The Clinical Outcomes in Patients With Atrial Isomerism Undergoing Single Ventricular Palliation: Insights From A Single-Center Study in Thailand.

Background: The management of atrial isomerism across various countries may impact survival outcomes. Methods: This retrospective study involved patients diagnosed with atrial isomerism undergoing single ventricular palliation between 2000 and 2021. The objective was to evaluate survival outcomes within the right atrial isomerism (RAI) and left atrial isomerism (LAI) groups, as well as overall survival. Results: Of the 125 patients diagnosed with atrial isomerism, 105 (84%) had RAI, and 20 (16%) had LAI. The median age at presentation was 3 days (range: birth to 7.1 years), with median follow-up of 6.6 years (range: 0.59 months to 30.8 years). In the overall cohort, survival rates at one, five, and ten years were 85.6%, 72.3%, and 66.8%, respectively, with no statistically significant difference between RAI and LAI groups (log rank P value = .293). Specifically, survival rates in the RAI group at one, five, and ten years were 83.8%, 69.0%, and 62.6%, respectively, while in the LAI group, there were 95.0%, 89.7%, and 81.6%, respectively. Following the third-stage operation, overall survival rates at one and five years were 76.4% and 76.4%, respectively. Among patients in the RAI group, survival rates at one and five years post-third stage operation were 80.2% and 80.2%, respectively. Conclusions: Despite resource limitations, the survival outcomes of patients with atrial isomerism were found to be similar with those observed in high-income countries. Although an increased mortality rate was noted within the first year of life, focusing efforts on neonatal and infant care holds potential for improving overall outcomes.

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.

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.

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.

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.

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.

Medium and long-term outcomes of Fontan operation.

BACKGROUND: The Fontan operation had been proposed as the final palliative surgery in the patients with single ventricle physiology. Even though modifications of the operation were developed to improve outcomes, long-term complications remain significant with time. The present study reviewed long-term survival rate, morbidities associated with time, and risk factors during the follow-up period after Fontan operation. MATERIAL AND METHOD: A retrospective study was conducted. Every patient who underwent the Fontan operation at Siriraj Hospital between January 1987 and December 2007 and had available data was included in the present study. The data was collected until the most recent follow-up in December 2008. Demographic data, diagnosis, echocardiographic data, cardiac catheterization data, surgical data, type of modified Fontan procedure, and perioperative data were collected. The follow-up clinical data, cardiac investigation data, complications, and management were also collected and analyzed. RESULTS: Survival rates were 88.7%, 85.3%, and 83.8% at 1 year, 5 years, and 10 years, respectively. The median follow-up time was 4.75 years (0-17.45). The 10-years survival rate of tricuspid atresia, single ventricle and the heterotaxy syndrome were 94.5%, 79%, and 83.3%, respectively, which were not significantly different (p = 0.09). The 10-years survival rates of the patients that underwent lateral tunnel, extracardiac conduit and atriopulmonary connection were 80.7%, 88% and 84.3%, respectively. A mean pulmonary artery pressure of more than 18 mmHg was the only factor that affected the survival rate after Fontan surgery (p = 0.008). The incidence of postoperative arrhythmia was 7.9%. Age at operation, diagnosis, type of operation, fenestration, systemic EDP, or PVR before operation did not significantly affect the survival rate. Diagnosis and type of surgery did not affect long-term outcome regarding arrhythmia, re-intervention, systemic atrioventricular valve regurgitation, and systemic ventricular dysfunction. Patients post Fontan operation had good survival rate. CONCLUSION: Cardiac diagnoses were not significantly different in the medium and long-term survival rate of post Fontan patients, freedom from arrhythmia, re-intervention and systemic atrioventricular regurgitation. Types of Fontan operation did not affect long-term survival rate or long-term complications. Mean pulmonary artery pressure of more than 18 mmHg was the only risk factor to the survival rate.