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Year : 2017  |  Volume : 14  |  Issue : 2  |  Page : 119-121

Cardiac cephalgia presenting as acute coronary syndrome: A case report and review of literature

Department of Cardiology, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication26-Oct-2017

Correspondence Address:
Biswajit Majumder
Department of Cardiology, R. G. Kar Medical College and Hospital, 1, Khudiram Bose Sarani, Kolkata - 700 004, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njc.njc_16_17

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Although chest pain is the most common presentation of ischemic heart disease, there are other rare presenting symptoms such as indigestion, diaphoresis, and rarely only with headache. High degree of clinical suspicion is needed in any patient presenting with headache to have ischemic heart disease as an etiology. Hereby, we report an unusual case of acute coronary syndrome in a young patient under the age of 50 presenting with only headache.

Keywords: Acute coronary syndrome, cardiac cephalgia, coronary artery disease, headache

How to cite this article:
Majumder B, Chatterjee PK, Sudeep K N, Ghosh S. Cardiac cephalgia presenting as acute coronary syndrome: A case report and review of literature. Nig J Cardiol 2017;14:119-21

How to cite this URL:
Majumder B, Chatterjee PK, Sudeep K N, Ghosh S. Cardiac cephalgia presenting as acute coronary syndrome: A case report and review of literature. Nig J Cardiol [serial online] 2017 [cited 2023 Jun 10];14:119-21. Available from: https://www.nigjcardiol.org/text.asp?2017/14/2/119/217272

  Introduction Top

Coronary ischemia typically presents with retrosternal pain that radiates to left arm. It may present atypically in various forms such as indigestion, otalgia, facial pain, and syncope and rarely with headache. Acute coronary syndrome presenting only with headache is rare; however, it has been reported in the literature previously. In a study of 150 patients with angina, about 6% were found to have concomitant headache.[1] The term cardiac cephalgia (CC) refers to a form of headache which is the only presentation of coronary artery disease (CAD). The diagnosis depends on the presence of severe headaches worsened by physical exercise or stress and relieved with rest and/or nitrate administration. In this article, we present a patient with acute coronary syndrome, whose main presentation was with headache and reviewed others similar cases published in the literature.

  Case Report Top

A 48-year-old female patient presented to the outpatient clinic with severe headache for the last 7 h. She had a history of exertional severe headache relieved after taking rest for the last 6 months. She was a nonsmoker with a history of diabetes mellitus for the last 6 years. Neurological and cardiological examinations were unremarkable. She was investigated for the headaches by computed tomography scan of the brain, which showed no abnormalities. Suspecting a possibility of CC, an electrocardiogram (ECG) was done which revealed normal sinus rhythm with ST segment depression with T-wave inversion in inferior leads. Troponin t-test was positive. Echocardiography showed no regional wall motion abnormality with normal left ventricular systolic function. Other biochemical parameters were within normal limits. A clinical diagnosis of acute coronary syndrome was made.

Coronary angiography showed significant disease in the mid left anterior descending artery (LAD) [Figure 1], normal left circumflex artery, and cutoff right coronary artery (RCA) from mid-part [Figure 2]. The LAD was successfully treated with a single drug-eluting stent [Figure 3] and RCA with two drug-eluting stents [Figure 4]. Thrombolysis in myocardial infarction 3 flow was achieved in both territories. The patient was put on aspirin, clopidogrel, atorvastatin, β-blocker along with oral antidiabetic medications. She is doing very well over the last 6 months' follow-up with no recurrence of headache.
Figure 1: Coronary angiography showing significant disease in mid left anterior descending coronary artery

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Figure 2: Coronary angiography showing total occlusion of the right coronary artery from mid-part

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Figure 3: Coronary angiography showing final result after successful angioplasty with drug-eluting stent to mid left anterior descending coronary artery

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Figure 4: Coronary angiography showing thrombolysis in myocardial infarction 3 flow in the right coronary artery after successful angioplasty with two drug-eluting stents

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  Discussion Top

The term CC was coined by Lipton et al.[2] in 1997 as a form of exertional headache that was relieved by taking rest. The diagnosis of CC depends on the presence of severe headaches worsened by physical exercise or stress and relieved with rest and/or nitrate administration. The International Headache Society has included CC as a specific entity in its international classification of headache disorders and proposed diagnostic criteria.[3]

  1. Headache, which may be severe, aggravated by exertion and accompanied by nausea and fulfilling criteria 3 and 4
  2. Documentation that acute myocardial ischemia has occurred
  3. Headache develops concomitantly with acute myocardial ischemia
  4. Headache resolves and does not recur after effective medical therapy for acute myocardial ischemia or coronary revascularization.

