Changes in causes of sudden deaths by decade in patients with coronary arterial lesions due to Kawasaki disease

Abstract

Over a 25-year period, we encountered 12 patients who died suddenly with coronary arterial lesions due to Kawasaki disease. We report their clinical course, and analyze the happenings of their deaths. Of the 12 patients, 10 were dead on arrival at hospital. Their age at death ranged from 13 months to 27 years, with a median of 16 years, and the interval from the onset of Kawasaki disease to death ranged from 2 months to 24 years. In 4 patients, death was found to be due to myocardial infarction, while in the remaining 8, it could not be determined. In 7 patients, coronary angiograms obtained less than 4 months after the acute onset of Kawasaki disease showed lesions bilaterally, most being giant aneurysms. Myocardial infarction had occurred in 6 patients prior to their death. In 1 patient of the late 1970s, who collapsed after running, cardiac sequels had not been suspected prior to autopsy. During the 1980s, 3 infants with bilateral giant aneurysms died within a year of the initial onset of Kawasaki disease, with acute myocardial infarction being the cause in 2 of them. In the late 1990s, and the 2000s, 5 patients died suddenly with left ventricular dysfunction, their ejection fractions being less than 40 percent more than 20 years after the initial onset of Kawasaki disease. Prior to their sudden deaths, they had had no cardiac events for many years, but had suffered previous myocardial infarctions. Multifocal premature ventricular contractions, and non-sustained ventricular tachycardia, are probable risk factors in such patients. Careful follow-up, checking for ventricular arrhythmia, is needed to prevent sudden death in patients suffering left ventricular dysfunction in the setting of Kawasaki disease.

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

“…Premature ventricular contractions and ventricular tachycardia have been taken as clinical markers of underlying myocardial damage and as potential predictors of long-term consequences, including late sudden death. 272,301 In particular, after MI, the incidence of ventricular tachycardia can be increased. Patients who have sustained severe myocardial injury or infarction may benefit from extended rhythm surveillance (Holter or other long-term electrocardiographic monitoring) to best assess the need for specific antiarrhythmic therapy.…”

When Children With Kawasaki Disease Grow Up

“…Long-term complications included stenosis, myocardial infarction, and death. Other series have reported both symptomatic and asymptomatic coronary artery occlusion in regions of previous aneurysms(22,23), coronary artery stenosis(24), coronary artery calcification(25), diastolic dysfunction(26), and sudden death(27). One study of 562 Japanese KD patients with known coronary artery lesions documented the appearance of new aneurysms 2-19 years after disease onset in 15 patients (3%)(28).…”

“…Increasing numbers of deaths that are attributed to left ventricular dysfunction and presumed ventricular arrhythmias are being reported in young adults from Japan with antecedent KD(27). Whether the diffuse fibrosis is a consequence of ischemic injury from microinfarcts or inflammatory cardiomyocyte injury or both is unknown.…”

Pediatric cardiac emergencies: Children are not small adults

“…[46] In 15 to 25% of untreated children, coronary artery aneurysms and progressive stenosis develop which can lead to significant morbidity and mortality from ischemic heart disease, myocardial infarction or sudden death. [47–49] The disease is diagnosed based on clinical criteria of at least five days of fever and at least four of the main clinical features including: polymorphous rash; limbic sparing non exudative conjunctivitis; mucocutaneous changes (e.g. strawberry tongue, dry cracked lips); cervical lymphadenopathy; and extremity changes with acute edema of the hands and feet or palmar erythema and after two to three weeks of illness periungual desquamation of the fingers and toes.…”