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Background and Objectives: During the last decades, the survival rates in patients with congenital heart disease have increased dramatically, particularly in patients with complex heart malformations. However, the survival in patients with simple defects is still unknown. We aimed to determine the characteristics and the risk of mortality in patients with isolated pulmonary valve stenosis (PS)., Methods: Swedish inpatient, outpatient and cause of death registries were used to identify patients born between 1970 and 2017 with a diagnosis of PS, without any other concomitant congenital heart lesion. For each patient with PS, 10 control individuals without congenital heart disease were matched by birth year and sex from the total population registry. We used median-unbiased method and Kaplan-Meier survival analysis to examine the risk of mortality., Results: We included 3910 patients with PS and 38 770 matched controls. The median age of diagnosis of PS was 0.7 years (IQR 0.3-7.0). During a median follow-up of 13.5 years (IQR 6.5-23.5), 88 patients with PS and 192 controls died; 500 patients with PS (12%) underwent at least one transcatheter or surgical valve intervention. The overall mortality rate was significantly higher in patients with PS compared with matched controls (HR 4.67, 95% CI 3.61 to 5.99, p=0.001). Patients with an early diagnosis of PS (0-1 year) had the highest risk of mortality (HR 10.99, 95% CI 7.84 to 15.45)., Conclusions: In this nationwide, register-based cohort study, we found that the risk of mortality in patients with PS is almost five times higher compared with matched controls. Patients with an early diagnosis of PS appears to be the most vulnerable group and the regular follow-up in tertiary congenital heart units may be the key to prevention., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Importance: The choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction., Objective: To compare 30-year outcomes of TOF repair after a VS procedure vs TAP., Design, Setting, and Participants: This retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021., Exposures: The study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis., Main Outcomes and Measures: The primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions., Results: Among 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P < .001) and patients who underwent a VS procedure with at least moderate residual stenosis (1.3 interventions [95% CI, 0.9-1.9 interventions]; MR, 0.65 [0.45-0.93]; P = .02). Results were similar for PVR, with a 30-year cumulative mean 0.3 PVRs [95% CI, 0.1-0.7 PVRs] for patients who underwent a VS procedure without residual pulmonary stenosis (MR, 0.22 [95% CI, 0.12-0.43]; P < .001) and 0.6 PVRs (95% CI, 0.2-1.5 PVRs) for patients with at least moderate residual stenosis (MR, 0.44 [95% CI, 0.21-0.93]; P = .03), compared with 1.4 PVRs (95% CI, 0.8-2.5 PVRs) for the TAP group. No statistically significant difference was found for cardiovascular hospitalizations., Conclusions and Relevance: This study found that patients who underwent a VS procedure had increased 30-year survival, fewer cardiovascular reinterventions, and fewer PVRs compared with individuals who underwent TAP, even in the presence of significant residual pulmonary stenosis. These findings suggest that it is beneficial to perform a VS procedure when possible, even in the presence of moderate residual stenosis, compared with the insertion of a TAP.

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.

Objective: To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease., Study Design: Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs., Results: Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation., Conclusions: Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: The long-term durability of the 2 most commonly used types of bioprosthetic valves in the pulmonic position, the porcine and pericardial valves, is unclear. We compared the long-term durability of the pericardial (Carpentier-Edwards PERIMOUNT [CE]) and porcine (Hancock II) valves in the pulmonic position in patients with congenital cardiac anomalies., Methods: We retrospectively reviewed the medical records of 258 cases (248 patients) of pulmonary valve implantation or replacement using CE (129 cases, group CE) or porcine (129 cases, group H) valves from 2 institutions between 2001 and 2009., Results: The patients' age at pulmonary valve implantation was 14.9 ± 8.7 years. No significant differences in perioperative characteristics were observed between groups CE and H. Follow-up data were complete in 219 cases (84.9%) and the median follow-up duration was 10.5 (interquartile range, 8.4∼13.0) years. Ten mortalities (3.9%) occurred. Sixty-four patients underwent reoperation for pulmonary valve replacement due to prosthetic valve failure; 10 of these 64 patients underwent reoperation during the study period. Patients in group CE were significantly more likely to undergo reoperation (hazard ratio, 2.17; confidence interval, 1.26-3.72; P = .005) than patients in group H. Patients in group CE showed had a greater prosthetic valve dysfunction (moderate-to-severe pulmonary regurgitation or pulmonary stenosis with ≥3.5 m/s peak velocity through the prosthetic pulmonary valve) rate (hazard ratio, 1.83; confidence interval, 1.07-3.14; P = .027) than patients in group H., Conclusions: Compared with the pericardial valve, the porcine valve had long-term advantages in terms of reduced reoperation rate and prosthetic valve dysfunction in the pulmonic position in patients with congenital cardiac anomalies., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Only few studies have reported long-term outcome of the transatrial-transpulmonary approach in the current era of management of tetralogy of Fallot (ToF). We investigated 15-year outcome of correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated in the 21st century. All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.1 (interquartile range 3.0-10.9) years. Data regarding postoperative complications, reinterventions, development of atrial and ventricular arrhythmia, cardiac function, and survival were evaluated. Prior shunting was performed in 10 patients (6%). The transatrial-transpulmonary approach resulted in valve-sparing surgery in 57 patients (32%). Postoperative surgical complications included junctional ectopic tachycardia (N = 12, 7%), pericardial (N = 10, 6%) or pleural effusion (N = 7, 3%), chylothorax (N = 7, 4%), bleeding requiring reoperation (N = 4, 3%), and superficial wound infection (N = 1). Fifty-one patients underwent 68 reinterventions, mainly due to pulmonary restenosis (PS) (N = 57). ToF correction at age <2 months and double outlet or double-chambered right ventricle variants of the ToF spectrum were independent predictors for reintervention. Patients undergoing valve-sparing ToF correction had a significant longer PR-free survival than those with a transannular patch (8.5 [95% confidence interval 6.8-10.3] years vs 1.1 [95% confidence interval 0.8-1.5] years; P < 0.001). Overall mortality was 2.8%; mortality rates were higher in premature/dysmature newborns (0.7% vs 9.5%; P < 0.001). Although the 15-year outcome of the transatrial-transpulmonary approach in terms of postoperative complications and mortality rates is excellent, the high incidence of moderate and severe PR is worrisome. Valve-sparing surgery was associated with a substantially lower incidence of PR, yet was surgically not possible in the majority of patients., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to report long-term outcomes after percutaneous pulmonary valve implantation (PPVI)., Background: Excellent short- and mid-term results after PPVI for right ventricular outflow tract (RVOT) dysfunction have been reported. Data on long-term results after PPVI are scarce., Methods: All 226 patients treated with PPVI at a single institution were prospectively enrolled in a database and included in the study. Follow-up information regarding outcomes was collected. Risk-factor analysis for death and reinterventions was performed., Results: Follow-up data (up to 11 years) were available in 96% of the patients. At the end of the study period, 219 patients (92.8%) still lived with the initially implanted valve. Seven patients died, 2 of them from procedure-related complications. Seventeen patients (7.2%) needed valve replacement surgically (n = 11) or percutaneously (n = 6) for infectious endocarditis (n = 10) or valve degeneration (n = 7). In the remaining patients, the valves retained excellent function, and right ventricular dimensions and exercise capacity improved. A post-interventional RVOT gradient >15 mm Hg was a risk factor for death (hazard ratio: 7.57; 95% confidence interval: 1.26 to 45.38; p = 0.027) and for valve failure (hazard ratio: 3.76; 95% confidence interval: 1.43 to 9.93; p = 0.007). The best outcome was achieved with RVOT pre-stenting and a post-interventional gradient <15 mm Hg, resulting in an estimated event-free survival rate of 88% at 10 years., Conclusions: Patients after PPVI have excellent long-term outcomes. Right ventricular volumes, function, and exercise capacity improve significantly. A residual RVOT gradient <15 mm Hg was associated with the best outcome., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: Carcinoid heart disease is characterized by tricuspid valve regurgitation and varying degrees of pulmonary valve regurgitation or stenosis. Valve replacement procedures may be complicated by systemic effects of carcinoid syndrome, as well as hepatic dysfunction and right heart failure. This study was performed to identify factors that might be associated with improving early mortality rates and late outcomes., Methods: Between November 1985 and January 2018, 240 adult patients underwent surgery for carcinoid heart disease at the Mayo Clinic. We analyzed the association of multiple clinical and echocardiographic variables on early mortality and late survival., Results: The median (interquartile range) age of patients was 63 years (55-69), and 117 patients (49%) were male. Before operation, 157 patients (70%) had New York Heart Association class III or IV limitation. Somatostatin analogs were used in 221 patients (92%), and long-acting somatostatins were used in 130 patients (54%). Loop diuretic therapy was used preoperatively in 125 patients (52%). Early mortality rate was 29% (9/22) between 1985 and 1994, but decreased to 7% (6/81) during 1995 to 2004, and to 5% (7/128) from 2005 onward. Overall survival estimates at 1, 3, and 5 years were 69%, 48%, and 34%, respectively. Older age, advanced New York Heart Association class, and a nonlinear effect of creatinine were independently associated with overall mortality., Conclusions: Valve replacement for carcinoid heart disease has acceptable short-term mortality, and early risk has decreased in the current era. Earlier intervention may improve overall survival., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

