Variant angina pectoris caused by coronary artery spasm

Variant Angina Pectoris Caused by Coronary Artery Spasm

NEIL J. HART, MARK

M.D.

E. SILVERMAN,

SPENCER

B. KING,

M.D M.D.

A Nanta, Georgia

A 58 year old man presented with Prinzmetal’s variant angina. Recurrent ventricular tachyarrhythmias, uncontrolled by various medical regimens, prompted cardiac catheterization. At catheterization a congenital anomaly of the coronary circulation was demonstrated, consisting of a single coronary artery. During coronary angiography, arterial spasm occurred associated with chest pain and ventricular ectopic beats. This was relieved by the acute administration of sublingual nitrates. Subsequently, despite the frequent administration of nitroglycerin and isosorbide dinitrate, chest pain and ventricular tachycardia continued to occur sporadically. Because of this, saphenous vein bypass surgery was performed. After an initial symptom-free interval, the chest pain recurred. Recatheterization revealed complete occlusion of the distal circumflex artery at the insertion of the implanted saphenous vein. The use of aortocoronary saphenous vein bypass grafting for relief of symptomatic coronary arterial spasm is questioned. Before the advent of coronary arteriography, the occurrence of coronary arterial spasm was only speculative. With the widespread use of coronary arteriography, coronary artery spasm has been amply documented; however, its clinical significance remains controversial. Recently, the theory of coronary arterial spasm causing or contributing to myocardial ischemia has gained new support [l-5]. Our purpose here is to present a patient with a congenital absence of the right coronary artery, Prinzmetal’s variant angina and arteriographically demonstrated focal, transient coronary artery narrowing associated with chest pain and arrhythmias. Subsequent inspection of the coronary artery at the time of saphenous vein bypass grafting did not reveal any organic obstructive disease. CASE REPORT

From the Division of Cardiology, Department of Medicine and the Department of Radiology, Emory University School of Medicine, Emory University Hospital and Piedmont Hospital, Atlanta, Georgia. This study was supported in part by National Heart and Lung Institute Training Grant HE-05731. Requests for reprints should be addressed to Dr. Mark E. Silverman, Piedmont Hopital, 1968 Peachtree Road, NW, Atlanta, Georgia 30309. Manuscript accepted December 3. 1973.

A 58 year old man was admitted to the hospital after a 30 minute episode of squeezing substernal ed into

the

upper

portion

of his chest

and

both

on April 17, 1972, pain which radiatshoulders.

no associated shortness of breath, nausea or vomiting. had never previously had chest pain or chest discomfort, nied

having

There was perlipidemia,

any no

symptoms

history however,

rettes/day and had physical examination

February

1974

referable

to

his

There

was

The patient and he de-

cardiovascular

system.

of hypertension, peripheral vasospasm he smoked about 1 to 1 l/2 packs

or hyof ciga-

done so for 40 years. At the time of admission, revealed a blood pressure of 160/90 mm Hg, a

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Figure 7. Electrocardiogram demonstrates sinus arrhythmia ventricular contraction.

ET AL.

obtained on, admission and a single premature

pulse rate of 90 beats/min, bilateral arcus normal cardiac size. An electrocardiogram

senilis

and

obtained

on admission was normal (Figure 1). Two hours later, the chest pain recurred and was accompanied by diaphoresis. Blood pressure was not palpable, and physical examination now revealed pulmonary rales, jugular venous distention and a ventricular gallop. An electrocardiogram showed complete heart block with narrow QRS complexes and a ventricular rate of 32 beats/min. There was transient S-T segment elevation in leads I I, Ill and aVF. A percutaneous temporary transvenous pacemaker was positioned in the apex of the right ventricle. The ventricle

was paced at 75 beatsfmin and the blood pressure returned to its previous level. Because the signs of congestive failure persisted, digitalis was administered. Later that day frequent ventricular premature contractions occurred for which the patient was treated with small doses of intravenous lidocaine. By the next day evidence of congestive failure was no longer present; however, persistence of frequent ventricular extrasystoles required further administration of lidocaine. Two days after admission substernal chest pain recurred and was associated with dyspnea, diaphoresis and hypotension. Ventricular tachycardia occurred, which reverted to the pacemaker rhythm after the intravenous administration of 100 mg of lidocaine. During the next 8 days, the patient experienced at least 30 separate episodes of ventricular tachycardia or ventricular fibrillation. On 11 occasions the ventricular arrhythmia was unresponsive to medication and required “thumpversion” and/or electrical defibrillation. These attacks would almost always occur at night and were characterized by an acute “crushing” substernal chest pain followed by diaphoresis, nausea and dyspnea. The systolic blood pressure would fall to 50 to 60 mm Hg, and numerous ventricular premature contractions would occur. Ventricular tachycardia and ventricular fibrillation frequently ensued (Figure 2). Monitored precordial leads revealed marked elevations in the S-T segment during the bouts of pain. Shortly after cessation of the pain the S-T segments would return to the isoelectric line and remain normal until the next attack

