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OBJECTIVES: Aberrant origin of the coronary arteries is rare but can be life threatening. It is an important cause of sudden death in athletes and other young adults, and may be treated surgically. Consensus exists that interarterial left coronary artery (LCA) should be surgically repaired. For interarterial right coronary artery (RCA), the discussion remains open. The purpose of this study was to analyse our surgical experience. METHODS: From 2001 until 2014, 31 patients were operated for interarterial RCA, interarterial LCA or intraseptal course of the LCA. Twenty-six patients had interarterial RCA, 4 patients interarterial LCA and 1 patient an intraseptal course of the LCA. Median age at operation was 38 years (range 9-66 years). Twenty-eight patients had previous or current symptoms. The most important were a life-threatening event with resuscitation in 3 and myocardial infarction in 3 others. Surgical repair of interarterial RCA consisted of unroofing of the ostium with or without reimplantation in 25 patients and CABG on the RCA with a venous graft in 1 patient. Reconstruction of interarterial LCA consisted of ostium reconstruction of the LCA with a venous patch in 4 patients. The patient with an intraseptal course had a complete release of the LCA out of the septum and reimplantation in the correct coronary sinus. Follow-up was done by analysis of outpatient records, direct patient contact, echocardiography, electrocardiography, CT-angiography and an exercise test. RESULTS: Median follow-up was 6 years (range 0-11 years). One patient was lost tofollow-up. No early or late mortality occurred. Three patients had ischaemia with ventricular fibrillation or ventricular tachycardia shortly after surgery. Two were immediately reoperated, 1 had a stent implantation 1.5 months after release of intraseptal LCA. Two of these patients show a slight dysfunction of the left ventricle at follow-up. All other patients are asymptomatic. CONCLUSIONS: Surgery for aberrant origin of coronary arteries is safe. There is a risk of cardiac ischaemia shortly after operation, especially in LCA reconstruction. We strongly believe that a slit-like coronary ostium and an intramural aortic course is an absolute indication for surgical repair, also in asymptomatic aberrant RCA. [ABSTRACT FROM AUTHOR]

The article discusses pulmonary hypertension (PH) and its diagnosis and treatment. Topics discussed include several causes of Ph which include hyperplasia, Heart valve diseases and lung disease, diagnosis processes like electrocardiography, imaging and echocardiography and treatments for PH such as anticoagulation therapy, Prostacyclin drugs and pulmonary endarterectomy surgery.

Abstract: The population of adult survivors with tetralogy of Fallot (TOF) is growing over the last decades due to improvements in perinatal management, intensive care and surgical techniques. Pulmonary regurgitation (PR) plays a crucial role in the long-term outcome of these patients. Although PR may be tolerated well for many years, eventually it may lead to right ventricular (RV) dilatation, RV dysfunction, exercise intolerance, arrhythmia and sudden cardiac death. Cardiovascular magnetic resonance (CMR) imaging is an important tool in the follow-up of patients after TOF repair, because biventricular volumes and function and PR volume and fraction can be measured with great accuracy and reproducibility. CMR imaging studies have identified risk factors for late adverse outcomes in patients after TOF repair. These include increased RV end-diastolic volume (EDV), reduced left ventricular ejection fraction and abnormal RV outflow tract function. Other applications of CMR include stress imaging, assessment of diastolic function and late gadolinium enhancement, which have provided additional insight in the function of the RV. Timing of pulmonary valve replacement (PVR) is controversial and should balance between the preservation of RV function and the need for subsequent PVR surgery, since the life-span of a homograft is limited. Based on CMR imaging studies, PVR will be considered if the RVEDV reaches a threshold of between 150 and 200ml/m2 in the presence of severe PR. However, timing of PVR should be based on multiple factors, other than RV size and PR fraction alone. [Copyright &y& Elsevier]

