Epileptic Heart

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Synonyms and keywords:

Overview

Chronic epileptic episodes and the subsequent catecholamine surges and hypoxic events may affect the heart and coronary vessels and result in the dysfunction of the heart. This condition is known as the "epileptic heart." This concept was first described by Dr. Richard L. Verrier and his colleagues in 2020.

Historical Perspective

• Absence of cardiac activity during epileptic seizure, first described by Dr. A.E. Russell, an English physician, in 1906.
• The epileptic heart was first described by Drs. Verrier, Pang, Nearing, and Schachter, in 2020.

Classification

• There is no established system for the classification of the epileptic heart.

Pathophysiology

• The exact mechanisms involved in the development of the epileptic heart are still being elucidated. However, the conceptual framework below provides helpful information on the development of heart disease in patients with epilepsy.

Chronic epilepsy

Repeated hypoxia and subsequent myocardial ischemia

Accelerated atherosclerosis

Myocardial stunning

Vacuolization of myocytes and fibrosis

Catecholamine-induced cardiotoxicity

Epileptic Heart ❑ Cardiac electrical instability ❑ T wave alternans

Epilepsy and Cardiac Arrhythmia

Cardiac arrhythmias have long been observed in patients with epilepsy. Three different mechanisms explain this association:

• Direct (causal) pathway
• Co-existence of epilepsy and cardiac arrhythmia in the context of genetic disorders:
  • Genes involved in epilepsy: SCN1A, KCNA1, and SCN8A
  • Genes involved in heart diseases: KCNQ1, KCNH2, SCN5A, RYR2
• Resultant association:
  • Certain anti-epileptic medications with sodium channel blocking properties have been known to cause arrhythmia.
  • Seizure may also cause arrhythmia.
    • Ictal phase may be associated with tachycardia, asystole, bradycardia, and AV block.
    • Postictal phase may be associated with asystole, AV block, atrial flutter or fibrillation, and ventricular fibrillation.

Epilepsy and Structural Heart Disease

Three mechanisms have been suggested to explain the association between epilepsy and structural heart disease:

• Direct (causal) pathway in which structural heart disease may result in embolic stroke and subsequent epilepsy.
• Common risk factors contribute to the development of both epilepsy and structural heart disease.
• Resultant pathway:
  • Epilepsy may stimulate myocardial ischemia and a Takotsubo-like syndrome.
  • Certain anti-epileptic medications contribute to the development of arteriosclerosis, weight gain, non-alcoholic fatty liver disease and metabolic syndrome and result in a poorer cardiovascular risk profile.
• On microscopic histopathological analysis of patients with epilepsy a range of pathologies has been reported. These changes include fibrosis, myofibrillar degeneration, ventricular hypertrophy, focal myocardial fibrosis, perivascular and interstitial myocardial fibrosis, and mild to moderate coronary atherosclerosis.

Causes

• Epileptic heart is caused by the chronic effects of epilepsy on the heart.

Causes

Disease name] may be caused by [cause1], [cause2], or [cause3].

OR

Common causes of [disease] include [cause1], [cause2], and [cause3].

OR

The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].

OR

The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [disease name].

• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

• [Disease name] affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

History and Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

Differentiating Epileptic heart from other Diseases

• Epileptic heart must be differentiated from other causes of sudden death. It should also be distinguished from sudden unexpected death in epilepsy.

Screening

Currently, there is no guideline statement that recommends routine cardiac evaluation of patients with epilepsy. However, a resting 12-lead EKG and/or ambulatory EKG patch recording may be useful in identifying the patients at risk of cardiac pathology and to further follow the progression of their cardiac pathology.