Management of sequelae of Kawasaki disease in adults

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 Late manifestations of Kawasaki disease (KD) in young adults include: 

1. Cardiomyopathy: Diseases of the heart muscle, where the walls of the heart chambers have become stretched, thickened, or stiff.  

2. Ischemia: An inadequate blood supply to an organ or part of the body, especially the heart muscles 

3. Infarction: Death of tissue resulting from a failure of blood supply, commonly due to obstruction of a blood vessel by a blood clot or narrowing of the blood-vessel channel. 

 

KD poses special management challenges for the adult cardiologist who must recognize the unique features of the cardiovascular lesions in this growing population of patients.

 

In the article signed by John Gordon and Jane Burns have been presented the results of the observations made on a population of 140 adult KD patients enrolled in the San Diego Adult KD Collaborative Study.

 

Coronary artery aneurysms (a bulge in a blood vessel caused by a weakness in the blood vessel wall) resulting from KD in childhood are associated with a high risk of thrombosis (clotting of blood within an artery or vein so that the blood flow is reduced or impeded) and stenosis (abnormal narrowing) at the inlet or outlet of the aneurysm. These aneurysms are often highly calcified and may contain a large thrombus burden that may obscure the true size of the aneurysm.
 

All patients with important coronary artery damage as a result of Kawasaki disease (KD) in childhood should be in follow up care with an adult cardiologist knowledgeable about the unique challenges in managing this patient population.

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Understanding KD lesions: Once the normal arterial wall architecture has been damaged during the acute, inflammatory phase of KD, the affected segment will always have abnormal characteristics. 

The pathologic changes in these lesions can include both:

1. Rapid production of myofibroblasts (cells of wound healing), and,

2. Layering of thrombus (blood clot) along the wall of the aneurysm (bulge in a blood vessel caused by a weakness in the blood vessel wall)

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These processes, alone or in concert, contribute to progressive stenosis (abnormal narrowing) of the vessel lumen. 

Intravascular ultrasound studies have documented the abnormal composition of the arterial wall, which cannot dilate under conditions of increased oxygen demand.

Thus, even though the lumen appears normal by angiography, the calcified wall acts as a functional stenosis (abnormal narrowing).

The highest complication rate is among patients with large aneurysms affecting both the left and right coronary arteries. This is a group that requires frequent monitoring and therapies to prevent thrombosis.

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Gordon JB, Burns JC. Management of sequelae of Kawasaki disease in adults. Global cardiology science & practice. 2017 Oct 31;2017(3).