The Journal of Thoracic and Cardiovascular Surgery, 2021
OBJECTIVES A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geo... more OBJECTIVES A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geometries has been reported in patients with repaired tetralogy of Fallot (rTOF). We aimed to investigate the associations between RVOT/PA geometries and outcome indicators in a large rTOF cohort receiving non-conduit repair. METHODS Three-dimensional magnetic resonance angiographic images of 206 patients with rTOF who had a pulmonary regurgitation (PR) fraction ≥20% were reviewed. Patients' RVOT geometry was quantitatively classified into 4 distinct shapes (tubular, hourglass, pyramid, and inverted trapezoid). Bilateral PA size discrepancy was defined as the diameter of the smaller side being less than 70% of that of the bigger side. RESULTS Based on lateral projection of the 3-dimensional images, patients with an inverted trapezoid-shaped RVOT had the smallest RV end-diastolic volume index (EDVi) (108.7 ± 24.3 mL/m2) and pulmonary valve annulus diameter, and shortest QRS duration, whereas those with a pyramid-shaped RVOT had the largest RV EDVi (161.0 ± 44.6 mL/m2) and pulmonary valve annulus diameter. Similar trends of differences were also observed if such classifications were based on the frontal projections. Multivariable analysis revealed that RVOT shapes, subvalvular diameter, PR fraction, QRS duration, and the presence of bilateral PA size discrepancy were independent determinants of RV EDVi. Furthermore, having bilateral PA size discrepancy (25.2%) was independently associated with lower peak oxygen consumption (P = .041). CONCLUSIONS Distinct RVOT morphologies and branch PA size discrepancy are associated with variations in RV remodeling and exercise capacity in patients with rTOF. These findings may aid decision-making regarding reintervention for PR and branch PA size discrepancy.
To analyze the efficacy of a novel palivizumab protocol for hemodynamically significant congenita... more To analyze the efficacy of a novel palivizumab protocol for hemodynamically significant congenital heart disease (hsCHD) in subtropical areas without clear respiratory syncytial virus seasonality. Since July 2013, the National Health Insurance program has provided reimbursement for palivizumab prophylaxis with a novel monthly protocol in selected patients with hsCHD under 1 year of age. We performed a multicenter study to assess the trend of respiratory syncytial virus hospitalizations in patients with hsCHD from 2010 to 2016 during the prepalivizumab, transition, and postpalivizumab periods, and compared treatment and propensity-matched control groups. A total of 747 patients were enrolled in the study group and 809 in the control group. The male:female was 836:720. Cyanotic CHD was observed in 42.9% of patients. The mean age at diagnosis of CHD was 32.9 days. After 516 685 patient-days of follow-up and a mean of 3.9 doses of palivizumab in the treatment group, respiratory syncytia...
Ventricular tachycardia (VT), which is related to haemodynamic and electrophysiological alteratio... more Ventricular tachycardia (VT), which is related to haemodynamic and electrophysiological alterations, is an important complication in repaired tetralogy of Fallot (rTOF) patients. We defined the role of implantable cardioverter defibrillator (ICD) therapy after pulmonary valve replacement (PVR) and the implications of coexisting long QT gene mutations/polymorphisms. From 2003 to 2016, rTOF patients with VT who received ICD implantation were enrolled. rTOF patients without VT served as a control group. We performed long QT gene mutation analysis through the direct sequencing method. In total, 12 (male/female, 8/4) patients with VT received ICD implantation. The mean age at TOF repair and ICD implantation was 4.4±4.5years and 27.1±11.5years, respectively. All but one patient received PVR for severe pulmonary regurgitation or repair for a residual ventricular septal defect. After 5.4±4.0years of follow-up, six patients (50%) had at least one episode of appropriate shock: two had receive...
