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There has been a remarkable increase in the number of pediatric ventricular assist device (VAD) implanted over the past decade. Asian pediatric heart centers had not participated in the multicenter registries among the Western countries. This article aimed to report the outcomes of pediatric VAD in our hospital.The study enrolled all patients aged18 years at the time of VAD implantation in our institution between 2008 and 2021.There were 33 patients with diagnosis of acute fulminant myocarditis (n = 9), congenital heart disease (n = 5), dilated cardiomyopathy (n = 16), and others. Paracorporeal continuous-flow pump was the most frequently implanted (n = 27). Most of the devices were implanted in patients with INTERMACS profile 1 (n = 24). The median duration on VAD was 22 days (range 2-254). The proportion of patients attaining positive outcomes (alive on device, bridge to transplantation or recovery) was 72.7% at 1 month, 67.7% at 3 months, and 67.7% at 6 months. Most of the deaths on device occurred within the first month post-implant (n = 9), with neurological complications being the most frequent cause of death. All recovered cases were successfully weaned off the device within the first month of implantation.We demonstrated a favorable outcome in pediatric patients supported with VAD at our institution.

IntroductionWith the improvement of long-term survival of patients with congenital heart disease (CHD), complications such as atrial fibrillation (AF) have become a concern. This study aimed to determine the epidemiology data and risk factors of AF in adult CHD (ACHD) patients and evaluate the impact of AF on late outcomes using a large ACHD cohort in Asia.MethodThis study enrolled all CHD patients older than 18 years of age diagnosed with CHD at National Taiwan University Hospital between 2007 and 2018. Data on patients’ clinical characteristics, electrocardiogram, Holter reports, and follow-up information were collected. AF status was classified as sustained AF, paroxysmal AF, or intra-atrial reentry tachycardia (IART). CHD was categorized as simple, severe, or complex CHD (single ventricle). Primary endpoint was defined as cerebrovascular accidents (CVA) or death.ResultThe study included 4403 patients (55.9% women), with 16.4% having severe and 2.9% having complex CHD. The cumulative incidence of AF was 6.9% (54.8% paroxysmal AF, 26.9% sustained AF, and 18.4% IART), which is comparable to the Western countries. The incidence increased with age and was higher in patients with pulmonary hypertension (PH, 27%), complex CHD (12.7%), and metabolic syndrome. The mean onset age of IART, paroxysmal, and sustained AF was 35.7±15.8, 48.4±19.3, and 56.9±14.2 years, respectively. Multivariate Cox regression analysis revealed that male sex, PH, and severe and complex CHD were the most critical risk factors for AF (odds ratio 1.67, 1.91, 3.55, and 12.6, respectively). The 70-year CVA-free survival rate was 67.1% in patients with AF (vs. 80.5% in those without AF, pConclusionsThis large ACHD cohort showed a high cumulative incidence of AF, similar to the Western countries, which increased with age, PH, and CHD severity. CVA-free survival was more closely associated with these factors than with AF.

Objectives To mitigate the shortage of homograft sources, the use of handmade trileaflet expanded polytetrafluoroethylene valves in pulmonary valve replacement has shown excellent results from multicentre studies conducted in Japan. However, world-wide data outside Japan is relative insufficient. This study presents the long-term results of a single surgeon’s use of flipped-back trileaflet method in a 10-year case series. Methods We have developed an efficient way to make a trileaflet-valved conduit utilizing flipped-back method for pulmonary valve replacement and have employed the technique since 2011. Retrospective data were studied between October 2010 and January 2020. Echocardiography, electrocardiogram, Pro-Brain Natriuretic Peptide, and Magnetic Resonance Imaging data were analyzed. Results 55 patients were reviewed and median follow-up duration was 2.9 years. The majority of diagnoses was Tetralogy of Fallot (n = 41), and these patients subsequently underwent secondary pulmonary valve replacement at a median age of 15.6 years. Survival was 92.7% with the longest follow-up period being 10 years. There was no need for reoperation, and freedom from reintervention was 98.0% at 10 years. There were 4 deaths (3 in-hospital and 1 outpatient). One patient eventually received transcatheter pulmonary valve implantation. Postoperative echocardiography showed mild or less pulmonary stenosis and pulmonary regurgitation degree in 92.2% and 92.0% of patients, respectively. Comparable magnetic resonance imaging data (n = 25) showed significant reduction in right ventricular volumes but not in ejection fractions. Conclusion Our series showed satisfactory long-term function of handmade flipped-back trileaflet valved conduit used in our patients. The simple design is efficiently reproducible without complex fabrication process.

