between IF and LA dysfunction in HCM patients with preserved systolic function. Methods 93 consecutive HCM patients with preserved EF underwent a standard CMR scan. Semi-automatic threshold-based quantification of ventricular volumes, function and mass was performed. LA volumes (LAV) and function were evaluated by CMR feature-tracking (FT) analysis. The three atrial phasic functions were analyzed: (i) passive strain (εe), (ii) active strain (εa) and (iii) total strain (εs). LGE was quantified using the standard deviations (SDs) method (≥4 SDs). IF was assessed by T1 mapping-derived ECV quantification in remote myocardium (r-ECV). A matched group of 15 healthy subjects (HS) served as controls. Results Compared to HS, HCM patients showed increased LAV (LAV max: HS 39±9ml, HCM 59±20 ml; LAV min: HS 16±4 ml, HCM 34±17 ml; p<0.001), reduced LA EF (HS 61±3%, HCM 45±12%, p<0.001), impaired εs (HS 40±7%, HCM 29±11%, p<0.001) and εe (HS 26±7%, HCM 15±7%, p<0.001). No differences in εa were observed (HS 13±4%, HCM 14±7%, p 0.56). HCM patients were divided into 2 groups according to the presence of IF, defined as r-ECV values ≥29%. The two ECV groups did not differ in terms of LV EF, LA EF, LAV, LA area, E/E', LGE, LV mass, maximal wall thickness and LVOTO (all p>0.05). HCM patients with increased r-ECV showed significantly impaired LA function in terms of all three strain parameters vs. normal r-ECV group (HCM r-ECV <29%: εs 31±12%, εe15±7%, εa 15±5%; HCM r-ECV≥29%: εs 24±7%, εe 12±4%, εa 12±5%; all p<0.05). Conclusions In HCM patients increased r-ECV correlates with LA dysfunction, hinting towards a possible role for IF in determining altered LV relaxation and DD. LA strain in controls and HCM ECV groups Funding Acknowledgement Type of funding source: None