Els Troost

Infective endocarditis of a transcatheter pulmonary valve in comparison with surgical implants

Background Melody valved stents (Medtronic Inc, Minneapolis, Minnesota, USA) have become a very competitive therapeutic option for pulmonary valve replacement in patients with congenital heart disease. After adequate prestenting of the right ventricular outflow tract (RVOT) Melody valved stents have a good medium term functional result but are exposed to infective endocarditis (IE). Patients and methods Retrospective study of tertiary centre Congenital Heart Disease database; to compare incidence of IE in three different types of valved conduits in RVOT: Melody valved stent, cryopreserved homograft (European Homograft Bank) and Contegra graft (Medtronic Inc). Results Between 1989 and 2013, 738 conduits were implanted in 677 patients. 107 Melody valved stents were implanted in 107 patients; IE occurred in 8 (7.5%) patients during a follow-up of 2.0 years (IQR 2.4 years, range 0.3–7.8 years). 577 Homografts were implanted in 517 patients; IE occurred in 14 patients (2.4%) during a median follow-up of 6.5 years (IQR 9.2 years; range 0.1–23.7 years). Finally, 54 Contegra grafts were implanted in 53 patients; 11 patients (20.4%) had IE during a follow-up of 8.8 years (IQR 7.7 years; range 0.2–3.5 years). Survival free of IE by Kaplan–Meier for homografts was 98.7% at 5 years and 97.3% at 10 years; for Contegra 87.8% at 5 years and 77.3% at 10 years and for Melody 84.9% at 5 years (log-rank test; p<0.001). Conclusions The Contegra conduit and Melody valved stents have a significantly higher incidence of IE than homografts. IE is a significant threat for long-term conduit function.

Prevalence of cardiovascular risk factors in adults with congenital heart disease

Background Empirical evidence indicates that patients with congenital cardiac anomalies may be prone to developing coronary heart disease. Although primary prevention of ischaemic heart disease in patients with congenital heart defects is important, data on the prevalence of cardiovascular risk factors in these patients are not available. The aims of this study are therefore to describe the prevalence of risk factors for coronary heart disease in a large sample of adults with congenital cardiac anomalies, and compare this with the prevalence in the general population. Design A retrospective analysis of computerized patient records. Methods At our outpatient clinic, all patients are examined by an advanced practice nurse and a congenital heart disease cardiologist. Data on smoking behaviour, sports participation, blood pressure, body mass index, and the diagnosis of diabetes are recorded systematically. Data on the general population were derived from national health surveys. Results In a 4-year period, we collected data on 1976 individual patients. Male patients had a significantly higher prevalence of smoking and elevated blood pressure, whereas women were less engaged in sports activities and were more often obese. In comparison with the general population, our patients reported less smoking and more participation in sports, but presented more often with hypertension or diabetes. Only 20.4% of men and 21.0% of women have a fully heart-healthy lifestyle, as they presented without any risk factor. Conclusion A substantial number of patients had one or more cardiovascular risk factors. Therefore, primary prevention by strengthening educational efforts becomes critically relevant in patients with congenital heart disease, to avoid the additional burden of coronary events in this growing population of patients.

Off-label use of percutaneous pulmonary valved stents in the right ventricular outflow tract: time to rewrite the label?

Percutaneous pulmonary valve implantation is now considered feasible and safe. “Native” right ventricular outflow tract (RVOT), small diameter conduits (<16 mm) and relatively large RVOT with a dynamic outflow aneurysm are currently considered off‐label uses. Extending indications creates concerns of safety, ethics, reimbursement, and liability.

Behavior of Unrepaired Perimembranous Ventricular Septal Defect in Young Adults

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 + or - 7% to 67 + or - 8% and from 41 + or - 11 to 44 + or - 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.

Long-term outcome after treatment of isolated pulmonary valve stenosis

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.

Intra-cardiac echocardiography in atrial septal interventions: impact on hospitalization costs.