Objective: To analyse the long-term course of QRS duration after pulmonary valve replacement in patients with a previous correction for tetralogy of Fallot. Setting: Tertiary referral centres. Methods: In a retrospective study, 99 adult patients with tetralogy of Fallot, who had undergone a first pulmonary valve replacement late after initial total correction, were identified from the CONCOR (CONgenital CORvitia) registry. Computer-generated QRS durations were obtained from 12-lead electrocardiogram ECG reports in the medical records. A mixed linear regression model was used to analyse the course of QRS duration over time and to identify risk factors for increase in QRS duration over time. Composite end point was created from sudden cardiac death, ventricular tachycardia or implantable cardioverter-defibrillator discharge. Results: In total, 99 patients (57% men, mean (SD) age at pulmonary valve replacement 29 (11) years) with a median follow-up of 4.9 (0.1-16) years were analysed. In patients with preoperative QRS <120 ms, surgery caused no significant change in QRS duration (increase 1.3 (7.9) ms; p=0.65), and after surgery, QRS duration remained stable overtime (increase 0.0064(0.059) ms/year; p=0.98). By contrast, in patients with a preoperative ORS of 150-180 ms or QRS ⩾ 180 ms, surgery resulted in QRS shortening (mean decrease 9.9 (SE 4.3) ms, p=0.021, and 12.2 (SE 2) ms; p<0.001, respectively). During follow-up, a QRS widening 1.1(1.3) ms/year (p<0.001) in both groups was observed. In patients with a preoperative QRS ⩾ 180 ms, no significant difference was observed in the number of patients reaching the composite end point compared with patients with a preoperative QRS of 150-180 ms (25% vs 7%; p = 0.08). However, the former more often reached QRS ⩾180 ms again after surgery compared with the latter (53% vs 13%; p=0.02, respectively). None of the patients with a preoperative QRS ⩾ 180 ms died during follow-up. Conclusion: In our study, we observed a decrease in QRS duration directly after surgery, followed by a steady increase, in patients with a preoperative QRS >150 ms. The beneficial effect of pulmonary valve replacement on QRS duration was transient. The risk of developing ventricular arrhythmias after surgery was substantial when preoperative QRS was ⩾180 ms, but mortality remained low. [ABSTRACT FROM AUTHOR]

Cardiovascular magnetic resonance (CMR) with flow velocity mapping has emerged as a noninvasive method to measure flow in saphenous vein coronary artery bypass grafts. The aim of the current study was to retrospectively test two previously described analysis methods on a large CMR data set and to compare their diagnostic accuracy in detecting diseased vein grafts. In 125 vein grafts of 68 patients, volume flow parameters (volume flow, systolic and diastolic peak flow, diastolic-to-systolic flow ratio at rest and during adenosine stress, and flow reserve) were derived from the velocity maps. Method 1 implemented basal flow [ABSTRACT FROM AUTHOR]

Objectives: The aim of this research was to assess right ventricular diastolic and systolic function before and after pulmonary valve replacement (PVR) in adult patients after repair of tetralogy of Fallot. Background: Pulmonary valve replacement (PVR) in adult patients late after repair of tetralogy of Fallot leads to rapid improvement of right ventricular (RV) systolic function. Methods: A total of 16 patients and 8 healthy subjects were included. Median age at initial repair was 4.9 (0.9 to 13.1) years, and mean age at PVR was 28.7 (19.5 to 45.6) years. Cardiac magnetic resonance imaging was performed before and 8 and 22 months after PVR. Right ventricular volumes and function as well as RV in- and outflow patterns were assessed. Results: The volume of the early filling of the RV (Evol) increased from 49.8 ± 14.7 ml to 53.8 ± 19.3 ml (not significant) and 62.0 ± 18.9 ml, respectively (p < 0.05), whereas the volume of the atrial contraction (Avol) remained unchanged. Consequently, the Evol/Avol ratio increased from 1.4 ± 0.7 before PVR to 1.6 ± 0.7 at 8 months (not significant) and 2.3 ± 1.2 at 22 months (p < 0.01). The Evol/Avol ratio was not significantly different from the healthy subjects at 22 months, indicating late recovery of diastolic function. Systolic function improved rapidly after PVR; the indexed RV end-systolic volume decreased from 93.7 ± 33.0 ml/m2 to 60.9 ± 18.4 ml/m2 (p < 0.01) and 54.8 ± 21.0 ml/m2 (p < 0.01). Conclusions: In adult patients late after total repair of Fallot, PVR leads to late improvement of diastolic function. We speculate that the rapid volume unloading after PVR increases systolic performance, whereas improvement in diastolic function requires long-term remodeling. [Copyright &y& Elsevier]

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance. [ABSTRACT FROM AUTHOR]

