Shuenn-Nan Chiu

National Taiwan University, T’ai-pei, Taipei, Taiwan

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Publications (62)229.02 Total impact

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    ABSTRACT: Objective: To compare the risk of respiratory syncytial virus (RSV)-associated hospitalization and analyze the epidemiology of RSV infection in patients with cyanotic and acyanotic congenital heart disease (CHD), we analyzed the nationwide health insurance database from 2005-2010. Study design: This study included 1050 patients with cyanotic CHD and 7077 patients with acyanotic CHD. Patients with acyanotic CHD were further classified into hemodynamically significant (hs)-acyanotic and non-hs-acyanotic groups according to whether they underwent surgery or took at least 2 anticongestive medications. Results: RSV-associated hospitalization was higher in the cyanotic group than in hs-acyanotic and non-hs-acyanotic groups both before 1 year of age (4.8% vs 2.1% vs 1.5%, P < .001) and between 1 and 2 years of age (0.9% vs 0.56% vs 0.14%, P = .003). The hospitalization duration, intensive care, ventilator support prevalence, hospitalization cost, and mortality rate were significantly higher in the cyanotic group than in the other 2 groups. Logistic regression revealed that cyanotic CHD was the most significant risk factor for the ventilator support and RSV-associated mortality. In both patients with cyanotic and acyanotic CHD, RSV-associated hospitalization rate was higher in patients aged younger than 1 year and in spring and autumn in Taiwan, a subtropical country. Conclusions: The results show that patients with cyanotic CHD have a higher risk of severe RSV infection than do those with acyanotic CHD. RSV prophylaxis is more important and may reduce costs more for patients with cyanotic CHD.
    No preview · Article · Jan 2016 · The Journal of pediatrics
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    ABSTRACT: Background: Patients with heterotaxy syndrome, commonly associated with complex congenital heart disease (CHD), exhibit a higher risk of severe bacterial infection (SBI). We sought to define the change of a novel immunologic marker, the immunoglobulin M (IgM) memory B cell percentage, and its association with SBI. Methods: We enrolled 46 (M/F 29/17) heterotaxy syndrome patients (42 right atrial isomerism and 4 left atrial isomerism) aged > 1 year during the period 2010-2012 in a tertiary care center. We analyzed IgM(+)CD27(+) memory B cell percentages. Patients with simple and complex CHD served as controls. Results: The mean IgM memory B cell percentages were the lowest in the heterotaxy syndrome group, compared with those in complex and simple CHD groups (1.8±2.1 vs 3.9±3.2 vs 5.1±4.7, P<.001). In the heterotaxy syndrome group, 41.3% had low IgM memory B cell percentages (<1% of B cells). Seven had a history of community-acquired SBI and 85.7% of these had low IgM memory B cell percentages, which was the only significant factors related to community-acquired SBI. (p=0.028). Conclusions: The memory B cell and IgM memory B cell percentages are low in patients with heterotaxy syndrome, and the presence of IgM memory B cell percentage < 1% correlates with community-acquired SBI.Pediatric Research (2015); doi:10.1038/pr.2015.221.
