Elsevier

Cardiovascular Pathology

Volume 22, Issue 5, September–October 2013, Pages 389-400
Cardiovascular Pathology

Original Article
Cardiomyopathy in neurological disorders

https://doi.org/10.1016/j.carpath.2012.12.008Get rights and content

Abstract

According to the American Heart Association, cardiomyopathies are classified as primary (solely or predominantly confined to heart muscle), secondary (those showing pathological myocardial involvement as part of a neuromuscular disorder) and those in which cardiomyopathy is the first/predominant manifestation of a neuromuscular disorder. Cardiomyopathies may be further classified as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, or unclassified cardiomyopathy (noncompaction, Takotsubo-cardiomyopathy). This review focuses on secondary cardiomyopathies and those in which cardiomyopathy is the predominant manifestation of a myopathy. Any of them may cause neurological disease, and any of them may be a manifestation of a neurological disorder. Neurological disease most frequently caused by cardiomyopathies is ischemic stroke, followed by transitory ischemic attack, syncope, or vertigo. Neurological disease, which most frequently manifests with cardiomyopathies are the neuromuscular disorders. Most commonly associated with cardiomyopathies are muscular dystrophies, myofibrillar myopathies, congenital myopathies and metabolic myopathies. Management of neurological disease caused by cardiomyopathies is not at variance from the same neurological disorders due to other causes. Management of secondary cardiomyopathies is not different from that of cardiomyopathies due to other causes either. Patients with neuromuscular disorders require early cardiologic investigations and close follow-ups, patients with cardiomyopathies require neurological investigation and avoidance of muscle toxic medication if a neuromuscular disorder is diagnosed. Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations.

Introduction

Cardiomyopathies (CMPs) are generally characterised by abnormal structure, dimension, or function of the left or right ventricular myocardium in the absence of arterial hypertension, coronary heart disease, valve abnormalities, or congenital heart disease [1]. CMPs may be hereditary or acquired and either confined to the heart or may represent cardiac involvement in systemic disease. Diagnosis of CMP relies on clinical, electrocardiographic, echocardiographic, coronary angiographic, or genetic findings. Complications of CMPs may include neurological disease and neurological disease may be accompanied by CMP. This review aims at summarising and discussing recent findings concerning the neurological implications in CMPs.

Section snippets

Classification of CMPs

There is no binding classification of CMPs but the frequently used classification of the American Heart Association classifies CMPs as primary (solely or predominantly confined to heart muscle), secondary [those showing pathological myocardial involvement as part of a neuromuscular disorder (NMD)] and those in which cardiomyopathy is the first/predominant manifestation of a NMD [2]. The European Society of Cardiology, on the contrary, differentiates according to the etiology between primary

Neurological disease and CMPs

CMPs may be related to neurological disease twofold. CMPs may either secondarily cause neurological disease or may represent the cardiac manifestation of an exclusively or predominantly neurological disease.

Definition and diagnostic criteria

HCM is present if there is left ventricular myocardial thickening >15 mm, with or without normal systolic function, in the absence of any other disease producing wall thickening (Fig. 1). HCM may be non-obstructive or obstructive (Table 2). Hypertrophy of the left ventricular myocardium may be symmetric or asymmetric. Asymmetric myocardial thickening frequently affects the interventricular septum, the mid-ventricular myocardium (MVHC) or the outflow tract. If myocardial thickening predominantly

Definition and diagnostic criteria

DCM is diagnosed if there is dilation of the left ventricle (LVEDD >57 mm), and systolic dysfunction (FS <25%) in the absence of abnormal loading conditions or coronary artery disease sufficient to cause global systolic impairment (Fig. 2). In some patients DCM may be accompanied by myocardial thickening.

Clinical presentation and treatment

Patients with DCM frequently develop heart failure or arrhythmias. Pharmacotherapy consists of medication with ACE-inhibitors, beta-blockers, or diuretics. In cases with systolic dysfunction (EF

Definition and diagnostic criteria

RCM is a rare form of CMP and diagnosed echocardiographically if there is dilation of the right and left atrium, a restrictive filling pattern (deceleration time <150 ms on transmitral flow), presence of normal or reduced diastolic and systolic ventricular volumes, normal LV wall thickness, and normal systolic function. RCM is either idiopathic or a manifestation of cardiac involvement in systemic disease, predominantly NMDs (Table 5) [121].

Clinical presentation and treatment

Patients with RCM may be asymptomatic but more

Definition and diagnostic criteria

ARVC, also known as arrhythmogenic right ventricular dysplasia or arrhythmogenic right ventricular dysplasia/CMP, is an inherited or acquired, non-ischemic CMP, which primarily involves the right ventricle affecting children and young adults. It is now well recognized that also the left ventricle may be affected. ARVC is characterized by structural abnormalities of the right ventricular myocardium, hypokinetic areas involving the free wall of the right ventricle, and progressive fibrofatty

Definition and diagnostic criteria

LVHT is characterised by hypertrabeculation of the left ventricular myocardium apically to the papillary muscles. The cause of LVHT is unknown but it is frequently associated with genetic disease and chromosomal abnormalities. LVHT is preferentially diagnosed on echocardiography. Several echocardiographic definitions have been proposed but none turned out to be binding so far. According to one frequently applied definition LVHT is present on echocardiography if there are more than three

Definition and diagnostic criteria

Takotsubo-CMP is defined as reversible left ventricular systolic dysfunction in the absence of coronary heart disease, triggered by stress [186]. It is echocardiographically characterized by left-ventricular ballooning, wall motion abnormalities, and severely reduced systolic function. Electrocardiogram (ECG) and clinical presentation mimic acute coronary syndrome or myocardial infarction. Blood tests may reveal cardiac enzyme-release. Typically, echocardiographic and ECG abnormalities resolve

Clinical implications

The presented data imply a number of clinical consequences. First, the risk of neurological disease from CMP is poorly investigated and requires a multicenter approach to assess if the stroke rate is truly increased in patients with CMP, which patients with CMP carry an increased stroke risk, or which are the optimal strategies to prevent stroke from CMP. Second, patients with primary and secondary NMD need to be obligatorily investigated for cardiac disease, including CMP. Cardiac

Conclusions

Patients with CMPs require special attention by the cardiologist and neurologist. CMPs may cause central nervous system disease, which can be prevented if adequate measures are taken in time. Additionally, neurological disease in particular NMDs may manifest with CMPs, why all these patients should be seen by the cardiologist as soon as the neurological diagnosis is established. Patients with cardiac involvement from a NMD require regular cardiac follow-ups not to miss the point when essential

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