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1.
Eur J Pediatr ; 182(2): 633-640, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36434403

RESUMEN

The long-term prognosis of patients with Kawasaki disease (KD) complicated by coronary artery aneurysms (CAA) is still unclear. The present, multicenter registry study aimed to study the factors associated with coronary events (CE) and determine an appropriate management method for patients with KD complicated with CAA. Patients with KD with onset after 2015 and with a medium-sized or large CAA having an actual diameter ≥ 4 mm or a Z-score ≥ 5.0 at 30 days and later after KD onset were included in the annual survey. The primary endpoint was the time-dependent incidence of CE. Associated factors were also examined. In total, 179 patients from 53 centers were enrolled and followed up for a median of 501 days. The median age at KD onset was 2.2 years, 137 patients were male (77%), 47 had incomplete KD (26%), and 36 had large CAA (20%). CE occurred in 13 patients (7%; 95% confidence interval: 4-12%); eight (62%) experienced CE within 1 year, and all the patients experienced a CE within 2 years. All but one patient received antiplatelet drugs and warfarin. Patients with a large CAA had significantly more CAA (2.8 vs. 1.7, p < 0.001), more cases of warfarin use (86% vs. 43%, p < 0.001), and were more likely to have CE (28% vs. 2%, p < 0.001) than those with a medium-sized CAA. On univariate Cox regression analysis, the factors significantly associated with CE were large CAA (hazard ratio (HR): 17.0), three or more CAA (HR: 23.3), and beaded CAA (HR: 15.9). Multivariable Cox regression analysis revealed that the only associated factor was a large CAA. CONCLUSION: Patients with a large CAA were more likely to have a CE within 2 years. Antithrombotic therapy with warfarin did not eliminate the CE risk, and better therapies are desirable. WHAT IS KNOWN: • Coronary artery aneurysms are a serious complication of Kawasaki disease, and coronary events are sometimes fatal. • In previous, retrospective studies in Japan, large aneurysms, male sex, and refractoriness to initial immunoglobulin therapy were considered risk factors for coronary events. WHAT IS NEW: • Of 179 patients with a medium sized or large aneurysm, 13 (7%) experienced coronary events, all of which occurred within 2 years of onset. Factors significantly associated with coronary events were large aneurysms, three or more aneurysms, and beaded aneurysms.


Asunto(s)
Aneurisma Coronario , Síndrome Mucocutáneo Linfonodular , Humanos , Masculino , Lactante , Preescolar , Femenino , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/epidemiología , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Estudios Retrospectivos , Warfarina/uso terapéutico , Vasos Coronarios , Aneurisma Coronario/epidemiología , Aneurisma Coronario/etiología , Inmunoglobulinas Intravenosas/uso terapéutico
2.
Pediatr Int ; 65(1): e15543, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37493267

RESUMEN

BACKGROUND: Kawasaki disease (KD) presents with gallbladder enlargement (GBE) or hydrops in the acute phase. Although GBE is highly specific to KD, epidemiological data on GBE have not been updated. In this study we evaluated the occurrence rate of GBE in KD and characterized the clinical features of patients who developed GBE. METHODS: The present study was a prospective, observational study. The maximum longitudinal area of the gallbladder and the common bile duct diameter in KD patients were measured by ultrasonography at the start of initial intravenous immunoglobulin (IVIG) therapy (day 0) and on days 7, 30, and 60 of therapy. The primary outcome was the complication rate of GBE (z- score ≥2.0) on day 0. The secondary outcome was the association of GBE with cholestasis, unresponsiveness to IVIG, and coronary artery lesions (CAL). RESULTS: Gallbladder enlargement occurred in 35% (35/101) of patients on day 0. Cholestasis and severe patients (Kobayashi score [KS] ≥5) were more common in the GBE group (20.6% vs. 1.6%, p = 0.002, and 54.3% vs. 15.2%, p < 0.001, respectively). In patients with a KS of ≤4, the non-responder rate was higher in the GBE group (44% vs. 20%, p = 0.0495) but did not differ in those with a KS of ≥5 (21% vs. 20%, p = 0.95). The rate of CAL occurrence did not differ significantly between the groups (8.6% vs. 6.1%, p = 0.64). CONCLUSIONS: Gallbladder enlargement occurred in 35% of KD patients and was associated with cholestasis. Gallbladder enlargement may not only be a diagnostic finding, but also a severity marker in KD patients.


