11 year old African American Boy

An 11 year old black boy reported to the emergency ward after 4 days of progressively severe episodes of chest pain.

ACC Vol. 5, No. 3
March 1985:761-4
761
Variant Angina in an 11 Year Old Boy
DAVID WILKES, MD, RICHARD DONNER, MD, FACC, IAIN BLACK, MD, FACC,
BLASE A. CARABELLO, MD, FACC
Philadelphia, Pennsylvania
Variant angina is a syndrome in which ischemic cardiac pain occurs with ST segment elevation. The syndrome is due at least in part to coronary vasospasm. Although well documented in adults, there are no known reports of this syndrome in children. The clinical, electrocardiographic and echocardiographic findings in an 11 year old boy with variant angina are reported.
(J Am Coll Cardiol 1985;5:761-4)
Variant angina is a syndrome in which ischemic chest pain occurs together with transient electrocardiographic ST segment elevation instead of depression (1). The coronary anatomy in patients with this syndrome varies widely from severe proximal fixed obstruction to the absence of discernible lesions. Variant angina usually is unrelated to exercise and often occurs at night. To our knowledge, this syndrome has been described only in adults. We now report a case of variant angina in an 11 year old boy.
Case Report
Clinical features. An 11 year old black boy reported to the emergency ward after 4 days of progressively severe episodes of chest pain. The pain was substernal in location and unrelated to exercise. There was no pleuritic component or radiation of the pain and it was not associated with diaphoresis, nausea or dyspnea. The episodes of chest pain lasted from 30 seconds to several minutes. In the 6 weeks before admission, the patient also noted the onset of frequent bifrontal headaches. The headaches were frequently precipitated by exertion and usually unrelated to the chest pain when they occurred. The headaches lasted 10 to 15 minutes and were usually relieved by rest. They were occasionally associated with blurred vision.
From the Section of Cardiology, Department of Pediatrics, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania and the Section of Cardiology, Department of Medicine, Temple University Health Sciences Center, Philadelphia, Pennsylvania. Manuscript received August 13, 1984; revised manuscript received October 16, 1984, accepted October 31, 1984.
Address for reprints: Blase A. Carabello, MD, Section of Cardiology, Temple University Hospital, 3401 North Broad Street, Philadelphia, Pennsylvania 19140.
©1985 by the American College of Cardiology
The past medical history was significant only for the presence of sickle cell trait. The patient was receiving no medications at the time of his admission. There was no family history of cardiac disease or migraine headache.
On physical examination, the patient was in distress with chest pain and mild dyspnea. Vital signs were pulse 130/min, blood pressure 84/62 mm Hg, respiration 24/min and temperature 37°C. There was no cyanosis. Funduscopic examination was normal. The carotid artery upstrokes were diminished in volume. The neck veins were not distended, the chest was clear and cardiac examination revealed no increased impulse, gallop, murmur or rub. There were no neurologic deficits.
The electrocardiogram demonstrated an anteroseptal injury current with ST segment elevation in leads I, aVR, aVL and V1 to V3 (Fig. 1A). In addition, junctional tachycardia was present at a rate of 128/min. The chest X-ray film was within normal limits.
The hematocrit was 35.6 ml/dl. The white blood cell count was 4,000/mm3. The erythrocyte sedimentation rate was 11 mm/h. Serum electrolytes and urinalysis were normal.
The chest pain subsided spontaneously after approximately 30 minutes. A repeat electrocardiogram was within normal limits (Fig. 1B). An echocardiogram performed during the chest pain (Fig. 2A) demonstrated septal akinesia. There was no mitral valve prolapse.
Hospital course. The chest pain did not recur. Serial cardiac enzyme determinations were within normal limits. A repeat echocardiogram on the sixth hospital day was normal (Fig. 2B). A radionuclide thallium myocardial scan was also within normal limits. Cardiac catheterization demonstrated no abnormalities; specifically, the coronary arteries were normal. Ergonovine was not infused. The patient was discharged, but returned on the same day with a severe headache. A neurology consultant found no neurologic deficit and suggested that the patient's headaches were most consistent with migraine. An electroencephalogram demonstrated prolonged diffuse slowing. The patient's headache resolved.
Follow-up. The patient was discharged while receiving nifedipine, 10 mg orally, three times a day. He continued this therapy for 3 months, but discontinued it when the capsules were all consumed. His angina did not recur and he has been angina-free for 14 months. His headaches have diminished in frequency and severity, occurring approximately once every 2 months.

Figure 1. A, Electrocardiogram during chest pain. The rhythm is junctional tachycardia. There is ST segment elevation in leads I, aVR, aVL and V, to V3, with reciprocal ST depression in leads II, III and aVF. B, The electrocardiogram has returned to normal after the subsidence of chest pain.


Figure 2. Parasternal long-axis views of the phased array echocardiogram at end-systole. A, During chest pain. Arrows point to an area of systolic bulging and thinning of the interventricular septum. B, Five days after admission in the absence of chest pain. Arrows point to the same area of the septum as in A. There now is normal contraction and thickening of the septum. Ao = aorta; LA = left atrium; LV = left ventricle.

 


Discussion
The diagnosis of variant angina in this 11 year old boy was made on the basis of a history of chest pain at rest and the documentation of transient ST segment elevation. The presence of a reversible left ventricular septal wall motion defect on the echocardiogram and normal cardiac enzyme determinations provided further evidence that the child had variant angina (2). To date, we have been unable to find a previous report of variant angina in a child.
Mechanism. It is widely believed that variant angina is due to coronary vasospasm (3-5). Ergonovine administration at the time of coronary arteriography has been employed to reproduce spasm and confirm the diagnosis (6-8). However, we did not attempt to provoke spasm in our patient because recent cautionary reports (9-11) note that complications, while rare, may occur during ergonovine provocation. We believed that we could not adequately assess the risk-benefit ratio of ergonovine administration in our young patient and would have elected to treat him with a calcium channel blocking agent or nitrates, even if the ergonovine test was negative. Thus, some other mechanism for transient transmural ischemia, such as transient thrombotic coronary occlusion, could have been operative. We believe, however, that the patient's migraine headaches and the recently documented association between migraine and vasospastic angina (12) further support vasospasm as a cause of his angina. The interaction of our patient's sickle cell trait with this event can be only speculated on. Although it is unlikely that sickling was the primary cause of the patient's angina, local cardiac ischemia could have precipitated sickling as a secondary event, which may have added to the Magnitude of vascular stasis.
Clinical implications. The clinical significahce of our case is uncertain. It may simply represent an isolated unusual event. However, coronary spasm has been hypothesized as a cause of atherosclerotic coronary disease in some patients (13,14). Marzilli et al. (14) postulated that spasm may be an important factor in the pathogenesis of fixed atherosclerotic lesions. They reported a case of a 60 year old woman with vasospastic angina and anatomically normal coronary arteries who subsequently developed fixed obstructive lesions at the sites of her previous vasospasm. In this context, our case may represent documentation of a risk factor for the premature development of coronary artery disease.