Renovascular hypertension due to fibromuscular dysplasia is an uncommon but important cause of pediatric hypertension. It is usually ignored and diagnosed after a long delay because blood pressure is ...infrequently measured in children. We report a case of previously undiagnosed renovascular hypertension complicated with right renal infarction in a 2-year-10-month-old child, who initially presented as a case of conscious disturbance. The patient's brain CT displayed brain stem hemorrhage, and a brain MRI showed acute hemorrhage and multiple old intracerebral hemorrhage. Therefore, intimal fibromuscular dysplasia of the right renal artery was diagnosed by computed tomography and confirmed after renal angiography. Her blood pressure was gradually normalized after medical therapy, including use of Losartan. She is presently asymptomatic on OPD follow-up. The importance of BP measurement can not be overemphasized in pediatric patients less than three years of age with underlying diseases.
Conscious disturbance; Fibromuscular dysplasia; Renovascular hypertension.
Prospective registry studies of congenital heart disease (CHD)-associated pulmonary artery hypertension (PAH) are rare. We established a multicenter registry of CHD-PAH: the TACHYON (TAiwan ...Congenital Heart disease associated with pulmonarY arterial hypertension) registry.
The prospective TACHYON registry was initiated in January 2016. Nine pediatric cardiology centers with 99 patients were included. Using this database, we evaluated clinical characteristics and outcomes.
Twelve patients with incomplete data were excluded. For the remaining 87 patients, mean age of enrollment was 37.4(SD 18.2) years, and the male to female ratio was 60:27. PAH after defect closure accounted for 46 (52.9%) and Eisenmenger syndrome for 30 (34.5%) cases. Atrial septal defect was the most common (48.3%) disease, followed by ventricular septal defect. Mean pulmonary artery pressure was 56.7 (SD 19.4) mmHg. PAH-targeted therapy was used in 95.4% of patients. Sildenafil and bosentan were the most common drugs. After mean 23.9 months of follow-up, the 2-year Kaplan–Meier survival rate was 93.2%. According to univariate Cox regression analysis, significant risk factors included right heart failure signs, symptom progression, high-risk baseline N-terminal pro–brain natriuretic peptide (BNP)/BNP, high-risk baseline 6-min walking distance (6MWD), and high baseline hemoglobin/hematocrit level. Using the three noninvasive parameters (functional class, 6MWD, NT-pro BNP/BNP) proposed by the European Society of Cardiology, the total number of high-risk criteria predicted survival rate reliably.
Using the TACHYON registry is feasible, but the physicians' adherences to guidelines are unsatisfactory. Midterm outcomes of PAH-target therapy are favorable and predictable using noninvasive parameters.
•This is the largest multicenter registry data from Asian country for CHD-PAH.•The physicians' adherence to the ESC guidelines of CHD-PAH patients is unsatisfactory.•We can predict outcome noninvasively by functional class, Pro-BNP, and 6MWD.•The number of high-risk parameters has higher predictive value than low risk ones.
Abstract We aimed to investigate which factors are associated with coronary artery lesions (CALs) during the acute and chronic stages in Taiwanese children with Kawasaki disease (KD). A total of 216 ...children with KD were enrolled. Clinical and laboratory data were obtained for each child within 7 days of illness. The patients were classified into KD children without acute CALs ( n = 135) and those with acute CALs ( n = 81) according to echocardiography data at Week 2 after treatment. Then, KD children with acute CALs were further divided into those without chronic CALs ( n = 55) and with chronic CALs ( n = 26) according to annual echocardiography data. During acute stage of KD, neutrophil count (<54%) odds ratio (OR) = 0.44, p = 0.041; second dose of intravenous immunoglobulin (IVIG) treatment (OR = 5.01, p = 0.009); and platelet count (≤400,000) (OR = 0.42, p = 0.006) were correlated with the risk of acute CALs. During chronic stage of KD, age (12–60 months) (OR = 0.25, p = 0.042); first dose of IVIG treatment (OR = 0.12, p = 0.005); and band count (≥3%) (OR = 3.51, p = 0.032) were correlated with the risk of chronic CALs. Our results suggest that the effects of neutrophil count, doses of IVIG treatment, and platelet count on CALs in acute KD are important. Age, doses of IVIG treatment, and band count are related to the persistence of CALs in chronic stage of KD.