A

B

D Monitored

E

Monitored

270

ECG

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Tracing

(rpiodified

V,)

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EGG

Tracing

(modified

V,)

Figure 2. Left, continuous recording before and during an acute angina/ attack. Line A shows sinus beats, paced beats and partially fused beats. In lines B and C there is progressive S-T segment elevation. In lines D and E ventricular tachycardia occurs with spontaneous resolution. Line F was recorded when the angina1 pain had subsided. Above, chest pain was present when this tracing was begun. S-T segment elevation is present in lines A and B. The rhythm degenerated to ventricular tachycardia (lines B, C and D) and spontaneously reverted to the paced rhythm.

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of pain. No pathologic “CT” waves ever developed. Arrhythmia was noted only at the time of chest pain. Serum potassium, glucose and arterial blood gases determined at the time of an attack were normal. Various medications were administered, alone and in combination, without appreciably altering the incidence or severity of attacks. Nitroglycerin, isosorbide dinitrate, quinidine sulfate, procainamide, propranolol and potassium chloride were all tried in addition to lidocaine. Several attempts at “over-drive” suppression of the ventricular extrasystoles at pacemaker rates as high as 110 beats/min were also unsuccessful. On April 27, 1972, the patient was transferred to Emory University Hospital for coronary arteriography and left heart catheterization. The first two injections of contrast material into the left coronary artery were normal. Approximately 5 minutes after the third selective left coronary artery injection, the patient experienced his typical chest pain with S-T segment elevation and frequent premature ventricular contractions. This last injection showed focal, smooth, 75 per cent narrowing of the mid-portion of the dominant circumflex artery (Figure 3). The chest pain terminated about 3 minutes after the sublingual administration of 0.4 mg nitroglycerin and 5 mg isosorbide dinitrate. On the following day the patient underwent catheterization again while receiving large doses of sublingual isosorbide dinitrate. The previously observed area of spasm was not detected (Figure 4). The circumflex artery was quite large and dominant to the posterior circulation giving rise to the posterior descending artery and the atrioventricular node branch. It then passed the crux of the heart and continued in the right atrioventricular groove terminating 2 cm from the right coronary cusp. Despite multiple injections into the right coronary cusp, the right coronary artery

Figure 3. Left anterior oblique view of the left coronary artery showing a focal smooth narrowing of the mid-portion of the circumflex artery (arrow). Immediately prior to this injection the patient had angina/ pain.

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could not be identified. Because of continued bouts of chest pain with threatening ventricular arrhythmias, it was decided that a saphenous vein bypass graft would be placed into that portion of the circumflex artery in the right atrioventricular groove in order to increase posterior circulation during periods of circumflex artery spasm. At surgery, inspection and palpation of the anterior descending and circumflex arteries were normal. Palpation in the fat pad by the right coronary cusp revealed no proximal right coronary artery. The coronary artery in the right atrioventricular groove was opened and a No. 5 French catheter was inserted into the lumen and was advanced across the crux of the heart and up into the left atrioventricular groove in the circumflex artery, thus demonstrating the continuity of the circumflex vessel across the crux of the heart to the right atrioventricular groove. The catheter was then removed and placed into the distal circumflex artery and passed as far as it would go toward the right

Figure 4. A, left anterior oblique view of the left coronary artery shows a widely patent circumflex artery. B, right anterior oblique view of the left coronary artery again demonstrates a large, patent circumflex branch. The patient was pretreated with isosorbide dinitrate.

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after sodes

the

onset

of chest

of

his

illness,

he

had

no further

pain and now leads an active

epi-

life.

COMMENTS

Vein graft

-

@ Ant descending coronary 0 0 Area of postoperative occlusion

Left

Anterior Oblique View Schematic illustration of the aortocoronary bypass graph inserted in the right atrioventricular groove, distal to the observed area of spasm. .Inset A shows the mid-portion of the circumflex artery without spasm and inset B the same area as it appeared during an angina1 attack. Postoperative occlusion occurred in the area of competitive flow in the native artery.