A subset of neonates with single ventricle (SV) physiology has antegrade pulmonary blood flow that is deemed unlikely to be reliable until Glenn. We have used systemic to pulmonary shunt (SPS) with pulmonary artery banding (PAB) to optimize pulmonary blood flow while maintaining reserve antegrade flow. We hypothesize that this is an effective strategy that can be accomplished without the routine need for cardiopulmonary bypass. We retrospectively reviewed the records of 60 neonates who underwent combined SPS + PAB between 2004 and 2015. Data are presented as median with quartiles. Children were 8 (4-19) days old at surgery and included 38 (63%) boys. Atresia or severe stenosis of the subpulmonary atrioventricular (AV) valve associated with pulmonary blood flow across a bulboventricular foramen was present in 37 (62%). In 20 (33%), heterotaxy-associated unbalanced AV canal with pulmonary stenosis with or without anomalous pulmonary venous drainage was present. First-stage palliation was accomplished without cardiopulmonary bypass in 44 patients (73%). There were 7 (12%) hospital deaths, 4 among the 20 (20%) with heterotaxy. Fifty-three children were followed for a median 5.1 (1.8-8.2) years. Three early reinterventions were required after initial palliation (1 PAB adjustment, 2 SPS balloon angioplasties). Five additional heterotaxy patients experienced late mortality during follow-up. There were no early or emergent Glenn. Thirty-nine patients have reached Fontan circulation with a median pre-Fontan PA pressure of 14 (12-18) mm Hg. One patient converted to biventricular physiology and the remaining await completion Fontan. Heterotaxy was the only independent predictor of mortality (hazard ratio 10 (2.3-44, P < 0.001). In SV patients with unreliable antegrade PA flow, SPS + PAB is an effective first-stage palliation. SV patients with heterotaxy are at increased risk for mortality., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: The objectives of this study were to characterize the epidemiological, clinical, and echocardiographic features of French bulldogs (FBs) with congenital pulmonic stenosis and document their survival times and risk factors for cardiac death (CD)., Animals: This study included 66 FBs with congenital pulmonic stenosis., Methods: Prospective cohort study including a survival analysis to assess time to CD., Results: In most cases (53/66, 80%), at least two obstructive lesions were observed, most commonly valvular and supravalvular (42/66, 64%), with pulmonary trunk hypoplasia in 40/66 (61%) of cases. The median Doppler-derived peak trans-stenotic pressure gradient (ΔP) was very high: 170 mmHg (range = 34-291 mmHg), with ΔP ≥ 200 mmHg in 33% of FBs. Among the 51 FBs with an available follow-up and that did not undergo surgical valvuloplasty, 21/51 (41%) died, 67% (14/21) of deaths being CD. The median survival time from diagnosis to CD was 2.8 years (interquartile range = 0.8-4.6 years). Univariate Cox proportional hazard analyses revealed that age (hazard ratio [HR] = 2.3 per 1 year increase; p = 0.02), clinical signs at presentation (HR = 3.7; p = 0.03), ΔP (HR = 1.2 per 10 mmHg increase; p = 0.01), right ventricular dilation (HR = 5.0; p = 0.04), severe tricuspid regurgitation (HR = 7.6; p = 0.001), and right-sided congestive heart failure (HR = 4.8; p = 0.05) were associated with time to CD. After adjustment for age and ΔP, tricuspid regurgitation remained significantly associated with time to CD (HR = 5.1; p = 0.02)., Conclusions: Pulmonic stenosis in FBs is commonly severe and complex, with at least 2 obstructive lesions in most cases, a high incidence of pulmonary trunk hypoplasia and CD, and strong association between prognosis and tricuspid regurgitation severity., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Risk factors for adverse clinical outcomes have been identified in patients with repaired tetralogy of Fallot before pulmonary valve replacement (PVR). However, pre-PVR predictors for post-PVR sustained ventricular tachycardia and death have not been identified., Methods: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort (International Multicenter TOF Registry), a 4-center international cohort study, who had a comprehensive preoperative evaluation and subsequently underwent PVR were included. Preprocedural clinical, ECG, cardiovascular magnetic resonance, and postoperative outcome data were analyzed. Cox proportional hazards multivariable regression analysis was used to evaluate factors associated with time from pre-PVR cardiovascular magnetic resonance until the primary outcome: death, aborted sudden cardiac death, or sustained ventricular tachycardia., Results: Of the 452 eligible patients (median age at PVR, 25.8 years), 36 (8%) reached the primary outcome (27 deaths, 2 resuscitated death, and 7 sustained ventricular tachycardia) at a median time after PVR of 6.5 years. Cox proportional hazards regression identified pre-PVR right ventricular ejection fraction <40% (hazard ratio, 2.39; 95% CI, 1.18-4.85; P=0.02), right ventricular mass-to-volume ratio ≥0.45 g/mL (hazard ratio, 4.08; 95% CI, 1.57-10.6; P=0.004), and age at PVR ≥28 years (hazard ratio, 3.10; 95% CI, 1.42-6.78; P=0.005) as outcome predictors. In a subgroup analysis of 230 patients with Doppler data, predicted right ventricular systolic pressure ≥40 mm Hg was associated with the primary outcome (hazard ratio, 3.42; 95% CI, 1.09-10.7; P=0.04). Preoperative predictors of a composite secondary outcome, postoperative arrhythmias and heart failure, included older age at PVR, pre-PVR atrial tachyarrhythmias, and a higher left ventricular end-systolic volume index., Conclusions: In this observational investigation of patients with repaired tetralogy of Fallot, an older age at PVR and pre-PVR right ventricular hypertrophy and dysfunction were predictive of a shorter time to postoperative death and sustained ventricular tachycardia. These findings may inform the timing of PVR if confirmed by prospective clinical trials.