    No preview · Article · Nov 2015 · Pediatric Research

  • No preview · Article · Nov 2015 · EuroIntervention: journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology
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    Kun-Tai Kang · Shuenn-Nan Chiu · Wen-Chin Weng · Pei-Lin Lee · Wei-Chung Hsu
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    ABSTRACT: In the present study, we aimed to verify associations between ambulatory blood pressure (ABP) and pediatric obstructive sleep apnea (OSA) in a hospital-based population. This was a cross-sectional observational study on children aged 4 to 16 years with OSA-related symptoms from a tertiary referral medical center. All children received overnight polysomnography and 24-hour recording of ABP. Severity of the disease was classified as primary snoring (apnea-hypopnea index, AHI <1), mild OSA (AHI 1–5), and moderate-to-severe OSA (AHI >5). For 195 children enrolled in this study (mean age, 7.8 ± 3.4 years; 69% boy), ABP increased as severity of OSA increased. During daytime, children with moderate-to-severe OSA had significantly higher systolic blood pressure (BP) (117.0 ± 12.7 vs 110.5 ± 9.3 mmHg), mean arterial pressure (MAP) (85.6 ± 8.1 vs 81.6 ± 6.8 mmHg), and diastolic BP load (12.0 ± 9.6 vs 8.4 ± 10.9 mmHg) compared with children with primary snoring. During nighttime, children with moderate-to-severe OSA had significantly higher systolic BP (108.6 ± 15.0 vs 100.0 ± 9.4 mmHg), MAP (75.9 ± 9.6 vs 71.1 ± 7.0 mmHg), systolic BP load (44.0 ± 32.6 vs 26.8 ± 24.5 mmHg), systolic BP index (0.5 ± 13.1 vs −6.8 ± 8.5 mmHg), and higher prevalence of systolic hypertension (47.6% vs 14.7 %) compared with children with primary snoring. Multiple linear regression analyses revealed an independent association between AHI and nighttime systolic BP and MAP after adjusting for adiposity variables. This large hospital-based study showed that children with moderate-to-severe OSA had a higher ABP compared with children who were primary snorers. As elevated BP in childhood predicts future cardiovascular risks, children with severe OSA should be treated properly to prevent further adverse cardiovascular outcomes.
    Full-text · Article · Oct 2015 · Medicine
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    ABSTRACT: Three risk estimation methods for predicting the cardiac outcomes of pregnancy in women with heart disease have been proposed. This study was designed to compare their prediction performance in an Asian cohort with congenital heart disease (CHD).Methods and Results:This study enrolled pregnant women with CHD who delivered their babies after the 20th gestational week between 1985 and 2011. Of 268 pregnancies in 190 women with CHD, 18 (6.7%) had cardiac complications. The incidence of maternal cardiac events among women with a CARPREG index of 0, 1 or 2 was 3.4%, 27.3% and 100%. The incidence was 2.7%, 8.6%, 11.1%, 40% and 17.6% for those with a ZAHARA score 0-0.5, 0.51-1.5, 1.51-2.5, 2.51-3.5 and >3.5. Among patients with a modified World Health Organization (WHO) classification I, II, III and IV, the incidence of maternal cardiac events was 0%, 4.0%, 12.2% and 25.7%. The c-statistic was 0.732 (95% confidence interval (CI): 0.589, 0.876; P<0.001) for the CARPREG score, 0.737 (95% CI: 0.611, 0.864; P=0.001) for the ZAHARA score and 0.827 (95% CI: 0.745, 0.909; P<0.001) for the WHO classification. All 3 risk estimation methods had good performance in predicting maternal cardiac outcomes; however, the modified WHO classification demonstrated superior discrimination and calibration.
    Full-text · Article · May 2015 · Circulation Journal
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    ABSTRACT: Treating obstructive sleep apnea in children is found to be associated with blood pressure decreases. However, exactly how adenotonsillectomy (T&A) affects blood pressure in obese and nonobese children remains unclear. This study assesses how obesity affects blood pressure in children with sleep apnea after T&A. Case series with chart review. A tertiary referral center. From 2010 to 2012, a total of 78 children were included. Based on propensity score methods (age, sex, and preoperative apnea-hypopnea index matched), children were assigned to either the obese group (n = 39) or the nonobese group (n = 39). All children received adenotonsillectomy. We recorded clinical symptoms, preoperative overnight polysomnography (PSG), and subsequent PSG within 3 months after T&A. We measured blood pressure 3 times before PSG (nocturnal blood pressure) and after PSG (morning blood pressure) in a sleep laboratory. Following surgery, the nonobese group had a significantly decreased nocturnal diastolic blood pressure (DBP) index (-12.0 to -18.8, P = .018), morning systolic blood pressure (SBP; 111.1 to 105.8 mm Hg, P = .014), SBP index (-5.4 to -10.9, P = .008), and DBP (-12.0 to -18.7, P = .023). Nevertheless, all blood pressure parameters in the obese group were not significantly changed postoperatively. The nonobese group improved more than obese group in nocturnal and morning DBP and DBP index by 2-way analysis of variance. Among the children receiving T&A as treatment for OSA, nonobese children improved more than obese children did in terms of blood pressure, allowing us to infer that obese children with OSA may benefit less from T&A in cardiovascular morbidities. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.