The characteristics of multiple accessory pathways in children have not been previously studied. Records were reviewed of 317 consecutive pediatric patients with Wolff-Parkinson-White syndrome who ...underwent electrophysiologic study and radiofrequency catheter ablation at our institution. Twenty-eight patients (9%) had multiple pathways (a total of 64 pathways: 21 patients had 2, 6 had 3, and 1 patient had 4 pathways). The locations were left free wall (22 pathways), right free wall (19 pathways), posteroseptal (17 pathways), and anteromidseptal (6 pathways). Of these 64 pathways, 55 were ablated successfully without complications, 5 failed ablation, and 4 fasciculoventricular fibers did not require treatment. Three patients had a newly found pathway at the repeat session. Three patients had atrioventricular nodal reentrant tachycardia and the slow pathway area was modified. One patient had failed initial ablation and had a successful ablation using a 3-dimensional electroanatomic mapping system. Compared with patients with a single pathway, those with multiple pathways exhibited a higher incidence of antidromic tachycardia, a shorter anterograde accessory pathway effective refractory period (<250 ms), a longer fluoroscopic time (65 ± 43 vs 39 ± 46 minutes, p <0.05), and a larger number of unsuccessful attempts (9 ± 16 vs 5 ± 8, p <0.05). Success rate (92% vs 93%) and recurrence rate (1.7% vs 2.1%) were similar in both groups. This study demonstrates that multiple pathways are not rare in pediatric patients and that multiple pathways contrast with a single pathway in a variety of conduction properties.
The relationship between infection and Kawasaki disease (KD) remains unclear. Infection has long been considered a key predisposing factor for KD. Bacterial and viral agents may be related to the ...onset of KD because of superantigen and cytokine production. Various bacterial and viral infections have been reported to be associated with KD, but the actual mechanism remains unknown. The higher association between KD and enterovirus has been well documented by using Taiwan National Health Insurance Research Database. However, no evidence has been obtained that various bacterial and viral infections induce KD. Comprehensive research, including infectious agents, should be conducted to elucidate the pathogenesis of KD.
Pulmonary hypertension (PH) is a hemodynamic and pathophysiologic condition, defined as a mean pulmonary arterial pressure exceeding 25 mmHg at rest. According to the recent classifications, it is ...grouped into pulmonary arterial hypertension (PAH), heart-related, lung-related, thromboembolic, and miscellaneous PH. In the past two decades, tremendous advances have occurred in the field of PH. These include (1) development of clinical diagnostic algorithm and a monitoring strategy dedicated to PAH, (2) defining strong rationales for screening at-risk populations, (3) advent of pulmonary specific drugs which makes PAH manageable, (4) recognition of needs of having proper strategy of combining existing pulmonary specific drugs, and/or potential novel drugs, (5) pursuit of clinical trials with optimal surrogate endpoints and study durations, (6) recognition of critical roles of PH/right ventricular function, as well as interdependence of ventricles in different conditions, especially those with various phenotypes of heart failure, and (7) for rare diseases, putting equal importance on carefully designed observation studies, various registries, etc., besides double blind randomized studies. In addition, ongoing basic and clinical research has led to further understanding of relevant physiology, pathophysiology, epidemiology and genetics of PH/PAH. This guidelines from the working group of Pulmonary Hypertension of the Taiwan Society of Cardiology is to provide updated guidelines based on the most recent international guidelines as well as Taiwan’s domestic research on PH. The guidelines aremainly for themanagement of PAH (Group 1) ; however themajority of content can be helpful for managing other types of PH.
The relationship between adenovirus infection and Kawasaki disease (KD) is unclear. The purpose of this study was to determine the relationship between adenovirus infection and KD using a cohort ...study in Taiwan.
We used Taiwan National Health Insurance data (from 2000 to 2008) to conduct a population-based cohort study, analyzing children that was under 18 years of age. In total, 5280 children had adenovirus infection, and 5280 children without adenovirus infection were matched and followed up. Subsequent KD was the major outcome event. The Cox proportional hazards model was used to estimate the hazard ratio (HR) with 95% confidence intervals (CIs) of developing KD associated with adenovirus infection.
There was a significantly higher cumulative incidence of KD in the adenovirus-infected cohort than that in the control cohort (log-rank test, p < 0.001). In the adenovirus-infected cohort, overall incidence of KD was 5.29 times higher than that of the control cohort (adjusted HR 5.29, 95% CI: 2.48-11.3). Increased KD risk was associated with previous adenovirus infection in children aged 3-5 years, in female patients, in those with a low urbanization level, and in those with allergies.
An association between previous adenovirus infection and KD was identified in Taiwanese children, but other potential risk factors were not fully analyzed. The relationship between infection and KD requires further study.
Whether low-risk Kawasaki disease (KD) patients are at increased risk of cardiovascular disease later in life remains controversial. The purpose of this study is to examine the arterial stiffness and ...exercise performance of KD patients in chronic stage.
