Recurrent Pediatric Respiratory Distress From a Challenging Vascular Anomaly: The Uncrossing Operation for Circumflex Aorta.

The authors report a case of circumflex aorta causing persistent respiratory distress in a 9-year-old boy who had previously undergone vascular ring division and multiple aortopexies. The uncrossing operation was performed, with symptomatic relief of both posterior and right-sided tracheal compression from the transverse aorta and right aortic arch, respectively.

Prognostic utility of a novel risk prediction model of 1-year mortality in patients surviving to discharge after surgery for congenital or acquired heart disease.

OBJECTIVE: We sought to develop a novel risk prediction model of 1-year mortality after congenital heart surgery that accounts for clinical, anatomic, echocardiographic, and socioeconomic factors. METHODS: This was a single-center, retrospective review of consecutive index operations for congenital or acquired heart disease, from January 2011 to January 2021, among patients with known survival status at 1 year after discharge from the index hospitalization. The primary outcome was postdischarge mortality at 1 year. Variables of interest included age, prematurity, noncardiac anomalies or syndromes, the Childhood Opportunity Index, primary procedure, major adverse postoperative complications, and the Residual Lesion Score. Logistic regression was used to develop a weighted risk score for the primary outcome. Internal validation using a bootstrap-resampling approach was performed. RESULTS: Of 10,412 consecutive operations for congenital or acquired heart disease, 8808 (84.6%) cases met entry criteria, including survival to discharge. There were 190 (2.2%) deaths at 1 year postdischarge. A weighted risk score was formulated on the basis of the variables in the final risk prediction model, which included all aforementioned risk factors of interest. This model had a C-statistic of 0.82 (95% confidence interval, 0.80-0.85). The median risk score was 6 (interquartile range, 4-8) points. Patients were categorized as low (score 0-5), medium (score 6-10), high (score 11-15), or very high (score 16-20) risk. The expected probability of mortality was 0.4% ± 0.2%, 2.0% ± 1.1%, 10.1% ± 5.0%, and 36.6% ± 9.6% for low-risk, medium-risk, high-risk, and very high-risk patients, respectively. CONCLUSIONS: A risk prediction model of 1-year mortality may guide prognostication and follow-up of patients after discharge after surgery for congenital or acquired heart disease.

Growth of the Neo-Aortic Root and Prognosis of Transposition of the Great Arteries.

BACKGROUND: Neo-aortic root dilatation can lead to significant late morbidity after the arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA). OBJECTIVES: We sought to examine the growth of the neo-aortic root in d-TGA. METHODS: A single-center, retrospective cohort study of patients who underwent the ASO between July 1, 1981 and September 30, 2022 was performed. Morphology was categorized as dextro-transposition of the great arteries with intact ventricular septum (d-TGA-IVS), dextro-transposition of the great arteries with ventricular septal defect (d-TGA-VSD), and double-outlet right ventricle-transposition of the great arteries type (DORV-TGA). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before the ASO and throughout follow-up. Trends in root dimensions over time were assessed using linear mixed-effects models. The association between intrinsic morphology and the composite of moderate-severe aortic regurgitation (AR) and neo-aortic valve or root intervention was evaluated with univariable and multivariable Cox proportional hazards models. RESULTS: Of 1,359 patients who underwent the ASO, 593 (44%), 666 (49%), and 100 (7%) patients had d-TGA-IVS, d-TGA-VSD, and DORV-TGA, respectively. Each patient underwent a median of 5 echocardiograms (Q1-Q3: 3-10 echocardiograms) over a median follow-up of 8.6 years (range: 0.1-39.3 years). At 30 years, patients with DORV-TGA demonstrated greater annular (P < 0.001), sinus of Valsalva (P = 0.039), and sinotubular junction (P = 0.041) dilatation relative to patients with d-TGA-IVS. On multivariable analysis, intrinsic anatomy, older age at ASO, at least mild AR at baseline, and high-risk root dilatation were associated with moderate-severe AR and neo-aortic valve or root intervention at late follow-up (all P < 0.05). CONCLUSIONS: Longitudinal surveillance of the neo-aortic root is warranted long after the ASO.

