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Objectives: Aortic valve repair procedures are technically challenging, and current intraoperative evaluation methods often fail to predict the final echocardiographic result. We have developed a novel intraoperative aortic valve visualization and pressurization (AVP) device, enabling valve inspection under physiological conditions, and measuring aortic valve insufficiency (AI) during cardioplegic arrest., Methods: The AVP device is attached to the (neo)aorta, after any type of aortic valve repair, while the heart is arrested. The root is pressurized (60-80 mmHg) using a saline solution and an endoscope is introduced. The valve is inspected, and the amount of valvular leakage is measured. Postoperative 'gold standard' transesophageal echocardiogram measurements of AI are performed and compared against regurgitation volume measured., Results: In 24 patients undergoing valve-sparing root replacement, the AVP device was used. In 22 patients, postoperative echocardiographic AI was ≤ grade 1. The median leakage was 90 ml/min, IQR 60-120 ml/min. In 3 patients, additional adjustments after visual inspection was performed. In 2 patients, with complex anatomy, the valve was replaced. In one, after evaluation with the device, there was undesirable result visually and residual AI of 330 ml/min, and in another, 260 ml/min residual AI was measured and valve restriction on visual inspection., Conclusions: The novel AVP device enables intraoperative evaluation of the valve under physiological conditions, while still on arrested heart, and allows for targeted adjustments. The AVP device can be an important aid for intraoperative evaluation of the aortic valve, during valve repair and valve-sparing procedures, thereby making the operative result more predictable and the operation more efficient., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Objectives: Delayed sternal closure is a routine procedure to reduce hemodynamic and respiratory instability in pediatric patients following cardiac surgery, particularly in neonates and infants. In this setting, the possible links between sternal wound infection and delayed sternal closure are still a matter of debate. As a part of our routine, there was a low threshold for delayed sternal closure, so we reviewed our experience with sternal wound infections with a focus on potentially related perioperative risk factors, particularly delayed sternal closure., Methods: We retrospectively identified 358 operated neonates (37%) and infants (mean age 3.6 months) in our local congenital heart disease database between January 2013 and June 2017. Potential risk factors for sternal wound infections, such as age, gender, complexity (based on Aristotle- and STS-EACTS mortality category), reoperation, use of cardiopulmonary bypass, extracorporeal membrane oxygenation, mortality and delayed sternal closure (163/358, 46%), were subjected to uni- and multivariate analysis., Results: A total of 26/358 patients (7.3%) developed a superficial sternal wound infection. There were no deep sternal wound infections, no mediastinitis or sepsis. Applying univariate analysis, the prevalence of sternal wound infections was related to younger age, more complex surgery and delayed sternal closure. However, in multivariate analysis, sternal wound infection was only associated with delayed sternal closure (p = 0.013, odds ratio 8.6). Logistic regression revealed the prevalence of delayed sternal closure to be related to younger age, complexity, and the use of extracorporeal membrane oxygenation., Conclusion: In patients younger than one year, sternal wound infections are clearly related to delayed sternal closure. However, in our cohort, all sternal wound infections were superficial and acceptable, considering the improved postoperative hemodynamic stability., Competing Interests: The authors have declared that no competing interests exist.

Objective: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect., Methods: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation., Results: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years., Conclusions: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives: To determine whether children with asymptomatic carriage of rhinovirus in the nasopharynx before elective cardiac surgery have an increased risk of prolonged PICU length of stay., Study Design: Prospective, single-center, blinded observational cohort study., Setting: PICU in a tertiary hospital in The Netherlands., Patients: Children under 12 years old undergoing elective cardiac surgery were enrolled in the study after informed consent of the parents/guardians., Interventions: The parents/guardians filled out a questionnaire regarding respiratory symptoms. On the day of the operation, a nasopharyngeal swab was obtained. Clinical data were collected during PICU admission, and PICU/hospital length of stay were reported. If a patient was still intubated 3 days after operation, an additional nasopharyngeal swab was collected. Nasopharyngeal swabs were tested for rhinovirus and other respiratory viruses with polymerase chain reaction., Measurements and Main Results: Of the 163 included children, 74 (45%) tested rhinovirus positive. Rhinovirus-positive patients did not have a prolonged PICU length of stay (median 2 d each; p = 0.257). Rhinovirus-positive patients had a significantly shorter median hospital length of stay compared with rhinovirus-negative patients (8 vs 9 d, respectively; p = 0.006). Overall, 97 of the patients (60%) tested positive for one or more respiratory virus. Virus-positive patients had significantly shorter PICU and hospital length of stay, ventilatory support, and nonmechanical ventilation. Virus-negative patients had respiratory symptoms suspected for a respiratory infection more often. In 31% of the children, the parents reported mild upper respiratory complaints a day prior to the cardiac surgery, this was associated with postextubation stridor, but no other clinical outcome measures., Conclusions: Preoperative rhinovirus polymerase chain reaction positivity is not associated with prolonged PICU length of stay. Our findings do not support the use of routine polymerase chain reaction testing for respiratory viruses in asymptomatic children admitted for elective cardiac surgery., Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2020 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Background: Despite the improved outcome in complete atrioventricular septal defect (AVSD) repair, reoperations for left atrioventricular valve (LAVV) dysfunction are common. The aim of this study was to evaluate the effect of fresh untreated autologous pericardium for ventricular septal defect (VSD) closure on atrioventricular valve function and compare the results with the use of treated bovine pericardial patch material., Methods: Clinical and echocardiographic data were collected of patients with complete AVSD with their VSD closed with either untreated autologous pericardial or treated bovine pericardial patch material between January 1, 1996, and December 31, 2003. Evaluation closed in September 2019., Results: A total of 77 patients were analyzed (untreated autologous pericardial VSD patch: 59 [77%], treated bovine pericardial VSD patch: 18 [23%]). Median age at surgery was 3.6 (interquartile range [IQR]: 2.7-4.5) months, and median weight was 4.5 (IQR: 3.9-5.1) kg. Trisomy 21 was present in 70 (91%) patients. Median follow-up time was 17.5 (IQR: 12.6-19.8) years. Death <30 days occurred in two (3%) patients. Reinterventions occurred in eight patients (early [within 30 days] in two, early and late in one, and late in five), all in the autologous pericardium group. Log-rank tests showed no significant difference in mortality ( P = .892), LAVV reinterventions ( P = .228), or LAVV regurgitation ( P = .770)., Conclusions: In AVSD, the VSD can safely be closed with either untreated autologous pericardium or xeno-pericardium. We found no difference in LAVV regurgitation or the need for reoperation between the two patches.

