Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle.

BACKGROUND: To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV. METHODS: Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding. RESULTS: Among the 359 patients with an sRV (32.2 (IQR 26.4-38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0-9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV. CONCLUSIONS: Despite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.

Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis: Modified REV versus Rastelli.

The objective of this study was to evaluate and compare the results of the modified réparation à l'ètage ventriculaire (REV) and the Rastelli operation for the treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS). Records of 38 patients who underwent the modified REV (n = 16) or the Rastelli operation (n = 22) for the treatment of TGA, VSD, and PS between 2010 and 2019 were reviewed. The median age was 2.2 years (range 0.6-8.0 years) and the median weight was 11.3 kg (range 6.4-22.0 kg). No in-hospital death occurred and there were 4 early reoperations (two in each group). Overall survival at 10 years was 97.4% (100% in Modified REV group and 95.5% in Rastelli group, P = 0.39). Freedom from left ventricular outflow tract (LVOT) reoperation was 100% in both groups. Freedom from right ventricular outflow tract (RVOT) reoperation was 100% in Modified REV group and 75.4% in Rastelli group (P = 0.073). Event-free survival was 100% in Modified REV group and 72.0% in Rastelli group (P = 0.048). The most recent echocardiography showed that LVOT peak gradient was less than 10 mmHg in all patients. In Modified REV group, 30.8% of patients (4/13) had either RVOT obstruction (RVOT peak gradient more than 40 mmHg) or moderate or severe pulmonary insufficiency, while conduit stenosis (peak gradient more than 40 mmHg) was found in 25.0% of patients (3/12) in Rastelli group. The modified REV and the Rastelli operation provide satisfactory early results, as well as long-term survival and LVOT performance. However, the modified REV has better RVOT performance.

Value of preoperative echocardiography for the diagnosis of coronary artery patterns in neonates with transposition of the great arteries.

BACKGROUND: Abnormal coronary pattern may complicate coronary transfer during arterial switch operation. OBJECTIVE: To evaluate the accuracy of echocardiography in assessing the anatomy of coronary arteries in neonates with transposition of the great arteries, and determine impact on outcomes. METHODS: We conducted a retrospective analysis of data in neonates with transposition of the great arteries. Preoperative echocardiographic coronary artery pattern and surgical intraoperative reports were compared. Mismatch between transthoracic echocardiography and surgical intraoperative reports and the impact on perioperative outcome were assessed. Coronary patterns were classified into four groups: type 1 (normal); type 2 (risk of coronary with intramural course); type 3 (coronary loop); and type 2+3. RESULTS: Overall, 108 neonates who underwent an arterial switch operation were included: 68 were classified as type 1; seven as type 2; 32 as type 3; and one as type 2+3. Overall, 10 adverse events occurred. Five patients died, three from coronary causes. Survival was 96% at 1 month. Transthoracic echocardiography and surgical intraoperative reports differed in 17.6% of cases. Mortality was 15.8% in case of inappropriate diagnosis and 2.2% for appropriate diagnosis (P=0.01). Mortality in type 2 was 66.7% in case of discordance versus 0% when concordant. Multivariable analysis found that inappropriate preoperative transthoracic echocardiography diagnosis of coronary pattern was the only significant risk factor for mortality (P=0.04). CONCLUSIONS: Echocardiography can assess coronary artery anatomy in neonates with transposition of the great arteries. Intramural coronary course is often misdiagnosed. Preoperative misdiagnosis of coronary artery anomaly may impact perioperative mortality. However, this assessment will have to be confirmed by further larger studies.

Outcomes and factors associated with early mortality in pediatric postcardiotomy veno-arterial extracorporeal membrane oxygenation.