Early Outcomes of Pulmonary Valve Replacement With the Edwards Inspiris Resilia Pericardial Bioprosthesis.

BACKGROUND: Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aortic bioprosthesis in the pulmonary position in pediatric and adult patients requiring PVR. METHODS: Data from consecutive patients who underwent PVR from February 2019 to February 2021 at our institution were retrospectively reviewed. Postoperative adverse events included paravalvular or transvalvular leak, endocarditis, explant, thromboembolism, valve thrombosis, valve-related bleeding, hemolysis, and structural valve degeneration. Progression of valve gradients was assessed from discharge to 30 days and one year. RESULTS: Of 24 patients with median age of 26 years (interquartile range [IQR]: 17-33; range: 4-60 years), 22 (91.7%) patients had previously undergone tetralogy of Fallot repair and 2 (8.3%) patients had undergone double-outlet right ventricle repair in the neonatal period or infancy. All patients had at least mild right ventricular (RV) dilatation (median RV end-diastolic volume index 161.4, IQR: 152.3-183.5 mL/m2) and at least moderate pulmonary insufficiency (95.8%) or stenosis (8.3%). Median cardiopulmonary bypass and cross-clamp times were 71 (IQR: 63-101) min and 66 (IQR: 60-114) min, respectively. At a median postoperative follow-up of 2.5 years (IQR: 1.4-2.6; range: 1.0-3.0 years), there were no mortalities, valve-related reoperations, or adverse events. Postoperative valve gradients and the severity of pulmonary regurgitation did not change significantly over time. CONCLUSIONS: At short-term follow-up, the bioprosthesis in this study demonstrated excellent safety and effectiveness for PVR. Further studies with longer follow-up are warranted.

Prognostic utility of a risk prediction model for predischarge major residual lesions or unplanned reinterventions following congenital mitral valve repair.

OBJECTIVE: We sought to develop a risk prediction model for predischarge major mitral valve (MV) residual lesions or unplanned MV reinterventions following congenital MV repair. METHODS: Patients who underwent congenital MV repair (excluding primary repair, but including secondary repair, of canal-type defects) at a single institution from January 2000 to December 2020 and survived to discharge were retrospectively reviewed. The primary outcome was major MV residua (mean gradient >6 mm Hg or moderate or greater regurgitation on the discharge echocardiogram) or predischarge unplanned MV reintervention. Risk factors of interest included age, single-ventricle physiology, preoperative and intraoperative postrepair MV stenosis and regurgitation severity, MV annular diameter z score, systemic ventricle ejection fraction, unfavorable anatomy, concomitant left-heart procedure, and various technique-related categories. Logistic regression was used to develop a weighted risk score for the primary outcome. Internal validation using bootstrap-resampling was performed. RESULTS: Of 866 patients who underwent congenital MV repair at a median age of 2.7 years (interquartile range, 0.7-9.1 years), 202 (23.3%) patients developed the primary outcome. The final risk prediction model had a C-statistic of 0.82 (95% confidence interval, 0.78-0.85). A weighted risk score was formulated per the variables in this model. The median risk score was 8 (interquartile range, 6-11) points. Patients were categorized as low (score 0-5), medium (score 6-10), high (score 11-15), or very high (score ≥16) risk. The probability of the primary outcome was 5.0 ± 1.7%, 15.2 ± 6.7%, 45.9 ± 12.6%, and 76.7 ± 8.8% for low-, medium-, high-, and very-high-risk patients, respectively. CONCLUSIONS: Our risk prediction model may guide prognostication of patients following congenital MV repair.

Impact of Residual Lesion Severity on Neurodevelopmental Outcomes Following Congenital Heart Surgery in Infancy and Childhood.

