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.

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.

Early Outcome of Simplified Standardized Trileaflet Polytetrafluoroethylene Valved Conduit Placement.

BACKGROUND: Use of the expanded polytetrafluoroethylene (ePTFE) valved conduit in the pulmonary position during the correction of congenital heart defects has significantly increased in popularity over the last decade due to its promising conduit longevity. We describe the standardized process to create and implant a trileaflet ePTFE pulmonary conduit along with the early outcomes of such procedures at our institute. METHODS: Records of 100 consecutive patients who underwent ePTFE valved conduit placement using our technique from April 2018 through February 2022 were retrospectively analyzed. The function of the conduit was evaluated by transthoracic echocardiography. RESULTS: The mean age of the patients was 28 ± 13.2 years old at the time of the operation. The conduit diameters ranged from 16 to 24 mm (mean 23.0 ± 1.9 mm). Conduit placement was utilized for pulmonary valve replacement in 68 patients, conduit change in 25 patients, and as a part of total repair in 7 patients. There were 2 in-hospital conduit-unrelated deaths from multi-organ dysfunction and pulmonary hypertensive crisis. From the postoperative echocardiography, the average peak pressure gradient across the conduit was 18.6 ± 9.0 mm Hg, and the conduit regurgitation was graded as none or trace in 81 patients, mild in 17 patients, and moderate in 2 patients. At 589 days of median follow-up, there was no conduit reoperation. Follow-up imaging of 60 available patients at a median time of 511 days did not show a significant change in conduit function. CONCLUSIONS: Our standardized ePTFE valved conduit creation and placement demonstrated satisfactory clinical and echocardiographic outcomes.

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.

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.

Using bilateral radial arteries in coronary artery bypass grafting is safe.

BACKGROUND: Recent studies have revealed that radial artery grafts have excellent patency rates. However, harvesting of the radial artery is generally limited to the non-dominant forearm. We would like to demonstrate the effectiveness and safety of bilateral radial artery harvesting. METHODS: We enrolled 173 patients undergoing coronary artery bypass. Bilateral RA were used in 64 cases and unilateral in 109. The primary endpoint was post-harvest forearm and hand complications. RESULTS: Forearm and hand complications occurred immediately postoperatively in 28.1% of the bilateral radial artery group, significantly more than in the unilateral radial artery group (8.3%). During follow-up, no overall difference was found in post-harvest forearm and hand complications. However, the forearm and hand perception score in the bilateral radial artery group was higher: 8.78 ± 1.45 vs. 8.35 ± 0.84 in the unilateral radial artery group. Subgroup analysis in the bilateral radial artery group revealed no significant difference in forearm and hand perception score between the dominant and non-dominant donor forearms (8.78 ± 1.45 in non-dominant and 8.66 ± 1.00 in dominant forearms). The bilateral radial artery group had more arterial coronary anastomoses, longer operative times, and longer cardiopulmonary bypass times. However, a backward multiple linear regression model revealed that only two factors related to operative time, these were the number of arterial distal coronary anastomosis and cardiopulmonary bypass time. CONCLUSIONS: This study demonstrated that bilateral radial artery conduits can be used effectively and safely with no difference in persistent complications related to the hands and forearms.

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.

Midterm results of anatomic repair in a subgroup of corrected transposition.

BACKGROUND: Anatomic repair has become the preferred option in the subgroup of patients with congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary obstruction. We report our 14-year experience with this approach. METHODS: From April 2001 to February 2014, 22 patients with congenitally corrected transposition with ventricular septal defect and pulmonary obstruction underwent anatomic repair. Nineteen patients had a modified Senning-Rastelli procedure, 2 had a Mustard-Rastelli procedure, and one had a hemi-Mustard-Glenn-Rastelli procedure. The mean age was 10.9 years, and 8 (36.4%) patients were male. RESULTS: There were 2 early deaths from sepsis and ventricular failure at 18 and 81 days postoperatively, and 3 late deaths from ventricular failure at 4, 33, and 113 months postoperatively. Left ventricular failure with mitral valve regurgitation was present in 3 of the 5 patients who died. Among the survivors, 3 underwent 4 transcatheter interventions for right ventricular outflow tract obstruction and 3 underwent 4 reoperations for atrial pathway obstruction, left and right ventricular outflow tract obstruction, or residual shunt. At a median follow-up of 64 months (range 14-167 months), 15 of 17 survivors were in functional class I. One patient had severe mitral valve regurgitation and was awaiting valve replacement. Another patient had right ventricular outflow conduit obstruction and was scheduled for reoperation. CONCLUSIONS: Results of atrial switch-Rastelli procedures in this subgroup of patients with corrected transposition are satisfactory but still imperfect. Mitral regurgitation might predict a poor outcome. Long-term follow-up is necessary.

