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20-year follow-up of a patient with coronary artery spasm.



Abstract: The authors present a 20-year follow-up of a patient with well-documented coronary artery spasm, who initially presented with syncope syncope

Effect of temporary impairment of blood circulation to a part of the body. It is often used as a synonym for fainting, which is loss of consciousness due to inadequate blood flow to the brain.
. The patient had excellent response to calcium channel antagonists and long-acting nitrates.

**********

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)

Case Report

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 precordial,
adj pertaining to the region over the heart or stomach: the epigastrium and inferior portion of the thorax.


precordial

pertaining to the precordium.
 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 asystole /asys·to·le/ (a-sis´to-le) cardiac standstill or arrest; absence of heartbeat.asystol´ic

a·sys·to·le
n.
The absence of contractions of the heart.
 (Fig. 1). Following the period of asystole, ST segment elevation gradually disappeared, and the electrocardiogram returned to normal. He had a coronary angiogram an·gi·o·gram
n.
An angiographic x-ray of blood vessels used in diagnosing pathological conditions of the cardiovascular system.//An x-ray of one or more blood vessels produced by angiography and used in diagnosing pathology in the cardiovascular
 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 circumflex /cir·cum·flex/ (serk´um-fleks) curved like a bow.

cir·cum·flex
adj.
1. Curving or bending around.

2. Bowed.



circumflex

curved like a bow.
 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 nitroglycerin (nī'trōglĭs`ərĭn), C3H5N3O9, colorless, oily, highly explosive liquid. It is the nitric acid triester of glycerol and is more correctly called glycerol trinitrate.  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 nifedipine /ni·fed·i·pine/ (ni-fed´i-pen) a calcium channel blocking agent used as a coronary vasodilator in the treatment of coronary insufficiency and angina pectoris; also used in the treatment of hypertension.  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 Shortness of Breath Definition

Shortness of breath, or dyspnea, is a feeling of difficult or labored breathing that is out of proportion to the patient's level of physical activity.
 when climbing one flight of stairs Noun 1. flight of stairs - a stairway (set of steps) between one floor or landing and the next
flight of steps, flight

staircase, stairway - a way of access (upward and downward) consisting of a set of steps
 and noted occasional ankle edema. Physical examination remained within normal limits, as did his electrocardiogram. Echocardiogram ech·o·car·di·o·gram
n.
A visual record produced by echocardiography.


Echocardiogram
A non-invasive ultrasound test that shows an image of the inside of the heart.
 showed normal left ventricle systolic Systolic
The phase of blood circulation in which the heart's pumping chambers (ventricles) are actively pumping blood. The ventricles are squeezing (contracting) forcefully, and the pressure against the walls of the arteries is at its highest.
 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]

Discussion

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 arteriovenous /ar·te·rio·ve·nous/ (-ve´nus) both arterial and venous; pertaining to or affecting an artery and a vein.

ar·te·ri·o·ve·nous
adj.
Abbr.
 block or sudden death. Coronary spasm is usually responsive to sublingual sublingual /sub·lin·gual/ (-ling´gwal) hypoglossal; beneath the tongue.

sub·lin·gual
adj. Abbr. SL
Below or beneath the tongue; hypoglossal.
 nitroglycerin, long-acting nitrates, and calcium channel antagonists. Diltiazem, verapamil verapamil /ve·rap·a·mil/ (ve-rap´ah-mil) a calcium channel blocker that dilates coronary arteries and decreases myocardial oxygen demand, used as the hydrochloride salt in the treatment of angina pectoris and of hypertension and the , 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 coronary artery disease, condition that results when the coronary arteries are narrowed or occluded, most commonly by atherosclerotic deposits of fibrous and fatty tissue. , left ventricular function, and disease activity. Patients with multi-vessel coronary artery disease had worse survival than patients with single vessel disease single vessel disease Univessel disease Cardiology  Coronary atherosclerosis that is very advanced in one artery  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 intimal

pertaining to or emanating from vascular intima.


intimal bodies
irregular mineralized masses covered by endothelium and protruding into the lumen of small arteries and arterioles of horses, especially in the intestinal
 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.
It's kind of fun to do the impossible.
--Walt Disney


Accepted May 5, 2004.