Figure 5.

coronary cusp. cusp. Palpation

The catheter stopped 2 cm short of the between this point and the right cusp

did not demonstrate a proximal right coronary The previously prepared saphenous vein was into the

the aorta circumflex

and anastomosed vessel in the

groove (Figure approximately the

cleared

due to technical

patient

had

following

mild

digitalis showed

operative

that

tracing,

is,

pattern

anterior

areas of myocardium being supplied by the left circumflex artery. Spasm in the mid-portion of this vessel was presumably responsible for the inferolateral injury current noted on the electrocardiogram and documented at catheterization. In addition, the artery to the atrioventricular node originated distal to this area of spasm, and this most likely accounted for the development of complete heart block during several of the attacks of pain. Prinzmetal considered this variant form of angina pectoris to be caused by atherosclerosis of a single major coronary artery [6]. This concept was supported by the coronary angiographic findings of MacAlpin [7] who reported focal stenotic lesions of a major coronary artery in 12 patients with variant angina pectoris. The vascular bed in-

Postoperafailure

which

Postoperatively

a similar

of

the graft was studies could

reasons.

congestive

therapy.

electrocardiogram across the creased in was minimal

continuation atrioventricular

5). The vessel receiving 2 mm in diameter. Flow

not be performed tively

to the right

artery. sutured

In 1959 Prinzmetal [6] described a variant form of angina pectoris. Our patient had lthe following features suggestive of this yariant form: severe, retrosternal chest pain occurring at rest with associated S-T segment elevation; a ,tendency for the pain to recur cyclically; the occurrence of arrhythmias following the chest pain, particularly ventricular tachycardia, ventricular fibrillation and atrioventricular block; an observed anatomic correlation of the location of myocardial ischemia to the inferolateral S-T segment elevation on electrocardiograms; and a response to nitrates. Because of the continued serious arrhythmLas, unresponsive to medical management, selective coronary arteriography was performed. This demonstrated a single coronary artery with the lateral and inferior

the

to the pre-

T wave

inversion

precordium; however, this gradually demagnitude and at the time of discharge (Figure 6). Postoperatively, until the time

of discharge, the patient had no more pain suggestive of angina pectoris and no further ventricular arrhythmias or S-T segment elevations. He remained tively

and

evation

asymptomatic

then

and

catheterization

the typical

ventricular the

for chest

8 weeks pain,

arrhythmias

native

artery

postopera-

S-T segment recurred.

distal

el-

At

to the vein

re-

insertion was found to be closed (Figure 5). Over the next several weeks the attacks of pain became less severe and were unaccompanied by arrhythmias. The patient was discharged and maintained on nitrate therapy. For tent

272

several

bouts

months

of angina

February 1974

he continued pectoris.

to have

However,

V4

V5

V6

graft

intermit-

by 10 months

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Figure 6.

The electrocardiogram on discharge shows changes in the S-T segment and T waves compatible with anterior-interior subendocardial ischemia. There is no initial force abnormality indic,ative of transmural myocardial infarction.

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volved was predicted by the location of the S-T segment elevation on the electrocardiogram. However, focal, high grade, obstructive lesions have not been invariably found. There have been several case reports emphasizing the normal or near normal coronary arteries at the time of coronary angiography in patients with the clinical findings of Prinzmetal’s angina [1,2,4,5,8]. Spasm occurring in atherosclerotic coronary arteries has been reported to produce angina pectoris and myocardial infarction [9]. Dhurandhar et al. [2] described a patient with variant angina arteriographically demonstrated pectoris and proximal right coronary artery spasm during an episode of chest pain. Repeat injection of contrast material after administration of nitroglycerin and isosorbide dinitrate showed some irregularities of the vessel but no spasm and adequate peripheral run-off. Despite insertion of a saphenous vein graft, which bypassed the area of spasm, the patient died during a subsequent attack of chest pain. At autopsy an eccentric plaque was found in the proximal right coronary artery which narrowed the vessel lumen by 75 per cent. Spasm of a nonatherosclerotic coronary artery has been postulated to cause angina pectoris and acute coronary insufficiency [1,4,5]. Recently, Cheng et al. [3] reported a case of inferior wall infarction which apparently resulted from severe spasm of the left circumflex artery during selective coronary arteriography. Repeat arteriography 3 months later showed the previously involved vessel to be normal. After reviewing their experience with coronary arterial spasm, Demany, Tambe and Zimmerman [lo] concluded that isolated coronary artery spasm occurring in a normal vessel could “if sufficiently severe, reduce the blood flow distally and produce an attack of angina pectoris which can terminate tragically,” We have seen, at coronary arteriography, spasm of a previously normal right coronary artery of such a degree that all flow in the artery terminated resulting in cardiac arrest. Our patient showed rather marked luminal narrowing of the circumflex artery on initial coronary study. Associated with this were transient S-T segment elevation, ventricular extrasystoles and severe chest pain which was relieved by nitroglycerin. On repeat angiography, following the administration of isosorbide dinitrate, the lumen was widely patent. This was confirmed by direct examination at the time of surgery. The remaining coronary branches were smooth and no atherosclerotic process was demonstrated. Our case is very similar to those described by Cheng et al. [4]. They studied four patients with “typical” Prinzmet-

