Infective endocarditis in patients after percutaneous pulmonary valve implantation with the stent-mounted bovine jugular vein valve: Clinical experience and evaluation of the modified Duke criteria.

AIMS: Percutaneous pulmonary valve implantation (PPVI) has proven good hemodynamic results. As infective endocarditis (IE) remains a potential complication with limited available clinical data, we reviewed our patient records to improve future strategies of IE prevention, diagnosis and treatment. METHODS: Medical records of all patients diagnosed with Melody® valve IE according to the modified Duke criteria were retrospectively analyzed in three Belgian tertiary centers. RESULTS: 23 IE episodes in 22 out of 240 patients were identified (incidence 2.4% / patient year) with a clear male predominance (86%). Median age at IE was 17.9 years (range 8.2-45.9 years) and median time from PPVI to IE was 2.4 years (range 0.7-8 years). Streptococcal species caused 10 infections (43%), followed by Staphylococcus aureus (n = 5, 22%). In 13/23 IE episodes a possible entry-point was identified (57%). IE was classified as definite in 15 (65%) and as possible in 8 (35%) cases due to limitations of imaging. Echocardiography visualized vegetations in only 10 patients. PET-CT showed positive FDG signals in 5/7 patients (71%) and intracardiac echocardiography a vegetation in 1/1 patient (100%). Eleven cases (48%) had a hemodynamically relevant pulmonary stenosis at IE presentation. Nine early and 6 late percutaneous or surgical re-interventions were performed. No IE related deaths occurred. CONCLUSIONS: IE after Melody® valve PPVI is associated with a relevant need of re-interventions. Communication to patients and physicians about risk factors is essential in prevention. The modified Duke criteria underperformed in diagnosing definite IE, but inclusion of new imaging modalities might improve diagnostic performance.

Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study.

BACKGROUND: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD). METHODS: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire). RESULTS: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0-2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4-2.0] (n = 6) per year, with 1.1% [95%CI 0.5-2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5-8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80-93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05). CONCLUSIONS: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease.

PREVENTION-ACHD: PRospEctiVE study on implaNTable cardioverter-defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease; Rationale and Design.

BACKGROUND: Many adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) may prevent SCD, but the evidence for primary prevention indications is still unsatisfactory. STUDY DESIGN: PREVENTION-ACHD is a prospective study with which we aim to prospectively validate a new risk score model for primary prevention of SCD in ACHD patients, as well as the currently existing guideline recommendations. Patients are screened using a novel risk score to predict SCD as well as current ICD indications according to an international Consensus Statement. Patients are followed up for two years. The primary endpoint is the occurrence of SCD and sustained ventricular arrhythmias. The Study was registered at ClinicalTrials.gov (NCT03957824). CONCLUSION: PREVENTION-ACHD is the first prospective study on SCD in ACHD patients. In the light of a growing and aging population of patients with more severe congenital heart defects, more robust clinical evidence on primary prevention of SCD is urgently needed.

Perceived health is partially associated with the symptomatological profile in patients with benign and severe conditions: the case of congenital heart disease.

PURPOSE: Individuals with serious medical conditions can perceive their health status as good. This might be explained by the symptomatology inherent to the condition. Research in this respect is scarce. Congenital heart disease (CHD) is a spectrum of mild, moderate, and complex heart defects, representing more benign and severe chronic conditions. We investigated (1) symptomatology (i.e., symptom frequency and symptom distress) of CHD patients; (2) the extent to which symptomatology was independently related to perceived health; and (3) the relative importance of individual symptoms for perceived health. METHODS: A secondary data analysis on two separate patient samples (629 Belgian and 1,109 Dutch patients) was conducted. Patients' symptomatology was measured with the TAAQOL-CHD. Perceived health was measured by the EQ-5Dvas in Belgian patients, and by a single item (EVGFP rating) of the SF-36 in Dutch patients. Linear regression analyses were performed to investigate the relationship between symptoms and perceived health, while controlling for sex, age, disease complexity, and functional status. RESULTS: The most frequently occurring symptoms were dizziness, palpitations, and nycturia. Symptom distress was associated with perceived health, independent of confounders. Symptom distress with respect to shortness of breath while walking; palpitations; and dizziness were independently related to perceived health. CONCLUSIONS: Perceived health in CHD patients is partially associated with their symptomatology. This finding underscores the possibility that differences in perceived health across patient groups with more benign and severe conditions may be caused by the different impact conditions have--in terms of symptoms--on the day-to-day life.

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.

Left atrial myxoma. Evaluation with transoesophageal echocardiographic and real time three-dimensional imaging.

Myxoma is the most common benign cardiac neoplasm. A 66-year-old woman presented with a large left atrial myxoma, which was detected by a real-time three-dimensional echocardiography (RT3DE) and a real-time three-dimensional transoesophageal echocardiography (RT3D-TEE) approach. RT3DE and RT3D-TEE proved to be techniques that can provide additional contributions to the diagnostic investigation of structural heart diseases.

