Genetic and clinical characteristics of catecholaminergic polymorphic ventricular tachycardia in a Taiwanese nationwide cohort.

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and lethal arrhythmia. Ryanodine receptor 2 (RYR2) mutation accounts for ∼60% of CPVT patients which is inherited in an autosomal dominant pattern. OBJECTIVE: This study aimed to identify CPVT-related mutations and clinical characteristics among Taiwanese CPVT patients and compare to other cohorts worldwide. METHODS: Clinical and genetic data were obtained from the Sudden Arrhythmia Death Syndrome Registry in Taiwan (SADS-TW). Forty clinically diagnosed Taiwanese CPVT patients were included. RESULTS: This is the first nationwide CPVT cohort in Taiwan. Among the 29 Taiwanese patients with CPVT-related gene mutations, 55% had RYR2 mutations, a rate similar to other ethnicities. Three out of 12 RYR2 variants were unreported. Exercise-induced symptoms including syncope and cardiac arrest were more frequent in East Asian cohorts (Taiwanese 79%, Japanese 91%), compared to Caucasian cohorts (59%) (p = 0.002). CONCLUSION: The discovery of diverse RYR2 mutations in the Taiwanese CVPT population demonstrates the importance of genetic testing in different ethnicities.

Multicenter registry of multisystem inflammatory syndrome in children (MIS-C) and Paired comparison with Kawasaki disease.

OBJECTIVES: This study aimed to identify clinical characteristics to differentiate multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) in Taiwan, an island with a delayed cluster of MIS-C and a high incidence of KD. Additionally, we studied risk factors for developing severe complications in patients with MIS-C. METHODS: We conducted a retrospective, multicenter, cohort, and observational study that linked data on patients with MIS-C between May and December 2022 and patients with KD between 2019 and 2021 from 12 medical centers. Hemodynamic compromise, defined as the need for inotropic support or fluid challenge, was recorded in patients with MIS-C. We also evaluated maximal coronary Z-scores before treatment and one month after disease onset. RESULTS: A total of 83 patients with MIS-C and 466 patients with KD were recruited. A 1:1 age and gender-matched comparison of 68 MIS-C and KD pairs showed that MIS-C patients had a lower percentage of positive BCG red halos, lower leukocyte/platelet counts, more gastrointestinal symptoms, and a higher risk of hemodynamic compromise. In Taiwan, 38.6% of MIS-C patients experienced hemodynamic compromise, with presence of conjunctivitis and elevated levels of procalcitonin (>1.62 ng/mL) identified as independent risk factors. CONCLUSIONS: We identified two independent risk factors associated with hemodynamic compromise in MIS-C patients. The comparison between matched MIS-C and KD patients highlighted significant differences in clinical presentations, like BCG red halos, which may aid in the differential diagnosis of the two disease entities, especially in regions with a high incidence rate of KD.

Atrial flutter/fibrillation in adult congenital heart disease: Insights from a large Asian cohort.

BACKGROUND: The incidence of atrial fibrillation/atrial flutter (AF/AFL) in general population is lower in Asia compared to Western countries. It is unclear whether a similar trend exists among adults with congenital heart disease (ACHD). We determine the profile, risk factors, and impact of AF/AFL in an Asian ACHD cohort. METHODS: We included all ACHD patients diagnosed in an Asia tertiary care center between 2007 and 2018, analyzing AF (sustained and paroxysmal AF) and AFL, collectively./Purpose. RESULTS: The study encompassed 4391 patients (55.9% women), with 81% having simple, 16.3% moderate and 2.8% severe CHD. AF/AFL was observed in 6.7% of the patients, with 54.6% having paroxysmal AF, 27.3% sustained AF, and 18.1% AFL. Incidence of AF/AFL increased with age and was higher in patients with pulmonary hypertension (PH), severe CHD, and metabolic syndrome. We found a progressive trend in the onset age of arrhythmia: AFL at a younger age, followed by paroxysmal and sustained AF. Risk factors for AF/AFL included severe and moderate CHD, PH, previous interventions, and male sex (odds ratio 11.2 and 3.15, 2.03, 1.75, and 1.71, respectively). When stratifying by CHD severity, PH and male sex were significant risk factors in simple CHD, while only PH in severe CHD. Patients with AF/AFL had a significantly lower major adverse cardiovascular events-free survival rate. CONCLUSIONS: This large ACHD cohort from Asia exhibited a high incidence of AF/AFL, similar to Western reports. The risk of AF/AFL was primarily associated with hemodynamic factors such as PH and CHD severity.

