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Objective: To evaluate if development of fragmented QRS (fQRS) complexes, a marker of inhomogeneous ventricular activation due to myocardial fibrosis, is associated with adverse outcome in adults after Mustard/Senning repair for d-transposition of the great arteries (d-TGA).Methods: Adults with atrial switch repair for d-TGA were selected from the database of a tertiary care hospital. Exclusion criteria were systemic right ventricular (RV) assist device or heart transplantation (HTx) before the age of 16, or fQRS already present at first visit to the Adult Congenital Heart Disease clinic. A blinded expert reader retrospectively analysed all available ECGs after the age of 16 for the presence of fQRS. The appearance of fQRS was modelled for each patient as a time-dependent variable. Cox regression was performed to assess the relationship between covariates and the composite endpoint of cardiovascular mortality, HTx or systemic RV assist device.Results: Records of 89 patients (34% female, 42% Mustard repair) were analysed. At latest follow-up, fQRS was noted in 26 patients (29%). Over a median follow-up time of 16.9 (IQR 12.6-22.9) years, the composite endpoint occurred in nine patients (10%). In multivariable Cox analysis, appearance of fQRS (HR 14.11; 95% CI 1.42 to 140.12) and development of severe RV dysfunction (HR 11.36; 95% CI 2.08 to 62.17) were significantly associated with the composite endpoint.Conclusions: Appearance of fQRS complexes on a 12-lead ECG is associated with adverse outcome in adults after atrial switch repair for d-TGA. In this population, fQRS detection might be a promising and easily implementable tool to identify patients at risk for adverse events. [ABSTRACT FROM AUTHOR]

Objectives: Studies evaluating the long-term outcome of adults with ventricular septal defect (VSD) are important to inform patients about prognosis. This study investigated the long-term outcome of patients with perimembranous VSD (pmVSD) followed in the Belgian Registry on Adult Congenital Heart Disease. Methods: All pmVSD patients in the registry were analyzed. Results: Two hundred and sixty-six patients were studied. Fifteen patients had Eisenmenger syndrome. One hundred and seventy-three had isolated pmVSD and 78 had pmVSD with concomitant lesions. Of the patients with isolated pmVSD, 52% were male, median age was 29 years (IQR 24-35 years) and median follow-up duration was 18 years (IQR 10-25 years). Fifty-three (31%) patients underwent VSD closure and 10 (19%) had a residual shunt. Most (93%) patients were in NYHA class I. No patients died. Two (4%) patients developed atrial arrhythmia and 2 (4%) re-quired pacemaker implantation. Seven (14%) developed left ventricular outflow tract obstruction (LVOTO). In the unrepaired pmVSD group, 4 developed endocarditis. In the entire group, moderate or severe aortic regurgitation (AR) occurred in 9 (5%) patients. Conclusions: Long-term survival in patients with isolated pmVSD was not uneventful. Moderate or severe AR might develop and endocarditis occurred in patients without VSD repair. Complications after VSD closure included atrial arrhythmia, pacemaker implantation and LVOTO. [ABSTRACT FROM AUTHOR]

Objective The clinical benefit of tricuspid annuloplasty (TA) in patients undergoing mitral valve surgery (MVS) is still debated. We evaluated the immediate surgical success, postoperative outcome and the medium-term effect of TA in MVS. Methods Patients were included between September 2003 and December 2009 and followed until September 2013 to achieve a median follow-up time of 5 years (IQR 3.7-6.9). The end point of mortality due to cardiac causes and combined end point of cardiac mortality or hospitalisation for heart failure were evaluated. Propensity score adjusted Cox regression was used to evaluate the clinical benefit of TA at the time of MVS. Results Of 150 patients (84 female; 67±12 years), 82 presented with tricuspid regurgitation (TR) <2/4 and underwent isolated MVS. Of 68 patients presenting with TR≥2/4, 31 underwent isolated MVS whereas 37 underwent additional TA. In patients with preoperative TR≥2/4, TR was significantly reduced until 5 years postoperatively (mean reduction 0.81±1.31; p=0.04) when additional TA was done. The combined end point occurred in 29% vs 6% at 1 year and in 57% vs 39% at 5 years follow-up for patients with isolated MVS and patients undergoing concomitant TA, respectively. Patients with preoperative TR≥2/4 had worse unadjusted survival than those with TR<2/4 (logrank p=0.009). In the patients with TR≥2/4, propensity score-adjusted risk for the combined end point was higher in those with isolated MVS versus MVS with additional TA (Cox HR 2.855 (1.082-7.532), p=0.035). Conclusions Additional TA is an effective surgical measure to reduce functional TR severity. This approach results in a decreased risk of cardiac mortality and hospitalisation in patients with preoperative TR≥2/4. [ABSTRACT FROM AUTHOR]