Surgical repair of Tetralogy of Fallot (ToF) is usually performed in the first months of life with low early postoperative mortality. During long-term follow-up, however, both right (RV) and left ventricular (LV) performances may deteriorate. Tissue Doppler imaging (TDI) and speckle tracking echocardiography (ST) can unmask a diminished RV and LV performance. The objective of the current study was to assess the cardiac performance before and shortly after corrective surgery in ToF patients using conventional, TDI and ST echocardiographic techniques. Thirty-six ToF patients after surgery were included. Transthoracic echocardiography including TDI and ST techniques was performed preoperatively and at hospital discharge after surgery (10 days to 4 weeks after surgery). Median age at surgery was 7.5 months [5.5-10.9]. Regarding the LV systolic function there was a significant decrease in interventricular septum (IVS) S' at discharge as compared to preoperatively (pre IVS S' = 5.4 ± 1.4; post IVS S' = 3.9 ± 1.2; p < 0.001) and in global longitudinal peak strain (GLS) (pre = - 18.3 ± 3.4; post = - 14.2 ± 4.1; p = 0.003); but not in the fractional shortening (FS). Both conventional and TDI parameters showed a decrease in diastolic function at discharge. Tricuspid Annular Plane Systolic Excursion and RV S' were significantly lower before discharge. When assessing the RV diastolic performance, only the TDI demonstrated a RV impairment. There was a negative correlation between age at surgery and postoperative LV GLS (R = - 0.41, p = 0.031). There seems to be an impairment in left and right ventricle performance at discharge after ToF corrective surgery compared to preoperatively. This is better determined with TDI and ST strain imaging than with conventional echocardiography.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: This study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to Mustard or Senning for transposition of the great arteries (TGA)., Methods: In 76 adult patients with TGA (59% male) after atrial correction, long-term survival and morbidity were investigated in three periods: early (<15 years postoperatively), midterm (15-30 years postoperatively) and late (>30 years postoperatively)., Results: The Mustard technique was performed in 41 (54%) patients, and the Senning technique was performed in 35 (46%) patients aged 3.1 (IQR: 2.1-3.8) and 1.0 (IQR: 0.6-3.1; p<0.01) years, respectively. Adult survival was 82% at 39.7 (IQR: 35.9-42.4) years postoperatively and exceeded 50 years in four patients. Supraventricular tachycardia (SVT) occurred in 51% of patients. The incidences of ventricular arrhythmia (0%, 8% and 13%; p<0.01), heart failure (0%, 5% and 19%; p<0.01) and surgical reinterventions (0%, 5% and 11%; p=0.01) increased from early to late follow-up. At last follow-up, RV function was depressed in 31 (46%) patients, and New York Heart Association functional class was ≥2 in 34 (48%) patients. Bradyarrhythmia, SVT and ventricular arrhythmia were associated with depressed RV function (OR: 4.47, 95% CI 1.50 to 13.28, p<0.01; OR: 3.74, 95% CI 1.26 to 11.14, p=0.02; OR: 14.40, 95% CI 2.80 to 74.07, p<0.01, respectively) and worse functional capacity (OR: 2.10, 95% CI 0.75 to 5.82, p=0.16; OR: 2.87, 95% CI 1.06 to 7.81, p=0.04; OR: 8.47, 95% CI 1.70 to 42.10, p<0.01, respectively)., Conclusions: In adult patients with TGA, survival was 82% at 39.7 (IQR: 35.9-42.4) years after atrial correction. Morbidity was high and included SVT as most frequent adverse event. Ventricular arrhythmias, heart failure and surgical reinterventions were common during late follow-up. Adverse events were associated with depressed right ventricle function and reduced functional class., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Pericardial effusion (PE) after pediatric cardiac surgery is common. Because of the lack of a uniform classification of the presence and severity of PE, we evaluated PE altering clinical management: clinically relevant PE. Risk factors for clinically relevant PE were studied. After cardiac surgery, children were followed until 1 month after surgery. Preoperative variables were studied in the complete cohort. Perioperative and postoperative variables were studied in a case-control manner. Patients with and without clinically relevant PE were matched on age, gender, and diagnosis severity in a 1:1 ratio. Multivariate analysis was conducted using important preoperative variables from the complete cohort combined with perioperative and postoperative variables from the case-control data. 1241 surgical episodes in 1031 patients were included. Clinically relevant PE developed in 136 episodes (11.0%). Multivariate correlation with the outcome was present for age, BSA (adjusted odds ratio: 1.6, 95% CI 0.9-2.8), right-sided heart defect (adjusted odds ratio: 1.3, 95% CI 0.9-1.9), history of previous operation (adjusted odds ratio: 0.5, 95% CI 0.3-0.7), cardiopulmonary bypass use (adjusted odds ratio: 2.1, 95% CI 0.9-4.5), duration of CPAP postoperatively, and an inotropic score (adjusted odds ratio: 1.01, 95% CI 0.998-1.03). In this large patient cohort, 11.0% of postoperative periods of pediatric cardiac surgery were complicated by PE requiring alteration of treatment. Secondly, we newly identified cardiopulmonary bypass use and right-sided heart defects as risk factors for clinically relevant PE and confirmed previously described risk factors: age, CPAP duration, BSA, and inotropic score and a previously described risk reductor: history of previous operation.

