Among Kawasaki disease patients with systemic artery aneurysms, the brachial and internal iliac arteries are the most commonly affected, and occlusions of both arteries are often found. However, the long-term fate of large common iliac artery aneurysms remains unknown, because their prevalence is very low. The long-term outcomes of common iliac artery aneurysms caused by Kawasaki disease in four patients (three females, one male) were investigated retrospectively based on their medical records and angiograms. Their ages ranged from 30 to 36 years-old. The onset age of Kawasaki disease ranged from 4 to 8 months, and the interval from the onset of Kawasaki disease to the latest angiogram ranged from 17 to 21 years. All patients had bilateral large coronary aneurysms and common iliac artery aneurysms with maximal diameters greater than 10 mm. Although all patients had multi-vessel coronary artery stenotic lesions and systemic artery aneurysms, they were asymptomatic. The three female patients underwent coronary artery bypass grafting, and the male patient underwent replacement of artificial vessels for large bilateral common iliac artery aneurysms at 3 years old of age. Over the long-term, common iliac artery aneurysms greater than 10 mm persisted as calcified aneurysms. However, they had no symptoms due to their common iliac artery aneurysms, and their ankle brachial pressure index was preserved, even if the stenosis of the common iliac artery developed as a late outcome, because the collateral arteries were well developed. The progression of stenosis of the common iliac artery after Kawasaki disease was slower.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

Whether the acute inflammation caused by Kawasaki disease will have an effect on the endothelial function of coronary arteries in the future is unknown.

We report the long-term results of percutaneous transluminal coronary balloon angioplasty for early appearing localised stenosis of the left anterior descending artery caused by Kawasaki disease in two 2-year-olds. One patient had 50% restenosis and newly appearing aneurysm at 21-year-old, and the other had no restenosis at 16-year-old. They had no symptoms or cardiac events for 20 years. It is useful in the early localised stenosis without aneurysm.

Thirteen boys and one girl, 5–30 years (median 13 years), underwent percutaneous transluminal coronary rotational atherectomy. The interval from the onset of Kawasaki disease to PTCRA ranged from 5 to 29 years (median 12 years). The follow-up period was 1–22 years (median 13 years). The target vessels were the right coronary artery (7), left anterior descending artery (3), left circumflex (2), and left main trunk (2). The maximum burr size used was 1.75 mm in four, 2.00 mm in four, and 2.15 mm in six. The immediate results of rotational atherectomy were successful in all patients, and the mean stenosis degree improved from 86 ± 15% (mean ± standard deviation) to 37 ± 14% (p < 0.001). Cardiac events in the late period were found in four patients (29%). Acute myocardial infarction occurred in two, and syncope and ventricular fibrillation in one each. The cardiac event-free rate at 10 and 20 years was 79% (95% confidence interval 50–92) and 39% (6–87), respectively, (n = 14). The overall 20-year patency rate was 54% (95% CI 28–78). That in patients more than 10 years old was 77% (95% CI 42–94, n = 10). PTCRA alone is suitable for severe localised stenosis with calcification caused by KD in young adults except for small children. Re-stenosis within the first year after PTCRA often develops because of reactive intimal thickening after the procedure. If a target vessel is a patent 1 year after the procedure, long-term patency may be expected in patients more than 10 years old.

Over a 50-year period from the first description of Kawasaki disease, we encountered three male patients with a history of Kawasaki disease, who had their first cardiac events in their forties. They were considered to have almost normal coronary arteries in the coronary angiograms when they were children and adolescents. They had no follow-up examinations after 20 years old. The 1st patient had an acute myocardial infarction, and the 2nd was a new appearance of coronary aneurysm and stenotic lesions with coronary artery calcification. The 3rd patient had unexpected sudden death. The interval from the onset of Kawasaki disease to the cardiac events ranged from 37 to 38 years. In the former two patients, coronary artery lesions could not be evaluated immediately after Kawasaki disease. Although the 3rd patient had bilateral medium-sized coronary artery aneurysms, his coronary aneurysms regressed 1 year after acute Kawasaki disease. The intimal thickening at a previous coronary aneurysm at the age of 19 was mild. The patients with regressed coronary aneurysms were asymptomatic for about 40 years after Kawasaki disease, prior to their cardiac events. Coronary artery calcification of the proximal portion of the major coronary arteries was a predictable marker in such patients. To prevent serious cardiac events in middle-aged adult patients, reevaluation of coronary artery lesions and restarting of anti-thrombotic therapy are needed. We must be aware that there are some differences in the clinical course and time of cardiac events between patients with giant aneurysms and those with medium aneurysms.

