Hemodynamic and Echocardiographic Characteristics and the Presence of Pulmonary Hypertension in Patent Ductus Arteriosus Patients who Underwent Transcatheter Closure.

We investigated the hemodynamic parameters of pediatric PDA patients and focused on the influence of PDA size on pulmonary arterial pressure and the prevalence of pulmonary hypertension. A total of 52 patients aged between 2 months and 20 years who received transcatheter closure of a PDA from January 2018 to June 2022 in our institution were retrospectively recruited. Their hemodynamic parameters collected both by echocardiography and by cardiac catheterization were analyzed to delineate the influence of PDA size on the pulmonary vascular system. The echocardiographic-based ductal size and indexed PDA size were 1.93 mm (1.15-6 mm) and 4.05 mm/m2 (2.03-25.47 mm/m2), respectively. The pulmonary artery pressure measured was 20.83 mmHg (8-45 mmHg). We found a positive correlation between indexed PDA size and mean pulmonary arterial pressure (mPAP) (Pearson correlation coefficient = 0.47, p < 0.001). A subgroup analysis showed that 28 patients (53.8%) developed pulmonary hypertension (PH) (defined as mPAP > 20 mmHg). The median age of the PH group was 1.02 years [range: 0.19-8.64], which was significantly younger than the non-PH group's median age of 3.43 years [range: 0.42-19.96] (p = 0.001). The indexed PDA size for the PH group, 4.69 mm/m2, was significantly higher than that of the non-PH group, 3.2 mm/m2 (p = 0.004). The major risk factor for patients with PH was the PDA/BSA index, with an OR of 2.181 (95% CI, 1.224-3.887). Our demographic data showed younger patients with a higher PDA/BSA index are more likely to develop pulmonary hypertension.

Diagnosis and Surgical Outcomes of Patients with Anomalous Left Coronary Artery from the Pulmonary Artery: A Single Taiwanese Medical Center Experience.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA), a very rare congenital cardiac anomaly, is associated with a high mortality rate among infants who are not diagnosed or treated in a timely manner. Surgical intervention with the reconstruction for a two-coronary-system circulation is the main treatment; however, there have been very few reported cases from Taiwan. In this study, we aim to describe the clinical manifestations, diagnostic methods, surgery types, and surgical outcomes in patients with ALCAPA from a single Taiwanese medical center. METHODS: We retrospectively reviewed patients diagnosed with ALCAPA who underwent surgery at our institution between January 2001 and October 2018. Clinical presentations, noninvasive and invasive study results, surgical methods, and postoperative follow-up results were assessed from medical records. Moreover, literature on this particular cardiovascular anomaly was reviewed. RESULTS: The study included 6 patients (5 children and 1 adult). The diagnosis was confirmed using cardiac catheterization and coronary angiography in four patients and only echocardiography in two patients. All patients underwent surgical correction and survived. Four patients showed improvements in left ventricular function and mitral regurgitation (MR). CONCLUSION: Early diagnosis and timely surgical intervention could avoid mortality regardless of the method of operation. ALCAPA can be definitively diagnosed using noninvasive echocardiography. Both left ventricular systolic function and mitral insufficiency could improve after the surgical intervention in pediatric patients. Repair or replacement of the mitral valve could be reserved for persistent MR complicated with congestive heart failure, particularly in patients who received the initial operation beyond infancy.

Distraction using virtual reality for children during intravenous injections in an emergency department: A randomised trial.

