Journal of the Practice of Cardiovascular Sciences

: 2019  |  Volume : 5  |  Issue : 1  |  Page : 64--65

Atrial cardiomyopathy: A new and novel concept

Rakesh Agarwal 
 Department of Cardiology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Correspondence Address:
Dr. Rakesh Agarwal
243, G.T Road (N), Laxmi Niketan, Flat-2E, Liluah, Howrah - 711 204, West Bengal

How to cite this article:
Agarwal R. Atrial cardiomyopathy: A new and novel concept.J Pract Cardiovasc Sci 2019;5:64-65

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Agarwal R. Atrial cardiomyopathy: A new and novel concept. J Pract Cardiovasc Sci [serial online] 2019 [cited 2019 Dec 11 ];5:64-65
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Dear Editor,

The term “cardiomyopathy” was first introduced by Brigden in 1957. He proposed it “to indicate isolated noncoronary myocardial disease.” Since then the term has been used to refer to any cardiac muscle disease, without specification of the chamber involved, though most commonly implying the ventricles.[1]

Recently, atrial cardiomyopathy as an entity has been much in the discussion, both as a subject of concern and controversy. Sekiguchi et. al. described the case of a 33-year-old male patient with sick sinus syndrome and a history of diphtheria in childhood who underwent the right atrial and ventricular endomyocardial biopsies. The right atrial biopsy revealed more advanced interstitial fibrosis than the right ventricular biopsy. The authors attributed it to be a form of “atrial cardiomyopathy.”[2]

However, the first case of fibrotic atrial cardiomyopathy (FACM) was described as long back as in 1972 in a family with first-degree heart block where three of five siblings progressed to persistent atrial standstill.[3] In 2012, Kottamp in his famous article described FACM as a specific disease with fibrotically destroyed atria that cannot be explained by age or underlying heart disease or a history of atrial fibrillation (AF). It was described as an independently progressing cardiomyopathy with the transition to persistent AF much faster than patients without FACM.[4]

Recently, EHRA/HRS/APHRS/SOLAECE jointly published expert consensus on atrial cardiomyopathies and defined it as: “any complex of structural, architectural, contractile, or electrophysiological changes affecting the atria with the potential to produce clinically relevant manifestations.”[5]

The causes of atria cardiomyopathy are varied and include but are not limited to AF (which may as well result from the cardiomyopathic process), chronic heart failure, myocarditis, valvular heart disease, hypertension, diabetes, and amyloidosis [Table 1]. Atrial cardiomyopathy has been classified by EHRAS into four subsets [Table 2].{Table 1}{Table 2}

However, the classification itself has its own limitations. To put a patient into a class, tissue specimens and histopathology are needed. There is considerable overlap among the EHRAS classes for the different proposed etiologies, for example, valvular heart disease can have features of all four classes. Hypertension, diabetes, and muscular dystrophies would fall into at least three classes. Only atrial amyloidosis probably would fit into a single class (Class IV). Even if a single class was assigned, the data would not be of much help in clinical assessment or management of patients.[6]

A clinically relevant classification would include etiological factors, key cardiomyopathic consequences, genetic and environmental expressions, and prognostic factors.

Clinical recognition of atrial cardiomyopathy can have a potential impact. It might help in the identification of individuals at risk of stroke, independent of AF. The annual stroke risk with the CHA2DS2-VASc score of 9 is around 15% only. Future identification of specific patients at risk of thromboembolism may spare patients from the toxicities of anticoagulation unnecessarily. Specific approaches to treatment, including ablation approach, antiarrhythmic drug therapy, and ancillary therapy for rhythm maintenance can be planned.[6]

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Conflicts of interest

There are no conflicts of interest.


1Richmond C. Wallace William Brigden. BMJ 2008;336:1382.
2Sekiguchi M, Hasegawa A, Hiroe M, Morimoto S, Nishikawa T. Inclusion of electric disturbance type cardiomyopathy in the classification of cardiomyopathy: A current review. J Cardiol 2008;51:81-8.
3Williams DO, Jones EL, Nagle RE, Smith BS. Familial atrial cardiomyopathy with heart block. Q J Med 1972;41:491-508.
4Kottkamp H. Fibrotic atrial cardiomyopathy: A specific disease/syndrome supplying substrates for atrial fibrillation, atrial tachycardia, sinus node disease, AV node disease, and thromboembolic complications. J Cardiovasc Electrophysiol 2012;23:797-9.
5Goette A, Kalman JM, Aguinaga L, Akar J, Cabrera JA, Chen SA, et al. EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: Definition, characterisation, and clinical implication. J Arrhythm 2016;32:247-78.
6Guichard JB, Nattel S. Atrial cardiomyopathy: A useful notion in cardiac disease management or a passing fad? J Am Coll Cardiol 2017;70:756-65.