    No preview · Article · May 2015 · Otolaryngology Head and Neck Surgery
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    ABSTRACT: Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients. We enrolled 248 KD (development dataset: 181, validation: 67) patients who received IVIG within 10 days after fever onset. IVIG unresponsiveness was defined by persistent fever beyond 24 hours after IVIG or recrudescent fever with KD symptoms. From the development dataset (181 patients), IVIG unresponsiveness was noted in 22 patients (12.1%). The preIVIG levels of albumin, percentage of neutrophils, and positive lymphadenopathy were identified with highest risk for IVIG unresponsiveness. These three variables were used to construct a three-variable logistic regression model, which yielded an area under the receiver-operating-characteristics curve of 0.87. These three variables were further used to generate a composite scoring model (Formosa score) which yielded a sensitivity of 90.9% and specificity of 81.3% for a cut-off point of three or more. Validation in an independent cohort (67 KD patients) yielded sensitivity and specificity of 71.4% and 81.0%, respectively. We have established a simple three-variable Formosa score for KD patients to identify early those at risk of IVIG unresponsiveness for timely aggressive immunomodulation initially. Copyright © 2015. Published by Elsevier B.V.
    No preview · Article · Apr 2015 · Journal of the Formosan Medical Association
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    ABSTRACT: Objective: We present the mid-term results of transcatheter treatment of unroofed coronary sinus (CS) using the Amplatzer septal occluder. Background: The unroofed CS is a rare atrial septal defect communicating the left atrium and CS. Surgery has been the mainstay of treatment. Methods: In a 4.5-year period, 9 patients (5 males) with ages ranging from 26 to 69 years (median 39 years) diagnosed with an unroofed CS but without a persistent left superior vena cava (LSVC) underwent transcatheter treatment. Computerized tomography (CT) was performed in 8 patients. Transesophageal echocardiography (TEE) was used to monitor the procedure. Results: The mean Qp/Qs ratio was 2.4 ± 1 and mean systolic pulmonary artery pressure was 35 ± 19 mmHg. An Amplatzer septal occluder was deployed in all 9 patients. The device was implanted in the defect in 1 patient and at the CS ostium in the other 8 patients. The median device size used was 22 mm (16-28 mm). The left disc herniated into the CS in the single patient in whom the device was implanted within the defect. All patients were available for the three-month follow-up. None had a residual shunt on the three-month follow-up echocardiography. One patient died of a stroke 4.5 months after the procedure. At mean follow-up 42.6 ± 18.3 months, symptomatic improvement was documented in the remaining 8 patients. All 8 patients had an O2 saturation above 96 %. Conclusion: Transcatheter treatment for unroofed CS without persistent LSVC using Amplatzer septal occluder is safe and feasible. © 2013 Wiley Periodicals, Inc.
    Full-text · Article · Feb 2014 · Catheterization and Cardiovascular Interventions
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    ABSTRACT: The epidemiology of infective endocarditis (IE) changes with the medical advances. The study aimed to evaluate the trends in a pediatric cohort. From hospital database 1983-2011, patients <18 years who fulfilled the modified Duke Criteria of IE were identified. We enrolled 112 patients (M/F 57/55) with 116 IE episodes. 86 patients (74.1%) had preexisting cardiac lesions, and 23 patients (19.6%) were immunocompromised hosts. Prior dental procedure was noted in 12 (10.3%) patients, including 4 with simple ventricular septal defect. The overall mortality was 10.7%. The risk factors included vegetations in both ventricles (odds ratio, OD = 7.81, P = .019) and prior use of broad-spectrum antibiotics (OD = 3.75, P = .055). Approximately one-third of the patients (29.3%) required surgical intervention. We identified an increasing trend in the proportion of hospital-acquired IE (HAIE; from 12% in 1983-1991 to 39% in 2002-2011), and the spectrum of offending pathogens showed a trend for fewer Streptococcus species, more Staphylococcus aureus and increased pathogen diversity. The leading pathogens were Gram-negative bacilli in HAIE and Streptococcus species in community-acquired IE (CAIE). HAIE was associated with younger age, a higher proportion of immunocompromised patients, a history of central line indwelling and higher mortality. In contrast, more surgical intervention and embolic events occurred in CAIE patients. The mortality of pediatric IE remains high. Dental procedures were noted in one-tenth of the patients. Though increased S. aureus-caused episodes and pathogen diversity were noted, Streptococcus species remain the most common pathogen.
