Comparing Strategies for Critical Congenital Heart Disease Newborn Screening.

OBJECTIVES: An extended newborn critical congenital heart disease (CCHD) screening program using oximetry has been implemented in Taipei, Taiwan since April 2014. This study was conducted to investigate the test accuracy and efficiency of this screening protocol. METHODS: This study analyzed data from 30 birthing facilities representing 87.9% of live births in Taipei. Positive screening was defined as oxygen saturation <95% in either extremity or a preductal-postductal oxygen saturation difference of >3%. This study cohort was used to retrospectively estimate outcomes on the basis of different CCHD screening protocols. RESULTS: During the study period, 93 058 of 94 204 (98.8%) infants who had no prenatal suspicion were screened. The referral rate was 0.17% (156/93 058), and up to 90% of test-positive infants were referred within 48 hours of life. Forty-two CCHD cases without prenatal suspicion were detected and 97.6% were diagnosed within 72 hours of life. Of the screened newborns, 4 CCHD cases passed the screening. The false positive and false negative rates were 0.12% and 0.04%, respectively. In addition, applying our database to Spanish and updated American Academy of Pediatrics screening strategies led to more CCHD case detection. CONCLUSIONS: The Taipei protocol provided an efficient and effective screening referral system in a community setting. For optimal efficiency, we advocated the updated American Academy of Pediatrics algorithm/Spanish recommendation with a modification of immediate referral if oxygen saturation ≤90% in either extremity. The updated protocol would be practicable for nationwide screening in Taiwan and could also be applied to other regions with similar medical care systems.

Development of a Newborn Screening Program for Critical Congenital Heart Disease (CCHD) in Taipei.

BACKGROUND: Early detection of critical congenital heart disease (CCHD) can significantly reduce morbidity and mortality among newborns. We investigate the feasibility of implementing a community-based newborn CCHD screening program in Taipei. METHODS: Twelve birthing facilities in Taipei participated in a trial screening program between October 1, 2013, and March 31, 2014. Newborns underwent pulse oximetry at 24-36 h old, with probes attached to the right hand and one lower limb. Any screening saturation ≥95% in either extremity, with an absolute difference of ≤3% between the right hand and foot, was accepted as a screening pass. A screening result was considered as a fail if the oxygen saturation was <95% at either probe site, on 3 separate occasions, each separated by 30 min or the first result was <95% at either probe site, and any subsequent oxygen saturation measurement was <90%. Public health nurses would follow up all missed or refused cases. RESULTS: Of the 6,387 live births, 6,296 newborns (coverage rate: 6,296/6,387 = 98.6%) underwent appropriate pulse oximetry screening. Sixteen newborns (0.25%) were reported to have a failed screening result. Five of these screen positive newborns were confirmed with CCHD; two of them were diagnosed solely attributed to the failed screening results. The false-positive rate was 0.18%. Implementing a 6-month screening program for CCHD produced good case detection rate, while using efficient screening and referral systems. CONCLUSION: This program was successful in integrating screening, referral and public health tracking systems. The protocol outlined in this report could provide a community-based model for worldwide implementation.

Efficacy and Safety of Transthoracic Echocardiography Alone in Transcatheter Closure of Secundum-Type Atrial Septal Defects in Adults.

BACKGROUND: On-site transthoracic echocardiography (TTE) to guide the transcutaneous closure of secundum-type atrial septal defects (ASDs) in the catheterization laboratory remains unclear, especially in adults. METHODS: Between 2005 and 2012, a total of 82 adults underwent transcutaneous closure of ASDs. The initial 15 cases underwent the procedure with both on-site transesophageal echocardiography (TEE) and TTE monitoring. Since January 2008, a total of 67 patients underwent on-site TTE alone to guide the procedure. RESULTS: Among the 82 adult patients who underwent a transcutaneous closure of the secundum-type ASD procedure, all had successful closure of the defects, and no periprocedural adverse complications occurred. No statistical significance was observed in the successful complete shunt closure rate between the TEE plus TTE and TTE groups during sequential follow-up (postprocedure 24 hour [87% vs. 92%],1 month [93% vs. 95%], 3 month [93% vs. 97%], and 12 month [93% vs. 97%], P > 0.05, respectively) nor was a significant difference observed between the two groups, including decreased right ventricular dimension (29.5 ± 3.3 vs. 32.0 ± 4.9 mm, 26.5 ± 3.0 vs. 28.7 ± 4.6 mm, 26.2 ± 3.1 vs. 28.2 ± 4.8 mm, and 25.6 ± 2.8 vs. 27.7 ± 4.7 mm, P > 0.05, respectively) or increased left ventricular end-diastolic dimension (41.1 ± 2.0 vs. 42.6 ± 3.0 mm, 44.3 ± 2.7 vs. 45.5 ± 3.1 mm, 44.2 ± 2.8 vs. 45.4 ± 3.1 mm, 44.9 ± 2.7 vs. 45.8 ± 2.6 mm, P > 0.05, respectively) before the procedure, and at the 3-, 6-, and 12-month follow-up evaluations. CONCLUSION: This study showed that TTE guidance alone may be considered efficacious and safe as TEE during a transcutaneous ASD occlusion procedure in select adults.

Current recommendations for the Japanese encephalitis vaccine.

