Emergent Intervention in a Life-Threatening Multivessel Coronary Artery Vasospasm

Abstract 

While coronary artery spasm (CAS) is not an uncommon event and spontaneous spams might occur in young patients as well as during percutaneous coronary intervention in elderly patients, extensive multivessel spasm rarely occurs. Recognizing and rescuing massive multivessel CAS is paramount, because it can cause devastating hemodynamic embarrassment and even death. When a 66-year-old female with recurrent chest pain, normal cardiac enzymes, and an unremarkable EKG had her left coronary artery cannulated, she suddenly developed bradycardia that immediately progressed into pulseless electrical activity (PEA), most likely directly related to the acute CAS event, despite being pretreated with sublingual nitroglycerin. Initial angiography of the left coronary system showed total occlusion of the left descending and left circumflex arteries. A standard cardiopulmonary resuscitation protocol was immediately initiated. After three intra-coronary 400 mcg nitroglycerin injections, the patient regained spontaneous circulation, and her heart rate and blood pressure recovered. Post-event left coronary artery angiography revealed complete reversal of the spasm. No potentially lethal ischemia-induced ventricular arrhythmias were noted. The patient’s vital signs remained stable and coronary angiography was continued as planned. CAS, especially multivessel spasm, requires accurate diagnosis and immediate treatment with intracoronary artery injection of high doses of nitroglycerin to relieve the spasm and restore spontaneous circulation. This measure may be warranted in high-risk patients both prior and during catheterization.

Introduction

Coronary artery spasm is not an uncommon event. Spontaneous vasospasm during percutaneous coronary intervention is well described¹; however, extensive multivessel spasm rarely occurs. Prompt recognition and immediate treatment of a massive coronary artery spasm is crucial in prevention of an acute devastating hemodynamic adverse condition that could result in death.

We report the case of a 66-year-old woman with hemodynamically insignificant coronary artery disease, who suffered from a spontaneous multivessel coronary artery spasm of the left anterior descending and left circumflex arterial trees during a cardiac catheterization. 

Case report

A 66-year-old woman with a history of recurrent progressive retrosternal chest pain over the last few months was scheduled for diagnostic coronary angiography. Four years prior, she had a high-grade stenosis of her proximal right coronary artery (RCA), requiring an angioplasty and a drug-eluting stent. Her current symptoms remained despite therapy with aspirin, atorvastatin, clopidogrel, metoprolol, and sublingual nitroglycerin. She is a non-smoker, and denies alcohol or illicit drug use. 

A 12-lead electrocardiogram prior to her cardiac catheterization showed normal sinus rhythm with no acute ST-T wave abnormalities. Serial cardiac enzymes, including troponin-I levels on admission, were within normal limits. A nuclear exercise stress study performed a week prior was normal. Her echocardiogram showed a non-dilated left ventricular cavity with a well-preserved systolic function and an ejection fraction of 60-65%.

In the cardiac catheterization laboratory, the patient received standard pre-procedural preparation, and a 6 French side-arm sheath was percutaneously advanced into the right femoral artery uneventfully. Prior to angiography, after conscious sedation and sublingual nitroglycerin therapy (0.4 mg), the patient’s baseline blood pressure was 120/60 mmHg. As a 6 French, soft-tipped FL4 diagnostic guide catheter was advanced near her left main coronary artery, the patient suddenly developed bradycardia, with a heart rate of 53 beats/min, and hypotension, with a drop in direct arterial blood pressure to 65/48 mmHg. She subsequently became pulseless, accompanied by electromechanical cardiac dissociation. Standard cardiopulmonary resuscitation protocol was immediately initiated. The clinical vignette of cardiovascular collapse raised differential diagnoses, such as perforation, tamponade, or vasovagal or anaphylactic response to contrast. However, an immediate angiography of the left coronary arterial system showed total occlusions of the proximal left anterior descending coronary artery (LAD) and left circumflex artery (LCX) (Figure 1). It was strongly felt that her pulseless episode was due to the acute coronary artery spasm, despite the pretreatment with sublingual nitroglycerin. While cardiopulmonary resuscitation was in tow, the first dose of 400 mcg of nitroglycerin was immediately administered into her left main coronary artery. Two more doses were administered intra-arterially within a minute. Within three documented minutes, her spontaneous circulation was restored. Her improved blood pressure was 116/78 mmHg and her pulse rate was 141 beats/min. An immediate left coronary artery angiography revealed complete restoration of coronary artery occlusion, suggesting resolution of the acute coronary spasm (Figure 2). Her proximal LAD had mild luminal irregularities, with up to 30% luminal stenosis, near the site of the previously seen spasm. Her right coronary angiogram was widely patent at the stent site, with only a 20-30% eccentric luminal narrowing at the distal end of the stent (Figure 3). She had a well-preserved left ventricular systolic function, with no significant regional wall motion and an estimated ejection fraction of 65-70%. The patient remained hemodynamically stable and her coronary angiography procedure was completed. She had an uneventful 12-month post-procedural course after combination therapy of verapamil, amlodipine, and nitrates.