Objectives: In repaired tetralogy of Fallot, surgical pulmonary valve replacement (PVR) is in certain cases required. Our institution reported earlier about 26 patients who received a pulmonary homograft via PVR. To date, we have data from more than 17 years of follow-up. The aim of this retrospective study was to evaluate the late haemodynamic and clinical outcomes in this predefined patient group., Methods: Between 1993 and 2001, 26 patients underwent PVR for pulmonary regurgitation (58% men; 30.4 ± 8.9 years). The rates of mortality and of complications (re-PVR, ablation and cardioverter defibrillator implants) were analysed. Other main study outcomes were haemodynamic parameters determined from cardiovascular magnetic resonance imaging: pulmonary regurgitation; right ventricular (RV) end-diastolic volume; RV ejection fraction; left ventricular (LV) end-diastolic volume; LV ejection fraction; New York Heart Association functional class at the latest follow-up visit; and echocardiographic parameters of the right ventricle., Results: The median follow-up time was 17 ± 1.1 years. Overall freedom from complications was 61.5% (95% confidence interval 47.5-78.6%). One patient died 18 months after surgery of unknown causes. Two patients needed replacement of the homograft at 24 and 39 months after PVR. The indication in both patients was recurrence of severe homograft regurgitation with important RV dilatation. Six patients received an implantable cardioverter defibrillator at a median age of 41 years (interquartile range 36-47); 12 patients experienced supra- and/or ventricular arrhythmias and 6 of these needed ablation. There was no significant deterioration of haemodynamic function or functional class., Conclusions: The patients who underwent PVR exhibited long-term follow-up stabilization of RV function and impressive functional durability of the graft. After a follow-up of 17 years, 23 out of 26 patients (89%) were alive without redo PVR. Event-free survival was good (61.5%).

Background: Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has generally been replaced by a Damus-Kaye-Stansel or Norwood procedure due to concerns of iatrogenic heart block, reobstruction, or ventricular dysfunction. Studies reporting long-term outcome after the direct approach are limited. The aim of our study was to describe and analyze our experience with direct relief of subaortic stenosis in single ventricle patients., Methods: Demographic data, characteristics, and pre-operative, operative and outcome details were collected for children undergoing direct relief of subaortic stenosis between 1989 and 2016., Results: Twenty-three patients (median age: 7.4 months, range: 10 days to 5.5 years) underwent direct relief of subaortic stenosis. Complete follow-up was available for all patients (median: 15.6 years, range: 34 days to 26.3 years). Seven (30%) patients had recurrence of subaortic stenosis. One (4%) patient developed complete heart block and one patient developed moderate ventricular dysfunction. Five (50%) patients developed a (pseudo)aneurysm at site of the patch and ventriculotomy. There were two perioperative deaths. Eighty-six percent of patients underwent a successful Fontan procedure., Conclusions: Direct relief of subaortic stenosis is associated with a substantial risk of reobstruction and patch (pseudo)aneurysm formation. However, risk of heart block is low and long-term outcome is good with the majority of patients reaching Fontan completion. In our opinion, the direct approach appears to be a good and relatively simple procedure in selected cases for the treatment of subaortic stenosis.

Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of 18 years could predict the chance of congenital heart surgery in adulthood. Design and methods Of 10,300 patients from the CONCOR national registry, we used general patient characteristics at age 18 years, underlying congenital heart defect, history of complications, and interventions in childhood as potential predictors of congenital heart surgery occurring from age 18 years up to age 40 and 60 years. Cox regression was used to calculate hazard ratios with 95% confidence intervals. Analyses were performed separately for all congenital heart surgery and for valvular surgery alone. Results Altogether 2427 patients underwent congenital heart surgery after age 18 years, 1389 of whom underwent valvular surgery. Underlying heart defect, male sex, multiple defects, childhood endocarditis, supraventricular arrhythmia, aortic complications and paediatric cardiovascular surgery, independently predicted adult congenital heart surgery. The mean chance of congenital heart surgery was 22% up to age 40 and 43% up to age 60 years; individual chances spanned from 9-68% up to age 40 and from 19-93% up to age 60 years. Conclusion At the time of transition from paediatric to adult cardiology, an easily obtainable set of characteristics of patients with congenital heart disease can meaningfully inform cardiologists about the patient's individual chance of surgery in adulthood. Our findings warrant validation in other cohorts.

Objective: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study., Design: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed., Results: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths., Conclusions: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period., (© 2016 Wiley Periodicals, Inc.)

Background: Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability., Methods: A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013., Results: Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts., Conclusions: Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: During normal left ventricular (LV) filling, a vortex ring structure is formed distal to the left atrioventricular valve (LAVV). Vortex structures contribute to efficient flow organization. We aimed to investigate whether LAVV abnormality in patients with a corrected atrioventricular septal defect (AVSD) has an impact on vortex ring formation., Methods: Whole-heart 4D flow MRI was performed in 32 patients (age: 26 ± 12 years), and 30 healthy subjects (age: 25 ± 14 years). Vortex ring cores were detected at peak early (E-peak) and peak late filling (A-peak). When present, the 3-dimensional position and orientation of the vortex ring was defined, and the circularity index was calculated. Through-plane flow over the LAVV, and the vortex formation time (VFT), were quantified to analyze the relationship of vortex flow with the inflow jet., Results: Absence of a vortex ring during E-peak (healthy subjects 0%, vs patients 19%; P = .015), and A-peak (healthy subjects 10% vs patients 44%; P = .008) was more frequent in patients. In 4 patients, this was accompanied by a high VFT (5.1-7.8 vs 2.4 ± 0.6 in healthy subjects), and in another 2 patients with abnormal valve anatomy. In patients compared with controls, the vortex cores had a more-anterior and apical position, closer to the ventricular wall, with a more-elliptical shape and oblique orientation. The shape of the vortex core closely resembled the valve shape, and its orientation was related to the LV inflow direction., Conclusions: This study quantitatively shows the influence of abnormal LAVV and LV inflow on 3D vortex ring formation during LV inflow in patients with corrected AVSD, compared with healthy subjects., (Copyright © 2015. Published by Elsevier Inc.)

