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Open in new tab Download slide OBJECTIVES Congenital supravalvular aortic stenosis (SVAS) is a rare form of congenital outflow tract obstruction and long-term outcomes are scarcely reported. This study aims to provide an overview of outcomes after surgical repair for congenital SVAS. METHODS A systematic review of published literature was conducted, including observational studies reporting long-term clinical outcome (>2 years) after SVAS repair in children or adults considering >20 patients. Early risks, late event rates and time-to-event data were pooled and entered into a microsimulation model to estimate 30-year outcomes. Life expectancy was compared to the age-, sex- and origin-matched general population. RESULTS Twenty-three publications were included, encompassing a total of 1472 patients (13 125 patient-years; pooled mean follow-up: 9.0 (6.2) years; median follow-up: 6.3 years). Pooled mean age at surgical repair was 4.7 (5.8) years and the most commonly used surgical technique was the single-patch repair (43.6%). Pooled early mortality was 4.2% (95% confidence interval: 3.2–5.5%) and late mortality was 0.61% (95% CI: 0.45–0.83) per patient-year. Based on microsimulation, over a 30-year time horizon, it was estimated that an average patient with SVAS repair (mean age: 4.7 years) had an observed life expectancy that was 90.7% (95% credible interval: 90.0–91.6%) of expected life expectancy in the matched general population. The microsimulation-based 30-year risk of myocardial infarction was 8.1% (95% credible interval: 7.3–9.9%) and reintervention 31.3% (95% credible interval: 29.6–33.4%), of which 27.2% (95% credible interval: 25.8–29.1) due to repair dysfunction. CONCLUSIONS After surgical repair for SVAS, 30-year survival is lower than the matched-general-population survival and the lifetime risk of reintervention is considerable. Therefore, lifelong monitoring of the cardiovascular system and in particular residual stenosis and coronary obstruction is recommended. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Our goal was to report the long-term serial follow-up after transatrial–transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome. METHODS We included all patients with TOF who had undergone transatrial–transpulmonary repair between 1970 and 2012. Records were reviewed for patient demographics, operative details and events during the follow-up period (death, pulmonary valve replacement, cardiac reinterventions and hospitalization/intervention for arrhythmias). In patients with elective early primary repair of TOF after 1990, a subanalysis of the optimal timing of TOF repair was performed. RESULTS A total of 453 patients were included (63% male patients; 65% had transannular patch); 261 patients underwent primary elective repair after 1990. The median age at TOF repair was 0.7 years (25th–75th percentile 0.3–1.3) and decreased from 1.7 to 0.4 years from before 1990 to after 2000, respectively (P  < 0.001). The median follow-up duration after TOF repair was 16.8 years (9.6–24.7). Events developed in 182 (40%) patients. In multivariable analysis, early repair of TOF (<6 months) [hazard ratio (HR) 3.06; P  < 0.001] and complications after TOF repair (HR 2.18; P  = 0.006) were found to be predictive for an event. In a subanalysis of the primary repair of TOF after 1990, the patients (n  = 125) with elective early repair (<6 months) experienced significantly worse event-free survival compared to patients who had elective repair later (n  = 136). In multivariable analysis, early repair (HR 3.00; P  = 0.001) and postoperative complications (HR 2.12; P  = 0.010) were associated with events in electively repaired patients with TOF. CONCLUSIONS Transatrial–transpulmonary repair of TOF before the age of 6 months may be associated with more events during the long-term follow-up period. [ABSTRACT FROM AUTHOR]

View large Download slide View large Download slide OBJECTIVES In patients with the Fontan circulation, systemic venous return flows passively towards the lungs. Because of the absence of the subpulmonary ventricle, favourable blood flow patterns with minimal energy loss are clinically relevant. The region where the inferior vena cava, the hepatic veins and the extracardiac conduit join (IVC–conduit junction) is a potential source of increased energy loss. The aim of this study was to evaluate the relationship between geometry and blood flow patterns in the IVC–conduit junction with associated kinetic energy and energy loss using 4-dimensional flow magnetic resonance imaging (MRI). METHODS Fourteen extracardiac conduit-Fontan patients underwent 4-dimensional flow MRI. The IVC–conduit junctions were ranked into 3 groups for 3 categories: the geometry, the flow complexity and the conduit mean velocity. The relative increase in the mean velocity from the IVC to the conduit (representing IVC–conduit mismatch) was determined. The peak kinetic energy and mean kinetic energy and energy loss were determined and normalized for volume. RESULTS In 4 of 14 patients, adverse geometries led to helical flow patterns and/or acute changes in flow direction. For each category, the most adverse IVC–conduit junctions were associated with an approximate 2.3–3.2-fold and 2.0–2.9-fold increase in kinetic energy and energy loss, respectively. The IVC–conduit mismatch is strongly correlated with the mean kinetic energy and energy loss (r   = 0.80, P   =  0.001 and ρ  = 0.83, P  <  0.001, respectively) and with body surface area in patients with 16- mm conduits (r   =  0.88, P   =  0.010). CONCLUSIONS The IVC–conduit junction is a potential source of increased energy loss. Junctions with increased energy loss showed: (i) a distorted geometry leading to adverse blood flow patterns and/or (ii) the IVC–conduit mismatch. Sixteen-millimetre conduits appear to be inadequate for older patients. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Rhythm disturbances are an important cause of morbidity in Fontan patients. Currently, the total cavopulmonary connection is performed by using the intra-atrial lateral tunnel (ILT) ('baffle ILT' or 'prosthetic ILT'), or the extracardiac conduit (ECC). The aim of the study was to evaluate rhythm abnormalities and compare the surgical techniques in a contemporary cohort. METHODS: In a cross-sectional multicentre study, 115 patients (age 12.5 ± 3.1 years) underwent rhythm evaluation using ECG, exercise testing and Holter, including heart rate variability. Medical history was reviewed for episodes of arrhythmia. RESULTS: Sinus node dysfunction (SND) was found in 29%, 3 of whom required pacemaker therapy. No difference was found in the incidence of SND between ILT and ECC patients. Sinus pauses occurred only in the ILT group. Exercise testing showed no difference in peak heart rate between the groups. Heart rate reserve (P = 0.023) and heart rate recovery (HRrecovery) (P < 0.001) were lower in ILT patients. Atrial arrhythmias were more common in ILT patients (15 vs 1%, P = 0.004), but only in those with a baffle ILT. One patient had symptomatic ventricular tachycardia (VT). Holter recordings showed sub-clinical VT in 6% of patients, which was associated with larger end-diastolic (P = 0.035) and end-systolic volumes (P = 0.029). CONCLUSIONS: The overall incidence of arrhythmia was low, although SND was frequently present in both Fontan groups. ILT patients had slower HRrecovery, and ILT patients with the more extensive baffle technique had more atrial arrhythmias and more sinus pauses. The significance of asymptomatic ventricular arrhythmias in this young population remains to be determined. [ABSTRACT FROM AUTHOR]

