Long-Term Follow-Up of Right Ventricle to Pulmonary Artery Biologic Valved Conduits Used in Pediatric Congenital Heart Surgery.

Valved conduit reconstruction between the right ventricle (RV) and the pulmonary circulation is often necessary in the surgical treatment of complex congenital heart defects. The aim of this study is to evaluate the long-term performance of the three types of conduits we have used and assess risk factors for conduit failure. Retrospective, single-center review of 455 consecutive pediatric patients with 625 conduits from 1990 to 2019 undergoing RV-to-pulmonary artery (PA) reconstruction with a valved conduit. The three conduit types investigated were pulmonary homograft, aorta homograft, and bovine jugular vein (BJV) graft. Overall patient survival was 91.4%, freedom from conduit replacement (FCR) was 47.4%, and freedom from reintervention (FFR) was 37.8% with a median follow-up of 8.7 years (interquartile range 4.3-13.3 years). For pulmonary homografts, 10-, 20-, and 28-year FCR was 79.6%, 68.6%, and 66.0%, respectively. For aortic homografts, 10-, 20-, and 30-year FCR was 49.8%, 31.5%, and 23.0%, respectively. For BJV grafts, 10- and 19-year FCR was 68.1% and 46.0%, respectively. When controlling for baseline variables, FCR was similar for pulmonary homografts and BJV grafts. Overall patient survival was excellent. Risk factors for conduit failure in patients operated with reconstruction of the RV-PA outflow tract included low age, low weight, small conduit size, and certain cardiac diagnoses. There was no evidence for a shorter life span of the second graft. Pulmonary homografts and BJV grafts performed similarly but the risk of endocarditis was greater in the BJV group.

Mitral valve repair using leaflet expansion and subpartial annuloplasty in children.

Objective: Mitral valve reconstruction in the pediatric population is a challenge due to the frequent combination of annular dilatation and leaflet restriction and the need for growth. We present a novel strategy using leaflet expansion and subpartial annuloplasty with polytetrafluoroethylene reinforcement. Methods: From January 2014 through May 2021, 11 children aged 5 months to 14 years (median, 24 months) underwent elective mitral valve repair due to severe mitral valve regurgitation. The mitral valve abnormalities included congenital malformations (n = 7), postoperative leakage following commissurotomy (n = 1), and functional mitral valve regurgitation due to dilated cardiomyopathy (n = 3). Surgery consisted of leaflet expansions with autologous, untreated pericardium and subpartial annuloplasty with polytetrafluoroethylene reinforcement. Results: All children survived their surgeries with uneventful postoperative courses, except for 1 patient who needed an early reoperation to resolve a functional stenosis due to a spinnaker phenomenon. At discharge, mean gradient was 3.5 ± 3.9 mm Hg, with trivial mitral regurgitation in 9 patients (82%). All patients were alive and asymptomatic during the median follow-up of 3 years (range, 1-7 years). Their echocardiographic data showed a mean transmitral gradient of 4.4 ± 1.7 mm Hg and remained unchanged. Residual mitral valve regurgitation was trivial or mild in 9 patients (82%) and moderate in 2 patients (18%). Conclusions: Leaflet expansion with autologous pericardium and subpartial annuloplasty with polytetrafluoroethylene reinforcement for mitral regurgitation in the pediatric population gives stable and satisfactory results both early and at intermediate follow-up, permitting growth of the mitral valve.

Evaluation and comparison of patch materials used for pulmonary arterioplasty in pediatric congenital heart surgery.