AIM AND OBJECTIVES: To determine whether the virtual reality as a distracting intervention could reduce pain and fear in school-age children receiving intravenous injections at an emergency department. BACKGROUND: An intravenous injection is the most common invasive procedure that paediatric patients encounter in emergency department. School-age children seldom show their fear or discomfort during the procedure which may be ignored. DESIGN: A randomised controlled trial was conducted from December 2017-May 2018 and performed according to the CONSORT guidelines. METHODS: One hundred and thirty-six children aged 7-12 years were randomly allocated to receive either a routine intravenous injection procedure or one with an immersive virtual reality experience. Children were asked to rate their pain and fear along with their caregivers and nurses on the Wong-Baker FACES Pain Rating Scale and Children's Fear Scale, respectively. The time required for successful intravenous insertion was also assessed in the emergency department. Clinical trial registration was done (ClinicalTrials.gov.: NCT04081935). RESULTS: Pain and fear scores were significantly lower in the virtual reality group, as were the children's ratings as perceived by their caregivers and nurses. The children's ratings of pain and fear were positively correlated with the caregivers' ratings and the nurses' ratings as well. The time required for successful intravenous insertion was significantly lower in the virtual reality group. CONCLUSION: Visual reality intervention can effectively reduce the pain and fear during intravenous procedure in school-age children in emergency department. RELEVANCE TO CLINICAL PRACTICE: The results of this study indicate the feasible clinical value of virtual reality interventions during the administration of intravenous injections in school-age children in emergency departments.

A Novel and Simple Algorithm Using Surface Electrocardiogram That Localizes Accessory Conduction Pathway in Wolff-Parkinson-White Syndrome in Pediatric Patients.

BACKGROUND: The location of the accessory pathway (AP) can be precisely identified on surface electrocardiography (ECG) in adults with Wolff-Parkinson-White (WPW) syndrome. However, current algorithms to locate the AP in pediatric patients with WPW syndrome are limited. OBJECTIVE: To propose an optimal algorithm that localizes the AP in pediatric patients with WPW syndrome. METHODS: From 1992 to 2016, 180 consecutive patients aged below 18 years with symptomatic WPW syndrome were included. After the exclusion of patients with non-descriptive electrocardiography (ECG), multiple APs, congenital heart diseases, non-inducible tachycardia, and those who received a second ablation, 104 patients were analyzed retrospectively. Surface ECG was obtained before ablation and evaluated by using previously documented algorithms, from which a new pediatric algorithm was developed. RESULTS: Previous algorithms were not highly accurate when used in pediatric patients with WPW syndrome. In the new algorithm, the R/S ratio of V1 and the polarity of the delta wave in lead I could distinguish right from the left side AP with 100% accuracy. The polarity of the delta wave of lead V1 could distinguish free wall AP from septal AP with an accuracy of 100% in left-side AP, compared to 88.6% in leads III and V1 for right-side AP. The overall accuracy was 92.3%. CONCLUSIONS: This simple, novel algorithm could differentiate left from right AP and septal from free wall AP in pediatric patients with WPW syndrome.

Improvement in the sensitivity of newborn screening for Fabry disease among females through the use of a high-throughput and cost-effective method, DNA mass spectrometry.

Many female carriers of Fabry disease are likely to develop severe morbidity and mortality. However, by our own estimation, around 80% of female newborns are missed by our current enzyme-based screening approach. Our team's aim was to develop an improved cost-effective screening method that is able to detect Fabry disease among female newborns. In Taiwan, based on a database of 916,000 newborns, ~98% of Fabry patients carry mutations out of a pool of only 21 pathogenic mutations. An Agena iPLEX platform was designed to detect these 21 pathogenic mutations using only a single-assay panel. A total of 54,791 female infants were screened and 136 female newborns with the IVS4 + 919G > A mutation and one female newborn with the c.656T > C mutation were identified. Using the current enzyme-based newborn screening approach as baseline, around 83% of female newborns are being missed. Through a family study of the IVS4 female newborns, 30 IVS4 adult family members were found to have left ventricular hypertrophy. Ten patients received endomyocardial biopsy and all were found to have significant globotriaosylceramide (Gb3) accumulation in their cardiomyocytes. All of these individuals now receive enzyme replacement therapy. We have demonstrated that the Agena iPLEX assay is a powerful tool for detecting females with Fabry disease. Furthermore, through this screening, we also have been able to identify many disease-onset adult family members who were originally undiagnosed for Fabry disease. This screening helps them to receive treatment in time before severe and irreversible cardiac damage has occurred.

Initial validation of a healthcare needs scale for young people with congenital heart disease.

