Progressive pulmonary vascular disease after pulmonary artery banding and total correction in a case of ventricular septal defect and pulmonary hypertension.

A 7-month-old infant with ventricular septal defect and pulmonary hypertension underwent pulmonary artery banding, which resulted in a decrease in the pulmonary arterial peak pressure from 102 to 54 mmHg. Lung biopsy findings showed at most an early grade 3 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.4, both of which indicated operability for total correction. Small pulmonary arteries less than 100 microns in diameter, however, showed marked hydropic changes in the medial smooth muscle cells. Total correction was performed at the age of 2 years, but the pulmonary arterial pressure failed to decrease. A lung biopsy taken just after the closure of the ventricular septal defect contraindicated operability due to progressive pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.4. The patient died at 8 months after the operation, and an autopsy revealed still more advanced pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.8. The pathogenesis of arterial changes is discussed.

Clinical and histopathological studies in children with supraventricular tachycardia.

To determine whether myocardial changes in patients with supraventricular tachycardia (SVT) are primary or secondary to persistent tachycardia, 11 patients with SVT were studied. These patients were divided into 2 groups with respect to the type of SVT. Group I consisted of 5 patients with incessant SVT and one with multifocal atrial tachycardia, while group II consisted of 4 patients with paroxysmal supraventricular tachycardia and one with short-run supraventricular premature contraction. All of the patients underwent electrophysiological study and endomyocardial biopsy from the right ventricle following routine cardiac catheterization. In group II, there were no significant abnormalities in the clinical and hemodynamic parameters. In group I, 3 patients had clinical features of dilated cardiomyopathy including abnormal ECG, chest X-ray and hypokinesis on left ventriculography. Induction and termination of SVT were possible in 2 patients in group I and in 4 of the 5 patients in group II. The only significant histologic difference between group I and group II was fibrosis. A high incidence of histopathological abnormalities, such as hypertrophy, degeneration, interstitial fibrosis and disarray was observed in both groups. The incidence of significant pathology was higher in group I than in group II. Almost all of the patients were given antiarrhythmic drugs. One patient underwent a successful surgical procedure and normal cardiac function returned after resection of the foci of the right atrium. Our present results suggest that patients with SVT who have incessant or recurrent SVT should undergo not only intracardiac electrophysiologic study but also endomyocardial biopsy for the evaluation of myocardial damage, since SVT might be the initial sign of cardiomyopathy.

Interruption of aortic arch type A in two siblings.

The recurrence of interruption of aortic arch (IAA) in siblings is rare. According to previous reports concerning siblings with IAA, all cases were IAA type B according to the classification proposed by Celoria and Patton. In this report we present the first cases of brothers with IAA type A. The type of IAA was confirmed by autopsy findings in the elder brother and by operative findings in the younger brother. The etiology of IAA was unclear. Monogenic inheritance was thought unlikely because congenital heart disease was not recognized in other members of the family. It would seem prudent to separate recurrence risks for IAA type A from IAA type B when genetic counselling is provided, but it must be borne in mind that the recurrence of IAA type A can occur among siblings.

Histopathologic evidence for cardiomyopathy in a child with mitral valve prolapse.

An intriguing relationship between idiopathic mitral valve prolapse (MVP) and cardiomyopathy has been reported in adult cases of MVP. This paper reports a girl with idiopathic MVP, who presented with progressive cardiomegaly and cardiomyopathic findings or, cardiac biopsy; right ventricular endomyocardial biopsy at the age of 15 years showed myocardial hypertrophy, disarray, myocardial degeneration, interstitial fibrosis and endocardial thickening. To our knowledge, this is the first case of idiopathic MVP in childhood that has been shown to have positive biopsy findings for cardiomyopathy.

Histopathological study on Kawasaki disease with special reference to the relation between the myocardial sequelae and regional wall motion abnormalities of the left ventricle.

Thirty-eight consecutive patients with Kawasaki disease (18 patients with normal coronary artery and 20 with coronary artery lesions) were studied in order to find the incidence of myocardial abnormalities and the influence of the myocardial damage on the regional wall motion of the left ventricle in patients with Kawasaki disease with or without coronary artery lesion. Abnormal regional wall motion of the left ventricle was found in 28% of the patients with normal coronary artery (NCA) and in 55% of those with coronary artery lesions (CAL). Hypertrophy of myocytes, degeneration of myocytes and disarray were found frequently in the patients with CAL. The histopathology of endomyocardial biopsy taken from patients more than 3 years after disease onset and less than 3 years after onset were compared. Histopathologic abnormalities were found even in the biopsies taken more than 3 years after onset, especially from patients with coronary artery lesions, although the incidence of abnormalities was less frequent. We consider that repeat endomyocardial biopsies may be necessary in the patients who have significant histopathologic changes in the first endomyocardial biopsy to clarify the natural course of myocardial abnormalities, regardless of whether or not they have CAL.

[Histopathological analysis of myocardial damages following Kawasaki disease with repeated endomyocardial biopsy].