Objective: Mortality in type 1 diabetic patients with end-stage renal failure is high and dominated by coronary atherosclerotic events. With regard to prognosis, simultaneous transplantation of pancreas and kidney (SPK) may be superior to kidney transplantation alone (KTA) in type 1 diabetic patients, because normalization of blood glucose levels may reduce progression of coronary atherosclerosis and because it is well known that progression of coronary atherosclerosis is one of the major factors that determines clinical prognosis. However, no data are available on progression of coronary atherosclerosis after SPK.Research Design and Methods: We performed an observational angiographic study comparing progression of coronary atherosclerosis, analyzed with quantitative coronary angiography, in patients with (n = 26) and those without (n = 6) a functioning pancreas graft after SPK, to test the hypothesis that normalization of blood glucose levels by SPK may indeed reduce progression of coronary atherosclerosis in type 1 diabetic patients and thereby improve prognosis.Results: Mean follow-up was 3.9 years. Average glucose control was significantly worse for the patients without a pancreas graft than for patients with a functioning pancreas graft: 11.3 (SD 3.5) vs. 5.9 mmol/l (SD 1.1) (P = 0.03). Mean segment diameter loss (progression of diffuse coronary atherosclerosis) was 0.024 mm/year (SD 0.067) in patients with a functioning pancreas graft, compared with 0.044 mm/year (SD 0.038) in patients in whom the pancreas graft was lost. Minimum obstruction diameter loss (progression of focal coronary atherosclerosis) was 0.037 mm/year (SD 0.086) in patients with a functioning pancreas graft compared with 0.061 mm/year (SD 0.038) in patients in whom the pancreas graft was lost. Regression of atherosclerosis occurred in 38% of patients with a functioning pancreas graft compared with 0% of patients of whom the pancreas graft was lost (P = 0.035).Conclusions: Our study provides, for the first time, evidence that in patients who have undergone SPK, progression of coronary atherosclerosis in patients with a functioning pancreas graft is reduced compared with patients with pancreas graft failure. Our observation is an important part of the explanation for the observed improved mortality rates reported in type 1 diabetic patients with end-stage renal failure after SPK compared with KTA. In light of these findings described above, SPK must to be carefully considered for all diabetic transplant candidates. [ABSTRACT FROM AUTHOR]

Investigates the additional value of magnetic resonance angiography to delineate the course of the coronary artery. Role of echomagnetic resonance angiography in coronary atheriography; Patient characteristics, origin, and course of coronary anomalies; Interpretation of magnetic resonance angiograms.

The study aim was to assess three-dimensional electrocardiogram (ECG) changes during development of pulmonary arterial hypertension (PAH). PAH was induced in male Wistar rats (n = 23) using monocrotaline (MCT; 40 mg/kg sc). Untreated healthy rats served as controls (n = 5). ECGs were recorded with an orthogonal three-lead system on days 0, 14, and 25 and analyzed with dedicated computer software. In addition, left ventricular (LV)-to-right ventricular (RV) fractional shortening ratio was determined using echocardiography. Invasively measured RV systolic pressure was 49 (SD 10) mmHg on day 14 and 64 (SD 10) mmHg on day 25 vs. 25 (SD 2) mmHg in controls (both P < 0.001). Baseline ECGs of controls and MCT rats were similar, and ECGs of controls did not change over time. In MCT rats, ECG changes were already present on day 14 but more explicit on day 25: increased RV electromotive forces decreased mean QRS-vector magnitude and changed QRS-axis orientation. Important changes in action potential duration distribution and repolarization sequence were reflected by a decreased spatial ventricular gradient magnitude and increased QRS-T spatial angle. On day 25, LV-to-RV fractional shortening ratio was increased, and RV hypertrophy was found, but not on day 14. In conclusion, developing PAH is characterized by early ECG changes preceding RV hypertrophy, whereas severe PAH is marked by profound ECG changes associated with anatomical and functional changes in the RV. Three-dimensional ECG analysis appears to be very sensitive to early changes in RV afterload. [ABSTRACT FROM AUTHOR]

The aim of this study was to analyze the effect of pressure and/or volume overload on right ventricular (RV) function and brain natriuretic peptide (BNP) levels in patients with surgically corrected congenital heart disease. Forty-two consecutive patients aged 17 to 57 years (median 30) with congenital heart disease (32 with tetralogy of Fallot and 10 with pulmonary stenosis) were examined. The RV systolic pressure was estimated using Doppler echocardiography. Cardiovascular magnetic resonance imaging was used to obtain the RV volumes, ejection fraction (EF) and corrected EF (cEF). Plasma BNP levels were determined by immunoradiometric assay. Patients were categorized as having volume overload when pulmonary regurgitation was ≥10% and pressure overload when the RV systolic pressure was >40 mm Hg. Patients with RV volume overload had a lower RVEF compared with patients with pressure overload (p = 0.02) and lower left ventricular EF (p <0.001). BNP was higher in patients with volume overload than in patients with pressure overload (p = 0.002). BNP correlated with pulmonary regurgitation, RVEF, RV cEF, and left ventricular EF. In linear regression analysis, RV cEF was an independent predictor for BNP, after adjustment for age. Without the parameter of RV cEF in the regression model, pulmonary regurgitation and RVEF were independently associated with BNP level, after adjustment for age. In conclusion, patients with RV volume overload had higher BNP levels and lower RV function than patients with RV pressure overload. BNP levels were independently associated with the degree of RV volume overload and RV function. [Copyright &y& Elsevier]