Objective: To evaluate the long-term performance of the patch materials we have used to augment the pulmonary arterial tree across a wide spectrum of diagnoses and anatomical locations. Methods: Retrospective, single-center review of 217 consecutive pediatric patients at a tertiary referral center from 1993 to 2020 who underwent patch arterioplasty of the pulmonary arterial tree from the pulmonary bifurcation to the distal pulmonary arterial branches. Reintervention data were collected and analyzed. Lesion-specific anatomy and other variables were analyzed as risk factors for reintervention. Results: There were 280 total operations performed (217 initial operations and 63 reoperations) and 313 patches used. The patches used were autologous pericardium (166, 53.0%), pulmonary homograft (126, 40.3%), and a heterogeneous group of other materials (21, 6.7%). Overall patient survival was 86.2%, freedom from reoperation was 81.0% and freedom from reintervention (FFR) was 70.6%, with a median follow-up of 13.8 years (interquartile range, 6.3-17.9 years). For all patches, 10-, 20-, and 27-year FFR was 76.6%, 70.6%, and 70.6%, respectively. FFR was similar among all 3 patch type groups (P = .29). Multivariable Cox regression analysis showed that diagnoses of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries and hypoplastic left heart syndrome, patches placed at initial cardiac operation, and increasing number of cardiac operations were risk factors for reintervention. Conclusions: Autologous pericardium and pulmonary homograft patches performed similarly. Although patch type conferred no difference in need for reintervention, other risk factors did exist. Namely, diagnoses of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries and hypoplastic left heart syndrome, patch placement at a patient's first cardiac operation, and increasing number of cardiac operations were risk factors for reintervention.

Reoperations After Repair for Atrioventricular Septal Defects: >25 Years Experience at a Single Center.

Our aim was to evaluate the total burden of reoperations after previous repair for atrioventricular septal defects, including long-term survival and identify risk factors for reoperation. All patients with surgical correction for atrioventricular septal defect (AVSD) 1993- 2020 underwent a follow-up in October 2020. Clinical data were obtained by retrospective review and evaluated with Kaplan-Meier and competing risk analysis. Of 477 patients who underwent initial repair, 53 patients (11.1%) underwent a total of 82 reoperations. The perioperative mortality at reoperation was 3.8% (2/53). There were no late deaths (0/51) during follow-up. In patients requiring reoperation for left atrioventricular valve regurgitation, a re-repair was performed in 90% (26/29) at first attempt. Estimated overall survival was 96.2 ± 2.6% (95% CI 91.2-100) in the Any reoperation group and 96.7 ± 0.9% (95% CI 94.9-98.5) in the No reoperation group at 20 years (P = 0.80). The cumulative incidence function of Any reoperation (with death as competing risk) was 13.0% (95% CI 9.4-16.5) at 20 years. Independent risk factors for Any reoperation included severe mitral regurgitation after primary repair (HR 40.7; 95% CI 14.9-111; P < 0.001). The risk of perioperative mortality in AVSD patients undergoing reoperation was low in the present study. Long-term survival was very good and not significantly different when compared to patients who did not need reoperation. Re-repair for left atrioventricular valve regurgitation was possible in most cases and showed long-term durability. Our data suggest that reoperations after primary repair of AVSD have very good long-term outcomes when performed at a high-volume pediatric cardiac surgery center.

Outcomes in neonatal critical and non-critical aortic stenosis: a retrospective cohort study.

OBJECTIVE: To compare long-term survival, reinterventions and risk factors using strict definitions of neonatal critical and non-critical valvular aortic stenosis (VAS). DESIGN: A nationwide retrospective study using data from patient files, echocardiograms and the Swedish National Population Registry. SETTING AND PATIENTS: All neonates in Sweden treated for isolated VAS 1994-2018. We applied the following criteria for critical aortic stenosis: valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function (fractional shortening ≤27%). Indication for treatment of non-critical VAS was Doppler mean gradient >50 mm Hg. MAIN OUTCOME MEASURES: Short-term and long-term survival, aortic valve reinterventions need of valve replacements, risk factors for reintervention and event-free survival. RESULTS: We identified 65 patients with critical VAS and 42 with non-critical VAS. The majority of the neonates were managed by surgical valvotomy. Median follow-up time was 13.5 years, with no patients lost to follow-up. There was no 30-day mortality. Long-term transplant-free survival was 91% in the critical stenosis group and 98% in the non-critical stenosis group (p=0.134). Event-free survival was 40% versus 67% (p=0.002) in the respective groups. Median time from the initial treatment to reintervention was 3.6 months versus 3.9 years, respectively (p=0.008). CONCLUSIONS: Critical VAS patients had significantly higher need for reintervention during the first year of life, lower event-free survival and lower freedom from aortic valve replacement at age ≥18 years, compared with neonates with non-critical stenosis.