Since Kawasaki disease was first reported 20 years ago, many investigations about cardiac complications and treatment have been carried out. However, etiology still remains unknown. Although many studies focused on coronary artery lesions (CAL) have been reported, there have been only a few reports of pathological studies on myocardial after-effects of Kawasaki disease using endomyocardial biopsy. In order to investigate the myocardial sequelae, repeated coronary arteriography and endomyocardial biopsy were performed in thirteen patients of Kawasaki disease with coronary artery lesions. Their ages ranged from 19 months to 12 years in the first study, 4 years to 15 years in the second study, and the male to female ratio was 8:5. In this study the interval between the first study and the second one was more than two years and six months. In the second study, progression of the CAL was noticed in 23% of the cases. It was unchanged in 54%, and regression was observed in 23%. Twenty-three percent of the patients were found to have an ejection fraction of less than 55%, and abnormal regional wall motion was found in 62% of the patients. Myocardial changes in the second endomyocardial biopsy were interstitial fibrosis in 62%, degeneration in 54%, disarray in 32% and inflammatory cell infiltration in 23%. One of the cases with Kawasaki disease, who had mild CAL, revealed massive inflammatory cell infiltration and myocytolysis in the second study performed seven years after onset, which suggested chronic myocarditis. Myocardial changes in the patients with advanced CAL were relatively mild, and low EF and abnormal regional wall motion were not always related to severity of CAL.(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary arterial calcification in Kawasaki disease.

The purpose of this paper is to describe the clinical characteristics and significance of the coronary arterial calcification due to Kawasaki disease (KD), based on our five patients. The coronary arterial lesions (CAL) were in segments 1, 2, 5, 6 and 11 according to the classification of the American Heart Association. The interval from the onset to the first appearance of coronary calcification was 17 months to 61 months. Progression of CAL into coronary arterial stenosis or myocardial infarction occurred in all five patients. In the comparative study between the patients with calcified CAL and those with non-calcified CAL, the maximal size of CAL of the former was 8 mm or larger in contrast with that of the latter (less than 8 mm). It is concluded that coronary arterial calcification is one of the important signs which suggest the presence of the advanced CAL in the follow-up of children with a history of KD.

[Clinical investigation on the patients with tachyarrhythmic syncope with special reference to comparative study between long QT syndrome and ventricular tachycardia without long QT interval].

The long QT syndrome (LQTS) is one of the important diseases that may lead to sudden death mainly in childhood, however etiology and pathogenesis are still poorly understood. The group studied consisted of 6 patients with a history of ventricular tachyarrhythmic syncope, 3 with long QT syndrome (LQTS) and 3 without long QT interval, and of 4 patients with ventricular tachycardia without syncopal episode. Their ages ranged from 5 years to 17 years. Histopathology of endomyocardial biopsy was nonspecific and mild in two cases but in one patient with LQTS, who had several episodes of syncope and refractory ventricular arrhythmia, remarkable subendocardial fibrosis, interstitial fibrosis and hypertrophy of myocytes were demonstrated. As far as ventricular tachycardia without long QT interval was concerned, in the patients with VT with syncope, histopathological abnormalities were more remarkable than in those without syncope. Electrophysiological findings in the patients with LQTS showed no characteristic findings, but only mild abnormalities with functional atrioventricular conduction disturbance on programmed atrial pacing. No inducible VT was demonstrated. Although electrophysiologic study and endomyocardial biopsy are of limited value, such studies are considered to be worthwhile for treating ventricular arrhythmias, and making a prognosis of the patients with tachyarrhythmic syncope and LQTS.

[Clinical investigation on childhood HCM; long-term follow-up study with special reference to electrophysiology, histopathology by endomyocardial biopsy and ambulatory and exercise electrocardiography].

In order to determine the prognosis and risk factors of childhood HCM, we investigated clinical parameters, such as the age of onset, symptoms and signs related to HCM, dysrhythmias and ST-T and changes by Holter ECG and exercise ECG, electrophysiological study and histopathological study by endomyocardial biopsy. Heart murmur was the commonest sign although only one patient revealed a significant left ventricular outflow tract gradient of more than 20 mmHg. The peak age of patients diagnosed as having HCM was 10 to 15 years. The major ECG manifestations of our series showed specific findings and in some cases ECG changed remarkably during the follow-up period. The diagnosis of apical hypertrophy should be made with caution in childhood. Holter ECG seemed to be indispensable for the follow-up of childhood HCM because Holter ECG in our patients manifested such significant findings as ventricular premature contractions or couplets or ST depression. The degree of clinical severity did not always correlate with the histopathological severity obtained by endomyocardial biopsy. As HCM in childhood is heterogeneous and multifaceted, there is no one therapy, and prognosis and proper treatment with close observation should be carried out in each individual case.

Endomyocardial biopsy in children with Kawasaki disease.