Failure of epicardial pacing leads in congenital heart disease: not uncommon and difficult to predict.

AIMS: We evaluate the incidence of epicardial lead failure and try to identify risk factors in patients with congenital heart disease. METHODS: 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. RESULTS: 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 death occurred in four patients (4.3%), in one a lead failure was documented. CONCLUSION: A high incidence of epicardial lead failures is found in patients with congenital heart disease. Unfortunately, it is difficult to predict this potentially life-threatening complication.

Transapical left ventricular access for difficult to reach interventional targets in the left heart.

OBJECTIVE: Interventional targets may be virtually "excluded" due to vascular access problems or complex previous surgical procedures. This study reviews our experience using transapical ventricular puncture to gain direct access to the systemic ventricle. PATIENTS: Patient 1 (74 years, 2 previous sternotomies), patient 2 (66 years, 5 previous sternotomies), and patient 5 (69 years, 3 previous sternotomies) with prosthetic valves had paravalvular mitral valve leaks. Patient 3 (6.3 years, 2 previous sternotomies) with an extracardiac Fontan conduit, had a significant residual leak after two previous surgical attempts of patch closure of a severely regurgitant right atrioventricular valve. Patient 4 (10 months) had failure of standard ablation of the posteroseptal region of the mitral valve with persistent life-threatening episodes of ventricular tachycardia. METHODS: Procedures were performed under general anesthesia. Entry site was percutaneous in three patients and in two (and one conversion) a mini-thoracotomy was used. Sheaths were placed (6 F) using standard Seldinger technique, followed by the procedure as required. Direct surgical closure of the puncture site was done in 4 patients and in patient 3, a percutaneous vascular occlusion device was used. RESULTS: Easy and immediate access was obtained in all patients. The paravalvular leaks were crossed within seconds and completely closed with Amplatzer occluders. In patient 3 the valve was crossed using a Brokenbrough needle and a 12-mm Amplatzer device was placed in the patch leak. Patient 4 was successfully ablated using a 7-F irrigated catheter endo- and epicardially. Complications were in the percutaneous puncture group: in one patient a coronary artery was punctured and in one a hemothorax developed. CONCLUSION: Direct left ventricular puncture offers a very useful alternative access site in selected patients to reach "inaccessible" targets for certain percutaneous interventions in patients where standard approaches may be impossible or difficult.

New-onset and persistent migraine early after percutaneous atrial septal defect closure disappear at follow-up.

AIMS: Recently we reported that percutaneous atrial septal defect (ASD) closure had no influence on the prevalence of migraine during a short followup period. 12 % of patients however developed a new-onset migraine after the ASD closure. As it has been suggested that the closing device might induce or maintain migraine temporarily, we were interested in the prevalence of migraine at longer follow-up. METHODS: All 75 patients included in the previous study, received the same structured headache questionnaire. A neurologist, blinded to previous data, diagnosed migraine with or without aura (MA+ or MA-) according to the International Headache Criteria. McNemar paired X2 test was used to evaluate changes in the occurrence of migraine. RESULTS: Seventy-one patients (94.7%) answer the questionnaire (55 women, mean age at closure 51 +/- 18 years). Mean follow-up time was 52 +/- 13 months. The overall migraine prevalence decreased from 30.7% before to 22.5% after closure (P=0.21). A significant reduction was noted in patients with new-onset migraine early after closure (n=7), where migraine disappeared in 6 patients (P=0.031). In the group with persistent migraine early after closure (n=13), another 6 patients became migraine-free (P=0.031). CONCLUSION: Percutaneous ASD closure was not related to a significant decrease in overall migraine prevalence. However, new-onset and persistent migraine early after closure disappeared.

Patent foramen ovale and migraine.

Migraine is a common neurological disorder with a great impact on the quality of life and social activities. The patent foramen ovale (PFO) is an intra-atrial right-to-left shunt with a prevalence of 25% in the general population. An increased prevalence is found in patients with migraine, especially in migraine with aura. Percutaneous PFO closure might decrease the prevalence of migraine. However, most of these observational studies were retrospective without a randomized design and the results need to be interpreted with caution. In this review we describe the association between PFO and migraine and the different pathophysiological hypotheses, which have been proposed to explain this relationship.

Stenting of hypoplastic aortic segments with mild pressure gradients and arterial hypertension.