This study included 158 subjects. They were divided into three groups: 37 KD patients with regressed coronary artery lesions (CALs) (M/F 23/14, 13.6 ± 6.5 years) (group I), 43 KD patients without CALs (M/F 26/17, 13.9 ± 6.2 years) (group II), and 78 age- and gender-matched normal controls (M/F 44/34, 13.2 ± 6.9 years) (group III). They all underwent brachial-ankle pulse wave velocity (baPWV), an exercise test, and blood sampling to measure the levels of high-sensitivity C-reactive protein (hs-CRP), triglycerides (TG), high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, and total cholesterol (TC). The differences among the groups were compared.
There were significant differences among the three groups in terms of right and left baPWV (p < 0.01 respectively), HDL level (p < 0.05), TC/HDL ratio (p < 0.05), and oxygen consumption (VO2) peak (p < 0.05). Moreover, group I subjects had significantly higher right and left baPWV (p < 0.05 respectively), lower HDL level (p < 0.05), and lower VO2 peak (p < 0.05) than group II subjects. Furthermore, baPWV was significantly correlated with TG level (r = 0.326, p < 0.05), TC/HDL ratio (r = 0.483, p < 0.01), LDL level (r = 0.386, p < 0.01), and VO2 peak (r = -0.385, p < 0.05) in group I subjects. Only the TC/HDL ratio was found to be a significant correlating factor for an increase of baPWV (beta = 0.68, p < 0.05) in KD patients after multiple linear regression.
Our results suggest that arterial stiffness is present late after KD and may adversely affect exercise performance, especially in patients with regressed CALs. Regular measurement of baPWV may be indicated in the long-term follow-up of KD patients.
Abstract Background The purpose of this article was to evaluate the effect of maternal smoking exposure during pregnancy on postnatal outcomes. Methods This prospective study enrolled 278 pregnant ...women in the third trimester, who were asked to complete a questionnaire which included inquires about the nature and extent of smoking exposure during their pregnancy. In addition to the questionnaire, each study subject provided urine sample for the measurement of cotinine. Using data generated from this inquiry, we analyzed the association between maternal smoking exposure and birth outcomes. Results From the 278 enrollees in this study, a minority of subjects (7.2%) smoked, while 40.6% of the study subjects were exposed to environmental tobacco smoke during pregnancy. There was significantly higher birth weight (3205.2 ± 373.1 vs 3089.7 ± 363.0 vs 2959.0 ± 403.7 g, p = 0.004), larger chest size (33.1 ± 1.7 cm vs 32.7 ± 1.5 cm vs 32.0 ± 1.7 cm, p = 0.009), higher bilirubin on postnatal day 3 (8.9 ± 1.6 vs 8.6 ± 1.5 vs 7.8 ± 1.4 mg/dL, p = 0.015), but lower maternal urinary cotinine level (83.7 ± 132.4 vs 153.2 ± 96.0 vs 800.5 ± 1027.8 μg/g creatinine, p < 0.001) in smoking-free status than in passive or active smoking status. Significant risks of birth weight < 2500 g (AOR 3.93 (95% CI 1.61–9.59), p = 0.003) and maternal urinary cotinine ≥ 143 μg/g creatinine (AOR 3.38 (95% CI 2.02–5.66), p < 0.001) were observed as smoking exposure increased. There was significantly higher birth weight ( p = 0.048), larger chest size ( p = 0.045), and higher bilirubin level on postnatal day 3 ( p < 0.001) in the group with cotinine <143 μg/g creatinine than in the group with cotinine ≥ 143 μg/g creatinine. Conclusion Our results demonstrated that maternal smoking exposure during pregnancy is associated with low birth weight and small chest circumference. Although the incidence of active smoking in Taiwanese pregnant women is low, most of them are exposed to passive smoking environment. Further studies are required to evaluate useful interventions to enhance a smoking-free environment during pregnancy.
Purpose To investigate parents’ views about new vaccines, we surveyed the attitudes and awareness toward immunization program among parents of children younger than 14 years in Taiwan. Methods ...Parents of children were invited to complete a questionnaire in a tertiary referral medical center in Kaoshiung, southern Taiwan from 2006 to 2008. A total of 535 questionnaires were completed. We used descriptive data for the analysis of parents’ views and attitudes toward the current vaccination program in Taiwan. Results Of the 535 respondents, most parents (93%) did not think the current vaccination program was satisfactory. Few (approximately 8%) preferred self-paid vaccines. About 63% of parents believed that the new (self-paid) vaccines provided more protection, whereas 48% deemed them too expensive. The most popular reason for preferring the new vaccines was greater protection (73%). One-half of parents considered the new vaccines to be expensive. Regarding parental awareness of side effect of vaccination, fever was the most well known and of greatest concern (91%). Most parents (68%) had good awareness of conjugated pneumococcal vaccines, and only few (13%) had heard of the human papilloma virus vaccine. Most parental information of vaccines came from pamphlets at the hospital (56%). Conclusion We found the awareness of parents about new vaccines to be insufficient. They also considered the new vaccines to be expensive.
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