Fifty years have passed since the first report of Kawasaki disease in 1967, and the prevalence of acute coronary syndrome in Kawasaki disease patients with coronary artery lesions exceeding 40 years old has increased. Primary coronary stent implantation is currently an acceptable method in ischaemic coronary heart disease in adults. However, it is unknown whether the stent implantation is effective or not in this population. As the clue to answer this question, I reviewed the references on Kawasaki disease patients who underwent the stent implantations between 1997 and 2019. Thirty-three patients underwent stent implantations for 34 coronary arteries. Adverse effects in the late period were found in 19 (68%) of 28 vessels with follow-up angiograms. There were complete occlusion 9, restenosis 8, and migration 2. A new aneurysm formation was found in 7 (37%) among the 19 vessels, and 6 (86%) of the 7 vessels were drug-eluting stent and 5 were found after the procedure for chronic total occlusion. The adverse effects free-rate at 1 year and 3 years were 57 and 25%, respectively. At present, the usefulness of stent implantation in the long-term results was scarce. Even if primary percutaneous coronary intervention without a stent implantation is performed for acute coronary syndrome, it can be expected to maintain the patency of the culprit lesion for several years. It is better to avoid a stent implantation as long as possible in this population. Knowing the long-term efficacy and complications of stent implantations is important for deciding the procedure.

We report two females with coronary artery occlusion caused by presumed Kawasaki disease that delivered children without any special treatment. After a 58-year-old female had ventricular tachycardia, a giant coronary artery aneurysm with calcification at the bifurcation of the left coronary artery and segmental stenosis of the right coronary artery were pointed out by CT angiography. She had an episode of sepsis when 3 years old. Further, she remembered chest pain during sleep after that episode. She had delivered twice without any complication during her 20s. Her diagnosis was undiagnosed coronary artery lesions caused by presumed Kawasaki disease and a previous myocardial infarction, and she underwent radiofrequency catheter ablation and implantable cardioverter defibrillator implantation. The other 48-year-old female was accidentally discovered to have coronary artery calcification on CT, while experiencing pneumonia. Her CT angiograms revealed a right coronary artery occlusion and coronary artery calcification at segments 1, 6, and 11. She had a history of “scarlet fever” before 12 months. Premature ventricular contractions were detected, while delivering her first child when 31 years old. However, she was not diagnosed as ischaemic heart disease and delivered twice by a vaginal delivery without any complication. Current guidelines recommend systemic anti-coagulation and anti-platelet therapy for all patients with giant aneurysms resulting from Kawasaki disease in childhood. The two women reported here were fortunate not to have had complications during pregnancy and delivery despite their severe coronary artery aneurysms, which were unrecognised clinically until later in life. They were lucky cases.

Eosinophilic myocarditis is rare in children, and consequently, it is difficult to diagnose eosinophilic myocarditis rapidly. We report the clinical course of acute eosinophilic myocarditis with pericarditis in two adolescent boys and their associated electrocardiograms. The two patients, 13- and 14-year-old boys, developed cardiomegaly and chest pain with vomiting. On examination by two-dimensional echocardiography, thickening of the ventricular septum and a pericardial effusion were detected. The eosinophil count had increased by the pericardial effusion. Acute eosinophilic myocarditis often complicates a moderate to severe pericardial effusion owing to acute pericarditis. A cellular fraction analysis of the pericardial effusion is easy and useful for the diagnosis of eosinophilic myocarditis. Some serial changes in the electrocardiogram occur during each stage of acute eosinophilic myocarditis. They are induced by eosinophilic granules, which are capable of inducing tissue damage and dysfunction, and those changes in the electrocardiogram resemble the changes after an acute myocardial infarction. It is important to know the characteristics of eosinophilic myocarditis in order to prevent lethal complications.

Severe valvulitis owing to acute Kawasaki disease leading to severe mitral regurgitation is a rare event in infants. Further, there is less information about underlying ruptured mitral chordae tendineae causing severe mitral regurgitation. We encountered ruptured mitral chordae tendineae in three female patients after Kawasaki disease. The age at the onset of Kawasaki disease ranged from 3 to 8 months, and detection of ruptured mitral chordae tendineae was from 24 to 90 days. Two patients had acute heart failure, and one was asymptomatic. One patient underwent mitral annuloplasty, and the others responded to medication. These ruptured mitral chordae tendineae occurred after the remission of the initial acute Kawasaki disease, in the early course and the convalescent of acute Kawasaki disease. Further, the recurrent fever was also detected in them. The ruptured mitral chordae tendineae in an infant within 6 months can be detected by systolic heart murmur around the convalescent stage of acute Kawasaki disease, although the prevalence is very low.