Objectives: The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position., Background: The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI)., Methods: From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included., Results: The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p < 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%)., Conclusions: Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: RASopathies are developmental disease caused by mutations in genes encoding for signal transducers of the RAS-MAPK cascade. The aim of the present study was to provide a comprehensive description of morbidity and mortality in patients with molecularly confirmed RASopathy., Methods: A multicentric, observational, retrospective study was conducted in seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET). Clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed. Mortality was described as crude mortality, cumulative survival and restricted estimated mean survival. Multivariable regression analysis was used to assess the impact of mutated genes on number of interventions and overall prognosis., Results: Cardiac defects occurred in 80.3% of cases, almost half of them underwent at least one intervention. Overall, crude mortality was 0.29/100 patients-year. Cumulative survival was 98.8%, 98.2%, 97.7%, 94.3%, at 1, 5, 10, and 20years, respectively. Restricted estimated mean survival at 20years follow-up was 19.6years. Ten patients died (2.7% of the entire cohort; 3.4% of patients with cardiac defect). Patients with hypertrophic cardiomyopathy (HCM) and age <2years or young adults, as well as subjects with biventricular obstruction and PTPN11 mutations had a higher risk of cardiac death., Conclusions: The risk of intervention was higher in individuals with Noonan syndrome and pulmonary stenosis carrying PTPN11 mutations. Overall, mortality was relatively low, even though the specific association between HCM, biventricular outflow tract obstructions and PTPN11 mutations appeared to be associated with early mortality, including immediate post-operative events and sudden death., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives: The aim of this study was to evaluate survival following catheter intervention in pediatric patients with pulmonary vein stenosis (PVS)., Background: Despite aggressive surgical and catheter intervention on PVS in children, recurrence and progression of stenosis can lead to right heart failure and death. Clinicians continue to seek effective treatment options for PVS., Methods: A single-center, retrospective study was performed including all patients <18 years of age who underwent catheter intervention (balloon angioplasty and bare-metal stent and drug-eluting stent insertion) on PVS. Endpoints included death, vein loss, and rate of reintervention., Results: Thirty patients underwent intervention (balloon angioplasty, n = 9; bare-metal stent, n = 5; drug-eluting stent, n = 16) at a median age of 6.4 months (4.3 to 9.9 months). Median follow-up duration was 30.6 months (77 days to 10.5 years). Fourteen patients (47%) died at a median of 2.0 months (0.4 to 3.2 months) following intervention. There was no association between DES placement and survival (p = 0.067). Reintervention (catheter or surgical) was associated with improved survival (p = 0.001), with a 1-year survival rate of 84% compared with 25% for no reintervention. Vein loss occurred in 34 of 58 (59%) veins at a median of 3.3 months (1.0 to 5.0 months). One-year vein survival was higher with DES implantation (p = 0.031) and with reintervention (p < 0.001)., Conclusions: DES implantation at first catheter intervention appears to be associated with improved vein survival but may not result in improved patient survival. However, reintervention appears to be associated with improved patient survival and vein patency, suggesting that despite mode of treatment, frequent surveillance is important in the care of these patients., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS)., Methods: Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years., Results: Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (<30 days), survival was 94% at 40 years. Of 46 eligible survivors, 29 participated in the in-hospital examination and 15 gave permission to use their hospital records (96% participation). Cumulative event-free survival was 68% after 40 years: 25% needed a reintervention, 12% underwent pacemaker implantation and 9% had supraventricular arrhythmias. Early reinterventions were mainly for residual PS, late reinterventions for pulmonary regurgitation. Subjective health status was good. Exercise capacity was normal in 74% (median 96 (82-107)% of expected workload). Right ventricular and left ventricular (LV) dysfunction was found in 13% and 41%, respectively. The use of a transannular patch and younger age at surgery were predictive for late events (HR 3.02 (95% CI 1.09 to 8.37) and HR 0.81/year (95% CI 0.66 to 0.98), respectively). Use of inflow occlusion compared with cardiopulmonary bypass showed a trend towards more reinterventions (HR 3.19 (95% CI 0.97 to 10.47))., Conclusions: Survival up to 40 years after successful PS repair is nearly normal. Subjective health status is good and there is a low incidence of arrhythmias. Reinterventions, however, are necessary in one-quarter and 40 years postoperatively several patients show LV dysfunction., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Aims: The pathophysiology of branch pulmonary artery (PA) stenosis after the arterial switch operation, most commonly on the left, is incompletely understood. This study examines factors associated with left PA (LPA) obstruction., Methods and Results: Cardiac magnetic resonance (CMR) imaging studies performed in patients after arterial switch operation (ASO) were retrospectively analysed. Blood flow was measured in both branch PAs and neo-pulmonary root position in relation to the aorta was expressed as an angle, relative to a line connecting the sternum and the spine. Sixty-six patients were included for analysis. Seventy per cent (n = 46) had balanced pulmonary blood flow, 28% (n = 18) had decreased flow to the left, and 2% (n = 2) had decreased flow to the right lung. LPA area indexed to body surface area (BSA) was smaller than RPA area (62 ± 37 vs. 120 ± 64 mm 2 /m 2 , P < 0.0001). Patients with reduced LPA flow were more likely to have required pulmonary arterioplasty at the time of ASO (17 vs. 2%, P = 0.04) and had a larger aortic root diameter (25 ± 7 vs. 22 ± 5 mm 2 /m 2 , P = 0.01). Greater rightward orientation of the neo-pulmonary root correlated inversely with LPA cross-sectional area (r = -0.39, P = 0.001) but not with LPA flow. Aortic root diameter correlated inversely with LPA flow (r = -0.43, P = 0.0004) but not with LPA cross-sectional area (P = 0.32). Patients with a rightward neo-pulmonary root and/or a dilated aortic root in the upper quartile range had a smaller LPA area (53 vs. 73 mm 2 /m 2 , P = 0.04) and less pulmonary blood flow (41 vs. 46%, P = 0.02) compared with patients without those risk factors., Conclusions: Neo-pulmonary to neo-aortic geometry as well as post-operative compression of the LPA by an enlarged aorta impact LPA size and perfusion of the left lung., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.)

Objective: To evaluate the rate of pulmonary stenosis and functional pulmonary atresia (PS/PA) in recipient twins prior to fetal surgery for twin-twin transfusion syndrome (TTTS) and their pre- and postnatal outcomes., Methods: We carried out a prospective study including 260 cases of TTTS. Echocardiography was performed before laser surgery to detect the presence of PS/PA. The outcomes of recipients with and without PS/PA were compared. The need of postnatal cardiac interventions and the survival rate at 6 months of age were also evaluated., Results: PS was observed in 16/260 (6.2%) of recipient twins and PA in 12/260 (4.6%). After fetal surgery, 10/28 (35.7%) recipients died, 9/28 (32.1%) showed in utero regression, and 9/28 (32.1%) had persistence of PS/PA. Postnatally, seven recipients underwent percutaneous balloon pulmonary valvuloplasty, one required surgical valvotomy and one palliative surgery. Pregnancies with recipient twins with PS/PA had lower survival of at least one twin (67.9 vs. 83.6%, p = 0.045) and lower overall survival (57.1 vs. 72.8%, p = 0.015) at 6 months of age., Conclusion: PS and PA were observed in 10.8% of recipients. Among these, about one third showed persistence of pulmonary valve pathology after delivery, which stresses the need for strict follow-up., (© 2016 S. Karger AG, Basel.)

Background: Surgical correction was the treatment of choice for pulmonary stenosis until three decades ago, when balloon valvuloplasty was implemented. The natural history of surgically relieved pulmonary stenosis has been considered benign but is actually unknown, as is the need for re-intervention. The objective of this study was to investigate the morbidity and mortality of patients with surgically treated pulmonary stenosis operated at Aarhus University Hospital between 1957 and 2000., Results: The total study population included 80 patients. In-hospital mortality was 2/80 (2.5%), and an additional four patients died after hospital discharge; therefore, the long-term mortality was 5%. The maximum follow-up period was 57 years, with a median of 33 years. In all, 16 patients (20%) required at least one re-intervention. Pulmonary valve replacement due to pulmonary regurgitation was the most common re-intervention (67%). Freedom from re-intervention decreased >20 years after the initial repair. In addition, 45% of patients had moderate/severe pulmonary regurgitation, 38% had some degree of right ventricular dilatation, and 40% had some degree of tricuspid regurgitation, which did not require re-intervention at the present stage., Conclusion: Surgical relief for pulmonary stenosis is efficient in relieving outflow obstruction; however, this efficiency is achieved at the cost of pulmonary regurgitation, leading to right ventricular dilatation and tricuspid regurgitation. When required, pulmonary valve replacement is performed most frequently >20 years after the initial surgery. Lifelong follow-up of patients treated surgically for pulmonary stenosis is emphasised in this group of patients, who might otherwise consider themselves cured.