References

1. Burns A. Observation on some of the most frequent and important diseases of the heart; on aneurysm of the thoracic aorta; on Preternatural pulsation pulsation /pul·sa·tion/ (pul-sa´shun) a throb, or rhythmic beat, as of the heart.

pul·sa·tion
n.
1. The act of pulsating.

2. A single beat, throb, or vibration.
 in the epigastric region; and on the unusual origin and distribution of some of the large arteries of the human body. London, printed by James Muirhead for Thomas Bryce and Co., Med Booksellers, 1809, p 138.

2. Latham PM. Collected works, Vol. 1. London, New Syndenham Society, 1876, p. 445.

3. Danielopolu D. The pathology and the surgical treatment of angina pectoris. Br Med J 1924;2:554.

4. Prinzmetal M, Kennamer R, Merliss R, et al. Angina pectoris. I. A variant form of angina pectoris. Am J Med 1959;27:357-388.

5. Prinzmetal M, Ekmecki A, Kennamer R, et al. Variant form of angina pectoris. Previously undelineated syndrome. JAMA JAMA
abbr.
Journal of the American Medical Association
 1960;14:1794-1800.

6. Beltran P, Lichstein E, Sanders M, et al. Coronary artery spasm appearing as syncope. Arch Intern Med 1982;142:192-194.

7. Schroeder JS, Lamb IH, Bristow MR, et al. Prevention of cardiovascular events in variant angina by long-term diltiazem therapy. J Am Coll Cardiol 1983;1:1507-1511.

8. Antman E, Muller J, Goldberg S, et al. Nifedipine therapy for coronary artery spasm. Experience in 127 patients. NEJM NEJM New England Journal of Medicine  1980;302:1269-1273.

9. Morikami Y, Yasue H. Efficacy of slow-release nifedipine on myocardial myocardial /myo·car·di·al/ (-kahr´de-al) pertaining to the muscular tissue of the heart.

myocardial

pertaining to the muscular tissue of the heart (the myocardium).
 ischemic Ischemic
An inadequate supply of blood to a part of the body, caused by partial or total blockage of an artery.

Mentioned in: Antiangiogenic Therapy, Subarachnoid Hemorrhage, Ventricular Fibrillation


ischemic
 episodes in variant angina pectoris. Am J Cardiol 1991;68:580-584.

10. Chahnine RA, Feldman RA, Giles TD, et al. Randomized ran·dom·ize  
tr.v. ran·dom·ized, ran·dom·iz·ing, ran·dom·iz·es
To make random in arrangement, especially in order to control the variables in an experiment.
 placebo-controlled trial of amlodipine in vasospastic angina. Amlodipine study 160 group. J Am Coll Cardiol 1993;23:1365-1370.

11. Winniford MD, Johnson SM, Mauritson DR, et al. Verapamil therapy for Prinzmetal's variant angina: comparison with placebo and nifedipine. Am J Cardiol 1982;50:913-918.

12. Severi S, Davies G, Maseri A, et al. Long-term prognosis of variant angina with medical treatment. Am J Cardiol 1980;46:226-232.

13. Waters DD, Miller DD, Szlachcic J, et al. Factors influencing the long-term prognosis of treated patients with variant angina. Circulation 1983;8:258-265.

14. Mark DB, Califf RM, Morris KG, et al. Clinical characteristics and long-term survival of patients with variant angina. Circulation 1984;69:880-888.

15. Walling A, Waters DD, Miller DD. Long-term prognosis of patients with variant angina. Circulation 1987;76:990-997.

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.

18. Nobuyoshi M, Tanaka M, Nosaka H, et al. Progression of coronary atherosclerosis: is coronary spasm related to progression? J Am Coll Cardiol 1991;18:904-910.

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 FACC Fellow, American College of Cardiology  

From the Department of Medicine, Maimonides Medical Center The Maimonides Medical Center is non-profit academic medical center in Brooklyn, New York. History
The institution was founded in 1911 as the New Utrecht Dispensary.
, New York, NY. 11219.

Reprint requests to Elsayed Mohamed, MD, Department of Medicine, Maimonides Medical Center, 4802 10th Ave, Brooklyn, NY 11219.
COPYRIGHT 2004 Southern Medical Association
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2004, Gale Group. All rights reserved. Gale Group is a Thomson Corporation Company.

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Title Annotation:Case Report
Author:Lichstein, Edgar
Publication:Southern Medical Journal
Date:Oct 1, 2004
Words:1633
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