Asunto(s)
Colestasis , Enfermedad de la Arteria Coronaria , Síndrome Mucocutáneo Linfonodular , Humanos , Lactante , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Vesícula Biliar/diagnóstico por imagen , Estudios Prospectivos , Estudios Retrospectivos
3.
J Pediatr ; 240: 158-163.e4, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34461064

RESUMEN

OBJECTIVE: To detect risk factors of coronary artery aneurysm (CAA) development in patients with Kawasaki disease determined to have a low risk for resistance to primary intravenous immunoglobulin (IVIG) treatment based on the Kobayashi score. STUDY DESIGN: This study included 1757 predicted IVIG responders from Prospective Observational study on STRAtified treatment with Immunoglobulin plus Steroid Efficacy for Kawasaki disease (Post RAISE), a large-scale, multicenter, prospective cohort study of Kawasaki disease in Japan. Predicted IVIG responders were defined as patients with Kawasaki disease with a Kobayashi score of <5, a predictive scoring system for IVIG resistance created in Japan. The primary outcome was CAA development at 1 month after disease onset. CAA was defined as a Z score of ≥2.5. Multivariable logistic regression was used to identify the independent risk factors of CAA. The variables for inclusion were identified based on univariate analysis results and previously reported risk factors of CAA. RESULTS: Among 1632 patients who had complete coronary outcome data, CAA developed in 90 patients (5.5%) at 1 month after disease onset. Multivariable analysis found that a baseline maximum Z score of >2.5, age of <12 months at fever onset, and nonresponsiveness to IVIG were significant, independent risk factors of CAA development at 1 month after disease onset. Among the risk factors, a baseline maximum Z score of >2.5 was most strongly associated with CAA development (OR, 7.1; 95% CI, 4.1-12.2; P ≤ .001). CONCLUSIONS: Predicted IVIG responders with CAA risk factors identified in this study may be candidates for future clinical trials of intensified primary IVIG treatment with prednisolone, cyclosporine or infliximab.


Asunto(s)
Aneurisma Coronario/etiología , Inmunoglobulinas Intravenosas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Preescolar , Aneurisma Coronario/epidemiología , Resistencia a Medicamentos , Femenino , Humanos , Inmunoglobulinas Intravenosas/efectos adversos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/efectos adversos , Lactante , Japón , Masculino , Síndrome Mucocutáneo Linfonodular/epidemiología , Estudios Prospectivos , Medición de Riesgo , Factores de Riesgo
4.
J Pediatr ; 244: 133-138.e1, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-34798077

RESUMEN

OBJECTIVE: To evaluate the optimal duration of echocardiographic follow-up in patients with Kawasaki disease without an initial coronary aneurysm. STUDY DESIGN: In this single-center, retrospective, observational study, we reviewed the results of follow-up echocardiography in children with Kawasaki disease enrolled in the Prospective Observational Study on Stratified Treatment with Immunoglobulin Plus Steroid Efficacy for Kawasaki Disease from a children's hospital. The main enrollment criterion was the absence of coronary aneurysms, defined as a maximum z-score (Zmax) ≥2.5, in the proximal right coronary artery and the proximal left anterior descending artery within 9 days from treatment initiation. The primary outcome was Zmax on follow-up echocardiography at up to 5 years. RESULTS: Among 386 patients, 106 (27.5%) received prednisolone with intravenous immunoglobulin for first-line therapy, and 57 (14.8%) showed a poor response. Echocardiography at 1 month detected 9 patients with a Zmax ≥2, including 3 (0.8%) with coronary aneurysms requiring additional antithrombotic treatment and observation. Of 7 patients (1.8%) with normal echocardiographic findings at 1 month but a Zmax ≥2 later, 2 were lost to follow-up and 5 experienced spontaneous resolution, but none of the 7 patients required any change in management. CONCLUSIONS: The optimal duration of echocardiographic follow-up may be 1 month in patients with no initial coronary aneurysms and a Zmax <2 at 1 month. Coronary artery abnormalities observed after 1 month are rare and mostly benign in this category of patients.