Objective: Patients with unoperated single ventricle (SV) rarely survive into adulthood with good functional status and may develop Eisenmenger's syndrome (ES). We report outcomes of a 30-year cohort of such patients., Methods: Adult patients with unoperated SV were identified by searching the Mayo Clinic medical record from 1984 to 2014. Clinical data were collected and compared between patients with pulmonary stenosis (PS) and ES., Results: 24 patients were identified (median peak-age 56 (31-77) years (11 ES, 13 PS); 22 had left ventricular morphology. 50-year transplant-free survival was 65% (95% CI 43 to 81). Median age at death was 55 years (31-77 years); 15 deaths (62%) occurred before oral pulmonary vasodilators were commercially available. Two-thirds of the cohort demonstrated preserved New York Heart Association functional class and median EF was 60% (49% to 62%). The majority of patients to survive into the fifth decade exhibited anatomy of double-inlet LV (DILV) with PS., Conclusion: Selected patients with unoperated SV with PS and ES can survive with good functional class up to the eighth decade with good medical management. DILV/PS appears to be the ideal phenotype for advanced survival. Our outcomes may be considered when such patients with SV having 'balanced' physiology are evaluated for Fontan palliation. However, additional prospective study will be necessary to verify this assertion., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Background: Stentless porcine roots (SPV) have been proposed for right ventricular outflow tract reconstruction in the Ross procedure due to the relative availability of pulmonary homografts in large diameters. We report here our experience with SPV used in the Ross procedure., Methods: Between March 1992 and February 2011, 360 patients had a Ross procedure; 61 patients received a SPV in pulmonary position and they represent the study population. Mean age was 38 ± 7.6 years. Indication for surgery was an infective endocarditis in 15 cases, there were 3 redo operations. Median SPV diameter was 29 mm (range, 25 to 29 mm). Pulmonary stenosis was defined as a peak transvalvular gradient of more than 50 mm Hg., Results: Perioperative mortality was 4.9% (3 patients) and late mortality was 3.3% (2 patients). Median follow-up was 4 years (range, 7 days to 14.9 years). There was no reoperation on the right ventricle outflow tract, and freedom from pulmonary stenosis was 100% at 5 years. Mean transpulmonary gradients were 7.1 ± 3.1 mm Hg and 13.5 ± 6.8 mm Hg postoperatively and at 5 years, respectively. Mean transpulmonary gradient increased faster over time when the SPV diameter was less than 29 mm (p = 0.03)., Conclusions: The SPV could represent an alternative to cryopreserved pulmonary homografts during the Ross procedure in adult patients. Hemodynamic results were improved by using large diameter SPV, but longer follow-up is mandatory to confirm those results., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Percutaneous implantation of pulmonary valves has recently been introduced into clinical practice., Aim: To analyse data of patients treated in France between April 2008 and December 2010., Methods: Prospective, observational, multi-centric survey by means of a database registry of the Filiale de cardiologie pédiatrique et congénitale., Results: Sixty-four patients were included, with a median (range) age of 21.4 (10.5-77.3) years. The majority (60.9%) of the patients were New York Heart Association (NYHA) class II. The most common congenital heart disease was tetralogy of Fallot with or without pulmonary atresia (50%). Indication for valve implantation was stenosis in 21.9%, regurgitation in 10.9% and association of stenosis and regurgitation in 67.2%. Implantation was successful in all patients. Pre-stenting was performed in 96.9% of cases. Median (range) procedure time was 92.5 (25-250) minutes. No significant regurgitation was recorded after the procedure, and the trans-pulmonary gradient was significantly reduced. Early minor complications occurred in five cases (7.8%). Three patients died during a median follow-up of 4.6 (0.2-5.2) years, two from infectious endocarditis and one from end-stage cardiac failure. Surgical reintervention was required in three patients. Follow-up with magnetic resonance imaging demonstrated significant improvements in right ventricular volumes and pulmonary regurgitation in mixed and regurgitant lesions., Conclusions: Transcatheter pulmonary valve implantation is highly feasible and mid-term follow-up demonstrates sustained improvement of right ventricular function. Late endocarditis is of concern, therefore longer follow-up in more patients is urgently needed to better assess long-term outcome., Clinical Trial Registration: NCT01250327., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objectives: To overcome the drawbacks of the Rastelli operation, the modified Lecompte procedure creates a connection from the left ventricle to the aorta with greater freedom from residual obstruction due to the resection of the outlet septum and avoids the implantation of an extracardiac valved conduit. We evaluated the effectiveness of this technique with analysis of our 25-year long-term results., Methods: We reviewed the records of 50 patients who underwent the modified Lecompte procedure during the past 25 years. The median age at operation was 1.95 years (range, 0.30-12.48 years). The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double outlet right ventricle, and double outlet left ventricle., Results: There were 2 early deaths (4.0%). During a mean follow-up of 14.2 ± 7.9 years (range, 0.2-25 years), there were 4 late deaths. Actuarial survival was 87.3% ± 4.9% at 25 years. The freedom from arrhythmia and reoperation at 25 years was 87.7% ± 6.2% and 25.2% ± 9.4%, respectively. The freedom from reoperation for left ventricular outflow tract obstruction and right ventricular outflow tract obstruction at 25 years was 88.5% ± 5.4% and 49.6% ± 9.0%, respectively. At last follow-up, 43 survivors (97.7%) are in New York Heart Association class I., Conclusions: The modified Lecompte procedure has excellent long-term results for treating anomalies of ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction. Early repair is possible with low mortality and morbidity in terms of arrhythmia, reoperation for right or left ventricular outflow tract obstruction, and functional class., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: This article reports outcomes of percutaneous balloon dilation in patients with severe pulmonary valve stenosis, in particular in those treated late with cyanosis, congestive heart failure, and pericardial effusion., Background: Percutaneous balloon dilation is the treatment of choice for pulmonary valve stenosis. Although earlier intervention may produce better results, patients may present late with congestive heart failure and cyanosis., Methods: Fifty-five patients who underwent pulmonary valve balloon dilation, were grouped into two groups, based on the presence or absence of congestive right heart failure and/or central cyanosis. Group I included 33 patients with severe pulmonary valve stenosis, but without clinical evidence of congestive right heart failure in the form of liver enlargement, raised jugular venous pressure, and peripheral edema and/or central cyanosis and group II included 22 patients with severe pulmonary valve stenosis and congestive right heart failure and/or central cyanosis. Their outcomes were compared., Results: Doppler measured transvalvar pressure gradient decreased from 110.2 ± 34.0 mm Hg before to 52.5 ± 28.7 mm Hg in group I after dilation (P < 0.001), and from 138.4 ± 32.3 mm Hg to 53.9 ± 19.3 mm Hg in group II, (P < 0.001). Complications included ventricular tachycardia/fibrillation in three patients and severe bradycardia in one patient in group II. Twelve patients in group II developed clinical and radiologic evidence of reperfusion injury/pulmonary edema within the first 24 hr of intervention and needed ventilation for 2-9 days. Three of these patients died from intractable pulmonary edema. On follow up, clinical and echocardiographic improvement parameters were similar in the two groups., Conclusion: Those patients with severe pulmonary valve stenosis with congestive right heart failure, especially those with pericardial effusion, ascites and cyanosis, represent an important technical and clinical challenge. They are a high-risk group with or without treatment. If they survive the procedure, they may still remain at a high risk in the first few days afterward. Maintaining their ventilator and inotropic support after balloon dilation may increase survival. However, excellent results can be obtained., (© 2013 Wiley Periodicals, Inc.)

Objective: Noonan syndrome (NS) is the second most common genetic syndrome associated with cardiac abnormalities, including, most notably, pulmonary stenosis (PS) and hypertrophic cardiomyopathy (HCM). Little is known about the natural history of heart disease in this unique subset of patients. We sought to contribute information on the natural history of NS by looking at how the cardiac disease progresses with time., Design: This is a retrospective review of the medical records of patients with NS seen at our institution between 1963 and 2011., Results: Records were available for 113 patients. Average length of follow-up was 14.16 years (2 months to 44 years, median 12.5 years). Sixty-six percent (75/113) of our patients had PS; within this subset, 57% (43) were classified as mild, 9% (7) moderate, and 33% (25) severe. None of the cases of mild PS worsened with time. All of the severe cases had an intervention, as did some moderate cases. Fourteen percent (16/113) of our patients had HCM; 56% (9/16) were mild, diagnosed at an average age of 3.8 years. Seven of these were stable with time, while one did progress. Forty-four percent (7/16) of cases were classified as severe, diagnosed at an average age of 4.2 months, and all were managed medically, surgically, or both. Our cohort had seven deaths (ages 6 months and 6, 10, 20, 40, 49, and 50 years)., Conclusion: Mild PS in patients with NS is nonprogressive. Severe, and in some cases moderate, PS will invariably require a therapeutic intervention. It is uncommon for HCM to progress or have new onset beyond early childhood. Prognosis of heart disease in NS is influenced most by the findings on presentation., (© 2013 Wiley Periodicals, Inc.)