Paradoxical embolism is a risk factor for cryptogenic stroke in young adults, and can be primaril... more Paradoxical embolism is a risk factor for cryptogenic stroke in young adults, and can be primarily attributed to the presence of patent foramen ovale (PFO). Although contrast echocardiography may facilitate diagnosis, it would be difficult to distinguish a PFO from a pulmonary arteriovenous fistula (PAVF), which could also result in paradoxical embolism. We report a 46-year-old woman with recurrent ischemic stroke, who was scheduled for PFO closure because of a right-to-left shunt detected by contrast echocardiography. The diagnosis turned out to be a PAVF confirmed by angiography. Thereafter, coil embolization of this PAVF was performed successfully. Embolic stroke; Patent foramen ovale; Pulmonary arteriovenous fistula; Right-to-left shunt.
Balloon angioplasty of native coarctation of the aorta, though effective, is frequently associate... more Balloon angioplasty of native coarctation of the aorta, though effective, is frequently associated with early restenosis, repeated interventions and aneurysm formation, especially in very young patients. However, available long-term data are limited. From Jan. 1986 to Dec. 2007, eighty-eight patients with simple aortic coarctation, isolated or associated with patent ductus arteriosus or ventricular septal defect, and survived angioplasty (n = 17) or surgery (n = 71) within 3 months of age in National Taiwan University Hospital were enrolled. The average length of follow-up was 10.1 ± 5.1 years. The long-term outcomes between these two groups were comparable in hypertension, left ventricular outflow tract obstruction, and aortic aneurysm. Patients in the angioplasty group had a greater likelihood of reintervention (64.7% vs. 42.3%, p = 0.096) and repeated reintervention (29.4% vs. 7.0%, p = 0.027) as compared with surgery group. The 10-year freedom from reintervention was 35.3% and 5...
Stent implantation using a Cheatham Platinum (CP) stent for coarctation of the aorta (CoA) is a p... more Stent implantation using a Cheatham Platinum (CP) stent for coarctation of the aorta (CoA) is a promising treatment alternative to traditional surgical repair. However, there are no earlier reports in the literature focusing on use of this stent in a Taiwanese patient. Herein we report a 16-year-old boy with CoA presenting with heart murmur and exercise intolerance who underwent successful CP stent placement. However, severe hypertension with arterial blood pressure increasing to 207/104 mmHg occurred four hours after stent implantation. There was no abdominal pain, nausea or vomiting. The patient's hypertension was controlled by intravenous nitroglycerin infusion, followed by an oral antihypertensitve agent for the following 7 days. Experience from this case highlighted the usefulness of CP stent implantation for native CoA, and the importance of early recognition and management of paradoxical hypertension after CoA stenting.
We investigated the mid-to-long-term results of transcatheter closure of atrial septal defect (AS... more We investigated the mid-to-long-term results of transcatheter closure of atrial septal defect (ASD) in patients ≥40 years since there are issues with patients presenting with pulmonary hypertension and arrhythmia at the time of closure. In an 8.8 year period, transcatheter closure of ASD was successful in 179 patients aged ≥40 years, but failed in 2. Of the 179 patients (44 males, median 53 years), NYHA functional class, presence of arrhythmia and severity of pulmonary hypertension were compared before and after closure. Patients with pulmonary hypertension (n = 43, 24 %) were significantly older (60 ± 11 vs. 52 ± 9 years, p < 0.01) and required larger devices (27 ± 7 vs. 24 ± 7 mm, p = 0.04) than those without. Arrhythmia before intervention was documented in 31 patients (17.3 %): 22 atrial fibrillation (AF), 4 atrial flutter and 5 supraventricular tachycardia. Patients with AF or atrial flutter (n = 26) were significantly older (63 ± 10 vs. 53 ± 10 years, p = 0.048) and had a higher pulmonary artery mean pressure (29.2 ± 12.6 vs. 20.2 ± 7.6 mmHg, p = 0.041) than those without. The mean follow-up period was 3.8 ± 2.1 years. Early new-onset arrhythmia was documented in 23 patients of whom 1 had persistent AF, 1 developed sick sinus syndrome and others were in sinus rhythm at latest visit. There was significant improvement in NYHA functional class after closure (p < 0.001). Of the 22 patients with AF, 10 were in sinus rhythm, 1 had paroxysmal AF, and 11 had persistent AF. Pulmonary hypertension persisted in 13 patients. Transcatheter closure of atrial septal defect in patients above 40 years is beneficial in terms of NYHA functional class, pulmonary artery pressure and cardiac rhythm.