Asunto(s)
Aneurisma Coronario , Síndrome Mucocutáneo Linfonodular , Niño , Aneurisma Coronario/diagnóstico por imagen , Aneurisma Coronario/etiología , Vasos Coronarios/diagnóstico por imagen , Ecocardiografía , Estudios de Seguimiento , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Lactante , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico por imagen , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Estudios Retrospectivos
5.
Circ J ; 87(1): 130-138, 2022 12 23.
Artículo en Inglés | MEDLINE | ID: mdl-35979562

RESUMEN

BACKGROUND: The prospective Control of HEART rate in inFant and child tachyarrhythmia with reduced cardiac function Using Landiolol (HEARTFUL) study investigated the effectiveness and safety of landiolol, a short-acting ß1 selective blocker, in children.Methods and Results: Twenty-five inpatients aged ≥3 months to <15 years who developed supraventricular tachyarrhythmias (atrial fibrillation, atrial flutter, supraventricular tachycardia, and inappropriate sinus tachycardia) were treated with landiolol. The primary endpoint, the percent of patients with a reduction in heart rate ≥20% from the initial rate of tachycardia, or termination of tachycardia at 2 h after starting landiolol, was achieved in 12/25 patients (48.0%; 95% CI 28.4-67.6), which exceeded the predetermined threshold (38.0%). At 2 h after starting landiolol administration, heart rate had decreased by ≥20% in 45.8% (11/24) and recovery to sinus rhythm was achieved in 40.0% (6/15) of the patients. Adverse reactions (ARs) occurred in 24.0% (6/25) of patients, and the study was discontinued in 4.0% (1/25) of the patients; however, none of these ARs were considered serious. The most common AR was hypotension (20.0% [5/25] of patients). CONCLUSIONS: The HEARTFUL study has demonstrated the efficacy of landiolol, by reducing heart rate or terminating tachycardia, in pediatric patients with supraventricular tachyarrhythmias. Although serious ARs and concerns were not identified in this study, physicians should be always cautious of circulatory collapse due to hypotension.


Asunto(s)
Fibrilación Atrial , Hipotensión , Humanos , Niño , Lactante , Frecuencia Cardíaca , Estudios Prospectivos , Taquicardia/tratamiento farmacológico , Urea/efectos adversos , Antagonistas Adrenérgicos beta/efectos adversos
6.
Radiographics ; 42(1): 268-288, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-34826255

RESUMEN

Kawasaki disease (KD) is a common pediatric vasculitis syndrome involving medium- and small-sized arteries that is especially prevalent in early childhood (ie, age 6 months to 5 years). The diagnosis of KD is made on the basis of clinical features, such as fever, characteristic mucocutaneous changes, and nonsuppurative cervical lymphadenopathy. However, early diagnosis is often challenging because many children with KD present with atypical symptoms. The most serious complication of KD is coronary artery aneurysm caused by coronary arteritis. Prompt intravenous immunoglobulin therapy reduces the risk of cardiac morbidity. In addition, the systemic extension of KD-related vasculitis during the acute phase causes a variety of multisystem manifestations, including encephalopathy, stroke, retropharyngeal edema, pericarditis, myocarditis, KD shock syndrome, pulmonary lesions, intestinal pseudo-obstruction, gallbladder hydrops, arthritis, and myositis. These complications tend to be more common in affected children with atypical presentation. Radiologists can play an important role in the timely identification of diverse KD-associated morbidities and thus may contribute to the early diagnosis of atypical KD. Online supplemental material is available for this article. ©RSNA, 2021.


Asunto(s)
Aneurisma Coronario , Enfermedad de la Arteria Coronaria , Síndrome Mucocutáneo Linfonodular , Niño , Preescolar , Edema , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/diagnóstico por imagen
7.
Heart Vessels ; 37(6): 1075-1084, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-34799788

RESUMEN

There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty institutions participated. A total of 180 patients were identified. The median age at diagnosis was 5.8 years old and median duration of observation was 8.3 years. Although six patients (3%) deteriorated into the dilated phase of HCM, no patient received heart transplantation. Freedom from death at 1, 5, 10, and 20 years were 97%, 92%, 84%, and 80%, respectively. There were 26 deaths. Among them, 11 patients died suddenly, presumably due to arrhythmia, and 15 patients died of heart failure. The presence of heart failure symptoms and a greater cardiothoracic ratio were significant risk factors for heart failure-related death. There were no significant risk factors identified for arrhythmia-related death. In conclusion, the prognosis of pediatric HCM in Japan is good and similar to those reported in population-based studies in the United States and Australia. Significant risk factors for heart failure-related death were identified in pediatric patients with HCM in Japan.


