Cardiovascular Sciences

Objective: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) usually results from a systemic-to-pulmonary shunt. Eisenmenger syndrome (ES) is characterised by severe irreversible PAH and reversal of a previous systemic-to-pulmonary shunt. A national registry of ES patients was initiated to optimise patient care and to provide epidemiological information regarding PAH and CHD in Belgium.

The number of adolescents and young adults with congenital heart defects, including ventricular septal defect (VSD), increases continuously. We evaluated the mid-term outcome of small and unclosed perimembranous VSDs (pmVSDs). All patients with a known unrepaired pmVSD at 16 years of age were selected from our database. The clinical, electrocardiographic, and echocardiographic changes between baseline and the latest follow-up examination were compared. A total of 220 patients (119 males, median age 18 years, interquartile range 7) could be included. During a median follow-up of 6 years (interquartile range 4, range 38), 2 patients died (1%; 1 from sudden death and 1 from end-stage heart failure). Endocarditis occurred in 8 patients (4%). One patient required pacemaker implantation (0.5%) and one required implantable cardioverter-defibrillator implantation (1%). Fifteen patients (7%) required a closing procedure. In 8 patients (4%), the pmVSD closed spontaneously. In the remaining 203 patients (93%), the QRS morphology changed in 5% and 1% lost sinus rhythm (p ‫؍‬ 0.0001 and p ‫؍‬ 0.015, respectively). The left ventricular ejection fraction and stroke volume index increased from 62 ؎ 7% to 67 ؎ 8% and from 41 ؎ 11 to 44 ؎ 15 ml/m 2 (p ‫؍‬ 0.0001 and p ‫؍‬ 0.035, respectively), the end-systolic diameter decreased, and the end-diastolic diameter did not change. Finally, patients with an open pmVSD developed more pulmonary arterial hypertension during follow-up (from 3% to 9%, p ‫؍‬ 0.002). In conclusion, mid-term follow-up of adolescents and young adults with a small and unrepaired pmVSD was not uneventful. Some patients required intervention, but in others, spontaneous closure occurred. Electrocardiographic and structural changes were noticed, for which the clinical significance needs to be determined.

Objectives: Ebstein's anomaly, characterized by an apical displacement of the tricuspid valve into the right ventricle, occurs in approximately 1/200,000 live births. Because longterm follow-up data of adults with Ebstein's anomaly are scarce, we evaluated the outcome of our Ebstein's anomaly patients. Methods: All patients 1 16 years and registered in the congenital heart disease database of our hospital with isolated Ebstein's anomaly were selected for the study. Records were reviewed for outcome. Results: Forty-nine patients (21 males, mean age at diagnosis 29.1 8 20.7 years) were followed for a mean time of 11.4 years (range 1.1-32.4). Twenty-five patients (51%) underwent tricuspid valve surgery (16 valvuloplasty and 9 valve replacement). Eight patients (32%) required redo tricuspid valve surgery. Twentysix patients (52.1%) exhibited supraventricular arrhythmia, and the typical Wolff-Parkinson-White syndrome occurred in 15 patients (31.2%). Seventeen patients (34.7%) underwent ablation therapy and 5 patients (10.4%) required pacemaker implantation. Conclusions: Half of the patients with Ebstein's anomaly needed tricuspid valve surgery and redo

Five (28%) needed reoperation. In the dcVSD closure group, left ventricular ejection fraction decreased from 69 8 12 to 61 8 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.

Background Closure of an atrial septal defect (ASD) mostly causes reverse remodelling of the right heart, a decrease in pulmonary artery pressures (PASP), and improves functional capacity. However, abolition of the left-to-right shunt may be unfavourable in older patients. This study aimed at evaluating patients older than 60 years who underwent ASD closure.

Aims: Atrial fibrillation and flutter remain an important cause of morbidity in adults with atrial septal defect (ASD). This study aimed at investigating predictors for late (≥1 month after repair) atrial arrhythmia. Methods: Patients who underwent ASD closure after the age of 18 years, were selected through the databases of three medical centres in Belgium. Preprocedural, periprocedural and follow-up data were extracted. Univariate and multivariate Cox-regression analysis was performed. Kaplan-Meier analysis was performed for any independent predictor of late atrial arrhythmia. Results: A total of 155 patients (38 men and 117 women) was included. Twenty-four patients (median age 48.3 years, range 19.9-79.8) underwent surgical and 131 (median age 57.6 years, range 18.2-86.9) underwent transcatheter closure. Thirty-nine patients (25.2%) presented with late atrial arrhythmia. Male gender (P= 0.008), creatinine (P= 0.002), atrial arrhythmia before (Pb 0.0001) and within 1 month after repair (P= 0.001) and a mean pulmonary artery pressure (mPAP) ≥25 mm Hg (Pb 0.0001) correlated with late atrial arrhythmia in univariate Cox-regression analysis. Multivariate analysis showed that mPAP ≥25 mm Hg (HR 3.72; 95%CI 1.82-7.59; P b 0.0001) and the presence of atrial arrhythmia before (HR 3.22; 95%CI 1.56-6.66; P = 0.002) and within 1 month after repair (HR 2.08; 95%CI 2.08-15.92; P = 0.001) were predictive of late atrial arrhythmia. Kaplan-Meier analysis showed that patients with a mPAP ≥25 mm Hg had a higher risk at developing late atrial arrhythmia (Pb 0.0001). Conclusion: In patients with ASD type secundum, a mPAP ≥25 mm Hg is an independent predictor of late atrial arrhythmia. The presence of pulmonary hypertension before repair should raise awareness for atrial arrhythmias and may be used to guide therapy.