Mortality and morbidity of children received veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support after cardiac surgery remain high despite remarkable advances in medical management and devices. The purpose of this study was to describe outcomes and risk factors of applying VA-ECMO in the surgical pediatric population. We retrospectively analyzed 85 consecutive pediatric patients (aged <18 years) who received postcardiotomy VA-ECMO from January 2010 to December 2018. Median (IQR) age at ECMO implantation in this cohort was 12.7 (6.4, 43.2) months, median weight was 8.5 (6.0, 12.8) kg, mean ECMO duration was 143.2 ± 81.6 hours and mean hospital length of stay was 48.4 ± 32.4 days. Seventy-five patients (88.2%) were indicated for postcardiotomy cardiogenic shock. The successful ECMO weaning rate was 70.6% and in-hospital mortality was 52.9%. The most common diagnosis was transposition of great arteries (n = 18, 21.2%), while acute kidney injury occurred most often (n = 64, 75.3%). Multivariate logistic regression analysis showed that thrombocytopenia, hemolysis, and nosocomial infection were positively correlated with in-hospital mortality. Multivariate Cox proportional hazard regression analysis presented that thrombocytopenia significantly increased the 180-day mortality in patients with successful weaning. Therefore, multiple factors had adverse effects on prognosis. Patient selection and procedures from ECMO implantation to weaning need to be closely monitored and performed in a timely manner to improve outcome.

Prenatal diagnosis and planned peripartum care improve perinatal outcome of fetuses with transposition of the great arteries and intact ventricular septum in low-resource settings.

OBJECTIVE: To report on the feasibility of establishing a regional prenatal referral network for critical congenital heart defects (CHDs) and its impact on perinatal outcome of fetuses with transposition of the great arteries and intact ventricular septum (TGA-IVS) in low-resource settings. METHODS: This was a retrospective study of consecutive fetuses with a diagnosis of TGA-IVS between January 2011 and December 2019 in Kochi, Kerala, India. A regional network for prenatal diagnosis and referral of patients with critical CHDs was initiated in 2011. Pregnancy and early neonatal outcomes were reported. The impact of the timing of diagnosis (prenatal or after birth) on age at surgery, perinatal mortality and postoperative recovery was evaluated. RESULTS: A total of 82 fetuses with TGA-IVS were included. Diagnosis typically occurred later on in gestation, at a median of 25 (interquartile range (IQR), 21-32) weeks. The majority (78.0%) of affected pregnancies resulted in live birth, most (84.4%) of which occurred in a specialist pediatric cardiac centers. Delivery in a specialist center, compared with delivery in a local maternity center, was associated with a significantly higher rate of surgical correction (98.1% vs 70.0%; P = 0.01) and overall lower neonatal mortality (3.7% vs 50%; P = 0.001). The proportion of cases undergoing arterial switch operation after prenatal diagnosis of TGA-IVS increased significantly, along with the prenatal detection rate, over the study period (2011-2015, 11.1% vs 2016-2019, 29.4%; P = 0.001). Median age at surgery was significantly lower in the prenatally diagnosed group than that in the postnatally diagnosed group (4 days (IQR, 1-23 days) vs 10 days (IQR, 1-91 days); P < 0.001). There was no significant difference in postoperative mortality (2.0% vs 3.6%; P = 0.49) between the two groups. CONCLUSIONS: This study demonstrates the feasibility of creating a network for prenatal diagnosis and referral of patients with critical CHDs, such as TGA, in low-resource settings, that enables planned peripartum care in specialist pediatric cardiac centers and improved neonatal survival. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Outcome of modified réparation à l'ètage ventriculaire (REV) based on anatomical characteristics for the anomalous ventriculoarterial connection with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: The present study evaluated the results of the modified réparation à l'étage ventriculaire (REV) based on the individual anatomical and pathological findings of the patients with an anomalous ventriculo-arterial connection with ventricular septal defect (VSD) and left ventricular outflow tract obstruction. METHODS: We reviewed a series of 24 patients who underwent modified REV between 2005 and 2019. Surgical indications included ventricles and atrioventricular valves suitable for biventricular repair, severe left ventricular outflow tract obstruction (peak gradient >30 mmHg), unrestrictive subaortic VSD and coronary arteries not suitable for reimplantation. RESULTS: The mean follow-up time was 7.0 ± 4.2 years (range 0.5-14.1 years). Kaplan-Meier analyses showed that overall survival was 100% and freedom from any reoperation was 93.3% ± 6.4%. Longitudinal analyses of the available postoperative echocardiographic data showed that the left ventricular outflow tract peak gradient was less than 10 mmHg in all patients (15/15) and the left ventricular ejection fraction was more than 50% in 93.3% of patients (14/15). The right ventricular outflow tract peak gradient was less than 40 mmHg in 73.3% of patients (11/15). CONCLUSIONS: The REV remains an option for selected patients despite the increasing use in recent years of the Nikaidoh procedure and its modifications. The surgical strategy needs to be determined by the specific anatomical and pathological findings of the patient. The modified REV had excellent long-term survival and freedom from reoperation for the treatment of anomalous ventriculo-arterial connection with VSD and left ventricular outflow tract obstruction. The long-term performance of the reconstructed left ventricular outflow tract and right ventricular outflow tract is satisfactory.

