Hung-Chi Lue

Epidemiologic Features of Kawasaki Disease in Taiwan, 2003–2006

OBJECTIVE. Kawasaki disease is the leading cause of acquired heart disease in children worldwide. This study characterizes the epidemiology of Kawasaki disease in Taiwan between 2003 and 2006. METHODS. Using Taiwans 2003–2006 national health insurance claims, we investigated the epidemiologic features of Kawasaki disease (ICD-9-CM code 446.1) and coronary artery aneurysm formation (International Classification of Diseases, Ninth Revision, Clinical Modification code 414.11) and compared the incidences of these diseases with those occurring between 1996 and 2002 in Taiwan and those reported by other countries. RESULTS. During our 4-year study period, 3877 children and adolescents <20 years of age were hospitalized for Kawasaki disease. Ninety percent of these children were <5 years of age, and the male/female ratio was 1.62:1. The annual incidence of Kawasaki disease was 153 in 100000 children <1 year of age, 111 in children 1 year of age, 58 in children 2 years of age, 30 in children 3 years of age, 19 in children 4 years of age, and 5.2 in children 5 to 9 years of age. The overall incidence was 69 cases per 100000 for children <5 years of age. Kawasaki disease recurred in 1.5% of all cases. Kawasaki disease occurred most frequently in the summer and least frequently in the winter. Coronary artery aneurysm occurred in 7.2% (279 of 3877) of all Kawasaki disease cases. CONCLUSIONS. The overall incidence of Kawasaki disease was 69 in 100000 children <5 years of age between 2003 and 2006 in Taiwan, comparable with the incidence of 66 in 100000 children between 1996 and 2002. Taiwan has the third highest incidence of Kawasaki disease in the world, after Japan and Korea. In Taiwan, it occurs more frequently during the summer.

Supraventricular tachycardia in patients with right atrial isomerism

OBJECTIVES To clarify the prevalence and mechanism of supraventricular tachycardia in patients with right atrial isomerism. BACKGROUND Paired SA and dual atrioventricular (AV) nodes have been described in patients with right atrial isomerism. However, the clinical significance remains unclear. METHODS From 1987 to 1996, a total of 101 patients (61 male, 40 female) and four fetuses were identified with right atrial isomerism. The diagnosis of supraventricular tachycardia exclude the tachycardia with prolonged QRS duration or AV dissociation, and primary atrial tachycardia. RESULTS The median follow-up duration was 38 months (range 0.2-270 months). Supraventricular tachycardia was documented in 25 patients (24.8%) and one fetus (25%) (onset age ranged from prenatal to 14 years old; median 4 years old). Actuarial Kaplan-Meier analysis revealed that the probability of being free from tachycardia was 67% and 50% at 6 and 10 years of age, respectively. These tachycardias could be converted by vagal maneuvers in one, verapamil in seven, propranolol in four, digoxin in two, procainamide in one, and rapid pacing in five. Spontaneous conversion was noted in six (including the fetus). Seven cases had received electrophysiological studies. Reciprocating AV tachycardia could be induced in five and echo beats in one. The tachycardia in three patients was documented as incorporating a posterior AV node (antegrade) and an anterior or a lateral AV node (retrograde). Two of them received radiofrequency ablation. Successful ablation in both was obtained by delivering energy during tachycardia, aimed at the earliest retrograde atrial activity and accompanied by junctional ectopic rhythm. The patient with echo beats developed tachycardia soon after operation. CONCLUSIONS Supraventricular tachycardia is common in patients with right atrial isomerism and can occur during the prenatal stage. Drugs to slow conduction through the AV node may help to terminate the tachycardia. Radiofrequency ablation is a safe and effective treatment alternative to eliminate tachycardia.

Transcatheter closure of moderate to large patent ductus arteriosus with the Amplatzer duct occluder

To investigate the acute and follow‐up results of transcatheter closure of moderate to large patent ductus arteriosus (PDA) with the Amplatzer duct occluder.

Rheumatic fever recurrences: controlled study of 3-week versus 4-week benzathine penicillin prevention programs

To compare the merits of 3-week versus 4-week injections of benzathine penicillin G in preventing recurrence of rheumatic fever, 179 patients aged 4 to 19 years were assigned to one of the two programs. Age, weight, cardiac status, and streptococcal infections among the patients and their family members studied in each program were comparable. Eight-two patients and their family members were monitored for streptococcal infections. Compliance in the two programs was comparable. Of the 63 patients who stayed in the 4-week program, RF recurred in six, as a result of prophylaxis failure in five and associated with partial compliance in one. Of the 90 patients in the 3-week program, RF recurred in one, associated with partial compliance; no failures occurred (P = 0.01). We recommended that for RF chemoprophylaxis in individuals at great risk, regardless of age, benzathine penicillin injections should be administered every 3 rather than every 4 weeks.

