[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
The Journal of pediatrics 10/2013; 164(1). DOI:10.1016/j.jpeds.2013.08.051 · 3.79 Impact Factor
[Show abstract][Hide abstract] 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.
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.
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).
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.
[Show abstract][Hide abstract] ABSTRACT: Objectives:
To delineate the long-term outcomes and mechanisms of pediatric sinus bradycardia.
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.
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.
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.
The Journal of pediatrics 04/2013; 163(3). DOI:10.1016/j.jpeds.2013.03.054 · 3.79 Impact Factor
[Show abstract][Hide abstract] 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.
Mayo Clinic Proceedings 01/2013; 88(3). DOI:10.1016/j.mayocp.2012.11.005 · 6.26 Impact Factor
[Show abstract][Hide abstract] 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.
The Annals of thoracic surgery 08/2012; 94(5):1627-33. DOI:10.1016/j.athoracsur.2012.06.063 · 3.85 Impact Factor
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
Human Genetics 03/2012; 131(8):1295-304. DOI:10.1007/s00439-012-1156-4 · 4.82 Impact Factor
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] 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.
European Journal of Pediatrics 11/2011; 171(3):579-86. DOI:10.1007/s00431-011-1621-4 · 1.89 Impact Factor
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] ABSTRACT: Uncorrected congenital heart defects (CHD) with severe pulmonary hypertension (sPH, systolic pulmonary artery>70% of systolic pressure) are usually considered inoperable. We are curious to know if some selected patients might benefit from palliative operation for those sPH with uncorrected CHD.
Adults or adolescents with sPH associated with ventricular septal defect (VSD) with/without great artery anomalies were selected for pulmonary artery banding (PAB) to reduce sPH. The target pulmonary pressure was less than half of the systolic blood pressure after arch or great arteries reconstruction. Repeated catheterization was performed to evaluate the feasibility of defect closure.
Consecutively, 8 patients (age 26 ± 9 years) received PAB as a palliative procedure in the past 8 years without mortality. The pre-PAB systolic pulmonary pressure was 119 ± 9 mmHg. Additional PAB had been applied in 4 of them. All patients showed significant improvement in function class (III to I or II). The mean post-PAB pulmonary pressure decreased significantly (77.5 ± 9.2 mmHg to 42.0 ± 9.0 mmHg) and 6-minute walk test was also found to have great improvement (270 ± 86 m to 414 ± 49 m), but the saturation at rest did not show a difference. Three of them received corrective surgery to close defects over 3-5 years.
For some selected adult sPH with uncorrected CHD, PAB can work as a palliative procedure to improve their functional class and even provide a chance of total repair.
International journal of cardiology 09/2011; 151(3):313-7. DOI:10.1016/j.ijcard.2010.05.067 · 4.04 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Pulmonary regurgitation and prolonged QRS duration of right bundle branch (RBB) block are common in repaired tetralogy of Fallot (TOF) and increase the risk of sudden death. We sought to establish an animal model to reflect both abnormalities. Twenty-one canines: group I (n = 7) received a surgical right ventricular outflow tract (RVOT) transannular patch plus pulmonary valve destruction; group II (n = 5) received RBB ablation and sham operation; and group III (n = 9) received combined interventions. Serial electrophysiological data were obtained up to 1 y. Procedure mortality was 27.6%. At 1 y, although severe pulmonary regurgitation was documented in most dogs in groups I (71%) and III (100%), progressive RVOT dilatation was noted in group III. RBB block was present in all dogs in groups II and III. However, the increments of QRS duration, QTc, JTc, and QT dispersion progression between 1 mo and 1 y were all greatest in group III. Ventricular arrhythmia events were frequent in group III (median 3.3/mo) but uncommon in groups I and II (median 1/mo). We have created a novel animal model that adequately reflects both the hemodynamic and electrophysiological characteristics of repaired TOF patients and can be applied to examine the risk of ventricular arrhythmias.
Pediatric Research 05/2011; 70(3):247-52. DOI:10.1203/PDR.0b013e318225fd8b · 2.31 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Microvolt T wave alternans (MTWA) is an indicator of repolarization heterogeneity and a predictor of ventricular arrhythmia in adults with ischemic or dilated cardiomyopathy. Its implication in patients with repaired tetralogy of Fallot (TOF) is still unclear. This study sought to define the changes and the clinical implication of MTWA in these patients.
Treadmill examination with modified moving average beat analysis (MMA) for MTWA was performed in 101 repaired TOF patients (60.4% male). Data from 103 age- and sex-matched subjects with normal hearts served as controls.
The age at latest follow-up was 20.0 ± 10.6 years. Total repair (60.4% received a transannular right ventricular outflow patch) was performed at a mean age of 4.8(± 5.8) years. After excluding 11 patients with indeterminate data, the MTWA data in 90 TOF patients revealed higher values than those in the control (25.1 ± 14.0 vs. 17.6 ± 9.2 μV, p<0.001). The values were best correlated to the presence of severe pulmonary regurgitation (p=0.006). Ten (9.9%) patients experienced late ventricular arrhythmic events. They tended to have higher MTWA values than those without (34.0 ± 16.5 vs. 24.2 ± 13.5 and p=0.053). Although the MTWA per se would not predict the late arrhythmia events better than QRS duration alone, the positive and negative predictive values increased slightly after adding the MTWA to QRS duration.
MTWA, as measured by MMA, increased in repaired TOF patients particularly in those with severe pulmonary regurgitation and late arrhythmia events. To predict late ventricular arrhythmia, MTWA however was not superior to QRS duration alone.
International journal of cardiology 03/2011; 159(3):220-4. DOI:10.1016/j.ijcard.2011.02.063 · 4.04 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Surgical repair of tetralogy of Fallot (TOF) in countries with sound medical care systems is seldom delayed until adolescence. This study investigated the clinical profile and the surgical outcomes in such a population from Taiwan. Between 1970 and 2009, 179 TOF patients (56% male) received total repair at 19.2 ± 8.3 (10-49) years of age. We reviewed the medical records and interviewed the patients concerning their current status. The survival was ascertained in all by a link to our national health database. Major morbidities before cardiac repair included atrial arrhythmia (1.1%), ventricular arrhythmia (3.9%), infective endocarditis (6.7%), brain abscess (4.6%) and pulmonary tuberculosis (3.3%). Ventricular arrhythmia and pulmonary tuberculosis occurred mainly after 20 years of age. Thirty patients (16.8%) received a palliative shunt. The preoperative QRS duration increment was 0.6 ms/year. Early mortality occurred in 4 (2.2%) and was related to previous shunt surgery (OR = 16.5, p < 0.05) and coronary artery crossing RVOT (OR 17.6, p < 0.05). After repair, the functional class improved in all patients. The median age at latest follow-up was 31.8 (32.8 ± 12.3) years. The survival was 92.7 and 89.3% at 20 and 30 years after operation, respectively. Late cardiovascular death could be predicted by the length of postoperative intensive care unit stay (OR = 1.3, p < 0.001). The freedom from ventricular arrhythmia 30 years after repair was 84.1% and was associated with a final QRS longer than 160 ms. Unrepaired TOF patients were at high risk of infective endocarditis, brain abscess, pulmonary tuberculosis and arrhythmias during their adolescence and adulthood. Cardiac repair in this age group was still safe and effective.
Heart and Vessels 02/2011; 27(1):65-70. DOI:10.1007/s00380-011-0119-3 · 2.07 Impact Factor