Prenatal Diagnosis of Transposition of the Great Arteries Reduces Postnatal Mortality: A Population-Based Study.

BACKGROUND: Transposition of the great arteries (TGA) may present as a life-threatening neonatal malformation. Although prenatal detection facilitates the perinatal management, the impact on outcome is controversial. METHODS: This study reviewed the differences in prenatal diagnosis of TGA from 2009 to 2014 among the 5 geographic areas in Ontario and compared the management, morbidity, and mortality among neonates with a prenatal (prenatal cohort; n = 70) vs a postnatal (postnatal cohort; n = 76) anomaly diagnosis. Cases were identified from prospective databases of the provincial cardiac tertiary centres and the coroner's office. RESULTS: Prenatal TGA detection rates varied significantly among areas (median: 50%; range: 14% to 72%; P = 0.03). Compared with the postnatal cohort, time from birth to tertiary care admission (1.4 vs 10.4 hours, P < 0.001), prostaglandin therapy (0.1 vs 5.3 hours; P < 0.001), balloon atrial septostomy (5.3 vs 14.9 hours; P <0.001), and arterial switch operation (6 vs 9 days, P = 0.002) was significantly shorter in the prenatal cohort. Although other preoperative variables-including the need of ventilation and mechanical support, morbidity score, and lowest pH and preductal oxygen saturations-were comparable, a prenatal diagnosis was associated with improved 1-year survival (odds ratio: 0.108; 95% confidence interval, 0.013-0.88; P = 0.0184). CONCLUSIONS: Prenatal diagnosis of TGA significantly shortened time intervals from birth to neonatal care and surgery and was associated with improved survival. The prenatal detection rate of TGA in Ontario was low (50% or less) outside of Metropolitan Toronto, suggesting the need for new strategies to further improve intraprovincial detection rates.

Perinatal and perioperative factors associated with mortality and an increased need for hospital care in infants with transposition of the great arteries: A nationwide 11-year population-based cohort.

INTRODUCTION: Newborn infants with transposition of the great arteries (d-TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11-year population-based cohort of d-TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care. MATERIAL AND METHODS: The national cohort consisted of all live-born infants with simple d-TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004-2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated. RESULTS: Preoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but the total mortality rate remained unchanged. All prenatally diagnosed infants (n = 27) survived. Lower gestational age (odds ratio 0.68, P = .012) and higher maternal age at birth (odds ratio 1.16, P = .036) were associated with increased mortality in multivariable analysis. Older infant age at time of operation (P = .002), longer aortic clamp time (P < .001), and higher maternal body mass index (P = .027) were associated with longer initial hospital stay. An extended need for hospital care during the first year of life was multi-factorial. CONCLUSIONS: In our cohort, none of the prenatally diagnosed d-TGA infants died. As a result of the limited prenatal detection rates, however, the sample size was insufficient to reach statistical significance. The d-TGA infants born with lower gestational age and to older mothers had increased mortality.

Twenty-Three-Year Experience With the Arterial Switch Operation: Expectations and Long-Term Outcomes.

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients <18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or <30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012), <2.5 kg (P< 0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P < 0.001), <2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of <1% and good long-term survival. Reinterventions are more frequent in patients <2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time.

Outcomes of the arterial switch operation in patients with aortic arch obstruction.