AIM: To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. BACKGROUND: As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. DESIGN: A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease. METHODS: The sample consisted of 500 patients with congenital heart disease, aged 15-24 years, from paediatric cardiology departments and covered the period from March-August 2015. The patients completed the 25-item Healthcare Needs Scale for Youth with Congenital Heart Disease, the questionnaire on health needs for adolescents and the WHO Quality of Life-BREF. Reliability and construct, concurrent, predictive and known-group validity were examined. RESULTS: The Healthcare Needs Scale for Youth with Congenital Heart Disease includes three dimensions, namely health management, health policy and individual and interpersonal relationships, which consist of 25 items. It demonstrated excellent internal consistency and sound construct, concurrent, predictive and known-group validity. CONCLUSION: The Healthcare Needs Scale for Youth with Congenital Heart Disease is a psychometrically robust measure of the healthcare needs of youth with congenital heart disease. It has the potential to provide nurses with a means to assess and identify the concerns of youth with congenital heart disease and to help them achieve a successful transition to adult care.

Atrial tachycardia originating from the atrial septum in a patient with dextrocardia and complex structural heart disease.

We report a case with dextrocardia, corrected transposition of the great arteries. He also had an atrial septum defect (ASD) with patch repair. Activation map showed a centrifugal activation from a focal origin on the systemic lower left atrial ASD patch. Ablation of the origin can terminate the atrial tachycardia.

Various modalities for evaluation of a fused heart in conjoined twins.

Conjoined twins are a rare congenital anomaly with an estimated incidence of 1/50,000 to 1/100,000. Among thoracopagus conjoined twins, 75% have a fused heart. We compare the usefulness of various modalities for evaluating cardiovascular structure in fused-heart conjoined twins. We report a series of 20 sets of thoracopagus conjoined twins as well as the results of a PubMed database literature review literature from 1982 to 2009. Twenty sets of fused-heart thoracopagus conjoined twins were evaluated by echocardiography, cardiac catheterization, magnetic resonance image (MRI), and three-dimensional computed tomography angiography (3D-CTA). Imaging results were compared to findings at surgery or autopsy. All sets of conjoined twins underwent postnatal echocardiography; 11 sets (55%) underwent cardiac catheterization; 4 sets (20%) underwent MRI; and 1 set (5%) underwent 3D-CTA. All intracardiac anatomy (ICA) was identified by echocardiography. Cardiac catheterization, MRI, and 3D-CTA were able to identify extracardiac vascular structures as well as the ICA. 3D-CTA, which can be performed as early as the first week of life, is a noninvasive, less expensive, and the safe examination with minimal risk due to its short procedural time. Three-dimensional CTA is an effective and safe modality for evaluating the cardiovascular anatomy of fused-heart conjoined twins before surgery.

Clinical manifestations vary with different age spectrums in infants with Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis with unknown etiology. The diagnosis of KD depends on clinical manifestations. The prevalence of coronary artery abnormality (CAA) is 11.0% and results in cardiac sequelae, such as myocardial infarction or coronary aneurysm, which are the most serious complications in KD. METHODS: We divided KD's children into different age groups: ≤6 months old, 7 months to 1 year old, and >1 year old, respectively. Different parameters were compared in each group. RESULTS: Infants ≤6 months old are less likely to fulfill KD's major diagnostic criteria within 10 days, are prone to develop incomplete KD with the lowest cholesterol level, and have the greatest chance to have CAA and the laboratory features associated with CAA, such as the longest time needed to confirm CA diagnosis, lower hemoglobin level, lower albumin level, and higher platelet count. Infants <1 year old develop higher percentage of leukocytosis and sterile pyuria. But this group has fewer patients with neck lymphadenopathy.

The specific characteristics in children with obstructive sleep apnea and cor pulmonale.

