Four Cases of Kawasaki Syndrome Complicated With Myocarditis

Abstract

yocarditis frequently occurs in the acute phase of Kawasaki syndrome (KS) and may be transient, 1,2 but there are a few cases of severe myocarditis. [3][4][5][6][7][8] We present 4 cases of myocarditis in KS that required additional catecholamine treatment for severe left ventricular dysfunction (LVD).Case Reports (Table 1, Fig 1) Patient 1 (Fig 1A) A 7-year-old-girl (height 115 cm, body weight 20 kg) was admitted to a general hospital because of 4 days of persistent fever and right cervical adenopathy (white blood cell count (WBC) 22,600 / l, Creactive protein (CRP) 10 mg/dl). Rash and edema of the hands and feet developed on the 5th day of illness. The echocardiography revealed a normal value of ejection fraction (EF) (69%). On the 8th day, the fever was persisting and she was referred to us. Physical examination revealed a cardiac gallop rhythm, nasal alar breathing, eyelid edema, bilateral conjunctival congestion, strawberry tongue, right cervical adenopathy and edema of the hands and feet. The liver was palpable at 7 cm below the right costal margin. The results of laboratory tests were: WBC 18,700 / l, hemoglobin (Hb) concentration 9.4 g/dl, hematocrit (Hct) 32.4%, platelets 18-10 4 / l, CRP 24 mg/dl, mild liver dysfunction (aspartate aminotransferase (AST) 51 IU/L, alanine aminotransferase (ALT) 81 IU/L), hyponatremia (129 mmol/L), and hypoproteinemia (total protein 4.6 g/dl, albumin 2.0 g/dl). Hypotension became obvious with pulmonary edema and a decreased EF (44%), which were noted on chest X-ray and echocardiography at admission. With a diagnosis of KS with myocarditis, we started treatment with aspirin (30 mg·kg -1 ·day -1 ), intravenous immunoglobulin (IVIG: 2 g/kg for 24 h) and dopamine (3 g· kg -1 ·min -1 ). Despite the medication, the hypotension did not improve and sufficient urinary output was not obtained. Dobutamine (3 g·kg -1 ·min -1 ) and diuretics were added and her hypotension and oliguria improved. The fever subsided on the 10th day, but re-emerged the next day. Additional IVIG (2 g/kg for 24 h) was administered and the fever subsided on the 12th day. Giant coronary aneurysms were observed during convalescence.Patient 2 (Fig 1B) A 6-year-old-girl (height 117 cm, body weight 21 kg) was admitted to Kagoshima Medical Association Hospital because of 3 days of persistent fever and right cervical adenopathy (WBC 30,700 / l, CRP 23 mg/dl). Altered consciousness was observed on the 4th day of illness. Bilateral conjunctival congestion, strawberry tongue and rash developed on the 5th day (WBC 15,800 / l, CRP 23 mg/dl). Echocardiography revealed normal coronary arteries and normal EF (64%). With the diagnosis of KS, IVIG (2 g/kg for 24 h) was started. Because of liver dysfunction, flurbiprofen (4 mg · kg -1 · day -1 ) instead of aspirin was also started. Although the fever subsided on the 6th day, the serum level of CRP increased (26 mg/dl) and EF decreased (48%). Hypotension and oliguria were also observed together with increases in blood urea nitrogen (BUN) (55 mg/dl) and creatinine (1.3 mg/dl). ...

Intravenous Immunoglobulin Nonresponsive Symptomatic Myocarditis during the Acute Stage of Incomplete Kawasaki Disease

“…The following are the common findings between our case and other patients with myocarditis 5,6) : 1) the patients with symptomatic myocarditis were older than 6 years, and 2) cervical lymphadenopathy and fever were the only initial features of KD.…”

“…In comparison, symptomatic myocarditis with congestive heart failure and inotropic treatment has been reported [5][6][7] . It is known that IVIG treatment reduced the prevalence of coronary disease and improved myocardial function in patients with KD to accompany myocarditis with clinical or subclinical left ventricular dysfunction 6,7) . Although IVIG therapy improved myocardial function, it may sometimes induce hypotension 8) .…”

Kawasaki Disease, Others Heart Injuries, Not Only Coronary Arteritis

“…Findings from the histological viewpoint have been diverse, and among these are found the following: hypertrophy; myocyte degeneration; fibrosis; infiltration of lymphocytes and plasma cells, and disarray of myocardial fibers and, in adults after KD, we find the description of kariomyocyte dropout and diffuse fibrosis not in the watershed distribution of the epicardial coronary arteries; it has been supposed that the fibrosis is due to ischemic damage because of microinfarcts or kariomyocyte inflammatory damage. Mycocarditis in KD is characterized by inflammatory cell infiltration from the coronary arteries to the myocardiac instersticium, and necrosis of the myocardium is infrequently observed (Yoshikawa, 2006); on the other hand, diffuse myocarditis followed by myocardial fibrosis can lead to diastolic dysfunction, and in the acute phase of KD, measurement of ventricular flow has revealed an abnormal relaxation and has been associated with increased levels of type B naturiuretic peptide. By other hand Myocarditis is recognized as a component on the half of KD patients, thought the left ventricular dysfunction ; Ajami, et al, evaluated myocardial function in patient during acute phase of illnees using the myocardial performance index also known as a Tei index, and they assessed the Tei index, the ejection fraction, shortening fraction and valvular regurgitation, pericardial effusion or coronary arterial involvement, they compared the changes in acute phase compared with pos-treatment data, confirming left ventricular dysfunction these index measures combined systolic and diastolic function is a simple sensitive and accurate tool for estimating myocardial function (Ajami 2010;Kurotobi, 2005).…”

“…By these methods and by echocardiography, it has been observed that there is improvement in the myocarditis after treatment. This situation probably is related with the fact that myocarditis has been considered as a transitory event, without its being studied more profoundly (MacMorrow, 2001;Newburger, 2004;Yoshikawa, 2006).…”

Different subgroups regarding the absence of rescue therapy in intravenous immunoglobulin-resistant Kawasaki disease

“…I excluded 29 patients who received concomitant IVIG and anti-inflammatory therapy, four patients who had developed CAL before the start of therapy, and seven patients with disease recurrence and one patient who developed left ventricular dysfunction because of their greater prevalence of CAL [9,10,11].…”