OBJECTIVE: Transposition of the great arteries or Taussig-Bing anomaly with concomitant aortic arch obstruction is uncommon, with limited data on long-term outcomes after arterial switch operation and aortic arch obstruction repair. This study sought to determine outcomes of patients undergoing arterial switch operation and aortic arch obstruction repair at a single institution. METHODS: From 1983 to 2015, 844 patients underwent an arterial switch operation for biventricular repair at The Royal Children's Hospital. Eighty-three (9.8%, 83/844) patients underwent an arterial switch operation and aortic arch obstruction repair. RESULTS: Fifty-five (66%, 55/83) patients had transposition of the great arteries. and 28 (34%, 28/83) patients had Taussig-Bing anomaly. Fifty-nine (71%, 59/83) patients underwent arterial switch operation and aortic arch obstruction repair as a single-stage procedure, and 24 (29%, 24/83) patients underwent arterial switch operation and aortic arch obstruction repair as a 2-stage procedure. There were 5 early deaths (6.0%, 5/83). Follow-up was available for 74 (95%) of the 78 survivors. Median follow-up was 13.3 years (interquartile range, 7.3-19.3 years; range, 1-30 years). There were no late deaths. Freedom from reintervention was 77%, 71%, and 68% at 5, 10, and 20 years, respectively. Reintervention was more common compared with patients without aortic arch obstruction (P < .001). Reintervention for right-sided obstruction was more common compared with patients without aortic arch obstruction (P = .006). CONCLUSIONS: Patients with transposition of the great arteries or Taussig-Bing anomaly with associated aortic arch obstruction have a higher reintervention rate, especially for right-sided obstruction. Closer monitoring of this subgroup of patients is warranted.

Longitudinal functional health status in young adults with repaired dextro-transposition of the great arteries: A Congenital Heart Surgeons' Society study.

OBJECTIVES: Improved survival has led to interest in functional health status (FHS) as patients with dextro-transposition of the great arteries (d-TGA) transition to adulthood. Our primary objectives were (1) evaluation of The Medical Outcomes Study Short Form-36 Health Survey (SF-36) results; (2) comparison with results of patients who completed the Child Health Questionnaire-Child Form 87 (CHQ-CF87) previously, or the PedsQL Generic Core Scales (PedsQL) survey subsequently; and (3) determination of factors associated with SF-36 domains. METHODS: Survivors from the d-TGA Congenital Heart Surgeons' Society cohort (1985-1989) completed the SF-36 (2010) as a measure of FHS (n = 210; age 21-26 years). Patient characteristics, medical history, psychosocial factors, and previous adolescent CHQ-CF87 FHS assessment (2000) were explored for association with SF-36 domains, along with comparison with recent PedsQL data (2017). RESULTS: Patients scored themselves the same/higher than published normative data in 10 of 10 SF-36 summary scores/domains and similar in 5 of 6 PedsQL summary scores/domains. Factors commonly associated with lower summary scores/domains of the SF-36 were presence of cardiac symptoms, heart condition impacting physical activity/overall health/quality of life, unemployment, and lack of postsecondary education. Less commonly associated factors were lower birth weight, greater total medication number, female sex, shorter procedure-free interval, poor health knowledge, lower family income, younger age at SF-36, living with parents, and being married. These factors accounted for 17% to 47% of the variation in FHS summary scores/domains. FHS was minimally related to d-TGA morphology and repair type. CONCLUSIONS: Patients with d-TGA surviving into adulthood, regardless of morphology or repair type, can primarily expect normal FHS. Addressing the challenges of patients with d-TGA entering adulthood requires consideration of psychosocial factors and clinical management.

Do infants with transposition of the great arteries born outside a specialist centre have different outcomes?

BACKGROUND: Infants born with undiagnosed transposition of the great arteries continue to be born in district general hospitals despite the improvements made in antenatal scanning. Evidence indicates improved outcomes with early definitive treatment after birth, hence the recommendation of delivery in a tertiary centre. The role of specialist paediatric and neonatal transport teams, to advise, stabilise, and transport the infants to a tertiary centre in a timely manner, is critical for those infants born in a district general hospital. This pilot study aims to compare outcomes between infants born in district general hospitals and those who were born in a tertiary maternity unit in South West England and South Wales. METHODS: This was a secondary data analysis of data collected from the local Paediatric Intensive Care Audit Network and the local transport database. Infants born with a confirmed diagnosis of transposition of the great arteries, that required an arterial switch operation as the definitive procedure between April, 2012 and March 2018 were included. RESULTS: Forty-five infants with a confirmed diagnosis of transposition of the great arteries were included. Statistical analysis demonstrated there were no significant differences in the time to balloon atrial septostomy (p = 0.095), time to arterial switch operation (p = 0.461), length of paediatric ICU stay (p = 0.353), and hospital stay (p = 0.095) or mortality between the two groups. CONCLUSIONS: We found no significant differences in outcomes between infants delivered outside the specialist centre, who were transferred in by a specialist team.