Asunto(s)
Cardiomiopatía Hipertrófica , Insuficiencia Cardíaca , Arritmias Cardíacas/complicaciones , Cardiomiopatía Hipertrófica/complicaciones , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/terapia , Niño , Preescolar , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/terapia , Humanos , Japón/epidemiología , Estudios Retrospectivos
8.
Pediatr Cardiol ; 2022 Nov 27.
Artículo en Inglés | MEDLINE | ID: mdl-36436005

RESUMEN

Although the treadmill and cycle ergometer are commonly used for exercise stress electrocardiography (ECG) testing, they are often difficult to perform with children. We herein evaluated the utility and safety of the 2-minute jump test (2MJT) as a simple, alternative exercise test. One hundred patients, including 60 male patients, with an average age at study commencement of 10.7±3.5 years (mean±standard deviation) and with no exercise restriction who underwent a cardiac check-up between November 2020 and March 2022 at the study center were included. After recording their resting ECG, they jumped for 2 minutes during ECG recording, and the change in heart rate (HR), ECG findings, and occurrence of adverse events were investigated. As a result, patients jumped 185±60 times in two minutes, and their HR increased from 76±13 beats/min at rest to 172±18 beats/min at peak during the test. Ninety (90%) patients attained the ideal target HR of > 150 beats/minute. During the recovery period after loading, five patients had abnormal ECG findings (ventricular extrasystoles, second-degree atrioventricular block, and atrial extrasystoles in two, two, and one patient, respectively) but completely resolved spontaneously within three minutes. Our findings suggested that the 2MJT is a useful and safe exercise test capable of inducing sufficient increase in HR in a short time in children.

9.
J Pediatr ; 236: 108-112.e5, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34004190

RESUMEN

OBJECTIVES: To evaluate electrocardiogram markers to predict coronary involvement in patients with Kawasaki disease by assessing measures of ventricular repolarization parameters on the 12-lead electrocardiogram. STUDY DESIGN: This cross-sectional study included 180 Spanish and Japanese patients ≤14 years of age with Kawasaki disease, with or without coronary involvement, from 2011 to 2016. We manually measured the Tp-Te/QT ratio and QTc interval (with Bazett's formula) in 12-lead electrocardiogram in the acute and recovery period and explored their potential association with coronary involvement. RESULTS: No association was found between Tp-Te/QT ratio obtained manually in V5 and V6 leads and coronary involvement in the acute (V5:0.25 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .80; V6:0.24 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .86) or the recovery (V5: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.19-0.25], P = .68; V6: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.17-0.25], P = .50) period. By contrast, QTc in V5 and V6 was significantly lower in patients with Kawasaki disease and coronary involvement in the acute period (V5: 378 ms [IQR, 364-395 ms] vs 390 ms [IQR, 371-411 ms], P = .04; V6: 377 ms [IQR, 364-392 ms] vs 390 ms [IQR, 371-410 ms], P = .01). A QTc interval of <385 ms in lead V6 was associated with a 2.5-fold increased risk of coronary involvement (OR, 2.5; 95% CI, 1.2-5.3; P = .02). CONCLUSIONS: Manually measured QTc interval may be a marker of coronary disease in the acute period of Kawasaki disease.


Asunto(s)
Cardiopatías/diagnóstico , Cardiopatías/etiología , Síndrome Mucocutáneo Linfonodular/complicaciones , Síndrome Mucocutáneo Linfonodular/fisiopatología , Preescolar , Estudios Transversales , Electrocardiografía , Femenino , Sistema de Conducción Cardíaco/fisiopatología , Cardiopatías/fisiopatología , Ventrículos Cardíacos/fisiopatología , Humanos , Lactante , Masculino , Síndrome Mucocutáneo Linfonodular/metabolismo , Reproducibilidad de los Resultados
10.
J Pediatr ; 237: 96-101.e3, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34147499

