Prinzmetal's variant angina

A 55-year-old man demonstrated typical reflux esophagitis, not esophageal spasm, by esophageal manometry, although he demonstrated classic migraine, positive Raynaud's scan, and proven coronary artery spasm. He suffered from severe chest pain by medication of ergotamine tartrate. Ergot alkaloids should be avoided in patients with symptomatic coronary artery spasm.

Coronary artery spasm may sometimes produce diffuse arterial narrowing[1] and the coincidence of Raynaud's phenomenon and migraine in certain patients with coronary artery spasm[2,3] suggests that coronary artery spasm may sometimes be a manifestation of a generalized vascular smooth muscle hypercontractility syndrome. Also, recently a high prevalence of esophageal motility disorders has been noted in patients with coronary artery spasm or microvascular angina, leading to the hypothesis of a generalized abnormality in smooth muscle function.[4-6] However, to our knowledge, coexistence of reflux esophagitis, migraine, and Raynaud's disease has not been reported variant angina.

CASE REPORT

A 55-year-old man came to our hospital on June 21, 1992, for evaluation of frequent chest pain, first noted about 2 years previously. He experienced substernal pain, which lasted several minutes, and heartburn while drinking cold water in the early morning. Angina was relieved by sublingual nitroglycerin at the time of hospital admission. He had no history of hypertension, diabetes mellitus, or hyperlipidemia, and no risk factor except cigarette smoking (60 pack-years).

The patient suffered from frequent throbbing headaches on one side of his head. His vision change (flashes and zigzag lines) and slurred speech occurred during a headache. He felt sleepy and confused during a headache. Also, he had Raynaud's disease. The very dramatic blanching was brought on by a cool environment and was limited to three or four digits of the left hand. Causes of secondary Raynaud's phenomenon were searched, including collagen vascular diseases (scleroderma, systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, polymyositis), arterial occlusive disease, blood dyscrasias, and so on. They were ruled out from clinical history, physical examination, and serologic tests--lupus erythematosus cell, fluorescent antinuclear antibody (FANA), anti-DNA, complement components (C3, C4, CH50), cryoglobulin, rheumatoid factor assay, serum globulins and proteins, routine hematologic and biochemical tests, and so on. Migraine was diagnosed if the score in part A totaled 7 or more of 26, and Raynaud's phenomenon was diagnosed if the score in part B totaled 4 or more of 11 by Miller et al.[3] His score in part A and part B totaled 19 and 4 each. [.sup.99m]Tc-RBC Raynaud's scan demonstrated that left over right finger ratio was 0.64 (normal group is 0.96 [+ or -] 0.15 and Raynaud group is 0.70 [+ or -] 0.40 in our laboratory) and confirmed Raynaud's disease (Fig 1).

Esophageal manometry and Bernstein test were performed for evaluation of heartburn. Infusion of acid, but not of saline solution, reproduced the symptoms of heartburn. Esophageal manometry demonstrated that peristaltic wave progagated well and peristaltic amplitude was normal and mean pressure of the lower esophageal sphincter (LES) was 37.0 (Katz et al[7] defined hypertensive LES as elevated LES pressure more than 45 mm Hg).

An ECG at rest was normal. The patient completed 11 metabolic equivalents of an exercise test (Bruce protocol) with no chest pain. Coronary angiography revealed normal findings and the ECG showed normal findings. Following two injections (total 0.4 mg) of ergonovine, 0.2 mg intravenously, he complained of severe chest pain in 2 min. The ECG showed 2-mm horizontal ST segment elevation in leads 2, 3, and VF, and reciprocal changes in leads 1, aVL, and [V.sub.2] to [V.sub.4] (Fig 2). Right coronary angiography revealed severe spasm (90 percent stenosis) of mid right coronary artery (Fig 3). Following two intracoronary injections of nitroglycerin, 200 [micro]g, chest pain was relieved and the ECG showed an isoelectric ST segment.

