Young 13 Years Old Boy with Vasospastic Angina

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1 Case Report J Jpn Coron Assoc 2013; 19: Young 13 Years Old Boy with Vasospastic Angina Shozo Sueda, 1 Hiroaki Kohno, 1 Naoto Ochi 2 The patient was 13 years old boy. He woke up early in the morning at 5 o clock because of chest oppression on September 10, He had sometimes chest oppression on dynamic exercise since 11 years old. His cardiac echocardiogram showed decreased percent fractional shortening at the near clinic. Long-acting calcium channel antagonist was administered in the morning once a day. Two weeks later, his percent fractional shortening on echocardiogram dramatically improved and also his chest discomfort on both rest and effort decreased about a half level. However he had residual chest oppression on exercise. Cardiac catheterization was performed under no medication at least 48 hours. Coronary angiogram showed no fixed stenosis. Intracoronary administration of acetylcholine caused diffuse narrowing on the right coronary and the left anterior descending artery accompanied by significant ECG changes and his chest pain. Intracoronary administration of ergonovine did not cause spasm or no chest pain/ecg changes. Adding intracoronary administration of acetylcholine after ergonovine injection caused severe spasm on the left anterior descending artery and the left circumflex artery (so called as withered branch), accompanied by severe chest pain/cold sweating and significant ECG changes. He was diagnosed as multiple coronary spastic angina. KEY WORDS: vasospastic angina, young boy, acetylcholine, paradoxical vasoconstriction, endothelial dysfunction I. Intorduction In Japan, coronary artery spasm has three times higher prevalence than Caucasian. 1 3) We recently reported that abnormal coronary artery response on acetylcholine tests increased in Japan. 4) It may be related to the increase of hypertension, diabetes mellitus, and dyslipidemia, so called metabolic syndrome. Japanese young boys and girls now may have endothelial disturbance compared with those of former Japanese due to the life style change. In this article, we reported a 13-years old boy with coronary endothelial dysfunction due to passive smoking and diagnosed as multiple coronary spastic angina. II. Case report He had no likes and dislikes except tomato and had no coronary risk factors, such as hypertension, dyslipidemia, diabetes mellitus, family history of ischemic heart disease, and obesity. His body height and weight were 164 cm and 48 kg. His serum total cholesterol, triglyceride, low-density-lipoprotein cholesterol, high-density-lipoprotein cholesterol, fasting plasma glucose and glycohemoglobin A1c, was 190 mg/dl, 51 mg/dl, 67 mg/dl, 91 mg/dl, 87 mg/dl, and 5.3%, respectively. He had been 1 Department of Cardiology, Ehime Niihama Prefectural Hospital, Hongou, Niihama City, Ehime, , Japan, 2 Department of Cardiology, Ochi Clinic Received July 4, 2012; Accepted September 14, 2012 doi: /jcoron very well until 11 years old. His father smoked >20 cigarettes/ day for 20 years and his mother also smoked cigarettes/ day for 18 years at home or at work. His both parents had history of smoking since 20 years old and were now active current smoker. He had experienced chest oppression sometimes on exercise since 11 years old. His chest oppression was disclosed often in the morning, in the winter, sometimes during exercise for 5 to 10 minutes. He recently experienced less endurance on exercise. He woke up early in the morning at 5 o clock because of severe chest pain for 10 minutes with slight cold sweating on September 10, He admitted to the near clinic. ECG, though showing inferior atrial rhythm and chest X ray finding were within normal limits, as shown in Fig. 1. However, his percent fractional shortening on cardiac echocardiogram decreased and wall motion had diffusely slight hypokinetic, as shown in Fig. 2-a. Long-acting calcium antagonist [diltiazem R (100) mg/ day] was administered in the morning once a day. A few days after taking long-acting calcium antagonist, his chest discomfort both diminished about in a half grade and in frequency. Two weeks later, his percent fractional shortening on cardiac echocardiogram improved from 28% to 41% within normal range, as shown in Fig. 2-b. Because of residual chest discomfort/chest oppression on both rest and effort, cardiac catheterization was performed in our hospital. Before cardiac catheterization, treadmill exercise test was done. He stopped exercise due to severe dyspnea without ischemic ECG change and his maximal heart rate was 134/min on Bruce s stage 4. As shown in Fig. 3, his 355