Objectives: To assess survival and risk factors in dogs with pulmonic stenosis., Methods: A retrospective review of medical case records of all cases of pulmonic stenosis >50 mmHg, undergoing pulmonary balloon valvuloplasty or not. Survival curves and multivariate analysis were calculated in the overall population and in subgroups., Results: One hundred and seventy-two cases were included. Factors negatively affecting survival were clinical signs [hazard ratio (HR) 3 · 44, P < 0 · 001], younger age at diagnosis (HR 3 · 96, P = 0 · 001) and valve morphology type B (HR 3 · 33, P = 0 · 001) in the overall population. In those that had pulmonary balloon valvuloplasty group only clinical signs was significant (HR 3 · 44, P < 0 · 001). In cases that did not undergo pulmonary balloon valvuloplasty group Doppler gradient (HR 1 · 02, P < 0 · 001), clinical signs (HR 5 · 41, P = 0 · 002), valve morphology type B (HR 10 · 20, P = 0 · 001) and younger age at diagnosis (HR 12 · 82, P < 0 · 001) negatively affected survival. Dogs with severe pulmonic stenosis undergoing pulmonary balloon valvuloplasty (HR 0 · 47, P = 0 · 047) and asymptomatic dogs with moderate pulmonic stenosis (HR 0 · 10, P = 0 · 042) had a better outcome. Younger age at diagnosis was correlated with poorer outcome in right-sided congestive heart failure dogs (HR 14 · 02, P = 0 · 01)., Clinical Significance: Clinical signs, valve morphology type B and age at diagnosis are risk factors in pulmonic stenosis patients. Pulmonary balloon valvuloplasty is a reasonable treatment choice in dogs with severe pulmonic stenosis., (© 2013 British Small Animal Veterinary Association.)

Background and Aims: Percutaneous implantation of pulmonary valve has been recently introduced in the clinical practice. Our aim was to analyze data of patients treated in Italy by using the Melody Medtronic valve., Methods: Prospective, observational, multi-centric survey by means of a web-based database registry of the Italian Society of Pediatric Cardiology (SICP)., Results: Between October 2007 and October 2010, 63 patients were included in the registry (median age: 24 years; range 11-65 years). Forty subjects were in NYHA class I-II while 23 were in NYHA class III-IV. Patients included had a history of a median three previous surgeries (range 1-5) and a median of one previous cardiac catheterization (range 0-4). A cono-truncal disease was present in 39 patients, previous Ross operation in 9, and other diagnosis in 15. Indication to valve implantation was pure stenosis in 21 patients, pure regurgitation in 12, association of stenosis and regurgitation in 30. Implantation was performed in 61 subjects (97%). Pre-stenting was performed in 85% of cases. Median procedure time was 170 minutes (range 85-360). No significant regurgitation was recorded after procedure while the trans-pulmonary gradient reduced significantly. Early major complications occurred in seven subjects (11%). One death occurred in the early post-operative period in a severely ill subject. At a median follow-up of 30 months (range 12-48 months), three patients died due to underlying disease. Major complications occurred in six patients during follow-up (external electric cardioversion: one patient; herpes virus encephalitis: two patients; Melody valve endocarditis needing surgical explant: two patients; major fractures of the stent and need second Melody valve implantation: two patients). Freedom from valve failure at latest follow-up was 81.4% ± 9%., Conclusion: Early results of the SICP registry on transcatheter Melody pulmonary valve implantation show that the procedure is safe and successful. Major concerns are related to the occurrence of stent fracture and bacterial endocarditis. Longer follow-up and larger series are needed., (Copyright © 2012 Wiley Periodicals, Inc.)

Objectives: Surgical reconstruction of the right ventricular outflow tract (RVOT) often requires implantation of a valved conduit. A single-centre 10-year experience with the Hancock porcine-valved Dacron conduit was retrospectively assessed., Methods: The records of 63 patients who underwent RVOT reconstruction with Hancock conduit implantation between August 2000 and July 2010 were retrospectively reviewed. The median age was 13 years (range, 4 months to 64 years) and the median weight 44 kg (range, 6.5-75 kg). Fifty-one patients (83%) had previous cardiac surgery, and conduit replacement was performed in 31 patients (49%). Patient and conduit survivals with respect to factors precipitating conduit degeneration were analysed. Conduit failure was defined as severe conduit regurgitation or stenosis with a main pulmonary artery systolic gradient over 60 mmHg., Results: Early mortality was 4.8% and not related to conduit failure. Follow-up was complete with a mean duration of 3.5 ± 2.6 years. Patient survival after conduit implantation was 93 [95% confidence interval (CI), 87-100], 90 (95% CI, 81-100) and 85% (95% CI, 74-98) after 1, 3 and 5 years, respectively. Conduit failure occurred in six patients after a median of 5.6 years (range, 2.7-9.0 years). Freedom from conduit failure was 100, 96 (95% CI, 89-100) and 83% (95% CI, 62-100%) after 1, 3 and 5 years, respectively. Mean systolic gradient over the stenotic conduit valve was 87 ± 11 mmHg. Neither RVOT-aneurysm formation nor distal conduit stenosis occurred. Univariate analysis revealed younger age and absent pulmonary valve syndrome as risk factors for conduit failure (P = 0.01 and P < 0.01). Stepwise logistic regression identified higher white blood cell count at postoperative day 8 as a significant risk factor for conduit failure (odds ratio, 0.7; 95% CI, 0.52-0.89; P < 0.01)., Conclusions: The Hancock conduit is a valuable option for pulmonary valve replacement. It is not associated with RVOT-aneurysm formation or distal conduit stenosis. A persisting perioperative inflammatory reaction may be a predictor for later conduit failure.

Introduction: Ductal stent (DS) in duct-dependent pulmonary circulation is less morbid than neonatal Blalock-Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair., Methods: This is a retrospective review of clinical follow-up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion., Results: Among 22 infants, four Group A patients followed for 26-54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short-term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5-14 months at a body weight of 5-7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6-8.5 kg after 8-15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow-up for hypoxia. Four patients had sudden death., Conclusions: The short-term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%., (© 2012 Wiley Periodicals, Inc.)

We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100 %) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65 %) were diagnosed with Williams-Beuren-Syndrome, six (17 %) had a diffuse form of SVAS and 10 (39 %) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p = 0.30) or diffuse form of SVAS (p = 0.13). Patients with PS were operated at younger age (p = 0.028). Median follow-up time was 14.6 years. Overall mortality was 11.5 %. One patient with preoperatively severely decreased LV-function died 27 days postoperatively. Two late deaths occurred 7 and 10 years after the initial operation. Reoperations were required in 4 patients (15 %), 4-19 years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p = 0.005) and for the combined reoperation/death end-point (p = 0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.

Background: We aimed to reveal late clinical features of patients with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (cPS) after biventricular repair (BVR) based on preoperative right ventricular (RV) end-diastolic volume (RVEDV) findings., Methods: Since 1985, 23 of 73 patients with PA/IVS (n=22) or cPS (n=1) with a tripartite RV and without major sinusoidal communication underwent BVR with a hybrid approach. The mean age and weight at BVR were 1.4±2.1 years and 6.9±5.9 kg, respectively. Mean follow-up was 10.1±6.4 years (range, 1.1 to 24.6 years)., Results: Overall survival, reintervention-free, and arrhythmia-free rates at 20 years were 90.6%, 75.4%, and 50.4%, respectively. In 19 patients with preoperative RVEDV of 60% to 120% of normal, echocardiography at 10 years after BVR showed well-maintained RV systolic function. However, RV volume was quantitatively dilated in 16 (88.9%) due to moderate or greater tricuspid regurgitation in 8 (44.4%), pulmonary regurgitation in 12 (66.7%), or both, which caused arrhythmia in 3 patients more than 10 years after BVR. Two patients with preoperative RVEDV of greater than 120% of normal required tricuspid valve replacement after BVR, after which refractory atrial tachyarrhythmia developed in both patients. Furthermore, 2 patients with preoperative RVEDV of less than 60% of normal showed a cardiac index value within 2.5 L/min/m2 at 1 year after BVR, which did not improve., Conclusions: Patients with PA/IVS or cPS and adequately sized RV showed good late clinical features after BVR. However, long-term follow-up examinations are necessary for RV dilatation and late-onset arrhythmia., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure., Methods: This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy., Results: There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure., Conclusions: The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Implantation of stents into the ductus arteriosus is an alternative treatment to palliative or corrective cardiac surgery in newborns with duct-dependent pulmonary circulation, although the use of this technique for congenital heart disease is limited., Methods: Between April 2010 and June 2011, 13 patients underwent patent ductus arteriosus stenting after full assessment by echocardiogram and angiogram, two of patients had pulmonary atresia (PA) and ventricular septal defect (VSD), six patients had PA with intact ventricular septum (IVS), four patients had critical pulmonary stenosis with IVS and one single ventricle physiology with PA and four patients had radiofrequency-assisted perforation of the pulmonary valve at the same time. All procedures were retrograde through the femoral artery, except one, which was by the femoral vein approach., Results: The mean age and weight during intervention were 10.5±5.7 days and 3.1±0.4 kg, respectively. The mean of procedure and scopy time, time of stay in intensive care, total out-of-hospital and total follow-up time were 138.88±67.11 min; 40.32±25.86 min; 4.88±6.07 days; 11.00±6.89 days and 86.40±73.21 days, respectively. The mean of the radiation amount was 1054.27±1106.91 cGy/cm2. The mean of saturation before and after intervention were 64.44±5.83; 81.88±6.95%, respectively. Procedure-related deaths were observed in two patients. The causes of death were pulmonary haemorrhage (n=1) and retroperitoneal haemorrhage (n=1). Two patients also died after discharge before surgery due to sepsis (n=1) and aspiration pneumonia (n=1). Eight of 13 patients achieved stent patency during 6 months of follow up and re-stenosis developed in one patient (1/8; 12.5%) who had undergone a Glenn operation at 4.5 months of age., Conclusions: Ductal stenting is a practicable, effective, safer and less invasive method compared palliative or corrective surgery. Patients with ductal stenting have growth of the pulmonary artery which provides additional time for surgical repair. Our data suggested that ductal stenting should be considered as a first treatment step in newborns with duct-depended pulmonary circulation. However, long-term palliation without stent re-stenosismight still be a concern especially in patients with hypoplastic pulmonary arteries.