Objectives: This study was designed to perform a head-to-head comparison between single-photon emission computed tomography (SPECT) and cardiovascular magnetic resonance (CMR) to evaluate hemodynamic significance of angiographic findings in bypass grafts. Background: The hemodynamic significance of a bypass graft stenosis may not always accurately be determined from the coronary angiogram. A variety of diagnostic tests (invasive or noninvasive) can further characterize the hemodynamic consequence of a lesion. Methods: Fifty-seven arterial and vein grafts in 25 patients were evaluated by angiography, SPECT perfusion imaging, and coronary flow velocity reserve determination by CMR. Based on angiography and SPECT, four different groups could be identified: 1) no significant stenosis (<50%), normal perfusion; 2) significant stenosis (>/=50%), abnormal perfusion; 3) significant stenosis, normal perfusion (no hemodynamic significance); and 4) no significant stenosis, abnormal perfusion (suggesting microvascular disease). Results: A complete evaluation was obtained in 46 grafts. Single-photon emission computed tomography and CMR provided similar information in 37 of 46 grafts (80%), illustrating good agreement (kappa = 0.61, p < 0.001). Eight grafts perfused a territory with scar tissue. When agreement between SPECT and CMR was restricted to grafts without scar tissue, it improved to 84% (kappa = 0.68). Integration of angiography with SPECT categorized 14 lesions in group 1, 23 in group 2, 6 in group 3, and 3 in group 4. Single-photon emission computed tomography and CMR agreement per group was 86%, 78%, 100%, and 33%, respectively. Conclusions: Head-to-head comparison showed good agreement between SPECT and CMR for functional evaluation of bypass grafts. Cardiovascular magnetic resonance may offer an alternative method to SPECT for functional characterization of angiographic lesions. [ABSTRACT FROM AUTHOR]

The Post-Pulmonary Embolism Syndrome (PPES) comprises heterogeneous entities, including chronic thromboembolic disease with/without pulmonary hypertension (CTEPH/CTEPD), and deconditioning. To assess underlying physiological determinants of PPES, and efficacy and safety of rehabilitation training in these patients. 56 consecutive PE patients with persistent dyspnea and/or functional limitations despite ≥3 months of anticoagulation underwent standardized diagnostic work-up including exercise testing as part of routine practice. All diagnostic (imaging and cardiopulmonary function) tests were interpreted by a core group of experienced clinicians. A subgroup of patients without CTEPH or other treatable conditions was referred for a 12-week personalized rehabilitation program, studying changes in physical condition and patient-reported outcome measures. Persistent vascular occlusions were observed in 21/56 patients (38%) and CTEPH was confirmed in ten (18%). Regarding those without CTEPH, impaired cardiopulmonary responses were evident in 18/39 patients with available CPET data (46%), unrelated to chronic thrombi. Rehabilitation was completed by 27 patients after excluding 29 (patients with CTEPH or treatable comorbidities, refusal, ineligibility, or training elsewhere). Training intensity, PE-specific quality of life (PEmb-QoL) and fatigue (CIS) improved with a median difference of 20 W (p = 0.001), 3.9 points (p < 0.001) and 16 points (p = 0.003), respectively. Functional status (Post-VTE Functional Status Scale) improved ≥1 grade in 18 (67%) patients, and declined in one (3.7%). Our findings suggest that abnormal cardiopulmonary responses to exercise are common in patients with PPES and are not limited to those with chronic thrombi. Offering pulmonary rehabilitation to patients not treated otherwise seems safe and promising. • We studied physiological determinants in patients with Post-Pulmonary Embolism Syndrome (PPES). • Insufficient cardiopulmonary responses to exercise were common in these patients. • These findings were not limited to patients with persistent vascular occlusions. • Offering a 12-week pulmonary rehabilitation program seems safe and promising. • 'Post-VTE Functional Status' improved after completing rehabilitation training. [ABSTRACT FROM AUTHOR]