Parameters from cardiopulmonary exercise test (CPET) are useful prognostic factors for patients w... more Parameters from cardiopulmonary exercise test (CPET) are useful prognostic factors for patients with repaired tetralogy of fallot (TOF). Its application in exercise prescription remains unclear. This study sought to define its role. We made current exercise recommendations in repaired TOF patients according to European Society of Cardiology (ESC) guideline, which were based on ventricular function, pressure/volume load, pulmonary artery pressure, hypoxemia and arrhythmic burden both at rest and during exercise. CPET parameters (peak oxygen consumption, oxygen uptake efficiency plateau, and E/CO2 slope), along with cardiothoracic ratio, ventricular arrhythmia, QRS duration and NYHA functional status, were used to calculate "score to exercise". 112 repaired TOF adolescent and adult aged 32.6 ± 10.8 (14.05- to 56.99-year-old, median 30.1) years received exercise recommendations by ESC guideline, which suggested high, moderate and low intensity sports for 45 (40.2 %), 45 (40.2 %), and 22 (19.6 %) patients, respectively. The optimal cut-off values were 67 and 77 % for peak VO2, 86 and 100 % for OUEP, 22 and 28 for E/CO2 slope to correlate to the exercise intensity recommendation. But, individual CPET parameter had low consistency (41-46 %) in making decisions of exercise intensity compared to ESC recommendations. Using the "score to exercise", the consistency rate could be increased to 74.1 %. With "score to exercise" recommended exercise intensity, follow-up result revealed no adverse event related to sports. Individual CPET parameter did not correlate well to the exercise recommendation from ESC. We proposed a scoring system, "score to exercise", which incorporates three CPET parameters with cardiothoracic ratio, ventricular arrhythmia, QRS duration and NYHA functional status. Score to exercise is easy to be assessed and provides useful information for exercise recommendation in patients with repaired TOF.
For children with a history of Kawasaki disease (KD), low grade inflammation was generally report... more For children with a history of Kawasaki disease (KD), low grade inflammation was generally reported to be associated with persistent coronary artery lesions (CAL). However, this association has not been clearly demonstrated to hold true in KD adolescents and young adults (10-25 years of age). We enrolled 104 subjects into our study, who were separated into the following 3 groups and controls: 1): 22 KD patients with angiography-confirmed CAL which persisted for an average of 12.5 years after onset of KD; 2) 38 KD patients with regressed aneurysms; 3) 44 KD patients without any coronary complications from the disease onset; and 4) 31 age-matched (18.7 ± 1.88 years old) healthy controls. Plasma levels of high-sensitivity C reactive protein (hs-CRP) were measured for all participants. Plasma levels of hs-CRP were significantly higher in KD patients than in the controls, regardless of their coronary severity. However, there was no significant difference in hs-CRP levels among KD patient...
Patients with repaired tetralogy of Fallot (TOF) usually experience progressive right ventricle (... more Patients with repaired tetralogy of Fallot (TOF) usually experience progressive right ventricle (RV) dysfunction due to pulmonary regurgitation (PR). This could further worsen the cardiopulmonary function. This study aimed to compare the changes in patient exercise cardiopulmonary test and cardiac magnetic resonance imaging, and consider the implication of these changes. Our study examined repaired TOF patients who underwent cardiopulmonary exercise test (CPET) to obtain maximal (peak oxygen consumption, peak VO2) and submaximal parameters (oxygen uptake efficiency plateau, oxygen uptake efficiency plateau (OUEP), and ratio of minute ventilation to carbon dioxide production, VE/VCO2 slope). Additionally, the hemodynamic status was assessed by using cardiac magnetic resonance. Criteria for exclusion included TOF patients with pulmonary atresia, atrioventricular septal defect, or absence of pulmonary valve syndrome. We enrolled 158 patients whose mean age at repair was 7.8 ± 9.1 years...