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al’s variant angina who had normal coronary arteries by arteriography. Coronary artery spasm was demonstrated by selective arteriography in two patients at the time of cardiac catheterization. One died of ventricular fibrillation and had normal coronary arteries at autopsy. The others improved on nitroglycerin and isosorbide dinitrate therapy. Our patient, in addition, had no atherosclerosis found on surgical inspection. Oliva et al. [I] also documented coronary artery spasm in Prinzmetal angina by coronary arteriography. Their coronary arteriograms showed not only variability in the severity of the spasm, but also that the location and extent of the coronary artery spasm was not constant. Because of this latter observation, they questioned the use of saphenous vein bypass therapy alone. The prognosis in patients with variant angina pectoris appears to be grave and justifies a vigorous therapeutic approach [ll]. In focal stenotic lesions, saphenous vein bypass grafting would warrant consideration. The role of surgery for alleviation of symptoms produced by spasm in a nonatherosclerotic coronary artery is less clear. Saphenous vein bypass grafting was performed in our patient because of his progressive deterioration unchecked by various medical interventions. Despite early surgical success, competition of flow between the graft and native circulation resulted in occlusion. This procedure is not recommended, therefore, in patients with spasm in nonatherosclerotic coronary arteries. The congenital anomaly of a single coronary artery is rare [12]. A single coronary artery, however, does not preclude longevity. The average age at death in nine recorded cases [ 131 of single coronary artery with death resulting from myocardial infarction was identical to that in all other patients in the series who died following myocardial infarction (49 years). Severe atherosclerotic disease could cause complete obliteration of a coronary artery lumen at or very close to its ostia. This was considered in our patient; atherosclerotic occlusion of the right coronary artery was searched for at angiography and at surgery, but we found no evidence of it. An anomalous origin of the right coronary artery from the pulmonary artery was not shown by angiography or by inspection at the time of surgery. In conclusion, we have described a patient with symptomatic coronary arterial spasm with no evidence of coronary atherosclerosis by angiography or by inspection at the time of surgery. Surgical therapy proved to be of no benefit. A congenital anomaly of a single coronary artery was demonstrated.

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ACKNOWLEDGMENTS

We thank Dr. A. Abernathy for permission to study and report this patient. The cooperation of Dr. E. 0. Wheeler of Boston, Massachusetts, who

provided follow-up data is appreciated. Grateful acknowledgment is made to the Piedmont Hospital CCU nurses and the Emory Unliversity Hospital nurses for their expert care of this patient.

REFERENCES 1. 2.

3.

4.

5. 6.

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Oliva PB, Potts DE, Pluss RG: Coronary arterial spasm in Prinzmetal angina. N Engl J Med 288: 745, 1973. Dhurandhar RW, Watt DL, Silver MD, et al.: Prinzmetal’s variant form of angina with arteriographic evidence of coronary arterial spasm. Am J Cardiol 30: 902, 1972. Cheng TO, Bashour T, Singh BK, et al.: Myocardial infarction in the absence of coronary arteriosclerosis. Am J Cardiol 30: 680, 1972. Cheng TO, Bashour T, Kelser GA, et al.: Variant angina of Prinzmetal with normal coronary arteriograms. A variant of the variant. Ann Intern Med 76: 862, 1972. Whiting RB, Klein MD, Vander Veer J, et al.: Variant angina pectoris. N Engl J Med 282: 709, 1970. Prinzmetal M, Kennamer R, Merliss R, et al.: A variant form of angina pectoris: preliminary report. Am J Med 27: 375, 1959.

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7. 8.

9. 10. 11. 12.

13.

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MacAlpin R: Variant angina pectoris (letter). N Engl J Med 282: 1491, 1970. Gianelly R, Mugler F, Harrison DC: Prinzmetal’s variant of angina pectoris with only slighlt coronary atherosclerosis. Calif Med 108: 129, 1968. Sewell WH: Coronary spasm as a primary cause of myocardial infarction. Angiology 17: 1, 1966. Demany MA, Tambe A. Zimmerman HA: Coronary arterial spasm. Dis Chest 53: 714, 1968. Silverman ME, Flamm MD: Variant angina pectoris. Ann Intern Med 75: 339, 1971. Alexander RW, Griffith GC: Anomalies of the coronary arteries of the heart and their clinical significance. Circulation 14: 800, 1956. Allen Maj GL, Snider Capt TH: Myocardial infarction with a single coronary artery. Arch Intern Med 117: 261, 1966.