    No preview · Article · Dec 2013 · The Pediatric Infectious Disease Journal
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    ABSTRACT: To determine the incidence of sepsis in patients with heterotaxy syndrome. From our institutional database, we identified patients with heterotaxy syndrome and other complex congenital heart disease (CHD) born between 2001 and 2011. Severe bacterial infection was defined as sepsis with positive culture result or infection with abscess formation. We enrolled 95 patients with heterotaxy syndrome (88 with right atrial isomerism and 7 with left atrial isomerism) and 142 patients with complex CHD. With 1026 person-years follow-up, the 5-year survival was 52% and 65.7% in heterotaxy and complex CHD groups, respectively (P = .239). Community-acquired severe bacterial infection occurred only in heterotaxy syndrome (13 episodes in 10 patients, 3 of whom had spleen noted at imaging study) with 2- and 5 years cumulative severe bacterial infection rate of 9.6% and 14.5%, respectively. The overall mortality rate of those with community-acquired severe bacterial infection was 31%. Pneumococcus and Citrobacter freundii were the most common pathogens. Nosocomial severe bacterial infection occurred in 33.3% of all patients and 12.5% of all procedures. The rates (0.59 and 0.52/100 hospitalization days in heterotaxy and complex CHD group) and the pathogens of nosocomial severe bacterial infection were similar between heterotaxy and complex CHD groups. Patients with heterotaxy syndrome are at high risk for community-acquired severe bacterial infection and also have high mortality rate whether the spleen is present or not. The risk of nosocomial severe bacterial infection seems similar to that of patients with other complex CHD.
    No preview · Article · Oct 2013 · The Journal of pediatrics
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    ABSTRACT: Objective: CATCH 22 syndrome is a medical acronym for multiple abnormalities, especially cardiac defect. The patients with congenital heart disease (CHD) usually have more complicated post-surgery course. Methods: We reviewed 4-year experience in our hospital to define the lower airway anomalies and the clinical implications in patients with CATCH 22 syndrome and CHD. From 2004 to 2007, 18 patients who underwent computed tomography for planning of cardiovascular treatment were enrolled. Detection of airway anomalies were performed on computed tomography. Results: Characteristic dysmorphic facial features were noted in 8 out of 18 patients (44%). Ten patients (55.6%) had mild to moderate tracheal or bronchial stenosis. Five patients (28%) had tracheomalacia or bronchomalacia. Comparing to control group, patients received the first major surgery with a median hospital stays of 42 days in study group had a significantly longer hospital stay (42 vs. 16, P = 0.005) and longer duration of ventilator usage (16 vs. 4, P = 0.014). The difference of the mean position of the carina on computed tomography image between study groups and control groups was about three-quarters of a vertebral body height (P < 0.05). Conclusions: Patients with CATCH 22 syndrome and CHD had a shorter length of trachea compared to that of the age-matched similar CHD. The incidence of associated lower airway anomalies in CHD children with CATCH 22 syndrome was higher. It may lead to longer time of ventilator support after surgery as well as total hospital stays than those of CHD without CATCH 22 syndrome.
    Full-text · Article · Jun 2013 · Pediatric Pulmonology
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    ABSTRACT: Objectives: To delineate the long-term outcomes and mechanisms of pediatric sinus bradycardia. Study design: Participants with sinus bradycardia who were identified from a survey of 432,166 elementary and high school students, were enrolled 10 years after the survey. The clinical course, heart rate variability, and hyperpolarization-activated cyclic nucleotide-gated potassium channel 4 (HCN4) gene were assessed. Results: A total of 104 (male:female was 60:44; prevalence, 0.025%) participants were observed to have sinus bradycardia at age 15.5 ± 0.2 years with a mean heart rate of 48.4 ± 0.4 beats per minute; 86 study participants (83%) responded to clinical assessment and 37 (36%) underwent laboratory assessment. Athletes composed 37.8% of the study participants. During the extended 10-year follow-up, 15 (17%) of the participants had self-limited syncopal episodes, but none had experienced life-threatening events. According to Holter recordings, none of the participants had heart rate <30 beats per minute or a pause longer than 3 seconds. Compared with 67 age- and sex-matched controls, the variables of heart rate based on the spectral and time domain analysis of the participants with sinus bradycardia were all significantly higher, indicating higher parasympathetic activity. The results of mutation analysis were negative in the HCN4 gene in all of our participants. Conclusions: The long-term outcomes of the children and adolescents with sinus bradycardia identified using school electrocardiographic survey are favorable. Parasympathetic hyperactivity, instead of HCN4 gene mutation, is responsible for the occurrence of sinus bradycardia.