Impact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect.

OBJECTIVE: We studied a cohort of patients with nonsyndromic complete atrioventricular septal defect with and without concomitant complex cardiac anatomy and compared the outcomes after surgical repair. METHODS: Between 1993 and 2018, 62 nonsyndromic patients underwent complete atrioventricular septal defect repair. Sixteen patients (26%) had complex complete atrioventricular septal defect with variables representing concomitant cardiac anatomic complexity: tetralogy of Fallot, double outlet right ventricle, total anomalous pulmonary venous return, concomitant aortic arch reconstruction, multiple ventricular septal defects, staged repair of coarctation of the aorta, and a persisting left superior vena cava. The mean follow-up was 12.7 ± 7.9 years. Baseline variables were retrospectively evaluated and analyzed using univariable logistic regression. Survival was studied using Kaplan-Meier estimates, and group comparisons were performed using the log-rank test. A competing-risk analysis estimated the risk of reoperation with death as the competing event. A Gray's test was used to test equality of the cumulative incidence curves between groups. RESULTS: The perioperative mortality was 3.2% (2/62). Actuarial survival was 100% versus 66.7% ± 14.9% at 10 years in the noncomplex and complex groups, respectively (P < .01). There was no significant difference in the overall reoperation rate between the noncomplex group (7/46; 15%) and the complex group (4/16; 25%) (odds ratio, 1.86; 95% confidence interval, 0.46-7.45; P = .30). The competing-risk analysis demonstrated no significant difference in reoperation between the groups (P = .28). CONCLUSIONS: Our data show that nonsyndromic patients without complex cardiac anatomy have a good long-term survival and an acceptable risk of reoperation similar to contemporary outcomes for patients with complete atrioventricular septal defect with trisomy 21. However, the corresponding group of nonsyndromic patients with concomitant complex cardiac lesions are still a high-risk population, especially regarding mortality.

Long-term outcome after early repair of complete atrioventricular septal defect in young infants.

OBJECTIVE: The long-term outcome after repair of complete atrioventricular septal defect in young infants is still not fully understood. The objective of this study was to evaluate data after repair for complete atrioventricular septal defect over a 25-year period to assess survival and identify risk factors for left atrioventricular valve-related reoperations. METHODS: A total of 304 consecutive patients underwent surgical correction for complete atrioventricular septal defect between April 1993 and October 2018. The results for young infants (aged <3 months; n = 55; mean age 1.6 ± 0.6 months) were compared with older infants (aged >3 months; n = 249; mean age, 5.1 ± 5.2 months). Mean follow-up was 13.2 ± 7.8 years (median, 14.0 years; interquartile range, 7.0-20.0). The Kaplan-Meier method was used to assess overall survival and freedom from left atrioventricular valve-related reoperation. RESULTS: Overall, 30-day mortality was 1.0% (3/304) with no difference between young and older infants (P = 1.0). Overall survival in the total population at 20-year follow-up was 95.1% (±1.3%). Independent risk factors for poor survival were the presence of an additional ventricular septal defect (P = .042), previous coarctation of the aorta (P < .001), persistent left superior vena cava (P = .026), and genetic syndromes other than Trisomy 21 (P = .017). Freedom from left atrioventricular valve-related reoperation was 92.6% (±1.7%) at 20 years. There was no significant difference in left atrioventricular valve-related reoperation in young infants compared with older infants (P = .084). CONCLUSIONS: Our data demonstrated that excellent long-term survival could be achieved with early repair for complete atrioventricular septal defect, and the need for reoperations due to left atrioventricular valve regurgitation was low. Primary correction in patients aged less than 3 months is, when clinically necessary, well tolerated. Palliative procedures can be avoided in the majority of patients.