With advances that have been made over the recent decades in transcatheter and surgical intervent... more With advances that have been made over the recent decades in transcatheter and surgical interventions, most patients with congenital heart disease (CHD) can survive into adulthood. Overall, probably half of these surviving patients are female. When these female CHD patients reach childbearing age, however, pregnancy management will be a major issue. In order to meet the demands of fetal growth, the maternal cardiovascular system starts a series of adaptations beginning in early pregnancy. These adaptations include: decreased systemic and pulmonary vascular resistances, decreased blood pressure, expansion of the blood volume, increased heart rate and increased cardiac output. For women with CHD, this hemodynamic alteration may increase the risks of adverse cardiovascular events as well as the fetal and neonatal complications. Therefore, proper risk stratification and effective counseling for women with CHD who are planning their pregnancies is an important undertaking. Congenital hea...
The aim of this study was to investigate the extent of diffuse myocardial fibrosis by measuring l... more The aim of this study was to investigate the extent of diffuse myocardial fibrosis by measuring left (LV) and right ventricular (RV) extracellular volume fraction (ECV) in patients with repaired tetralogy of Fallot (rTOF) and to explore its association with ventricular remodeling, hemodynamic load, and clinical parameters. Focal myocardial fibrosis is prevalent in patients with rTOF. However, little is known about the extent of diffuse myocardial fibrosis and its clinical implications in this population. We measured ECV by pre- and post-gadolinium T1 measurements using a 1.5-T scanner in 84 patients with rTOF (median age 23.3 years). LV ECV was determined by averaging values from 6 short-axis mid-ventricular segments, and RV ECV was calculated by averaging values from the anterior-inferior and the diaphragmatic RV wall segments. LV ECV above the upper limit of normal (>28%) was observed in 11 patients and for RV ECV (>41%) in 9 patients. LV ECV correlated positively with RV EC...
Sleep disturbance is common in children with atopic dermatitis (AD), but effective clinical manag... more Sleep disturbance is common in children with atopic dermatitis (AD), but effective clinical management for this problem is lacking. Reduced levels of nocturnal melatonin were found to be associated with sleep disturbance and increased disease severity in children with AD. Melatonin also has sleep-inducing and anti-inflammatory properties and therefore might be useful for the management of AD. To evaluate the effectiveness of melatonin supplementation for improving the sleep disturbance and severity of disease in children with AD. This randomized clinical trial used a double-blind, placebo-controlled crossover design to study 73 children and adolescents aged 1 to 18 years with physician-diagnosed AD involving at least 5% of the total body surface area. The study was conducted at the pediatric department of a large tertiary care hospital in Taiwan from August 1, 2012, through January 31, 2013. Forty-eight children were randomized 1:1 to melatonin or placebo treatment, and 38 of these ...
Circulation journal : official journal of the Japanese Circulation Society, Jan 9, 2015
Three risk estimation methods for predicting the cardiac outcomes of pregnancy in women with hear... more 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 (9...
The 6-minute walking test (6MWT) is a simple method used to evaluate exercise capacity in adults ... more The 6-minute walking test (6MWT) is a simple method used to evaluate exercise capacity in adults and children with cardiac diseases. Normal reference values in pediatric populations have been reported, but significant variations in the walking distance (6MWD) were noted among different studies. We aimed to provide and validate normal reference values of the 6MWD for healthy Taiwanese pediatric population between 7 and 17 years of age. Healthy children and adolescents were recruited from 13 randomly selected schools in Kaohsiung City. From that recruitment effort, 762 participants (50.1% male) were included, and the 6MWT was conducted using standardized protocols. The main outcome measure utilized was the 6MWD, which was used to construct centile charts and Z score equations. Data from additional 64 healthy volunteers recruited from the National Taiwan University Children's Hospital were used to validate these standards. There was an overall linear trend of increase in the 6MWD b...
