Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome.

Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison. In 1995-2021, a total of 142 patients with ES and 192 children with PAH-CHD-predominantly L-R shunt were eligible for our analysis. The PVRi in ES patients was 26.7 ± 16.8 WU m2. Most patients (91%) received PAH-targeted therapy. Of the 192 children with PAH-CHD-predominantly L-R shunt, the baseline PVRi was 9.2 ± 5.8 WU m2. A total of 64 patients (33.3%) had borderline PVRi (4-8 WU m2) and 98 patients (51%) had high PVRi (> 8 WU m2). Most patients (88.5%) responded to acute pulmonary vasodilatory testing and underwent repair, with 158 undergoing defect closure and 12 having fenestrated closure. A treat-and-repair strategy was used in 33 children (17.1%). The 10- and 15-year survival rates for patients with ES were 79.3% and 72.4%, respectively, which was significantly inferior to children with borderline PVRi [97.3% and 87.8% (p = 0.02)]; and high PVRi [91.6% and 89.5% (p = 0.06)], respectively. The survival rate of children receiving treat-and-repair was slightly higher than that of ES (p = 0.16). The independent mortality risk in children with PAH-CHD-predominantly L-R shunt was persistent PAH following the defect correction (adjusted hazard ratio 5.8, 95% CI 1.7-19.9, p = 0.005).Trial registration: TCTR20200420004.

Manifestations of Rheumatic Carditis, Regression of Valvular Regurgitation, and Independent Predictors of Mitral Regurgitation Improvement After Rheumatic Carditis in Thai Children.

Background: Acute rheumatic fever (ARF) with carditis can lead to the development of rheumatic heart disease in children and young adults. Objective: This study aimed to investigate the manifestations of rheumatic carditis, clinically significant regression of valvular regurgitation as assessed by echocardiography, and the independent predictors of mitral regurgitation (MR) improvement after rheumatic carditis in Thai children. Method: Children diagnosed with rheumatic carditis during 2005-2020 at Siriraj Hospital (Bangkok, Thailand) were retrospectively enrolled. Trivial, and mild regurgitation were grouped as non-clinically significant (NCS) regurgitation. Valvular regression was defined moderate-severe regurgitation improving to NCS regurgitation. Results: Eighty-one patients (mean age: 10 years, range: 8-12 years) were included. At presentation, 59 (72.8%) patients had combined mitral regurgitation (MR) and aortic regurgitation (AR), 20 (24.6%) patients had MR alone, and 2 (2.4%) patients had AR alone. Concerning severity, 28 (34.6%) and 30 (37%) patients presented with severe and moderate MR, respectively. Severe and moderate AR was found in 9 (11.1%) and 16 (19.8%) patients, respectively. At the one-year follow-up, 43.4% of moderate-severe MR, and 41.7% of moderate-severe AR improved to NCS regurgitation. Multivariate analysis revealed high erythrocyte sedimentation rate (ESR) (p = 0.01) and severe carditis (p = 0.05) at presentation to be independent predictors of MR improvement. Conclusion: Thai children with rheumatic carditis had a high incidence of valvular regurgitation; however, the valvular damage was improved in most patients. High ESR and severe carditis independently predict MR improvement.

Aortic Root Dilatation Measured by Cardiac Magnetic Resonance in Patients with Repaired Tetralogy of Fallot.

BACKGROUND: Aortic root dilatation (AoD) frequently occurs following repaired tetralogy of Fallot (rTOF). The objective of this study was to assess aortic dimensions, investigate the prevalence of AoD, and identify predictors of AoD in rTOF patients. METHODS: A cross-sectional retrospective study was conducted in repaired TOF patients from 2009 to 2020. Aortic root diameters were measured by cardiac magnetic resonance (CMR). Severe AoD of the aortic sinus (AoS) was defined as a Z-score (z) of >4, reflecting a mean percentile ≥99.99%. RESULTS: Two hundred forty-eight patients, with a median age of 28.2 years (10.2-65.3 years), were included in the study. The median age at the time of repair was 6.6 years (0.8-40.5 years) and the median interval between the repair and CMR study was 18.9 years (2.0-54.8 years). The prevalence of severe AoD was found to be 35.2% when defined by an AoS z greater than 4 and 27.6% when defined by a AoS diameter ≥40 mm, respectively. A total of 101 patients (40.7%) had aortic regurgitation (AR), with 7 patients (2.8%) having moderate AR. Multivariate analysis revealed that severe AoD was only associated with the left ventricular end diastolic volume index (LVEDVi) and a longer duration after repair. The age at the time of repair for TOF was found not to be correlated with the development of AoD. CONCLUSIONS: After repair of TOF, severe AoD was found to be prevalent, but no fatal complications were observed in our study. Mild AR was also commonly observed. Larger LVEDVi and a longer duration after repair were identified as factors associated with the development of severe AoD. Therefore, routine monitoring of AoD is recommended.