Sub-valvular aortic stenosis (SAS) is a common form of left ventricular outflow tract (LVOT) obstruction, which can lead to aortic valve damage. Although surgery for SAS is an accepted treatment, the timing of surgical intervention of SAS remains controversial. This review aims to establish an overview of the natural history and outcome after surgery and factors associated with prognosis in paediatric SAS patients. We searched PubMed and EMBASE for studies that reported factors that negatively affected the prognosis of patients with SAS. Studies were included if they were written in English, published between 1 January 1997 and 31 December 2012 and the mean patient age was <18 years at the time of study entry. Studies were excluded if the study size was <20 patients. A distinction was made between natural history and surgical cohorts. Twenty-four studies were included in this review, encompassing a total of 809 natural history and 1476 surgical patients. Fifty-one percent of natural history patients required surgery. After surgery, there was a substantial reoperation rate. Higher LVOT gradient and the presence of aortic regurgitation (AR) were identified as the foremost independent predictors of a worse outcome. Valve-to-membrane distance was also found to be associated with prognosis, although the results were contradictory. This systematic review underlines the importance of LVOT gradient, aortic valve-to-membrane distance and AR in surgical decision-making in paediatric SAS patients. There is need for collaborative effort to further study the optimal timing of surgery based on LVOT gradient, valve-to-membrane distance and the presence of AR. [ABSTRACT FROM AUTHOR]

OBJECTIVES The surgical approach to repair of tetralogy of Fallot (ToF) has shifted over the years. We aimed to report the long-term follow-up after ToF repair with the transatrial–transpulmonary approach and to determine predictors of long-term outcomes. METHODS Retrospective analysis of patients operated on in two tertiary referral centres. Primary outcome measures were: death, pulmonary valve replacement (PVR), reintervention for other reasons, internal cardiodefibrillator and/or pacemaker placement. Kaplan–Meier assessment of overall and event-free survival as well as uni- and multivariate analyses of risk factors for outcomes were performed. RESULTS Four hundred and fifty-three patients were included. Median age at operation was: 0.6 years (range 0–19.6) and median age at the last follow-up was 14.3 years (range 0.1–42.1). Median age at repair decreased from 1.2 years (range 0.6–5.8) (1970–80) to 0.3 years (range 0–4.7) (2000–12). A transannular patch (TP) was used in 65% of all patients. The use of a TP showed a decline from 89% in the initial years of the cohort to 64% in 2000–12. Early mortality was 1.1% (5 patients) for the entire cohort and late mortality 2.4% (11 patients). Overall survival for the entire cohort was 97.3% (95% CI 95.7–98.8) and 91.8% (95% CI 85.9–97.7) at 10 and 25 years, respectively. For patients with a TP (n = 294) vs non-TP (n = 159), this was 97.2% (95% CI 95.2–99.2) vs 97.5% (95% CI 95.1–99.9) at 10-year and 91.0% (95% CI 83.9–98.1) vs 96.3% (95% CI 93.0–99.6) at 25-year follow-up (P = 0.958). Fifty-two patients underwent PVR, and in 5 a pacemaker was inserted. Event-free survival for TP versus non-TP patients was 80.2% (95 CI% 75.5–84.9) vs 81.7% (95% CI 75.2–88.2) at 10-year and 27.9% (95% CI 17.7–38.1) vs 78.5% (95% CI 71.4–85.6) at 25-year follow-up (P = 0.016). In multivariate analysis, both the use of a TP (HR 1.705, 95% CI 1.023–2.842) and the year of surgical repair of tetralogy of Fallot (HR 1.039, 95% CI 1.006–1.073) were associated with a higher probability of an event. CONCLUSIONS ToF patients corrected with the transatrial–transpulmonary approach have good long-term survival. PVR is a frequent event at longer follow-up, and other events are limited. The use of a TP is a predictor for poorer event-free outcomes, increasing the risk of the composite endpoint 1.7 times. [ABSTRACT FROM PUBLISHER]

A response from the author of the article "Long-term outcomes of transatrial-transpulmonary repair of tetralogy of Fallot" in the 2015 issue is presented.