An anomalous coronary artery is reported in 2% to 23% of patients with tetralogy of Fallot (TOF). Knowledge of coronary anatomy prior to corrective surgery is vital to avoid damage to vessels crossing the right ventricular outflow tract (RVOT). A meta-analysis on the prevalence of anomalous coronary arteries in TOF is lacking to date. Here, an overview of coronary anomalies in TOF is provided and implications for patient management are discussed. PubMed, Embase and Web of Science were searched. Analysis was done using Revman 5.3 (Cochrane Community, London). The primary analysis focused on the origin and proximal course of the right and left coronary arteries. In addition, the prevalence of large conus arteries and coronary arteriovenous fistulas (CAVF) was calculated. Twenty-eight studies, encompassing 6956 patients, were included; 6% of TOF patients have an anomalous coronary artery. Hereof, 72% cross the RVOT; the majority of the remaining 28% courses behind the aorta. Six percent of patients have a large conus artery and 4% a CAVF. Other coronary anomalies include a left or right coronary artery from the pulmonary trunk or left or right pulmonary artery, coronary tree hypoplasia and anastomoses between coronary and bronchial arteries. The prevalence of coronary anomalies in TOF is 4–6%. In patients with an anomalous coronary artery, 72% cross the RVOT. The combined risk of encountering an anomalous coronary artery or a large conus artery crossing the RVOT is 10.3%. Coronary anatomy should be defined before surgery and the surgical approach adapted accordingly. Unlabelled Image • The prevalence of coronary anomalies in TOF is 4–6%. • In patients with an ACA, 72% of the anomalous arteries cross the RVOT. • The risk of encountering an ACA or large conus artery crossing the RVOT is 10.3%. • Before surgery, define the coronary anatomy to avoid damage of anomalous vessel. • Surgical approach should be adapted to the course of an anomalous coronary artery. [ABSTRACT FROM AUTHOR]

Objective: Maternal heart disease (HD) complicates 1-4 % of pregnancies and is associated with adverse maternal and fetal outcomes. Although vaginal birth is generally recommended in the guidelines, cesarean section (CS) rates in women with HD are often high. Aim of the present study was to evaluate mode of birth and pregnancy outcomes in women with HD in a tertiary care hospital in the Netherlands.Study Design: The study population consisted of 128 consecutive pregnancies in 99 women with HD, managed by a pregnancy heart team between 2012-2017 and ending in births after 24 weeks' gestation. Pregnancy risk was assessed per modified World Health Organization class. Mode of birth (planned and performed) and maternal and fetal complications (cardiovascular events, postpartum hemorrhage, prematurity, small for gestational age and death) were assessed for each pregnancy.Results: Pregnancy risk was classified as modified World Health Organization class I in 23 %, class II in 50 %, class III in 21 % and class IV in 6% of pregnancies. Planned mode of birth was vaginal in 114 pregnancies (89 %) and CS in 14 (11 %; nine for obstetric and five for cardiac indication). An unplanned CS was performed in 18 pregnancies (16 %; 16 for obstetric and two for cardiac indications). Overall mode of birth was vaginal in 75 % and CS in 25 %. Twelve cardiovascular events occurred in eight pregnancies (6 %), postpartum hemorrhage in nine (7 %) and small for gestational age in 14 (11 %). No maternal or fetal deaths occurred.Conclusions: Findings of this study indicate that - given that pregnancies are managed and mode of birth is meticulously planned by a multidisciplinary pregnancy heart team - vaginal birth is a suitable option for women with HD. [ABSTRACT FROM AUTHOR]

Abstract Patients presenting with chest pain suggestive of coronary artery disease (CAD) who at coronary arteriography appear to be free of obstructive disease have presented a diagnostic and therapeutic challenge since the 1970's. Studies in female patient populations have suggested that this is predominantly a women's syndrome usually caused by microvascular endothelial dependent and independent dysfunction. A critical review of the literature focusing on studies including both women and men revealed that apart from a higher incidence of this syndrome in women there are no clinical relevant differences between both sexes. In women a lower coronary flow reserve has been reported but this appears to be mainly due to a higher basal flow. Important questions with regard to the clinical implications of microvascular dysfunction have yet to be resolved in studies involving women as well as men in which a distinction is made between patients with normal coronary arteries and those with nonobstructive disease. Highlights • Sex specificity of microvascular angina has not been proven. • Women more often than men have chest pain without coronary obstructions. • Hypertension is a common cause of microvascular disease and in part reversible. • Irrespective of sex normal coronary arteries indicates a favorable prognosis. [ABSTRACT FROM AUTHOR]

Abstract Objectives In the VAL-SERVE (Valsartan in Systemic Right Ventricle) trial, three-year valsartan treatment improved systemic ventricular function only in symptomatic patients with congenitally or with an atrial switch corrected transposition of the great arteries. The aim of the current study was to investigate the longer-term clinical outcomes after valsartan treatment. Methods From 2006 to 2009, 88 adults were randomly allocated 1:1 to either valsartan or placebo for three consecutive years. Endpoints were defined as overall survival and freedom from clinical events (arrhythmia, heart failure, tricuspid valve surgery, death). Results Cardiac drug use and median follow-up after trial close-out (8.3 years) was similar between the randomization groups. Six patients (valsartan n = 3, placebo n = 3) died in 364 and 365 person-years (P = 0.999). No difference in the composite or separate clinical endpoints was found between the randomization groups, with corresponding long-term event-free survival rates of 50% and 34%. Nevertheless, in symptomatic patients valsartan significantly reduced the risk for events compared to placebo (HR 0.37, 95% CI 0.17–0.92). Analysis for repeated events and on-treatment analysis with any renin-angiotensin-aldosterone-system-inhibitor did not alter these results. Conclusions Valsartan treatment in systemic RV patients did not result in improved survival at longer-term follow-up, but was associated with decreased risk of events in symptomatic patients. Highlights • Systemic right ventricular failure is a major problem in transposition of the great arteries. • Limited data on heart failure treatment in this patient group are inconclusive. • The study shows a reduction in morbidity in symptomatic patients with angiotensin II receptor blockers. • In line with the previous trial results, valsartan has a favorable treatment effect in symptomatic patients. [ABSTRACT FROM AUTHOR]