BACKGROUND: The prevalence of obstructive sleep apnea (OSA) in the pediatric population is currently estimated at 1-2% of all children. The purpose of this study was to investigate the clinical and hemodynamic characteristics in pediatric patients with cor pulmonale and OSA. METHODS: Thirty children with the diagnosis of OSA were included. These patients consisted of 26 male and 4 female children with a mean age of 7 ± 4 years old. Five of those children were found to be associated with cor pulmonale, and 25 had OSA but without cor pulmonale. RESULTS: The arousal index was much higher in children with OSA and cor pulmonale. The children with OSA and cor pulmonale had much lower mean and minimal oxygen saturation and a higher incidence of bradycardia events. All 5 patients with OSA and cor pulmonale underwent an adenotonsillectomy, and the pulmonary arterial pressure dropped significantly after the surgery. CONCLUSION: This study demonstrated that the OSA pediatric patients with cor pulmonale had the different clinical manifestations and hemodynamic characteristics from those without cor pulmonale. The adenotonsillectomy had excellent results in both the OSA pediatric patients with and without cor pulmonale.

Resuscitated Sudden Cardiac Arrest of a Neonate with Congenital LQT Syndrome-Associated Torsades de Pointes: A Case Report and Literature Review.

Sudden infant death syndrome (SIDS), the most common cause of infant death in developed countries, is attributed to diverse trigger factors. Malignant cardiac dysrhythmias are potentially treatable etiologies, and congenital long QT syndrome (LQTS) is the most common cardiac ionic channelopathy confronted. ß-Blockers or class Ib agents are the drugs of choice for the control of arrhythmias, and an implantable cardioverter defibrillator (ICD) should be considered for secondary prevention in survivors of lethal cardiac death. We report the case of a 4-day old neonate, later genetically confirmed as LQT type 3 (LQT3), who survived a pulseless torsades de pointes (TdP) attack and was successfully treated with propranolol, mexiletine, and ICD implantation.

Implantation of monocusp valve prolongs the duration of chest tube drainage in children with tetralogy of fallot after corrective surgery.

BACKGROUND: Right ventricular outflow tract obstruction relief is one of the major procedures during the total correction of tetralogy of Fallot (TOF). Pulmonary insufficiency (PI) is usually inevitable after a transannular incision with a patch repair is performed. Therefore, some surgeons advocate to place a monocusp valve within the transannular patch (TAP) in order to decrease the severity of the PI. However, the monocusp valve seemed not be very effective in some patients who underwent the complete TOF repair. METHODS: Patients who had the classic form of TOF between January 2009 and January 2017 and underwent the corrective surgery with a TAP by the same cardiovascular surgeon were identified for further analysis. Clinical information including demographics at operation, perioperative data, and postoperative outcome were collected retrospectively and compared between the group with and without a monocusp valve. RESULTS: A total of 24 TOF cases were included in the final analysis, and 16 (66.7%) patients received a monocusp valve placement. The patients' characteristics before and during the surgery were similar between the two groups. The median duration of chest tube drainage after the total correction in the monocusp group was longer than those without the valve (p = 0.04). There was no difference in the immediate postoperative data, including the inflammation/infection status, the duration of mechanical ventilation, and the length of ICU and hospital stay. CONCLUSION: Implantation of a monocusp valve during the total TOF correction using a TAP did not bring benefit to improve the immediate postoperative outcomes, especially the duration of the pleural drainage. Further study with a prospective design and a larger number of cases is needed.

Pharmacologic interventions for Kawasaki disease in children: A network meta-analysis of 56 randomized controlled trials.