Children with congenital heart disease are at increased risk of neurodevelopmental delay throughout their lifespan. This risk is exacerbated following congenital heart surgery (CHS) in infancy. However, there are few modifiable risk factors for postoperative neurodevelopmental delay. In this study, we assessed the Residual Lesion Score (RLS), a quality assessment metric that evaluates residual lesion severity following CHS, as a predictor of neurodevelopmental delay. This was a single-center, retrospective review of patients who underwent CHS from 01/2011 to 03/2021 and post-discharge neurodevelopmental evaluation from 12 to 42 months of age using the Bayley Scales of Infant Development, 3rd Edition (BSID-III). RLS was assigned per published criteria: RLS 1, no residua; RLS 2, minor residua; and RLS 3, major residua or pre-discharge reintervention. Associations between RLS and BSID-III scores, as well as trends in neurodevelopmental outcomes over time, were evaluated. Of 517 patients with median age at neurodevelopmental testing of 20.0 (IQR 18.0-22.7) months, 304 (58.8%), 146 (28.2%), and 67 (13.0%) were RLS 1, 2, and 3, respectively. RLS 3 patients had significantly lower scaled scores in the cognitive, receptive, and expressive communication, and fine and gross motor domains, compared with RLS 1 patients. Multivariable models accounted for 21.5%-31.5% of the variation in the scaled scores, with RLS explaining 1.4-7.3% of the variation. In a subgroup analysis, RLS 3 patients demonstrated relatively fewer gains in cognitive, expressive communication, and gross motor scores over time (all p < 0.05). In conclusion, RLS 3 patients are at increased risk for neurodevelopmental delay, warranting closer follow-up and greater developmental support for cognitive, language, and motor skills soon after surgery.

Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants.

OBJECTIVE: We sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF). METHODS: A single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. RESULTS: Of 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045). CONCLUSIONS: Patients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up.

Natural History of Truncal Root Dilatation and Truncal Valve Regurgitation in Truncus Arteriosus.

BACKGROUND: The natural history of the dilated truncal root in repaired truncus arteriosus (TA) is incompletely understood. METHODS: A single-center review of patients who underwent TA repair between January 1984 and December 2018 was performed. Echocardiographically determined root diameters and derived z scores were measured at the annulus, sinus of Valsalva (SoV), and sinutubular junction (STJ) immediately before TA repair and throughout follow-up. Linear mixed-effects models assessed trends in root dimensions over time. RESULTS: Of 193 patients who underwent TA repair at a median age of 12 days (interquartile range, 6-48 days) and survived to discharge, 34 (17.6%), 110 (57.0%), and 49 (25.4%) patients had bicuspid, tricuspid, and quadricuspid truncal valves, respectively. Median postoperative follow-up was 11.6 years (interquartile range, 4.4-22.0 years; range, 0.1-34.8 years). Truncal valve or root intervention was required in 38 patients (19.7%). The mean rates of annular, SoV, and STJ growth were 0.7 ± 0.3 mm/y, 0.8 ± 0.5 mm/y, and 0.9 ± 0.4 mm/y, respectively. Root z scores remained stable with time. At baseline, compared with patients with tricuspid leaflet anatomy, bicuspid patients had larger diameters at the SoV (P = .003) and STJ (P = .029), whereas quadricuspid patients had larger STJ diameters (P = .004). Over time, the bicuspid and quadricuspid cohorts demonstrated comparatively greater annular dilatation (both P < .05). Patients with ≥75th percentile root growth rates had a higher incidence of moderate-severe truncal regurgitation (P = .019) and truncal valve intervention (P = .002). CONCLUSIONS: Root dilatation in TA persisted for up to 30 years after primary repair. Patients with bicuspid and quadricuspid truncal valves demonstrated greater root dilatation over time and required more valve interventions. Continued longitudinal follow-up is warranted in this higher-risk cohort.

Impact of Neighborhood Socioeconomic Status on Outcomes Following First-Stage Palliation of Single Ventricle Heart Disease.

