The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–4, 2014 Copyright Ó 2014 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter
http://dx.doi.org/10.1016/j.jemermed.2013.11.082
Clinical Communications: Adults MYOCARDIAL INFARCTION IN A YOUNG MAN DUE TO CORONARY ARTERY ANEURYSMS AFTER AN UNDIAGNOSED KAWASAKI DISEASE Sumbul Javed Mirza, MD, Khalifa Omar Mohammed, MD, and Fahad Omar Baslaib, MD, FACC Department of Cardiology, Rashid Hospital, Dubai Health Authority, Dubai, United Arab Emirates Reprint Address: Sumbul Javed Mirza, MD, Department of Cardiology, Rashid Hospital, Dubai Health Authority, Oud Metha Road, Dubai, United Arab Emirates
, Abstract—Background: Kawasaki disease usually affects infants and young children. It often goes unrecognized in adults due to varying symptoms and lack of definite diagnostic criteria. Objectives: To describe the potential for acute myocardial infarction as a complication of antecedent Kawasaki Disease (KD). Case Report: We describe a case of a 19-year-old man who presented to the Emergency Department (ED) with an acute myocardial infarction that was subsequently determined to be the result of previously untreated KD. Conclusion: Kawasaki disease can cause coronary complications in a teenager. A high level of suspicion in the ED can help in proper management of these patients. Ó 2014 Elsevier Inc.
in severity and nonradiating. He had no other symptoms. He denied trauma and joint pains, and had no flu-like symptoms. He had no history of alcohol or drug abuse. On examination, he had normal vital signs and an unremarkable cardiovascular and systemic examination. An electrocardiogram was obtained and demonstrated sinus rhythm with biphasic to negative T wave in leads V2V3-V4. Initial cardiac enzymes were elevated (creatine phosphokinase: 435, creatine kinase-MB: 26, and troponin T: 0.17 ng/mL) and indicative of cardiac injury; accordingly, he was admitted to the Cardiology team for evaluation and management. An echocardiogram was obtained and demonstrated a dilatated aortic root of 38 mm and normal mitral and aortic valves. A prominent right coronary artery measuring 8 mm and left anterior descending artery measuring 11 mm could be seen on a short-axis view (Figures 1, 2). Mild septal hypokinesia was also seen, but left ventricular function was preserved. Due to echocardiographic suspicion of coronary artery abnormalities and evidence of cardiac injury, he underwent coronary angiography, which showed segmental and fusiform dilatations of all coronary vessels and a proximal occlusion of the left anterior descending artery (Figures 3, 4). Review of the patient’s medical records showed that he was admitted 2 years prior when he presented with fever, erythematous rash over the extremities, arthralgia, and abdominal pain of 5 days duration. His hospital course was complicated with respiratory failure and hemodynamic compromise requiring mechanical ventilation,
, Keywords—Kawasaki disease (KD); Coronary artery aneurysms (CAA); coronary angiography (CAG)
INTRODUCTION Kawasaki disease is a febrile illness that affects infants and young children under the age of 5 years. The disease was first described by Tomisaku Kawasaki in 1967. It is common in Japan, Taiwan, and Korea, with approximately 112 cases per 100,000 of the population. CASE REPORT A 19-year-old man presented to the Emergency Department (ED) complaining of chest pain of 1-h duration. The pain started while playing football. It was moderate
RECEIVED: 2 December 2012; FINAL SUBMISSION RECEIVED: 26 October 2013; ACCEPTED: 17 November 2013 1
2
S. J. Mirza et al.
Figure 1. Echocardiogram (ECHO): showing dilatated aortic root, left anterior descending (LAD) and left circumflex artery (LCX).
Figure 2. Echocardiogram (ECHO): showing dilatated right coronary artery (RCA).
inotropic support, and wide-spectrum antibiotic therapy for a suspected septic illness. Despite an extensive septic and immunological work-up, which was negative, the clinical suspicion for sepsis and immunological process remained high. Given the coronary aneurysms seen on angiography and the preceding febrile illness, he was diagnosed with an acute myocardial infarction secondary to Kawasaki disease.
young children. The diagnosis in children requires fever for 5 or more days with presence of four out of five clinical manifestations that include erythema of hands and feet, polymorphous exanthema, bilateral conjunctival injection, erythema of the oral and pharyngeal mucosae, and cervical lymphadenopathy (1). The diagnostic criterion is not validated in adults, so it is easy to overlook or misdiagnose the disease. In view of varying clinical and laboratory features, the disease in adults may be reported as complete KD and an incomplete KD (2). Gomard et al. studied 10 cases of adult KD in which four fulfilled the criteria of complete KD, with fever and four clinical features; and six others were reported to have
DISCUSSION Kawasaki disease (KD) is a vasculitic condition of small and medium-sized vessels, usually affecting infants and
Figure 3. Coronary angiography (CAG): showing fusiform dilatation of right coronary artery (RCA) and obliteration of left anterior descending (LAD).