OBJECTIVESAn 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.METHODSThis study analyzed data from 30 birthing facilities representing 87.9% of live births in Taipei. Positive screening was defined as oxygen saturation 3%. This study cohort was used to retrospectively estimate outcomes on the basis of different CCHD screening protocols.RESULTSDuring 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.CONCLUSIONSThe 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.

The purpose of this study is to determine the ECG parameter change and the efficacy of ECG screening for cardiac adverse effect after the second dose of BNT162b2 vaccine in young population. In December 2021, in cooperation with the school vaccination system of Taipei City government, we performed a ECG screening study during the second dose of BNT162b2 vaccines. Serial comparisons of ECGs and questionnaire survey were performed before and after vaccine in four male-predominant senior high schools. Among 7934 eligible students, 4928 (62.1%) were included in the study. The male/female ratio was 4576/352. In total, 763 students (17.1%) had at least one cardiac symptom after the second vaccine dose, mostly chest pain and palpitations. The depolarization and repolarization parameters (QRS duration and QT interval) decreased significantly after the vaccine with increasing heart rate. Abnormal ECGs were obtained in 51 (1.0%) of the students, of which 1 was diagnosed with mild myocarditis and another 4 were judged to have significant arrhythmia. None of the patients needed to be admitted to hospital and all of these symptoms improved spontaneously. Using these five students as a positive outcome, the sensitivity and specificity of this screening method were 100% and 99.1%, respectively. Conclusion: Cardiac symptoms are common after the second dose of BNT162b2 vaccine, but the incidences of significant arrhythmias and myocarditis are only 0.1%. The serial ECG screening method has high sensitivity and specificity for significant cardiac adverse effect but cost effect needs further discussed. What is Known: • The incidence of cardiac adverse effects was reported to be as high as 1.5 per 10 000 persons after the second dose BNT162b2 COVID-19 vaccine in the young male population based on the reporting system. What is New: • Through this mass ECG screening study after the second dose of BNT162b2 vaccine we found: (1) The depolarization and repolarization parameters (QRS duration and QT interval) decreased significantly after the vaccine with increasing heart rate; (2) the incidence of post-vaccine myocarditis and significant arrhythmia are 0.02% and 0.08%; (3) The serial ECG screening method has high sensitivity and specificity for significant cardiac adverse effect.

In patients with pulmonary artery hypertension (PAH) associated with atrial septal defect (ASD), closure of ASD may carry significant risks. We aimed to evaluate the safety and efficacy of transcatheter closure of ASD in selected patients with PAH using a fenestrated device followed by pulmonary vasodilator therapy. During the 14.8-year period, 51 ASD patients (10 males, age 46 ± 18 years) with a mean pulmonary artery pressure (PAP) ≥ 35 mm Hg and/or systolic PAP ≥ 60 mm Hg, underwent closure with a fenestrated device. Of them, mean Qp/Qs ratio, systolic PAP and mean PAP were 2.6 ± 1.2, 73 ± 14 mm Hg, and 44 ± 8 mm Hg, respectively. A total of 35 patients received pulmonary vasodilator therapy. The New York Heart Association (NYHA) functional class improved at 3 to 6 months follow-up. (p0.001) Nineteen patients underwent repeated catheterization. A comparison of the hemodynamic parameters between baseline and repeated catheterization revealed significant decreases in both systolic and mean PAP (77 ± 11 vs 55 ± 14 mm Hg, p0.000148 ± 7 vs 37 ± 8 mm Hg, p = 0.001, respectively), pulmonary vascular resistance (PVR) (5.1 ± 2.3 vs 4.0 ± 1.7 WU, p = 0.011) and PVRi (7.7 ± 3.3 vs 6.2 ± 2.4 WU*m2, p = 0.024). After a follow-up period of 84 ± 45 months, 6 mortalities were noted in which 2 were due to cardiac causes. In conclusion, catheter closure of ASD in patients with PAH using a fenestrated device followed by vasodilator therapy is safe and effective.

