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Curr Pain Headache Rep (2015) 19:14

DOI 10.1007/s11916-015-0481-4

UNCOMMON AND/OR UNUSUAL HEADACHES AND SYNDROMES (J AILANI, SECTION EDITOR)

Cardiac Cephalgia
Yasar Torres-Yaghi & Justin Salerian & Carrie Dougherty

# Springer Science+Business Media New York 2015

Abstract Cardiac cephalgia is a type of secondary headache disorder, usually initiated by exertion that is related to
myocardial ischemia. Primary exertional headaches such as
sex-, cough-, or exercise-induced headaches are typically benign. Cardiac cephalgia, on the other hand, can have lifethreatening complications. Due to overlapping features and
similarities in presentation, cardiac cephalgia can be
misdiagnosed as a primary headache disorder such as migraine. However, the management of these conditions is
unique, and treatment of cardiac cephalgia with vasoconstrictors intended for migraine can potentially worsen myocardial
ischemia. Thus, it is important to make the correct diagnosis
by evaluating cardiac function with an electrocardiogram and/
or stress testing. In this review, we examine reported cases of
cardiac cephalgia from the past 5 years to highlight the importance of this condition in the differential diagnosis of a headache in a patient with a history of cardiovascular risk factors,
as well as to discuss the appropriate approach to diagnosis and
the proposed pathogenic mechanisms of this condition.
Keywords Cardiac cephalgia . Cardiac cephalalgia .
Migraine . Migrainous thoracalgia

been many cases of headache associated with acute coronary


syndromes described in the literature. Cases of cardiac
cephalgia show complete resolution following treatment of
the myocardial ischemia. Postulated mechanisms to explain
this phenomenon include convergence of nerve fibers within
the spinal cord, increased intracranial pressure secondary to
decreased venous return from the brain, and increased inflammatory mediators causing vasodilation. The purpose of this
review is to summarize the key similarities and conclusions
from such cases with emphasis on those published since the
last similar review by Bini et al. in 2009 [2].

Materials and Methods


We performed searches in the Ovid and PubMed databases
using the terms cardiac cephalgia, cardiac cephalalgia,
headache and angina, headache and acute coronary syndrome, and headache and myocardial infarction. The following limitations were used: full text, English language only,
and published after 2009. We selected articles pertinent to our
topic and included seven reports in our review.

Introduction

Clinical Features

The term cardiac cephalgia was introduced in 1997 by Lipton


et al. to describe the phenomenon of a secondary headache
occurring in the setting of cardiac ischemia [1]. There have

Cardiac cephalgia is classified as a secondary headache syndrome. According to the International Classification of Headache Disorders (ICDH-II) diagnostic criteria of 2004, it is
characterized as a headache that is aggravated by exertion,
accompanied by nausea, and develops in the setting of acute
myocardial ischemia [3]. It also, by definition, resolves without recurrence after effective medical or surgical therapy for
the myocardial ischemia. Diagnostic tools are used to evaluate
myocardial ischemia when cardiac cephalgia is suspected. A
neurologic workup is routinely performed for the headache

This article is part of the Topical Collection on Uncommon and/or


Unusual Headaches and Syndromes
Y. Torres-Yaghi : J. Salerian : C. Dougherty (*)
Department of Neurology, MedStar Georgetown University
Hospital, 3800 Reservoir Road NW, 7 PHC,
Washington, DC 20007, USA
e-mail: carrie.o.dougherty@guh.georgetown.edu

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Curr Pain Headache Rep (2015) 19:14