Objective: Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR., Methods: In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included. Preoperative and postoperative imaging data and a composite of adverse clinical events (death, sustained ventricular tachycardia, heart failure, or supraventricular tachycardia) were collected. Multivariate Cox hazards regression analysis was used to determine which factors were predictive for adverse events after PVR., Results: A total of 129 patients (61% men, age at PVR 32.9±10.4 years) were included. The composite endpoint occurred in 39 patients during 8.4±4.2 years of follow-up. In multivariate analysis, severe preoperative TR (HR 2.49, 95% CI 1.11 to 5.52), right ventricular end-systolic volume (HR 1.02/mL/m(2), 95% CI 1.01 to 1.03) and age at PVR (HR 1.07/year, 95% CI 1.04 to 1.09) were predictive for adverse events. Early postoperative TR was not predictive for adverse events (p=0.96). In patients without any risk factor (age >40 years, right ventricular end-systolic volume >90 mL/m(2) or severe TR), 5-year event-free survival was 100% as compared with 61% in patients with two or three risk factors., Conclusions: In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objectives: Valuable treatment modalities for branch pulmonary artery (BPA) stenoses are surgical patch angioplasty, percutaneous BPA stenting and hybrid stent placement. The purpose of this study was to report our institutional experience with hybrid stent placement to relieve BPA stenoses., Methods: Between August 2007 and May 2014, 7 adults (5 females) with congenital heart disease (CHD) had elective intraoperative BPA stent placement. All 7 patients had undergone previous surgery [6 tetralogy of Fallot repairs and 1 arterial switch operation for transposition of the great arteries (TGAs)]. A total of 7 stents (4 right pulmonary artery, 3 left pulmonary artery) were implanted under direct vision, concomitant with a pulmonary homograft implantation (tetralogy of Fallot patients), or because percutaneous stenting was not feasible (TGA patient). Retrospective analysis of clinical data, procedural details and outcomes was performed., Results: Overall, the mean age was 35 ± 7.2 years. Stent implantation was successful in all procedures. The mean postinflation stent diameter was 13.3 ± 2.0 mm. No stent migration, fracture, stent thrombosis, reintervention or deaths occurred. In 1 case the procedure was complicated by a right pulmonary artery tear just distal to the stent which was repaired by surgical patch angioplasty. At a mean follow-up of 55.6 ± 26 months no patient required catheter reintervention or surgery. Echocardiography (mean follow-up 47.1 ± 22 months), MRI (mean follow-up 43.8 ± 37 months) and CT data (mean follow-up 14.8 ± 10 months) demonstrate a BPA diameter increment from a mean 5.57 ± 2.29 to 10.71 ± 2.56 mm., Conclusions: Hybrid intraoperative BPA stent placement is safe and effective and can be used as an alternative for surgical patch angioplasty or if percutaneous BPA stenting is not feasible. Short- to mid-term results are good., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: To characterize and directly quantify regurgitant jets of left atrioventricular valve (LAVV) in patients with corrected atrioventricular septal defect (AVSD) by four-dimensional (4D)Flow Cardiovascular Magnetic Resonance (CMR), streamline visualization and retrospective valve tracking., Methods: Medical ethical committee approval and informed consent from all patients or their parents were obtained. In 32 corrected AVSD patients (age 26 ± 12 years), echocardiography and whole-heart 4DFlow CMR were performed. Using streamline visualization on 2- and 4-chamber views, the angle between regurgitation and annulus was followed throughout systole. On through-plane velocity-encoded images reformatted perpendicular to the regurgitation jet the cross-sectional jet circularity index was assessed and regurgitant volume and fraction were calculated. Correlation and agreement between different techniques was performed with Pearson's r and Spearman's rho correlation and Bland-Altman analysis., Results: In 8 patients, multiple regurgitant jets over the LAVV were identified. Median variation in regurgitant jet angle within patients was 36°(IQR 18-64°) on the 2-chamber and 30°(IQR 20-40°) on the 4-chamber. Regurgitant jets had a circularity index of 0.61 ± 0.16. Quantification of the regurgitation volume was feasible with 4DFlow CMR with excellent correlation between LAVV effective forward flow and aortic flow (r = 0.97, p < 0.001) for internal validation and moderate correlation with planimetry derived regurgitant volume (r = 0.65, p < 0.001) and echocardiographic grading (rho = 0.51, p = 0.003)., Conclusions: 4DFlow CMR with streamline visualization revealed multiple, dynamic and eccentric regurgitant jets with non-circular cross-sectional shape in patients after AVSD correction. 4DFlow with retrospective valve tracking allows direct and accurate quantification of the regurgitation of these complex jets.