Background/purpose: Prognostic factors remain unclear in patients undergoing transcatheter implantation of Venus P-valve for their severe pulmonary regurgitation associated with native right ventricular (RV) outflow tract. Method: Between January 2017 and October 2018, we prospectively collected data of patient characteristics, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) before and 6–12 months after valve graft implantation. Results: Fifteen patients (male: 8, median age: 24.8 years) were enrolled. The procedure success rate was 100%. The median follow-up was 16.3 months without any dysfunction of the valve graft. The cohort demonstrated a significant improvement in cardiac index (from 3.3 to 3.9 L/min/m2) and increase of percentage of New York Heart Association functional class I (P

In this international study, we (1) compared patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) who had versus had not been hospitalized during the previous 12 month, (2) contrasted PROs in patients who had been hospitalized for cardiac surgery versus nonsurgical reasons, (3) assessed the magnitude of differences between the groups (i.e., effect sizes), and (4) explored differential effect sizes between countries. APPROACH-IS was a cross-sectional, observational study that enrolled 4,028 patients from 15 countries (median age 32 years; 53% females). Self-report questionnaires were administered to measure PROs: health status; anxiety and depression; and quality of life. Overall, 668 patients (17%) had been hospitalized in the previous 12 months. These patients reported poorer outcomes on all PROs, with the exception of anxiety. Patients who underwent cardiac surgery demonstrated a better quality of life compared with those who were hospitalized for nonsurgical reasons. For significant differences, the effect sizes were small, whereas they were negligible in nonsignificant comparisons. Substantial intercountry differences were observed. For various PROs, moderate to large effect sizes were found comparing different countries. In conclusion, adults with CHD who had undergone hospitalization in the previous year had poorer PROs than those who were medically stable. Researchers ought to account for the timing of recruitment when conducting PRO research as hospitalization can impact results. ispartof: AMERICAN JOURNAL OF CARDIOLOGY vol:145 pages:135-142 ispartof: location:United States status: published

Central sleep apnea is a rare disorder in the pediatric population with various initial presentations and is secondary to many underlying diseases. We report on a 4-year-old boy with episodes of syncope. He also had pulmonary hypertension and cardiomegaly. Polysomnography showed the finding for central sleep apnea with a high apnea-hypopnea index (up to 138.1 events/h). Brain magnetic resonance imaging showed an ill-defined area near the medulla oblongata and lower pons. The lesion from a brainstem biopsy confirmed the diagnosis of low-grade glioma. Conservative medical follow-up was suggested, and brain magnetic resonance imaging 6 months later showed no obvious tumor progression. To our best knowledge, this is the first case report that workup on the cause of syncope and pulmonary hypertension led to the final diagnosis of central sleep apnea and a brain neoplasm. CITATION: Huang S-W, Lee P-L, Fan P-C, et al. Diffuse midline glioma presenting with central sleep apnea and pulmonary hypertension in a 4-year-old patient: a case report. J Clin Sleep Med. 2021;17(2):325–328.

Plant volatile terpenoids (PVTs) are the main aroma components of flowers, fruits, and vegetables. Citronellol, geraniol, and nerol are the three major components of rose oil, which has wide applications in aromatherapy, perfumes, and cosmetics. However, the current production of rose oil through the distillation of flowers is not sustainable and cannot meet the increasing market demand. Here, we firstly reconstructed the biosynthetic pathway of citronellol in the platform yeast strain HP001, which overexpresses the MVA-pathway genes, resulting in the production of 7.87 mg L−1 citronellol. To further increase the production, the native promoter of ERG20 was replaced by the promoter of ERG7, and the expression of the geraniol-synthesis module was enhanced. The resulting strain exhibited a remarkable 4450% increase of the citronellol titer, reaching 0.36 g L−1 in shake flasks and 6.37 g L−1 in a bioreactor. Furthermore, the entire synthetic pathways of these three rose oil monoterpenoids were reconstituted in yeast, resulting in Rose-yeast 1.0, with a titer of 124.0 mg L−1 in shake flasks and 4.87 g L−1 in a bioreactor. Rose-yeast 1.0 produced a synthetic rose oil comprising a correct ratio of citronellol (62.30%), geraniol (27.39%), and nerol (10.31%), closely matching that of Rosa × damascena MILLER. Additionally, the ERG7 promoter was inserted in front of the native ERG9 gene, and the metabolic flux of the sesquiterpenoid synthesis was assessed by introducing the biosynthetic pathways of nerolidol and valencene. The maximal titer of the resulting strain reached 912.72 mg L−1 nerolidol and 5.30 mg L−1 valencene in shake flasks. This synthetic biology platform for mono- and sesquiterpenoid production can serve as a basis for creating a sustainable microbial source of important PVTs.