The Journal of Thoracic and Cardiovascular Surgery, 2021
OBJECTIVES A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geo... more OBJECTIVES A wide variety of right ventricular outflow tract (RVOT) and pulmonary artery (PA) geometries has been reported in patients with repaired tetralogy of Fallot (rTOF). We aimed to investigate the associations between RVOT/PA geometries and outcome indicators in a large rTOF cohort receiving non-conduit repair. METHODS Three-dimensional magnetic resonance angiographic images of 206 patients with rTOF who had a pulmonary regurgitation (PR) fraction ≥20% were reviewed. Patients' RVOT geometry was quantitatively classified into 4 distinct shapes (tubular, hourglass, pyramid, and inverted trapezoid). Bilateral PA size discrepancy was defined as the diameter of the smaller side being less than 70% of that of the bigger side. RESULTS Based on lateral projection of the 3-dimensional images, patients with an inverted trapezoid-shaped RVOT had the smallest RV end-diastolic volume index (EDVi) (108.7 ± 24.3 mL/m2) and pulmonary valve annulus diameter, and shortest QRS duration, whereas those with a pyramid-shaped RVOT had the largest RV EDVi (161.0 ± 44.6 mL/m2) and pulmonary valve annulus diameter. Similar trends of differences were also observed if such classifications were based on the frontal projections. Multivariable analysis revealed that RVOT shapes, subvalvular diameter, PR fraction, QRS duration, and the presence of bilateral PA size discrepancy were independent determinants of RV EDVi. Furthermore, having bilateral PA size discrepancy (25.2%) was independently associated with lower peak oxygen consumption (P = .041). CONCLUSIONS Distinct RVOT morphologies and branch PA size discrepancy are associated with variations in RV remodeling and exercise capacity in patients with rTOF. These findings may aid decision-making regarding reintervention for PR and branch PA size discrepancy.
To analyze the efficacy of a novel palivizumab protocol for hemodynamically significant congenita... more To analyze the efficacy of a novel palivizumab protocol for hemodynamically significant congenital heart disease (hsCHD) in subtropical areas without clear respiratory syncytial virus seasonality. Since July 2013, the National Health Insurance program has provided reimbursement for palivizumab prophylaxis with a novel monthly protocol in selected patients with hsCHD under 1 year of age. We performed a multicenter study to assess the trend of respiratory syncytial virus hospitalizations in patients with hsCHD from 2010 to 2016 during the prepalivizumab, transition, and postpalivizumab periods, and compared treatment and propensity-matched control groups. A total of 747 patients were enrolled in the study group and 809 in the control group. The male:female was 836:720. Cyanotic CHD was observed in 42.9% of patients. The mean age at diagnosis of CHD was 32.9 days. After 516 685 patient-days of follow-up and a mean of 3.9 doses of palivizumab in the treatment group, respiratory syncytia...
Ventricular tachycardia (VT), which is related to haemodynamic and electrophysiological alteratio... more Ventricular tachycardia (VT), which is related to haemodynamic and electrophysiological alterations, is an important complication in repaired tetralogy of Fallot (rTOF) patients. We defined the role of implantable cardioverter defibrillator (ICD) therapy after pulmonary valve replacement (PVR) and the implications of coexisting long QT gene mutations/polymorphisms. From 2003 to 2016, rTOF patients with VT who received ICD implantation were enrolled. rTOF patients without VT served as a control group. We performed long QT gene mutation analysis through the direct sequencing method. In total, 12 (male/female, 8/4) patients with VT received ICD implantation. The mean age at TOF repair and ICD implantation was 4.4±4.5years and 27.1±11.5years, respectively. All but one patient received PVR for severe pulmonary regurgitation or repair for a residual ventricular septal defect. After 5.4±4.0years of follow-up, six patients (50%) had at least one episode of appropriate shock: two had receive...