OBJECTIVE: To determine the safety, feasibility and effectiveness of stent expansion of hypoplastic aortic segments with pressure gradients in patients with arterial hypertension. DESIGN: Non-randomised prospective clinical trial. SETTING: Tertiary referral centre, congenital cardiac unit. PATIENT SELECTION: 20 consecutive patients (median age 14.5 years, range 11.6-38.8 years) with arterial hypertension and a hypoplastic segment of the aorta. Seventeen patients had successful previous arch interventions in a coarctation site. INTERVENTIONS: Stent deployment in hypoplastic arch segments. MAIN OUTCOME MEASURES: Gradient across the aortic arch; complications early and during follow up; residual hypertension. RESULTS: 23 stents were deployed: 13 in the cross and 10 in the isthmus. The mean gradient across the aortic arch decreased from 16 (SD 6) (median 17) to 3 (4) (median 1) mm Hg (p < 0.001). In a few patients a mild gradient persisted just distal to the left carotid artery due to residual orificial narrowing or acute angulation. No complications occurred during or after the procedure. During follow up of 2.2 years (range 0.2-4.8 years) arterial hypertension resolved in 10 patients and 10 required residual drug treatment with better control of blood pressures. CONCLUSIONS: Pressure loss due to residual hypoplastic aortic segments can be treated effectively and safely with stent expansion. Some patients remain mildly hypertensive and require additional drug treatment.

Embolization of pulmonary arteriovenous malformations and decrease in prevalence of migraine.

OBJECTIVE: To determine if embolization of pulmonary arteriovenous malformations (PAVMs) decreases the occurrence of migraine. METHODS: All 105 patients with hereditary hemorrhagic telangiectasia (HHT), minimum age 16 years, who underwent an embolization of PAVM in our hospital between June 1995 and April 2004, were included in this study. A structured headache questionnaire was sent to all patients and focused on two periods: 1 year before and at least 6 months after embolization. Two independent neurologists diagnosed migraine according to the International Headache Society criteria. RESULTS: Eighty-four patients (80%) sent back the questionnaire (mean age 47.8 +/- 15.1 years, 51 female). Median follow-up time was 48 months (first quartile: 23 months; third quartile: 66 months). The overall prevalence of migraine decreased from 45.2% before to 34.5% after embolization (p = 0.01). The prevalence of migraine with aura decreased from 33.3% before to 19.0% after embolization (p = 0.002). The severity of headache attacks decreased in patients who still had migraine (p = 0.15) or migraine with aura after embolization (p = 0.11). CONCLUSION: Embolization of pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia seems to be related to a decrease in prevalence of migraine, suggesting that the presence of a right-to-left shunt rather than the localization of this shunt plays a causative role in the pathogenesis of migraine.

Changes in QRS duration are associated with maximal exercise capacity in adult patients with repaired tetralogy of Fallot.

OBJECTIVE: In adult patients with repaired tetralogy of Fallot (TF) QRS duration at rest seems to be a predictor of maximal exercise. We examined the relationship between QRS duration during exercise and exercise performance. DESIGN: In 57 consecutive TF patients QRS duration in V1 (ms) was measured at rest, at maximal exercise (Wmax, W), and at peak oxygen consumption (peak VO2, ml/min). Stroke volume (SV) was calculated from cardiac output, obtained by CO2 rebreathing. Spearman rank correlation was used to describe the relationship between QRS duration and exercise performance. Statistical significance was defined as P<0.05. RESULTS: Seven patients, who didn't pass the anaerobic threshold, and one outlier (Wmax=340 W) were excluded, resulting in a sample of 49 patients (75.5% male; median age=24 years, range 16-43 years). QRS duration at rest (median=160 ms, range 78-194 ms) and at maximal exercise (median=153 ms, range 80-193 ms) did not differ significantly. The median change of QRS duration during exercise was -5 ms (range -31 to +83 ms). This was negatively correlated with Peak VO2 (2081+/-577 ml/min; rho=-0.33, P=0.02) and Wmax (182+/-53 Watt; rho=-0.33, P=0.02). In patients with QRS shortening peak VO2 and the exercise induced increase in SV were significantly higher than in patients with QRS shortening. CONCLUSIONS: This study indicates that QRS shortening during exercise in TF patients is related with a better exercise performance. Lower increase in stroke volumes may be responsible for this difference. Further research is needed to elaborate these findings.

Is the severity of congenital heart disease associated with the quality of life and perceived health of adult patients?

OBJECTIVE: To explore whether the severity of congenital heart disease is associated with the quality of life and perceived health status of adult patients. DESIGN: Descriptive, cross sectional study. SETTING: Adult congenital heart disease programme in one tertiary care centre in Belgium. PATIENTS: 629 patients (378 men, 251 women) with a median age of 24 years. MAIN OUTCOME MEASURES: Disease severity was operationalized in terms of initial diagnosis (classification of Task Force 1 of the 32nd Bethesda Conference), illness course (disease severity index), and current functional status (New York Heart Association (NYHA) class, ability index, congenital heart disease functional index, and left ventricular ejection fraction). Quality of life was measured by a linear analogue scale, the satisfaction with life scale, and the schedule for evaluation of individual quality of life. Perceived health status was also assessed with a linear analogue scale. RESULTS: Scores derived from the disease severity classification systems were weakly negatively associated with quality of life and health status, ranging from -0.05 to -0.27. The NYHA functional class and ability index were consistently associated with quality of life and perceived health. CONCLUSIONS: This study showed that the severity of congenital heart disease is marginally associated with patients' quality of life and perceived health. Functional status was more related to patients' assessment of their quality of life than was the initial diagnosis or illness course.