Objectives: To evaluate long-term results of tracheoplasty using autologous pericardial patch and strips of costal cartilage for relieving severe long-segment tracheal stenosis., Methods: Data were collected retrospectively by clinical chart review. Between 1995 and 2013, 21 patients underwent tracheoplasty. Follow-up was performed by outpatient chart review; otherwise, referring physicians and parents were contacted and asked to fill in a questionnaire., Results: Median age at the time of operation was 0.9 (range 0.5-44) years. Aetiology of tracheal stenosis was double aortic arch in 9 patients, right arch with a left ductus in 3, innominate artery compression in 1 patient, complete tracheal rings in 7, 3 of whom with pulmonary artery sling and 2 with agenesis of one lung, and other causes in 1 patient. Previous surgery was performed in 6 (29%) patients. Patch tracheoplasty was performed using autologous pericardial patch and external stenting using costal cartilage. Major complications were mediastinitis and patch dehiscence in 2 patients, 2 patients needed tracheal cannula and 1 patient had stent implantation. Three (14%) patients died in the late postoperative period: 1 patient died of sepsis, 1 had patch dehiscence and 1 erosion of tracheal stent and consequently intractable bleeding. Follow-up was 6.1±2.7 years (0.75-10 years). At follow-up, 2 (11%) patients were still symptomatic, 4 (22%) had occasionally mild symptoms and 12 (67%) were free of symptoms., Conclusions: Treatment for severe tracheal stenosis remains challenging. With tracheoplasty using autologous pericardial patch and strips of costal cartilage, long and narrow tracheal stenosis can be repaired. There are no limitations as to the length and location and severity of the stenosis. Tracheoplasty is associated with a high complication rate. A multidisciplinary approach is mandatory to ensure favourable long-term outcomes., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Management of pulmonary atresia with intact ventricular septum (PAIVS) is challenging and depends on the individual patient's morphologic characteristics. We evaluated outcomes of 25 years of experience in morphologically driven management of PAIVS., Methods: Between January 1985 and December 2011, 60 patients were treated for PAIVS. Patients were retrospectively categorized into mild (n=27), moderate (n=18), and severe (n=15) right ventricular (RV) hypoplasia subgroups based on morphologic characteristics at first presentation. Tricuspid valve (TV) annulus z score increases were analyzed in patients who underwent biventricular repair and in patients who underwent 1.5-ventricular repair. Median follow-up time was 9.8 years (range, 0-25 years). One patient was lost to follow-up., Results: Fifty-nine (98.3%) patients underwent operation. There were 7 early postprocedure deaths and 1 late death. Estimated overall survival was 86.5% at 10 years after initial admission for the total group, and 96.3%, 77.8%, and 79.4% for the subgroups, respectively. In the respective subgroups, biventricular repair was achieved in 24 (88.9%), 3 (16.7%), and 0 patients. In the patients who underwent biventricular repair, there was a significant increase of the TV annulus z score. At latest follow-up, mean New York Heart Association (NYHA) class was 1.3 for the total group and 1.1, 1.2, and 1.5 for the patients who underwent biventricular, 1.5-ventricular, and univentricular repair, respectively., Conclusions: Our individualized management approach to PAIVS showed good early and long-term results. Early decision on the management goal, determined by individual morphologic characteristics at presentation, is of paramount importance. In specific patients, however, taking into account RV development at a later point in time may improve outcome., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Radiofrequency catheter ablation (RFCA) of ventricular tachycardia (VT) in repaired Tetralogy of Fallot focuses on isthmuses in the right ventricle but may be hampered by hypertrophied myocardium or prosthetic material. These patients may benefit from ablation at the left side of the ventricular septum., Methods and Results: Records from 28 consecutive repaired Tetralogy of Fallot patients from 2 centers who underwent VT ablation were reviewed. Ablation targeted anatomic isthmuses containing VT re-entry circuits, which were identified by 3-dimensional substrate, pace, and entrainment mapping. A left-sided approach was considered beneficial if (1) right-sided RFCA failed, (2) part of the circuit was mapped to the left side, and (3) left-sided RFCA resulted in isthmus transection and prevention of VT induction. In 4 of 28 patients (52±13 years; 75% men), inducible for 1.5 (quartiles, 1.0 - 2.0) VTs (335±58 ms), left-sided RFCA was performed. In 3 patients, RFCA at aortic sites terminated VT related to a septal isthmus and prevented reinduction. In 1 patient, with prior biventricular implantable cardioverter-defibrillator, diastolic activity was recorded at the left side of the septum in proximity to the His-bundle. RFCA prevented VT reinduction with anticipated complete atrioventricular block. The left-sided approach resulted in complete procedural success (transection of anatomic isthmus and noninducibility) and freedom of VT recurrence during follow-up (20±15 months) in all patients. Right-sided RFCA failure was likely because of septal hypertrophy in 2, overlying pulmonary homograft in 1, and overlying ventricular septal defect patch in 1., Conclusions: Left-sided RFCA for VTs dependent on septal anatomic isthmuses improves ablation outcome in repaired Tetralogy of Fallot., (© 2014 American Heart Association, Inc.)