Major infections following pediatric cardiac surgery pre- and post-CLABSI bundle implementation.

Background: Postoperative infection contributes to the worsening of congenital cardiac surgery (CCS) outcomes. Surgical site infection (SSI), bloodstream infection (BSI) and ventilator associated pneumonia (VAP) are common. An additional bundle of preventive measures against central-line associated bloodstream infection (CLABSI) bundle was implemented in April 2019. Objectives: To compare the incidence of major infections after pediatric CCS before and after the implementation of the CLABSI bundle and to identify risk factors for major infections. Methods: We conducted a single-center, retrospective study to assess the incidence of major infections including bloodstream infection (BSI), surgical site infection (SSI), and ventilator-associated pneumonia (VAP) after pediatric CCS one year before and after implementation of the CLABSI bundle during April 2018-March 2020. The demographics and outcomes of the patients were explored, and risk factors for major infections were identified using multivariate analysis. Results: A total of 548 children (53% male) underwent CCS with a median age of 1.9 years (range 0.01-17.5 years). The median Aristotle Basic Complexity score was 7.1 (range 3-14.5). The CLABSI bundle was applied in 262 patients. Overall mortality was 5.5%. 126 patients (23%) experienced major postoperative infections. During the year after the implementation of the CLABSI bundle, BSI was reduced from 8.4% to 3.1% (p = 0.01), with a smaller reduction in VAP (21% to 17.6%; p = 0.33). The incidence of SSI was unchanged (1.7% to 1.9%; p = 0.77). The independent risk factors for major infections were age at surgery <6 months (p = 0.04), postoperative ventilator usage >2 days (p < 0.01), central line usage >4 days (p = 0.04), and surgery during the pre-CLABSI bundle period (p = 0.01). Conclusion: Following the implementation of the CLABSI prevention package in our pediatric CCS unit, the incidence of BSI was significantly reduced. The incidence of VAP tended to decrease, while the SSI was unchanged. Sustainability of the prevention package through nurse empowerment and compliance audits is an ongoing challenge.

The First Successful Transcatheter Closure of an Inferior Sinus Venosus Defect with Anomalous Drainage of the Right Lower Pulmonary Vein Using Bare and Covered Stents: A Single-Case Report.

Inferior sinus venosus defect (SVD) is less common than a superior one. The lower edge of the defect straddles the orifice of the inferior vena cava, and this makes surgical repair via bicaval cannulation a technical challenge. The orifice of the unroofed right pulmonary vein is caused by the interatrial communication in sinus venosus defects which results in partial anomalous pulmonary vein drainage (PAPVD). Novel transcatheter closure of a superior SVD has recently been described; however, transcatheter closure of an inferior SVD has not yet been reported in the published literature. Here, we report the first successful transcatheter closure of an inferior SVD with bare and covered stents and the rerouting of a PAPVD into the left atrium to avoid occlusion of the hepatic veins. In this single-case report, we carefully describe the planning process, how the procedure was performed, and the steps taken to recapture and reposition a migrated stent. Careful patient selection and intensive assessment of pulmonary and hepatic vein anatomy before and during the procedure were necessary to achieve a successful outcome.

A retrospective cohort study of major adverse cardiac events in children affected by Kawasaki disease with coronary artery aneurysms in Thailand.