MI after Undiagnosed Kawasaki Disease
3
Figure 4. Coronary angiography (CAG): showing proximal occlusion of left anterior descending (LAD) with faint retrograde filling.
incomplete KD, having only two to three clinical features. In the same series, the diagnosis of KD was made in two cases retrospectively due to myocardial infarction in the presence of coronary aneurysms (2,3). In adults, the cardiac manifestations of KD have been reported in up to 25% of untreated cases and up to 5% in cases treated with immunoglobulins with persistent coronary artery aneurysms (CAAs) as the main cardiac feature (4). Daniels et al. studied 261 angiograms and found definite aneurysms in 5% of angiograms due to previous KD (4,5). The exact pathology of how coronary aneurysms develop as sequelae to the vasculitic process is not known. Some have hypothesized that bacterial infections like Staphylococcal aureus toxins may trigger an inflammatory response, but this has not been established (6). Myocardial ischemia or even myocardial infarction may be seen in KD due to flow abnormalities occurring in the CAAs. An anteroseptal infarction in a young man with a calcified CAA due to childhood KD, and another case of triple vessel disease requiring bypass surgery in a 20-year-old man due to KD have been reported (7–9). Echocardiography is used in infants as a primary tool for evaluation and follow-up of coronary abnormalities and may be helpful in the ED for young adults presenting with symptoms of chest pain and myocardial injury (10,11). Coronary angiography remains a priority once there is evidence of acute coronary syndrome. The role of immunoglobulins in young adults to reduce the cardiac complications is not as established as in children (12). Once coronary aneurysms are formed, long-term use of aspirin is recommended to prevent thrombus formation and future ischemic events (13).
CONCLUSION Undiagnosed KD may present in teenagers with myocardial ischemia due to CAAs. Our case is unusual in its late childhood presentation and early manifestation of cardiac ischemia. Though chest pain in teenagers is usually benign, a high level of suspicion is needed for patients with prior illnesses suggestive or diagnostic of KD, given the potential for significant cardiac complications from untreated KD. REFERENCES 1. Council on Cardiovascular Disease in the Young; Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease; American Heart Association. Diagnostic guidelines for Kawasaki disease. Circulation 2001;103:335–6. 2. Gomard-Mennesson E, Landron C, Dauphin C, et al. Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore) 2010;89:149–58. 3. Brecker SJ, Gray HH, Oldershaw PJ. Coronary artery aneurysms and myocardial infarction: adult sequelae of Kawasaki disease? Br Heart J 1988;59:509–12. 4. Daniels LB, Tjajadi MS, Walford HH, et al. Prevalence of Kawasaki disease in young adults with suspected myocardial ischemia. Circulation 2012;125:2447–53. 5. Pongratz G, Gansser R, Bachmann K, et al. Myocardial infarction in an adult resulting from coronary aneurysms previously documented in childhood after an acute episode of Kawasaki’s disease. Eur Heart J 1994;15:1002–4. 6. Leung DY, Schlievert PM, Meissner HC. The pathogenesis and management of Kawasaki syndrome. Arthritis Rheum 1998;41:1538–47. 7. Mirza I, Gribbin B, Forfar C. A young man with myocardial infarction and a calcified coronary artery aneurysm on chest radiograph. Int J Clin Pract 2004;58:1050–1. 8. Lim AL, Lam HY, Kareem BA, et al. Ayoung patient with history of Kawasaki disease presenting with triple vessel disease. Med J Malaysia 2012;67:219–21. 9. Shaukat N, Ashraf S, Mebewu A, et al. Myocardial infarction in a young adult due to Kawasaki disease. A case report and review of
4 the late cardiological sequelae of Kawasaki disease. Int J Cardiol 1993;39:222–6. 10. Allal J, Vieyres C, Guihem JP, et al. Value of echocardiography in the diagnosis of a complicated form of Kawasaki’s disease. Arch Mal Coeur Vaiss 1989;82:1443–9. 11. Alva-Espinosa C, Diaz-Arauzo A, Mojarro-Rios JA, et al. Kawasaki disease: the echocardiographic diagnosis of coronary aneu-
S. J. Mirza et al. rysms. A report of 2 cases [Spanish]. Arch Inst Cardiol Mex 1995;65:75–7. 12. Wolff AE, Hansen KE, Zakowski L, et al. Acute Kawasaki disease, not just for kids. J Gen Intern Med 2007;22:681–4. 13. Dauphin C, Motreff P, Souteyrand G, et al. Kawasaki disease is also a disease of adults: report of six cases. Arch Mal Coeur Vaiss 2007; 5:439–47.