Background: The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot.Methods: The REDEFINE trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) is an investigator-initiated, multicenter, prospective, 1:1 randomized, double-blind, placebo-controlled study. Adults with repaired tetralogy of Fallot and RV dysfunction (RV ejection fraction [EF] <50%) but without severe valvular dysfunction were eligible. Patients were randomly assigned between losartan (150 mg daily) and placebo with target treatment duration between 18 and 24 months. The primary outcome was RV EF change, determined by cardiovascular MRI in intention-to-treat analysis.Results: Of 95 included patients, 47 patients received 150 mg losartan daily (age, 38.0±12.4 years; 74% male), and 48 patients received placebo (age, 40.6±11.4 years; 63% male). Overall, RV EF did not change in patients allocated to losartan (n=42) (44.4±5.1% to 45.2±5.0%) and placebo (n=46) (43.2±6.3% to 43.6±6.9%). Losartan did not significantly improve RV EF in comparison with placebo (+0.51%; 95% confidence interval, -1.0 to +2.0; P=0.50). No significant treatment effects were found on secondary outcomes: left ventricular EF, peak aerobic exercise capacity, and N-terminal pro-brain natriuretic peptide (P>0.30 for all). In predefined subgroup analyses, losartan did not have a statistically significant impact on RV EF in subgroups with symptoms, restrictive RV, RV EF<40%, pulmonary valve replacement, or QRS fragmentation. However, in a post hoc analysis, losartan was associated with improved RV EF in a subgroup (n=30) with nonrestrictive RV and incomplete remodeling (QRS fragmentation and previous pulmonary valve replacement) (+2.7%; 95% confidence interval, +0.1 to +5.4; P=0.045).Conclusions: Losartan had no significant effect on RV dysfunction or secondary outcome parameters in repaired tetralogy of Fallot. Future larger studies may determine whether there might be a role for losartan in specific vulnerable subgroups.Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02010905. [ABSTRACT FROM AUTHOR]

OBJECTIVES: In patients with congenitally corrected transposition of the great arteries (ccTGA) or after atrial (Mustard or Senning) correction for transposition of the great arteries (acTGA), the right ventricle (RV) supports the systemic circulation. The tricuspid valve (TV) (systemic atrioventricular valve) is prone to regurgitation in these patients and this is associated with impending RV failure and decreased survival. This study evaluates mid-term functional improvements, echocardiographic findings and survival after TV surgery in this patient group. METHODS: From July 1999 to November 2014, 26 patients (mean age 37.1 ± 12.3 years, 14 females) with ccTGA (n = 15) or acTGA (n = 11) had TV surgery. All patients had RV dysfunction and more-than-moderate TV regurgitation (TR); 14 underwent TV replacement (TVR) and 12 had valvuloplasty (TVP). Main outcomes were New York Heart Association (NYHA) functional class, TR and RV dysfunction at 1 year postoperatively and at latest follow-up. Complications and freedom from the composite end-point of death or recurrent TR were analysed. RESULTS: The median follow-up time was 5.9 years (range, 0-16.1 years). Mean NYHA functional class significantly improved to 1.7 [95% confidence interval (CI): 1.3-2.1] at 1 year (P = 0.004) and was 2.1 (95% CI: 1.7-2.6) at latest follow-up (P = 0.14). TV competence significantly improved to a mean TR grade of 1.1 (95% CI: 0.5-1.7) at latest follow-up (P < 0.001). The mean grade for RV function at latest follow-up was 2.7 (95% CI: 2.3-3.0). Most encountered postoperative complications were arrhythmias and temporary haemodynamic instability due to low cardiac output. Early mortality was 11.5% (n = 3); late mortality was 15.4% (n = 4). Estimated freedom from the composite end-point of death or recurrent TR was 76.9% (95% CI: 55.7-88.9%) at 1 year and 64.8% (95% CI: 43.2-79.9%) at 5 years. In TVP patients, TV function at 1 year and at latest follow-up was significantly worse than in TVR patients (P < 0.001 and P = 0.003, respectively). Also, TVP patients had a significantly lower composite end-point survival curve compared with TVR patients (P = 0.018). CONCLUSIONS: In this patient group, TV surgery showed stabilization of RV function and improvement of NYHA functional class for at least several years. In this series, TVR appears superior to TVP with respect to occurrence of recurrent TR. Early and late mortality after TV surgery is substantial, and we believe that patients with significant TR should be referred earlier for surgery for better outcome. [ABSTRACT FROM AUTHOR]