BACKGROUND: Although the current consensus recommends a standard treatment of high-dose intravenous immunoglobulin with high-dose aspirin to manage Kawasaki disease (KD), the use of different adjunctive therapies remains controversial. The aim of the current network meta-analysis (NMA) was to compare the efficacy and tolerability of different existing interventions for the initial and refractory stages of KD. METHODS: An NMA of randomised controlled trials (RCTs) was conducted using the frequentist model applied after electronic searches in PubMed, Embase, ScienceDirect, ProQuest, ClinicalTrials.gov, ClinicalKey, Cochrane CENTRAL, and Web of Science. The main outcomes were reduced fever duration/diminished severity of fever subsided. The initial stage of KD was defined as the first stage to treat patients with KD; the refractory stage of KD represents KD patients who failed to respond to standard KD treatment. The cut-off points for intravenous immunoglobulin (IVIG) were low (100-400 mg), medium (1 g), and high (at least 2 g). FINDINGS: A total of fifty-six RCTs with 6486 participants were included. NMA demonstrated that the medium-dosage IVIG + aspirin + infliximab [mean difference=-1.76 days (95% confidence intervals (95% CIs): -3.65 to 0.13 days) compared to high-dosage IVIG + aspirin] exhibited the shortest fever duration; likewise, the medium-dosage IVIG + aspirin + infliximab [odds ratio (OR)=0.50, 95% CIs: 0.18-1.37 compared to high-dosage IVIG + aspirin] exhibited the smallest incidence of coronary artery lesion (CAL) in the initial-stage KD. In the refractory-stage KD, the high-dosage IVIG + pulse steroid therapy (OR=0.04, 95% CIs: 0.00-0.43 compared to the high-dosage IVIG only) had the best rate of decline of fever; likewise, the high-dosage IVIG + ciclosporin [OR=0.05 (95% CIs: 0.00-1.21) compared to the high-dosage IVIG only] exhibited the smallest incidence of CAL. Infliximab significantly improved resolution compared to the high-dosage IVIG only group (OR=0.20, 95%CIs: 0.07-0.62) in refractory-stage KD. INTERPRETATION: The NMA demonstrated that the combination therapy with the standard therapy of IVIG and aspirin might have an additional effect on shortening the duration of fever and lowering the CAL incidence rate in patients with acute KD. Moreover, the combination therapy with high-dose IVIG and pulse steroid therapy or cyclosporine therapy might have an additional effect on improving the rate of decline of fever and lowering the incidence rate of CAL in children with refractory KD. Because some of the findings of this NMA should be considered hypothesis-generating rather than confirmatory, further evidence from de novo randomised trials is needed to support our results. FUNDING: None.

The impact of age on the electrophysiological characteristics and different arrhythmia patterns in patients with Wolff-Parkinson-White syndrome.

INTRODUCTION: Information about the impact of age on the electrophysiological characteristics of accessory pathways (APs) in patients with Wolff-Parkinson-White (WPW) syndrome remains limited. METHODS AND RESULTS: A total of 1,885 consecutive patients (mean age 43 ± 17 years, male 61.5%) with WPW syndrome who were referred to the tertiary center for an electrophysiological study and radiofrequency catheter ablation were investigated. The patients were divided into 4 groups based on their age (Group 1: <20; Group 2: 20-39; Group 3: 40-59; Group 4: ≧60 years old). With age, more left-sided APs (53.2%, 67.7%, 71.7%, 75.7%, P < 0.001) and a longer duration of the arrhythmia (4.3 ± 2.8 years, 10.1 ± 7.0 years, 12.4 ± 10.9 years, 14.0 ± 12.4 years, P < 0.001) were noted. The incidence of concealed APs (53.5%, 53.0%, 57.8%, 60.9%, P = 0.01), and orthodromic atrioventricular (AV) reentrant tachycardia (92.4%, 94.2%, 96.5%, 96.3%, P = 0.023) increased with age. The tachycardia cycle length, antegrade (275.5 ± 42.2 ms, 286.7 ± 62.7 ms, 302.5 ± 66.5 ms, 315.2 ± 80.2 ms, P < 0.001) and retrograde AP effective refractory periods (APERPs) (254.0 ± 42.5 ms, 263.3 ± 51.8 ms, 274.5 ± 100.5 ms, 292.7 ± 57.0 ms, P < 0.001), atrial ERP, antegrade AV node effective refractory period (AVNERP), and ventricular effective refractory period (VERP) lengthened as the age increased. The incidence of decremental APs, multiple APs, and a catecholamine response were similar. The duration of the catheter ablation, total fluoroscopy time, acute success rate, complication rate, and incidence of a secondary procedure were similar between the different age groups. CONCLUSION: The electrophysiological characteristics and pattern of the arrhythmic attack associated with the AP changed with age.