Secular trend of Kawasaki disease and its correlation with viral activity in Taiwan: a nationwide population-based study.

BACKGROUND: Kawasaki disease (KD) is the most important acquired heart disease in children. This study investigated annual incidence, seasonality, secular trend and the correlation of KD incidence with viral activity in Taiwan. METHODS: Through the national health insurance database, we identified KD during 2001-2020. The viral activity was obtained from nationwide surveillance database. We analyzed KD age-specific annual incidence, secular trends, seasonality and the correlation between KD incidence and common enteric or respiratory viral activity. RESULTS: The KD incidence of subjects younger than 18 years significantly increased from 2001 to 2020 (11.78 and 22.40 per 100,000 person-years, respectively), and substantially decreased with age. Infants younger than 1 year presented the highest KD annual incidence at 105.82 to 164.34 per 100,000 person-years from 2001 to 2020. For all KD patients, the most frequently occurring season was summer followed by autumn. The KD incidence of infants younger than 1 year had significantly positive correlation with enteric (r = 0.14) and respiratory (r = 0.18) viral activity. CONCLUSIONS: This study demonstrates the increasing trend of KD annual incidence and seasonality (more in summer and autumn) in Taiwan. The activity of common respiratory and enteric viruses was significantly correlated with KD incidence in infants.

Liver fibrosis detected by diffusion-weighted magnetic resonance imaging and its functional correlates in Fontan patients.

OBJECTIVES: The evaluation of Fontan-associated liver disease is often challenging. Diffusion-weighted magnetic resonance imaging can detect hepatic fibrosis from capillary perfusion and diffusion abnormalities from extracellular matrix accumulation. This study investigated its role in the evaluation of liver disease in Fontan patients and explored possible diagnostic methods for early detection of advanced liver fibrosis. METHODS: Stable adult Fontan patients who could safely be examined with magnetic resonance imaging were enrolled, and blood biomarkers, transient elastography were also examined. RESULTS: Forty-six patients received diffusion-weighted imaging; and 58.7% were diagnosed with advanced liver fibrosis (severe liver fibrosis, 37.0%, and cirrhosis 21.7%). Two parameters of hepatic dysfunction, platelet counts (Spearman's ρ: -0.456, P = 0.001) and cholesterol levels (Spearman's ρ: -0.383, P = 0.009), decreased with increasing severity of fibrosis. Using transient elastography, a cut-off value of 14.2 kPa predicted the presence of advanced liver fibrosis, but with a low positive predictive value. When we included platelet count, cholesterol, post-Fontan years and transient elastography values as a composite, the capability of predicting advanced liver fibrosis was the most satisfactory (C statistic 0.817 ± 0.071, P < 0.001). A cut-off value of 5.0 revealed a sensitivity of 78% and a specificity of 82%. CONCLUSIONS: In Fontan patients, diffusion-weighted imaging was helpful in detecting liver fibrosis that was correlated with hepatic dysfunction. A simple score was proposed for long-term surveillance and early detection of advanced liver disease in adult Fontan patients. For adult Fontan patients with a calculated score > 5.0, we may consider timely diffusion-weight imaging and early management for liver complications.

Risk Factors of Long-Term Sequelae After Transcatheter Closure of Perimembranous Ventricular Septal Defect in Young Children.

BACKGROUND: Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR), have been observed. However, the associated risk factors remain unclear. This study identified risk factors linked with residual shunts and AR following transcatheter closure of pmVSD in children aged 2-12 years. METHODS AND RESULTS: The medical records of 63 children with pmVSD and a pulmonary-to-systemic blood flow ratio <2.0 who underwent transcatheter closure between 2011 and 2018 were analyzed with a minimum 3-year follow-up. The success rate of transcatheter closure was 98.4%, with no emergency surgery, permanent high-degree atrioventricular block, or mortality. Defects ≥4.5 mm had significantly higher odds of persistent residual shunt (odds ratio [OR] 6.85; P=0.03). The use of an oversize device (≥1.5 mm) showed a trend towards reducing residual shunts (OR 0.23; P=0.06). Age <4 years (OR 27.38; 95% confidence interval [CI] 2.33-321.68) and perimembranous outlet-type VSD (OR 11.94, 95% CI 1.10-129.81) were independent risk factors for AR progression after closure. CONCLUSIONS: Careful attention is crucial for pmVSDs ≥4.5 mm to prevent persistent residual shunts in transcatheter closure. Assessing AR risk, particularly in children aged <4 years, is essential while considering the benefits of pmVSD closure.