    No preview · Article · Apr 2013 · The Journal of pediatrics
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    ABSTRACT: OBJECTIVE: To assess the tolerability and efficacy of the investigational use of the angiotensin II receptor blocker losartan added to β-blockade (BB) to prevent progressive aortic root dilation in patients with Marfan syndrome (MFS). PATIENTS AND METHODS: Between May 1, 2007, and September 31, 2011, 28 patients with MFS (11 males [39%]; mean ± SD age, 13.1±6.3 years) with recognized aortic root dilation (z score >2.0) and receiving BB (atenolol or propranolol) treatment were enrolled. They were randomized to receive BB (BB: 13 patients) or β-blockade and losartan (BB-L: 15 patients) for 35 months. RESULTS: In the BB-L group, aortic root dilation was reduced with treatment, and the annual dilation rate of the aortic root was significantly lower than that of the BB group (0.10 mm/yr vs 0.89 mm/yr; P=.02). The absolute aortic diameters at the sinus of Valsalva, annulus, and sinotubular junction showed similar trends, with a reduced rate of dilation in the BB-L group (P=.02, P=.03, and P=.03, respectively). Five patients (33%) treated with BB-L were noted to have a reduced aortic root diameter. However, the differences between the groups regarding changes in aortic stiffness and cross-sectional compliance were not statistically significant. CONCLUSION: This randomized, open-label, active controlled trial mostly based on a pediatric population demonstrated for the first time that losartan add-on BB therapy is safe and provides more effective protection to slow the progression of aortic root dilation than does BB treatment alone in patients with MFS. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00651235.
    Full-text · Article · Jan 2013 · Mayo Clinic Proceedings
  • Shuenn-Nan Chiu · Chi-Wei Chang · Chun-Wei Lu · Mei-Hwan Wu

    No preview · Article · Sep 2012 · International journal of cardiology
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    ABSTRACT: Genetic syndromes, especially 22q11 deletion (del22q11) syndrome, are common in patients with pulmonary atresia and ventricular septal defect (PA-VSD), but their association with long-term outcomes varies. The purpose of this study was to evaluate the long-term outcome after complete repair of PA-VSD and to determine the impact of genetic syndromes. We reviewed our experience of 125 patients with PA-VSD who received primary or staged repair between 1978 and 2010. Evaluations for genetic syndromes included clinical features, cytogenetic analysis, and fluorescence in situ hybridization or multiplex ligation-dependent probe amplification. Genetic syndromes were documented in 26 patients (20.8%), including del22q11 in 16 patients, trisomy 21 in 2 patients, and other syndromes in 8 patients. The prevalence of hypoplastic pulmonary arteries was not significantly different between the syndromic and nonsyndromic groups. After 1,069 patient-years of follow-up, 20-year survival was 90% ± 6% in patients without syndromes and 14% ± 23% in patients with syndromes (p < 0.01). Multivariate analysis identified the presence of a genetic syndrome as an important risk factor for hospital and late mortality. Subgroup analysis showed that genetic syndromes other than del22q11 were associated with worse outcome. The rate of 10-year freedom from cardiac reintervention after repair was 53% ± 11%, with hypoplastic pulmonary arteries before repair as a major risk factor (p = 0.02). Genetic syndromes significantly affect survival after repair of PA-VSD, whereas genetic syndromes do not represent additional risk for reintervention. Repair is feasible in patients with syndromes, but suboptimal long-term outcome should be addressed when counseling parents.