Kawasaki disease (KD) is a common form of vasculitis in children that can be complicated by coronary artery aneurysms (CAAs). Data of long-term outcomes and major adverse cardiac events (MACE) in children with CAAs following KD in developing country are limited. Our aims were to determine the rates of MACE and identify risk factors associated with MACE in children with KD and CAAs in Thailand. We performed a retrospective analysis of data from 170 children diagnosed with KD and CAAs in two tertiary hospitals between 1994 and 2019. During a median (range) follow-up of 5.4 years (22 days to 23 years), 19 patients (11.2%) experienced MACE, that included 12 coronary artery bypass grafting, 2 percutaneous coronary intervention and 5 children with evidence of myocardial ischemia and coronary occlusion. Coronary interventions were performed at a median time of 4 years (0.01 to 9.5 years) after KD diagnosis. Forty-nine patients (28.8%) had giant CAAs. No MACE was reported in children with small CAAs. Independent risks of MACE were from the absence of intravenous immunoglobulin treatment (HR 7.22; 95% CI 2.21 to 23.59; p = 0.001), the presence of giant aneurysms (HR 13.59; 95% CI 2.43 to 76.09; p = 0.003), and CAAs that involved bilateral branches of coronary arteries (HR 6.19; 95% CI 1.24 to 30.92; p = 0.026). Among children with giant CAAs, the intervention-free rate was 93.8%, 78.7% and 52.2%, at 1, 5 and 10 years, respectively. Of note, 81% of the small CAAs regressed to a normal size, and for medium CAAs, 50% regressed to normal size. Overall, ~10% of children with CAAs following KD experienced MACE in this cohort. Timely IVIG treatment in children with KD following symptom onset will reduce the risk of MACE. Cautious surveillance to identify cardiac complications should be recommended for children once medium or giant CAAs develop. Trial registration: TCTR20190125004.

Risk factors and long-term outcomes after tetralogy of Fallot repair at an Asian tertiary referral center.

BACKGROUND: Tetralogy of Fallot is the most common type of cyanotic congenital heart disease. More postoperative tetralogy of Fallot patients grow up than in the past, and these patients need to be followed-up. OBJECTIVE: To investigate the survival and long-term outcomes of patients who underwent total repair of tetralogy of Fallot, and to identify the risk factors for reoperation with pulmonic valve replacement. METHOD: A total of 403 patients who underwent total tetralogy of Fallot repair at our center during 1997 to 2016 were retrospectively included. Demographic, clinical, treatment, outcome, and follow-up data were collected and analyzed. RESULTS: Median age and body weight at the time of tetralogy of Fallot repair was 4.41 years (range: 0.85-55.28) and 13.58 kg (range: 5.5-68), respectively. The median follow-up was 9.0 years, and overall mortality was 3.2%. The actuarial survival rates at 10 and 20 years were 96.4% and 95.2%, respectively, and the freedom from pulmonic valve replacement was 93.4% and 57.4%, respectively. The median time to indicate pulmonic valve replacement was 13.9 years (range: 6.2-20.5). Multivariate analysis revealed transannular patch technique (hazard ratio: 3.023, 95% confidence interval: 1.34-6.83; p = 0.008) and palliative shunt (hazard ratio: 2.39, 95% confidence interval: 1.16-4.91; p = 0.018) to be independent risk factors for reoperation with pulmonic valve replacement. CONCLUSION: The rates of overall survival and freedom from pulmonic valve replacement were both high in this study, and both were comparable to the rates reported from other studies. Overall mortality was as low as 3.47%. The need for a transannular patch or palliative shunt should be considered risk factors for a consequent reoperation.

Improved low-risk criteria scores for combination therapy of sildenafil and generic bosentan in patients with congenital heart disease with severe pulmonary hypertension: A prospective open label study.