Information about acute coronary syndrome caused by Kawasaki disease-related coronary artery lesions in adults is sketchy. We reviewed the clinical features of 50 adult patients who had an acute coronary syndrome caused by coronary artery lesions due to Kawasaki disease or probable Kawasaki disease from 1980 to 2008. Of the 50 patients, 43 (90%) were male and seven were female (10%). Their ages at the onset of acute coronary syndrome ranged from 18 to 69 years, with a median of 28 years. The culprit lesion in 43 patients was thrombotic occlusion of an aneurysm, and 40 patients had giant aneurysms. In the three patients in whom no aneurysms were seen in coronary angiograms performed at the time of acute myocardial infarction, either giant aneurysms or aneurysms had been visualised in childhood. The initial treatment of acute coronary syndrome was as follows: intracoronary thrombolysis, 11; primary percutaneous coronary intervention, 9; emergency coronary artery bypass grafting, 3; and medication, 26. Elective coronary artery bypass grafting was performed in 15 patients. Three patients (6%) died. Of the 27 patients with additional coronary risk factors, 20 were smokers. Giant aneurysms due to Kawasaki disease continued to cause acute coronary syndrome in adult life with onset at a younger age than typifies that due to atherosclerosis in the general population, especially in male population rather than female population. Even when giant aneurysms regressed after the acute phase, a few patients still developed acute coronary syndrome in adult life. Smoking appears to be the most prominent additional risk factor.

There exists a population of adults with undiagnosed coronary arterial lesions due to Kawasaki disease occurring before 1967. We report the clinical features in 6 adult males with coronary arterial lesions caused by presumed Kawasaki disease, whose dates of birth ranged from 1945 to 1963. The age of the diagnosed coronary arterial lesions due to presumed Kawasaki disease ranged from 26 to 48 years. In 4 patients, there was a history of probable Kawasaki disease. The presenting features were chest pain in 2; syncope in 1, an abnormal electrocardiogram in 2; a history of presumed Kawasaki disease in 1, and symptomatic myocardial infarction in the final patient. Coronary angiograms revealed multi-vessel disease in 5 patients, with segmental stenosis in 5, and calcified giant aneurysms in the proximal portion of the coronary arteries also in 5. Low left ventricular ejection fractions of less than 40% were found in 3. Of the patients, 3 had undergone coronary arterial bypass grafting. A defibrillator had been implanted in 2 because of rapid ventricular tachycardia with syncope induced during electrophysiologic studies. We conclude that, in patients with multi-vessel disease or left ventricular dysfunction caused by presumed Kawasaki disease, symptoms and serious cardiac events occur in adult life with the onset of ageing, although the patients had been asymptomatic for many years after the onset of Kawasaki disease itself.

Background: Our purpose was to determine the outcome of pregnancy and delivery in patients with coronary arterial lesions caused by Kawasaki disease. Methods and Results: We surveyed by mail the Japanese national experience of pregnancy and delivery in patients known to have Kawasaki disease. The first questionnaire was returned by 154 of 207 (74%) institutions, and 16 of the 154 had knowledge of deliveries in their patients. Based on a second questionnaire, and previous Japanese case reports, we identified 46 deliveries in 30 patients from 16 institutions. The age at delivery ranged from 18 to 35 years, with a median of 27 years. Of the patients, 4 had undergone coronary arterial bypass grafting. Low-dose aspirin was given in 16 patients. The deliveries, 27 in all, had been vaginal in 20 patients, albeit that 7 required assistance by forceps or vacuum extraction under epidural anesthesia. Caesarean section had been performed in 11 patients, 3 for obstetric indications, and 1 for chest discomfort in the third trimester. Although there were no cardiac events, obstetric complications occurred in 2. Conclusion: The results of pregnancy and delivery were favourable. The mode of delivery should be primarily determined by obstetrical considerations, rather than the coronary arterial lesions caused by Kawasaki disease. Excessive anticoagulant therapy may not be needed for this population.

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.

Objectives: We evaluated the efficacy of percutaneous transluminal coronary angioplasty for anastomotic stenosis after coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease. Subjects and Methods: From July 1997 to April 2000, four boys and one girl underwent percutaneous transluminal coronary angioplasty for 6 anastomotic lesions following coronary arterial bypass grafting using the left or right internal thoracic artery. Progressive severe stenosis of the grafts in the follow-up angiograms after grafting, and evidence of ischemia, were regarded as indications for percutaneous transluminal coronary angioplasty. Age at coronary angioplasty ranged from 4.2 to 16.7 years, with a median of 6.9 years, while the interval from operation ranged from 0.3 to 3.0 years, with a median of 1.1 years. The diameter of the balloon catheter employed varied from 1.5 to 2.5 mm, and the pressure of inflation ranged from 8 to 16 atmospheres. Results: The degree of stenosis decreased from 63 to 99%, with a median of 88%, to 0 to 40%, with a median of 17% immediately after angioplasty. A follow-up angiogram either 3 months or 1 year later revealed no restenosis in any patient. Conclusion: Percutaneous transluminal coronary angioplasty is a feasible and useful procedure for treating anastomotic stenosis following coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease.