Taipei consensus on integrative traditional Chinese and Western Medicine.

BACKGROUND: Traditional Chinese medicine and western medicine have coexisted since 1958 in Taiwan. Integrative traditional Chinese and western medicine (TC&WM) remains to be studied and promoted. In response to the documentary report of WHO Traditional Medicine Strategy 2002-2005, the present study was planned and carried out. METHODS: During 2004-2008, 19 integrative TC&WM dialogue forums were held, in which 219 TC&WM scholars and professionals participated by invitation. The proceedings of the forums in Chinese were published. A study team was organized in 2009 to collect the consensus opinions, utilizing a Delphi method. The opinions collected were discussed in an international TC&WM forum held on November 1, 2014. RESULTS: The opinions of TC&WM experts and professionals on the integrative issues and values were quite divergent. Of the 39 integrative issues, 34 (87.8%) reached consensus, agreeing that WM is excellent in the diagnosis and treatment of diseases/disorders, yet is still evolving, and not perfect without defects. TCM is patient-centered, wellness-oriented, inadequate for acute, critical and life-threatening diseases, but has a complementary and alternative role to WM. Of the 44 diseases/disorders, 36 (81.8%) reached consensus, worthy for integrative clinical use or trials. CONCLUSIONS: Integrative TC&WM, combining the best features of two systems, could be a most useful and advanced healthcare medicine in the future, requiring development of regulations and guidelines for the use of TCM and more rigorous efforts have to be made in clinical trials.

Changing Spectrum of Cardiac Diseases in Children: An Extended Longitudinal Observation Study of a Pediatric Cardiac Screening Program.

BACKGROUND: The spectrum of cardiac diseases in children is distinct from that in adults, and changes with advances in medical care and socioeconomic conditions. Such data are important for the allocation of medical resources, but are unclear. Based on a longitudinal pediatric cardiac screening program, we sought to delineate the spectrum and the trends. METHODS: From 2002 to 2018, citywide screening programs of cardiac diseases for children were performed in Taipei, Taiwan. Participants, mainly students entering the first grade of elementary school, were evaluated using questionnaires, simplified 4-lead electrocardiography (EKG), phonocardiography, and physical examinations. Those suspected of having abnormal cardiac lesions received detailed evaluations by pediatric cardiologists for a final diagnosis. RESULTS: The median prevalence of cardiac lesions in the children was 19.60/1000 (range from 14.9 to 24.65/1000), including congenital heart disease (32.02%), EKG abnormalities (33.34%), mitral valve prolapse (15.17%), Kawasaki disease (6.89%), rheumatic heart disease (0.17%) and others (12.41%). A significant time trend was only observed in a trend of decline in rheumatic heart disease. The median prevalence rates of congenital heart disease, Kawasaki disease, rheumatic heart disease, mitral valve prolapse, and EKG abnormalities were 6.20, 1.37, 0, 3.12, and 6.46 per 1000 children, respectively. CONCLUSIONS: This study, based on a citywide cardiac screening program, indicates that the spectrum of cardiac diseases in children has not changed in recent decades, except for a decline or disappearance of rheumatic heart disease in the Taipei metropolitan area. While congenital heart disease and EKG abnormalities were the most common, Kawasaki disease was the most commonly acquired heart disease in children.

Cardiac Screening for High Risk Sudden Cardiac Death in School-Aged Children.

BACKGROUND: Sudden cardiac death (SCD) is an uncommon but significant cause of death in the young. Citywide cardiac screening of school-aged children has been performed in Taipei since 1989. In this study, we investigate the efficacy of this screening method for identifying those at high risk of SCD. METHODS: This study analyzed the data from the results of cardiac screening for school-aged children in Taipei from 2003 to 2014. The cardiac screening included: Stage I, questionnaire surveys, simplified phonocardiography test and simplified electrocardiography (ECG) test; Stage II, physical examination and auscultation by a pediatric cardiologist for all children who had abnormal findings in stage I screening; Stage III, referral to a pediatric cardiologist for further examinations. Logistic regression and decision tree analyses were performed. RESULTS: A total of 566,447 students were screened, of whom 685 were identified as being at high risk of SCD. The most common causes of being at high risk of SCD included Wolff-Parkinson-White syndrome, long QT syndrome, cardiomyopathy and Marfan's syndrome. Using logistic regression analysis, the simplified ECG test was identified as being the most effective tool (odds ratio = 16.4, p < 0.001) and past history as the second most crucial factor (odds ratio = 3.95, p < 0.001) for detecting a high risk of SCD. Decision tree analysis showed that serial studies with a past history and the simplified ECG test could accurately identify those at high risk of SCD. CONCLUSIONS: Questionnaire survey and simplified electrocardiography test-based cardiovascular screening in school-aged children can identify those at high risk of SCD.

Study on ECG in the Adolescent.

Normal ECG values in newborns, infants, and children have been collected and published. ECG in the adolescent, however, remains, to be collected and studied. The present study was designed and carried out to establish the normal ECG standards in male and female adolescents. A total of 898 school children and adolescents screened and examined as healthy were divided by age and sex into 6-9, 9-13, and 13-18 years age-groups. A 12 lead conventional ECG was recorded in 10 mm/mV and 25 mm/s, utilizing an automated Fukuda Denshi FCP-4301, MS-DOS/IBM-AT ECG machine. Lead V3R was not taken. Analog-to-digital conversion was performed by Fukuda signal acquisition module at a sampling rate of 500 Hz. The data on 69 ECG parameters were analyzed for the mean, standard deviation, 2nd to 98th percentiles, 95% confidence intervals, and sex difference. Normal values on 69 ECG parameters, sex-specific heart rate, P-QRS-T interval, duration, axis, wave amplitude, and calculated R/S amplitude ratio and ventricular activation time by age-group and sex were established. Male and female difference was noted in 49 (71.0%) parameters, of which 3 (6.1%) began in 6-9 years age-group, 30 (61.2%) began in 9-13 years age-group, and 16 (32.7%) in 13-18 years age-group. No sex difference occurred in 20 (29.0%) parameters. Normal male and female ECG standards on 69 ECG parameters in the adolescent were established. ECG sex difference began to appear the earliest at ages 6-9 years, and it occurred mostly at ages 9-13 years and 13-18 years, reflecting the anatomical and physiological consequences of puberty.

Six-Minute Walking Test: Normal Reference Values for Taiwanese Children and Adolescents.

BACKGROUND: 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. METHODS: 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. RESULTS: There was an overall linear trend of increase in the 6MWD between 7 and 17 years of age (p < 0.001). Males covered significantly more distance than females after the age of 14 years, when the 6MWD essentially plateaued in female adolescents. Upon multivariate analysis, height was the most significant positive predictor of the 6MWD, while body mass index negatively correlated with the 6MWD. The height-based normal reference values of the 6MWD, derived from the 6MWT conducted in the school settings, were validated by a second cohort who received 6MWT inside the hospital. CONCLUSIONS: Normal reference values of the 6MWD in healthy Taiwanese children and adolescents may serve as useful references for future clinical and research studies. KEY WORDS: Adolescents; Children; Six-minute walking test; Taiwan.

Long-term outcomes of pediatric sinus bradycardia.

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.

Normal ECG standards and percentile charts for infants, children and adolescents.

BACKGROUND: Normal ECG standards in newborns, infants, children and adolescents have been collected and published by many authors. Only those by Davignon et al., Rijinbeek et al. and our two studies covered all ages from birth to adolescence. The standards reflecting the growth and development of the heart in infants, children and adolescents remained to be studied and explored. METHODS: We selected from our ECG database, after discussions and consultation, 15 key ECG parameters and analyzed for their age- and sex-specific mean, standard deviation and 2nd to 98th percentiles and their percentile charts were constructed. RESULTS: The ranges and distributions of the normal ECG standards, means and 2nd to 98th percentiles of 15 key parameters were established. CONCLUSION: A complete set of normal ECG standards of 15 key parameters from birth to adolescents is available for clinicians and researchers.

A shift from underweight to overweight and obesity in Asian children and adolescents with congenital heart disease.

BACKGROUND: In Western countries, obesity is a common problem in children with congenital heart disease (CHD). However, this problem may have racial difference, and little is known about the shift of this trend as patients grow up. The present study sought to investigate the prevalence and trends of being underweight, overweight and obesity in an Asian CHD cohort using a 5-year citywide school survey database. METHODS: Patient group consisted of 705 first grade elementary school students (children) and 219 first grade senior high school students (adolescents), while 18753 healthy children and 15014 healthy adolescents served as controls. Body mass index (BMI) percentile was calculated to define underweight (BMI < 15(th) percentile) and overweight (BMI 85(th) -95(th) percentile)/obesity (BMI ≥ 95(th) percentile). RESULTS: In CHD children, the prevalence of underweight and overweight/obesity was 21.0% (control 16%, P < 0.001) and 14.5% (control 19.8%, P < 0.001), respectively. Children with moderate to severe CHD, especially cyanotic CHD, were more underweight and less overweight/obese than children with non-cyanotic CHD. The prevalence of underweight (23.3%) and overweight/obesity (26.5%) in CHD adolescents became close to that in controls. From childhood to adolescence, different shifts in BMI distribution were noted; controls became more underweight and overweight/obese for males and more underweight and less overweight/obese for females, while CHD patients became more overweight/obese for both genders, including cyanotic CHD. CONCLUSIONS: In this Asian CHD cohort, we demonstrates a shift of BMI distribution from more underweight and less overweight/obese compared with healthy children, to a pattern similar to that in healthy adolescents.

Comparison of a Chinese Herbal Medicine (CCH1) and Lactulose as First-Line Treatment of Constipation in Long-Term Care: A Randomized, Double-Blind, Double-Dummy, and Placebo-Controlled Trial.

Many institutionalized patients and their healthcare providers are dissatisfied with current laxative therapy. This study compared therapeutic efficacy, safety, and laxative cost of an herbal formula (CCH1) and lactulose for long stay patients with constipation. In this double-blind, double-dummy, and placebo-controlled trial, we randomized 93 residents with chronic constipation from two long-term care facilities in Taiwan to receive either CCH1 with lactulose placebo or CCH1 placebo with lactulose for 8 weeks, then followed up for 4 weeks without study medication. Both treatments were effective and well tolerated for patients, but CCH1 produced more spontaneous bowel movements, less rectal treatments, less amount of rescue laxative, and lower laxative cost than lactulose during treatment. No significant differences were found in stool consistency, stool amount, global assessment, and safety concerns. In conclusion, our results suggest that CCH1 may have better efficacy and could be used as an alternative option to lactulose in the treatment of constipation in long-term care.

A pediatric clinic-based approach to early literacy promotion--experience in a well-baby clinic in Taiwan.

BACKGROUND/PURPOSE: Reach Out and Read (ROR) is an evidence-based intervention situated in pediatric offices and can help pediatricians to promote parents' reading to their children. The objective of this study was to determine if the program could also achieve good results in different culture, such as in Taiwan. METHODS: The intervention group (n=205) was enrolled from a well-baby clinic participated in a program modified from ROR (receiving anticipatory guidance and an appropriate children's book at a well-baby clinic) at a mean age of 9 months. The control group (n=210) was recruited from a general pediatric outpatient service at the compatible age. Both groups were queried about the reading habits of primary caregivers and the frequency of book sharing with their child. When children were at aged 12 to 18 month, follow-up questionnaires were collected. RESULTS: At follow-up, ANCOVA analysis indicated that the intervention group exhibited significantly greater increase in child-centered literacy scores (frequency of shared reading, reading as one of their three favorite interaction activities and child interested in shared reading). Caregivers were willing to accept their pediatrician's advice to read to their infants. CONCLUSION: In this study, the simple intervention, implemented at a well-baby clinic in Taiwan, changed Taiwanese parents' attitudes toward the importance of reading with their infants and toddlers. As primary health care providers, pediatricians are in a unique position to affect and encourage parental behaviors that foster early literacy development in children.

Severe hyponatremia due to ACTH insufficiency in a 14 year-old girl with growth hormone deficiency.

SIADH-like hyponatremia as the presenting manifestation of ACTH deficiency is rare in childhood. Here we report a 14 year-old girl who, after 8 years of GH replacement and subsequent treatment for subclinical secondary hypothyroidism, presented with confusion and disorientation due to severe hyponatremia. When her pituitary axis was re-assessed, she was diagnosed as having ACTH deficiency associated with multiple pituitary hormone deficiencies (MPHD) (including GH, FSH, LH, and subclinical TSH deficiencies). She responded poorly to treatment with only hypertonic fluid, but improved after addition of hydrocortisone replacement. The purpose of this paper is to emphasize the importance of suspecting ACTH insufficiency in children with GH deficiency if hyponatremia develops.

Dilated cardiomyopathy after long-term right ventricular apical pacing in children with complete atrioventricular block: role of setting of ventricular pacing.

BACKGROUND: Pediatric patients paced for complete atrioventricular block (CAVB) are at risk of developing dilated cardiomyopathy (DCM). We sought to explore the role played by the setting of ventricular pacing. METHODS AND RESULTS: A total of 35 patients <18 years of age at diagnosis were enrolled. Twenty-eight (80%) received pacemakers after a mean follow-up of 10 years, and most were paced from right ventricular (RV) apex (n = 23). None of the 7 patients without pacemakers developed DCM, whereas 8 (35%) paced from RV apex had DCM 2.6 +/- 2.2 years after pacing. The percentage of ventricular pacing was >90% in all patients. Multivariate analysis revealed that the cumulative Z score of lower ventricular rate setting was the single most important risk factor for DCM (HR, 3.14; 95% CI, 1.07-9.19; P = .037). Subgroup analysis in patients with VVI/VVIR modes revealed an even stronger predictive value of the cumulative Z score of lower ventricular rate setting (HR, 9.12; 95% CI, 1.53-54.24; P = .015). CONCLUSIONS: Higher setting of the lower ventricular rate, though still within the age-appropriate range, was associated with increased risk of developing DCM in pediatric patients with CAVB and chronic RV apical pacing.

Short-term sulpiride treatment of children and adolescents with Tourette syndrome or chronic tic disorder.

BACKGROUND/PURPOSE: Tourette syndrome (TS) is characterized by motor and vocal tics, and its diagnosis is based on clinical criteria. Dopamine-blocking neuroleptics are regarded as the most effective drugs for the treatment of TS. Sulpiride is a selective dopamine D2 antagonist. However, only one study with a large number of patients has reported the effect of treatment of TS with sulpiride. The purpose of this study was to evaluate prospectively the effect of sulpiride treatment of children and adolescents with TS or chronic tic disorder. METHODS: The inclusion criteria were patients who fulfilled the diagnosis of TS or chronic tic disorder, and who had not received previous treatment. The severity of TS was assessed by the Yale Global Tic Severity Score (YGTSS) every 2 weeks for a total of 6 weeks. The patients started treatment with low-dose sulpiride according to their age on the first visit. The adverse effects of sulpiride were evaluated by subjective complaints from the patients themselves or their parents. The change in scores between each assessment point was analyzed by repeated measures one-way analysis of variance, with SPSS version 12.0 software. RESULTS: One hundred and eighty-nine patients were enrolled. Their average age was 8.0 +/- 2.5 years (range, 3-15 years). Most patients were male (n = 165, 87.3%). Six weeks' treatment significantly improved motor tics (p < 0.05), vocal tics (p < 0.05) and total YGTSS (p < 0.05). The most commonly encountered adverse effect was sedation (n = 31, 16.4%). CONCLUSION: Sulpiride is effective for short-term treatment of children and adolescents with TS or chronic tic disorder, and has few adverse effects.

Cardiac conduction disturbance detected in a pediatric population.

OBJECTIVE: To delineate the prevalence and clinical implications of cardiac conduction disturbance (CCD) in school-age children. STUDY DESIGN: Between 1999 and 2001, a citywide survey of 432,166 elementary and high school students had been performed in Taipei by questionnaire, electrocardiography, phonocardiography, and physical examination. Patients with any abnormalities on this survey were referred for final diagnosis. RESULTS: After excluding those with congenital heart disease (CHD), the prevalence of CCD was 0.75%, higher in males than in females (0.78% vs 0.71%). Incomplete right bundle branch block (IRBBB; 0.32%), complete right bundle branch block (CRBBB; 0.11%), ventricular premature contraction (0.11%), and Wolff-Parkinson-White syndrome (0.067 %) were the most common diagnoses. Second-degree atrioventricular block, IRBBB, CRBBB, and intraventricular conduction delay were more common in males; and atrial premature contraction was more common in females. The prevalence of CCD increased with age, from 0.48% in elementary school students to 0.97% in high school students. After detection of CCD, 39 patients with previously undiagnosed atrial septal defect (ASD) and 15 high-risk patients were found. The sensitivity of IRBBB in screening for ASD was 34.67%. CONCLUSIONS: The prevalence of CCD in children without CHD was 0.75%. Detection of CCD helped identify patients with unrecognized ASD and high-risk cardiac patients.

Resolution of pathologic Q wave, left ventricular dysfunction and mitral regurgitation after dual coronary repair of the anomalous origin of the left coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.

A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system.

PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.

Trends in birth weight-specific and -adjusted infant mortality rates in Taiwan between 2004 and 2011.

BACKGROUND: A yearly increase in the proportion of very low birth weight (VLBW) live births has resulted in the slowdown of decreasing trends in crude infant mortality rates (IMRs). In this study, we examined the trends in birth weight-specific as well as birth weight-adjusted IMRs in Taiwan. METHODS: We linked three nationwide datasets, namely the National Birth Reporting Database, National Birth Certification Registry, and National Death Certification Registry databases, to calculate the IMRs according to the birth weight category. Trend tests and mortality rate ratios in the periods 2010-2011 and 2004-2005 were used to examine the extent of reduction in birth weight-specific and birth weight-adjusted IMRs. RESULTS: The proportion of VLBW (<1500 g) infants among live births increased from 0.78% in 2004-2005 to 0.89% in 2010-2011, thus exhibiting a 15% increase. The extents of the decreases in birth weight-specific IMRs in the 500-999, 1000-1499, 1500-1999, 2000-2499, and 2500-2999 g birth weight categories were 15%, 33%, 43%, 30%, and 28%, respectively, from 2004-2005 to 2010-2011. The reduction in IMR in each birth weight category was larger than the reduction in the crude IMR (13%). By contrast, the IMR in the <500 g birth weight category exhibited a 56% increase during the study period. The IMRs were calculated by excluding all live births with a birth weight of <500 g. The birth weight-adjusted IMRs, which were calculated using a standard birth weight distribution structure for adjustment, exhibited similar extent reductions. CONCLUSION: In countries with an increasing proportion of VLBW live births, birth weight-specific or -adjusted IMRs are more appropriate than other indices for accurately assessing the real extent of reduction in IMRs.

Progression of aortic regurgitation after surgical repair of outlet-type ventricular septal defects.

BACKGROUND: Progression of aortic regurgitation (AR) in repaired outlet ventricular septal defects (VSDs) remains unclear, especially for muscular outlet and perimembranous outlet VSDs. We tried to identify the risk factors for AR progression and aortic valve replacement (AVR) at long-term follow-up. METHODS: Four hundred patients with complete follow-up after the repair of their outlet VSD between 1987 and 2002 were studied. RESULTS: Juxta-arterial VSD, perimembranous outlet VSD, and muscular outlet VSD were noted in 190, 148, and 62 patients, respectively. There were 377 patients with none to mild AR (group I) and 23 with moderate to severe AR (group II) preoperatively. Aortic valve replacement was performed on 11 patients (all from group II), with 10 having received AVR concomitantly with VSD repair and 1 having received it 4 years later. Only severity of preoperative AR and older age (>15 years) at VSD repair were significant predictors of AVR. With a total follow-up of 2230 person-years, the 10-year freedom from AVR after VSD repair for group I was 100% and that for group II was 50.2%. In group I, AR progressed in 4 patients only (1.2%, 2 juxta-arterial and 2 perimembranous outlet) and aortic valvular (aortic valve prolapse or ruptured sinus Valsalva aneurysm) or subvalvular anomalies were present in all. The event-free (AR or AVR) survival rates among the 3 outlet-type VSDs however showed no difference. CONCLUSIONS: Aortic regurgitation progression modes after surgical VSD repair were similar among the 3 outlet-type VSDs. Aortic valve replacement was rarely necessary for patients who were operated on when they were younger than 15 years. Aortic regurgitation of a less-than-moderate degree preoperatively rarely progressed after VSD repair.

Coronary artery diameters in infants and children with congenital heart disease as determined by computed tomography.

This study was conducted to establish reference curves and formulas for the diameters of the coronary arteries in infants and children using computed tomography. A total of 145 children (57 female, 88 male) ranging in age from 2 days to 19 years, 11 months (mean 5 years, 10 months), were retrospectively identified, and the diameters of their coronary arteries were quantified. The measurability of the coronary arteries, coronary arterial size differences between the genders, and relations of the diameters of the coronary arteries to age, height, weight, body surface area, and the diameter of the descending aorta were examined. Independent-samples Student's t tests, 2-tailed Pearson's correlations, and linear regression were used in statistical analysis. The measurability of all coronary arteries was 73.3%. No difference in coronary arterial size was found between the genders. The diameter of the descending aorta correlated most strongly with coronary arterial size. In conclusion, predictive formulas and reference curves for coronary arterial diameters in infants and children were obtained.

Morphologic substrates for first-branch pulmonary arterial hypoplasia in transposition of the great arteries.

BACKGROUND: Distal right-sided outflow obstruction remains a problem after arterial switch operation. We studied the anatomical features of the pulmonary trunk (PT) and its branches that are susceptible to right and left pulmonary arterial (RPA and LPA) hypoplasia in transposition of the great arteries (TGA). METHODS: One hundred and one angiograms of TGA performed between 1981 and 1996 were viewed, and Polaroid photos were taken at end-systole. The diameters of RPA, LPA, PT, duct, ascending aorta, and angles between PA and PT were measured, and the ductal flow direction was recorded. RESULTS: Forty-eight cases (47.5%) had a PA/PT diameter ratio (both PAs had same size) below 0.49. A smaller PA/PT was significantly related to posterior inclination of the proximal PT [narrower right (r = 0.50, p < 0.00001) and left (r = 0.48, p < 0.00001) PA-PT angle in lateral view] and a larger duct (r = 0.37, p < 0.0001). Eighteen patients had a follow-up angiogram after a mean period of 8.5 months. Those with a closed duct had evident PA growth (n = 12, 0.51 +/- 0.09 to 0.74 +/- 0.17, p < 0.0001), but four patients with an attenuated duct had no significant change (0.58 +/- 0.06 to 0.68 +/- 0.08, p = NS), and one with a persistent large duct had even regression of PA/PT (0.36-0.19). The direction of ductal flow was toward the aorta during early systole on cineangiogram. CONCLUSIONS: First-branch PA hypoplasia, which is frequently seen in TGA, was related to the right-to-left shunt through a duct resulting in hemodynamic starvation, and to posterior inclination of the proximal PT in this setting. Natural regression of the duct facilitated PA growth.