OBJECTIVE: We evaluated the efficacy and safety of the bosentan as a sequential add-on therapy with sildenafil in pulmonary arterial hypertension with congenital heart disease (PAH-CHD) patients. MATERIAL AND METHOD: Twenty patients who were receiving sildenafil were given generic bosentan for up to a year. Hemodynamic data was collected from cardiac catheterization at pretreatment and at three months. Comparisons were made between the total scores of the four, low-risk criteria adapted from the 2015 ESC/ERS pulmonary hypertension guidelines, which are: 1) WHO functional class of I or II, 2) 6MWD of more than 440 m, 3) right atrial pressure of less than 8 mm Hg, and 4) cardiac index ≥2.5 L/min/m2, performed at the beginning of therapy, 3-months, 6-months, and 1 year. RESULTS: Patients' average age was 27 ± 11 years old (12-53). PVRi decreased from 16.7 ± 9.5 to 12.7 ± 10.3 Wood unit (WU) m2 (p = 0.025) and PVRi/SVRi decreased from 0.69 ± 0.33 to 0.49 ± 0.32 (p = 0.001). During the follow-up, the composite scoring of the low risk scores for 19 patients was increased significantly from 1.8 ± 1.0 at baseline to 2.3 ± 0.9 at 3 months, to 2.9 ± 0.8 at 6 months, and 3 ± 0.7 at 1 year (p = 0.001). CONCLUSION: We demonstrated intermediate term benefits for generic bosentan as an add-on therapy to sildenafil in patients with PAH-CHD by improving PVRi, and PVRi/SVRi at three months. A significant improvement was also seen in the combined scores of the low-risk criteria from below 2 to 3 at one year (p = 0.001).Thai Clinical Trials Registry (TCTR): TCTR identification number is TCTR20200506006.

Long-term outcomes of repaired and unrepaired truncus arteriosus: 20-year, single-center experience in Thailand.

BACKGROUND: Truncus arteriosus (TA) is a complex congenital heart disease that carries morbidities in the first year of life. Previous authors have reported an operative mortality of 50%. In this report, we aim to report on the survival of patients with TA in our medical center in the recent era. METHODS: A retrospective review of all patients diagnosed with TA in Siriraj Hospital, Thailand from August 1995 to March 2018 was performed. Patients with single ventricle, hemiTA were excluded. The characteristics and outcomes of repaired and unrepaired TA patients with a known recent functional status in 2018 were reviewed. Operative mortality risks were analyzed using a multivariate model. RESULTS: A total of 74 patients (median age at referral: 70 days) were included in the cohort. One-third of the patients had associated anomalies including DiGeorge syndrome (13.5%). Anatomical repair was not performed in 22 patients (29.7%). The median age at time of repair for the 52 patients was 133 days (range: 22 days to 16.7 years). Complex TA was 10%. Early mortality occurred in 16 patients (30.8%). Five patients (9.6%) had late deaths at 0.3-1.2 years. Significant mortality risk was weight at time of operation <4 kg (HR 3.05, 95% CI [1.05-8.74], p-value 0.041). Of the 31 operation survivors, 17 required re-intervention within 0.4-11.4 years. Eight patients had reoperation at 8.7 years (range: 2.7-14.6 years) post-repair. Freedom from reoperation was 93%, 70.4%, and 31%, at 5, 10, and 15 years, respectively. All late survivors were in functional class I-II. Of the 22 unrepaired TA patients, 11 patients (50%) died (median age: 13.6 years; range: 14 days-32.8 years). Survival of unrepaired TA patients was 68.2%, 68.2%, and 56.8, at 5, 10, and 15 years of age, respectively. At the end of study, 11 survivors of TA with palliative treatment had a recent mean oxygen saturation value of 84.1 ± 4.8% and a mean weight for height of 81.4 ± 12.7%, which were significantly lower than those of 31 late-survivors who had undergone anatomical repair. CONCLUSION: Contemporary survival rates of patients with TA following operation in the center has been gradually improved over time. Most of the operative mortality occurs in the early postoperative period. Compared to patients with TA who had palliative treatment, operative survivors have a better functional status even though they carry a risk for re-intervention.

Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand.

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We therefore conducted a retrospective study to assess survival rates and mortality risks of 90 children with PA/VSD at Siriraj Hospital, Thailand during 2005-2016. Patients with single ventricle were excluded. Survival and mortality risks were analyzed at the end of 2018. The median age of diagnosis was 0.5 (0-13.8) years. The patients' PAs were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD with confluent PAs and MAPCAs (n = 21), 3) PA/VSD with non-confluent PAs and MAPCAs (n = 12), and 4) PA/VSD with small native PAs and MAPCAs (n = 17). Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. Significant mortality risks were the presence of associated anomalies and non-confluent PAs.

Association of Echocardiographic Parameters of Right Ventricular Remodeling and Myocardial Performance With Modified Task Force Criteria in Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy.