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and is supplemented with a cardiac workup that can include an


electrocardiogram (EKG), cardiac enzymes, nuclear or treadmill stress testing, and coronary angiography.
Lipton et al. were the first to suggest the term cardiac
cephalgia to describe this condition in 1997 [1]. Prior to this,
it may have been believed that such headaches characteristically were pathogenically similar to benign exertional headache, benign cough headache, and headache associated with
sexual activity, all of which rarely have serious sequelae. Lipton et al. observed in their case series that the headache of
cardiac cephalgia was relieved by treatments for acute coronary syndrome, such as the administration of nitroglycerine
and/or surgical interventions such as coronary artery bypass
grafting or percutaneous angioplasty. Thus, they deemed it a
rare type of exertional headache that if left undiagnosed may
have life-threatening implications.
Cardiac cephalgia can vary widely in presentation and often can have features and inciting events (i.e., exertion) that
overlap with other headache disorders, both primary and secondary. As illustrated in the recent literature, the clinical presentation of cardiac cephalgia can vary in location, timing, and
associated symptoms, as well as the presence of cardiac risk
factors (Table 1).
With regard to location, cardiac cephalgia can be bifrontal,
bitemporal, or occipital and onset can be acute, subacute, or
intermittent over time. Duration can range from minutes to
hours, and severity can range from mild to severe. As expected, all hospitalized cases examined in our review had a headache intensity described as severe. Mathew et al. describe a
patient with cardiac cephalgia who had intermittent headaches
that were triggered by exertion and activities such as walking
five blocks, tossing hay bales, and sexual activity. Depending
on the degree of activity, headache intensity ranged from 1 to
6 out of 10 and lasted a few minutes [4]. In contrast, other
reported cases describe acute headaches that persist with severe pain for several hours to days, prompting patients to seek
urgent medical attention [5, 6, 7, 11, 12].
Although Liptons definition of cardiac cephalgia as well
as that of the ICHD-II involves an exertional trigger, a 2009
review of the literature notes that 33 % percent of the cases
included exhibited headaches where exertional activity was
not, in fact, always an inciting factor, further reinforcing the
variable nature of cardiac cephalgias [2]. However, cases reported since have mostly described headaches triggered by
exertion [4, 5, 6, 7, 10].
Given the relation of cardiac cephalgia to myocardial ischemia, one might expect patients to have concurrent cardiac
symptoms such as chest pain. However, although chest pain is
present in many cases [7, 8], there have been reports of cardiac cephalgia occurring as the sole symptom of the myocardial ischemia [4, 6, 9, 10, 1217]. One case describes an 86year-old man with a history of cardiac risk factors who presented with a severe occipital headache, and despite the

absence of chest pain, dyspnea, or other symptoms of angina


was found to have an acute myocardial infarction [6].
Cardiac risk factors such as advanced age, hypertension,
hyperlipidemia, diabetes, smoking, and family history of heart
disease are often present in cases of cardiac cephalgia [9, 10,
14, 18]. However, myocardial ischemia and resultant cardiac
cephalgia have been reported to occur in the absence of concurrent cardiac risk factors, in settings where suspicion for
acute coronary syndromes is low [7, 8]. Three cases reported
in the past 5 years describe patients in their fourth decade of life
with no cardiac comorbidities that presented with headaches
and were found to have acute coronary events [4, 7, 8].

Diagnosis
Neurologic workup for refractory or unusual headache presentation often includes brain imaging and/or lumbar puncture.
When such studies are negative, or in cases where cardiac
origin of the headache is suspected secondary to presence of
cardiac risk factors or concurrent cardiac symptoms such as
chest pain, a cardiac workup is also warranted.
Laboratory studies, EKG, echocardiogram, stress testing,
and coronary angiography are the most frequently used tools
to evaluate myocardial function. Youngsoon et al. report a
case of a patient with a severe headache whose workup revealed normal results for routine laboratory tests and neurologic workup, including computed tomography of the head,
magnetic resonance imaging of brain and cerebral arteries,
electroencephalogram, and cerebral spinal fluid studies. The
presence of chest pain in this case prompted cardiac testing,
and after an EKG was found to be normal, a stress test revealed ST segment depression and the diagnosis of cardiac
cephalgia was made [8].
Although cardiac stress testing is a valuable tool that often
leads to the diagnosis of myocardial ischemia [8, 15], in the
setting of an acute coronary syndrome, there is often not
enough time to perform stress testing. EKG testing is rapid
and useful but can result in a normal initial reading despite the
presence of myocardial ischemia that is later revealed upon
further testing [7, 8, 15, 19].
Coronary angiography is important for the diagnosis of
an acute coronary event and is often performed as both a
diagnostic and therapeutic measure if EKG or stress testing demonstrates evidence of myocardial ischemia. A case
of ongoing intermittent headaches presented with a severe
headache and chest pain was found to be secondary to
variant angina after an angiogram with acetylcholine
provocation revealed coronary vasospasm. During the angiogram, the vasospasm directly induced both chest pain
and headache. Symptoms resolved completely after administration of intra-arterial nitroglycerine and subsequent
vasodilation [8].