Association between Cesarean section delivery and increased risk of childhood Kawasaki disease.

AIM: Cesarean section delivery is associated with microbiota disruption and immuno-dysregulation during childhood, but the association with Kawasaki disease remains uncertain. We aimed to evaluate the association between Cesarean section and Kawasaki disease. METHODS: We examined the association between Kawasaki disease between six and eighteen months and Cesarean section within a birth cohort of 15,796 mother-infant pairs in Taiwan. The associations were assessed with Poisson regression in the study population, in the 1:2 propensity score-matched subpopulation, and compared with febrile convulsion, trauma and accidents during the same interval as negative control outcomes. RESULTS: Cesarean section was found to increase the risk of Kawasaki disease among overall population (adjusted relative risk [aRR]: 2.22, 95 % confidence interval (CI): 1.14-4.34) and the matched subpopulation (aRR: 2.29, 95 % CI: 1.14-4.68 in PS-matched subpopulation). Meanwhile, there was no association between Cesarean section and the clinic visits for febrile convulsion, trauma and accidents. CONCLUSION: In conclusion, this study identified a potential association between Cesarean section delivery and a higher risk of Kawasaki disease during six-to eighteen months of the prospective birth cohort in Taiwan.

Atrial fibrillation in adult congenital heart disease and the general population.

BACKGROUND: Atrial fibrillation (AF) in adult patients with congenital heart disease (ACHD) may appear early, depending on individual characteristics. OBJECTIVES: The goals of this study were to investigate the epidemiological spectrum of AF in the entire cohort of ACHD and compare it with that in the general population. METHODS: A retrospective study was performed in the nationwide cohort 2000-2014 with AF onset during 2003-2014. RESULTS: In the cohort of ACHD, 2350 patients had AF; the incidence increased with age, plateauing around age 70. In patients aged 25-29, 45-49, 65-69, 75-79, and ≥80 years, the annual incidence was 1.3, 7.9, 20.6, 23.7, and 21.4/1000 per year, respectively. In the general population without CHD, 347,979 patients had AF; the annual incidence was <1/1000 per year in those aged <55 years but increased steadily with age (3.6, 8.6, and 14.2/1000 per year in aged 65-69, 75-79, and ≥80 years, respectively). Compared with individuals without ACHD, ACHD patients aged <50 years and those aged both 50-54 and 55-59 years exhibited a 20-fold and 10-fold higher incidence of AF, respectively. Patients with complex congenital heart disease and Ebstein's anomaly had the highest risk of AF (cumulative risk >10% by age 50 and >20% by age 60), followed by those with tetralogy of Fallot, tricuspid atresia, endocardial cushion defect, and secundum atrial septal defect (cumulative risk >5% by age 50 and >10% by age 60). CONCLUSION: Compared with individuals without ACHD, AF in patients with ACHD likely appeared 30 years earlier, with a 10- to 20-fold higher incidence plateauing around age 70. Yet, incidence in individuals without ACHD continued to increase. AF burden in patients with ACHD is not expected to increase in a never-ending way.

Combination of slow pathway late activation maps and voltage gradient maps in guidance of atrioventricular nodal reentrant tachycardia cryoablation.

BACKGROUND: The optimal strategy for electroanatomic mapping-guided cryoablation of atrioventricular nodal reentry tachycardia (AVNRT) remains unclear. OBJECTIVE: The purpose of this study was to investigate the effectiveness of slow pathway late activation mapping (SPLAM) and voltage gradient mapping for AVNRT cryoablation. METHODS: From June 2020 to February 2022, all consecutive patients with AVNRT underwent SPLAM to define the wave collision point and voltage gradient mapping to define the low-voltage bridge (LVB). Conventional procedures performed from August 2018 to May 2020 served as control. RESULTS: The study and control groups comprised 36 patients (age 16.5 ± 8.2 years) and 37 patients (age 15.5 ± 7.3 years), respectively. Total procedural times were comparable, and acute success rates were 100% in both groups. Compared to controls, the number of cryomapping attempts (median 3 vs 5; P = .012) and cryoablation applications (median 1 vs 2; P <.001) were significantly lower in the study group. At median follow-up of 14.6 and 18.3 months, recurrence rates were 5.6% (2 patients) and 10.8% (4 patients) in the study and control groups (P = .402), respectively. Mapping of the Koch triangle took 11.8 ± 3.6 minutes, during which 1562 ± 581 points were collected. In SPLAM, wave collision points were defined and compatible with the final successful lesion sites in all patients, including those with multiple slow pathways. LVB could not be defined in 6 patients (16.7%), and LVB was not compatible with the final successful lesion in another 6 (16.7%). CONCLUSION: For AVNRT cryoablation, SPLAM could effectively guide the localization of slow pathway ablation sites and was particularly beneficial in patients with multiple slow pathways.