Paradoxical embolism is a risk factor for cryptogenic stroke in young adults, and can be primaril... more Paradoxical embolism is a risk factor for cryptogenic stroke in young adults, and can be primarily attributed to the presence of patent foramen ovale (PFO). Although contrast echocardiography may facilitate diagnosis, it would be difficult to distinguish a PFO from a pulmonary arteriovenous fistula (PAVF), which could also result in paradoxical embolism. We report a 46-year-old woman with recurrent ischemic stroke, who was scheduled for PFO closure because of a right-to-left shunt detected by contrast echocardiography. The diagnosis turned out to be a PAVF confirmed by angiography. Thereafter, coil embolization of this PAVF was performed successfully. Embolic stroke; Patent foramen ovale; Pulmonary arteriovenous fistula; Right-to-left shunt.
Balloon angioplasty of native coarctation of the aorta, though effective, is frequently associate... more Balloon angioplasty of native coarctation of the aorta, though effective, is frequently associated with early restenosis, repeated interventions and aneurysm formation, especially in very young patients. However, available long-term data are limited. From Jan. 1986 to Dec. 2007, eighty-eight patients with simple aortic coarctation, isolated or associated with patent ductus arteriosus or ventricular septal defect, and survived angioplasty (n = 17) or surgery (n = 71) within 3 months of age in National Taiwan University Hospital were enrolled. The average length of follow-up was 10.1 ± 5.1 years. The long-term outcomes between these two groups were comparable in hypertension, left ventricular outflow tract obstruction, and aortic aneurysm. Patients in the angioplasty group had a greater likelihood of reintervention (64.7% vs. 42.3%, p = 0.096) and repeated reintervention (29.4% vs. 7.0%, p = 0.027) as compared with surgery group. The 10-year freedom from reintervention was 35.3% and 5...
Stent implantation using a Cheatham Platinum (CP) stent for coarctation of the aorta (CoA) is a p... more Stent implantation using a Cheatham Platinum (CP) stent for coarctation of the aorta (CoA) is a promising treatment alternative to traditional surgical repair. However, there are no earlier reports in the literature focusing on use of this stent in a Taiwanese patient. Herein we report a 16-year-old boy with CoA presenting with heart murmur and exercise intolerance who underwent successful CP stent placement. However, severe hypertension with arterial blood pressure increasing to 207/104 mmHg occurred four hours after stent implantation. There was no abdominal pain, nausea or vomiting. The patient's hypertension was controlled by intravenous nitroglycerin infusion, followed by an oral antihypertensitve agent for the following 7 days. Experience from this case highlighted the usefulness of CP stent implantation for native CoA, and the importance of early recognition and management of paradoxical hypertension after CoA stenting.
We investigated the mid-to-long-term results of transcatheter closure of atrial septal defect (AS... more We investigated the mid-to-long-term results of transcatheter closure of atrial septal defect (ASD) in patients ≥40 years since there are issues with patients presenting with pulmonary hypertension and arrhythmia at the time of closure. In an 8.8 year period, transcatheter closure of ASD was successful in 179 patients aged ≥40 years, but failed in 2. Of the 179 patients (44 males, median 53 years), NYHA functional class, presence of arrhythmia and severity of pulmonary hypertension were compared before and after closure. Patients with pulmonary hypertension (n = 43, 24 %) were significantly older (60 ± 11 vs. 52 ± 9 years, p &amp;amp;lt; 0.01) and required larger devices (27 ± 7 vs. 24 ± 7 mm, p = 0.04) than those without. Arrhythmia before intervention was documented in 31 patients (17.3 %): 22 atrial fibrillation (AF), 4 atrial flutter and 5 supraventricular tachycardia. Patients with AF or atrial flutter (n = 26) were significantly older (63 ± 10 vs. 53 ± 10 years, p = 0.048) and had a higher pulmonary artery mean pressure (29.2 ± 12.6 vs. 20.2 ± 7.6 mmHg, p = 0.041) than those without. The mean follow-up period was 3.8 ± 2.1 years. Early new-onset arrhythmia was documented in 23 patients of whom 1 had persistent AF, 1 developed sick sinus syndrome and others were in sinus rhythm at latest visit. There was significant improvement in NYHA functional class after closure (p &amp;amp;lt; 0.001). Of the 22 patients with AF, 10 were in sinus rhythm, 1 had paroxysmal AF, and 11 had persistent AF. Pulmonary hypertension persisted in 13 patients. Transcatheter closure of atrial septal defect in patients above 40 years is beneficial in terms of NYHA functional class, pulmonary artery pressure and cardiac rhythm.
