Long-Term Management of Kawasaki Disease

Age: 19 years

Gender: Female

Occupation: Student

Working diagnosis: Kawasaki disease


The patient presented acutely with Kawasaki disease at the age of 20 months and was treated with aspirin. At age 23 months, she experienced chest pain with ST elevation seen in ECG leads II, III, and aVF, and had an inferior myocardial infarction. The infarction was followed by mild mitral regurgitation and impaired LV systolic function.

At 3 years of age, coronary angiography showed complete occlusion of the right coronary artery, with a giant aneurysm at the bifurcation of the left coronary artery. Repeat coronary angiography at age 10 showed in addition segmental stenosis of the left circumflex artery, though the patient was clinically stable. Segmental stenosis, which is a typical characteristic lesion as sequelae after Kawasaki disease, implies the development of several new small vessels representing recanalization after coronary artery occlusion.

By age 15, the patient had signs of heart failure due to ischemic cardiomyopathy. LV end-diastolic volume was increased. Multifocal premature ventricular contractions, as well as nonsustained ventricular tachycardia, were noted. Within a year she underwent coronary artery bypass grafting to the left anterior descending artery using the left internal mammary artery concomitantly with conventional reduction of the mitral valve annulus (by means of the so-called Kay-Reed method). The patient was said to be in NYHA class III in the early postoperative period. Warfarin was meticulously given for 1 year after surgery, in addition to aspirin.

One year after bypass surgery her functional class had improved to NYHA class II. A beta-blocker was added to her medications. She had no chest pain. Catheterization showed that the LV end-diastolic volume had decreased. The left internal mammary graft remained patent, but was extremely slender (“string sign”).

Comments: Kawasaki disease is an infantile acute febrile mucocutaneous lymph node syndrome. The inflammatory process can cause arteritis and, in a large proportion of patients, involves the coronary arteries in particular. This frequently leads to the formation of large aneurysms in the proximal coronaries, eventually with calcification of the wall of the aneurysm. Giant aneurysms (>8 mm) are likely to occlude with thrombosis, and this can lead to acute myocardial infarction. Acute myocardial infarction occasionally causes sudden death or impairs LV function, a major determinant of outcome in this population.

In this patient, mitral regurgitation worsened the LV dilatation and vice versa. Although surgical mitral valvuloplasty improved the regurgitation, LV dysfunction persisted.

In children undergoing coronary revascularization for Kawasaki disease, it is not rare for the bypass graft to become narrow and atretic. This likely reflects competition from native coronary flow. This phenomenon indicates the importance of a justifiable indication for coronary artery bypass grafting. It should be performed only when the native flow is severely impaired.


Mild exertional dyspnea. The patient was finding it more difficult to keep up with the demands of her full-time university education.

NYHA class: II


  • Aspirin 200 mg daily

  • Furosemide 40 mg daily

  • Spironolactone 50 mg twice daily

  • Metoprolol 25 mg twice daily

Comments: Today, anticoagulation with warfarin is routinely recommended for patients with Kawasaki disease, since giant aneurysms may become thrombosed, often within the first year after the acute phase of the disease. This was not known in the past, however, and this is the reason that this patient was not on warfarin at the time.


  • BP 104/60 mm Hg, HR 60 bpm, oxygen saturation 95%

  • Height 159 cm, weight 41 kg, BSA 1.35 m 2

  • Neck veins: Normal waveform, not elevated

  • Lungs/chest: Clear

  • Heart: The heart had a regular rhythm. The second heart sound was normally split. There was a grade 2/6 holosystolic murmur at the apex. Peripheral pulses were normal.

  • Abdomen: Liver was not palpable.

  • Extremities: Normal, no edema was present

Comments: The murmur is consistent with mitral regurgitation.


Hemoglobin 13.5 g/dL (11.5–15.0)
Hematocrit/PCV 39% (36–46)
Sodium 140 mmol/L (134–145)
Potassium 3.5 mmol/L (3.5–5.2)
Creatinine 0.6 mg/dL (0.6–1.2)
Blood urea nitrogen 2.8 mg/dL (2.5–6.5)


Protein 6.5 g/dL (6.5–8.2)
Albumin 3.9 g/dL (3.6–5.5)
Total bilirubin 1.0 mg/dL (0.2–1.2)
AST 5 U/L (0–40)
ALT 7 U/L (0–35)
LDH 102 U/L (100–225)
CPK 34 U/L (30–200)
T-chol 130 mg/dL (130–220)
TG 38 mg/dL (30–130)
hANP 87 pg/mL (<40)
BNP 84.1 pg/mL (<20.0)


Figure 79-1



  • Heart rate: 60 bpm

  • QRS axis: −10°

  • QRS duration: 80 msec

  • Sinus rhythm. Leftward axis. Probable prior posterolateral infarction. T-wave inversion in the anterolateral leads.


Figure 79-2

Posteroanterior projection.


Cardiothoracic ratio: 47%

Previous sternotomy. Normal cardiac size. There was a heavily calcified left coronary artery aneurysm, seen in cardiac silhouette.

Comments: The calcification seen is in the wall of the aneurysmal left coronary artery, indicative of a giant coronary aneurysm.

Another round and calcified lesion is vaguely seen around the third sternal wire from the top, which outlines the aneurysmal right coronary artery. This lesion is not as vivid as the one from the left coronary artery, probably because the right coronary one was more hidden between the sternum and vertebrae.


Exercise protocol: Modified Bruce
Duration (min:sec): 5:05
Reason for stopping: Dyspnea
ECG changes: Multifocal PVCs

Rest Peak
Heart rate (bpm): 60 175
Percent of age-predicted max HR: 87
O 2 saturation (%): 95 97
Blood pressure (mm Hg): 104/60 122/74
Double product: 22,400
Peak V o 2 (mL/kg/min): 15.3
Percent predicted (%): 31
Ve/V co 2 : 48
Metabolic equivalents: 4.2

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Sep 11, 2019 | Posted by in CARDIOLOGY | Comments Off on Long-Term Management of Kawasaki Disease
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