RESUMEN

OBJECTIVES: To test the performance of the Son risk score, which was created to predict coronary artery abnormalities from baseline variables in North American patients with Kawasaki disease. STUDY DESIGN: The dataset from Post RAISE, the largest prospective cohort study of Japanese patients with Kawasaki disease to date, was used for the present study. With high risk defined as ≥3 points, sensitivity, specificity, positive predictive value, and negative predictive value for coronary artery abnormality development were calculated. To evaluate the effect of each risk factor in the Son score, the OR and 95% CIs were calculated using logistic regression analysis with the presence of coronary artery abnormality at 1 month after disease onset. RESULTS: Post RAISE enrolled 2628 consecutive patients with Kawasaki disease, and 304 patients had a high-risk score, of whom 15.1% showed coronary artery abnormality. At the cutoff ≥3 points, the sensitivity was 37.7%, and the specificity was 87.2%. The maximum z score at baseline ≥2.0 (OR 3.5, 95% CI 2.3-5.2) and age <6 months at disease onset (OR 3.2, 95% CI 1.9-5.4), were significantly associated with coronary artery abnormality development. However, a high concentration of C-reactive protein was not associated with coronary artery abnormality. The area under the receiver operating characteristic curve for the Son score was 0.65 (95% CI 0.59-0.71). CONCLUSIONS: The Son score had insufficient sensitivity and good specificity in a Japanese cohort of patients with Kawasaki disease. Among the variables comprising the Son score, a large baseline z score and young age at disease onset were significant, independent predictors of coronary artery abnormality development.


Asunto(s)
Reglas de Decisión Clínica , Aneurisma Coronario/diagnóstico , Aneurisma Coronario/etiología , Síndrome Mucocutáneo Linfonodular/complicaciones , Índice de Severidad de la Enfermedad , Niño , Preescolar , Femenino , Humanos , Lactante , Japón , Modelos Logísticos , Masculino , Estudios Prospectivos , Medición de Riesgo , Factores de Riesgo , Sensibilidad y Especificidad
11.
J Cardiovasc Electrophysiol ; 32(2): 279-286, 2021 02.
Artículo en Inglés | MEDLINE | ID: mdl-33382508

RESUMEN

INTRODUCTION: Given that few studies investigated the efficacy of catheter ablation (CA) in patients with paroxysmal atrial fibrillation (AF) and atrial septal defect (ASD), this study evaluated its effectiveness in patients with paroxysmal AF and ASD. METHODS AND RESULTS: Of the 216 patients who underwent ASD device closure at two hospitals, 36 patients had paroxysmal AF. After April 2012, CA for AF was performed before ASD device closure (ASD-CA group; n = 20). The ASD-CA group had a significantly higher AF-free survival rate after ASD device closure compared to patients without CA for AF before ASD device closure (ASD-non-CA group; n = 16) (ASD-CA group: 2 patients vs. ASD-non-CA group: 9 patients; follow-up period: 4.2 ± 2.5 years; log-rank p = .01). In addition, the AF-free survival rates were similar between the ASD-CA group and 80 paroxysmal AF patients who underwent CA without any detectable structural heart disease (non-SHD-CA group). The two groups were matched by propensity scores for age, sex, and left atrium dimension (ASD-CA group: 2 patients vs. non-SHD-CA group: 5 patients; follow-up period: 3.3 ± 1.8 years; log-rank p = .28). CONCLUSION: CA for AF before ASD device closure might be an effective treatment option for patients with paroxysmal AF and ASD.


Asunto(s)
Fibrilación Atrial , Ablación por Catéter , Defectos del Tabique Interatrial , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/cirugía , Ablación por Catéter/efectos adversos , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/diagnóstico , Defectos del Tabique Interatrial/cirugía , Humanos , Resultado del Tratamiento
12.
Circ J ; 86(1): 128-135, 2021 12 24.
Artículo en Inglés | MEDLINE | ID: mdl-34657926

RESUMEN

BACKGROUND: The correlation between the Z-score of the left ventricular (LV) diameter and the LV volume-overload due to pulmonary over-circulation in children with ventricular septal defect (VSD) or patent ductus arteriosus (PDA) remains unclear.Methods and Results:The present, retrospective study enrolled 70 children (aged 0.3-16.8 years; 33 males, 37 females) with a diagnosis of isolated VSD and/or PDA who underwent cardiac catheterization (CC) between 2015 and 2019. Patients with chromosomal/genetic anomalies, growth disorder, right-ventricular enlargement or other conditions causing LV enlargement were excluded. Echocardiographic parameters were retrospectively evaluated from the medical records, converted to a Z-score, then compared with CC data. The pulmonary-systemic flow ratio on CC (cQp/Qs) correlated significantly with the Z-score of both the LV end-diastolic diameter (Zd) (r=0.698, P<0.0001) and LV end-systolic diameter (r=0.593, P<0.0001). Regression analysis and curve-fitting were used to predict the cQp/Qs based on the Zd, and a significant regression equation was found on cubic regression (R2of 0.524, P<0.0001) showing a strong correlation with the cQp/Qs (r=0.724, P<0.0001). CONCLUSIONS: The Z-score of the LV diameter can be a useful, non-invasive marker for evaluating LV volume overload and determining the surgical indications in children with VSD or PDA because of its strong correlation with the cQp/Qs.


