The Classification Concept of the ESC Working Group on Myocardial and Pericardial Diseases for Dilated Cardiomyopathy

Abstract

In the WHO/ISFC classification of 1996, cardiomyopathies were defined as primary myocardial disorders of unknown cause. Heart muscle disorders of known etiology or associated with systemic disorders were classified as secondary or specific heart muscle diseases. An expert panel of the American Heart Association has recently suggested a new scheme that combines genetic and clinical criteria. In this system, the term primary is used to describe cardiac diseases in which the heart is the sole or predominantly involved organ and secondary to describe diseases in which myocardial dysfunction is part of a systemic disorder. In a radical departure from convention, they also suggested that ion channelopathies and disorders of conduction should be considered cardiomyopathies as well. The ESC Working Group on Myocardial and Pericardial Diseases has taken a different approach based on the belief that a clinically oriented classification system in which heart muscle disorders are grouped according to ventricular morphology and function remains the clinically most useful method for diagnosing and managing patients and families with heart muscle disease. In the ESC position statement, cardiomyopathies are defined as myocardial disorders in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality. In this article, this is illustrated by examples of dilated cardiomyopathy as familial/genetic forms and nonfamilial/nongenetic forms.

Influence of co-existing atrial fibrillation on the efficacy of atorvastatin treatment in patients with dilated cardiomyopathy: a pilot study

“…In a prospective, randomized study 69 patients (men and women aged 18 years or older) with DCM (according to ESC 2007 [5]) with a left ventricular ejection fraction (LVEF) ≤40% as documented by echocardiography were included. Patients had been on stable doses of HF medications for three weeks before enrollment.…”

“…Hypertension, coronary artery disease (CAD), and cardiomyopathy represent the most prevalent underlying pathologies of AF and congestive heart failure (HF), implying a coincidence of both in many patients [2-4]. According to the European Society of Cardiology (ESC) 2007 guidelines [5], dilated cardiomyopathy (DCM) is recognized based on dilation and systolic dysfunction of the left ventricle unless a patient simultaneously suffers from CAD, hypertension, valvular heart disease or congenital heart disease which is so significant that it leads to an observed pathology of the myocardium. Within the last few years, more and more evidence has been presented that autoimmunological processes, cellular as well as humoral ones, are involved in the pathogenesis of DCM [6].…”

Myocardial Infarction as Typical Presentation of Noncompaction Cardiomyopathy

“…Нозологическая принадлежность НКМ остается не вполне понятной: он рассматривается и как само-стоятельный вариант генетически детерминирован-ной КМП, чаще всего по типу дилатационной (ДКМП), (в этом качестве НКМ присутствует, в част-ности, в последних европейской и американской классификациях КМП [4,5]), и как один из феноти-пов разнообразных по своим проявлениям саркомер-ных КМП (особенно в тех случаях, когда НКМ выяв-ляется при наличии рестриктивного или гипертрофи-ческого вариантов КМП, [6]), и даже как вторичный феномен на фоне выраженной систолической дис-функции левого желудочка (ЛЖ) и его дилатации, исчезающий в результате успешного лечения (при этом появление некомпактного слоя объясняют рас-тяжением ЛЖ с улучшением визуализиции на фоне ослабленных сокращений, компенсаторной гипер-трофией трабекул) [7,8].…”

Dilated Cardiomyopathy – a Syndrome, not a Homogeneous Disease

“…Goodwin has contributed. It may also apply to the quantitative definition of inflammatory cardiomyopathy by the World Heart Federation's (WHF) Task Forces in 2000 [2,3] or the first classification of the American Heart Association (AHA) in 2006 [4] and the (counter) position statement of the Working Group on Myocardial and Pericardial Diseases of the European Society of Cardiology (ESC) [5,6]. It is true for recent AHA and ESC guidelines on heart failure (see also issue 9/2006 of Herz) or those on the management of pericardial disease by the ESC Task Force 2005 [7], the Japanese approach to cardiomyopathies (Matsumori in this issue [8]) or recommendations in Herz in 2006 for the management of inflammatory cardiomyopathy [9].…”

“…This approach is futuristic and still far away from the clinical work-up of a patient who comes to consultation with clinical symptoms, to receive an ECG and an echocardiogram, but not a complete family tree or the genetic determination of mutations for HCM, DCM or ARVC. This has also been one of the major objections of the ESC Working Group on Myocardial and Pericardial Diseases [5] and the contribution by Kaski & Elliott in this issue of Herz [6]. The ESC position paper therefore keeps the traditional but well-accepted hemodynamic classification for cardiomyopathies (HCM, DCM, ARVC, unclassified) and separates between familial/genetic and nonfamilial/nongenetic forms.…”