Self-Expanding Pulmonary Valves in 53 Patients With Native Repaired Right Ventricular Outflow Tracts.

BACKGROUND: Self-expanding pulmonary valve grafts have been designed for percutaneous pulmonary valve implantation (PPVI) in patients with native repaired right ventricular (RV) outflow tracts (RVOTs). However, their efficacy, in terms of RV function and graft remodelling remain unclear. METHODS: Patients with native RVOTs who received Venus P-valve (N = 15) or Pulsta valve (N = 38) implants between 2017 and 2022 were enrolled. We collected data on patient characteristics and cardiac catheterization parameters as well as imaging and laboratory data before, immediately after, and 6 to 12 months after PPVI and identified risk factors for RV dysfunction. RESULTS: Valve implantation was successful in 98.1% of patients. The median duration of follow-up was 27.5 months. In the first 6 months after PPVI, all patients exhibited resolution of paradoxical septal motion and a significant reduction (P < 0.05) in RV volume, N-terminal pro-B-type natriuretic peptide levels, and valve eccentricity indices (-3.9%). Normalization of the RV ejection fraction (≥ 50%) was detected in only 9 patients (17.3%) and was independently associated with the RV end-diastolic volume index before PPVI (P = 0.03). Nine patients had residual or recurrent pulmonary regurgitation or paravalvular leak (graded as ≥ mild), which was associated with a larger eccentricity index (> 8%) and subsided by 12 months postimplantation. CONCLUSIONS: We identified the risk factors likely to be associated with RV dysfunction and pulmonary regurgitation following PPVI in patients with native repaired RVOTs. RV volume-based patient selection is recommended for PPVI of a self-expanding pulmonary valve, along with monitoring of graft geometry.

The implications of airway anomalies on children with congenital heart disease and bronchiolitis.

BACKGROUND: Bronchiolitis is a common airway infection in young children. Hemodynamically significant congenital heart disease (CHD) predicts a more complicated course. However, the role of airway anomalies remains unknown. METHODS: We retrospectively reviewed the records of patients under 2 years old, diagnosed with CHD, and admitted between January 2011 and December 2013, before the palivizumab era. Records of bronchiolitis admissions were also extracted. Patients were grouped according to CHD condition and airway anomalies. RESULTS: A total of 230 patients with CHD were enrolled. A total of 180 (78%) and 71 (31%) patients had hemodynamically significant CHD and airway anomalies, respectively. A total of 52 (22.6%) patients were admitted for bronchiolitis 78 times. Among them, 33 (63.5%) had hemodynamically significant CHD, and 28 (53.8%) had airway anomalies. In patients with bronchiolitis admissions, the mean ventilator use, intensive care unit stay, and hospital stay were 1.08, 4.08, and 15.19 days, respectively. When compared, the mean hospital stay for bronchiolitis patients with airway anomalies was significantly longer than that of those without airway anomalies (19.8 vs. 9.9 days, p = 0.008). When further divided the patients by the presence hemodynamic significance, patients with hemodynamically significant CHD and airway anomaly had longer hospital stay than those who had neither. (21.7 vs. 8.3 days, p = 0.004) Airway anomaly was a significant risk factor for longer hospital stay in linear regression model (p = 0.007). CONCLUSIONS: Airway anomalies are common in children with CHD and are associated with longer hospital stays on bronchiolitis admission. An active survey for airway anomalies and adequate prophylaxis for bronchiolitis infection might be important in the care of children with CHD associated with airway anomalies.

Changes of ECG parameters after BNT162b2 vaccine in the senior high school students.