Surgical Outcomes of Adult Congenital Heart Disease with Left to Right Shunt at Siriraj Hospital.

Background: Outcomes of surgical treatment of adult congenital heart disease (ACHD) in developing country are scant but are of critical importance for caring of these patients. Objective: We studied characteristic and surgical repair outcomes of simple ACHD at Siriraj Hospital. Material and Method: We studied 297 adults with atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA) whom underwent surgical repair between January 2006 and September 2014. There were 211 ASD, 80 VSD, and 6 PDA. We focused on perioperative data, follow-up data, and factor(s) associated with adverse events. Results: Of 297 patients, 37.4% were males; mean age was 41.8 years (range, 20-78 years). Pulmonary hypertension and valvular regurgitation (mitral, tricuspid, or aortic) were presented in 74.7% and 35.0% of the patients, respectively. Majority of defects were closed with patching (85.4% for ASD and 82.7% for VSD), while the rest were closed directly. Of the 190 concomitant procedures, most of them were tricuspid repair (28.9%), and mitral repair (17.4%). There were eight hospital deaths (2.7%). At median follow-up of 25 months (range, 1-102 months), the mean functional classification was reduced from 1.9 to 1.1 (p<0.01). There were five re-interventions and four late deaths. Overall survival was 96.1% at five years and 76.1% at eight years. Concomitant procedure(s), tricuspid, and mitral regurgitation were associated with worse survival. Concomitant procedure(s), mitral, and aortic regurgitation were associated with re-intervention. Conclusion: Outcomes of surgical repair of simple ACHD were good. Valvular regurgitation and concomitant procedure(s) adversely affected the outcomes.

Validation of EuroSCORE for coronary artery bypass grafting at Siriraj Hospital.

OBJECTIVE: To access the performance of the EuroSCORE when applied to CABG patients at Siriraj hospital. MATERIAL AND METHOD: One thousand five hundred forty nine patients diagnosed with coronary artery disease (CAD) who underwent isolated CABG between January 2007 and December 2009 was prospectively studied. RESULTS: The patients included 1,102 men and 447 women and had a mean age of 67 years old. The mean additive score in expired and survived groups were 9.65 +/- 5.14 and 3.87 +/- 3.06. In logistic, score were 25.43 +/- 26.31 and 4.88 +/- 7.88 respectively (p < 0.001). The best cut-off value of EuroSCORE for prediction of a death rate was 6 for additive score and 10 for logistic score. Area under the curve was 0.831 for the additive score and 0.823 for the logistic score. The observed overall mortality rate was 2.0% while the predict mortality was 5.27%. The difference between observed and predicted deaths was significant with additive score and logistic score (p < 0.001). CONCLUSION: Our results suggest that EuroSCORE is not valid for CABG in Thai patient due to over prediction.

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.

Early results of anatomic repair in a subgroup of corrected transposition.

The atrial switch operation with the Rastelli procedure is becoming popular for treatment of the subgroup of corrected transposition of the great arteries with ventricular septal defect and pulmonary obstruction. This technique eliminates the problem of short- and long-term right ventricular failure, and decreases the incidence of iatrogenic atrioventricular heart block. Between April 2001 and November 2002, this technique was used in 3 patients aged 5 to 7 years. Two had a Senning operation and one had a Mustard operation. There was no operative death. The first patient needed re-operation to close the sternum. The last patient was re-explored for bleeding. All patients were in New York Heart Association functional class I at their last follow-up. The atrial switch plus Rastelli procedure is feasible in this subgroup of corrected transposition, but longer follow-up is necessary to determine whether this approach is indeed warranted.

Spontaneous rupture of the left subclavian artery in neurofibromatosis.

Two patients with neurofibromatosis presented with expanding masses at the left supraclavicular region. Computed tomography (CT) scans revealed vascular masses. The patients underwent surgery and ruptures of the left subclavian artery were found. Both patients were treated by ligation of subclavian artery.