Intracoronary urokinase in Kawasaki disease: treatment and prevention of myocardial infarction.

The main cause of death in Kawasaki disease is myocardial infarction due to thrombotic occlusion of a coronary aneurysm. Intracoronary thrombolytic treatment was performed in 15 patients with Kawasaki disease with giant coronary aneurysms. Three patients had acute myocardial infarction, four demonstrated silent myocardial infarction, three suffered chest pain and five did not show ischemia features but had massive thrombus in the coronary aneurysms. Urokinase was infused into the coronary aneurysms as a bolus of 8,000 to 10,000 units/kg via a catheter over 10 minutes. Partial but significant coronary recanalization was achieved after injection of urokinase in a patient with acute myocardial infarction. Complete resolution of massive intracoronary thrombi was observed in 3 of 15 patents, and partial resolution was recognized in 4 cases. In 7 patients, the size of thrombus did not change. Recurrence of the thrombus was observed in 4 patients by serial two-dimensional echocardiography. Urokinase was readministered and two showed significant reduction in the thrombus. All patients have been followed for more than 2 years with longest 8 years (mean: 3.3 yrs), and none have had a recurrence of myocardial infarction or died. These findings suggest that intracoronary urokinase is useful for the treatment and prevention of myocardial infarction in Kawasaki disease.

Infective endocarditis in children: a current diagnostic trend and the embolic complications.

Twenty-three cases of IE, of which 91% had underlying congenital heart diseases were diagnosed by positive blood culture or vegetative lesion detected by 2-D ECHO when a patient revealed clinically suspicious episodes. VSD and TOF were the most common underlying heart diseases. The vegetation was detected successfully by 2-D ECHO in 91% of the cases. The causative organisms were identified in 83% of the cases. Streptococcus viridans and Staphylococcus aureus were the two major organisms detected. Candida albicans was found in 3 postoperative and fatal cases. Embolic complications occurred in 7 cases (30.5%): five cases of pulmonary infarction and two of cerebral infarction. The overall mortality was 22%. We recommend early surgical intervention whenever a patient reveals a very large vegetation with animated movability, and emphasize the importance of a proper prophylaxic regimen.

[Unusual cardiac hypertrophy in neonates with congestive heart failure: report of three cases].

We experienced three cases of unusual cardiac hypertrophy of the right ventricle or interventricular septum with severe congestive heart failure in the neonatal period. One patient had congenital heart disease consisting of membranous tricuspid atresia, absent pulmonary valve, patent ductus arteriosus, left single coronary artery and a hypoplastic pouch-like right ventricle. Very marked cardiac hypertrophy was observed in the right ventricle and interventricular septum. Histologically, there was no appreciable disorganization of the cardiac muscle. The etiology of the unusual hypertrophy of cardiac muscle in this patient is uncertain. The other two patients had asymmetrical septal hypertrophy of the left ventricle evidenced by two-dimensional echocardiography. Cardiac catheterization was performed for these two patients. There was no evidence of congenital heart disease; however, one patient had a significant pressure gradient in the outflow tracts of the left and right ventricles. These two patients' faces appeared unusual and they had minor anomalies of their fingers and ears. This unusual cardiac hypertrophy associated with cardiac anomalies and minor anomalies of the face and extremities comprise a specific type of cardiomyopathy in neonates. This should be distinguished from hypertropic cardiomyopathy of older children and adults.

Myocardial infarction in Kawasaki disease: clinical analyses in 195 cases.

We analyzed clinical data from 195 patients (141 boys) with myocardial infarction complicating Kawasaki disease, collected from 74 major hospitals in Japan. The myocardial infarction usually occurred within the first year of illness, but 27.2% of the patients had myocardial infarction more than 1 year later. In 63% of the patients it occurred during sleep or at rest. The main symptoms of acute myocardial infarction were shock, unrest, vomiting, abdominal pain, and chest pain; chest pain was much more frequently recognized in the survivors and in older patients. The myocardial infarctions were asymptomatic in 37% of the patients. Twenty-two percent of the patients died during the first attack. Sixteen percent of the survivors of a first attack had a second attack. Forty-three percent of all survivors of the first or subsequent attack are doing well; however, others have some type of cardiac dysfunction, such as mitral regurgitation, decreased ejection fraction of the left ventricle, or left ventricular aneurysm. Coronary angiographic studies indicate that in most of the fatal cases there was obstruction either in the main left coronary artery or in both the main right coronary artery and the anterior descending artery. In survivors, one-vessel obstruction was frequently recognized, particularly in the right coronary artery.

[Intracoronary thrombolytic therapy in Kawasaki disease and the usefulness of two-dimensional echocardiography in detecting intracoronary thrombi].

