Diffuse or segmental spasm in the coronary arteries causes Printzmetal angina. There is a decrease in blood supply to the myocardium generating symptoms like chest pain. The coronary arteries may develop spasm as a result of exposure to cold weather, exercise, or a substance that promotes vasoconstriction as alpha-agonists (pseudoephedrine and oxymetazoline). Recreational drug use, for example, cocaine use, is associated with the development of vasospastic angina, especially when used concurrently with cigarette smoking.
Incidence or prevalence of Prinzmetal angina is still unknown. The reason for this is the possible misdiagnosis or confusion with other conditions that might present with the same symptoms, and further evaluation is not sought. Some studies show that the Japanese population has an increased risk of developing vasospastic angina when compared with Caucasian populations. The difference between the Japanese population and the Caucasian population is that the former has a three times higher risk. The average age of presentation of vasospastic angina is around the fifth decade of life. Females are more likely within the Japanese population to experience vasospastic angina.
The underlying mechanism in the development of coronary artery spasm is multifactorial. One mechanism that might generate the spasm is an increased reactivity of the vessels to vasoconstrictive stimuli and subsequent high-grade stenosis in a segment of the coronary or diffusely, creating low coronary flow and with this myocardial injury due to ischemia. The etiology of the hyperreactivity of the coronary vessels is unclear but could be related to endothelial dysfunction and primary smooth muscle cells of the coronary vessels that might have impaired regulatory mechanism for vasoconstriction and vasodilation. Balances within the sympathetic and parasympathetic tone is also an important factor that regulates flow in the coronaries. Imbalance in this system can predispose exaggerated vasoconstriction under normal circumstance and during exposure of acetylcholine and methacholine.
Typical cardiovascular risk factors have not directly been associated with the presence of vasospastic angina, except for cigarette smoking and inflammatory states determined by high hs-CRP levels. A metabolic disorder such as insulin resistance has also been associated with vasospastic angina.
Multiple triggers have been associated with the development of vasospastic angina.
Several drugs such as ephedrine and sumatriptan can cause typical chest pain due to coronary spasm. Recreational drugs like cocaine, amphetamines, alcohol, and marijuana are also possible precipitating factors.
Environmental factors such as cold water can cause spasms in the coronaries. Valsalva maneuver, hyperventilation, and coronary manipulation through cardiac catheterization also can produce hyperreactivity of the coronaries.
Patients with vasospastic angina present with the following:
Other vasospastic disorders, like Raynaud phenomenon or a migraine, can be associated with this subset of patients. Patients may complain of recent or past episodes with some symptom-free periods.
The international study group of coronary vasomotion disorders known as COVADIS, created a diagnostic criterion to determine the presence of Prinzmetal angina. These include:
Because of the lack of electrocardiographic evidence, further studies might be considered. The initial workup should be to evaluate the possibility of fixed obstructive coronary artery disease.
If appropriated, a noninvasive stress test can be performed. Most of these patients will have a normal stress test or negative for ischemia. However, a subgroup of patients (10% to 30%) can have exercise-induced spasms with electrocardiographic changes for ischemia that are not specific for vasospastic angina versus fixed coronary obstruction. This group of patients should undergo coronary catheterization to determine obstructive coronary disease. Also, a negative stress test with suspicion for coronary obstruction should be evaluated with cardiac catheterization.
During cardiac catheterization, coronary spasms can be visualized spontaneously or under drug induction.
Ergonovine, acetylcholine, and hyperventilation, can be used in the catheterization laboratory in an attempt to confirm the diagnosis of coronary vasospasm. These tests are warranted only when the diagnosis of vasospastic angina is suspected but not firmly established. At present, provocative pharmacologic testing is not frequently performed.
An ambulatory electrocardiogram also can be used to record transient electrocardiographic changes during acute spontaneous events.
It is important to remember that when a patient has acute chest pain or angina, the focus is to evaluate for possible fixed coronary obstruction.
Treatment is focused on decreasing episodes of angina and preventing complications like myocardial injury and arrhythmia. Lifestyle modifications should be encouraged, especially smoking cessation. This is one of the critical interventions in reducing the frequency of episodes. Avoiding medications or drugs that can trigger coronary vasospasm, for example, cocaine, marijuana, and ephedrine-based products) is also important.
An interprofessional approach to Prinzmetal angina is recommended.
Prinzmetal angina is not an easy diagnosis to make and may require tests including cardiac catheterization with provocation. An interprofessional team of healthcare workers best manages the disorder.
In general, the long-term prognosis for vasospastic angina is good if patients get adequate treatment. Overall, 75% of patients can be free of myocardial infarct at 5 years. The factor that might independently determine the free infarct survival includes the presence and severity of pre-existing coronary stenosis, the number of vessels with hyperreactivity or spams, and the use of calcium channel blockers. Half of the patients with angina will have persistent symptoms.
Half of the patients with vasospastic angina will have recurring symptoms, even during treatment, within the first 3 years after onset. The development of arrhythmias with the episodes of vasospastic angina has been associated with a poor prognosis, with the main association with ventricular fibrillation and sudden cardiac death.