Background: Few data are available on very long-term follow-up after treatment for isolated pulmonary valve stenosis (PVS), either surgically or by percutaneous balloon angioplasty (PBA). Methods and results: All patients with isolated PVS were selected from our database of congenital heart defects. Their records were reviewed systematically. We identified 79 surgically treated patients with a median follow-up of 22.5 years (range 0-45 years) and 139 PBA patients with median follow-up of 6.0 years (range 0-21 years). Echocardiographic and catheterization parameters indicate excellent results of both techniques in relieving the transpulmonary gradient. However, after initial surgery 20.3% of patients needed a cardiac re-intervention: 81% for severe pulmonary valve regurgitation, but none for residual pulmonary stenosis. After initial PBA a cardiac re-intervention was needed in 9.4% of patients. In 85% the indication was residual pulmonary stenosis, in none of them pulmonary regurgitation, although almost all patients developed a mild pulmonary regurgitation. Freedom of re-intervention after surgery was 98.4%, 93.5%, 87.7%, 70.9% and 55.7% at 5, 10, 20, 30 and 40 years postoperatively. Freedom of re-intervention in the PBA group was 95.1%, 87.5% and 84.4% at 5, 10 and 20 years post-procedure. Conclusions: Both surgery and PBA are safe and successful in relieving the acute transpulmonary gradient. Long-term results of surgery are worse than previously thought due to severe PR. After PBA re-interventions for residual stenosis are frequently needed and the incidence of mild PR is high. Very long-term results of PBA are still unknown.

Objectives: The value of the right heart morphology is not well established for the evaluation of elevated pulmonary artery pressure (PAP). We aimed (1) to assess the relationship between right heart morphology and PAP and (2) to evaluate whether morphology can help to exclude elevated PAP. Methods: From 11-2010 until 01-2011, 1640 consecutive patients were included from the database of echocardiography. Tricuspid regurgitation (TR) severity, right ventricular (RV) dilatation and right atrial (RA) dilatation were evaluated for (1) tricuspid regurgitant gradient (TRG) ≤ 30 mm Hg; (2) TRG = 30-40 mm Hg, and TRG N 40 mm Hg. A weighted score model was developed to diagnose TRG N 30 mm Hg. The model was validated with data from right heart catheterization in 100 patients. Results: TR severity and RA diameter increased significantly from group 1 to group 2 and to group 3 whereas RV diameter differed only significantly from group 2 to group 3. To integrate TR severity, RA dilatation and RV dilatation, a point-based model was constructed. A total score ≥ 3 was associated with a sensitivity and specificity of 95% and 31% and with a positive and negative likelihood ratios of 1.37 and 0.17, respectively to diagnose TRG N 30 mm Hg. Negative predictive value for TRG N 30 mm Hg was 92%. Prediction numbers could be reproduced when right heart catheterization was used as a reference standard. Conclusions: Increasing TRG is characterized by a steady increase in TR severity and RA dilatation. However, the RV dilates only significantly when TRG is markedly elevated. Integrating morphological parameters could reliably exclude the presence of elevated TRG and thus can be useful in screening for elevated PAP.

We evaluate the incidence of epicardial lead failure and try to identify risk factors in patients with congenital heart disease. All patients with a congenital heart defect and an epicardial pacing system, implanted within a timeframe of 25 years, were included in this study. Patients' medical records and lead data were reviewed. Lead failure was defined as the primary endpoint. In total 198 active epicardial leads (atrial 40, ventricular 158) were implanted in 93 patients (median age at implantation 4.4 years (range 0-58.6)). During a total follow-up of 1235 lead-years, 29 lead failures (14.6%, 4 atrial, 25 ventricular) were documented in 22 patients (23.7%). Lead failure occurred at a median time period of 4.8 years (range 1.2-24.1) after implantation. Five-year freedom of lead failure was 88%. The only independent predictor for lead failure was the age at implantation (HR 0.44; 95%CI 0.20-0.97, p = 0.04), other characteristics failed to predict lead failure. Sudden cardiac de...

The Fontan procedure has become the procedure of choice for patients with one functional ventricle. Although perioperative mortality has decreased, late failure of the Fontan circulation remains a major concern. We aimed at (i) describing Fontan patient characteristics and (ii) identifying simple risk factors for outcome. Seventy-three patients (median age 23 y (IQR 19-29 y), 60.3% male) were selected from the database of congenital heart defects. Followup data were collected. The primary end point was composed by death, resuscitation, or heart transplantation. The most frequently occurring defect was tricuspid atresia (41.1%). Twenty-five (34.2%) and 48 (65.8%) patients received an intra- and extracardiac conduit, respectively. Ten patients reached the primary end point (13.7%) after a median follow-up time of 16 years (IQR 14-19 y). NYHA classification (OR 63.0; 95% Cl 6.7-592.4; P < or =0.001), atrioventricular-valve regurgitation (OR 10.6; 95% Cl: 1.2-94.1; P = 0.034), ventri...

Although tricuspid valve (TV) surgery has become more popular, isolated TV surgery is infrequently performed. The aims of this study were (1) to evaluate the postoperative and long-term mortality of patients undergoing isolated TV surgery, (2) to compare the outcomes of patients undergoing their first TV surgery or TV reoperation, and (3) to assess the additive value of echocardiographic and invasive hemodynamic evaluations for predicting postoperative outcome. We followed a contemporary cohort of patients undergoing isolated TV surgery from January 1, 1995, through December 31, 2011. Preoperative demographic, echocardiographic, hemodynamic, and operative data were included. Outcome was all-cause mortality. Ninety-two patients (38% male; mean age: 56±14 years) were included. Kaplan-Meier survival analyses showed that 30-day, 3-month, 5-year, and 10-year mortality were 7.9%, 15.2%, 25.7%, and 53.7%, respectively. No difference in outcome was found between patients undergoing first TV...