2 Fig. 1 Chest X ray and ECG findings. Fig. 2 Echocardiogram before and after the administration of long-acting calcium channel antagonist for 14 days. a: Echocardiogram before administration of long-acting calcium channel antagonist. b: Echocardiogram after 2 weeks administration of long-acting calcium channel antagonist, showing evident improvement of percent fractional shortening from 28% to 41%. coronary arteriogram showed almost normal findings. Spasm provocation test was performed under no medication at least 48 hours cessation of long-acting calcium channel antagonist. Intracoronary injection of acetylcholine caused transient luminal narrowing on the right coronary and the left anterior descending artery accompanied by chest pain and ischemic ECG changes, as shown in Fig. 4, 5. Coronary spasm was not provoked after intracoronary administration of ergonovine on both coronary arteries. Adding intracoronary injection of acetylcholine after ergonovine test caused severe spasms on the left anterior descending artery and the left circumflex artery and the right coronary artery accompanied by severe chest pain, cold sweating and ischemic ECG changes, as shown in Fig After intracoronary injection of nitrate, his coronary artery dilated slightly compared 356

3 Fig. 3 Coronary arteriograms before and after nitrate. a: Control (LCA) b: Control (RCA) c: After nitrate (LCA) d: After nitrate (RCA) Fig. 4 Spasm provocation tests in the left coronary artery. a: After 100 μg acetylcholine b: After 64 μg ergonovine c: Adding intracoronary injection of 100 μg acetylcholine after 64 μg ergonovine d: After nitrate 357

4 Fig. 5 Spasm provocation tests in the right coronary artery. a: After 50 μg acetylcholine b: After 40 μg ergonovine c: Adding intracoronary injection of 50 μg acetylcholine after 40 μg ergonovine d: After nitrate Fig. 6 ECG changes during spasm provocation tests. a: Control b: After 100 μg acetylcholine (LCA) c: After 64 μg ergonovine (LCA) d: Adding intracoronary injection of 100 μg acetylcholine after 64 μg ergonovine (LCA) e: After 50 μg acetylcholine (RCA) f: After 40 μg ergonovine (RCA) g: Adding intracoronary injection of 50 μg acetylcholine after 40 μg ergonovine (RCA) 358

5 with that of control state, as shown in Fig. 3. Finally, he diagnosed as multiple coronary spastic angina. After the medications including two calcium channel antagonists (diltiazem R 100 mg and nifedipine CR 20 mg) once a day, minor tranqulizer (lorazepam 2 mg/day) and ethyl icosapentate (Epadel S 1800 mg/day), his chest symptom gradually diminished over one year. III. Discussion In this patient, left ventricular wall motion on echocardiogram improved after the administration of a long-acting calcium channel antagonist. Multiple spasms may cause transient left ventricular wall motion abnormality and heart failure. 5 7) The echocardiogram was very useful to detect abnormal finding in a young boy who complained chest pain on both rest and effort. Irrespective of normal ECG finding and chest X ray finding, cardiac echocardiogram should be performed in even younger patients who had chest pain or chest oppression. His coronary endothelium showed abnormal response during spasm provocation tests. If having normal coronary endothelium, coronary artery not only dilate but increase coronary flow after the administration of acetylcholine. 8, 9) However, this patient showed abnormal vasoconstriction after acetylcholine injection on the right and the left anterior descending coronary arteries. We could not obtain the typical provoked spasm during acetylcholine or ergonovine test alone. Adding intracoronary injection of acetylcholine after ergonovine test 10) caused severe spasms on all 3 coronary arteries (so called as withered branch), accompanied by severe chest pain, cold sweating and ischemic ECG changes. We speculate that the mechanism of provoked severe spasm adding intracoronary injection of acetylcholine after ergonovine test is a dual vasoconstrictive effect in which ergonovine acts on smooth muscle via the serotonergic receptor and acetylcholine acts on the endothelium and smooth muscle via the muscarinergic receptor. However, this may be related to the low disease activity, less endothelial disturbance compared with the older patients, residual effect of long-acting calcium channel antagonist irrespective of 48 hours cessation, or limitations of pharmacologic agents. This patient have coronary endothelial disturbance even at 13 years old. Moreover, intracoronary injection of nitrate dilated coronary arteries slight compared with that of control state. The cause of endothelial disturbance is unclear. However, his parent s habitual smoking may led to their son s passive smoking and may have caused coronary endothelial disturbance. Passive smoking intimately linked the association with dose-related impairment of endothelium-dependent dilatation in young healthy adults, suggesting earlier arterial damage. 11) Sumida, et al. reported that coronary endothelial dysfunction may occur diffusely in passive smokers as well as in active smokers in even middle aged women. 12) Smoking is the most important risk factor for coronary artery spasm. 13, 14) We strictly suggested the stop smoking of his both parents and avoid habitual smoking in this patient in the future forever. If chest symptom recurred in the future, it would be necessary to dose up and administer another long-acting calcium channel antagonist or select other approaches in this case. 15) In the near future, we may encounter younger people less than 10 or 20 years old with typical coronary spastic angina in the clinical ground in Japan. 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