The main cause of death in patients with Kawasaki disease is myocardial infarction due to thrombotic occlusion of a coronary aneurysm. Intracoronary thrombolytic therapy was administered to dissolve the intracoronary thrombi of one infarcted patient and five non-infarcted patients who had massive intracoronary thrombus formations which were detected by two-dimensional echocardiography (2-D echo). Intracoronary injections of Urokinase ranged in dose from 2000 to 240000 IU. Systemic Urokinase infusions were performed for two patients in addition to intracoronary injections. Coronary angiography revealed complete obstruction of coronary aneurysms in two patients and partial obstruction in one patient. Although coronary angiography failed to visualize the intracoronary thrombi in three patients, 2-D echocardiography imaged massive thrombus formations in coronary aneurysms. Partial but significant coronary arterial recanalization was achieved after injecting Urokinase in a patient with myocardial infarction. Complete resolution of massive intracoronary thrombi was observed in three of five patients using 2-D echocardiography. A decrease in size of the intracoronary thrombus in one patient was observed after thrombolytic therapy. In another patient, the size of a thrombus did not change after thrombolytic therapy. Recurrence of thrombus formation in coronary aneurysms was observed in three patients using serial 2-D echocardiography. Urokinase was readministered to them and one showed significant reduction in the thrombus size. We conclude that 1) 2-D echocardiography is more sensitive and reproducible than coronary angiography. Therefore, serial 2-D echocardiography should be performed for patients with Kawasaki disease to detect intracoronary thrombus formation and to evaluate serial changes in thrombi. 2) Intracoronary thrombolytic therapy is useful for patients who have intracoronary thrombi to treat or prevent myocardial infarction.

[Kawasaki disease: new and important problems in cardiology].

Kawasaki disease or mucocutaneous lymph node syndrome (MCLS) is a recently recognized clinical entity in infants and young children with fever and characteristic mucocutaneous involvements accompanied by swelling of the cervical lymph nodes. It has aroused much interest because it may cause sudden death due to coronary arteritis with subsequent aneurysmal formation and thrombotic occlusion. Between January 1973 and September 1982, 611 patients with Kawasaki disease were evaluated with coronary angiography after the acute stage of illness. Of these patients, 136 (22%) were diagnosed as having coronary aneurysms, which were the most common abnormal finding at this stage. Serial two-dimensional echocardiography was useful to evaluate noninvasively the lesions of the coronary artery, and it was discovered that coronary aneurysms appeared in the eighth to 15th day of the illness, and some of them revealed an early restoration. Pericardial effusion appeared in 35% of the patients in the second to third week of the illness. Follow-up coronary angiography was performed in 72 cases who previously had coronary aneurysms five to 18 months after the acute illness. Thirty-nine cases showed completely normal findings at the second study, suggesting the regression of coronary aneurysms in this entity within one or two years after the onset of the illness. The remaining 33 patients showed abnormal findings such as stenotic or obstructed lesions, the irregular arterial wall and persistent aneurysms of coronary arteries at the follow-up study. Among the patients with abnormal angiographic findings myocardial infarction and mitral regurgitation were occasionally present. Three patients died suddenly from myocardial infarction at four months, four and six years after the onset of the illness, respectively. Early initiation of aspirin therapy (10-30 mg/kg) remains the mainstay to prevent thrombus formation and ischemic heart disease. The intracoronary thrombolysis by Urokinase was useful for prevention or treatment of acute myocardial infarction. Patients with Kawasaki disease are mostly in Japan, however, an increasing number of patients have recently been published in the foreign literatures, and this entity has become an important cause of heart disease in children. The long-term follow-up study and establishment of the effective treatment as well as elucidation of the etiology of this disease are essential.

[Usefulness of contrast echoangiographic diagnosis of congenital tricuspid stenosis].

Congenital tricuspid stenosis is an uncommon congenital anomaly and usually associated with other lesions such as severe pulmonic stenosis or atresia. Sometimes, it is difficult to diagnose this malformation non-invasively, but the differential diagnosis from tricuspid atresia is essential for the surgical considerations. Described here were three patients with congenital tricuspid stenosis who were successfully diagnosed by contrast echoangiography using peripheral venous injection of the contrast medium. Contrast echoes appeared in the right atrium initially and then in the right ventricle through the stenotic tricuspid valve. In conclusion, contrast echoangiography is mandatory in the diagnosis of this entity.

Neurological complications in Kawasaki disease.

We have experienced 540 cases with Kawasaki disease over the past 10 years. Six of them (1.1%) had neurological complications with clinical manifestation. Two infants had central nervous system involvement with remarkable changes of cerebrum on CT scan during an acute stage, and these findings disappeared completely within six months. One of these two patients showed no abnormal changes on cerebral angiography. The other four infants had lower motor neuron facial palsy of acute onset and improved within two months.

[Echocardiographic assessment of anatomical detail in patients with hypoplastic left heart syndrome].