BACKGROUND: The usefulness of echocardiographic indices, including those already used by modified Task Force Criteria (mTFC), and others such as strain imaging, to identify arrhythmogenic right ventricular cardiomyopathy (ARVC) in adolescence is not well established. METHODS: Echocardiograms from 120 adolescents investigated for ARVC (13±4 years) were retrospectively analyzed. According to the mTFC, patients were classified into definite (n=38), borderline (n=39), or possible (n=43) ARVC. Results were compared with 35 healthy controls. mTFC echocardiographic parameters were analyzed, as well as comprehensive right ventricular (RV) and left ventricular assessment of function including parameters not included in mTFC such as pulsed-wave tissue Doppler and RV 2-dimensional speckle strain. RESULTS: mTFC parameters indexed for body surface area were significantly more abnormal in patients with possible, borderline, or definite ARVC compared with controls for parasternal long-axis view of the RV outflow tract. RV end-diastolic diameters were significantly larger in patients versus controls, a difference that increased with likelihood of ARVC. Left ventricular ejection fraction, tricuspid annular peak systolic excursion, and systolic and diastolic pulsed-wave tissue Doppler imaging indices were similar to controls for all groups. Average and segmental RV peak longitudinal systolic strain was significantly lower in patients with definite ARVC (-21±4%) and disease subgroups versus controls (-25±3%). Multivariable risk analysis showed that reduced RV strain was significantly associated with ARVC diagnosis and its likelihood (multivariable odds ratio [95% CI]=1.23 [1.1-1.37]; P<0.001) as was increased end-diastolic diameter at the apical third of the RV (multivariable odds ratio [95% CI]=1.51 [1.33-1.72]; P<0.001). CONCLUSIONS: mTFC echocardiographic criteria are significantly different between patients and controls and between the different diagnostic groups. However, in our cohort, current echocardiographic mTFC are not met by the majority of adolescent ARVC patients, particularly when indexed to body surface area. Measurement of RV apical dimensions and strain may increase the diagnostic yield of echocardiography for ARVC.

Contemporary outcomes and mortality risks of Ebstein anomaly: A single-center experience in Thailand.

BACKGROUND: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival. OBJECTIVES: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era. METHODS: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardiographic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks. RESULTS: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow-up [median time of 5.2 years (3 days-23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day-64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA surgery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predictors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z-score >3.80, TV displacement >19.5 mm/m2 , presence of severe tricuspid regurgitation, and absence of forward flow across the pulmonic valve at the initial diagnosis. CONCLUSION: Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population.

Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts.

OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis. RESULTS: A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2. CONCLUSION: Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.

Left Ventricular Function in Children and Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy.

The aim of this study was to determine if left ventricular (LV) contractility is reduced in children with arrhythmogenic right ventricular cardiomyopathy (ARVC). For this retrospective study, children and adolescents undergoing a workup for ARVC were characterized according to the revised Task Force Criteria (rTFC). LV strain, rotation, and torsion were measured by feature-tracking cardiovascular magnetic resonance imaging (CMR). Of 142 pediatric patients, 41% had no, 23% possible, 20% borderline, and 16% definite ARVC. LV ejection fraction (EF) did not differ between rTFC categories. Patients in higher rTFC categories had lower right ventricular (RV) EF z-scores (Z-), higher Z-RV end-diastolic volumes (EDVs) and larger Z-LVEDVs (p <0.001, p = 0.002 and 0.013, respectively). LV global circumferential strain was lower in higher rTFC categories (p = 0.018). Z-LVEDV correlated with Z-RVEDV, and Z-LVEF correlated with Z-RVEF (r = 0.69 and r = 0.55, both p <0.001). Z-LVEF and Z-RVEF correlated with LV global circumferential strain (r = 0.48 and r = 0.46, both p <0.001). Forty-eight patients (34%) underwent follow-up CMR investigations after a mean of 3.2 ± 1.9 (0.4 to 8.4) years. A decrease of Z-LVEF over time correlated with that of Z-RVEF (r = 0.35), and Z-LVEDV increase correlated with Z-RVEDV increase (r = 0.57). In conclusion, LV myocardial dysfunction is present in young patients with suspected ARVC. Progressive LV dysfunction assessed by conventional CMR and feature-tracking and enlargement over time parallel adverse remodeling of the RV.

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot.