Background The impact of neighborhood socioeconomic status (SES) on outcomes following first-stage palliation of single ventricle heart disease remains incompletely characterized. Methods and Results This was a single-center, retrospective review of consecutive patients who underwent the Norwood procedure from January 1, 1997 to November 11, 2017. Outcomes of interest included in-hospital (early) mortality or transplant, postoperative hospital length-of-stay, inpatient cost, and postdischarge (late) mortality or transplant. The primary exposure was neighborhood SES, assessed using a composite score derived from 6 US census-block group measures related to wealth, income, education, and occupation. Associations between SES and outcomes were assessed using logistic regression, generalized linear, or Cox proportional hazards models, adjusting for baseline patient-related risk factors. Of 478 patients, there were 62 (13.0%) early deaths or transplants. Among 416 transplant-free survivors at hospital discharge, median postoperative hospital length-of-stay and cost were 24 (interquartile range, 15-43) days and $295 000 (interquartile range, $193 000-$563 000), respectively. There were 97 (23.3%) late deaths or transplants. On multivariable analysis, patients in the lowest SES tertile had greater risk of early mortality or transplant (odds ratio [OR], 4.3 [95% CI, 2.0-9.4; P<0.001]), had longer hospitalizations (coefficient 0.4 [95% CI, 0.2-0.5; P<0.001]), incurred higher costs (coefficient 0.5 [95% CI, 0.3-0.7; P<0.001]), and had greater risk of late mortality or transplant (hazard ratio, 2.2 [95% CI, 1.3-3.7; P=0.004]), compared with those in the highest tertile. The risk of late mortality was partially attenuated with successful completion of home monitoring programs. Conclusions Lower neighborhood SES is associated with worse transplant-free survival following the Norwood operation. This risk persists throughout the first decade of life and may be mitigated with successful completion of interstage surveillance programs.

A Risk Prediction Model for Reintervention After Total Anomalous Pulmonary Venous Connection Repair.

BACKGROUND: Outcomes after total anomalous pulmonary venous connection (TAPVC) repair remain suboptimal due to recurrent pulmonary vein (PV) obstruction requiring reinterventions. We sought to develop a clinical prediction rule for PV reintervention after TAPVC repair. METHODS: Data from consecutive patients who underwent TAPVC repair at a single institution from January 1980 to January 2020 were retrospectively reviewed after Institutional Review Board approval. The primary outcome was postdischarge (late) unplanned PV surgical or transcatheter reintervention. Echocardiographic criteria were used to assess PV residual lesion severity at discharge (class 1: no residua; class 2: minor residua; class 3: major residua). Competing risk models were used to develop a weighted risk score for late reintervention. RESULTS: Of 437 patients who met entry criteria, there were 81 (18.5%) reinterventions at a median follow-up of 15.6 (interquartile range, 5.5-22.2) years. On univariable analysis, minor and major PV residua, age, single-ventricle physiology, infracardiac and mixed TAPVC, and preoperative obstruction were associated with late reintervention (all P < .05). The final risk prediction model included PV residua (class 2: subdistribution hazard ratio [SHR], 4.8; 95% CI, 2.8-8.1; P < .001; class 3: SHR, 6.4; 95% CI, 3.5-11.7; P < .001), age <1 year (SHR, 3.3; 95% CI, 1.3-8.5; P = .014), and preoperative obstruction (SHR, 1.8; 95% CI, 1.1-2.8; P = .015). A risk score comprising PV residua (class 2 or 3: 3 points), age (neonate or infant: 2 points), and obstruction (1 point) was formulated. Higher risk scores were significantly associated with worse freedom from reintervention (P < .001). CONCLUSIONS: A risk prediction model of late reintervention may guide prognostication of high-risk patients after TAPVC repair.

Intraoperative Technical Performance Score Predicts Outcomes After Congenital Cardiac Surgery.

BACKGROUND: The utility of the intraoperative technical performance score (IO-TPS) in predicting outcomes after congenital cardiac surgery remains unknown. METHODS: Data from patients undergoing surgery for congenital heart disease from January 2011 to December 2019 at a single institution were retrospectively reviewed. Intraoperative echocardiograms were used to assign IO-TPS for each index operation (class 1, no residua; class 2, minor residua; class 3, major residua). The primary outcome was a composite of in-hospital mortality, transplant, unplanned reintervention in the anatomic area of repair, and new permanent pacemaker implantation. Secondary outcomes included postdischarge (late) mortality or transplant and unplanned reintervention. Associations between IO-TPS and outcomes were assessed using logistic (primary) and Cox or competing risk (secondary) models, adjusting for preoperative patient- and procedure-related covariates. RESULTS: The primary outcome was observed in 784 (11.5%) of 6793 patients who met entry criteria. On multivariable analysis, IO-TPS was a significant predictor of the primary outcome (class 2: odds ratio, 1.7 [95% CI, 1.4-2.0; P < .001]; class 3: odds ratio, 6.0 [95% CI, 4.0-8.9; P < .001]). Among 6661 transplant-free survivors of hospital discharge observed for up to 10.5 years, there were 185 (2.8%) deaths or transplants and 1171 (17.6%) reinterventions. Class 3 patients had a greater adjusted risk of late mortality or transplant (hazard ratio, 2.2; 95% CI, 1.2-4.2; P = .012) and late reintervention (subdistribution hazard ratio, 2.5; 95% CI, 1.8-3.3; P < .001) vs class 1 patients. CONCLUSIONS: IO-TPS is significantly associated with adverse early and late outcomes after congenital heart surgery and may serve as an important adjunct for self-assessment and quality improvement.

