Long-term Outcomes of Staged Complete Repair of Pulmonary Atresia With Ventricular Septal Defect.

BACKGROUND: The optimal strategy for pulmonary atresia with ventricular septal defect (PA-VSD) without major aortopulmonary collateral arteries is yet to be defined. It remains unclear how primary complete repair compares with staged repair. METHODS: This study reviewed the records of 107 patients with PA-VSD who were managed with systemic-to-pulmonary shunts from February 1989 to November 2019. Tables presenting summary statistics of patient and surgical data and Kaplan-Meier curves showing repair, mortality, and reoperation were created. Logistic regression analysis was performed to identify risk factors for repair, morbidity, and mortality. RESULTS: A total of 91 (85%) patients achieved complete repair at a median age of 1 year (interquartile range [IQR], 8-20 months). Survival was as follows: 97 (90%) patients at 6 months, 91 (85%) at 20 months, and 87 (81%) at 10 years. A shunt diameter of 3.5 mm or less (P = .006) and shunt after 2009 (P < .001) were associated with earlier progression to complete repair. A shunt diameter of 3.5 mm or less was an independent risk factor for interstage morbidity (hazard ratio, 4.5; IQR, 1.1-18.5; P = .039), but it was not associated with complete repair (P = .75) or mortality (P = .62). The median follow-up period was 10.5 years (IQR, 3.6-18.8 years) from birth. CONCLUSIONS: The complete repair and long-term survival rates of staged PA-VSD repair are high. The use of smaller shunts has resulted in earlier complete repair and is associated with increased risk of morbidity but not mortality.

Propensity Score Matched Analysis of Cleft Closure in Complete Atrioventricular Septal Defect Repair.

BACKGROUND: Repair of complete atrioventricular septal defect (cAVSD) is achieved with low mortality. However, there is a high rate of reoperation on the left atrioventricular valve (LAVV), which is often attributed to nonclosure of the cleft. Although nonclosure of the cleft has been reported to be a risk factor for reoperation, no randomized controlled or propensity-matched trials have ever been performed. We investigated the effect of cleft closure on outcomes after cAVSD repair. METHODS: We reviewed 455 patients who underwent cAVSD repair between 1990 and 2019. To determine the effect of cleft closure, propensity score matching was performed on risk factors for reoperation after cAVSD repair. RESULTS: Median age was 3.6 months (mean, 9.6 ± 20.4 months), median weight was 4.3 kg (mean, 4.7 ± 4.3 kg) and 41.9% (191 of 455) were male. Early mortality was 2.9% (13 of 455), and survival was 89.8% ± 1.9% at 20 years. Early reoperation was a risk factor for mortality (P = .004). Freedom from reoperation was 72.5% ± 4.0% at 20 years. Freedom from LAVV reoperation was 74.1% ± 4.0% at 20 years. Preoperative severe LAVV regurgitation (P < .001) and early postoperative moderate or greater LAVV regurgitation (P = .007) were risk factors for reoperation, while trisomy 21 (P = .03) and recent era of surgery (P = .02) were protective. Propensity score matching yielded 106 pairs. There were no differences in long-term survival (P = .71) or reoperation (P = .26) between the 2 groups. CONCLUSIONS: Repair of cAVSD can be achieved with low mortality and good long-term survival, although the reoperation rate remains high. Similar freedom from reoperation can be achieved with or without closure of the LAVV cleft.

Arterial Switch Operation in Patients With Taussig-Bing Anomaly and Aortic Arch Obstruction.

BACKGROUND: Arterial switch operation (ASO) in children with Taussig-Bing anomaly and associated aortic arch obstruction (AAO) is technically demanding. This study sought to determine outcomes of patients with Taussig-Bing anomaly and AAO who underwent ASO at a single institution. METHODS: From 1983 to 2015, 844 patients underwent an ASO for biventricular repair at The Royal Children's Hospital. Twenty-eight patients (3.3%) had Taussig-Bing anomaly and AAO. RESULTS: Of 28 patients, 21 (75%) underwent ASO and AAO repair as a single-stage procedure and 7 (25%) had arch repair before ASO. There were no early or late deaths. Follow-up after hospital discharge was available for all patients. Median follow-up was 17.5 years (interquartile range, 9.2-22.3; range, 4.0-31.5). All patients were in New York Heart Association class I at last follow-up. Freedom from reintervention was 61% (95% confidence interval, 40-76) and 56% (95% confidence interval, 36-73) at 5 and 15 years, respectively, after ASO. The most common reinterventions were repair of recurrent AAO, branch pulmonary artery stenosis, and neoaortic valve repair. Four of 28 patients (14%) required 5 reinterventions (3 reoperations and 2 catheter reintervention) for recurrent AAO at a median of 2.2 years after ASO (interquartile range, 2.1-2.3). CONCLUSIONS: Patients with Taussig-Bing anomaly and AAO can undergo ASO with excellent survival. Reintervention is equally common for both left- and right-sided obstruction.

