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Review
. 2023 Dec 9;11(12):3259.
doi: 10.3390/biomedicines11123259.

Management Strategies in Arrhythmogenic Cardiomyopathy across the Spectrum of Ventricular Involvement

Yash Maniar  1 , Nisha A Gilotra  1 , Paul J Scheel 3rd  1
Affiliations
Review

Management Strategies in Arrhythmogenic Cardiomyopathy across the Spectrum of Ventricular Involvement

Yash Maniar et al. Biomedicines. .

Abstract

Improved disease recognition through family screening and increased life expectancy with appropriate sudden cardiac death prevention has increased the burden of heart failure in arrhythmogenic cardiomyopathy (ACM). Heart failure management guidelines are well established but primarily focus on left ventricle function. A significant proportion of patients with ACM have predominant or isolated right ventricle (RV) dysfunction. Management of RV dysfunction in ACM lacks evidence but requires special considerations across the spectrum of heart failure regarding the initial diagnosis, subsequent management, monitoring for progression, and end-stage disease management. In this review, we discuss the unique aspects of heart failure management in ACM with a special focus on RV dysfunction.

Keywords: arrhythmogenic cardiomyopathy; arrhythmogenic right ventricular cardiomyopathy; guideline-directed medical therapy; heart failure.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
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.
Figure 2
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.
Figure 3
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.
Figure 4
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.
See this image and copyright information in PMC

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