What is the prognosis of Kawasaki disease?

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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Answer

Patients who do not develop CAAs recover fully. In those who develop CAAs, the severity of aneurysms determines the prognosis. More than half of all aneurysms resolve within 2 years. Endovascular ultrasonography has shown that, even when aneurysms resolve, marked intimal thickening is present in some. Vessel flow may be abnormal. These patients may have an increased risk of premature coronary atherosclerotic disease. [53]

Coronary artery bypass grafting (CABG) has been required in some children with severe perfusion deficits. Follow-up of children and adolescents 20-25 years after CABG has shown a 95% survival rate, though some patients have required repeat CABG or percutaneous coronary intervention. [54] Transplant has been performed in some children who had large aneurysms in vessels not amenable to bypass.

The greatest risk of cardiac damage occurs in children younger than 1 year and in older children, which may be related to the incomplete presentation often seen in the older age group that leads to delay in diagnosis and, consequently, treatment. Providers must be aware of both the complete and incomplete presentations of this disease to ensure timely diagnosis and treatment. Therapy with IVIG should be started within 10 days, and ideally within 7 days, of fever onset to prevent cardiac complications. [55]

Recurrence of KD is unusual: the recurrence rate in Japan is 3%, and it is approximately 1% in North America. Most relapses occur within 2 years from the initial episode. The highest incidence is in younger children and those who had cardiac sequelae from the initial episode.

The mortality from KD is low at less than 0.5%, with the highest risk in the first year after disease onset. Death is typically due to acute MI in the setting of giant aneurysms. Aneurysm rupture is rare and typically occurs within the first few months after the illness began. In the first week, severe myocarditis leading to hemodynamic instability or arrhythmias can occur. [1]


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