Objective: After the arterial switch operation (ASO), disproportional neo-aortic growth during childhood has been reported. Even though it has been suggested neo-aortic dilation will stabilise in adulthood, data are lacking. The aim of this study was to assess the change in neo-aortic dimensions, prevalence of neo-aortic dilation >40 mm and long-term outcome in adults who underwent ASO in childhood., Methods: All 116 ASO patients operated in a tertiary referral centre and born before 1995 were included. Of these, 83 (72%) survived to adulthood (>17 years) and six were lost to follow-up. Neo-aortic measurements performed in adulthood were collected from available echocardiographic, cardiovascular magnetic resonance and CT images. The time trend was analysed using a mixed model, adjusted for imaging modality., Results: Clinical data with at least one measurement of the neo-aortic diameter were available in 77 (93%) adult patients and serial measurements in 65 (78%). At baseline (median age 18.1 years), mean neo-aortic diameter was 36±5 mm. Mean neo-aortic growth was 0.31 mm/year (p<0.001 compared with normal value 0.08 mm/year) and was linear over time. Freedom from neo-aortic dilation beyond a diameter of 40 mm was 23% at 28 years of age. During a mean clinical follow-up in adulthood of 7.2 years (IQR 4.0 to 10.1), 3 (4%) patients underwent neo-aortic replacement. No other neo-aortic complications occurred., Conclusions: In early adulthood, neo-aortic growth was on average linear and did not stabilise over time., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objective: Recent descriptions of decreased exercise capacity 10 to 15 years after arterial switch operation (ASO) suggest subclinical hemodynamic restrictions. Persistent impairment of ventricular performance following ASO may add to this. We aimed to characterize the time course of changes in biventricular performance within the first year following ASO., Methods: We prospectively included 26 patients with transposition of the great arteries undergoing ASO and 20 age-matched controls. Left and right ventricular systolic and diastolic performance was assessed using tissue Doppler imaging-derived peak systolic velocity, peak diastolic velocity, and peak early wave Doppler flow velocity/early diastolic tissue Doppler imaging velocity as well as mitral and tricuspid annular plane systolic excursion. Furthermore, left ventricular longitudinal, radial, and circumferential strain were assessed using speckle tracking strain imaging. Studies were performed preoperatively, 1 day postoperatively, at discharge, and at medium-term follow-up (9 months [interquartile range, 6-23 months] postoperatively)., Results: After an initial decrease in biventricular systolic and diastolic performance 1 day postoperatively versus preoperatively, recovery was observed in all parameters during medium-term follow-up. At medium-term follow-up left ventricular systolic and diastolic performance parameters were comparable in patients and controls. In contrast, right ventricular systolic and diastolic performance were still impaired in patients versus controls roughly 1 year postoperatively (tricuspid annular plane systolic excursion, 11.6 ± 2.2 vs 18.6 ± 3.1 mm; right ventricular peak systolic velocity, 8.1 ± 2.3 vs 12.6 ± 1.8 cm/second; right ventricular peak diastolic velocity, 12.4 ± 3.0 vs 18.2 ± 4.2 cm/second; and right ventricular peak early wave Doppler flow velocity/early diastolic tissue Doppler imaging velocity, 6.7 ± 2.1 vs 4.3 ± 1.3; all Ps < .001)., Conclusions: If early ASO is performed, left ventricular performance recovers to control values within the first postoperative year. In contrast, right ventricular systolic and diastolic performance remained impaired during follow-up, which stresses the importance of postoperative follow-up of right ventricular performance., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Patient satisfaction with care has received little attention within the field of congenital heart disease. Our objective was to examine patient satisfaction with the care received when undergoing open-heart surgery in order to identify the best and worst aspects of peri-operative care. Moreover, we examined whether having contact with a specialised nurse in addition to usual care is associated with higher patient satisfaction levels., Methods: Patient satisfaction was measured by the Satisfaction with Hospital Care Questionnaire, evaluating nine aspects of care by answering individual items and giving overall grades. A top 10 of the best and worst items was selected. Linear regression analyses were used to examine the relationship between having contact with a specialised nurse and patient satisfaction (9 grades), independent of patient characteristics--sex, age, educational level, and health status., Results: Data were available for 75 patients. Grades ranged from 6.74 for "discharge and after care" to 8.18 for "medical care". In all, 21% of patients were dissatisfied with the clarity of the information about lifestyle adjustments given by the surgeon. However, patients who had contact with a specialised nurse were more satisfied with the provided information (B-coefficient is 0.497, p-value is 0.038), independent of patient characteristics., Conclusions: Patients were satisfied with the received care, although there is room for improvement, especially in discharge and after care and the clarity of the information provided by the surgeon. This gap in care can be compensated for by specialised nurses, as patients who were counselled by a specialised nurse were more satisfied with the provided information.

Objectives: Right ventricular (RV) failure after cardiac surgery is a clinical entity with high morbidity and mortality. Patients with congenital heart disease (CHD) often undergo right-sided cardiac surgery. The authors aimed to identify determinants of RV failure after cardiac surgery to differentiate patients with increased risk., Design: A retrospective chart review., Setting: University hospital., Participants: Adults with CHD operated on between January 2001 and January 2011., Interventions: Clinical characteristics, laboratory tests, surgical data, and intensive care unit outcome were obtained from medical records., Measurements and Main Results: The diagnosis of clinical RV failure was made by careful review of the medical records by 2 independent physicians. Patients only were identified as having RV failure if (1) they had elevated jugular venous pressure, (2) they had impaired postoperative RV function on transthoracic echocardiography, and (3) a diagnosis of RV failure was documented clearly in the medical charts by the treating physician. Data of 412 consecutive patients (median age 36 [range 18-74] years, 56% male) were studied. Eighteen patients had clinical RV failure (4.4%) postoperatively, of whom 6 patients died. Patients undergoing left- and both-sided surgery had an equal risk of developing clinical RV failure as compared with patients undergoing right-sided surgery. In multivariate logistic regression analysis, preoperative impaired RV function, supraventricular tachycardia, and cardiopulmonary bypass time >150 minutes were the strongest determinants of clinical RV failure (p<0.05, for all)., Conclusions: RV failure after cardiac surgery is a serious complication, and occurs regardless of the side of surgery. A tailored approach in patients with CHD at highest risk of RV failure should be considered., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Cardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time course of ventricular performance deterioration and restoration after correction of a congenital heart defect (CHD) in pediatric patients is sparse. Therefore, the authors perioperatively quantified left ventricular (LV) and right ventricular (RV) performance using echocardiography. Their study included 141 patients (ages 0-18 years) undergoing CHD correction and 40 control subjects. The study assessed LV systolic performance (fractional shortening) and diastolic performance (mitral Doppler flow) in combination with RV systolic performance [tricuspid annular plane systolic excursion (TAPSE)] and diastolic performance (tricuspid Doppler flow). Additionally, systolic (S') and diastolic (E', A', E/E') tissue Doppler imaging (TDI) measurements were obtained at the LV lateral wall, the interventricular septum, and the RV free wall. Echocardiographic studies were performed preoperatively, 1 day postoperatively, and at hospital discharge after 9 ± 5 days. Although all LV echocardiographic measurements showed a deterioration 1 day after surgery, only LV TDI measurements were impaired in patients at discharge versus control subjects (S': 5.7 ± 2.0 vs 7.1 ± 2.7 cm/s; E': 9.8 ± 3.9 vs 13.7 ± 5.1 cm/s; E/E': 12.2 ± 6.4 vs 8.8 ± 4.3; p < 0.05). In the RV, TAPSE and RV TDI velocities also were impaired in patients at discharge versus control subjects (TAPSE: 9 ± 3 vs 17 ± 5 mm; S': 5.2 ± 1.7 vs 11.4 ± 3.4 cm/s; E': 7.3 ± 2.5 vs 16.3 ± 5.2 cm/s; E/E': 12.5 ± 6.8 vs 4.8 ± 1.9; p < 0.05). Furthermore, longer aortic cross-clamp times were associated with more impaired postoperative LV and RV performance (p < 0.05). In conclusion, both systolic and diastolic biventricular performances were impaired shortly after CHD correction. This impairment was detected only by TDI parameters and TAPSE. Furthermore, a longer-lasting negative influence of cardiopulmonary bypass on myocardial performance was suggested.