Paradoxical aging changes of the atrioventricular nodal properties in patients with atrioventricular nodal re-entrant tachycardia.

BACKGROUND: This study aimed to investigate the impact of aging on electrophysiological characteristics in patients with atrioventricular nodal re-entrant tachycardia (AVNRT). METHODS AND RESULTS: The 2,111 patients who underwent an electrophysiological study and radiofrequency (RF) catheter ablation of AVNRT were enrolled. The patients were divided into 4 groups according to age (group 1: < 20 years; group 2: 20-39 years; group 3: 40-59 years; and group 4: ≥ 60 years). The gender distribution differed with age. The atrio-Hisian interval, and effective refractory periods (ERP) of the right atrium, ventricle, antegrade slow pathway, retrograde slow pathway and fast pathway, and tachycardia cycle length all increased with age. However, a paradoxical change in the fast pathway ERP was noted. The fast pathway ERP was significantly longer in group 2 than in other groups, and was associated with the largest tachycardia window. The response to catecholamines was similar between different age groups. Procedure time, radiation time, and complications did not differ. However, the number of RF impulses was higher in group 2 compared with other groups (7.6 ± 9.3, P=0.04), which might imply a differing complexity during the ablation. CONCLUSIONS: Paradoxical aging changes of AVN electrophysiological characteristics were associated with a different atrioventricular nodal conduction property and the number of RF impulses.

Aortic regurgitation in Marfan syndrome patients who underwent prophylactic surgery: A single-center experience.

BACKGROUND: Marfan syndrome is an inherited connective tissue disease that causes aortic root dilatation and dissection and requires surgical intervention. Apart from emergent surgery for aortic dissection or aortic aneurysmal rupture, prophylactic surgical intervention can also be administered, depending on the severity of aortic root dilatation. The direct relationship between surgical intervention and aortic regurgitation was seldom mentioned in previous studies. METHODS: A retrospective cohort study was designed to determine the clinical presentations of prophylactic surgery in patients with Marfan syndrome. Between January 2009 and May 2019, 112 patients, adolescents and young adults, treated in the Department of Pediatric Cardiology of Taipei Veterans General Hospital, were enrolled. All patients' sex, body measurements, echocardiography reports, and surgical notes were collected for statistical analysis. RESULTS: Among the participants, nine patients (8%) underwent the Bentall procedure, and the other 103 did not receive surgical intervention. The operation group had a larger aortic root size (4.89 vs 2.86 cm, p < 0.001), more dilated left ventricle (4.81 vs 4.1 cm, p = 0.002), and higher prevalence of moderate and severe aortic regurgitation (66% vs 1%, p < 0.001) than the nonoperation group. CONCLUSION: Among adolescents and young adults with Marfan syndrome, echocardiographic presentation of aortic root dilatation, left ventricular dilatation, and significant aortic regurgitation was significantly associated with prophylactic surgical intervention. According to the study, significant aortic regurgitation should also be considered as an important indication for prophylactic surgery.

Association Between Tetralogy of Fallot and Psychiatric Disorders: A Nationwide Cohort Study.

OBJECTIVE: The tetralogy of Fallot (TOF) has been reported to be associated with some neurodevelopmental impairment and psychiatric disorders. Nevertheless, a nationwide study to clarify the risk between TOF and comorbid psychiatric disorders is lacking. Using a nationwide database in Taiwan, this study aimed to explore the role of TOF in various psychiatric disorders and analyze whether there are patient-related risk factors. METHODS: A total of 16,824 enrolled patients, including 4,206 study subjects who were diagnosed with TOF and 12,618 controls with TOF matched (1:3) for sex, age, hospital visits, and index year, were randomly selected from the Taiwanese National Health Insurance Research Database (NHIRD) between 2000 and 2015. Patients' diagnoses in the NHIRD were encoded using International Classification of Diseases, 9th Revision, Clinical Modification codes. RESULTS: Of patients with TOF, 256 (6.09%) developed psychiatric disorders compared to 394 (3.12%) in the control group. After adjusting for covariates, the adjusted hazard ratio of psychiatric disorders for patients with TOF was 3.192 (95% CI, 2.683-3.798; P < .001). After exclusion of psychiatric diagnoses within the first 5 years, TOF was associated with an increased risk of anxiety (P < .001), depression (P < .001), bipolar disorder (P < .001), and sleep disorders (P = .005). CONCLUSIONS: This study revealed that TOF patients have a nearly 3-fold higher risk of psychiatric disorders, including anxiety, depressive, bipolar, and sleep disorders, than the general population. Therefore, continued mental health screening and surveillance are warranted in TOF patients.