Background/purpose Outlet-type VSD is frequently associated with aortic valve prolapse that surgery is frequently required. The literature regarding outcomes of transcatheter closure of outlet-type VSDs is scant. This study was conducted to know the safety and efficacy of transcatheter closure of outlet-type ventricular septal defects (VSDs) with Amplatzer Duct Occluder II (ADO II). Methods Medical records of patients underwent attempted transcatheter closure of outlet-type VSD with ADO II between October 2013 and August 2019 were retrospectively reviewed. Results Among 49 patients, transcatheter closure was successful in 45 (91.8%; 33 males and 12 females; mean [± standard deviation] age and body weight: 15.8 (±17.7) years and 36.6 (±23.3) kg, respectively). The median VSD diameter was 4.0 mm (range: 1.2–6.0 mm). Device closure failed in four because the sheath could not be advanced through a prograde or retrograde route in one patient, occluder embolization in the two patients, and failed right ventricular disc anchoring in one patient. After a mean follow-up of 22.7 months (range: 0.3–51.1 months), only nine (20.0%) patients had increased severity in aortic regurgitation (AR) on the echocardiography. Preprocedural AR decreased in severity or even disappeared in 11 (24.4%) patients. No heart block or device failure occurred during follow-up. A trivial-to-small residual shunt was detected in 19 patients (42.2%) in the most recent echocardiography. Conclusion Transcatheter closure of outlet-type VSDs with ADO II is feasible. Although no significant aggravation of AR was observed in the short-to-mid-term follow-up, long-term follow-up is mandatory.

Cardiovascular complications after Pfizer-BioNTech COVID-19 (BNT) vaccination are a concern, especially in adolescents. We analyzed the risk factors for myocarditis after BNT vaccination.We used a special evaluation protocol for all patients aged 12-18 years who presented to our emergency department with cardiovascular symptoms after BNT vaccination.A total of 195 patients (109 boys and 86 girls) were enrolled. Eleven (5.6%) patients presented with arrhythmia (arrhythmia group), 14 (7.2%) had a diagnosis of pericarditis/myocarditis (the peri/myocarditis group), and the remaining 170 were controls (no cardiac involvement). Chest pain (77.6%) was the most common symptom. The median time from vaccination to symptom onset was 3 days. In the peri/myocarditis group (13 myocarditis and 1 pericarditis), the median time to the peak troponin T level was 5 days after vaccination. Abnormal electrocardiographic changes, including ST-T changes and conduction blocks, were more commonly detected in the peri/myocarditis group (85.7% vs. 12.4% in the control group, p 0.01). Echocardiography revealed normal ventricular function in all patients. Symptoms were resolved before discharge in all, with the median duration of hospital stay being 4 days. The electrocardiography was the most appropriate screening tool for myocarditis, with a sensitivity and specificity of 85.7% and 87.6%, respectively.Pericarditis or myocarditis was diagnosed in 7.2% of adolescents presenting to the emergency department with cardiovascular symptoms after BNT vaccination. In addition to the troponin T level, ECG change listed above can be used as a screening tool for vaccine-induced cardiac complications.

Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient‐report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH‐IS (Assessment of Patterns of Patient‐Reported Outcomes in Adults with Congenital Heart disease—International Study), we collected data on HF status and patient‐reported outcomes in 3959 patients from 15 countries across 5 continents. Patient‐report outcomes were: perceived health status (12‐item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence‐13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter‐defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient‐reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov ; Unique identifier: NCT02150603.