Objective — Intra-cardiac echocardiography (ICE) is used to guide percutaneous interventions on the atrial septum. However, ICE catheters are expensive.We questioned the impact of the use of ICE catheters on hospitalization costs for patent foramen ovale (PFO) or atrial septal defect (ASD) closure. Methods and results — Patients, scheduled for atrial septal closure, were randomly selected to use the AcuNav catheter (Biosense Webster Inc, Diamond Bar, CA, US) on top of the standard procedure (three or two days of hospitalization, procedure with transoesophageal echocardiography (TEE) and general anaesthesia). The AcuNav catheter was provided for free and the total hospitalization cost for each patient was calculated by verification of the bills, sent to the patient and the national health insurance.This was compared with a fictive hospitalization cost when ICE alone would have been used (three or two days of hospitalization, procedure without TEE, and local anaesthesia). Feasibility and safety were also evaluated. Three PFOs and two ASDs were successfully closed (3F/2M, age 55 ± 12 years). The total hospitalization cost for a standard closing procedure was EUR 9345 ± 132 and EUR 9303 ± 132 for three and two days of hospitalization, respectively. With a free ICE catheter and no general anaesthesia, hospitalization cost lowered to EUR 8464 ± 131 and EUR 8422 ± 131, respectively. Cost saving would be EUR 881 ± 3, but the price of a single-use ICE catheter varies between EUR 2000 and 2500. In all patients, adequate images were obtained and no complications related to the ICE catheter occurred. Conclusions — Single-use ICE catheters remain expensive in percutaneous ASD or PFO closure. However, general anaesthesia might be avoided, which could open a discussion on cost savings.

BMPR1A is a candidate gene for congenital heart defects associated with the recurrent 10q22q23 deletion syndrome

Congenital heart defects (CHD) are associated with the recurrent 10q22q23 deletion syndrome and with partially overlapping distal 10q23.2.q23.31 microdeletions. We report on a de novo intragenic deletion of the BMPR1A gene in a normally developing adolescent boy with short stature, delayed puberty, facial dysmorphism and an atrioventricular septal defect. Based on this finding, complemented with computational prioritization data and molecular evidence in literature, the critical region for CHD on 10q23 can be downsized to a single gene, BMPR1A. Although loss-of-function mutations in BMPR1A typically result in juvenile polyposis syndrome, none of the patients with the typical 10q22q23 microdeletion syndrome, comprising this gene, were reported to have juvenile polyposis thus far. We reason that, even in the absence of juvenile polyposis syndrome, sequencing and copy number analysis of BMPR1A should be considered in patients with (atrioventricular) septal defects, especially when associated with facial dysmorphism and anomalous growth.

Behavior of Ebstein's Anomaly: Single-Center Experience and Midterm Follow-Up

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 long-term follow-up data of adults with Ebstein’s anomaly are scarce, we evaluated the outcome of our Ebstein’s anomaly patients. Methods: All patients >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 ± 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. Twenty-six 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 surgery was not uncommon. Supraventricular arrhythmia occurred frequently and ablation therapy was often indicated. Careful follow-up is obligate, as some complications occur for the first time in adulthood.

Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries

BACKGROUND Pulmonary outflow tract obstruction (POTO) reduces systemic atrioventricular valve (SAVV) regurgitation severity in congenitally corrected transposition of the great arteries (ccTGA). Therefore, pulmonary artery banding is proposed as a palliative intervention. We aimed to investigate the effect of native or surgically induced POTO on event-free survival, defined as the composite of all-cause mortality, heart transplantation, or congestive heart failure (CHF). METHODS AND RESULTS Patients with ccTGA (n=62; median age 27.5 (IQR 18.4-39.4) years; 39% with POTO) were selected from the Adult Congenital Heart Disease database of a tertiary hospital. At first visit, SAVV regurgitation ≥ 3/4, systemic RV dysfunction ≥ moderate, and CHF were present in 26%, 26%, and 15% of patients, respectively. Over a mean follow-up time of 10.1 ± 6.1 years, all-cause mortality, rate of heart transplantation, and CHF were 18%, 8% and 40%, respectively. SAVV regurgitation (HR: 1.99; 95% CI: 1.01-3.92; P=0.048) and systemic RV dysfunction severity (HR: 1.89; 95% CI: 1.05-3.37; P=0.033) were associated with the composite endpoint, independently of age at baseline, POTO, Ebstein-like malformation, and systemic RV dilatation. Patients with POTO had lower risk for developing SAVV regurgitation ≥ 3/4 (HR: 0.18; 95% CI: 0.05-0.58; P=0.004) and moderate systemic RV dysfunction (HR: 0.34; 95% CI: 0.15-0.78; P=0.011). When POTO was present, the mean progression-free interval for the composite endpoint increased from 11.2 to 18.1 years (P=0.035). CONCLUSIONS POTO is associated with an improved event-free survival in adults with ccTGA.

Functional and haemodynamic assessment of mild-to-moderate pulmonary valve stenosis at rest and during exercise

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 (Pearsons 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. Clinical trial number: NCT01444222