Objective: Optimal surgical treatment of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) remains a matter of debate. This study evaluated the clinical outcome and right ventricle outflow tract performance in the long-term follow-up of patients subjected to pulmonary root translocation (PRT) as part of their surgical repair., Methods: From April 1994 to December 2010, we operated on 44 consecutive patients (median age, 11 months). All had malposition of the great arteries as follows: TGA with VSD and PS (n = 33); double-outlet right ventricle with subpulmonary VSD (n = 7); double-outlet right ventricle with atrioventricular septal defect (n = 1); and congenitally corrected TGA with VSD and PS (n = 3). The surgical technique consisted of PRT from the left ventricle to the right ventricle after construction of an intraventricular tunnel that diverted blood flow from the left ventricle to the aorta., Results: The mean follow-up time was 72 ± 52.1 months. There were 3 (6.8%) early deaths and 1 (2.3%) late death. Kaplan-Meier survival was 92.8% and reintervention-free survival was 82.9% at 12 years. Repeat echocardiographic data showed nonlinear growth of the pulmonary root and good performance of the valve at 10 years. Only 4 patients required reinterventions owing to right ventricular outflow tract problems., Conclusions: PRT is a good surgical alternative for treatment of patients with TGA complexes, VSD, and PS, with acceptable operative risk, high long-term survivals, and few reinterventions. Most patients had adequate pulmonary root growth and performance., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: Describe the short and midterm outcome and to determine the predictors of reintervention in neonates with critical pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS)., Background: The transcatheter intervention for critical PS and PA/IVS resulted in improvement in the patient's survival and the quality of life. The procedure is not free of complications and there is still a significant rate of reintervention., Method: All neonates with critical PS or PA/IVS who underwent interventional cardiac catheterization between November 2004 and January 2009 were reviewed retrospectively. We performed a comparison between those who required reintervention and those who did not, to identify the predictors of reintervention., Results: Forty-three neonates were included, 23 (53.5%) had critical PS and 20 (46.5%) had PA/IVS. Twenty-six patients (60%) were males, the mean age was 11 ± 8 days, and the mean weight was 3.2 ± 0.6 kg. Two patients died (4.6%). The mean follow-up period was 19 ± 13 months for 42 patients. Fifteen patients (36%) required reintervention, 11 of them (73%) had PA/IVS, and 4 (27%) had critical PS. Reintervention was more in patients with PA/IVS than those with critical PS (P = 0.003). Other predictors for reintervention included hospital stay ≥ 7.5 days (P = 0.001) and tricuspid valve regurgitation peak gradient in day one post first intervention (TR1) ≥ 43 mm Hg (P = 0.03)., Conclusion: Interventional cardiac catheterization shows favorable outcome for patients with critical PS and PA/IVS. Predictors for reintervention included the diagnosis of PA/IVS, hospital stay ≥7.5 days after first intervention and TR1 gradient ≥ 43 mm Hg., (Copyright © 2011 Wiley Periodicals, Inc.)

Background: Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid., Methods and Results: We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases., Conclusions: Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

Objective: To determine the outcome, independent predictors of cardiac death, and the Doppler-derived pressure gradient cut-off for predicting cardiac death in dogs with pulmonic stenosis, with or without tricuspid regurgitation, that do not undergo balloon valvuloplasty or valve surgery., Methods: Review of medical records of two UK referral centres between July 1997 and October 2008 for all cases of pulmonic stenosis that had no balloon valvuloplasty or valve surgery. Inclusion criteria included a diagnosis of pulmonic stenosis; spectral Doppler pulmonic velocity greater than 1·6 m/s; characteristic valve leaflet morphological abnormalities. Exclusion criteria included concurrent significant cardiac defects, including tricuspid dysplasia. Dogs with tricuspid regurgitation were included. Dogs were classified according to Doppler-derived pressure gradients into mild, moderate or severe pulmonic stenosis categories., Results: Presence of tricuspid regurgitation and severe stenosis were independent predictors of cardiac death. A pulmonic pressure gradient of more than 60 mmHg was associated with 86% sensitivity, and 71% specificity of predicting cardiac death., Clinical Significance: There is an increased probability of cardiac death in those cases which have a pulmonary pressure gradient greater than 60 mmHg and tricuspid regurgitation, though the effect of severity of tricuspid regurgitation on outcome was not measurable because of small sample sizes. These animals might benefit from intervention., (© 2011 British Small Animal Veterinary Association.)

Background: Doubly committed ventricular septal defect (dcVSD) is the least common type of VSD. Because published studies are rather scarce, this study aimed at evaluating the midterm outcome of dcVSDs., Methods: The records of all patients registered in the database of Paediatric and Congenital Cardiology, University Hospitals Leuven, with a dcVSD at 16 years of age were reviewed. Clinical, electrocardiographic and transthoracic echocardiographic changes from baseline, defined as of the age of 16 years, until the latest follow-up were compared., Results: Thirty-three patients (20 males, median age 26 years, interquartile range 12) were followed for a median time of 7.9 years (interquartile range 9.8, time range 2-25.9). No deaths occurred. In 15 patients (45%), the defect remained patent at baseline. During follow-up, two spontaneous closures (13%) occurred. Eighteen patients (55%) required closure before the age of 16 years. Five (28%) needed reoperation. In the dcVSD closure group, left ventricular ejection fraction decreased from 69 ± 12 to 61 ± 6% (p = 0.028). No significant changes in pulmonary arterial hypertension were noticed., Conclusions: Patients with persistently patent dcVSD remained nearly event free during follow-up. Event-free survival after dcVSD closure was markedly lower. These patients developed reduced left ventricular function and had a high risk of reintervention., (Copyright © 2011 S. Karger AG, Basel.)