Influence of intraoperative residual lesions and timing of extracorporeal membrane oxygenation on outcomes following first-stage palliation of single-ventricle heart disease.

BACKGROUND: Data regarding the influence of intraoperative residual lesions on extracorporeal membrane oxygenation (ECMO) following the Norwood procedure are limited. Moreover, the significance of postoperative ECMO timing on in-hospital outcomes remains incompletely characterized. METHODS: This was a single-center, retrospective review of consecutive patients who underwent the Norwood operation from January 1997 to November 2017. Patients with at least minor residual lesions based on the intraoperative postcardiopulmonary bypass echocardiogram were identified. The association between residual lesions and postoperative ECMO was assessed with logistic regression, adjusting for age, weight, prematurity, various preoperative system-specific and procedural risk factors, shunt type, and era. Among patients receiving ECMO, associations between late ECMO (≥3 days post-Norwood) and in-hospital mortality or transplant, postoperative hospital length-of-stay, and cost of hospitalization were evaluated using logistic regression or generalized linear models with a gamma distribution and logarithmic link. RESULTS: Among 500 patients, 78 (15.6%) received ECMO postoperatively. On multivariable analysis, the presence of at least minor residual lesions (odds ratio, 4.4; 95% CI, 2.1-9.3; P < .001) was associated with postoperative ECMO. In the ECMO subpopulation, there were 44 (56.4%) deaths or transplants. Late ECMO was associated with increased risk of in-hospital mortality or transplant (adjusted odds ratio, 6.2; 95% CI, 1.5-26.0), longer postoperative hospital length of stay (regression coefficient, 0.7; 95% CI, 0.3-1.1), and greater cost (regression coefficient, 0.6; 95%, CI 0.4-0.7), versus early ECMO (all P values < .05). CONCLUSIONS: The presence of even minor intraoperative residua significantly increases the risk of ECMO following the Norwood operation. Among patients receiving ECMO postoperatively, early institution of ECMO is associated with lower mortality and resource utilization.

Residual Lesion Severity Predicts Midterm Outcomes After Congenital Aortic Valve Repair.

BACKGROUND: We sought to validate the technical performance score (TPS) as a predictor of midterm outcomes after congenital aortic valve repair. METHODS: This was a single-center, retrospective review of consecutive patients who underwent aortic valve repair between January 1, 2011, and December 31, 2019. Predischarge echocardiograms were used to assign a TPS for each index operation as class 1, no aortic valve residua; class 2, minor aortic valve residua; or class 3, major aortic valve residua or predischarge reintervention for major residua. The primary outcome was postdischarge (late) unplanned aortic valve reintervention. Secondary outcomes included late mortality and at least moderate aortic regurgitation or stenosis at the latest follow-up or before the earliest reintervention. Associations between TPS and outcomes were assessed using competing risk, Cox proportional hazards, or logistic regression models, adjusting for preoperative patient- and procedure-related covariates. RESULTS: Of 507 patients, there were 110 (21.7%) reinterventions, 22 (4.3%) deaths, and 67 (13.2%) cases of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention. On multivariable analysis, class 3 patients had a greater risk of reintervention (subdistribution hazard ratio, 2.6; 95% CI, 1.3-5.1; P = .005) and mortality (hazard ratio, 5.3; 95% CI. 1.1-25.2; P = .038) compared with class 1 patients. Adjusting for duration of follow-up, class 3 patients also had a greater risk of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention (odds ratio, 7.7; 95% CI, 2.5-24.2; P < .001) vs class 1 patients. CONCLUSIONS: Patients with major residua after congenital aortic valve repair have significantly worse midterm outcomes compared with those with no residua, warranting closer follow-up.