The purpose of this study is to determine the ECG parameter change and the efficacy of ECG screening for cardiac adverse effect after the second dose of BNT162b2 vaccine in young population. In December 2021, in cooperation with the school vaccination system of Taipei City government, we performed a ECG screening study during the second dose of BNT162b2 vaccines. Serial comparisons of ECGs and questionnaire survey were performed before and after vaccine in four male-predominant senior high schools. Among 7934 eligible students, 4928 (62.1%) were included in the study. The male/female ratio was 4576/352. In total, 763 students (17.1%) had at least one cardiac symptom after the second vaccine dose, mostly chest pain and palpitations. The depolarization and repolarization parameters (QRS duration and QT interval) decreased significantly after the vaccine with increasing heart rate. Abnormal ECGs were obtained in 51 (1.0%) of the students, of which 1 was diagnosed with mild myocarditis and another 4 were judged to have significant arrhythmia. None of the patients needed to be admitted to hospital and all of these symptoms improved spontaneously. Using these five students as a positive outcome, the sensitivity and specificity of this screening method were 100% and 99.1%, respectively.  Conclusion: Cardiac symptoms are common after the second dose of BNT162b2 vaccine, but the incidences of significant arrhythmias and myocarditis are only 0.1%. The serial ECG screening method has high sensitivity and specificity for significant cardiac adverse effect but cost effect needs further discussed. What is Known: • The incidence of cardiac adverse effects was reported to be as high as 1.5 per 10 000 persons after the second dose BNT162b2 COVID-19 vaccine in the young male population based on the reporting system. What is New: • Through this mass ECG screening study after the second dose of BNT162b2 vaccine we found: (1) The depolarization and repolarization parameters (QRS duration and QT interval) decreased significantly after the vaccine with increasing heart rate; (2) the incidence of post-vaccine myocarditis and significant arrhythmia are 0.02% and 0.08%; (3) The serial ECG screening method has high sensitivity and specificity for significant cardiac adverse effect.

Computed tomography jellyfish angiography in pediatric endovascular interventions.

Contrast pooling (CP) reconstruction is widely used in computed tomography (CT) studies of congenital heart diseases. However, endovascular devices are usually obscured in CP. To improve visualization of the vascular lumen, we developed jellyfish angiography (JFA), a semitransparent blood pool inversion technique. Ten CT studies of patent ductus arteriosus (PDA) or coarctation of the aorta (CoA) were selected retrospectively for reconstruction using both CP and JFA. Four of the studies were conducted before the endovascular intervention, and six were conducted after the intervention. Radiology residents and pediatric cardiologists completed questionnaires regarding the reconstruction models. For radiology residents, JFA was superior to CP in postintervention PDA diagnosis, device evaluation, and overall satisfaction. For pediatric cardiologists, JFA outperformed CP in both PDA and CoA postintervention cases. Our findings show that JFA overcomes the disadvantages of CP and can improve the visualization of intraluminal devices which is essential for endovascular treatment evaluation.

Management of cardiovascular symptoms after Pfizer-BioNTech COVID-19 vaccine in teenagers in the emergency department.

BACKGROUND: Cardiovascular complications after Pfizer-BioNTech COVID-19 (BNT) vaccination are a concern, especially in adolescents. We analyzed the risk factors for myocarditis after BNT vaccination. METHODS: We used a special evaluation protocol for all patients aged 12-18 years who presented to our emergency department with cardiovascular symptoms after BNT vaccination. RESULTS: A total of 195 patients (109 boys and 86 girls) were enrolled. Eleven (5.6%) patients presented with arrhythmia (arrhythmia group), 14 (7.2%) had a diagnosis of pericarditis/myocarditis (the peri/myocarditis group), and the remaining 170 were controls (no cardiac involvement). Chest pain (77.6%) was the most common symptom. The median time from vaccination to symptom onset was 3 days. In the peri/myocarditis group (13 myocarditis and 1 pericarditis), the median time to the peak troponin T level was 5 days after vaccination. Abnormal electrocardiographic changes, including ST-T changes and conduction blocks, were more commonly detected in the peri/myocarditis group (85.7% vs. 12.4% in the control group, p < 0.01). Echocardiography revealed normal ventricular function in all patients. Symptoms were resolved before discharge in all, with the median duration of hospital stay being 4 days. The electrocardiography was the most appropriate screening tool for myocarditis, with a sensitivity and specificity of 85.7% and 87.6%, respectively. CONCLUSION: Pericarditis or myocarditis was diagnosed in 7.2% of adolescents presenting to the emergency department with cardiovascular symptoms after BNT vaccination. In addition to the troponin T level, ECG change listed above can be used as a screening tool for vaccine-induced cardiac complications.