http://dx.doi.org/10.1016/j.jemermed.2013.11.082
Clinical Communications: Adults MYOCARDIAL INFARCTION IN A YOUNG MAN DUE TO CORONARY ARTERY ANEURYSMS AFTER AN UNDIAGNOSED KAWASAKI DISEASE Sumbul Javed Mirza, MD, Khalifa Omar Mohammed, MD, and Fahad Omar Baslaib, MD, FACC Department of Cardiology, Rashid Hospital, Dubai Health Authority, Dubai, United Arab Emirates Reprint Address: Sumbul Javed Mirza, MD, Department of Cardiology, Rashid Hospital, Dubai Health Authority, Oud Metha Road, Dubai, United Arab Emirates
, Abstract—Background: Kawasaki disease usually affects infants and young children. It often goes unrecognized in adults due to varying symptoms and lack of definite diagnostic criteria. Objectives: To describe the potential for acute myocardial infarction as a complication of antecedent Kawasaki Disease (KD). Case Report: We describe a case of a 19-year-old man who presented to the Emergency Department (ED) with an acute myocardial infarction that was subsequently determined to be the result of previously untreated KD. Conclusion: Kawasaki disease can cause coronary complications in a teenager. A high level of suspicion in the ED can help in proper management of these patients. Ó 2014 Elsevier Inc.
in severity and nonradiating. He had no other symptoms. He denied trauma and joint pains, and had no flu-like symptoms. He had no history of alcohol or drug abuse. On examination, he had normal vital signs and an unremarkable cardiovascular and systemic examination. An electrocardiogram was obtained and demonstrated sinus rhythm with biphasic to negative T wave in leads V2V3-V4. Initial cardiac enzymes were elevated (creatine phosphokinase: 435, creatine kinase-MB: 26, and troponin T: 0.17 ng/mL) and indicative of cardiac injury; accordingly, he was admitted to the Cardiology team for evaluation and management. An echocardiogram was obtained and demonstrated a dilatated aortic root of 38 mm and normal mitral and aortic valves. A prominent right coronary artery measuring 8 mm and left anterior descending artery measuring 11 mm could be seen on a short-axis view (Figures 1, 2). Mild septal hypokinesia was also seen, but left ventricular function was preserved. Due to echocardiographic suspicion of coronary artery abnormalities and evidence of cardiac injury, he underwent coronary angiography, which showed segmental and fusiform dilatations of all coronary vessels and a proximal occlusion of the left anterior descending artery (Figures 3, 4). Review of the patient’s medical records showed that he was admitted 2 years prior when he presented with fever, erythematous rash over the extremities, arthralgia, and abdominal pain of 5 days duration. His hospital course was complicated with respiratory failure and hemodynamic compromise requiring mechanical ventilation,
, Keywords—Kawasaki disease (KD); Coronary artery aneurysms (CAA); coronary angiography (CAG)
INTRODUCTION Kawasaki disease is a febrile illness that affects infants and young children under the age of 5 years. The disease was first described by Tomisaku Kawasaki in 1967. It is common in Japan, Taiwan, and Korea, with approximately 112 cases per 100,000 of the population. CASE REPORT A 19-year-old man presented to the Emergency Department (ED) complaining of chest pain of 1-h duration. The pain started while playing football. It was moderate
RECEIVED: 2 December 2012; FINAL SUBMISSION RECEIVED: 26 October 2013; ACCEPTED: 17 November 2013 1
2
S. J. Mirza et al.
Figure 1. Echocardiogram (ECHO): showing dilatated aortic root, left anterior descending (LAD) and left circumflex artery (LCX).
Figure 2. Echocardiogram (ECHO): showing dilatated right coronary artery (RCA).
inotropic support, and wide-spectrum antibiotic therapy for a suspected septic illness. Despite an extensive septic and immunological work-up, which was negative, the clinical suspicion for sepsis and immunological process remained high. Given the coronary aneurysms seen on angiography and the preceding febrile illness, he was diagnosed with an acute myocardial infarction secondary to Kawasaki disease.
young children. The diagnosis in children requires fever for 5 or more days with presence of four out of five clinical manifestations that include erythema of hands and feet, polymorphous exanthema, bilateral conjunctival injection, erythema of the oral and pharyngeal mucosae, and cervical lymphadenopathy (1). The diagnostic criterion is not validated in adults, so it is easy to overlook or misdiagnose the disease. In view of varying clinical and laboratory features, the disease in adults may be reported as complete KD and an incomplete KD (2). Gomard et al. studied 10 cases of adult KD in which four fulfilled the criteria of complete KD, with fever and four clinical features; and six others were reported to have
DISCUSSION Kawasaki disease (KD) is a vasculitic condition of small and medium-sized vessels, usually affecting infants and
Figure 3. Coronary angiography (CAG): showing fusiform dilatation of right coronary artery (RCA) and obliteration of left anterior descending (LAD).