Bini et al.'s [4] review of 30 cases with CC showed a mean age of 62.4 (range 35–85 years). Pain is usually not localized and may be unilateral or bilateral. Pain is almost always severe and has been described as having different characteristics.

There may or may not be other accompanying symptoms and if there are, 30% may be autonomic in nature. In 27% of the cases, headache is the only manifestation of a cardiovascular ischemia. The headache starts immediately after physical exertion or on stress and disappears gradually after resting. In 33% of cases, headache appeared at rest. The frequency of this headache is highly variable and is concomitant with the acute cardiovascular event. Fifty-seven percent of patients show ECG abnormalities at rest [5] as well as elevated cardiac enzymes,[6] and in the remaining, ECG changes appear only during stress.

It is not surprising that CC does not respond to simple painkillers but promptly responds to nitrates. Triptans and ergot derivatives are contraindicated. In doubtful cases, the only test that can confirm the diagnosis is coronary angiography. If revascularization is done, CC usually disappears and may recur again in the event of coronary artery restenosis.

Four theories have been proposed as a pathophysiologic mechanism. The first theory [1],[7] suggests that CC is a referred pain as there is a connection between the central cardiac pathway (vagus nerve) and the cranial pain afferents (trigeminal nerve) in the upper part of the spinal cord. The second theory [2] proposes that CC is secondary to elevated intracranial pressure due to venous stasis caused by transient decrease in cardiac output due to ischemic ventricular dysfunction. According to the third theory, it may be secondary to the local release in the heart muscle of chemical mediators capable of inducing remote pain in this case headache. Among others, serotonin, bradykinin, histamine, and substance P have been proposed as potential pain-producing substances. The increase in intracardiac pressure during angina attacks could also result in release of natriuretic peptides with consequent vasodilatation of the cerebral vasculature resulting in headache. Finally, CC could be due to the concomitant presence of vasospasm in both coronary and cerebral vascular beds. When the headache occurs as the only manifestation of an acute coronary event, the diagnosis could be difficult; useful clues are older age at onset, no previous history of headaches, and presence of risk factors for cardiovascular disease and the onset of headache during exertion and relived after taking rest. Knowledge of CC is scarce and it is very underrecognized and underreported.

Differential diagnosis is from migraine with or without autonomic symptoms, tension-type headache, primary and secondary forms of exertional headache, thunderclap headache, and exacerbation of headache or migraine attacks by the use of nitroglycerine. It is extremely important to differentiate CC from other noncardiac diseases as the use of triptans or ergot derivatives could be serious. Our case is unique as the patient was younger than most of the cases described to date and presented initially with worsening of stable angina manifested by exertional headache. Also, during admission, headache at rest was the sole manifestation of acute coronary syndrome. Symptoms were relieved by revascularization without any recurrence.

  Conclusion Top

Headache could be an uncommon but important presentation of coronary ischemia. It may be the only manifestation without associated chest pain and response to rest and nitrates aids diagnosis. Useful clues are older age, no previous history of headache, presence of CAD risk factors, and symptoms on exercise or stress. Distinguishing CC from migraine is important, as triptans and ergot derivatives are contraindicated. Awareness of this condition is scarce as it is underrecognized and underreported. Idea of reporting this case is to aware the cardiologists and physicians that headache may be the sole presentation of ischemic heart disease missing which might have an adverse consequence.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Lanza GA, Sciahbasi A, Sestito A, Maseri A. Angina pectoris: A headache. Lancet 2000;356:998.  Back to cited text no. 1
Lipton RB, Lowenkopf T, Bajwa ZH, Leckie RS, Ribeiro S, Newman LC, et al. Cardiac cephalgia: A treatable form of exertional headache. Neurology 1997;49:813-6.  Back to cited text no. 2
Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders: 2nd ed. Cephalalgia 2004; 24 Suppl 1: 9-160.  Back to cited text no. 3
Bini A, Evangelista A, Castellini P, Lambru G, Ferrante T, Manzoni GC, et al. Cardiac cephalgia. J Headache Pain 2009;10:3-9.  Back to cited text no. 4
Seow VK, Chong CF, Wang TL, Ong JR. Severe explosive headache: A sole presentation of acute myocardial infarction in a young man. Am J Emerg Med 2007;25:250-1.  Back to cited text no. 5
Korantzopoulos P, Karanikis P, Pappa E, Dimitroula V, Kountouris E, Siogas K. Acute non-ST-elevation myocardial infarction presented as occipital headache with impaired level of consciousness – A case report. Angiology 2005;56:627-30.  Back to cited text no. 6
Meller ST, Gebhart GF. A critical review of the afferent pathways and the potential chemical mediators involved in cardiac pain. Neuroscience 1992;48:501-24.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
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[Pubmed] | [DOI]
2 Cardiac cephalalgia: a case series of four patients and updated literature review
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