No
No

Severe
Severe

Frontal and
bitemporal

Bi-frontal
Occipital
Frontal, radiating Severe
to jaw,
neck, back

Yiannis
42/M 4 h
et al. [7].

Yang
44/F 10 month prior
et al. [8].
Costopoulos 55/M 6 weeks
et al. [9].
Sendovski 61/F 3 weeks
et al. [10].

Yes

Variable

No

Exertion L

Positive

Stress test

Normal

ST depression
laterally,
ST elevation

Left anterior
descending
artery occlusion

Triple vessel
disease

Left anterior
descending
artery stenosis

Left anterior
descending
artery stenosis

Coronary
angiogram

Outcome Discharge
status

Alive
Resolved Alive

Resolved Alive

Resolved Alive

Percutaneous
Resolved Alive
coronary
angioplasty,
stenting
Percutaneous
Resolved Alive
coronary
angioplasty,
stenting
Coronary artery *
Alive
bypass graft

Therapy

Percutaneous
coronary
angioplasty,
stenting
ST depression Coronary
Intra-arterial
artery spasm
nitroglycerine
*
Triple vessel disease Coronary artery
bypass graft
*
Triple vessel disease Coronary artery
bypass graft

ST depression
*
anteriorly,
and ST elevation
posteriorly
Initially normal,
*
repeat Q waves,
and ST elevation

Q wave

Exertion D, H, L, S T wave inversions

Exertion None

Exertion None

EKG
findings

Risk factors for coronary artery disease are listed and include H hypertension, L hyperlipidemia, D diabetes, and S smoking

This table lists the clinical features seen in cases of headache and cardiac ischemia from articles published since 2009. Diagnostic tests, therapeutic interventions, and outcomes are described for each case.
An asterisk (*) indicates that the data was not included in the publication

Severe

Severe

Occipital

Dimitros
86/M New onset
et al. [6].

Risk
Factors

Exertion *

Trigger

Right sided, *
pleuritic

Severe

No

Intensity Chest
pain

Elgharably 55/M New onset 12 h Frontal


et al. [5].

Site

Severe

Age/ HA onset,
sex history

Clinical features of cardiac cephalgia

Mathew
47/M HAs 7 years,
Occipital
et al. [4].
diag. migraine

Reference

Table 1

Curr Pain Headache Rep (2015) 19:14


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All reviewed cases of cardiac cephalgia describe resolution


of headache after reinstating flow in the cardiac vessels by
medical or surgical interventions, further establishing the diagnosis by virtue of response to management.

Differential Diagnosis
The differential diagnosis of cardiac cephalgia remains broad.
It includes the primary headache syndromes such as migraine
and tension headache, as well as other secondary headache
syndromes such as exertional headache.
Factors that aid in diagnosis include older age, concurrent
medical conditions, presence of cardiac risk factors, occurrence of chest pain in the patient, and the absence of typical
features associated with other headache syndromes (such as
migraine aura). As described above, however, cardiac
cephalgia can occur without these risk factors, and diagnostic
studies to evaluate cardiac function are helpful in diagnosis.
Clinically, it is a difficult task to distinguish between cardiac
cephalgia and migraine specifically. Often, the diagnosis of cardiac cephalgia is missed, as the only definitive measure of diagnosing this condition is response to treatment of cardiac ischemia. Mathew et al. describe a patient who carried the diagnosis
of migraine for several years before experiencing an acute myocardial infarction. The patient experienced complete resolution
of his headaches after coronary angioplasty, which revealed that
the etiology of his headaches was cardiac cephalgia.
It is also important to remember that since both primary
headache syndromes and coronary artery disease are common,
Fig. 1 Pathways of referred pain
in coronary ischemia: This figure
illustrates various somatic
afferents that have potential
convergence with visceral
afferents in the spinal cord [5].
Reprinted with permission.
Elgharably Y, Iliescu C, Sdringola
S, Yusuf S. Headache: A
Symptom of Acute Myocardial
Infarction. European Journal of
Cardiovascular Medicine. 2012;
11 (111): 170174

