March29, 2023

Abstract Volume: 4 Issue: 1 ISSN:

Arrhythmic cardiomyopathy    

Dr. Belhamerche*

Corresponding Author: Dr. Belhamerche, GHEF Marne la vallée.

Copy Right: © 2022 Dr. Belhamerche. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received Date: December 27, 2021

Published Date: January 04, 2022

Arrhythmic cardiomyopathy


We have known for decades that rhythm disturbances can induce dilation and alteration of ventricular function. Arrhythmic cardiomyopathy (ARCM) is an exclusionary diagnosis and retrospective.

This means that a patient who is taking care of heart failure, with ventricular dysfunction must have a certain number of examinations to rule out a cause other than the rhythmic disorder. The diagnosis is most often done retrospectively.

We must eliminate ischemic heart disease with the performance of coronary angiography. From valvular heart disease by echocardiography. And MRI to rule out another heart disease. The treatment of the arrhythmia should lead to clinical improvement and parameters of the ventricular function.

will see, the definition, the pathophysiology, the prevalence, the etiology, and the management of this pathology.

Abbreviations And Acronyms:

ARCM: Arrhythmic cardiomyopathy, AF atrial fibrillation, AT atrial tachycardia, AVNRT: atria ventricular nodal reentry tachycardia, PJRT: paroxysmal junctional reentry tachycardia, WPW wolf Parkinson white,  VT:  ventricular tachycardia, VF ventricular fibrillation, PVC premature ventricular contraction, EF: Ejection fraction, LVEF Left  ventricular  ejection  fraction, LBB : Left bundle brunch

Definition: (1,2,3,4,6,20)

ARCM is a form of dilated cardiopathy with ventricular dysfunction induced by arrhythmias. They are classified in 2 forms. The pure form which corresponds to the absence of underlying heart disease, and the impure form which occurs on a preexisting heart disease and will worsen It is characterized by the reversible aspect after the treatment of the arrhythmia which is why the diagnosis is retrospective. Some authors have proposed this definition:

(Arrhythmic cardiomyopathy results from atrial and/or ventricular dysfunction—secondary to rapid and/or asynchronous/irregular myocardial contraction, partially or completely reversed after-treatment of the causative arrhythmia).


Pathophysiological mechanisms: (2,3,26,27,28,53,56)

We have known from Whipple’s work, that rapid stimulation of the dog’s heart causes dilation and alteration of left ventricular function as well as a cascade of neuroendocrine and metabolic modifications.

Alteration of sympathetic regulation due to adrenergic hyperactivity leads to down-regulation of beta receptors which will induce a decrease in adenylate cyclase and associated cyclic AMP production.

A decrease in energy reserves is observed with a rate of creatine and ATP levels as well as the decrease in the Na / K ATPase pump are linked to an alteration in cell metabolism linked to hyperactivity of the Krebs cycle and changes in mitochondrial function by a reduction in cytochromes oxidases. Electrophysiological alterations accompany a decrease and dysfunction of type L calcium channels to a reduction in the concentration of the Gs protein.

Alongside this cellular dysfunction, and alteration of the extracellular matrix is observed.

Leading to oxidative stress responsible for a decrease in the production of Proxynitrite results in cavitary dilation.


Tachycardia-induced Cardiomyopathy. (26,27,28,29)

Changes in cellular level in T-CMP. (B and C) Echocardiographic and gross change in pacing-induced HF animal model. (A) Following sustained tachycardia, intracellular and extracellular remodeling leads to LV remodeling and worsening contractility. Decrease in L-type Ca2+ channel causes abnormal excitation-contraction coupling. Myocardial fibrosis persists even after recovery of LV function.


Epidemiology:  (6,9,11,12,14,30,46,48)

The incidence and prevalence of ARCM is uncertain. AF is present in 10 to 50% of patients with heart failure, most of them have worsening symp- toms that require rate control. In cases of focal atrial tachycardia, the incidence is in the order of 8 to 10% and 28% in children.

In the event of PVC, the ARCM is present from 8% (ie over 10,000 PVC / 24) , can reach 34% in some series.


Etiologies of arhythmic cardiomyopathy (ARCM) (30,46,48,65,66,)


 Atrial fibrillation (AF):15,16,17,18,19,20,21,22,23,24,26,44,45,46, 68

The heart rate plays a central role as shown by the AFFIRM (60) study (even if there are re- serves) but the irregularity of the rhythm, the loss of atrioventricular asynchrony even intra- ventricular due to the appearance of a complete functional left bundle branch block leads to asyn- chronous contact which alters the ventricular function.

- CAMARA Study (62,64): The goal of this study was to determine whether catheter ablation (CA) for AF could improve LVSD compared with medical rate control (MRC) where the etiology of the LVSD was unexplained.