Background: The optimal surgical treatment of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is controversial. Although the Rastelli operation has been standard surgical management of this lesion, aortic root translocation with right ventricular outflow tract (RVOT) reconstruction (Nikaidoh) and the pulmonary artery translocation (Lecompte) or REV (réparation a l'étage ventriculaire) are surgical alternatives more recently introduced to treat this complex lesion. This report reviews our 20-year experience with the Rastelli procedure and attempts to compare our outcomes with those recently published using the Nikaidoh and REV procedures., Methods: Between 1988 and 2008, 40 patients (median age, 4 years; range, 9 months to 17 years) underwent Rastelli operation at our institutions. The RVOT was obstructed in 32 and atretic in 8. Follow-up was available for all but one patient (mean follow-up, 8.6±5.6 years). The RVOT was reconstructed with homograft (n=25), bovine jugular vein (n=8), nonvalved Dacron tube (n=5), or a porcine valved conduit (n=2). Two patients required a pacemaker., Results: There were no early, but three late deaths and one heart transplantation 12 years postoperative the Rastelli operation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Univariate risk factors for death or transplantation included surgery before 1998 (p=0.03) and concomitant noncardiac anomalies (p=0.001). Sixteen patients (40%) had reoperation for right ventricular-pulmonary artery conduit stenosis (mean, 7.8±3.8 years) without mortality. Freedom from conduit replacement was 86%, 74%, 63%, and 59% at 5, 10, 15, and 20 years, respectively. Multivariate analysis revealed that the risk factors of conduit replacement were younger age at operation (p=0.001) and surgery before 1998 (p<0.001). Two patients (5%) required reoperation for left ventricular outflow tract obstruction. At follow-up, there were no sudden unexplained deaths, and New York Heart Association functional class is I or II., Conclusions: The Rastelli procedure is a low-risk operation with regard to early and late mortality and reoperation for left ventricular outflow tract obstruction. Conduit change operations will be required in most patients regardless of the technique of repair, but currently can be performed with low morbidity and mortality. These midterm outcomes after the Rastelli operation should serve as a basis for comparison with surgical alternatives more recently introduced for transposition of the great arteries and ventricular septal defect with RVOT obstruction., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The bidirectional Glenn shunt operation is conventionally performed under cardiopulmonary bypass. Between June 2007 and September 2009, 218 consecutive patients underwent off-pump bidirectional Glenn shunt institution for single ventricle with pulmonary stenosis complex. Their mean age was 4.72 ± 1.80 years (range, 4 months to 6 years) and median weight was 10.12 kg (range, 4.1-19 kg). A temporary shunt was created between the innominate vein and the right atrium, with a 3-way connector for de-airing. Fifty-five patients had bilateral cavae. The mean internal jugular venous pressure on clamping the superior vena cava was 24.69 ± 1.81 mm Hg. Continuous end-tidal CO₂ and O₂ saturation were monitored. Adequate oxygen saturation and blood pressure were maintained by optimizing inotropics, volume, and inspired oxygen. The mean duration of ventilation was 10.17 ± 8.96 h (range, 1-73 h). There were no gross neurological complications. Postoperative pleural effusion developed in 6 (2.75%) patients, and 4 (1.83%) had nodal rhythm. Four (1.83%) patients died in the immediate postoperative period due to low cardiac output syndrome. Venoatrial shunt-assisted bidirectional Glenn shunt surgery can be performed safely by optimizing intraoperative management strategies. It is economical and avoids the deleterious effects cardiopulmonary bypass.

Objectives: This study focused on the anatomical features and outcomes of percutaneous pulmonary valve balloon dilation (PVD) in newborns < or = 3 kg in weight., Background: Although PVD is the treatment of choice for isolated pulmonary valve stenosis, there are no studies detailing technical, anatomical, and outcome variables in the smallest of infants undergoing the procedure., Methods: A retrospective, consecutive review of all initial and follow-up echocardiograms, catheterization data, cineangiograms, and surgical records of all neonates less than 3 kg., Results: Fifty infants underwent 55 PVD's, median age 7 days and weight 2.7 kg. Congestive heart failure and/or cyanosis were present in 55%, 46% prostaglandin dependent, with 28% having a hypoplastic right ventricle. The pulmonary valve diameter median z-score was -2.6, (range -5.8 to 1.2, P < 0.0001 vs. normal). The procedure was accomplished in all but one child, using a balloon to annulus ratio of 1.3 (range 1.1 to 1.7). There was a reduction in both the peak right ventricular systolic pressure (P < 0.0001) and outflow gradient (P < 0.0001). There was no procedure related mortality, although six children died in long-term follow-up (14%). Reintervention was necessary in 15 infants within 8 months of the procedure. Pulmonary regurgitation, present in 36% after the procedure, showed a gradual increase over time while outflow gradients decreased and valve annular dimensions increased toward normal., Conclusions: In infants < or = 3 kg, PVD is safe and effective. A large proportion remains free from surgery (76% at 10 years). Small valve annular dimensions show catch up growth with time., (Copyright 2009 Wiley-Liss, Inc.)

Background: The Rastelli procedure is the standard surgical treatment of d-transposition of great arteries (d-TGA), ventricular septal defect (VSD), and pulmonary stenosis. Late morbidity is significant due to recurrent left ventricular outflow obstruction (LVOTO), early conduit obstruction, and arrhythmias, with troublesome late mortality. To avoid recurrent LVOTO, we routinely enlarge the VSD and resect the infundibular septum before LV baffling to the aorta. We examined the efficacy of this approach in mitigating recurrent LVOTO risk., Methods: Late echocardiographic and time-related clinical results of patients undergoing the Rastelli procedure were examined. Demographics and operative variables affecting outcomes were analyzed., Results: The Rastelli cohort comprised 36 patients with d-TGA, VSD, and pulmonary stenosis. Median age at operation was 2.4 years (range, 0.3 to 8.3 years). Pulmonary stenosis was present in 31 and atresia in 5. Twenty-two patients had undergone a previous aortopulmonary shunt, and 6 had an atrial septectomy. No operative or late deaths occurred. Time-related freedom from permanent pacemaker implantation, recurrent LVOTO on echocardiogram, and conduit replacement at 10 years was 82%, 100%, and 49%, respectively. Systolic function was normal in all but 3 patients and 92% were in New York Heart Association functional class I and II. None of the patients had late arrhythmias or required heart transplantation., Conclusions: Early and midterm survival after the Rastelli procedure is satisfactory. Aggressive resection of the infundibular septum to enlarge the VSD has mitigated the risk of LVOTO recurrence. Late conduit obstruction remains an important source of morbidity and frequently requires reintervention.

Objective: To assess the efficacy of an open patch-graft technique under cardiopulmonary bypass (CPB) in small dogs., Design and Methods: A retrospective analysis of 10 dogs with pulmonic stenosis. Records between 1992 and 2002 were reviewed. The effect of surgical correction was evaluated and perioperative parameters were compared between survivors and non-survivors., Results: The postoperative pulmonary pressure gradient was reduced in all seven surviving patients. Mean +/- SE was 21.5 +/- 7.4 mmHg (range 3.0-54.2 mmHg) and 6/7 dogs were < 40 mmHg at 3 months postoperatively. Comparing the data between those patients that survived and those that did not, the preoperative pressure gradient (P = 0.04) and volume of the Glucose-Insulin-Kalium solution used (P = 0.001) were significantly higher in those that did not survive., Conclusion: Open patch-grafting can be performed in small-breed dogs and decreased the pulmonary pressure gradient in survivors at 3 months postoperatively. However, this technique is more invasive than balloon valvuloplasty and should be used cautiously in severely stenosed patients.

Williams syndrome is a multifaceted disorder that includes a spectrum of cardiovascular anomalies. Due to its rare occurrence, outcome data for operations and cardiac catheterization are limited. We reviewed data from 242 individuals from the Pediatric Cardiac Care Consortium (PCCC) with Williams syndrome and associated cardiovascular lesions, and their frequency, and assessed their effects on mortality. In the PCCC, from 1984 to 1999 there were approximately 100,000 entries for cardiac procedures, involving more than 62,000 patients. The diagnosis of Williams syndrome was based on clinical features and determined by each site. Most patients were diagnosed with the availability of the FISH probe for region 7q11.23. Using a spreadsheet application, Microsoft Excel, the selected patients were analyzed for various types of cardiac anomalies. The most common cardiovascular lesions and the mortality rate in patients with Williams syndrome were examined. A complete tabulation of all cardiovascular lesions was assembled. There were 292 catheterizations and 143 operations reported to the PCCC. One hundred six patients had both an operation and a catheterization. The three main cardiovascular anomalies were supravalvular aortic stenosis (SVAS; 169), pulmonary artery stenosis (PAS; 130), and coarctation or aortic arch hypoplasia (Arch; 32). One hundred five patients had a single lesion, 70 with SVAS, 29 with PAS, and 6 with an arch anomaly. Ninety-two had more than one lesion: 80 with SVAS and PAS, 7 with PAS and Arch, and 5 with SVAS and Arch. Seventy individuals have only SVAS, 29 PAS, and 6 Arch alone. There was a total of 15 deaths. The mortality rate was highest in the group with the combination of SVAS and PAS (7 surgical and 5 catheter; 12 of 80 patients [15%]; p = 0.0001, chi(2)). In conclusion, our data represent the largest collection of individuals with Williams syndrome who underwent cardiac catheterization and/or operation. The data suggest that children with Williams syndrome and bilateral outflow tract obstruction have statistically and clinically significantly higher mortality associated with catheterization or operation.