Biventricular repair versus Fontan completion for patients with d- or l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: D-transposition of the great arteries and l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction are complex biventricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biventricular procedures in these patients. METHODS: We analyzed 129 patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biventricular repair) undertaken between 1990 and 2015. RESULTS: Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biventricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. Biventricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biventricular groups. CONCLUSIONS: Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction and patients with l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction. Both Fontan and biventricular pathways are associated with excellent mortality and functional outcomes. Biventricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biventricular repair.

Aortic translocation procedure: Early outcomes from a single center.

BACKGROUND/AIM: The data on the Nikaidoh procedure is limited in the literature. This study presents our experiential findings with aortic translocation (Nikaidoh) procedure and its modifications. METHODS: We retrospectively reviewed the data of all the patients who underwent aortic translocation in our institution. RESULTS: Between September 2014 and November 2018, eight patients underwent aortic translocation surgery. The median age was 3.2 years (14 months and 9 years). The diagnosis was transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) in five patients (63%); double-outlet right ventricle (DORV), VSD, and LVOTO in two patients (25%) and DORV and remote VSD in one patient (12%). Additionally, two patients had tricuspid straddling. In terms of surgical procedures, six patients underwent standard Nikaidoh procedures, while one patient underwent double root translocation and one patient underwent a half-turned truncal switch operation. Hospital mortality was recorded for one patient (12.5%). Median intensive care and hospital stay duration was 3 days (interquartile range [IQR]: 2-5 days) and 11 days (IQR: 8-17 days). Median follow-up duration was 10 months (IQR: 10-24 months). One of the patients, who underwent standard Nikaidoh procedure, died in the late follow-up. CONCLUSIONS: Aortic translocation procedure is an option for TGA, VSD, and LVOTO patients with a distal VSD or tricuspid straddling and DORV patients with a small VSD. Avoiding the use of a conduit through modifications, such as double root translocation and half-turned truncal switch operation, may decrease the risk of long-term recurrent interventions.

Late Survival and Patient-Perceived Health Status of the Congenital Heart Surgeons' Society dextro-Transposition of the Great Arteries Cohort.

BACKGROUND: Improved survival for patients with dextro-transposition of the great arteries (d-TGA) has led to an increased focus on functional health status (FHS). We assessed late survival and patient-perceived FHS for repaired TGA patients. METHODS: From 1985-1990, 830 neonates admitted to 24 Congenital Heart Surgeons' Society (CHSS) institutions with d-TGA underwent repair, including 516 arterial switch, 110 Mustard, 175 Senning, and 29 Rastelli operations. Median follow-up was 24.0 years (range, 0-32.7 years). We performed multiphase parametric hazard analysis for death after repair. Patients completed Pediatric Quality of Life Inventory (PedsQL) Core Scales and Cardiac Module Adult Forms. Patient and operative factors and CHSS General Questionnaire responses were analyzed for association with FHS using multiple linear regression. RESULTS: Survival at 30 years after repair was arterial switch, 80% ± 2%; Mustard, 81% ± 5%; Senning, 70% ± 4%; and Rastelli, 86% ± 8%. The arterial switch had the lowest hazard for late death. TGA patients reported FHS similar to a healthy population in all domains except physical health (lower scores). Symptoms, including chest pain and fainting, and having a pacemaker were associated with lower, and being employed with higher, self-reported physical health. Arterial switch patients reported higher FHS than the atrial switch patients in all domains. CONCLUSIONS: Arterial switch patients have a lower risk of premature death and better FHS than those with an atrial switch. Increased surveillance in atrial switch patients is warranted because of their increased risk of late death. Presence of symptoms, pacemaker, and lack of employment are associated with reduced FHS.

Surgical Age and Morbidity After Arterial Switch for Transposition of the Great Arteries.