Discussion

Incidence of CAS varies worldwide. In the United States, the frequency of vasospastic angina is low (4%). Vagal withdrawal is thought to be the most common mechanism leading to spontaneous spasms. Other causes include abnormal nitric oxide synthesis, enhanced phospholipase C enzyme activity, elevated C-reactive protein level, magnesium deficiency, and illicit use of drugs such as cocaine and alcohol. 

Patients classically present with chest pain at rest, and symptoms are not triggered by physical exertion or emotional stress. Most patients have evidence of mild angiographic atherosclerotic coronary artery disease. The provocation tests have an acceptable level of safety and the assessment of spasm type may provide useful information for the risk prediction of patients with vasospastic angina.

CAS occurs in 1-5% of patients during percutaneous coronary intervention (PCI) and may even be induced solely by guide wire manipulations.¹ Vascular hyperactivity is thought to be the underlying autogenic mechanism for spasm. During PCI, the coronary arterial endothelium can become hypersensitive to trauma caused by various instruments such as guide wires, balloons, stents, and Rotablator devices (Boston Scientific).² Focal coronary artery spasm, defined as over a 75% reduction in coronary artery diameter after administration of ergonovine, may commonly occur within 1-4 cm of an angiographically apparent lesion. Conversely diffused spasm, defined as more than 20 mm in length, and multivessel, epicardial coronary artery spasm are rarely reported. The latter are potentially lethal and can lead to cardiogenic shock.³

Catheter-induced coronary artery spasm can be distinguished from a spontaneous coronary artery spasm, as the former is usually confined to a solitary artery, usually the RCA, and is within 2 cm of the catheter tip. The spasm is usually smooth and concentric, and in most cases, readily reversed by intracoronary vasodilators. A focal spasm of the coronary artery is often associated with an atherosclerotic lesion near the spasm site. 

Once the diagnosis of CAS is made, nitroglycerin administered by any route (intracoronary, intravenous, topical, or sublingual) effectively treats angina and myocardial ischemia within minutes. Long-acting nitrate preparations reduce the frequency of recurrent events.⁴ Coronary angioplasty performed in CAS patients produced results similar to those without variant angina.⁵ There was no significant improvement with angioplasty compared with medical therapy in patients with diffuse, multivessel spasm.⁶

In our patient, a massive, multivessel coronary artery spasm occurred simultaneously in the LAD and LCX, despite pre-treatment with a conventional dose of sublingual nitroglycerin, which then progressed to electromechanical dissociation and cardiac arrest. Attempts at prevention, immediate recognition of CAS, and therapy with intracoronary administration of high doses of nitroglycerin are critical. Our patient has mild coronary artery disease and therefore, although unlikely, a diffuse coronary spasm might have been catheter-induced due to catheter manipulation. Regardless, after recognition of life-threatening CAS, she was treated with double calcium channel blocker and nitrate, which eliminated her frequent chest pain symptom. In known high-risk patients, continuous intravenous infusion of nitroglycerin may reduce CAS during PCI. Interventions such as coronary artery stenting and bypass surgery may be recommended when fixed coronary occlusions have been demonstrated. In rare occasions, in patients with severe CAS, potentially lethal ischemic-induced arrhythmias may justify the application of automatic defibrillator implantation.⁷ Therapy with ranolazine, in combination with amlodipine and beta-blockers, may exert potent protective effects against chronic stable angina; however, its role in vasospatic angina is uncertain.⁸ 

Conclusion

Massive CAS may lead to a severe unstable hemodynamic situation and can be lethal. Prompt recognition and immediate treatment with intracoronary administration of high doses of nitroglycerin can prevent such a crisis by relieving coronary artery spasms and restoring spontaneous circulation. Continuous infusion of nitroglycerin may be warranted during coronary angiography in selected high-risk patients.

This article received a double-blind peer review from members of the Cath Lab Digest editorial board. 

The authors may be contacted via Dr. Koroush Khalighi at koroushkhalighi@gmail.com.

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*Internal Medicine, Easton Hospital, Easton, Pennsylvania; †Clinical Professor of Medicine, Drexel University, Director of the Electrophysiology Lab, Easton Hospital, Easton, Pennsylvania