LONG-TERM OUTCOME OF PATIENTS WITH RHEUMATIC FEVER RECEIVING BENZATHINE PENICILLIN G PROPHYLAXIS EVERY THREE WEEKS VERSUS EVERY FOUR WEEKS

OBJECTIVE To compare the efficacy of injections of 1.2 million units of benzathine penicillin G given every 3 weeks versus every 4 weeks for secondary prevention of rheumatic fever, based on the long-term outcome of patients receiving such prophylaxis. METHODS A total of 249 consecutive patients with rheumatic fever, randomly assigned to either a 3-week or a 4-week regimen, were examined every 3 to 6 months, and followed for 794 and 775 patient-years, respectively. RESULTS Compliance with each regimen was comparable: 83 (66.9%) of 124 patients in the 3-week group versus 92 (73.6%) of 125 patients in the 4-week group stayed in the program (p > 0.05). Streptococcal infections occurred less frequently in those receiving the 3-week regimen: 7.5 versus 12.6 per 100 patient-years (p < 0.01). Prophylaxis failed in 2 patients receiving the 3-week regimen and in 10 receiving the 4-week regimen (0.25 and 1.29 per 100 patient-years respectively; p = 0.015). Serum penicillin levels were adequate (> or = 0.02 micrograms/ml) in 100 (56%) of 179 samples obtained 21 days after penicillin injection in the 3-week regimen, and in 51 (33%) of 155 samples obtained 28 days after injection in the 4-week regimen (p < 0.01). Of 71 patients with mitral regurgitation in the 3-week regimen, 47 (66%) no longer had the murmur; of 87 patients in the 4-week regimen, 40 (46%) no longer had the murmur (p < 0.05). CONCLUSIONS This 12-year controlled study indicates that the outcome of patients with rheumatic fever is better with a 3-week than with a 4-week penicillin prophylaxis regimen. Greater emphasis and more widespread use of the 3-week regimen should be recommended.

Posttransfusion hepatitis C virus infection in children

Hepatitis C virus (HCV) infection was investigated retrospectively and prospectively in children who had open heart surgery. The retrospective study included 196 patients who had open heart surgery 2 to 6 years before enrollment and were regularly followed. Antibody to HCV was detected in eight cases, and seven of these eight patients had viremia caused by HCV infection. The other patients anti-HCV titer gradually decreased and HCV ribonucleic acid was absent throughout the course. Four of these eight had persistent viral replication without clinical evidence of hepatitis; the other three had viremia and chronic hepatitis. The HCV infection rate in the retrospective study group was about 4%. The HCV was of genotype 2 in five cases and of genotype 3 in two cases. The clinical outcome was not related to sex, age, amount of transfusion, or HCV genotype. For the prospective study, we enrolled 94 patients. Of the 56 enrolled after the initiation of anti-HCV screening in the blood bank, none was infected by HCV. Of the 94 patients, 38 were enrolled before screening; 4 had abnormal liver function 1 to 3 months after operation, and 2 were infected with HCV. One patient had an acute but resolving course; the other had persistent anti-HCV and HCV viremia, although the liver function test results returned to the normal range. The HCV in both cases belonged to genotype 2. We conclude that in Taiwan the seroconversion rate of HCV in children who underwent open heart surgery was 4% to 5%. Anti-HCV screening in donor blood significantly decreased the incidence of posttransfusion hepatitis C.

Cardiac rhythm disturbances in patients with left atrial isomerism.

WU, M.‐H., et al.: Cardiac Rhythm Disturbances in Patients with Left Atrial Isomerism. This long‐term study sought to determine the clinical implication of defective sinus node and AV conduction tissue in patients with left atrial isomerism (LAI). From 1984 to 1998, a total of 22 patients were identified as LAI. Patient age at the last follow‐up ranged from 2 to 276 months (90 ± 70 months). Associated cardiac anomalies were interruption of the inferior vena cava (n = 18, 82%), common atrium (n = 9, 41%), AV canal (n = 14, 64%), double‐outlet right ventricle (n = 8, 36%), and pulmonary stenosis (n = 15, 68%). Palliative interventions were performed in 16 patients (Fontan‐type operation in 4 patients, shunt followed by Fontan‐type operation in 2, repair of septal defect in 4, and extracardiac intervention in 6). During the follow‐up, over half of the patients (n = 14, 64%) developed bradyarrhythmia (onset age: from 1 to 264 months; median 78 months): junctional rhythm (n = 11), sinus bradycardia (n= 8) (5 patients also had junctional rhythm), and AV block (n = 2, both also had junctional rhythm). The probability free from bradyarrhythmia was 80% and 46% at the age of 2 and 6 years, respectively. None of the bradyarrhythmias were directly related to open‐heart surgery. Besides, junctional ectopic tachycardia occurred after Fontan‐type operation in three of six patients. In two patients, a Mahaim‐like pathway was identified during the electrophysiological study. The patients with LAI had a high probability of developing bradyarrhythmias due to abnormal sinus node function. Varied AV conduction abnormalities may include compromised AV conduction, junctional ectopic tachycardia after Fontan‐type operation, and an association of Mahaim‐like pathway.