BACKGROUND: Transposition of the great arteries (TGA) is a complex congenital heart disease that requires early diagnosis as well as advanced surgical repair and postoperative support. This study sought to investigate the impact of surgical timing on early postoperative morbidity. METHODS: This study reviewed all patients with TGA repaired at a single institution (Skåne University Hospital, Lund, Sweden) by arterial switch operation (ASO) between June 2001 and June 2017. Major postoperative morbidity (MPM) and death within 30 days after ASOs were documented. Patients with double-outlet right ventricle, chromosomal abnormalities, and noncardiac diseases were excluded. MPM was defined as the presence of at least 1 of the following: delayed sternum closure, reoperation, prolonged mechanical ventilation, noninvasive ventilation after extubation, peritoneal dialysis, extracorporeal membrane oxygenation, and readmission. RESULTS: A total of 241 patients were included, with medians for birth weight, gestational week, and age at surgery of 3.5 kg, 39 weeks, and 5 days, respectively. MPM was encountered in 32.3% of patients. Prematurity (P = .001) and need for aortic arch repair at the time of ASO (P = .04) were associated with a significant increase in MPM. Non-A coronary anatomy, associated ventricular septal defect requiring surgical closure, and fetal diagnosis of TGA had no significant impact on MPM (P = .35, .08, and .21, respectively). There was no significant difference in MPM among the surgical groups (P = .49). CONCLUSIONS: Early complications after ASO do occur and are mostly associated with prematurity and the need for aortic arch repair. Timing of surgical repair does not seem to influence the rate of these complications.

Pulmonary artery banding and venous bidirectional cava-pulmonary shunt for two-stage arterial switch procedure in late referral of patients with transposition of the great arteries and intact ventricular septum: midterm results.

OBJECTIVE: Two-stage arterial switch operation and left ventricle retraining are necessary for the patients with left ventricle dysfunction and transposition of great vessels with intact ventricular septum (TGA-IVS) who are referred late. MATERIAL AND METHODS: Forty-seven patients with the diagnosis of TGA-IVS and left ventricle dysfunction who underwent arterial switch operation in our centre between July 2013 and August 2017 were analysed retrospectively. The inclusion criteria for left ventricle retraining were patients older than 2 months of age at presentation, having an echocardiographic left ventricle mass index of less than 35 g/m², and having an echocardiographic "banana-shaped" left ventricle geometric appearance. The patients were divided into two groups: pulmonary artery banding and Blalock Taussig shunt were performed as the initial surgical procedure for later arterial switch operation in Group I (n = 19) and pulmonary artery banding and bidirectional cava-pulmonary shunt in Group 2 (n = 28). RESULTS: The average age was found to be 122.3 ± 45.6 days in Group I and 145.9 ± 37.2 days in Group II. There was no statistically significant difference (p = 0.232 versus p = 0.373) between the average left ventricle mass index of the two groups neither before the first stage nor the second stage (26.6 ± 4.8 g/m² versus 25.0 ± 4.9 g/m² and 70.5 ± 12 g/m² versus 673.8 ± 12.0 g/m², respectively). The average time interval for the left ventricle to retrain was 97.7 ± 42.9 days for Group I and 117.3 ± 40.3 days for Group II, significantly lower in Group I (p = 0.027). The time spent in ICU, length of the period during which inotropic support was required, and the duration of hospital stay were significantly higher in Group I (p<0.001, p < 0.001, and p < 0.00, respectively). CONCLUSION: Pulmonary artery banding and bidirectional cava-pulmonary shunt can be performed as a safe and effective alternative to pulmonary artery banding and arterial Blalock Taussig shunt for patients with TGA-IVS in whom arterial switch operation is needed beyond the neonatal period. This approach involves a shorter hospital stay and fewer post-operative complications.

Ductal Stenting to Retrain the Involuted Left Ventricle in d-Transposition of the Great Arteries.

