Chest pain is often benign in children and adolescents, but it is crucial to recognise cardiac reasons because they carry a significant risk of developing into life-threatening illnesses. To rule out cardiac causes, chest pain characteristics, cardiac enzyme, and electrocardiographic results can be employed. Reference Chen, Duan, Li, Yang, Jiao, Sun and Jin1 A cardiac cause, such as myocarditis, myocardial ischemia, or structural heart disease, is suspected due to the chest discomfort radiating to the left arm and jaw, the sensation of pressure on the chest, and the concomitant symptoms. Reference Collins, Griksaitis and Legg2 Vasospastic angina is a very rare and a difficult-to-diagnose cardiac cause of adolescent chest pain. We report of a 17-year-old Afghan refugee child who had 6 months of myocarditis treatment before being diagnosed with vasospastic angina after experiencing ongoing chest pain.
Case report
A 17-year-old male was referred to our unit with myocarditis suspicion because of chest pain, an increase in cardiac enzymes, and abnormal electrocardiographic results. The patient was an Afghan refugee. His medical history revealed that he had presented with chest pain two more times within a year, that his tests showed elevated cardiac enzymes. He had started treatment for myocarditis with aspirin and metoprolol, and that he had been taking these medications for about 5 months. The characteristics of chest pain was pressure on his chest, no change with respiration nor position, radiating in his left shoulder, left arm, and jaw. The chest pain was discovered to be predominantly in the evening and to last for around 5 minutes when at rest. The patient also did not have any significant family medical history, such as hyperlipidemia or early myocardial infarction. He had never had a fever for Kawasaki disease, any other systemic disease, been overweight, smoked, substance use, or physical inactivity.
Upon examination, he had a heart rate of 60 bpm, a respiratory rate of 20 bpm, and blood pressure of 119/69 mmHg. A grade 2/6 short systolic murmur was audible on the mitral region during cardiac auscultation. On blood examination, N-terminal pro-B-type natriuretic peptide 419 ng/L, hs troponin T level 121 ng/L (0–14), HDL 36 mg/dL (40–85), creatine kinase mb 2,5 μg/L (0–4,94), white blood cell count 5,5 × 109/L (4–10), and CRP 0,7 mg/L (<5) were detected. The erythrocyte sedimentation rate, kidney and liver function tests, and other laboratory parameters were within the normal range. Chest X-ray was unremarkable. Electrocardiogram revealed T wave inversion and pathologic Q wave in the inferior leads, R dominance and ST elevation in the right precordial leads with a rate of 56 bpm. Echocardiography revealed posterolateral wall motion abnormality, mild left ventricular posterior wall thickness (PW:12 mm + 2 z score, S:12 mm + 1,5 z score) with a low left ventricular ejection fraction (50%), and mild mitral regurgitation. We first assumed that the results of the electrocardiographic and echocardiogram indicated recurrent myocarditis, and we continued to provide a combination of metoprolol and acetylsalicylic acid as treatment. On the fourth day of his hospitalisation, we had a cardiac MRI for myocarditis. Cardiac MRI revealed an increase in thickness of anteroseptal, inferolateral, and inferoseptal of left ventricle. Subepicardial hypoperfusion on early postcontrast images and subepicardial contrast enhancement on delayed post-contrast images was observed in the left ventricle basal and midventricular inferoseptal, inferior and lateral walls suitable for left circumflex artery and right coronary artery areas (Fig. 1). Hypokinesia was also observed at sites corresponding to this region. Findings were consistent with subacute infarct. Selective coronary angiography was planned immediately with the suspicion of infarct in cardiac MRI. Selective coronary angiography showed vasospasm in the distal branch of circumflex and mid right coronary artery (Fig. 2) After the administration of glyceryl trinitrate, opening of vasospasm was observed. The constellation of findings described above established a diagnosis of vasospastic angina. Previous medications were discontinued, and the combination of nitrate and calcium blocker treatment was started. Cardiac enzymes and other laboratory results for the patient were back to normal. Throughout the patient’s hospitalisation, there were no angina attacks after maximising doses. He was discharged after 12 days with diltiazem (180 mg daily), isosorbide mononitrate (20 mg daily), statin, and physical activity restriction recommendations. Four months after being discharged, the patient reported no symptoms. Since infarct repair began after 5 months, an MRI was scheduled to be done at a later time.
Figure 1. Images from selective coronary angiography showing a spasm in the coronary arteries and how it improved after being treated with intracoronary nitrate. ( a ) Spontaneous vasospasm was seen in the right coronary artery in the left anterior oblique position. ( b ) The spasm resolves after intracoronary nitrate administration. ( c ) A left coronary angiogram’s anterior-posterior (AP) cranial view demonstrating circumflex artery vasospasm. ( d ) The spasm resolves after intracoronary nitrate administration.
Figure 2. ( a ) Short-axis early postcontrast images shows subepicardial hypoperfusion in Lcx and RCA territory. ( b ) Contrast enhancement suitable for infarct observed on short-axis delayed postcontrast images. ( c ) T1 map.