Background: To deliver adequate care to patients with congenital heart disease (CHD), it is important to know which patients use what type of care. This knowledge is valuable, as modification of these factors may be used as means to regulate healthcare use. Our objective was to examine the predictive value of psychological characteristics for future healthcare use, independent of clinical characteristics. Methods: In total 845 adult CHD-patients participated in a longitudinal questionnaire study, with a two-year follow-up period. Linear regression analyses with negative binomial log link function were performed predicting healthcare used during the previous year. Psychological predictors were Type D personality, quality of life (QoL), depressive symptoms, trait-anxiety, happiness, optimism, and illness perceptions, independent of the number of co-morbidities, disease complexity and functional status. To control for clustering we included the variable type of centre (regional versus tertiary referral). Results: Patients who reported more healthcare use had a complex defect, a poor functional status, no Type D personality, and a poor QoL. They moreover felt their CHD had a severe impact on their life and believed their CHD could be managed by themselves or treatment. Conclusions: Healthcare use is not entirely determined by disease complexity and functional status but also by psychological patient characteristics. It can by hypothesised that reducing the negative impact experienced and informing patients about strategies to manage their CHD, will modify their future healthcare use. Additional research is necessary to examine this possibility. [ABSTRACT FROM AUTHOR]

Background: Survival data that are applicable to the current population of adults with congenital heart disease (CHD) are not available.Objectives: Using an alternative survival analysis with age as the primary time scale, we assessed the contemporary survival of adult patients with CHD.Methods: Survival was assessed using prospective data of the national registry of adult patients with CHD of the Netherlands. Survival was stratified by severity and lesion, and compared with a standardised general population.Results: Mean age at inclusion was 37 years, and 49% of the study population was male. During a cumulative prospective follow-up of 90, 270 patient-years in 14, 327 patients, 535 deaths occurred. Median survival was 53.4 (95% CI 49.9 to 60.7), 75.4 (95% CI 72.9 to 79.1) and 84.1 (95% CI 81.9 to 87.0) years for patients with severe, moderate and mild lesions, respectively. Survival of most patients with mild lesions did not differ from the general population, while, as expected, survival of patients with severe and moderate lesions was substantially lower (<0.001).Conclusions: The present study gives insight in the contemporary survival of adults with CHD. This may aid patient counselling, timing of interventions and future research. [ABSTRACT FROM AUTHOR]

Background In patients with repaired tetralogy of Fallot (rTOF), multiple reoperations or percutaneous interventions after pulmonary valve replacement (PVR) may be necessary due to limited homograft durability. However, data to guide individualised prediction of homograft durability remain scarce. The aim of this study was to provide risk models for RV to pulmonary artery homograft durability. Methods This retrospective multicentre study included consecutive patients with rTOF who had undergone PVR at an age of >12 years. Homograft dysfunction was defined as at least moderate pulmonary regurgitation (PR) or pulmonary stenosis (PS) (pressure gradient ≥36 mm Hg) as assessed by echocardiography. Reintervention was defined as percutaneous intervention or redo-PVR. Results A total of 153 patients with rTOF were included (62% male, mean age at PVR 31±11 years, pulmonary homograft 96%, follow-up 9.6 years (IQR 5.9, 13.3)). Average freedom from homograft dysfunction and reintervention after 10 years was 74% and 89%, respectively. In multivariable Cox proportional hazards analysis, postoperative PS ≥20 mm Hg (HR 6.52, 95% CI 3.09 to 13.7), postoperative PR ≥ grade 1 (HR 3.13, 95% CI 1.45 to 6.74) and age at PVR <18 years (HR 3.52, 95% CI 1.64 to 7.53) were independently predictive for homograft dysfunction. In patients without any risk factor, 10-year freedom from homograft dysfunction and reintervention was excellent (91% and 96%, respectively) in contrast to patients with ≥2 risk factors (25% and 73%, respectively). Conclusions Individualised prediction of homograft durability in patients with rTOF can be guided by early postoperative echocardiography. In adult patients without early postoperative PS or PR, homograft dysfunction and reintervention are unlikely to occur within 10 years, and follow-up may be less stringent. [ABSTRACT FROM AUTHOR]