Background: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients. The aim of the current study was to determine the role of residual PS in the need for PVR during follow-up in cToF patients., Methods: cToF patients were included if clinical follow-up after primary surgical correction had taken place for a minimum of 5 years. Patient characteristics, surgical factors, and postoperative factors were reviewed, with a special focus on the transpulmonic systolic gradient. Cox proportional hazards regression analysis was performed to identify predictors of PVR., Results: Of 171 cToF patients, 71 (41.5%) underwent PVR after 24.2 years (interquartile range, 16.8-31.6 years). Year of birth, older age at corrective operation, and patch use significantly predicted PVR during follow-up. By contrast, a mild residual PS in cToF patients (peak systolic gradient, 15-30 mm Hg) independently reduced the risk of PVR, as compared with patients without PS (hazard ratio, 0.47; p=0.02) and with moderate-to-severe PS (hazard ratio, 0.35; p=0.01)., Conclusions: In addition to the known risks factors for PVR, a postoperative mild residual PS reduces the risk of PVR during follow-up of cToF patients. This finding provides clinical evidence for a conservative PS relief during correction of ToF., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Low cardiac output syndrome is common after paediatric cardiac surgery. Previous studies suggested that hydrocortisone administration may improve haemodynamic stability in case of resistant low cardiac output syndrome in critically ill children. This study was set up to test the hypothesis that the effects of hydrocortisone on haemodynamics in children with low cardiac output syndrome depend on the presence of (relative) adrenal insufficiency., Methods: A retrospective study was done on paediatric patients who received hydrocortisone when diagnosed with resistant low cardiac output syndrome after paediatric cardiac surgery in the period from 1 November 2005 to 31 December 2008. We studied the difference in effects of treatment with hydrocortisone administration between patients with adrenal insufficiency defined as an exploratory cut-off value of total cortisol of <100 nmol/l and patients with a serum total cortisol of ≥ 100 nmol/l., Results: A total of 62 of patients were enrolled, meeting the inclusion criteria for low cardiac output syndrome. Thirty-two patients were assigned to Group 1 (<100 nmol/l) and 30 were assigned to Group 2 (≥ 100 nmol/l). Haemodynamics improved after hydrocortisone administration, with an increase in blood pressure, a decrease in administered vasopressors and inotropic drugs, an increase in urine production and a decrease in plasma lactate concentrations., Conclusions: The effects of treatment with hydrocortisone in children with low cardiac output after cardiac surgery was similar in patients with a low baseline serum cortisol concentration and those with normal baseline cortisol levels. A cortisol value using an exploratory cut-off value of 100 nmol/l for adrenal insufficiency should not be used as a criterion to treat these patients with hydrocortisone.

Right ventricular function (RVF) is often selectively declined after coronary artery bypass graft surgery. In adult patients with congenital heart disease (CHD) the incidence and persistence of declined RVF after cardiac surgery is unknown. The current study aimed to describe RVF after cardiac surgery in these patients. Adult CHD patients operated between January 2008 and December 2009 in the Academic Medical Centre in Amsterdam were studied. Clinical characteristics, laboratory tests, surgical data and intensive care unit outcome were obtained from medical records. RVF was measured by trans-thoracic echocardiography (TTE) and expressed by tricuspid annular plane systolic excursion (TAPSE), tissue Doppler imaging (RV S') and myocardial performance index (MPI) pre-operatively and direct, at intermediate and late follow up. Of a total of 185 operated, 86 patients (mean age 39 ± 13 years, 54% male) had echo data available. There was a significant fall in RVF after cardiac surgery. TAPSE and RV S' were significantly higher and MPI was significantly lower pre-operatively compared to direct post-operative values (TAPSE 22 ± 5 versus 13 ± 3 mm (P < 0.01), RV S' 11 ± 4 versus 8 ± 2 cm/s (P < 0.01) and MPI 0.36 ± 0.14 vs 0.62 ± 0.25; P < 0.01). There were no significant differences in left ventricular function pre-operatively compared to post-operative values. Right-sided surgery was performed in 33, left-sided surgery in 37 and both sided surgery in 16 patients. Decline in RVF was equal for those groups. Patients with severe decline in RVF, were patients who underwent tricuspid valve surgery. Decline in RVF was associated with post-operative myocardial creatine kinase level and maximal troponin T level. There was no association between decline in RVF and clinical outcome on the intensive care unit. 18 months post-operatively, most RVF parameters had recovered to pre-operative values, but TAPSE which remained still lower (P < 0.01). CHD patients have a decline in RVF directly after cardiac surgery, regardless the side of surgery. Although a gradual improvement was observed, complete recovery was not seen 18 months post-operatively.