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.

Outcomes of pediatric patients supported with ventricular assist devices single center experience.

BACKGROUND: There has been a remarkable increase in the number of pediatric ventricular assist device (VAD) implanted over the past decade. Asian pediatric heart centers had not participated in the multicenter registries among the Western countries. This article aimed to report the outcomes of pediatric VAD in our hospital. METHODS: The study enrolled all patients aged <18 years at the time of VAD implantation in our institution between 2008 and 2021. RESULTS: There were 33 patients with diagnosis of acute fulminant myocarditis (n = 9), congenital heart disease (n = 5), dilated cardiomyopathy (n = 16), and others. Paracorporeal continuous-flow pump was the most frequently implanted (n = 27). Most of the devices were implanted in patients with INTERMACS profile 1 (n = 24). The median duration on VAD was 22 days (range 2-254). The proportion of patients attaining positive outcomes (alive on device, bridge to transplantation or recovery) was 72.7% at 1 month, 67.7% at 3 months, and 67.7% at 6 months. Most of the deaths on device occurred within the first month post-implant (n = 9), with neurological complications being the most frequent cause of death. All recovered cases were successfully weaned off the device within the first month of implantation. CONCLUSION: We demonstrated a favorable outcome in pediatric patients supported with VAD at our institution.

Microrna-486-5P Regulates Human Pulmonary Artery Smooth Muscle Cell Migration via Endothelin-1.

Pulmonary arterial hypertension (PAH) is a fatal or life-threatening disorder characterized by elevated pulmonary arterial pressure and pulmonary vascular resistance. Abnormal vascular remodeling, including the proliferation and phenotypic modulation of pulmonary artery smooth muscle cells (PASMCs), represents the most critical pathological change during PAH development. Previous studies showed that miR-486 could reduce apoptosis in different cells; however, the role of miR-486 in PAH development or HPASMC proliferation and migration remains unclear. After 6 h of hypoxia treatment, miR-486-5p was significantly upregulated in HPASMCs. We found that miR-486-5p could upregulate the expression and secretion of ET-1. Furthermore, transfection with a miR-486-5p mimic could induce HPASMC proliferation and migration. We also found that miRNA-486-5p could downregulate the expression of SMAD2 and the phosphorylation of SMAD3. According to previous studies, the loss of SMAD3 may play an important role in miRNA-486-5p-induced HPASMC proliferation. Although the role of miRNA-486-5p in PAH in in vivo models still requires further investigation and confirmation, our findings show the potential roles and effects of miR-486-5p during PAH development.

Epidemiology of Cardiomyopathy in Taiwanese Population Aged Younger Than 65 Years.

Background: Few studies have investigated the epidemiology of cardiomyopathy (CMP) in the general population in Taiwan. The aim of this study was to investigate this issue. Methods: We identified patients aged < 65 years and diagnosed with CMP between 2001 and 2014 from the National Health Insurance Database of Taiwan 2000-2014. Those with known or presumed causes of CMP were further identified. Results: We identified 38,868 CMP patients (male/female = 2.13). Half had known or presumed causes of CMP, including coronary artery disease (23.6%), congenital heart disease (1.6%), metabolic disease (8.4%), conduction disturbance/dyssynchrony (2.2%), myocarditis (0.5%), muscular dystrophy (1.42%), Kawasaki disease (0.2%), nutrition problems or alcoholism (2.9%), and unspecified causes (12.4%). The incidence rates of CMP without known causes were 1.13 and 8.70 per 100,000 person-years in pediatric (0-19 years) and adult (20-64 years) populations, respectively. After an initial peak during infancy (9.16 per 100,000 person-years), the incidence declined to a nadir in those aged from 5 to 14 years, and then steadily increased during adulthood (26.51 per 100,000 person-years in those aged 60-64 years). Although mortality was higher in the pediatric (11.4%) than in the adult (1.5%) patients, the proportion of sudden death to all deaths was similar in the pediatric (9.9%) and adult (10.5%) patients. Conclusions: This study provides an epidemiological continuum of CMP in a Taiwanese population aged < 65 years. The results revealed an initial peak during infancy, followed by a decline in adolescence and a subsequent steady rise. The prognosis was poorer in the pediatric patients, and poorest in the infants. However, the risk of sudden death was the same in the adult and pediatric patients.