Solving the Riddle of Sudden Cardiac Death in Hypertrophic Cardiomyopathy: The Added Role of Cardiac Magnetic Resonance

Figures

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  Figure 1. Initial work-up with electrocardiography and transthoracic echocardiography. (A) A 12−lead electrocardiogram. (B) A 2−dimensional (2D) transthoracic echocardiography (TTE) 4−chamber apical view showing biventricular hypertrophy with an “ace of spades” morphology of the left ventricular (LV) cavity. (C) A 2D−TTE parasternal short-axis view at the level of the apex showing biventricular hypertrophy. (D) A 2D speckle-tracking echocardiography (STE) map showing reduced global longitudinal strain in the mid-apical segments of the left ventricle. (E) A 2D right ventricle (RV) −focused 4−chamber apical view. (F,G) Conventional indices of longitudinal RV systolic function, i.e., TAPSE (F) and S’ TDI (G). (H) A 2−D STE map showing reduced RV free wall longitudinal strain.

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  Figure 2. Cardiac magnetic resonance morphological and fibrosis characterization. (A–C) Balanced Steady State Free Precession (bSSFP) cine 4−, 2− and 3−chamber views showing hypertrophy of the mid-apical segments of the left ventricle. Hypertrophy of the right ventricular wall and a right ventricular papillary muscle (red asterisk; panel (A)) is also noted. (D–F) Magnitude reconstruction (MAG) LGE 4−, 2− and 3−chamber views showing extensive late gadolinium enhancement (LGE) of the left ventricular mid-apical segments and the right (red arrow; panel (D)) ventricle. Ao = aorta, LV = left ventricle, RV = right ventricle, LA = left atrium, and RA = right atrium.

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  Figure 3. Multiparametric cardiac magnetic resonance tissue characterization. Basal (upward), mid (mid), and apical (downward) short-axis views are shown. (A–C) Balanced Steady State Free Precession (bSSFP) short-axis views showing hypertrophy of the mid-apical segments of the left ventricle (LV) and hypertrophy of the right ventricular (RV) wall. (D–F) Modified Look-Locker inversion recovery (MOLLI) T1-mapping showing elevated native T1 with increasing values from base to apex; elevated T1 values of the RV free wall are also shown. (G–I) True fast imaging with steady-state precession (True-FISP T2) mapping showing elevated native T2 values at the apex. (J–L) MOLLI post-contrast T1-mapping showing elevated post-contrast T1 and elevated extracellular volume (ECV) with increasing values from base to apex. (M–O) Magnitude reconstruction (MAG) late gadolinium enhancement (LGE) showing LGE of the mid-apical segments of the LV and RV.

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  Figure 4. Sudden cardiac death risk stratification and class of recommendation to ICD in the present patient according to traditional assessment (panel (A)) and advanced assessment including CMR (panel B). For both panels, the European Society of Cardiology and American Heart Association/American College of Cardiology algorithms are respectively shown on the left and right side of the figure. Parameters that changed between baseline and advanced evaluation are colored in “red”(panel (B)). BP: blood pressure; FH: family history; ICD: implantable cardiac defibrillator; LA: left atrium; LGE: late gadolinium enhancement; LV; left ventricle; LVEF: left ventricular ejection fraction; LVH: left ventricular hypertrophy; LVOT: left ventricular outflow tract; MLVWT: maximum left ventricular wall thickness; NSVT: non-sustained ventricular tachycardia; SCD: sudden cardiac death.