Hypoplastic left heart syndrome is a disease with poor prognosis, which is characterized by severe heart failure in early neonatal period. However, there are some patients who survive for relatively longer period. In the light of this fact, 18 patients with this syndrome were studied by echocardiography, and the anatomical and functional classification was attempted. Hypoplastic left heart syndrome was classified into three types: type I was mitral atresia and aortic atresia, type II was mitral atresia and aortic stenosis, and type III was mitral atresia with an abnormality of cono-truncal relationship, respectively. M-mode echocardiography was performed in 15 of the 18 patients, and two-dimensional echocardiograms were recorded in seven cases. Heart catheterization was performed in all patients. Autopsy was performed on 14 of the 18 patients, and its findings were compared with the findings obtained by echocardiography. Two-dimensional echocardiographic classification of this syndrome was possible in all the patients using above-mentioned criteria. Patent ductus arteriosus was detected in two of six cases having this syndrome, and foramen ovale in 4 of 5 cases. The morphological evaluation of both the interatrial and interventricular septum was made by two-dimensional echocardiography, and it contributed to decide the indication of balloon-atrial septostomy. The patients with type I had the poorest prognosis, and the patients with type III survived longer period. Anatomical classification of this syndrome and detection of associated anomalies by two-dimensional echocardiography are recommended to consider the prognostic and surgical considerations.

Fate of coronary aneurysms in Kawasaki disease: serial coronary angiography and long-term follow-up study.

Between January 1973 and December 1979, 290 patients with Kawasaki disease were evaluated with coronary angiography after the acute stage of illness. Of these patients, 43 (15 percent) were diagnosed as having coronary aneurysms. Forty-two patients have been followed up for an average of 4 years (range 15 months to 8 years). One 8 month old girl died of myocardial infarction after 4 months of illness. Follow-up coronary angiography was performed in 42 patients 5 to 18 months after the acute illness. Four groups can be distinguished. Group I: In 21 (50 percent) of 42 patients angiography showed that the coronary aneurysms had regressed, so that no observable lesions were seen. During convalescence, electrocardiography, exercise stress testing and thallium scintigraphy were within normal limits. In the other 21 patients abnormal findings persisted on follow-up angiography. Group II: Ten patients showed persistent coronary aneurysms, although reduced in size. Group III: In seven patients the aneurysms had disappeared, but complete obstruction or marked stenosis of coronary arteries was found. Group IV: In four patients, irregularities of the coronary arterial wall without stenosis were seen. Among patients with abnormal angiographic findings myocardial infarction and mitral regurgitation were also seen. Early initiation of aspirin therapy aneurysms show regression on angiography in 1 or 2 years in about half of patients. The remaining patients are at risk for ischemic heart disease. Thus, Kawasaki disease should be considered an important cause of ischemic heart disease in children and a possible risk factor of premature coronary atherosclerosis.

[Two-dimensional echocardiographic study of coronary artery lesion in Kawasaki disease: a new approach to visualize the right coronary artery].

Recently nonivasive visualization of the left main coronary artery by two-dimensional echocardiography has developed. However, previous reports has described the difficulty to evaluate a right coronary aneurysm and coronary stenosis or obstruction. Therefore two-dimensional echocardiographic studies using 5 MHz linear scanner were performed in 48 patients of Kawasaki disease, and the findings were compared with coronary angiographic findings. Their ages ranged from 3 months to 15 years (mean 3.2 years of age). In coronary angiographic findings, the left main coronary aneurysm was demonstrated in 23 cases, and stenosis of the left coronary artery in 2 cases. While a right coronary aneurysm was demonstrated in 17 cases, and stenosis or obstruction of the right coronary artery in 7 cases. The diagnosis of a coronary aneurysm by linear scanner two-dimensional echocardiography was established in 22 of 23 (96%) patients on the left main coronary artery and in all 17 patients on the right coronary artery. Stenosis or obstruction of the left and right coronary arteries was demonstrated in 1 of 2 (50%) patients and 6 of 7 (86%) patients, respectively. These results were superior to the results of the two-dimensional echocardiographic study by 3.5 mHz sector scanner. The right coronary artery was visualized effectively and extensively by the linear scanner than the sector scanner. We conclude that two-dimensional echocardiography using high frequency linear scanner is a very useful method to diagnose stenotic lesions as well as aneurysmal formation in the right and left coronary arteries and to follow up aneurysms in acute phase and inspect the growth of coronary aneurysms.

[Application of two dimensional echocardiography for the intracardiac manipulation: the evaluation of atrial septal movement before and after balloon atrial septostomy (author's transl)].

Two-dimensional echocardiography was applied to intracardiac catheter manipulation (especially balloon atrial septostomy: BAS) in 6 babies with complete transposition of the great arteries and 2 with total anomalous pulmonary venous drainage. Under the usual roentgenoscopic investigation, intracardiac catheter manipulation was observed by two-dimensional echocardiography directly representing the right atrium, left atrium, atrial septum, right ventricle, left ventricle and interventricular septum by the subcostal approach. Also, formation and movement of the atrial septum and intra-atrial shunt before and after BAS was evaluated by contrast echocardiography. 1. It was confirmed that the catheter tip was inserted into the left atrium from the right atrium. 2. It was clearly demonstrated that the balloon was expanding in the left atrium. 3. When the balloon was pulled out to the right atrium from the left atrium, the movement of the atrial septum and the balloon was observed. 4. The formation and movement of atrial septal defect by BAS was demonstrated and the shunt of this site was estimated by using contrast echocardiography. The relation between the catheter tip and the intracardiac structures can be judged more correctly by two-dimensional echocardiography than roentgenoscopic investigation. Also the structural and hemodynamic changes by BAS can be clearly estimated by the observation of the atrial septum by two-dimensional echography.