The Role of Collaboration in Prenatal Congenital Heart Disease Diagnosis: A Comparison of Maternal-Fetal Medicine Specialist and Pediatric Cardiologist Performance.

OBJECTIVE: This study compared the accuracy of prenatal congenital heart disease (CHD) diagnosed by maternal-fetal medicine specialists (MFMs) and pediatric cardiologists (PCs), using postnatal cardiac findings as the reference standard. METHODS: This retrospective analysis at Siriraj Hospital, Bangkok, Thailand, involved 125 pregnancies with fetal CHD diagnosed by MFMs and evaluated by PCs later. Prenatal CHD diagnoses by either MFM or PC were compared with postnatal diagnoses obtained through echocardiography, cardiac surgery/catheterization, or autopsy. Diagnostic accuracy was classified as (A) correct diagnosis, (B) minor differences not impacting clinical management or outcomes, or (C) major differences affecting prognosis or treatment. RESULTS: Cardiac sonography by MFM achieved diagnostic accuracies of 73.6% (A), 16% (B), and 10.4% (C), while fetal echocardiography by PC resulted in accuracies of 72% (A), 20% (B), and 8% (C). No statistically significant differences were found between MFM and PC in each category (P = .375-.832). The MFMs' accuracy was highest for tetralogy of Fallot (94.4%; 95% CI, 72.7-99.9%) and lowest for right atrial isomerism (71.4%; 95% CI, 29-96.3%) and pulmonary atresia with ventricular septal defect (57.1%; 95% CI, 18.4-90.1%). CONCLUSIONS: MFMs and PCs demonstrated high and comparable accuracy in prenatal CHD diagnosis. Although PCs tended to outperform MFMs in cases where misdiagnosis could significantly impact neonatal care and outcomes, MFMs can effectively perform primary screening for fetal CHD in all pregnancies. Collaboration with PCs remains essential when fetal CHD is suspected, particularly in complex cases.

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.

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.

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.

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.

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.

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.

An interarterial course of anomalous coronary artery and an alternative surgical option.

An interarterial course of anomalous left main coronary artery originating from the right aortic coronary sinus of valsalva is a rare condition among anomalous aortic origin of the coronary artery. Various surgical options are available. We performed an alternative procedure, that is, mobilisation of the pulmonic root, pulmonary bifurcation, and plication of the pulmonic root adjacent to the left main coronary artery, in a 12-year-old boy. Favourable results were achieved after 43 months of follow-up.

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.

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.

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.

Outcomes of Transcatheter Pulmonary Valve Replacement and Surgical Pulmonary Valve Replacement: A Cohort Analysis.

Background: Transcatheter pulmonary valve replacement (TPVR) has become an alternative to surgical pulmonary valve placement (SPVR) for patients after tetralogy of Fallot repair. This study compared the outcomes of TPVR with those of SPVR. Methods: We reviewed data from patients who underwent pulmonary valve replacement with a median of 2 years of follow-up. Results: Between 2010 and 2021, 215 patients underwent pulmonary valve replacement (72 TPVR and 143 SPVR). The median size of the right ventricular end-diastolic volume index in the TPVR group was 165 mL/m2 (IQR, 136-190) and 184 mL/m2 (IQR, 163-230) in the SPVR group (P = .001). The median value of the maximum landing zone at the right ventricular outflow tract (RVOT) in patients with native RVOT was 26 mm (IQR, 24-28) in the 43 patients in the TPVR group and 31 mm (IQR, 28-34) in the 101 patients in the SPVR group (P < .001). The median size of the pulmonary valve implant for the native RVOT in the TPVR group was 29.0 mm (IQR, 26.0-29.0) and 24.0 mm (IQR, 24.0-24.0) in the SPVR group (P < .001). There were no deaths in the TPVR group and 8 deaths in the SPVR group (P = .041). Major complications and the length of hospitalization were lower in the TPVR group (P = .001). After 2 years, the mean decrease in QRS duration was 5 milliseconds (IQR, 1-14) in the TPVR group and 1 millisecond (IQR, -4 to 10) in the SPVR group (P = .006). Conclusions: TPVR allows for larger implants, resulting in lower mortality, shorter hospital stays, and fewer major cardiac events. SPVR may be preferable in patients with larger (>30 mm) native RVOT and in those who require concomitant surgical procedures.

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.

Neonatal lupus erythematosus: clinical character, investigation, and outcome.