Timing of reintervention influences survival and resource utilization following first-stage palliation of single ventricle heart disease.

OBJECTIVE: Outcomes after first-stage palliation of single-ventricle heart disease are influenced by many factors, including the presence of residual lesions requiring reintervention. However, there is a dearth of information regarding the optimal timing of reintervention. We assessed if earlier reintervention would be favorably associated with in-hospital outcomes among patients requiring unplanned reinterventions after the Norwood operation. METHODS: This was a single-center, retrospective review of all patients who underwent the Norwood procedure from January 1997 to November 2017 and required a predischarge unplanned surgical or transcatheter reintervention on 1 or more subcomponent areas repaired at the index operation. Outcomes of interest included in-hospital mortality or transplant, postoperative hospital length of stay, and inpatient cost. Associations between timing of reintervention and outcomes were assessed using logistic regression (mortality or transplant) or generalized linear models (postoperative hospital length of stay and cost), adjusting for baseline patient-related and procedural factors. RESULTS: Of 500 patients who underwent the Norwood operation, 92 (18.4%) required an unplanned reintervention. Median time to reintervention was 12 days (interquartile range, 5-35 days). There were 31 (33.7%) deaths or transplants, median postoperative hospital length of stay was 49 days (interquartile range, 32-87 days), and median cost was $328,000 (interquartile range, $204,000-$464,000). On multivariable analysis, each 5-day increase in time to reintervention increased the odds of mortality or transplant by 20% (odds ratio, 1.2; 95% confidence interval, 1.1-1.3; P = .004). Longer time to reintervention was also significantly associated with greater postoperative hospital length of stay (P < .001) and higher cost (P < .001). CONCLUSIONS: For patients requiring predischarge unplanned reinterventions after the Norwood operation, earlier reintervention is associated with improved in-hospital transplant-free survival and resource use.

Outcomes of Repair of Congenital Aortic Valve Lesions Using Autologous Pericardium vs Porcine Intestinal Submucosa.

BACKGROUND: Outcomes following congenital aortic valve (AoV) repair are plagued by progressive dysfunction of currently available leaflet substitute materials. OBJECTIVES: We compared the long-term outcomes of congenital AoV repair using porcine intestinal submucosa vs autologous pericardium (AP). METHODS: This was a single-center retrospective review of all patients who underwent congenital AoV repair with either porcine intestinal submucosa or AP from October 2009 to March 2013. The primary outcome was postdischarge (late) unplanned AoV reintervention. Secondary outcomes included number of late AoV reinterventions and a composite of at least moderate aortic regurgitation or stenosis at latest follow-up or before the first reintervention. Associations between leaflet repair material and outcomes were assessed using multivariable regression models, adjusting for prespecified patient-related and operative variables. RESULTS: Of 26 porcine intestinal submucosa and 49 AP patients who met entry criteria, the median age was 11.0 years (IQR: 4.7-16.6 years). At a median follow-up of 8.5 years (IQR: 4.4-9.6 years), 17 (65.4%) porcine intestinal submucosa and 22 (44.9%) AP patients underwent at least 1 AoV reintervention. On multivariable analysis, porcine intestinal submucosa use was significantly associated with unplanned AoV reintervention (HR: 4.6; 95% CI: 2.2-9.8; P < 0.001), number of postdischarge AoV reinterventions (incidence rate ratio: 1.7; 95% CI: 1.0-2.9; P = 0.037), and at least moderate aortic regurgitation or stenosis at latest follow-up or before the first reintervention (OR: 5.0; 95% CI: 1.2-21.0; P = 0.027). CONCLUSIONS: Aortic valvuloplasty with porcine intestinal submucosa is associated with earlier time to reintervention compared with autologous pericardium. The search for the ideal AoV leaflet repair material continues.