Quality of life in adults with congenital heart disease: biopsychosocial determinants and sex-related differences

Objectives To assess the quality of life (QoL) in adults with congenital heart disease (ACHD) and to explore the sex-related differences and biopsychosocial determinants in an Asian cohort. Design Prospective cross-sectional clinical study. Setting One tertiary medical centre in Taiwan. Patients and methods The QoL of ACHD (≥20 years) was investigated using the Taiwanese version of the QoL questionnaire designed by the WHO, which assesses four domains of QoL (physical, psychological, social and environmental). Personality, psychological distress and family support were assessed using the Maudsley Personality Inventory, Brief Symptom Rating Scale, and the Family APGAR score, respectively. Results A total of 289 patients (age 33.2±10.6 years; 36% men) were studied. ACHD women had significantly lower QoL scores in the physical and psychological domains compared to the age-matched general population, whereas no differences were observed between ACHD men and the general population. Multivariate analysis showed that female gender was associated with poorer physical QoL; the sex difference in the psychological QoL was mediated by psychological distress. Interaction analysis showed that the effect of family support on the psychological domain of QoL may be different by sex. The determinants of QoL varied between different domains. Extroversion personality trait, psychological distress and family support were common determinants of most domains of QoL. Conclusions In ACHD, female gender was associated with poor physical and psychological QoL. The common denominators for QoL were primarily personality trait, psychological distress and family support, but not disease severity.

Usefulness of Electron Beam Computed Tomography in Children With Heterotaxy Syndrome

Children with heterotaxy syndrome frequently have complex cardiac and noncardiac malformations requiring detailed diagnostic evaluation for management planning. Direct delineation of these structures by electron beam computed tomography (EBCT) is validated as a means of diagnosis. From July 1995 to March 1997, 32 patients (16 girls, 16 boys) with clinically impressed heterotaxy syndrome were enrolled in this study. After evaluation by echocardiography, EBCT studies were performed. Interpretation of cardiac anomalies was performed by sequential analysis based on these cross-sectional images. The diagnoses were subsequently confirmed by angiocardiography and surgical findings. Twenty-eight patients had bilateral trifurcated bronchi, and most of these (24 of 28) did not have a spleen. Four patients had bilateral bifurcated bronchi, 2 patients had polysplenia, and the other 2 patients had a lobulated single spleen. We found that laterality could be identified by EBCT in all patients. Comparison of diagnostic yield between echocardiography, catheterization, and EBCT showed that EBCT is superior to echocardiography and catheterization in demonstration of pulmonary venous anatomy and presence of a very small rudimentary ventricle. In addition, associated visceral, bronchopulmonary, mediastinal, and intracardiac anomalies could all be clearly delineated by EBCT at the same time. Thus, EBCT is a promising complementary modality for an overall understanding of heterotaxy syndrome.

Outcomes of transcatheter valvotomy in patients with pulmonary atresia and intact ventricular septum

The results of transcatheter valvotomy in pulmonary atresia with intact ventricular septum (PA-IVS) patients are presented with an attempt to identify the predictive factors for pulmonary valvotomy alone as definitive treatment. Between June 1995 and December 1997, 14 PA-IVS neonates with tripartite right ventricle underwent an attempted pulmonary valvotomy. For perforation of the pulmonary valve, a guidewire was used in 4, and a radiofrequency guidewire in 10 patients. Two outcome groups were identified. Group I included those in whom transcatheter treatment achieved a definitive success; group II patients required surgery despite an initial successful valvotomy. The attempt failed in 3 patients, 1 of whom had pericardial effusion. Perforation of the pulmonary valve was achieved in 11 patients: 2 with a guidewire and 9 with a radiofrequency guidewire. A subsequent balloon valvuloplasty was performed in these 11 patients. After valvuloplasty, mean right ventricular pressure decreased from 124 +/- 24 to 60 +/- 15 mm Hg (p <0.01). One died of heart failure and infection 10 days later, despite successful weaning from prostaglandin E1. Group I patients (n = 6) were treated with transcatheter valvotomy alone. Group II patients (n = 4) required right ventricular outflow patch. Significant differences between the 2 groups (group I vs II) were identified in tricuspid valve Z value (0.52 +/- 0.37 vs -1.25 +/- 0.48, p <0.05), pulmonary valve Z value (-3.47 +/- 0.59 vs -5.43 +/- 0.94, p <0.05), and ratio of right-to-left ventricular area on the apical 4-chamber view (0.73 +/- 0.06 vs 0.49 +/- 0.03, p <0.05). There were no significant differences in hemodynamic characteristics between the 2 groups. After a follow-up period ranging from 7 to 35 months (mean 18 +/- 10.3), the most recent echocardiograms in the 10 patients showed a mean pressure gradient across the pulmonary valve of 17 +/- 15 mm Hg. All 10 patients had an oxygen saturation of >92%. Transcatheter valvotomy using a radiofrequency guidewire is a safe and effective treatment in selected patients with PA-IVS. Transcatheter valvotomy can be a definitive treatment in PA-IVS patients with a tricuspid valve Z value > or = -0.1, pulmonary valve Z value > or = -4.1 and ratio of right-to-left ventricular area > or = 0.65.