Gender differences in the clinical characteristics and atrioventricular nodal conduction properties in patients with atrioventricular nodal reentrant tachycardia.

INTRODUCTION: The detailed electrophysiological characteristics of the gender differences associated with atrioventricular nodal reentrant tachycardia (AVNRT) have not been clarified. This study investigated the gender-related electrophysiological differences in a large series of patients undergoing radiofrequency catheter ablation. METHODS AND RESULTS: A total of 2,088 consecutive AVNRT patients (men/women 869/1,219) who underwent catheter ablation were enrolled in this study. We evaluated the gender differences in their electrophysiological characteristics. Women had a significantly younger age of onset, higher incidence of multiple jumps, shorter AH interval, atrial effective refractory period (ERP), anterograde fast pathway ERP, anterograde slow pathway ERP, and retrograde slow pathway ERP, and longer ventricular ERP than men. The incidence of baseline ventriculoatrial dissociation was lower in women than in men. Women needed less isoproterenol/atropine to induce AVNRT. No gender differences in the radiation exposure time, procedure time, complication rate, acute success rate, or second procedure rate were noted. Both typical and atypical AVNRT were more predominant in women. In the patients with atypical AVNRT, there was no significant gender difference in incidence of baseline ventriculoatrial dissociation; however, the retrograde slow pathway ERP was significantly shorter in women than in men. Women of premenopausal age (≤50 years old) had a significantly higher incidence of anterograde multiple jumps and a retrograde jump phenomenon, and a shorter anterograde slow pathway ERP and retrograde slow pathway ERP than those of women over 50 years old. CONCLUSION: Gender differences in the anterograde and retrograde AV nodal electrophysiology were noted in the patients with AVNRT.

Enzyme assay and clinical assessment in subjects with a Chinese hotspot late-onset Fabry mutation (IVS4 + 919G→A).

Newborn screening for Fabry disease in Taiwan Chinese has revealed a high incidence of the late-onset GLA mutation IVS4 + 919G→A (∼1 in 1,500-1,600 males). We studied 94 adults with this mutation [22 men, 72 women; mean age: men 57.8 ± 6.0 (range 42-68), women 39.1 ± 14.1 years (range 19-82)]. Plasma α-galactosidase A activity assay was 10.4 ± 11.2% of normal in the men and 48.6 ± 19.5% of normal in the women. Echocardiography in 90 of the adults revealed left ventricular hypertrophy (LVH) in 19 (21%), including 14 of 21 men (67%) and 5 of 69 women (7%). Microalbuminuria, based on the urine albumin-to-creatinine ratio measured on at least two occasions, was present in 17 of 86 subjects (20%) (men: 5/20, 25%; women 12/66, 18%). At least one ocular manifestation consistent with Fabry disease was present in 41 of 52 subjects (79%) who underwent ophthalmologic examination, including 8 (15%) with conjunctival vessel tortuosity, 15 (29%) with cornea verticillata, 10 (19%) with Fabry cataract, and 34 (65%) with retinal vessel tortuosity. Among subjects over 40 years of age, men were more likely than women to have LVH [14/21 (67%) vs 5/25 (20%), p < 0.001]. Cardiovascular, renal and ocular abnormalities are highly prevalent in adult Taiwan Chinese subjects with the Fabry mutation IVS4 + 919G→A. Our findings contribute to the limited understanding of the course of this late-onset disease variant and underscore the need for close follow up in such patients.