To study the histopathology of the myocardium in Kawasaki Disease (KD) with various coronary angiographic findings, right endomyocardial biopsy specimens were evaluated on 20 boys and 16 girls with this disorder. KD with coronary artery lesions (CAL) showed various histopathological abnormalities, such as myocellular hypertrophy in 44%, degeneration in 61%, interstitial fibrosis in 44% and endomyocardial changes in 22%. KD without CAL revealed myocellular hypertrophy in 44%, degeneration in 50%, disarray in 28% and interstitial fibrosis in 6%. As in patients with long-standing KD, more than three years after onset, myocardial changes such as myocellular hypertrophy, disarray and interstitial fibrosis persisted, it is suggested that cardiomyopathy may develop in some patients with KD.

[Clinical studies on cefteram pivoxil in pediatrics].

Cefteram pivoxil (CFTM-PI), a newly developed oral cephem antibiotic was administered to treat 16 children with various infections. The results were summarized as follows. 1. The clinical responses were "excellent" in 7, "good" in 5, "fair" in 3 and "poor" in 1, with 75.0% efficacy rate. 2. No side effects and no abnormal laboratory findings were observed.

[Histopathological studies of endomyocardial biopsy on the children with Kawasaki disease].

Since there has been many reports on the coronary artery lesions in the patients with Kawasaki disease, but only a few reports on histopathological investigation employing endomyocardial biopsy, histopathological study was performed in eighteen children of Kawasaki disease with coronary artery lesions and eighteen children of Kawasaki disease with intact coronary artery, aged from one year and ten months to fourteen years. There were twenty males and sixteen females. Comparing Kawasaki disease who had coronary artery lesions with those not, there was no striking difference in histologic findings. Kawasaki disease without coronary artery lesions revealed myocellular hypertrophy, degeneration of myocytes, disarray, interstitial fibrosis and endocardial changes in 44%, 61%, 11% and 44%, respectively. Among Kawasaki disease who had coronary artery lesions, children with coronary artery stenosis showed a higher incidence of histological abnormalities than those with coronary artery aneurysm. Some cases of Kawasaki disease were recognized to have significant myocardial abnormalities even in three to six years after onset, which seemed to persist whether or not they would have coronary artery lesions. Since Kawasaki disease who would have myocardial damages might have the potential for developing cardiomyopathy, careful attention should be payed to myocardial lesions as well as sequelae of coronary artery lesions.

[Electrophysiologic and histopathologic investigations in children with idiopathic ventricular arrhythmias].

Between April 1984 and December 1987, electrophysiological studies and endomyocardial biopsy were performed in 14 pediatric patients, aged from 7 to 15 years, with idiopathic ventricular arrhythmias in whom diagnostic evaluation had revealed no structural heart disease. They were 8 boys and 6 girls. Cardiac catheterization revealed regional wall motion abnormalities of the left ventricle in 3 patients, one of whom showed decreased ejection fraction (EF). Electrophysiologic examination showed sinus node dysfunction in 21%. AV nodal dysfunction in 14% and dual AV nodal pathway in 21%. Histopathologic examination by endomyocardial biopsy showed myocellular hypertrophy, degeneration of myocytes, interstitial fibrosis and endomyocardial thickness in 86%, 36%, 35% and 14%, respectively. Since idiopathic ventricular arrhythmias in pediatric age group included relatively high electrophysiologic and histopathologic abnormalities, which were suggestive of occult myocardial disease and might be the early stage of cardiomyopathy, careful follow-up should be required.

[Clinical results of a rokitamycin dry syrup in pediatric infections].

Clinical efficacies of a new macrolide antibiotic, rokitamycin (RKM, TMS-19-Q), were studied in acute pediatric infections. Responses to the RKM administration were evaluable in 62 out of 68 patients consisted of 7 patients with pharyngitis (efficacy rate of 85.7%, 6/7 patients), 4 with bronchitis (25.0%, 1/4), 9 with tonsillitis (100%, 9/9), 13 with mycoplasmal pneumonia (100%, 13/13), 13 with hemolytic streptococcal infections (92.3%, 12/13), 14 with pneumonia (57.1%, 8/14), one with pertussis (100%, 1/1) and another with Chlamydia pneumonia (100%, 1/1) thus an overall efficacy rate of 82.3% was achieved. Urticaria was observed in one of the patients as an adverse reaction to the drug, while abnormal laboratory test results were noted in 3 patients, but none of such changes were severe. The drug, even when administered in combination with a theophylline preparation, exerted no effects on the serum concentration of the latter.

Dilated cardiomyopathy: diagnostic accuracy of endomyocardial biopsy.

A histopathological index of contractility failure, which was reported to be accurate for the diagnosis of idiopathic dilated or congestive cardiomyopathy in Japan, was used to assess endomyocardial biopsy specimens from 41 Dutch patients with suspected dilated cardiomyopathy. The calculated index had a sensitivity of 62.5% and a specificity of 68% for idiopathic dilated or congestive cardiomyopathy. These disappointing results indicate that the histopathological index of contractility failure does not reliably distinguish between idiopathic dilated cardiomyopathy and other diseases that cause congestive heart failure.