Kawasaki Syndrome

Kawasaki disease is the most common cause of acquired heart disease. and this question will be likely on your Peds exam. it is a form of vasculitis that affects not only the coronary arteries, but other arteries as well, such as the brachial and carotid arteries.

It is called “Kawasaki Disease” because it is named after the person who discovered it, Dr. Kawasaki (not the motorcycle).

For the purpose of the Peds test, it is an inflammation of the coronary artery that leads to aneurysm of the left main coronary artery in particular.

For some reason that is not understood, it is far more common in Asians than Caucasians, by a about 3:1.

Myocardial infarction is the most common cause of death with this disease, and usually happens in the first few months of life.

The acute phase consists of fever and infection of some sort that particularly affects the endothelium of the coronary artery and other arteries, but can lead to thrombosis, aneurism, myocarditis, abnormalities of the conduction system, pericarditis, ischemic heart disease, valvular stenosis, and aneurysmal rupture.

This disease actually affects the entire cardiovascular system, rather than just one coronary artery.

After a few months, the body will “correct” the disease by overlaying the diseased arterial endothelium with scar tissue.

In the end, most people affected by this disease eventually die from acute coronary stenosis.

Aneurisms of the coronary artery usually appear within seven days of the onset of fever, and are considered severe if the aneurism is more than 8mm in diameter.

Myocarditis is the most common abnormality seen with these patients. It is important to look for valvular dysfunction, especially regurgitation.

Echocardiography should be done in order to provide a baseline measurement of the coronary artery (and to look for other cardiac abnormalities mentioned above), and done serially to follow up. Try to visualize all of the coronary arteries and measure if necessary to evaluate their size. The most common sites of aneurisms involve the proximal segments of the coronary arteries.

The coronary artery is abnormal if it measures more than 3mm in diameter, and is considered extremely severe if it measures 8mm in diameter.

Treatment involves immune globulin therapy, aspirin, reduction of fever, and steroids.

Palliative treatment is really the only effective treatment/


Ken Heiden RDCS