Background: The ideal age for correction of tetralogy of Fallot is still under discussion. The aim of this study was to analyze morbidity and mortality in patients who underwent early primary repair of tetralogy of Fallot at the age of less than 4 months and to assess whether neonates, who needed early repair within the first 4 weeks of life, faced an increased risk., Methods: From 1995 to 2006, 90 consecutive patients with tetralogy of Fallot and pulmonary stenosis underwent early primary repair. Patient charts were analyzed retrospectively for two groups: group A, 25 neonates younger than 28 days who needed early operation owing to duct-dependent pulmonary circulation or severe hypoxemia; and group B, 65 infants younger than 4 months of age who underwent elective early repair., Results: There was no 30-day mortality; late mortality was 2% after a median follow-up time of 4.7 years. Seven of 88 patients (8%) needed reoperation and twelve of 88 patients (14%) needed reintervention. Groups A and B did not differ significantly in terms of intensive care unit stay, days of mechanical ventilation, overall hospital stay, major or minor complications, or reoperation. Significant differences were found in a more frequent use of a transannular patch (p = 0.045) and more reinterventions (p = 0.046) in group A., Conclusions: Early primary repair of tetralogy of Fallot can be performed safely and effectively in infants younger than 4 months of age and even in neonates younger than 28 days with duct-dependent pulmonary circulation or severe hypoxemia.

Objectives: In older children (>four years) and adults the strategy of repair of tetralogy of Fallot (TOF) should have a low rate of transannular patch to avoid pulmonary insufficiency (PI), and in addition, we developed a new method of reconstruction of the right ventricular outflow tract (RVOT) to reduce PI., Methods: From 2001 through 2005, 74 patients (50 male, 24 female; mean age of 13.6 +/- 0.8 years, ranging from four to 34 years) with TOF (67) or double outlet RV (DORV)-type-TOF (seven) underwent complete repair. The resection of RVOT stenosis +/- pulmonary valvotomy was principally through RA. A new two-patch technique to create a folded mono-cusp valve by using autologous pericardium was applied since August 2004., Results: The repair was through RA in 52 patients and through RA + RVOT/PA in 22 patients (18/4). TAPR was performed in 17 patients (23%, eight non-valved and nine valved). The aortic cross-clamp time was 130.1 +/- 6.8 min in TAPR group and 85.8 +/- 4.6 min in non-TAPR group (p < 0.0001). The operative mortality was 4.0% (3/74) in all and 2.9% (2/67) in TOF patients (due to low output, uncontrollable bleeding, and repeated bleeding from the pulmonary collateral vessels late) and was similar in TAPR or non-TAPR. The valved patch-repaired patients had mild PI and good RV function postoperatively up to eight to 12 months., Conclusions: Repair of TOF in older children/adults should include low rate of TAPR of RVOT. If indicated, the new folded monocusp-patch technique is recommended. It is an effective and simple way to markedly reduce postoperative PI with no additional cost.

Objective: Midterm follow-up is analyzed after the aortic translocation (Nikaidoh) procedure, an alternative to the Rastelli procedure for ventriculoarterial discordance, ventricular septal defect, and pulmonary stenosis., Methods: Nineteen patients underwent a Nikaidoh procedure at a median age of 3.3 years (0.9-9.3 years). The native aortic valve was translocated from the right to the left ventricular outflow tract by full (n = 6) or partial (n = 13) mobilization of the aortic root. Seven patients with partial mobilization had the right coronary artery reimplanted as a button. The conal septum was divided in 13 patients. The right ventricular outflow tract was reconstructed with either a homograft (n = 4) or a right ventricular outflow tract patch (n = 15). The median follow-up was 11.4 years (0.1-23 years), and the median age at follow-up was 17.4 years (1-30 years). Left ventricular outflow tract obstruction and aortic insufficiency were assessed by echocardiography., Results: One patient died of right coronary arterial ischemia. All remaining patients (95%) survived. The median survival was 13.6 years (longest, 23.0 years). Seven right ventricular outflow tract reoperations were required in 5 patients (6 with obstruction and 1 with pulmonary insufficiency). No reoperations have been performed on the left ventricular outflow tract or aortic valve. No patient had any left ventricular outflow tract obstruction or aortic insufficiency more than mild (mild in 9 patients, trivial in 3 patients, and absent in 6 patients)., Conclusions: Midterm actuarial survival was 95% after the Nikaidoh procedure. Reintervention for the right ventricular outflow tract is more common when valved conduits are used versus valveless reconstruction; however, the Nikaidoh procedure provides complete freedom from important aortic insufficiency and left ventricular outflow tract obstruction.

Background: The Ross operation is increasingly accepted as an alternative to conventional valve prostheses for children, adolescents, and young adults. We review patients younger than 20 years of age., Methods: Of 404 Ross operations done before November 2004, 60 were young patients with a median age of 12 years (range, 1 to 20 years). The pulmonary autograft technique universally was as a free root. A cryopreserved pulmonary homograft reconstructed the right ventricular outflow tract., Results: Early postoperative complications were reentry for bleeding in 2 patients and one pacemaker insertion. No thromboembolic or hemorrhagic events occurred during the follow-up of 42 +/- 27 months. Two late deaths occurred, one from myocardial infarction after 3 months and another sudden death after 5 years, probably from critical pulmonary homograft stenosis. Echocardiographic follow-up revealed a median peak gradient of 6.3 +/- 3 mm Hg across the autograft. The median pulmonary homograft peak gradient of 19.1 +/- 13.7 mm Hg was increased to more than 30 mm Hg in 6 patients. Another 6 patients had moderate but clinically insignificant pulmonary homograft regurgitation. Altogether, 6 patients required reoperation for replacement of stenotic homografts. No autograft related reoperation occurred., Conclusions: Young patients with the Ross operation had good mid-term autograft function and no perioperative mortality. Factors that justify the choice of the Ross operation for young patients are the normal physiologic hemodynamics and growth of the autograft as well as freedom from anticoagulation. A 10% reoperation rate, elevated pulmonary homograft gradients, and the surgical complexity remain limiting factors.

Aims: Long-term (>20 years) survival and clinical outcome are only partly documented in patients who underwent surgical repair for isolated pulmonary stenosis. Yet, such data are of critical importance for the future perspectives, medical care, employability, and insurability of these patients., Methods and Results: Ninety consecutive patients underwent surgery for pulmonary stenosis between 1968 and 1980 at the Thoraxcenter. A systematic follow-up study was performed in 1990 and again in 2001. Survival after 25 years was 93%. Re-intervention was necessary in 15% of the patients, mainly for pulmonary regurgitation. Right atrial and ventricular dilatation and paradoxical septal motion were associated with the need for reoperation. No major ventricular arrhythmias occurred. Supraventricular arrhythmias occurred, only in patients with severe pulmonary regurgitation and disappeared after reoperation. At last follow-up, 67% of the patients was in NYHA Class I and maximal exercise capacity was 90% of normal. Moderate or severe pulmonary regurgitation was present in 37% of the patients., Conclusion: Although long-term survival and quality of life are good, pulmonary regurgitation is found in a third of the patients 22-33 years after surgical repair for isolated pulmonary stenosis and reoperation for pulmonary regurgitation was necessary in 9%, especially after the transannular patch technique.

The records of 43 dogs presenting with severe pulmonic stenosis in which balloon valvuloplasty was attempted were reviewed. Thirty-four dogs (79 per cent) were symptomatic at initial presentation. All patients were selected for balloon valvuloplasty on the basis of a Doppler-derived trans-stenotic pressure gradient of over 80 mmHg and concurrent evidence of mild to severe right ventricular hypertrophy. Forty dogs underwent balloon valvuloplasty; the procedure was not performed in three dogs because of an aberrant coronary artery in two cases and because catheterisation of the pulmonary artery was not possible in the third. Overall, 37 out of the 40 dogs (93 per cent) were successfully ballooned, resulting in a mean reduction in the pressure gradient of 46 per cent, with a mean pressure gradient of 124 mmHg on presentation and 67 mmHg six months after the procedure. Three dogs died during balloon valvuloplasty (all of which had a concurrent defect) and three dogs showed a poor clinical response to the procedure. Thus balloon valvuloplasty was successful and resulted in a sustained clinical improvement in 80 per cent of previously symptomatic cases. This study was undertaken to document the results of balloon valvuloplasty in a larger population of dogs than has previously been published.