    Full-text · Article · Aug 2012 · The Annals of thoracic surgery
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    ABSTRACT: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which the connection between the pulmonary vein (PV) and left atrium needs to be surgically created. This study investigated the spectrum and outcome of a Taiwanese cohort. Isolated TAPVC cases were identified from our institutional database between 1995 and 2009. We reviewed the medical chart and conducted telephone interviews with those lost to follow-up. There were 78 patients (52% male). The anomalous drainage sites were mainly supracardiac type (42.3%) and cardiac type (39.8%). Before operation, PV stenosis was found in 100% of infracardiac type, and in 69.7% of supracardiac type. Among the 75 patients undergoing operation, the surgical mortality was 9% (7/75). Perioperative arrhythmias (mainly of atrial origin) occurred in 35% of the patients. Of the 68 patients who survived the first operation, 28 (41%) developed pulmonary vein restenosis. Half of them progressed to severe PV stenosis, which required reintervention or resulted in mortality. Preoperative PV stenosis was the most significant predictor for postoperative PV restenosis and PV re-intervention. For the cohort, the 1-year and 5-year survivals were 78.9% and 74.2%, respectively, and the predictor for survival was again preoperative PV stenosis. The surgical mortality of isolated TAPVC is now low. Preoperative PV stenosis not only increased the risk of late PV restenosis and its reintervention, but also the overall mortality. The spectrum of PV drainage, per se, was not associated with worse outcome. PV restenosis remained the most important issue after correction of TAPVC.
    Full-text · Article · Jun 2012 · Pediatrics & Neonatology
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    ABSTRACT: Coexisting long QT gene mutations/polymorphisms in Tetralogy of Fallot (TOF) patients may aggravate the repolarization abnormality from cardiac repair. We investigated the impact of these genes on the risk of life-threatening events. Genetic variants of the three common long QT genes were identified from patients with repaired TOF. Life-threatening events were defined as sudden cardiac death and hemodynamic unstable ventricular arrhythmia. Biophysical characterization of the alleles of the genetic variants was performed using a whole-cell voltage clamp with expression in Xenopus oocytes. A total of 84 patients (56.0 % male with 1,215 patients-year follow-up) were enrolled. Six rare variants and six non-synonymous single nucleotide polymorphisms (SNPs) were found in 40 (47.6 %) patients. Life-threatening events occurred in five patients; four received implantable cardioverter defibrillator and one died of sudden cardiac death. Life-threatening events occurred more often in those with genetic variants than those without (5/40 vs. 0/44, P = 0.021); particularly, the hERG or SCN5A gene mutations/polymorphisms (2/5 vs. 3/79, P = 0.027 and 5/27 vs. 0/57, P = 0.003, respectively). Among the five patients with life-threatening events, three had compound variants (hERG p.M645R/SCN5A p.R1193Q, hERG p.K897T/SCN5A p.H558R, and KVLQT1 p.G645S/SCN5A p.P1090L), that also increased the risk of events. Their QTc and JTc were all prolonged. Functional study of the novel variant (hERG gene p.M645R) from patients with life-threatening events revealed a dominant negative effect. In conclusion, in repaired TOF patients, coexisting long QT mutations/polymorphisms might have additive effects on the repolarization abnormality from surgery and thereby increase the risks of life-threatening events.
    No preview · Article · Mar 2012 · Human Genetics
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    ABSTRACT: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in Taiwan. This study investigates the long-term survival and risks of TOF in an Asian cohort. This study enrolled 819 consecutive patients with TOF (61.1% male), who received total correction between 1970 and 2002, as participants. Patient medical records were reviewed, and the survival status of those out of contact was confirmed by death records retrieved from the National Health database. The mean (±SD) patient age at cardiac repair was 6.5±7.6 years, and a prior shunt operation was performed in 119 (14.5%) of the patients. At cardiac repair, a transannular patch for right ventricle outlet reconstruction was required in 444 (54.2%) of the patients. After 13,808 patient-years of follow-up, the 30-year survival rate was 90.5%. The annual mortality rate increased from 0.123% in the initial 15 years after repair to 0.395% thereafter (P<0.05). The presence of major aortopulmonary collateral arteries, older operative age, and previous shunt operation are independent risks of late cardiac deaths. Secondary to cardiac mortality, unnatural deaths (accident and suicide) accounted for 27.6% of late deaths, significantly higher compared with that of the general population (odds ratio, 2.18; P=0.028). In this Asian TOF cohort, except for a late decrease after 15 years, long-term survival after cardiac repair was satisfactory. Although cardiac death was the most common cause of late death, accidents or suicide may also be associated with late mortality, suggesting a potential role for psychosocial support.