BACKGROUND: Unfavorable left ventricular (LV) remodelling may be associated with adverse outcomes after Tetralogy of Fallot (TOF) repair. We sought to assess T1 cardiovascular magnetic resonance (CMR) markers of diffuse LV myocardial fibrosis in children after TOF repair, and associated factors. METHODS: In this prospective, cross-sectional study, native (=non-contrast) T1 times and extracellular volume fraction (ECV) were quantified in the LV myocardium using CMR. Results were related to ventricular volumes and function, degree of pulmonary regurgitation, as well as surgical characteristics, and exercise capacity. RESULTS: There was no difference in native T1 times or ECV between 31 TOF patients (age at CMR 13.9 ± 2.4 years, 19 male) and 15 controls (age at CMR 13.4 ± 2.6 years, 7 male). Female TOF patients had higher ECVs than males (25.2 ± 2.9 % versus 22.7 ± 3.3 %, p < 0.05). In the patient group, higher native T1 and ECV correlated with higher Z-Scores of right and left ventricular end-diastolic volumes, but not with reduced left and right ventricular ejection fraction or higher pulmonary regurgitation fraction. Longer cardiopulmonary bypass and aortic cross clamp times at surgery correlated with increased native T1 times and ECVs (r = 0.48, p < 0.05 and r = 0.65, p < 0.01, respectively). Maximum workload (percent of predicted for normal) correlated inversely with ECV (r = -0.62, p < 0.05). Higher native T1 times correlated with worse LV longitudinal (r = 0.50, p < 0.05) and mid short axis circumferential strain (r = 0.38, p < 0.05). CONCLUSIONS: As compared to controls, TOF patients did not express higher markers of diffuse fibrosis. Longer cardiopulmonary bypass and aortic cross clamp times at surgery as well as biventricular enlargement and reduced exercise tolerance are associated with markers of diffuse myocardial fibrosis after TOF repair. Female patients have higher markers of diffuse myocardial fibrosis than males.

Fetal long QT syndrome manifested as atrioventricular block and ventricular tachycardia: a case report and a review of the literature.

Fetal onset of congenital long QT syndrome (LQTS) is a rare manifestation, and prenatal diagnosis is difficult. This report describes a boy who presented with both atrioventricular (AV) block and ventricular tachycardia during the antenatal period. The early postnatal electrocardiogram showed prolongation of the QT interval and AV block, subsequently leading to a polymorphic ventricular tachycardia torsade de pointes. This unique feature of congenital LQTS has a poor outcome, but the boy was successfully treated with beta-blockers and implantation of an automated cardioverter-defibrillator. The intrauterine manifestation of fetal AV block and ventricular tachycardia should raise a high suspicion of congenital LQTS, and the strong association with a malignant clinical course should warrant special evaluation. The literature on the prenatal diagnosis, fetal therapy, and neonatal outcome of this condition also are reviewed.

Transcatheter closure of atrial septal defects in children, middle-aged adults, and older adults: failure rates, early complications; and balloon sizing effects.

Objectives. To compare the failure ratio and inhospital complications across three age groups of patients and to investigate the effects of balloon sizing on the success and the device diameter. Methods. This retrospective review was of 665 patients who had been listed for transcatheter-based closure of ASD between 1999 and 2010. The patients were divided into three age groups: children (<18 years; n = 183), adults (18-50 years; n = 337), and older adults (>50 years; n = 145). Procedural outcomes and early complications were reviewed. Use of balloon sizing was explored for its benefits. Results. Overall, failure of closure was 6.6% (n = 44). Use of balloon sizing tended to lead to a smaller device/defect ratio that was comparable to procedures without balloon sizing, though it did not predict the success rate (OR 1.4, 95% CI 0.7-2.3). Seven patients reported device embolization (1%). No mortalities were noted. In-hospital complications were 3.4%, with common complications, being vascular complications (1.4%) and cardiac arrhythmia (1.1%). No differences in failure rate or events were found among the three groups. Conclusion. Transcather closure of ASD is feasible and safe, regardless of the patient's age. A low rate of early complications was noted. Balloon sizing does not aggravate an oversizing of the device, but does not predict success.

Postoperative fever and major infections after pediatric cardiac surgery.