Aortic valve repair in children without use of a patch.

BACKGROUND: We aimed to assess the long-term outcomes of children in whom the aortic valve could be repaired without the use of patch material. We hypothesized that if the aortic valve is of sufficiently good quality to perform repair without patches, a durable repair could be achieved. METHODS: All children (n = 102) who underwent aortic valve repair without the use of a patch between 1980 and 2016 were reviewed. RESULTS: The median patient age at operation was 2 years (interquartile range, 1 month to 9.6 years). There were 25 neonates and 17 infants. There was no operative mortality. Mean overall survival at 10 years was 97.7% ± 0.01% (95% confidence interval, [CI] 91.0%-99.4%). Forty-three patients (42.2%) required 56 aortic valve reoperations, including 24 redo aortic valve repairs, 22 Ross procedures, 8 mechanical aortic valve replacements, and 2 homograft aortic valve replacements. Mean freedom from aortic valve reoperation at 10 years was 57.4% ± 0.06% (95% CI, 44.9%-68.1%), and freedom from aortic valve replacement at 10 years was 74.5% ± 0.05% (95% CI, 63.0%-82.9%) at 10 years. Freedom from aortic valve reoperation at 10 years was 33.1% ± 0.1% (95% CI, 14.5%-53.2%) in neonates and 68.9% ± 0.06% (95% CI, 54.5%-79.6%) in older children (P < .01). CONCLUSIONS: In approximately one-third of children undergoing aortic valve repair, the repair could be achieved without patches. In these children, aortic valve repair was achieved without operative mortality. Infants and older children have low reoperation rates, whereas reoperation rates in neonates are higher. Initial repair allows valve replacement to be delayed to later in childhood, when a more durable result may be achieved.

Outcomes of Interrupted Aortic Arch Repair in Children With Biventricular Circulation.

BACKGROUND: To evaluate the outcomes after surgical repair of interrupted aortic arch in patients with biventricular circulation. METHODS: We reviewed all children from a single institution (N = 177) who had interrupted aortic arch repair between 1978 and 2018. Patients were separated into simple (n = 122) and complex (n = 55) group based on their concomitant anomalies. RESULTS: Median age at repair was 6 days (range, 1-298 days) and median weight was 3.1 kg (range, 0.95-5.1 kg). Median follow-up time was 11.5 years (mean 12.6 years; range, 0.1-35.9 years). Overall early mortality was 11.9% (21 of 177) and there were 5 late deaths. Era of surgery did not impact on overall survival (P = .37). Between 2000 and 2018, there was a significant difference in early mortality between the simple and complex group (3.2% [2 of 62] vs 24.1% [7 of 29], P = .002). There was an improvement in mortality in the simple group over time (P = .03). Competing risks analysis showed at 15 years after the initial operation 14% had died without arch reoperation, 15.2% had undergone aortic arch reoperation, and 70.8% were alive without arch reoperation. Reoperation on the aortic arch was more common in the complex group compared to the simple group (20.0% [11 of 55] vs 9.0% [11 of 122], P< .001). CONCLUSIONS: Survival of patients with interrupted aortic arch and associated simple anomalies has improved over time, although mortality in patients with complex congenital cardiac lesions remains high.

The quadricuspid truncal valve: Surgical management and outcomes.

OBJECTIVE: To determine the outcomes of patients with a quadricuspid truncal valve (TV) and durability of TV repair. METHOD: We reviewed 56 patients with truncus arteriosus and a quadricuspid TV who underwent complete repair between 1979 and 2018. RESULTS: TV insufficiency was present in 39 patients (mild, n = 22; moderate, n = 14; and severe, n = 3). Fourteen patients had concomitant TV surgery. Early mortality in patients who had concomitant TV surgery was 14% (2 out of 14 patients) and overall survival was 77.1% ± 11.7% at 15 years. Freedom from TV reoperation was 30.3% ± 14.6% at 15 years. Early mortality in patients who did not undergo concomitant TV surgery was 9.5% (4 out of 42 patients) and overall survival was 74.9% ± 6.9% at 15 years. Progression of TV insufficiency requiring TV surgery occurred in 16.7% (7 out of 42 patients). Freedom from TV reoperation was 77.1% ± 7.8% at 15 years. The most common method of repair was tricuspidization of the TV. Freedom from TV reoperation was 64.3% ± 21.0% at 10 years after tricuspidization and 0% at 6 years after other types of TV surgery. Overall follow-up was 97.6% (41 out of 42 patients) complete for survivors with median follow-up of 16.6 years. At last follow-up there was no TV insufficiency in 16 patients, mild insufficiency in 24 patients, and moderate insufficiency in 1 patient. CONCLUSIONS: More than one-third of patients with a quadricuspid TV require TV surgery. Tricuspidization of the quadricuspid TV appears to be a durable repair option with good long-term outcomes.

Surgery for Truncus Arteriosus: Contemporary Practice.

Surgery for truncus arteriosus has an early mortality of 3% to 20%, with a long-term survival of approximately 75% at 20 years. Nowadays, truncus arteriosus repair is mostly done in the neonatal period together with a single-staged repair of concomitant cardiovascular anomalies. There are many challenging subgroups of patients with truncus arteriosus, including those with clinically significant truncal valve insufficiency, an interrupted aortic arch, or a coronary artery anomaly. In fact, truncal valve competency appears to be the most important factor influencing the outcomes after truncus arteriosus repair. The use of a conduit during truncus arteriosus repair invariably requires reoperation on the right ventricular outflow tract. Through improvements in perioperative techniques over time, many children are now living well into adulthood after repair of truncus arteriosus, albeit with a high rate of reoperation. Despite this, the long-term outcomes of truncus arteriosus repair are good, with many patients being asymptomatic and with a quality of life comparable to the general population.

Truncus arteriosus repair: A 40-year multicenter perspective.

OBJECTIVE: To examine the long-term surgical outcomes of patients who underwent truncus arteriosus (TA) repair. METHODS: Between 1979 and 2018, a total of 255 patients underwent TA repair at 3 Australian hospitals. Data were obtained by review of medical records from initial admission until last cardiology follow-up. RESULTS: At the time of TA repair, the median patient age was 44 days, and median weight was 3.5 kg. Early mortality was 13.3% (34 of 255), and overall survival was 76.8 ± 2.9% at 20 years. Neonatal surgery and low operative weight were risk factors for early mortality. Most deaths (82.5%; 47 of 57) occurred within the first year following repair. A coronary artery anomaly and early reoperation were identified as risk factors for late mortality. A total of 175 patients required at least 1 reoperation, with overall freedom of reoperation of 2.9 ± 1.5% at 20 years. Follow-up of survivors was 96% complete (191 of 198). The median duration of follow-up was 16.4 years. At the last follow-up, 190 patients were categorized as New York Heart Association class I/II, and 1 patient was class III. CONCLUSIONS: TA repair during the neonatal period presents significant surgical challenges. Neonates with signs of overcirculation should be operated on promptly. A coronary artery anomaly is a risk factor for late mortality. Survival beyond the first year following repair is associated with excellent outcomes.

Long-term outcomes following repair of truncus arteriosus and interrupted aortic arch.

OBJECTIVES: We aim to evaluate the long-term outcomes following repair of truncus arteriosus with an interrupted aortic arch. METHODS: We reviewed all children (n = 24) who underwent repair of truncus arteriosus and an interrupted aortic arch between 1979 and 2018 in a single institution. The morphology of the interrupted aortic arch was type A in 5, type B in 18 and type C in 1. RESULTS: The median age at repair was 10 days and the median weight was 3.1 kg. Direct end-to-side anastomosis of the ascending and descending aorta was performed in 16 patients (67%, 16/24), patch augmentation in 5 patients (21%, 5/24) and direct anastomosis with the use of an interposition graft to the descending aorta in 2 patients (8%, 2/24). One patient, the first in the series, underwent interrupted aortic arch repair via subclavian flap aortoplasty prior to truncus repair. A period of deep hypothermic circulatory arrest was used in 16 patients, and isolated cerebral perfusion was used in 8 patients. The early mortality rate was 17% (4 out of 24 patients). There were no late deaths and overall survival was 83 ± 8% [95% confidence interval (CI) 61-93] at 20 years. Freedom from any reoperation was 33 ± 11% (95% CI 14-54) at 5 years and 13 ± 9% (95% CI 2-34) at 10 years. Six patients underwent 10 aortic reoperations. Freedom from aortic arch reoperation was 69 ± 11% (95% CI 42-85) at 10 and 20 years. Follow-up was 95% complete (19/20), with a median follow-up time of 20 years. At last follow-up, no clinically significant aortic arch obstruction was identified in any patient, and all patients were in New York Heart Association Class I/II. CONCLUSIONS: Repair of truncus arteriosus with an interrupted aortic arch with direct end-to-side anastomosis results in good survival beyond hospital discharge. Although the long-term functional state of patients is good, reoperation rates are high.

Ross Operation in Children: 23-Year Experience From a Single Institution.

