Management Strategies in Arrhythmogenic Cardiomyopathy across the Spectrum of Ventricular Involvement

Figures

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  Figure 1. Representative heart explant pathology in arrhythmogenic cardiomyopathy (ACM). (A): Gross pathology focusing on the right ventricle demonstrating significant dilation and wall thinning with fatty replacement. (B): Hematoxylin and eosin (H&E) stain of right ventricle myocardium at 2×x magnification showing significant fibrofatty replacement surrounding residual cardiomyocytes. (C): Masson trichrome stain of the right ventricle at 4×x magnification showing increased fibrosis along with fatty infiltration.

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  Figure 2. Venn diagram highlighting similarities and differences for symptoms and objective data between predominant right ventricle and left ventricle dysfunction. CO, cardiac output; FAC, fractional area change; JVD, jugular venous distension; LV, left ventricle; LVEF, left ventricle ejection fraction; NT-proBNP, N-terminal prohormone brain natriuretic peptide; PAPI, pulmonary artery pulsatility index; PCWP, pulmonary capillary wedge pressure; PND, paroxysmal nocturnal dyspnea; PMI, point of maximal impulse; pVO2, peak oxygen uptake; RAP, right atrial pressure; RV, right ventricle; TAPSE, tricuspid annular plane systolic excursion; TR, tricuspid regurgitation.

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  Figure 3. Heart failure management considerations across the arrhythmogenic cardiomyopathy disease spectrum. ACM, arrhythmogenic cardiomyopathy; CMR, cardiac magnetic resonance; CPET, cardiopulmonary exercise test; ICD, implantable cardioverter defibrillator; LVAD, left ventricular assist device; RHC, right heart catheterization; RV, right ventricle.

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  Figure 4. Global overview of heart failure management in arrhythmogenic cardiomyopathy. ACEi, angiotensin converting enzyme inhibitor; ACM, arrhythmogenic cardiomyopathy; ARB, angiotensin receptor blocker; CPET, cardiopulmonary exercise test; CRT, cardiac rexynchronization therapy; GDMT, guideline-directed medical therapy; ISDN, isosorbide dinitrate; LVAD, left ventricular assist device; LVEF, left ventricle ejection fraction; MRA, mineralocorticoid receptor antagonist; RV, right ventricle; SGLT2i, sodium/glucose cotransporter-2 inhibitor; TV, tricuspid valve; VT, ventricular tachycardia.