A diagnosis of coronary artery spasm, classic migraine, Raynaud's disease, and reflux esophagitis was made, and the patient was discharged from the hospital taking isosorbide dinitrate, nicardipine, antacids, and ranitidine. One month after commencing treatment with this medication, the patient was well with very few symptoms.

On August 4, 1992, the patient suddenly suffered from severe throbbing headache on left side of his head and visited a local clinic. The headache was completely relieved by ergotamine tartrate, but at 4 AM on August 5, 1992, the patient began to suffer from severe chest pain with four attacks at 1-h intervals. Four attacks of angina were relieved by inhalation of isosorbide dinitrate spray. Therefore, the patient visited our hospital earlier than on schedule and we added amlodipine and recommended that he not take ergotamine tartrate.

DISCUSSION

Robertson and Oates[2] described three patients with variant angina and Raynaud's disease. Among 30 patients described by Heupler,[8] 8 had migraine and 3 had Raynaud's phenomenon. Leon-Sotomayor[9] described several patients with both migraine and variant angina. Miller et al[3] described the high prevalence of migraine (26 percent) and Raynaud's phenomenon (24 percent) in variant angina. These reports suggest the possibility that a common underlying defect or mechanism may partially account for all three conditions.[2,3,8,9]

Rasmussen et al[5] observed that in the basal state, esophageal spasms were recorded in six of ten patients with coronary artery spasm but in none of ten patients without coronary artery spasm. Lipton et al[6] described a patient with variant angina and diffuse esophageal spasm. These reports suggest that there may be an association in some patients between epicardial coronary spasm and esophageal motility disorders.[5,6] Cannon et al[4] observed the association of dynamic abnormalities of coronary microvascular function and a trend toward higher mean and peak distal esophageal pressure in patients with microvascular angina. These reports suggest that a high prevalence of esophageal motility disorders has been noted in patients with coronary artery spasm or microvascular angina, leading to the hypothesis of a generalized abnormality in smooth muscle function.[4-6]

However, our patient demonstrated typical reflux esophagitis, not esophageal spasm, by esophageal manometry, although he demonstrated classic migraine, positive Raynaud's scan, and proven coronary artery spasm. We suppose that concomitant reflux esophagitis should represent a coincidence rather than a previously unrecognized syndrome.

In addition, vasoconstricting drugs, especially ergot alkaloids, should be avoided in patients with symptomatic coronary arterial spasm.

REFERENCES

[1] Koh KK. Various 12-lead electrocardiographic findings of diffuse three-vessel coronary artery spasm. Am Heart J 1992; 123:1187-91

[2] Robertson D, Oates JA. Variant angina and Raynaud's phenomenon. Lancet 1978; 1:452

[3] Miller D, Waters DD, Warnica W, Szlachcic J, Kreeft J, Theroux P. Is variant angina the coronary manifestation of a generalized vasospastic disorder. N Engl J Med 1981; 304:763-66

[4] Cannon RO, Cattau EL Jr, Yakshe PN, Maher K, Schenke WH, Benjamin SB, et al. Coronary flow reserve, esophageal motility, and chest pain in patients with angiographically normal coronary arteries. Am J Med 1990; 88:217-22

[5] Rasmussen K, Funch-Jensen P, Ravnsbaek J, Bagger JP. Oesophageal spasm in patients with coronary artery spasm. Lancet 1986; 1:174-76

[6] Lipton SA, Markis JE, Pine MB, Paulin S, Lindsay HE. Cessation of smoking followed by Prinzmetal's variant angina and diffuse esophageal spasm. N Engl J Med 1978; 299:775-76

[7] Katz PO, Dalton CB, Richter JE, Wu WC, Castell DO. Esophageal testing of patients with noncardiac chest pain or dysphagia. Ann Intern Med 1987; 106:593-97

[8] Heupler FA Jr. Syndrome of symptomatic coronary arterial spasm with nearly normal coronary arteriograms. Am J Cardiol 1980; 45:873-81

[9] Leon-Sotomayor LA. Cardiac migraine--report of 12 cases. Angiology 1974; 25:161-71

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