Parameters from cardiopulmonary exercise test (CPET) are useful prognostic factors for patients w... more Parameters from cardiopulmonary exercise test (CPET) are useful prognostic factors for patients with repaired tetralogy of fallot (TOF). Its application in exercise prescription remains unclear. This study sought to define its role. We made current exercise recommendations in repaired TOF patients according to European Society of Cardiology (ESC) guideline, which were based on ventricular function, pressure/volume load, pulmonary artery pressure, hypoxemia and arrhythmic burden both at rest and during exercise. CPET parameters (peak oxygen consumption, oxygen uptake efficiency plateau, and E/CO2 slope), along with cardiothoracic ratio, ventricular arrhythmia, QRS duration and NYHA functional status, were used to calculate &amp;amp;quot;score to exercise&amp;amp;quot;. 112 repaired TOF adolescent and adult aged 32.6 ± 10.8 (14.05- to 56.99-year-old, median 30.1) years received exercise recommendations by ESC guideline, which suggested high, moderate and low intensity sports for 45 (40.2 %), 45 (40.2 %), and 22 (19.6 %) patients, respectively. The optimal cut-off values were 67 and 77 % for peak VO2, 86 and 100 % for OUEP, 22 and 28 for E/CO2 slope to correlate to the exercise intensity recommendation. But, individual CPET parameter had low consistency (41-46 %) in making decisions of exercise intensity compared to ESC recommendations. Using the &amp;amp;quot;score to exercise&amp;amp;quot;, the consistency rate could be increased to 74.1 %. With &amp;amp;quot;score to exercise&amp;amp;quot; recommended exercise intensity, follow-up result revealed no adverse event related to sports. Individual CPET parameter did not correlate well to the exercise recommendation from ESC. We proposed a scoring system, &amp;amp;quot;score to exercise&amp;amp;quot;, which incorporates three CPET parameters with cardiothoracic ratio, ventricular arrhythmia, QRS duration and NYHA functional status. Score to exercise is easy to be assessed and provides useful information for exercise recommendation in patients with repaired TOF.
For children with a history of Kawasaki disease (KD), low grade inflammation was generally report... more For children with a history of Kawasaki disease (KD), low grade inflammation was generally reported to be associated with persistent coronary artery lesions (CAL). However, this association has not been clearly demonstrated to hold true in KD adolescents and young adults (10-25 years of age). We enrolled 104 subjects into our study, who were separated into the following 3 groups and controls: 1): 22 KD patients with angiography-confirmed CAL which persisted for an average of 12.5 years after onset of KD; 2) 38 KD patients with regressed aneurysms; 3) 44 KD patients without any coronary complications from the disease onset; and 4) 31 age-matched (18.7 ± 1.88 years old) healthy controls. Plasma levels of high-sensitivity C reactive protein (hs-CRP) were measured for all participants. Plasma levels of hs-CRP were significantly higher in KD patients than in the controls, regardless of their coronary severity. However, there was no significant difference in hs-CRP levels among KD patient...
Patients with repaired tetralogy of Fallot (TOF) usually experience progressive right ventricle (... more Patients with repaired tetralogy of Fallot (TOF) usually experience progressive right ventricle (RV) dysfunction due to pulmonary regurgitation (PR). This could further worsen the cardiopulmonary function. This study aimed to compare the changes in patient exercise cardiopulmonary test and cardiac magnetic resonance imaging, and consider the implication of these changes. Our study examined repaired TOF patients who underwent cardiopulmonary exercise test (CPET) to obtain maximal (peak oxygen consumption, peak VO2) and submaximal parameters (oxygen uptake efficiency plateau, oxygen uptake efficiency plateau (OUEP), and ratio of minute ventilation to carbon dioxide production, VE/VCO2 slope). Additionally, the hemodynamic status was assessed by using cardiac magnetic resonance. Criteria for exclusion included TOF patients with pulmonary atresia, atrioventricular septal defect, or absence of pulmonary valve syndrome. We enrolled 158 patients whose mean age at repair was 7.8 ± 9.1 years...