Curr Pain Headache Rep (2015) 19:14

many patients can carry these diagnoses independently of one


another. One study reported that 34 % of a population of
patients with coronary artery disease had a history of primary
headache [20], making cardiac cephalgia even more difficult
to distinguish.
Another entity described in the literature, which may further confound the diagnosis of cardiac cephalgia, is migrainous thoracalgia [4]. As described by Mathew, this is a migraine accompanied by an aura of chest pain and arm paresthesias, mimicking the symptoms experienced during an acute
myocardial infarction. This aura, like many migraine auras,
can occur in the absence of the headache, potentially proposing a diagnostic challenge to physicians. In the case described,
the patient was treated with topiramate and this led to resolution of the chest pain.
The management of migraine often includes the use of
vasoconstrictive medications, which are contraindicated in
the presence of cardiovascular disease. On the other hand,
the management of cardiac ischemia and cardiac cephalgia
often includes the use of vasodilators, which can exacerbate
headaches in patients that suffer from migraines. Therefore, a
thorough history and workup should be undertaken to try to
avoid misdiagnosis [2].

Pathophysiology
Although the pathogenesis behind cardiac cephalgia remains
unknown, there are three proposed mechanisms for cephalgia
in the setting of heart disease. The first possible etiology

Curr Pain Headache Rep (2015) 19:14

involves potential convergence in the spinal cord of visceral


afferent nerves from the heart with somatic afferent nerves
from the head (Fig. 1). This is analogous to the wellrecognized referred left arm and jaw pain that can occur during cardiac ischemia, whose proposed mechanism is similar.
The term headache angina proposed by Blacky et al. in
1987 to describe this diagnosis prior to the accepted current
terminology (cardiac cephalgia) implies this possible etiology
of referred pain [17]. The second proposed etiology is that the
headaches are the result of a reduction of cardiac output during
cardiac ischemia, leading to decreased venous return from the
brain to the heart, resulting in increased intracranial pressure.
A third possible etiology is the effect of increased inflammatory markers such as bradykinin, serotonin, and histamine that
are released in higher quantities during cardiac ischemia, causing vascular changes in the brain and leading to headaches [5,
8, 16, 19, 21].

Conclusions
In addition to clinical reasoning, various diagnostic tools are
used in the diagnosis of cardiac cephalgia. Given the heterogeneity of presentation, diagnosis is difficult. Diagnostic tests
employed by neurologists may fail to elucidate positive findings. Neurologists, widely responsible for the workup and
evaluation of patients with headaches, may need to include
diagnostic studies for cardiac conditions in their armamentarium for headache disorders.
Compliance with Ethics Guidelines
Conflict of Interest Dr. Yasar Torres-Yaghi, Dr. Justin Salerian, and Dr.
Carrie Dougherty each declare no potential conflicts of interest.
Human and Animal Rights and Informed Consent This article does
not contain any studies with human or animal subjects performed by any
of the authors.

References
Papers of particular interest, published recently, have been
highlighted as:
Of importance
Of major importance
1.

Lipton RB, Lowenkopf T, Bajwa ZH, et al. Cardiac cephalgia: a


treatable form of exertional headache. Neurology. 1997;49:8136.

Page 5 of 5 14
2.

Bini A, Evangelista A, Castellini P, et al. Cardiac cephalgia. J


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