Background The population of adults with congenital heart disease (CHD) has increased dramatically with a high prevalence of acquired cardiac and non-cardiac comorbidities. However, the relationship among congenital heart disease, physical comorbidities, and psychological health in this population is not well studied. Aims The purpose of this study was to investigate (a) the association between adult congenital heart disease and the occurrence of depression and (b) whether physical comorbidities mediated the association between congenital heart disease and the occurrence of depression. Methods This retrospective cohort study was followed from 1 January 2010–31 December 2013, based on the data from the National Health Insurance Research Database 2010 in Taiwan. We used mediation analysis in survival data to assess the mediated effect. The hazard ratios were adjusted by age, sex, area of residence, and estimated propensity scores. Results We recruited 2122 adult congenital heart disease patients and 8488 matched controls. Nearly half of patients diagnosed with simple congenital heart disease, 39.0% had complex congenital heart disease, and 11.2% had unclassified congenital heart disease. Adult congenital heart disease patients had a significantly higher risk of depression than matched controls (adjusted hazard ratio = 1.43 and 1.48, for all and complex congenital heart disease, respectively, p Conclusions Helping patients to prevent psychological and physical acquired disease is imperative. Coronary artery disease is a potent mediator between congenital heart disease and depression, especially for patients with complex congenital heart disease.

Background 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. Methods 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. Results 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. Conclusions 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.

Background Transcatheter closure of patent ductus arteriosus (PDA) is an effective alternative to surgical ligation in preterm infants. However, data on device deformation and risk of left pulmonary artery (LPA) obstruction remain scant. This study describes the outcomes and complications of transcatheter closure of PDA in preterm infants weighing Methods Amplatzer Piccolo Occluder and Amplatzer Vascular Plug were used. Echocardiography was repeated at prespecified intervals. The device waist and length were assessed through lateral fluoroscopy immediately and at least 3 months after deployment. Results Fourteen infants were prospectively enrolled (mean procedural weight 1335 g, procedural age 24 days), and all procedures were successful. There was no obstruction of adjacent vessels immediately after deployment. At follow-up, three infants developed aortic coarctation, all of which resolved gradually. Obstruction of the LPA occurred in eight infants, with five being severe cases. Compared with the shape immediately after deployment, the devices became significantly more flattened and lengthened at follow-up in patients with LPA obstruction. This deformation was nonsignificant in infants without any LPA obstruction. The ratio of the device waist after deployment to the nominal waist ( Conclusions Device deformation occurring late at follow-up is common and may be associated with LPA obstruction in preterm infants after transcatheter PDA closure. Meticulous device selection and implantation technique are crucial for minimizing the associated risks.

The objective of this study is to assess the prevalence and risk factors for attention-deficit hyperactivity disorder (ADHD) in a large cohort of patients with congenital heart disease (CHD). Patients (n = 695) with CHD who were aged 6–15 years and visited the outpatient clinics in our hospital from June 2015 to May 2017 were enrolled. Their medical records were collected, and the Chinese version of the Swanson, Nolan, and Pelham rating scale (SNAP-IVc) and a questionnaire about neuropsychiatric care-seeking behavior were completed by parents and counselors. Of the 695 patients, the overall prevalence of ADHD was 12.4%, including 3.2% for the combined subtype, 6.8% for the inattentive-predominant subtype, and 2.4% for the hyperactivity/impulsive-predominant subtype. Only the inattention-predominant subtype was significantly more prevalent than in the general population. The prevalence of the inattention-predominant subtype was highest in the patients with cyanotic CHD, high severity index, and in those who had received surgery or cardiopulmonary bypass. Multivariate regression analysis indicated that the risk factors for inattention-related symptoms included postoperative seizure and previous cardiopulmonary bypass (odds ratio: 3.22 and 3.82; P = 0.027 and

The aims of this study were to examine the relationship between healthcare needs, health-promoting behaviors and quality of life (QoL) of youths with congenital heart disease (CHD), and to determine whether health-promoting behaviors were a mediating variable.This cross-sectional descriptive study was conducted recruiting 205 youths aged 15 to 24 with either ventricular septal defect (VSD) or tetralogy of Fallot (TOF). A self-reported questionnaire was used to collect demographic information; furthermore, the thickness of the triceps skinfold was measured. The Healthcare Needs Scale for Youth with CHD, Adolescent Health Promotion-short form scale, and World Health Organization QoL-BREF scale were administered, and Pearson's correlation, multiple regressions, and the Sobel test were conducted.The triceps skinfold of participants with VSD was significantly thicker than that of participants with TOF (p 0.05). Healthcare needs, health-promoting behaviors, and QoL were positively correlated. The overall health-promoting behaviors served as a partial mediator between overall healthcare needs and QoL, accounting for 40.1% of the total variation.The study illustrates that health-promoting behaviors have an impact on healthcare needs and QoL of youths with CHD. The development of tailored health consulting interventions to fulfill healthcare needs is required to improve physical and psychosocial wellness among youths with CHD.Targeting individual and public health strategies for enhancing health-promoting behaviors should be integrated in CHD care planning.

Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study. PH was determined by cardiac catheterization data or echocardiography reports. In accord with the World Symposia on Pulmonary Hypertension, CHD was further categorized as simple, severe, or complex CHD (including pulmonary atresia‐ventricular septal defect and single‐ventricle anomalies). There were 4301 patients (55.6% women), 15.7% with severe and 3.9% with complex CHD. The cumulative incidence of PH was 4.4% (95% CI, 3.8–5.0). Our multivariable regression model indicated 4.2‐fold mortality increase (95% CI, 3.0–5.9) in the presence of PH, with age, female sex, and severe or complex CHD linked to higher incidence of PH. Only 49% of patients received PH‐specific therapy. Five‐ and 10‐year survival rates of patients with PH (n=190) were 72.3% (95% CI, 65.1%–78.4%) and 58.8% (95% CI, 50.1%–66.5%), respectively. Survival rates in those with Eisenmenger syndrome, PH after defect correction, and complex CHD were similar. Low oxygen saturation and high uric acid levels were associated with increased mortality. Conclusions In this sizable Asian adult CHD cohort, the cumulative incidence of PH was aligned with that of Western countries. Mortality proved higher in patients with PH versus without PH. Although complex CHD carried greater risk of PH compared with other adult CHD subsets, survival rate was similar.

Monoterpenes are widely used in cosmetics, food, medicine, agriculture and other fields. With the development of synthetic biology, it is considered as a potential way to create microbial cell factories to produce monoterpenes. Engineering Saccharomyces cerevisiae to produce monoterpenes has been a research hotspot in synthetic biology. In S. cerevisiae, the production of geranyl pyrophosphate(GPP) and farnesyl pyrophosphate(FPP) is catalyzed by a bifunctional enzyme farnesyl pyrophosphate synthetase(encoded by ERG20 gene) which is inclined to synthesize FPP essential for yeast growth. Therefore, reasonable control of FPP synthesis is the basis for efficient monoterpene synthesis in yeast cell factories. In order to achieve dynamic control from GPP to FPP biosynthesis in S. cerevisiae, we obtained a novel chassis strain HP001-pERG1-ERG20 by replacing the ERG20 promoter of the chassis strain HP001 with the promoter of cyclosqualene cyclase(ERG1) gene. Further, we reconstructed the metabolic pathway by using GPP and neryl diphosphate(NPP), cis-GPP as substrates in HP001-pERG1-ERG20. The yield of GPP-derived linalool increased by 42.5% to 7.6 mg·L~(-1), and that of NPP-derived nerol increased by 1 436.4% to 8.3 mg·L~(-1). This study provides a basis for the production of monoterpenes by microbial fermentation.

Background: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardio-vascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II.Methods/design: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments.Discussion: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD., KU Leuven - University of Leuven; Research Foundation Flanders [1159522N, 12E9819N]; Swedish Heart and Lung Foundation [20190525]; Research Council of Aga Khan University in Karachi in Pakistan; Maltese Cardiac Society; Children's Heart Unit Fund; UnIC (Unidade de Investigacao Cardiovascular da FMUP); Italian Ministry of Health, This work is supported by KU Leuven - University of Leuven, Research Foundation Flanders through grants 1159522N and 12E9819N, Swedish Heart and Lung Foundation through grant 20190525, the University Research Council of the Aga Khan University in Karachi in Pakistan, Maltese Cardiac Society, UnIC (Unidade de Investigacao Cardiovascular da FMUP) supported the costs of the translations into Portuguese used in Portugal, Children's Heart Unit Fund ( www.chuf.org.uk), Newcastle upon Tyne through their funding of the CHUF Fontan nurse specialist. This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.