Emergency pacing via the umbilical vein of a neonate with congenital complete atrioventricular block: a report of two cases.

We report two cases of successful emergency pacing via the umbilical vein in neonates with congenital complete atrioventricular block. The first patient, a neonate with normal cardiac anatomy, underwent emergency temporary pacing via the umbilical vein under echocardiographic guidance. The patient underwent permanent pacemaker implantation on postnatal day 4. The second patient, a neonate with heterotaxy syndrome, underwent emergency temporary pacing through the umbilical vein under fluoroscopic guidance. The patient underwent permanent pacemaker implantation on postnatal day 17.

Coronary Revascularization of Giant Aneurysms in Children With Kawasaki Disease: A Report of Two Cases.

In recent years, >100 cases of coronary artery stenotic lesions due to Kawasaki disease were treated with coronary artery bypass grafting (CABG). Surgical indications include stenosis of >75%, myocardial infarction history, electrocardiographic changes, and ischemia, as detected by myocardial scintigraphy and electrocardiography, due to drugs or exercise. Some centers have reported good patency rates, even in infants. The advantages of CABG in younger patients are minimal loss of left ventricular function, early elimination of post-operative ischemia risk, and improved quality of life. However, the disadvantage of performing CABG in younger patients is the small coronary artery diameter and the thin vessel wall, which can lead to post-operative occlusion, especially when performed by inexperienced surgeons. The optimal timing of CABG varies by institution and case, which depends on the presence or absence of complications, such as left ventricular dysfunction or valve regurgitation, and surgeon's experience. Importantly, unlike adult surgery, childhood CABG needs to be kept open for the very longest possible period of time to determine the optimal conditions for surgery. We report two pediatric cases of giant coronary artery aneurysms diagnosed in infancy. During school age, the patients had a mild decline of left ventricular ejection function. In one case, there were no clinical symptoms because of the development of collateral vessels and limitation of exercise. Both patients underwent surgery with good results. The gastric gastroepiploic artery could be anastomosed owing to the development of collateral blood vessels, although it was obstructed. At 1 year after surgery, both patients had a good post-operative course without complications of anastomotic stenosis or myocardial damage due to aneurysm resection. If conditions are favorable, bypass surgery can be postponed to several years until the coronary arteries are sufficiently large to warrant a delay in coronary artery stenosis in cases of infantile Kawasaki disease.

Thrombolytic therapy in Kawasaki disease: a report of four cases.

Four patients with a thrombus in a coronary artery aneurysm due to Kawasaki disease (KD) were treated at our hospital between 1994 and 2009. All the cases were treated with intravenous coronary thrombolysis (IVCT) therapy and the cases with acute myocardial infarction were treated with additional intracoronary thrombolysis therapy. Although both thrombolytic therapies were effective, IVCT required more time than intracoronary thrombolysis to resolve the thrombus. We concluded that IVCT can be used as the first-line thrombolytic therapy for KD, except in cases with acute myocardial infarction.

National Japanese survey of thrombolytic therapy selection for coronary aneurysm: intracoronary thrombolysis or intravenous coronary thrombolysis in patients with Kawasaki disease.

BACKGROUND: Thrombolytic therapy for coronary aneurysm thrombosis of Kawasaki disease (KD) includes antiplatelet and anticoagulants, i.v. coronary thrombolysis (IVCT), and i.c. thrombolysis (ICT). Therapeutic methods, drugs and doses vary among medical facilities. METHODS AND RESULTS: A nationwide survey of thrombolytic therapy was conducted under the auspices of the Ministry of Health, Labour and Welfare Research Program to Intractable Diseases Research Grants. A secondary survey targeted 14 facilities conducting thrombolytic therapy during a 5 year period (2004-2009). The primary survey investigated performance of thrombolytic therapy for 23 KD patients at 14 facilities, and of these, five with acute myocardial infarction had received ICT and combined therapy. The secondary survey investigated the pre-treatment condition of aneurysm and thrombosis and actual treatment methods. ICT was effective for small thrombi (≤ 10 mm), while IVCT was effective even for giant thrombi (>10 mm). ICT was also effective for thrombi within several hours after formation. CONCLUSION: In the present nationwide survey, thrombolytic therapy was more effective in cases of a shorter duration between thrombus formation and the start of treatment. It was found that many facilities used only IVCT for thrombus alone. Medications given to KD children did not cause serious hemorrhagic complications, unlike in adults. Although doses exceeded recommended levels in many cases, the only complications were nasal bleeding and fever.