Discussion
Vasospastic angina (previously known as Prinzmental or variant angina) is coronary artery spasm characterised by anginal symptoms at rest, with transient ST segment elevation or depression and angina that is responsive to nitrates during the time of the episode. Reference Beltrame, Crea and Kaski3 It is thought that coronary arteries are more sensitive to vasoconstrictor factors and the pathogenesis is influenced by hyperactive vascular smooth muscles, abnormalities in the autonomic nervous system, magnesium deficiency, microvascular dysfunction, and endothelial dysfunction. Reference Beltrame, Crea and Kaski3-Reference Picard, Sayah, Spagnoli, Adjedj and Varenne5 These factors may cause localised or widespread spasm, which can lead to dynamic coronary blockage.
The clinical presentation is usually with anginal symptoms; however, it may cause syncope, myocardial infarction, arrhythmias (ventricular fibrillation or AV block), or sudden cardiac death if it persists. Reference Tandon, Mosebach, Kumar and Joshi4-Reference Stern and Bayes de Luna7 Chest pain is characterised by chronic and recurrent bouts and responds favourably to nitrates. Chest pain can last for more than a year, with the longest recorded period being 36 months. Reference Sueda6 Chest pain is often seen at rest (more so at night or in the morning) and feels like pressure in the chest that spreads to the arm, neck, upper extremities, and back, which does not change with breathing. Reference Picard, Sayah, Spagnoli, Adjedj and Varenne5 Chest pain may accompany nausea, palpitations, shortness of breath, and sweating during the anginal episode. Reference Picard, Sayah, Spagnoli, Adjedj and Varenne5 For about a year, our patient complained of chest pain that was characterised by pressure on the chest, and spreading to the left shoulder, left arm, and jaw, experienced chest pain during rest and in the evening. He visited the emergency room three times with this complaint.
A significant risk factor for coronary spasm is smoking. Hyperventilation, the cold, exercise, stress, magnesium deficit, and several medications (triptans, ephedrine, beta blockers, ergonovine, and high doses of aspirin) are also known to trigger cardiac vasospasm. Reference Tandon, Mosebach, Kumar and Joshi4,Reference Picard, Sayah, Spagnoli, Adjedj and Varenne5 Our patient had never smoked before, and we believe that his situation as a refugee and his living environment may have been a trigger as a stress factor. Release of vasopressor neurotransmitters such noradrenaline, platelet activation, and serotonin, and abnormal autonomic nervous system function leads to vasospasm in response to stress. 8 Ethnic differences are another risk factor, with Asian groups having a higher incidence. Reference Picard, Sayah, Spagnoli, Adjedj and Varenne5
In the absence of anginal symptoms in vasospastic angina, physical examination, electrocardiography, and laboratory findings are unremarkable. Depending on where the damaged vasospastic coronary artery is located and how long the episodes last, pathologic findings are seen in the electrocardiography. The presence of a brief ST depression or elevation and a negative u wave during vasospastic angina is diagnostic. Reference Beltrame, Crea and Kaski3 Selective coronary angiography may be planned on high suspicion of chest pain and spasm may be induced by provocative tests with ergonovine and acetylcholine. Reference Sueda6 Because of the patient’s young age, the prevalence of myocarditis, the difficulty in detecting vasospastic angina, the chronic nature of the condition and findings of echocardiography and electrocardiography, we believed that the patient was misdiagnosed with the diagnosis of recurrent myocarditis that he may have had complaints related to multivessel coronary spasm in his previous admissions. Our patient’s incorrect diagnosis of myocarditis resulted in him being treated with beta-blockers, which are known to make symptoms worse by causing a stronger vasoconstrictive reaction in cases of vasospastic angina. Selective coronary angiography showed spontaneous coronary spasm and the spasm disappeared with intracoronary nitroglycerine.
Both pharmacological therapy and lifestyle adjustments should be used to control the vasospastic angina. Triggers (stress, alcohol, and cold) should be avoided, and smoking should be discontinued. Improved anginal symptoms and relief from vasospasm are achieved with calcium canal blocker (diltiazem, amlodipine, or long acting nifedipine) and nitrate (isosorbide mononitrate). Statins may be chosen due to their effects on acetylcholine, one of the causes of coronary spasm, as well as their impact on endothelial activities. Reference Stern and Bayes de Luna7 In refractory cases with persistent symptoms, where Mg deficiency may also be one of the reasons, long-term Mg support may be given. Reference Stern and Bayes de Luna7 Class I recommendations for calcium channel blockers, class IIa recommendations for long-acting nitrates, and class IIb recommendations for statin therapy are made in the 2013 Japanese Circulatory Society Guidelines for VSA. 8 Following the recommended protocol, we gave our patient these three medications.
In conclusion, the patient we are presenting had already received a diagnosis of myocarditis about a year before, but the chest pain persisted. Although coronary artery disease in teenagers is extremely rare, it is crucial to maintain a high threshold for suspicion when chest pain persists and there are pathologic echocardiography and electrocardiography signs indicative of cardiac conditions. The results of the patient’s cardiac MRI were also very useful in confirming the diagnosis, and we were able to establish vasospastic angina by selective coronary angiography based on the MRI infarct findings. Angina episodes resolved with calcium channel blocker and nitrate treatment.
Ethical standards
The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human medical regulations and with Helsinki Declaration of 1975, as revised in 2008.
Informed consent
Written informed consent was obtained from all patient’s parents.