    Full-text · Article · Jan 2012 · Circulation Cardiovascular Quality and Outcomes
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    ABSTRACT: Our aim was to evaluate the feasibility of using computed tomography (CT) to define the pulmonary artery anatomy in patients with tetralogy of Fallot and pulmonary atresia (TOF-PA). We retrospectively reviewed 110 patients with TOF-PA between 1995 and 2008. Those who received cardiac catheterization and surgery within 3 months of their CT examinations were enrolled. Based on Dr. Somerville's classification, the pulmonary arterial pattern was determined, including identifiable pulmonary trunk (type I), the presence of both left and right pulmonary arteries without trunk (II), only left or right pulmonary artery present (III), and absent intrapericardial pulmonary arteries (IV). The accuracy of both imaging modalities was evaluated with operation findings as the golden standard. The effective radiation doses and adverse events were also recorded. In the 64 eligible patients (median age, 23 months), CT and catheterization demonstrated accurate pulmonary arterial morphology in 60 (60/64) and 53 (53/64) TOF-PA patients, respectively. Thirty-two of 35 type I patients were correctly identified by CT, whereas 26 were correctly identified by catheterization (p = 0.03). Of the 20 type II TOF-PA patients, 19 were diagnosed by CT, whereas 18 were diagnosed by catheterization. CT and catheterization both successfully defined six type III and three type IV patients. The median calculated radiation doses caused by CT and catheterization were 4.5 and 5.6 mSv, respectively (p > 0.05). CONCLUSIONS: For patients with TOF-PA, CT could accurately delineate pulmonary arterial morphology with the same level of accuracy as cardiac catheterization. Therefore, CT can be considered a reasonable diagnostic alternative for such patients.
    Full-text · Article · Nov 2011 · European Journal of Pediatrics
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    ABSTRACT: Enzyme replacement therapy (ERT) for infantile-onset Pompe disease effectively reduces the left ventricular (LV) mass. This study sought to explore detailed process of LV reverse remodeling after ERT with the use of tissue Doppler and stain rate imaging. Nine infants and children with Pompe cardiomyopathy undergoing ERT for ≥1 year, as well as 36 healthy control subjects, were studied. Global systolic and diastolic function was evaluated by peak systolic and early-diastolic velocity at mitral annulus. Temporal systolic and diastolic dyssynchrony was evaluated by the coefficient of variation of the time from the QRS complex to peak systolic and early-diastolic strain rate among 12 LV segments. All pre-ERT patients had impaired global systolic and diastolic function as well as increased regional dyssynchrony (P < .001 for each of all). During the regression of LV hypertrophy, all of these functional indices improved (P for trend <.001), with temporal diastolic dyssynchrony being a significant factor linking to LV mass index in multivariate analysis (P < .001). ERT improved global LV function and dyssynchrony in Pompe patients. The relationship between LV mass and temporal diastolic dyssynchrony during reverse remodeling suggested a pathophysiologic role of dyssynchrony in Pompe cardiomyopathy.
    Full-text · Article · Nov 2011 · Journal of cardiac failure

Publication Stats

452 Citations
229.02 Total Impact Points

Institutions

  • 2005-2015
    • National Taiwan University
      • College of Medicine
      T’ai-pei, Taipei, Taiwan
    • Min-Sheng General Hospital
      Hsin-chu-hsien, Taiwan, Taiwan
  • 2002-2015
    • National Taiwan University Hospital
      • • Department of Internal Medicine
      • • Department of Surgery
      T’ai-pei, Taipei, Taiwan
  • 2007
    • National Taiwan University Hospital
      Hsin-chu-hsien, Taiwan, Taiwan