301 patients with LVEF <45% with persistent AF are divided into 2 groups after having opted for medical treatment, in particular an average HR of 73b / mi. One medical treatment group and medical treatment and radio-frequency ablation group. A cardiac MRI is performed at 6 months to reassess LVEF.


Castel-AF study (44,45,46)

This study focused on mortality and morbidity in patients with heart failure with LVEF <35% enrolled in 2 groups medical treatment group the other medical treatment and ablation by radiofrequency.

The results showed a decrease in the primary endpoint (mortality and hospitalization for heart failure) with an HR of 0,62.

A decrease in hospitalizations with an HR of 0.56 and cardiac mortality with an HR of 0.53.

The studies on rate control versus rhythm control in AF had not shown inferiority on a hard primary endpoint which is global mortality. The AFFIRM (62) and RACE (63) trials were two of them.

The AATAC study even showed that the frequency control by ablation of the NAV associated with the implantation of a PM or ICD CRT was superior to the medical treatment which was Amiodarone to  slow down and maintain the SR.



Kaplan–Meier curve comparing AF-free survival between patients undergoing catheter ablation (group 1) and those receiving Amiodarone therapy (group 2). At end of the study, 71 (70%; 95% CI, 60%–78%) patients in group 1 were recurrence free in comparison with 34 (34%; 95% CI, 25%–44%) in group 2 (log-rank P<0.001). AF indicates atrial fibrillation; AT, atrial tachycardia; and CI, confidence interval.

PABA- CHF (57,58,59,60,61,64) study showed the superiority of AF ablation over atrioventricular node ablation with PM implantation showing that it is not only the rate control that improves this form of heart disease. The rhythm control is better.

In total, in AF, medical treatment to rate control and restore the sinus rhythm is necessary but it must be combined with the AF ablation because the results are better both in terms of mortality, hospitalization and the quality of the patient life. The ablation of the NAV is reserved for the elderly or patients with very dilated atria. Moreover, the new recommendations of the ESC and AHA place the ablation of AF in the first intention in this heart disease with a level Ia.

Others Supraventricular arrhythmia: (31,32,33,34,35,36)

(A Flutter, AT, AVNRT, PJRT, RAP, WPW).

For supraventricular arrhythmia, catheter ablation is recommended by the ESC / AHA in the first intention with a level of evidence from IA or IB.

Because the success rate of the ablation is of the order of 90%. Particularly in junctional arrhythmias such as PJRT which can induce multiviscérale failure tables due to the multi-current and incessant nature in children.


 PVCs and VT (9,10,11,13,14,28,53,56 68)

VT and PVCs can induce ARCM, often they come from the hunting chamber of the RV but sometimes from other locations. The mechanisms that induce this heart disease are unclear. In animal models, stopping the pacing results in complete recovery in 2 to 4 weeks of ventricular function. Tissue scans did not show fibrosis, inflammation or apoptosis.

This suggests an intraventricular desynchrony especially if the PVCs have an aspect of LBB, abnormal Ca2+ handling from the short coupling intervals, and abnormal ventricular filling from the post-PVC pause.

Many retrospective studies have investigated associated factors that may favor the onset of ARMC.   Age, male sex, increased body mass index PVCs asymptomatic PVCs, higher PVC coupling interval dispersion, and presence of retrograde P waves. 38 %

A high PVC burden has been variably defined as ranging from >10,000 to 25,000 PVCs/day and as

>10% to 24% of total heartbeats/day. The threshold would be 10000 PVCs per day.


Treatment involves reducing the number of PVCs either through medical treatment and or catheter ablation.

For medical treatment, beta-blockers are in the first line in this context, along with Amiodarone, other anti-arrhythmic treatments are not recommended because of the pro-arrhythmogenic risk. Catheter ablation gives excellent results, especially in the case of right infundibular PVCs with success rates of around 70 to 90%. In the new recommendations, catheter ablation is a class I indication if they induce ARMC or Ila if they worsen heart disease. The question is there an indication for implantation of an ICD? I will say yes if the patient comes for a VT or VF and we don’t know if it is an ARMC. In other patients who have recovered ventricular function, there is no indication but there are a few reported clinical cases of sudden death.

RV pacing (38,59,61,64,68)

In the event of heart disease induced by RV pacing, resynchronization is indicated and makes it possible to correct the ventricular function and improve the prognosis. The recommendation level is Ia if the LVEF is <35% and Ila if it is between 35 and 50%.


Management (10,12,13,14,15,31,32, 68)


ARMC disease is a real entity whose diagnosis is made retrospectively after having eliminated the other causes and obtained an improvement in ventricular function. The medical treatment combines with catheter ablation must be associated, for better results, whether in pure or impure forms.

The message is:(ablation, must be associated, for better results, whether in pure or impure forms).



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