Asunto(s)
Conducto Arterioso Permeable , Insuficiencia Cardíaca , Defectos del Tabique Interventricular , Niño , Conducto Arterioso Permeable/diagnóstico por imagen , Ecocardiografía/métodos , Femenino , Defectos del Tabique Interventricular/diagnóstico por imagen , Ventrículos Cardíacos , Humanos , Masculino , Estudios Retrospectivos
13.
Circ J ; 86(1): 109-115, 2021 12 24.
Artículo en Inglés | MEDLINE | ID: mdl-34588404

RESUMEN

BACKGROUND: There has been no nationwide survey on the prognosis of pediatric dilated cardiomyopathy (DCM) in Japan. Therefore, we designed this retrospective multicenter study to investigate the long-term survival rate in pediatric patients with DCM in Japan.Methods and Results:In this multicenter retrospective observational study, data were reviewed for 106 patients aged <18 years who had been diagnosed with DCM at any 1 of 18 Japanese institutions between 1990 and 2014. The median age at diagnosis was 2.0 years and the median duration of observation was 3.3 years. Most DCM patients were diagnosed because of symptoms of heart failure. On echocardiography, the median left ventricular end-diastolic dimension z score was 5.4 and fractional shortening was 0.10. Freedom from death or transplantation rates at 1, 3, 5, 10, and 20 years after diagnosis were 76%, 66%, 64%, 58%, and 43%, respectively. Freedom from death rates at 1, 5, 10, and 20 years after diagnosis were 81%, 75%, 72%, and 53%, respectively. The incidence of heart transplantation at 1, 5, 10, and 20 years after diagnosis was 6%, 15%, 20%, and 20%, respectively, suggesting that only 15% of patients in Japan underwent heart transplantation within 5 years of diagnosis. CONCLUSIONS: In Japan, the prognosis of pediatric DCM is poor and the rate of heart transplantation is low.


Asunto(s)
Cardiomiopatía Dilatada , Insuficiencia Cardíaca , Trasplante de Corazón , Niño , Trasplante de Corazón/efectos adversos , Humanos , Japón/epidemiología , Pronóstico , Estudios Retrospectivos
15.
Acta Paediatr ; 107(6): 1049-1054, 2018 06.
Artículo en Inglés | MEDLINE | ID: mdl-29385646

RESUMEN

AIM: We examined whether the paediatric assessment triangle (PAT) could predict the severity of Kawasaki disease. METHODS: We enroled patients diagnosed with Kawasaki disease between July 2012 and June 2016 at the emergency department of Tokyo Metropolitan Children's Medical Center in Tokyo, Japan. Triage nurses assigned participants to unstable or stable PAT groups. We compared the incidence of coronary artery aneurysms (CAA), the Kobayashi score, which measures resistance to intravenous immunoglobulin treatment, and the incidence of initial treatment resistance. RESULTS: Of the 420 participants, who were aged 0-145 months with a mean age of 31.2 ± 23.9 months, 66 (16%) were assigned to the unstable PAT group. The incidence of CAA was similar between the two groups. The percentage of unstable PAT group participants with a Kobayashi score of at least five points (39 versus 18%, p < 0.001) and initial treatment resistance (25 versus 15%, p = 0.047) were significantly higher than in the stable PAT group. Unstable PAT was an independent risk factor for initial treatment resistance (odds ratio 2.02, 95% confidence interval 1.05-3.90, p = 0.035). CONCLUSION: An unstable PAT was able to predict the severity of Kawasaki disease when measured by a higher rate of initial treatment resistance.