BACKGROUND: Ductal stenting is performed to retrain involuted left ventricles (LVs) in patients with d-transposition of the great arteries and intact ventricular septum (TGA-IVS). However, its efficacy is largely unknown. This study aimed to determine the safety and efficacy of ductal stenting in retraining of the involuted LV in patients with TGA-IVS. METHODS: This was a single-center, retrospective study. Echocardiographic assessment of the LV geometry, mass, and free wall thickness was performed before stenting and before the arterial switch operation. Patients then underwent the arterial switch operation, and the postoperative outcomes were reviewed. RESULTS: There were 11 consecutive patients (male, 81.8%; mean age at stenting, 43.11 ± 18.19 days) with TGA-IVS with involuted LV who underwent LV retraining by ductal stenting from July 2013 to December 2017. Retraining by ductus stenting failed in 4 patients (36.3%). Two patients required pulmonary artery banding, and another 2 had an LV mass index of less than 35 g/m2. Patients in the successful group had improved LV mass index from 45.14 ± 17.91 to 81.86 ± 33.11g/m2 (p = 0.023) compared with 34.50 ± 10.47 to 20.50 ± 9.88 g/m2 (p = 0.169) and improved LV geometry after ductal stenting. The failed group was associated with an increased need for extracorporeal support (14.5% vs 50%, p = 0.012). An atrial septal defect-to-interatrial septum length ratio of more than 0.38 was associated with failed LV retraining. CONCLUSIONS: Ductal stenting is an effective method to retrain the involuted LV in TGA-IVS. A large atrial septal defect (atrial septal defect-to-interatrial septum length ratio >0.38) was associated with poor response to LV retraining.

Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle.

INTRODUCTION: Knowledge gaps exist in the life expectancy and functional outcome of patients with congenitally corrected transposition (ccTGA) presenting early in life, which is relevant in the evaluation of early anatomic repair. METHODS: In a single-center analysis, 91 patients with ccTGA were identified over 25 years, of which 31 presented with biventricular anatomy in the first year of life and formed the study cohort. End points for analysis included survival, moderate or worse tricuspid valve regurgitation, and systemic right ventricle (RV) dysfunction. Median follow-up was 4.9 years (range: 7 days to 20 years). RESULTS: Among 31 patients presenting in the first year of life, 9 (29%) never received cardiac surgery, while 22 (71%) underwent 36 cardiac operations. Overall freedom from moderate or severe systemic RV dysfunction was 75% at 10 years. Overall survival was 82% at 10 years. Surgical mortality was 5.6% (2/36). Among survivors with a systemic RV, 23 (100%) of 23 were Ross or NYHA class I or II at last follow-up. CONCLUSIONS: Congenitally corrected transposition presenting in the first year of life and maintaining a systemic RV can expect (1) long-term survival of more than 80% at 10 years, (2) low expected surgical mortality (overall 6%), and (3) 75% late freedom from major RV dysfunction at 10 years. Pending multi-institutional analyses, this experience with a systemic RV in ccTGA provides an initial benchmark for comparison when considering early elective anatomic correction.

Sudden death in transposition of the great arteries with atrial switch surgery: Autopsy evidence of acute myocardial ischemia despite normal coronary arteries.

BACKGROUND: Sudden death is the leading cause of mortality in patients with transposition of the great arteries (TGA) and atrial switch surgery. Understanding underlying mechanisms could contribute to identifying high-risk patients and preventing such catastrophic deaths. METHODS: A total of 144 adults (≥18 years) with TGA and atrial switch surgery were followed at our adult congenital center since 1989. Four patients were excluded: two with double-outlet right ventricles and two with subsequent arterial switch surgery in childhood. RESULTS: Of the remaining 140 patients, age 37.6 ±â€¯7.8 years, 37.1% female, 8 (6%) had a cardiac arrest of presumed arrhythmic etiology of whom 3 were resuscitated. The arrests occurred in 3 women and 5 men at age 30.5 ±â€¯8.6 (range 22 to 50) years. None had established coronary artery disease, sustained ventricular arrhythmias, or syncope. Four (50%) had atrial arrhythmias and 6 (75%) had at least moderate systemic right ventricular dysfunction. For 5 patients in whom circumstances surrounding the arrests were documented, 3 occurred on exertion, 1 after consuming recreational methamphetamine, and 1 in the context of an atrial tachyarrhythmia. Autopsies were performed in 2 of 5 patients. Both revealed acute massive myocardial infarction of the hypertrophied systemic right ventricle with normal coronary arteries and chronic subendocardial ischemic lesions. CONCLUSION: This is the first report to provide histopathological evidence in support of a myocardial ischemia hypothesis as a cause of sudden death in this patient population, despite the absence of coronary atherosclerosis.