OBJECTIVE: 1) to identify the current status of major infections and other etiologies of postoperative fever from pediatric cardiac surgery 2) to determine the risk factors of major infections. MATERIAL AND METHOD: Databases of pediatric cardiac surgery patients in 2005 were retrospectively reviewed. The main outcomes of interest were postoperative fever and its etiologies. Potential predictors were analyzed by comparing patients who developed or did not have infections. RESULTS: Two hundred thirty patients, 43% (n = 99) developed postoperative fever. Major infections occurred in 13.5% (n = 31), and postpericardiotomy syndrome (PPS) was seen in 8.7% (n = 20) of the patients. The infection rate was 16.9/100 procedures, including pneumonia (29 episodes) and bloodstream infection (6 episodes). Risk factors were infancy, prolonged ventilator support > 2 days, hospital length of stay (LOS) > 14 days, intensive care unit (ICU) LOS > 3 days, re-open procedure, and extubation failure rate. Conversely, cyanosis and high complexity operations were not associated. Positive erythrocyte sedimentation rate was related to infections or to PPS (the area under the ROC = 0.72). CONCLUSION: Following pediatric cardiac surgery major infections are still problematic. The risks increase with infancy, prolonged ventilator support, prolonged hospital and ICU LOS, re-open procedure, and extubation failure.

Beneficial effects of transcatheter closure of atrial septal defects not only in young adults.

OBJECTIVES: To compare cardiac events and remodeling effects after transcatheter closure of atrial septal defects (ASD) in pediatric, adult, and older adult patients. METHODS: A retrospective review was conducted of 353 patients who underwent transcatheter ASD closure between February 1999 and December 2007 at Siriraj Hospital. The patients were divided into 3 groups according to age: children (<18 years; n = 99); adults (18-50 years; n = 169); and older adults (>50 years; n = 85). Cardiac events at 1 year, and changes in left and right ventricular dimensions between preprocedure and 6 months and 1 year postprocedure were compared between groups. RESULTS: Of the 353 patients, the average size of ASD was 22.1 ± 6.6 mm. Device: ASD diameter was 1.25 ± 0.28 mm. At 1 year postprocedure, the prevalence of chest discomfort and atrial fibrillation (AF) was higher in older adult patients, compared to the other age groups. Device embolization, cardiac erosion, pericardial effusion, syncope, migraine, thrombus formation, and residual shunt did not differ between groups. Within the first 6 months, the right ventricular (RV) dimension tended to dramatically decrease, while the left ventricular (LV) dimension increased in all age groups. These changes leveled off in children and in older adults, but in the adult group (18-50 years), RV shrinkage and LV expansion continued for 1 year. A low rate of early and late complications was noted. CONCLUSION: Transcather closure of ASD can cause cardiac remodeling, regardless of the patient's age at the time of the procedure. For older adult patients, the long-term risk of AF continuation and chest discomfort is likely.

The results of transcatheter closure of VSD using Amplatzer® device and Nit Occlud® Lê coil.

OBJECTIVE: We retrospectively reviewed the result of patients who underwent transcatheter closure of ventricular septal defect (VSD) using Amplatzer® Perimembranous or Amplatzer® muscular VSD device (the Amplatzer® group) and Nit Occlud® Lê VSD Coil (the Pfm group). BACKGROUND: Perimembranous type (PmVSD) and doubly committed subarterial (DCSA) VSD were the major isolated congenital heart defects in Thai children. Transcatheter device closure technique for both types of VSD has emerged as an alternative treatment to surgery. METHODS: Retrospectively, data was reviewed between 2003 and 2009. RESULTS: 116 patients were enrolled. Device or coil was successfully implanted in 94%. Complete closure at 24 hr is slightly higher in the Amplatzer® group. The average size of VSD in the Amplatzer® group was larger than the Pfm group (P = 0.001). The Pfm coil was primarily deployed in DCSA VSD when compared with the Amplatzer® group (P < 0.01). At 6 months follow-up the residual shunt was comparable (P = 0.054). There was only one transient AV block (AVB) in the Pfm group and 5 AVB in the Amplatzer® group. Four pacemakers were placed in the Amplatzer® group. CONCLUSIONS: Transcatheter closure of VSD in both Pm VSD and DCSA can be achieved by using either of the device. The Amplatzer® VSD device had the advantage of closure of larger defects with immediate less residual shunt but appeared to have a significant number of 3° AVB, which required pacemaker implantation. The Nit Occlud® Lê VSD Coil had the advantage of closure of both types of defects, in particular DCSA VSD with only small residual shunt.