MI after Undiagnosed Kawasaki Disease
3
Figure 4. Coronary angiography (CAG): showing proximal occlusion of left anterior descending (LAD) with faint retrograde filling.
incomplete KD, having only two to three clinical features. In the same series, the diagnosis of KD was made in two cases retrospectively due to myocardial infarction in the presence of coronary aneurysms (2,3). In adults, the cardiac manifestations of KD have been reported in up to 25% of untreated cases and up to 5% in cases treated with immunoglobulins with persistent coronary artery aneurysms (CAAs) as the main cardiac feature (4). Daniels et al. studied 261 angiograms and found definite aneurysms in 5% of angiograms due to previous KD (4,5). The exact pathology of how coronary aneurysms develop as sequelae to the vasculitic process is not known. Some have hypothesized that bacterial infections like Staphylococcal aureus toxins may trigger an inflammatory response, but this has not been established (6). Myocardial ischemia or even myocardial infarction may be seen in KD due to flow abnormalities occurring in the CAAs. An anteroseptal infarction in a young man with a calcified CAA due to childhood KD, and another case of triple vessel disease requiring bypass surgery in a 20-year-old man due to KD have been reported (7–9). Echocardiography is used in infants as a primary tool for evaluation and follow-up of coronary abnormalities and may be helpful in the ED for young adults presenting with symptoms of chest pain and myocardial injury (10,11). Coronary angiography remains a priority once there is evidence of acute coronary syndrome. The role of immunoglobulins in young adults to reduce the cardiac complications is not as established as in children (12). Once coronary aneurysms are formed, long-term use of aspirin is recommended to prevent thrombus formation and future ischemic events (13).
CONCLUSION Undiagnosed KD may present in teenagers with myocardial ischemia due to CAAs. Our case is unusual in its late childhood presentation and early manifestation of cardiac ischemia. Though chest pain in teenagers is usually benign, a high level of suspicion is needed for patients with prior illnesses suggestive or diagnostic of KD, given the potential for significant cardiac complications from untreated KD. REFERENCES 1. Council on Cardiovascular Disease in the Young; Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease; American Heart Association. Diagnostic guidelines for Kawasaki disease. Circulation 2001;103:335–6. 2. Gomard-Mennesson E, Landron C, Dauphin C, et al. Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore) 2010;89:149–58. 3. Brecker SJ, Gray HH, Oldershaw PJ. Coronary artery aneurysms and myocardial infarction: adult sequelae of Kawasaki disease? Br Heart J 1988;59:509–12. 4. Daniels LB, Tjajadi MS, Walford HH, et al. Prevalence of Kawasaki disease in young adults with suspected myocardial ischemia. Circulation 2012;125:2447–53. 5. Pongratz G, Gansser R, Bachmann K, et al. Myocardial infarction in an adult resulting from coronary aneurysms previously documented in childhood after an acute episode of Kawasaki’s disease. Eur Heart J 1994;15:1002–4. 6. Leung DY, Schlievert PM, Meissner HC. The pathogenesis and management of Kawasaki syndrome. Arthritis Rheum 1998;41:1538–47. 7. Mirza I, Gribbin B, Forfar C. A young man with myocardial infarction and a calcified coronary artery aneurysm on chest radiograph. Int J Clin Pract 2004;58:1050–1. 8. Lim AL, Lam HY, Kareem BA, et al. Ayoung patient with history of Kawasaki disease presenting with triple vessel disease. Med J Malaysia 2012;67:219–21. 9. Shaukat N, Ashraf S, Mebewu A, et al. Myocardial infarction in a young adult due to Kawasaki disease. A case report and review of
4 the late cardiological sequelae of Kawasaki disease. Int J Cardiol 1993;39:222–6. 10. Allal J, Vieyres C, Guihem JP, et al. Value of echocardiography in the diagnosis of a complicated form of Kawasaki’s disease. Arch Mal Coeur Vaiss 1989;82:1443–9. 11. Alva-Espinosa C, Diaz-Arauzo A, Mojarro-Rios JA, et al. Kawasaki disease: the echocardiographic diagnosis of coronary aneu-
S. J. Mirza et al. rysms. A report of 2 cases [Spanish]. Arch Inst Cardiol Mex 1995;65:75–7. 12. Wolff AE, Hansen KE, Zakowski L, et al. Acute Kawasaki disease, not just for kids. J Gen Intern Med 2007;22:681–4. 13. Dauphin C, Motreff P, Souteyrand G, et al. Kawasaki disease is also a disease of adults: report of six cases. Arch Mal Coeur Vaiss 2007; 5:439–47.