Japanese encephalitis (JE) is a mosquito-borne flavivirus infection and an important cause of encephalitis in most of Asia and parts of the western Pacific. Most people infected with the JE virus (JEV) are asymptomatic or seemingly suffer from a nonspecific, flu-like illness; in others, JE can cause illness ranging from fever and headache to severe encephalitis. Although it can cause significant morbidity and mortality, JE is a vaccine-preventable disease, and vaccination programs have proven most effective in preventing and diminishing the burden of disease. Such JE vaccines have been available for decades with four types of JE vaccines-live attenuated SA14-14-2 vaccine, inactivated mouse brain-derived vaccine (JE-MB), inactivated Vero cell culture vaccine (JE-VC), and live attenuated chimeric vaccine (IMOJEV)-and are currently used in most countries. In some Asian countries such as Japan, China, Taiwan, Korea, and Thailand, immunization programs have been conducted for children and so the ongoing incidence of JE has declined considerably in recent decades. Until quite recently, the primary JE vaccine in use internationally has been the JE-MB, which is now commonly replaced by cell culture-based vaccines.

Can probiotics be used to treat allergic diseases?

Probiotics are proprietary formulations of specific microorganisms and quantified populations of live bacteria that are intended to confer a health benefit on the host. These different strains and combinations of microorganisms have a wide and varying range of clinical and immunologic capacities that can modify intestinal microbial populations in ways that can benefit the host. The enhanced presence of probiotic bacteria in the intestinal microbiota has been found to correlate with protection against atopy. The prevalence of allergic diseases such as asthma, allergic rhinitis, and atopic dermatitis has increased sharply over the past 2-3 decades in many countries, and allergies are now the most common chronic disease among children throughout the world. In the past few years, probiotics have been advocated for the management of allergic diseases in many parts of the world. So far, probiotics have shown more promise, albeit limited, in the primary prevention of allergic disease rather than in the treatment of established disease.

2014 Guidelines of Taiwan Society of Cardiology (TSOC) for the Management of Pulmonary Arterial Hypertension.

UNLABELLED: 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 are mainly for the management of PAH (Group 1) ; however the majority of content can be helpful for managing other types of PH. KEY WORDS: Pulmonary arterial hypertension; Taiwan guidelines.

Color Doppler echocardiographic study on the incidence and natural history of early-infancy muscular ventricular septal defect.

BACKGROUND: Most small muscular ventricular septal defect (M-VSD) types have been diagnosed using color Doppler echocardiography. The purpose of this study was to understand the incidence of small M-VSD in the neonatal period and analyze the natural history of these M-VSDs. MATERIALS AND METHODS: All individuals in our study were neonates delivered at term who had a normal healthy appearance. Each accepted neonate had an examination with complete color Doppler echocardiography once before discharge. If the examination was confirmed for M-VSD, the study participants were then classified according to defect type. Further examination was arranged with color Doppler echocardiography at 1 month, 2 months, 4 months, 6 months, 9 months, and 12 months of age or until there was complete spontaneous closure. RESULTS: Among 2891 neonates, we found that 72 (24.9/1000) were diagnosed with M-VSD. Among this group, 38 were male and 34 were female. Only six infants were lost to follow-up. Fifty-four of the 66 infants (81.8%) had M-VSD closed spontaneously at 12 months' follow-up. Significantly, 33 of 37 infants (89.2%) with mid-muscular type, the most common type of M-VSD, closed within the 1(st) year of life compared with apical type (17/24:70.8%). Four of the five infants (80%) had anterior type M-VSD closed. Infants with posterior type M-VSD were not seen during this study period. CONCLUSION: Although the incidence of M-VSD was common in the neonatal period, there was also a high rate of spontaneous closure. Therefore, comparison of M-VSD appearance with the incidence of congenital heart disease in neonates had a decisive influence on analysis.

The prevalence and clinical impact of pulmonary artery sling on school-aged children: a large-scale screening study.

BACKGROUND: Airway obstruction is a leading cause of mortality and morbidity in patients with pulmonary artery sling (PAS). Early identification of PAS is imperative for prompt initiation of appropriate treatments. OBJECTIVE: Our aim was to investigate the prevalence and clinical impact of PAS on school-aged children through a large-scale pre-sports participation cardiovascular screening (PPCVS) study. METHODS: A total of 186,213 school-aged children underwent portable two-dimensional echocardiography (2DE) study in a PPCVS study between 2001 and 2004 in Taichung City, Taiwan. We prospectively collected data on the prevalence of PSA, demographics, clinical features, tracheo-bronchial anomalies (TBA), on computed tomography (CT), pulmonary function test (PFT), follow-up data in 1 year, and clinical outcomes. RESULTS: PAS was identified in 11 children (8 males/3 females) with a median age of 13 years (range: 7.2-13.7 years). The prevalence of PAS was 1 in 17,000 school-aged children. The majority of children with PAS had recurrent broncho-pulmonary infections (90.9%) and asthmatic cough (81.8%), which had a low positive predictive value of PAS (<1%). Seven patients had diffused complete cartilaginous rings. The other four patients were found to have prominent localized external compression of the trachea or bronchus. No coexisting intracardiac anomalies were found. Two patients had mental retardation. Seven patients underwent surgical correction without mortality or major complications. After surgery, significant improvement in PFT was shown in forced expiratory volume in 1 s (FEV1) and functional vital capacity (FVC). CONCLUSIONS: This is the first study to determine the prevalence of PAS as 59 per million school-aged children by a large-scale screening using 2DE. Although the majority of children with PAS have associated TBA and impaired pulmonary function, all patients with PAS were unrecognized due to non-specific symptoms. Early identification of PAS and surgery result in significant improvement in pulmonary function.