Aims Patients at risk of pulmonary arterial hypertension (PAH)may present with abnormal dynamic pulmonary vascular resistance (PVR) during exercise. However, its clinical significance remains unclear. The present study aimed at analysing the meaning of dynamic PVR in two populations at risk of PAH: secundum atrial septal defect (ASD) and systemic sclerosis (SSc). Methods and results Adult patients with corrected ASD were consecutively selected from the database of Pediatric and Congenital Heart Disease of the University Hospitals Leuven. Patients with SSc were consecutively selected from the rheumatology database of the University Hospital Liège. At inclusion, all underwent a rest and bicycle stress echocardiography to obtain baseline right heart characteristics and dynamic PVR. Routine follow-up echocardiography was performed. Twenty-eight patients with corrected ASD (mean age 41±17 years, 79% female) were followed for a median time of 3.7 [inter-quartile range (IQR) 2.9-4.1] years. No patient developed PAH. Dynamic PVR was significantly associated with right atrial dilatation at latest follow-up (Spearman's ρ 0.51, P = 0.013). Forty-five SSc patients (mean age 54±13 years, 76% female) were followed for a median time of 2.4 (IQR 0.8-2.9) years. Thirteen patients (30%) developed PAH. Dynamic PVR was the only independent predictor of PAH (hazards ratio 1.22, 95% confidence interval 1.01- 1.47). No significant right heart morphometric changes occurred. Conclusion Dynamic PVR predicted PAH development in patients with SSc, whereas dynamic PVR was associated with right heart morphometric changes after ASD closure. The predictive role of dynamic PVR might depend on the underlying disease type. Larger studies are needed to confirm this hypothesis. [ABSTRACT FROM AUTHOR]

Objective In adult patients with mild-to-moderate pulmonary valve (PV) stenosis, exercise capacity and haemodynamics have not been extensively studied, although regular exercise is recommended. Therefore, we aimed to assess exercise capacity to study the increase in PV gradient during exercise and to evaluate the impact of this increased pressure load on the RV. Methods Nineteen patients (8 female; 29±6.4 years) with isolated mild-to-moderate PV stenosis and no prior cardiac interventions were consecutively enrolled from the outpatient clinic of adult congenital heart disease. All patients underwent cardiopulmonary exercise testing, transthoracic echocardiography and bicycle stress echocardiography. Results for exercise testing were compared with age-matched and gender-matched control patients. Results In the studied population, resting heart rate (89±11 vs 75±14 bpm; p=0.001), peak power (199±66 vs 263±68 W; p=0.006); peak VO2 (31.2±9.9 vs 39 ±7.4 mL/kg/min; p=0.011); oxygen uptake efficiency slope (2430±913 vs 3292±943(mL/min)/(L/min); p=0.007) and VE/VCO2 slope (26.8±5.2 vs 22.6±4.3; p=0.01) differed significantly from controls. A linear increase of peak PV gradient with increasing flow was observed in the pooled dataset (Pearson's R=0.947; p<0.0001) and slopes identical as for control patients were obtained for the oxygen pulse-workload relationship. Right heart morphology and function were preserved in the studied patients. Conclusions Patients with mild-to-moderate PV stenosis have decreased exercise capacity. A linear increase in PV gradient with flow suggests a fixed valve area throughout the exercise. Although systolic RV pressure load increases during exercise, good ventricular performance was observed without signs of functional or morphological changes of the right heart. [ABSTRACT FROM AUTHOR]

Background When elevated pulmonary artery pressure (PAP) is assessed by echocardiography, right heart morphology is always considered. The goal of this study was to evaluate how right heart geometry changes with increasing right ventricular pressure load. Subjects and methods Data from patients undergoing transthoracic echocardiography with subsequent right heart catheterization within a time period of 6 months were retrospectively analysed. First, Spearman-rho coefficients between mean PAP and right heart parameters were calculated. Second, the population was divided into tertiles according to mean PAP and Kruskal-Wallis variance analysis between variables was performed. Results Fifty-four patients (23 female, median age 77 years, IQ range 63-83) were selected. Mean PAP (median 27mmHg, IQ range 24-36), right atrial (RA) dilatation (median 1, IQ range 0-2), tricuspid insufficiency (TI) severity (median 1.5, IQ range 0-2) and right ventricular (RV) dilatation (median 0, IQ range 0) were included. Significant correlations with mean PAP were found for RA dilatation (ρ=0.380; P=0.005) and TI severity (ρ = 0.294; P=0.032). No correlation with RV dilatation could be shown (ρ = 0.241; P=0.081). Across the tertiles [(1) mean PAP ≤ 25 mmHg; (2) mean PAP 26-30 mmHg; (3) mean PAP ≥ 31 mmHg)], variance analysis showed a significant increase in RA dilatation (P=0.009) and TI severity (P=0.040). No change in RV dilatation across groups was observed (P=0.216). Conclusions RA dilatation and TI severity significantly increase with increasing RV pressure load. No increase in RV dilatation was observed in the studied population. Hence, absence of RV dilatation does not exclude the presence of elevated PAP. [ABSTRACT FROM AUTHOR]

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 [ABSTRACT FROM AUTHOR]