BACKGROUND: Data on the long-term outcomes in children after the Ross operation are limited. This study aimed to assess the long-term outcomes in children who underwent the Ross operation at a single institution. METHODS: The study reviewed all children (n = 140) who underwent the Ross operation at the Royal Children's Hospital in Melbourne, Australia between 1995 and 2018. RESULTS: Mean follow-up time was 8.9 years. Median age at operation was 7.4 years. The root replacement (n = 120, Ross-Konno; n = 38), root inclusion (n = 17), and subcoronary implantation (n = 3) techniques were used. Operative mortality was 5.0% (7 of 140; 3 neonates and 4 infants). There were 6 late deaths. Overall survival at 10 years was 96.2% in children older than 1 year of age and 78.9% in children younger than 1 year of age at operation (P = .003). Freedom from autograft reoperation was 86.0% at 10 years. Age younger than 1-year at operation was a risk factor for autograft reoperation (P = .02). Patients younger than 1 year of age at operation experienced a higher incidence of moderate or greater aortic insufficiency compared with patients who were older than 1 year of age (P = .006). In patients who had a poly-(p-dioxanone)-filament band placed around the sinotubular junction, freedom from moderate or greater aortic insufficiency at 10 years was 100%, compared with 83.1% in patients with no band (P = .09). CONCLUSIONS: In children older than 1 year of age, the Ross operation has excellent outcomes with no operative mortality and a low incidence of aortic insufficiency. In children younger than 1 year of age, the Ross operation is associated with higher operative mortality and a higher incidence of aortic insufficiency. Where possible, the Ross operation should be delayed beyond infancy. Poly-(p-dioxanone)-filament banding may reduce the incidence of aortic insufficiency after the Ross operation.

Long-term quality of life in adults following truncus arteriosus repair.

OBJECTIVES: To date, few studies have assessed the quality of life following congenital cardiac surgery. In this study, we aimed at determining the quality of life after truncus arteriosus (TA) repair using the Short Form 36 questionnaire in adult survivors. METHODS: Seventy-three patients (age >18 years) who underwent TA repair at the Royal Children's Hospital, Australia were identified for the study. Of these, 42 patients (58%, 42/73) participated in the study and completed the Short Form 36 questionnaire. The results of the 8 domains and the derived health state summary score (Short Form Six Dimension, SF-6D) were compared with age-matched Australian population controls, and with patients who underwent the arterial switch operation (ASO). RESULTS: Compared with the age-matched Australian population, 18- to 24-year-old TA patients (31%, 13/42) had lower scores in 6 of 8 domains; 25- to 34-year-old TA patients (36%, 15/42) scored lower in 5 of 8 domains; and 35- to 44-year-old TA patients (33%, 14/42) scored lower in 4 of 8 domains. SF-6D scores were not significantly different between TA patients and the age-matched Australian population. Compared with patients who underwent ASO, 18-to 24-year-old TA patients scored lower in 3 of 8 domains; and 25- to 34-year-old TA patients scored lower in 2 of 8 domains. There was no significant difference in SF-6D scores between TA and patients who underwent the ASO. CONCLUSIONS: Adult survivors of TA have similar quality of life compared with age-matched Australian controls measured by SF-6D. Despite a higher reoperation rate in TA patients, they have similar quality of life compared with ASO patients.

Impact of truncal valve surgery on the outcomes of the truncus arteriosus repair.

OBJECTIVES: Preoperative moderate or greater truncal valve (TV) insufficiency is one of the most important factors influencing mortality in children with truncus arteriosus. We therefore sought to determine the impact of TV insufficiency and concomitant TV surgery on children who underwent truncus arteriosus repair at a single institution. METHODS: We reviewed 180 patients who underwent truncus arteriosus repair between 1979 and 2016. Preoperative echocardiography demonstrated TV insufficiency in 80 patients (mild: 33.9%, 61/180; moderate: 9.4%, 17/180 and severe: 1.1%, 2/180). RESULTS: Twenty-one patients had concomitant TV surgery with an early mortality of 19% (4/21) and overall survival of 70.8 ± 10.1% at 25 years. There were 60 neonates, 11 of whom had concomitant TV surgery with an early mortality of 27% (3/11) and overall survival of 62.3 ± 15.0% at 20 years. Concomitant TV repair (P = 0.5) was not a risk factor for death. TV reoperation was common in those who had concomitant TV surgery, with freedom from reoperation of 19.2 ± 14.9% at 20 years. In the remaining 159 patients, 14 required subsequent TV surgery, and the freedom from TV surgery was 84.0 ± 4.6% at 20 years. At a median follow-up of 18.5 years, TV insufficiency was none or trivial in 79.6% (109/137) and mild or less in 98.5% (135/137) of patients. CONCLUSIONS: Most patients with mild TV insufficiency are free from TV surgery up to 25 years. The durability of TV repair is poor. Most patients with moderate or greater TV insufficiency and a quadricuspid TV will require TV surgery.