Analysis of arterial function in adults with a history of Kawasaki disease.

BACKGROUND AND PURPOSE: It remains controversial whether Kawasaki disease (KD) is a risk factor for the early onset of atherosclerosis. The purpose of the present study was to assess endothelial function and arterial stiffness as markers of the early onset of atherosclerosis in adult patients with a history of KD. METHODS AND SUBJECTS: We compared 14 adult patients with a history of KD with 41 healthy controls. To assess arterial endothelial function, we measured the reactive hyperemia-peripheral arterial tonometry (RH-PAT) index and augmentation index adjusted to 75 bpm (AIx@75) using the Endo-PAT 2000 (Itamar Medical, Caesarea, Israel). In addition, we analyzed medical history, blood pressure, lifestyle habits, and atherosclerosis-related serum biochemical markers [asymmetric dimethylarginine, adiponectin, lipoprotein (a), cholesterols, atherogenic index of plasma]. RESULTS: There was no difference between the KD and control groups with regard to the RH-PAT index values (2.10 ± 0.43 and 1.84 ± 0.49, respectively; p=0.19). However, in the KD group, the RH-PAT index values were negatively correlated with the febrile period in the acute phase of disease (r(2)=0.458, p=0.048). In addition, the AIx@75 values were higher in KD patients compared to healthy controls (-7.69 ± 11.86% and -15.87 ± 8.72%, respectively; p=0.01). No significant differences existed between the KD and control groups with regard to the serum biomarkers of atherosclerosis. CONCLUSIONS: We speculate that endothelial dysfunction in former KD patients is affected by the febrile period of the acute phase, and antiplatelet drugs may improve endothelial function. The increased arterial stiffness of patients caused by post-inflammatory fibrotic changes in the arterial wall indicates that adults with a history of KD have an increased risk of developing atherosclerosis.

Histopathological study of lymph node lesions in the acute phase of Kawasaki disease.

AIMS: To review the histopathological features of cervical LNs, and to clarify the changes in extracervical LNs, in acute Kawasaki disease (KD). METHODS AND RESULTS: The samples were obtained from 33 patients with acute-phase KD. We divided the LNs into those in the neck (n = 23) and those located elsewhere (n = 26), and investigated them histologically. Changes occur not only in the cervical region, but also in LNs throughout the body. Most lymphadenopathy is non-specific, caused by sinus expansion and paracortical zone enlargement, but there are also necrotic lesions of various sizes that can be surmised to result from ischaemic changes in some LNs. Necrotic foci start to develop immediately below the capsule, and are accompanied by fibrin thrombi in the small vessels and perivascular nuclear debris. Especially in the case of cervical LNs with necrosis, a high degree of non-purulent inflammation develops in the LN capsule and surrounding connective tissue. CONCLUSIONS: In addition to lymphadenopathy with necrosis, KD should be suspected if there is non-purulent inflammation of the LN capsule and/or surrounding connective tissue featuring mainly monocytes/macrophages.

Histopathological characteristics of myocarditis in acute-phase Kawasaki disease.

AIMS: To elucidate the histopathological characteristics of myocarditis in acute-phase Kawasaki disease (KD). METHODS AND RESULTS: The examined materials were from 29 autopsied KD patients who died within 40 disease days following onset. Each heart was divided into three levels: base, middle and apex. At each of these levels, the myocardium was divided further into the epicardial, middle and endocardial layers, and the time-courses of the changes in the myocarditis and the distribution of inflammation were analysed. Inflammatory cell infiltration, consisting mainly of lobulated leucocytes and large mononuclear cells, was seen in the myocardial interstitium in all cases. Inflammatory cell infiltration was already seen by disease day 6 in a patient with no coronary arteritis; it became prominent after day 10 and gradually disappeared after day 20. Myocarditis was initially distributed diffusely throughout the heart, but after day 10 it was localized in the base and epicardial layer. CONCLUSIONS: In KD, myocarditis develops even earlier than epicardial coronary arteritis; it peaks by disease day 10 and then disappears gradually after day 20. The myocarditis is distributed unevenly, ranging from the entire heart to the epicardial layer of the base of the heart.