Neonatal lupus erythematosus is an uncommon maternal auto-antibody-associated disease characterized by cutaneous, cardiac, hepatic, hematological, neurological, and pulmonary involvement. A retrospective study was performed to review clinical manifestations, investigation results, outcomes of neonatal lupus erythematosus patients and their mothers at the Department of Pediatrics, Siriraj Hospital during 1993 to 2008. Seventeen neonatal lupus erythematosus patients (10 girls and seven boys) were identified. Cutaneous, cardiac, hepatobiliary, and hematological involvement was found in 70.6%, 64.7%, 52.9%, and 35.3% of infants, respectively. Skin lesions were erythematous patches (91.7%), subacute cutaneous lupus erythematosus (50%), petechiae (41.7%), persistent cutis marmorata (16.7%), and discoid lesions (8.3%). Congenital heart block was found in nine cases, and structural abnormalities were found in nine cases. All sera of patients were positive for antinuclear antibodies. Patients (87.5%) showed positive antiRo/SSA, and 50% had positive antiLa/SSB antibodies. Most neonatal lupus erythematosus mothers (64.7%) were asymptomatic. Five mothers were diagnosed with systemic lupus erythematosus, and one mother was diagnosed with mixed connective tissue disease. All maternal sera was positive for antinuclear antibodies and antiRo/SSA antibody. Seven patients required pacemaker implantation. The mortality rate was 11.8%, caused by congestive heart failure and pneumonia. Antinuclear antibody tests should be used as one of the screening tests in mothers or patients suspected of having neonatal lupus erythematosus.

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.

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.

Clinical manifestations of Kawasaki disease: what are the significant parameters?

The initial differential diagnosis of Kawasaki disease (KD) from other acute febrile illnesses infants and children is particularly difficult in patients who exhibit incomplete criteria. The objective of this study was to determine the differences in the clinical and laboratory findings between KD patients and those who were initially sus- , pected of having KD but eventually had other diagnoses. One hundred and fourteen pediatric patients who were initially diagnosed with suspected KD were included. Eighteen cases were finally diagnosed with another disease. The only demographic data that were significantly different between the groups were body height and the duration of fever. The KD group exhibited more classical clinical criteria than those who were finally diagnosed with another disease. The erythrocyte sedimentation rate (ESR) and platelet count were significantly higher in the KD group than in the non-KD group. An ESR > or = 40 mm/hour had a diagnostic sensitivity of 90.5%, a specificity of 66.6%, a positive predictive value of 93.4%, and a negative predictive value of 57.1%. The incidence of coronary aneurysm in this study was 6.2%. There was no correlation between ESR and coronary aneurysm. We conclude that the clinical criteria are the basis for the diagnosis of Kawasaki disease but the ESR can be helpful in pediatric patients with acute febrile illness who do not exhibit all clinical criteria.

N-terminal pro brain natriuretic peptide and cardiac function in doxorubicin administered pediatric patients.

OBJECTIVE: Assess the use of N terminal pro brain natriuretic peptide (NT-pro BNP) to early diagnose ventricular dysfunction in doxorubicin-administered children. MATERIAL AND METHOD: Fifty-five cancer patients who received accumulative dose of doxorubicin <300 mg/m2 (group 1), 49 cases with accumulative dose > or = 300 mg/m2 (group 2) and 52 cases as a control group (group 3) were included in the study. Electrocardiogram, chest roentgenogram, echocardiogram, and serum NT-pro BNP were studied. RESULTS: At age 1-10 years, there were significantly higher NT-pro BNP in group 2 than group 1 (384 +/- 291 vs. 92.2 +/- 89 pg/ml; p = 0.001), and than group 3 (79 +/- 92 pg/ml; p = 0.001). Patients with NT-pro BNP level > 1 SD of the control group were more likely to have abnormal > or = 2 echocardiographic parameters of left ventricular diastolic dysfunction than patients with NT-pro BNP < or = 1 SD (OR = 3.8, 95% CI 1.18-12.5). Patients in group 2 were more likely to have abnormal > or = 2 parameters of left ventricular diastolic dysfunction than patients in group 1 (OR = 2.8, 95% CI 1.07-7.7) and more likely to have NT-pro BNP >1 SD than group 1 (OR = 8, 95% CI 1.96-38.4). There were association of NT-pro BNP > 1 SD, accumulative dose of doxorubicin > or = 300 mg/m2, and early left ventricular diastolic dysfunction by echocardiogram. CONCLUSION: Serum NT-pro BNP > 1 SD has a high probability to diagnose early doxorubicin-induced cardiomyopathy in patient 1-10 years old.