Objective: Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. Clinical outcomes of biventricular repair seem favorable to univentricular palliation, but data on superiority of biventricular repair regarding exercise capacity are conflicting. We investigated the response to physical and pharmacologic stress in patients with surgically corrected pulmonary atresia with intact ventricular septum., Methods: Sixteen patients (7 patients after univentricular palliation, age 11.8 ± 2.6 years; 7 patients after biventricular repair, age 12.9 ± 2.7 years; and 2 patients after 1.5 ventricular repair, age 12 and 19 years) underwent cardiopulmonary exercise test, dobutamine stress magnetic resonance imaging, and delayed contrast-enhanced magnetic resonance imaging., Results: The univentricular group showed impaired exercise capacity in contrast with normal exercise capacity in the biventricular group. Left ventricular ejection fraction increased in both groups. With dobutamine, left ventricular stroke volume increased only in the biventricular group (+11.3 ± 5.0 mL, P < .001) and not in the univentricular group (-0.04 ± 3.6 mL, P = .9). Heart rate increase was inadequate in the univentricular group. Maximum oxygen consumption and oxygen pulse were strongly correlated with left ventricular stroke volume during stress but not at rest. The results of the 2 patients after 1.5 ventricular repair were comparable to those of the univentricular group. No myocardial fibrosis was detected., Conclusions: Impaired exercise capacity in children and adolescents with pulmonary atresia with intact ventricular septum after univentricular palliation is related to decreased cardiac reserve and inadequate chronotropic response. Young patients with pulmonary atresia with intact ventricular septum after biventricular repair show normal exercise capacity and cardiac reserve., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Neo-coarctation following arterial switch operation (ASO) for transposition of the great arteries (TGA) is a complication that is not regularly described, but may occur. We describe five patients who developed a neo-coarctation after operation. They were diagnosed with TGA, either with or without ventricular septal defect without signs or symptoms of a coarctation. Except for one patient, all patients were reoperated for a neo-coarctation within one year after the ASO. Several explanations are discussed as a possible cause for this phenomenon.

Background: We evaluated the results of the arterial switch operation (ASO) being performed at our institution for more than 30 years and identified risk factors for mortality and reoperation., Methods: Clinical outcome of 332 consecutive patients with transposition of the great arteries undergoing ASO was retrospectively analyzed, using surgical reports, medical charts, and latest follow-up echocardiography. Statistical analysis was performed using the Kaplan-Meier method and univariable and multivariable binary logistic and Cox regression analyses., Results: In-hospital mortality was 11.4%. At 15 years, estimated overall survival was 85.2%, and estimated freedom from reoperation was 74.0%. Cross-clamp time (p=0.001) and absence of the Lecompte maneuver (p=0.001) were identified as independent risk factors for in-hospital mortality, whereas coronary problems during surgery (p=0.009) and postoperative pacemaker implantation (p<0.001) were independent risk factors for late mortality. Independent risk factors for reoperation were higher age at the time of the ASO (p=0.002), presence of arch abnormalities (p<0.001), coronary problems during surgery (p=0.005), and duration of ventilation (p<0.001). At latest echocardiography, moderate or severe neoaortic regurgitation was present in 3.4% of the patients., Conclusions: Overall, 30 years of experience with the ASO shows good survival and event-free survival rates. Coronary transfer problems during surgery were found to be an important risk factor for late mortality and reoperation. However, coronary anatomy other than 1LCx-2R and an intramural course of the left coronary artery or left anterior descending artery were not risk factors for mortality or reoperation. Neoaortic regurgitation does not seem to form a major problem., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We report on an uncommon case of a 10-month-old patient who required early surgical closure of an ostium secumdum atrial septal defect due to the concomitant presence of a big subcarinal bronchogenic cyst compressing the left atrium and, therefore, increasing the left-to-right shunt. It led to refractory congestive heart failure symptoms, establishing thereby an earlier indication of surgical treatment.

Background: This study evaluated the diameters and distensibility of the aortic root as well as the degree of aortic regurgitation (AR) and its effect on left ventricular (LV) function in patients 8.2 +/- 3.1 years after they underwent the Ross procedure, with a comparison of these parameters between patients and matched healthy subjects., Methods: Eighteen Ross procedure patients (16 male patients, age [mean +/- SD] 19.2 +/- 3.8 years) and 18 matched healthy subjects (16 male patients, age [mean +/- SD] 19.7 +/- 4.2 years) underwent magnetic resonance imaging. Measurements for diameters (at 4 levels) and the distensibility of the aortic root were performed using a steady-state free precession sequence. Aortic flow was assessed with a velocity-encoded phase-contrast sequence. Left ventricular systolic function was assessed with a gradient-echo sequence in the short-axis plane. Comparison of parameters was performed using the Mann-Whitney U test. Correlations between diameters, distensibility, AR fraction, and LV systolic function were expressed with Spearman rank correlation coefficients. Linear regression analysis was used to identify predictors of LV systolic dysfunction., Results: Aortic root diameters were increased in Ross procedure patients as compared with healthy subjects (mean difference 6.3-11.6 mm, P < or = .02 at all 4 levels). Distensibility of the aortic root was lower in patients (1.9 +/- 1.1 vs 7.8 +/- 3.3 mm Hg(-1), P < .01). An AR fraction > 5% was present in 14 of the 18 patients (mean AR fraction 8% +/- 5% vs 1% +/- 1%, P < .01). Left ventricular ejection fraction was lower in patients (50% +/- 6% vs 57% +/- 6%, P < .01). Dilatation, decreased distensibility, and AR fraction were correlated with impaired LV systolic function (P < .05 for all). The AR fraction predicted impaired LV systolic function (P < .01)., Conclusions: Magnetic resonance imaging shows dilatation and decreased distensibility of the aortic root, AR, and consequent impaired LV systolic function in patients after the Ross procedure.