20-year follow-up of a patient with coronary artery spasm.
Coronary artery spasm was first described at the beginning of the 19th century, (1) and was noted in the literature in the 19th and the beginning of the 20th centuries. (2,3) It was not accepted as a clinical entity until publication of the work by Prinzmetal et al describing variant angina in 1959. (4,5) Although reports of variant angina have become more frequent in the literature, there has been little written regarding the long-term prognosis (more than 10 yr) of patients with this condition.
This report presents a 20-year follow-up of a patient with well-documented coronary artery spasm, who initially presented with syncope, and was first reported in 1982. (6)
This 56-year-old male presented in November 1980, with a history of unresponsiveness. The patient's wife claims that she was awoken at 4:00AM by her husband making grunting noises, and found him to be unresponsive when she tried to wake him. He regained consciousness within 5 minutes, and was brought to the hospital even though he was asymptomatic at that time. When interviewed, the patient claimed that he "dreamt that he had chest pain." He had no significant medical history, and was a nonsmoker. His father died as a result of a myocardial infarction at age 51. Physical examination showed an intermittent [S.sub.4] gallop, and a grade I early systolic murmur at the apex. The remainder of the physical examination was within normal limits. The resting electrocardiogram showed normal sinus rhythm, and was within normal limits. During a Holter recording which was done later that day, the patient described an episode of precordial heaviness, and then became dizzy and lost consciousness for 15 seconds. This episode occurred while the patient was at rest. Review of the Holter recording showed an episode of significant ST elevation, followed by a progressive sinus bradycardia, and finally a 10 second period of asystole (Fig. 1). Following the period of asystole, ST segment elevation gradually disappeared, and the electrocardiogram returned to normal. He had a coronary angiogram later that day which showed normal cardiac pressures, cardiac output, and wall motion. Coronary angiography showed a 30% narrowing of the distal left main coronary artery. The remainder of the coronaries were normal. The artery to the sinus atrial node arose from the proximal left circumflex artery. Ergonovine provocation test was performed, and after the third bolus of ergonovine, the left main coronary artery showed evidence of coronary spasm with at least a 75% obstruction (Fig. 2). The patient did not complain of chest pain or have any ST changes, but was given nitroglycerin 0.4 mg sublingually, and the angiogram was repeated five minutes later. After nitroglycerin, the obstruction had returned to the 30% noted before the ergonovine stimulation. The patient was started on nifedipine 10 mg orally four times a day, and nitroglycerin 6.5 mg orally four times a day. He later was switched to long-acting nifedipine 30 mg daily.
He continued to do well until 1996, when he was diagnosed with diabetes mellitus and started on metformin. In June 2002, he complained of shortness of breath when climbing one flight of stairs and noted occasional ankle edema. Physical examination remained within normal limits, as did his electrocardiogram. Echocardiogram showed normal left ventricle systolic function and normal left ventricle compliance. Stress thallium showed an ejection fraction of 66%, and a mild inferior defect with no evidence of ischemia.
[FIGURE 1 OMITTED]
[FIGURE 2 OMITTED]
Attacks of variant angina usually resolve spontaneously. However, if the coronary spasm is prolonged, it can lead to myocardial infarction, life-threatening ventricular arrhythmias, high-degree arteriovenous block or sudden death. Coronary spasm is usually responsive to sublingual nitroglycerin, long-acting nitrates, and calcium channel antagonists. Diltiazem, verapamil, and nifedipine, as well as amlodipine, have all been shown to be effective in preventing episodes of variant angina. (7-11)
The long-term prognosis of patients with variant angina has been addressed in a few studies, with the longest average follow-up being 7 years. In a review of four series, the overall survival of variant angina ranged from 88 to 98% at 1 year, and from 84 to 97% at 3 years. (12-16) The survival without myocardial infarction ranged from 70 to 86% at 1 year, and from 63 to 85% at 3 years. In a study by Walling et al, (15) the overall survival and survival without myocardial infarction at 5 years was 89% and 69% respectively. This study included 217 patients. Of these patients, 25% developed myocardial infarctions, 40% of them in the first 3 months of follow-up. Three factors significantly predicted the survival in these patients: extent of concomitant coronary artery disease, left ventricular function, and disease activity. Patients with multi-vessel coronary artery disease had worse survival than patients with single vessel disease and those without coronary artery disease. Patients with spontaneous attacks associated with arrhythmias had 25% mortality compared with 5% in patients with spasm documented by ergonovine provocation only. Patients with impaired left ventricular function had worse survival than patients with normal left ventricular function. Treatment with calcium channel antagonists has improved survival without myocardial infarction as compared with other medical therapy. However, calcium channel antagonists did not improve the overall survival. In a series by Yasue et al, (16) multivariate analysis yielded independent predictors of survival without myocardial infarction that included treatment with calcium channel antagonists, extent of coronary artery disease, and site of ST elevation. A study by Bory et al (17) that included 277 patients with documented coronary artery spasm and normal or near-normal coronary arteries, with a median follow-up of 89 months, failed to show significant predictive factors for sudden death, myocardial infarction, or persistent angina on multivariate analysis.
Another point of interest is the relationship between coronary spasm and progression of coronary atherosclerosis. In a study by Nobuyoshi et al, (18) a positive response to ergonovine provocation test was the strongest predictor for new myocardial infarction and disease progression without infarction. It was hypothesized that coronary spasm caused intimal rupture and allowed penetration of inflammatory cells, leading to the progression of the atherosclerotic process.
Our patient presented with a dramatic manifestation of coronary spasm with an excellent response to calcium channel antagonists and nitrates. He remained symptom free for a follow-up period of 20 years, with no documentation of disease activity or progression of his coronary artery atherosclerosis.
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Accepted May 5, 2004.
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10. Chahnine RA, Feldman RA, Giles TD, et al. Randomized placebo-controlled trial of amlodipine in vasospastic angina. Amlodipine study 160 group. J Am Coll Cardiol 1993;23:1365-1370.
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16. Yasue H, Takizawa A, Nagao M, et al. Long-term prognosis for patients with variant angina and influential factors. Circulation 1988;78:1-9.
17. Bory M, Pierron F, Panagides D, et al. Coronary artery spasm in patients with normal or near normal coronary arteries. Long-term follow up of 277 patients. Eur Heart J 1996;17:1015-1021.
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RELATED ARTICLE: Key Points
* Coronary aretery spasm can present as syncope.
* Patients respond to treatment with calcium channel antagonist and long-acting nitrates.
* Patients can remain event-free for up to 20 years on medical management.
Elsayed Mohamed, MD, and Edgar Lichstein, MD, FACC
From the Department of Medicine, Maimonides Medical Center, New York, NY. 11219.
Reprint requests to Elsayed Mohamed, MD, Department of Medicine, Maimonides Medical Center, 4802 10th Ave, Brooklyn, NY 11219.
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|Title Annotation:||Case Report|
|Publication:||Southern Medical Journal|
|Date:||Oct 1, 2004|
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