Asunto(s)
Síndrome Mucocutáneo Linfonodular , Índice de Severidad de la Enfermedad , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos
16.
Kyobu Geka ; 71(3): 190-194, 2018 Mar.
Artículo en Japonés | MEDLINE | ID: mdl-29755072

RESUMEN

We experienced 2 cases of primary pulmonary vein stenosis(PVS),which developed after a bidirectional Glenn procedure was performed for complex heart defects with normal pulmonary venous return. Although the patients successfully underwent primary sutureless repair for left PVS, restenosis of the affected pulmonary veins occurred several months after surgery in both patients. Stent implantation followed by balloon angioplasty was performed for stent stenosis in 1 patient without effect. However, the patient later underwent a successful fenestrated Fontan procedure. Catheter intervention was contraindicated in the 2nd patient due to almost complete obstruction of the left pulmonary veins with upstream hypoplasia. To improve the results of PVS treatment, earlier diagnosis by quantitative lung perfusion scintigraphy, magnetic resonance imaging, and close echocardiographic observation together with earlier, aggressive treatment combining surgery and catheter interventions are recommended.


Asunto(s)
Cardiopatías Congénitas/cirugía , Complicaciones Posoperatorias/cirugía , Estenosis de Vena Pulmonar/etiología , Estenosis de Vena Pulmonar/cirugía , Femenino , Procedimiento de Fontan , Humanos , Recién Nacido , Masculino , Resultado del Tratamiento
17.
J Pediatr ; 185: 106-111.e1, 2017 06.
Artículo en Inglés | MEDLINE | ID: mdl-28343657

RESUMEN

OBJECTIVE: To identify the prevalence of bradycardia associated with use of prednisolone in patients with Kawasaki disease and analyze the association between bradycardia and responsiveness to intravenous immunoglobulin (IVIG). STUDY DESIGN: We performed a retrospective cohort study of 176 patients with severe Kawasaki disease admitted to the Tokyo Metropolitan Children's Medical Center between March 2010 and December 2015. The group treated with IVIG plus prednisolone therapy from February 2012 was compared with the control group who received IVIG monotherapy before this date. The primary outcome was the prevalence of bradycardia, defined as heart rate less than the first percentile for normal children. Next, we determined whether bradycardia was associated with the clinical course in the patient subgroup treated with IVIG plus prednisolone therapy. RESULTS: The prevalence of bradycardia was significantly higher in the IVIG plus prednisolone subgroup than in the IVIG group (79.1% vs 7.1%; P?

Asunto(s)
Bradicardia/inducido químicamente , Glucocorticoides/efectos adversos , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Prednisolona/efectos adversos , Preescolar , Estudios de Cohortes , Quimioterapia Combinada , Femenino , Glucocorticoides/administración & dosificación , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Lactante , Modelos Logísticos , Masculino , Prednisolona/administración & dosificación , Estudios Retrospectivos , Índice de Severidad de la Enfermedad
18.
Circ J ; 82(1): 239-246, 2017 12 25.
Artículo en Inglés | MEDLINE | ID: mdl-28855435

RESUMEN

BACKGROUND: Giant coronary aneurysm is the most severe sequela in Kawasaki disease, occurring in approximately 0.2% of patients in Japan. Regression is rare, while myocardial infarction (MI) and sudden death are relatively common. Herein, we reviewed patients with giant coronary aneurysm in a 10-year period.Methods and Results:A nationwide questionnaire survey was conducted based on a national epidemiological database from 1999 to 2010. We identified 355 giant coronary aneurysm patients, of whom 209 were analyzed. The 5- and 10-year total cardiac event-free rates were 0.72 and 0.68, respectively. Twelve patients died, and MI was observed in 32 patients (18.1%). Five and 6 deaths were due to coronary rupture and MI, respectively. All ruptures occurred within 1 month of onset, while most MI occurred within 18 months. There was no death beyond 2 years. Aneurysm size was significantly related to the occurrence of MI in both the right and left coronary arteries. At the time of writing, 55% of patients had no exercise limitations. And including patients who cannot perform strenuous exercises, 81% of patients were leading ordinary lives. CONCLUSIONS: Severe cardiac events are likely to occur within 2 years from onset of Kawasaki disease, while no deaths occurred beyond this time. Hence, careful monitoring is needed especially for the first 2 years. Most patients with giant coronary aneurysms can lead ordinary lives with appropriate management.


Asunto(s)
Aneurisma Coronario/etiología , Síndrome Mucocutáneo Linfonodular/patología , Niño , Preescolar , Femenino , Humanos , Lactante , Japón/epidemiología , Masculino , Síndrome Mucocutáneo Linfonodular/terapia , Infarto del Miocardio , Evaluación de Resultado en la Atención de Salud , Encuestas y Cuestionarios , Análisis de Supervivencia , Factores de Tiempo
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