With advances that have been made over the recent decades in transcatheter and surgical intervent... more With advances that have been made over the recent decades in transcatheter and surgical interventions, most patients with congenital heart disease (CHD) can survive into adulthood. Overall, probably half of these surviving patients are female. When these female CHD patients reach childbearing age, however, pregnancy management will be a major issue. In order to meet the demands of fetal growth, the maternal cardiovascular system starts a series of adaptations beginning in early pregnancy. These adaptations include: decreased systemic and pulmonary vascular resistances, decreased blood pressure, expansion of the blood volume, increased heart rate and increased cardiac output. For women with CHD, this hemodynamic alteration may increase the risks of adverse cardiovascular events as well as the fetal and neonatal complications. Therefore, proper risk stratification and effective counseling for women with CHD who are planning their pregnancies is an important undertaking. Congenital hea...
The aim of this study was to investigate the extent of diffuse myocardial fibrosis by measuring l... more The aim of this study was to investigate the extent of diffuse myocardial fibrosis by measuring left (LV) and right ventricular (RV) extracellular volume fraction (ECV) in patients with repaired tetralogy of Fallot (rTOF) and to explore its association with ventricular remodeling, hemodynamic load, and clinical parameters. Focal myocardial fibrosis is prevalent in patients with rTOF. However, little is known about the extent of diffuse myocardial fibrosis and its clinical implications in this population. We measured ECV by pre- and post-gadolinium T1 measurements using a 1.5-T scanner in 84 patients with rTOF (median age 23.3 years). LV ECV was determined by averaging values from 6 short-axis mid-ventricular segments, and RV ECV was calculated by averaging values from the anterior-inferior and the diaphragmatic RV wall segments. LV ECV above the upper limit of normal (>28%) was observed in 11 patients and for RV ECV (>41%) in 9 patients. LV ECV correlated positively with RV EC...
Sleep disturbance is common in children with atopic dermatitis (AD), but effective clinical manag... more Sleep disturbance is common in children with atopic dermatitis (AD), but effective clinical management for this problem is lacking. Reduced levels of nocturnal melatonin were found to be associated with sleep disturbance and increased disease severity in children with AD. Melatonin also has sleep-inducing and anti-inflammatory properties and therefore might be useful for the management of AD. To evaluate the effectiveness of melatonin supplementation for improving the sleep disturbance and severity of disease in children with AD. This randomized clinical trial used a double-blind, placebo-controlled crossover design to study 73 children and adolescents aged 1 to 18 years with physician-diagnosed AD involving at least 5% of the total body surface area. The study was conducted at the pediatric department of a large tertiary care hospital in Taiwan from August 1, 2012, through January 31, 2013. Forty-eight children were randomized 1:1 to melatonin or placebo treatment, and 38 of these ...
Circulation journal : official journal of the Japanese Circulation Society, Jan 9, 2015
Three risk estimation methods for predicting the cardiac outcomes of pregnancy in women with hear... more 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 (9...
The 6-minute walking test (6MWT) is a simple method used to evaluate exercise capacity in adults ... more The 6-minute walking test (6MWT) is a simple method used to evaluate exercise capacity in adults and children with cardiac diseases. Normal reference values in pediatric populations have been reported, but significant variations in the walking distance (6MWD) were noted among different studies. We aimed to provide and validate normal reference values of the 6MWD for healthy Taiwanese pediatric population between 7 and 17 years of age. Healthy children and adolescents were recruited from 13 randomly selected schools in Kaohsiung City. From that recruitment effort, 762 participants (50.1% male) were included, and the 6MWT was conducted using standardized protocols. The main outcome measure utilized was the 6MWD, which was used to construct centile charts and Z score equations. Data from additional 64 healthy volunteers recruited from the National Taiwan University Children's Hospital were used to validate these standards. There was an overall linear trend of increase in the 6MWD b...
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