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.

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.

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.

Arrhythmia in early post cardiac surgery in pediatrics: Siriraj experience.

OBJECTIVES: To determine the incidence, risk factors and outcome of early postoperative arrhythmias in pediatric patients with congenital heart disease. MATERIAL AND METHOD: A prospective study was conducted in every pediatric patient who consecutively underwent open-heart surgery at Siriraj Hospital from January 1st to December 31st, 2006. The collected data were demographic data, diagnosis, pre-operative arrhythmia, cardiac surgical data and continuous electrocardiographic monitoring data throughout the post operative intensive care period. RESULTS: A total of 191 pediatric patients underwent cardiac surgery. Forty-five cases (23.5%) developed early post operative cardiac arrhythmias i.e. junctional ectopic tachycardia 18 cases (40%), heart block 7 cases (15.6%), supraventricular tachycardia 2 cases (4.4%). Cardiac arrhythmia occurred mostly within 24 hours after the operation. Patients with single ventricle physiology repaired developed the highest incidence of acute post operative arrhythmia (36.4%). Longer, cardiopulmonary bypass time- and redo-operation were the risk factors. Thirty-nine cases were treated with medications, 7 cases with temporary pacing, and 1 case with electrical cardioversion. Four patients needed long-term anti-arrhythmic agents. Cardiac arrhythmia played role in the causes of death in 2 cases (1.1% of total cases). CONCLUSIONS: Post operative arrhythmias remained common and important complications of pediatric open-heart surgery. Long cardiopulmonary bypass time and redo-operation were risk factors for early post operative arrhythmia.

Permanent cardiac pacing in pediatrics: experience in Thailand.

UNLABELLED: Permanent cardiac pacing in pediatrics is uncommon. There has been limited data in Thailand. A retrospective study of cardiac data and pacing parameters during follow-up periods in patients who underwent permanent pacemaker implantation at the Department of Pediatrics, Siriraj Hospital, from January 1997 to December 2004 was conducted. There were 31 patients in total who have been followed-up for the median period of 34.4 (1.07-91.13) months. All patients had atrio-ventricular block prior to implantation. The etiology were; post cardiac surgery 38.7%, maternal autoimmune diseases 19.4%, post radiofrequency ablation 3.2%, and unknown 38.7%. Twenty three cases (74.2%) were implanted by epicardial approach, and 18 (25.8% were implanted by endocardial approach. Modes of permanent pacemaker were WIR 45.2%, VVI 35.5%, and DDD 19.4%. Age and body sized of the patients using epicardial approach were significantly lower than endocardial approach. Minor complications occurred in 3 cases (9.6%) i.e. 2 with surgical wound infection, 1 with post pericardiotomy syndrome. Minimum energy threshold, sensitivity, and impedance at implantation and during follow up periods were not different statistically. There was significantly less in minimum energy threshold of endocardial lead than epicardial lead. Epicardial lead failure was found in 3 cases (11.5%) at the median time of 8.9 (7.9-62) months post implantation, but was not significant different from endocardial leads. Survival of epicardial leads were 82% at 8 years. CONCLUSION: Permanent pacemaker implantation in pediatrics was rare (4.4 cases/year). It was feasible in almost all body size and a rather safe procedure. There was no significant change in pacing parameters at the medium-term follow-up period for both epicardial and endocardial leads. Minimum energy threshold of epicardial lead was significantly higher than endocardial lead.

Heart murmur in the first week of life: Siriraj Hospital.

Congenital heart disease (CHD) is the most common form of cardiovascular diseases in children. In Thailand, there has not been available information about congenital heart disease in neonates. Between January 1st and December 31st, 2000, all full-term babies born at Siriraj Hospital with detected heart murmur within the first week of life were consulted to pediatric cardiologists. Echocardiography was performed for diagnosis in every baby. Total livebirths during that period were 11,245 cases. Heart murmurs were detected in 83 cases. The incidence of heart murmur within the first week of life was 7.38:1,000 livebirths. Innocent murmurs were found in 34 cases and echocardiogram revealed no detectable cardiac anomalies (2 cases), mild tricuspid regurgitation (2 cases), physiologic branch pulmonary stenosis (4 cases), and small size PDA (< 2 mm., 26 cases). Forty-nine cases had CHDs. The incidence of CHD was 4.36:1,000 livebirths. At the time of initial diagnosis, 22 cases (44.8%) were asymptomatic. Among these patients, 1 case had serious cardiac anomaly, i.e., tetralogy of Fallot. There were 27 cases with symptoms, including 15 cases (30.6%) with tachypnea, 8 cases (16.4%) with cyanosis and 4 cases (8.2%) with congestive heart failure. The 3 most common cardiac diseases were ventricular septal defect (9 cases, 18.4%), patent ductus arteriosus greater than 2 mm. (8 cases, 16.3%), and atrial septal defect (8 cases, 16.3%). Those with CHDs were treated with anticongestive medications (22 cases, 44.8%), prostaglandin E1 (5 cases, 10.2%), laser pulmonary vulvulotomy (1 case, 2%), palliative surgery within the first week of life (4 cases, 8.2%) and corrective surgery (4 cases, 8.2%). During follow-up for the period of 1 year, 2 cases died from sepsis. Early diagnosis and proper management are important to reduce morbidity and mortality in the newborn with CHD.

Coronary artery bypass graft in Kawasaki disease patients: Siriraj experience.

Kawasaki disease (KD) is an acute systemic vasculitis. It is one of the most common acquired heart diseases in children. Its important acute complication is coronary artery aneurysm. Although most of these aneurysms resolved overtime but some never did. Coronary artery stenosis and myocardial insufficiency or infarction are late complications. Coronary artery bypass graft (CABG) and catheter intervention are the treatment for these patients. We report our first five cases of Kawasaki patients with myocardial insufficiency who underwent CABG at Siriraj Hospital. Patients' ages ranged from 3.3 to 14.4 years old. Only two patients (40%) had angina. Internal thoracic artery was used as a bypass graft in most patients and postoperative course was uneventful. Coronary bypass operation is a safe and reliable surgical modality for coronary artery sequelae in children with Kawasaki disease. A long-term follow-up is necessary to study the long-term outcome of bypass.