Objective Patients with surgically repaired tetralogy of Fallot (rTOF) may develop functional tricuspid regurgitation (TR) due to annulus dilation. Guidelines suggest pulmonary valve replacement (PVR) in patients with rTOF with progressive TR, but data on clinical outcomes are lacking. Our objective was to determine whether TR was predictive for adverse events after PVR. Methods In this retrospective, multicenter cohort study, patients with rTOF who had undergone PVR after preoperative echocardiographic assessment of TR grade were included. Preoperative and postoperative imaging data and a composite of adverse clinical events (death, sustained ventricular tachycardia, heart failure, or supraventricular tachycardia) were collected. Multivariate Cox hazards regression analysis was used to determine which factors were predictive for adverse events after PVR. Results A total of 129 patients (61% men, age at PVR 32.9±10.4 years) were included. The composite endpoint occurred in 39 patients during 8.4±4.2 years of follow-up. In multivariate analysis, severe preoperative TR (HR 2.49, 95% CI 1.11 to 5.52), right ventricular end-systolic volume (HR 1.02/mL/mm², 95% CI 1.01 to 1.03) and age at PVR (HR 1.07/year, 95% CI 1.04 to 1.09) were predictive for adverse events. Early postoperative TR was not predictive for adverse events (p=0.96). In patients without any risk factor (age >40 years, right ventricular end-systolic volume >90 mL/m² or severe TR), 5-year event-free survival was 100% as compared with 61% in patients with two or three risk factors. Conclusions In patients with rTOF, severe preoperative TR was predictive for adverse events after PVR. Close surveillance is warranted in these patients irrespective of postoperative TR. [ABSTRACT FROM AUTHOR]

Background Adult post-coarctectomy patients (CoA) demonstrate increased cardiovascular morbidity and mortality. The carotid intima-media thickness (CIMT), a marker for atherosclerosis, is increased in CoA. The aim was to evaluate the predictive value of CIMT for cardiovascular events. Methods and results Consecutive CoA patients were prospectively studied during 10.1 ± 0.7 years follow-up. At baseline and follow-up echocardiography, MRI imaging and CIMT imaging were performed, while cardiovascular events were registered. CIMT data were compared with controls. The composite endpoint included: myocardial infarction, cerebrovascular events (CVAs), and (sudden) cardiac death. 160 CoA patients were studied (median age 31.7 (18-74 years), 64% male). Events occurred in 11 patients (7%), five (3%) with myocardial infarction, four (2.5%) with an ischemic CVA and two (1%) died suddenly. An increased CIMT (≥ 0.8 mm) (HR = 15.44, P = < 0.001) was predictive for the occurrence of cardiovascular events. Baseline CIMT was increased in CoA compared to controls (0.64 ± 0.12 mm vs 0.57 ± 0.07 mm, P = 0.005). CIMT progression rates were similar (0.0091 ± 0.016 mm/year vs 0.0097 ± 0.018 mm/year, P = 0.84). Signs of atherosclerosis occurred significantly earlier in CoA patients. Conclusion The contemporary cardiovascular event rate in CoA is 11% in 10 years. Atherosclerosis seems to appear earlier in CoA patients as compared to controls. CoA patients with a CIMT exceeding 0.8 mm have a fifteen fold higher cardiovascular risk. CIMT seems to be a useful tool for cardiovascular risk assessment in CoA. [ABSTRACT FROM AUTHOR]

Objectives Adequate prepregnancy prediction of maternal cardiovascular and offspring risk is important for counselling and management of pregnancy in women with congenital heart disease (CHD). Therefore we performed a study to identify the optimal assessment strategy for estimating the risk of pregnancy in women with CHD. Methods In this prospective study, we determined the outcomes of 213 pregnancies in 203 women with CHD. The ZAHARA I (Zwangerschap bij Aangeboren HARtAfwijkingen I) and CARPREG (CARdiac disease in PREGnancy) risk scores were calculated for each pregnancy, as was the total number of cardiovascular (TPc) or offspring risk predictors (TPo) from these and other studies combined. Pregnancies were also classified according to the modified WHO classification of maternal cardiovascular risk and according to disease complexity (DC). Results Maternal cardiovascular events occurred during 22 pregnancies (10.3%). Offspring events occurred during 77 pregnancies in 81 children (37.3%). Cardiovascular and offspring event rates increased with higher risk scores, higher TPc or TPo, higher WHO class and greater DC. The highest area under the curve (AUC) for maternal cardiovascular risk was achieved by the WHO class (AUC: 0.77, p<0.0001). AUC for the ZAHARA I risk score was 0.71 (p=0.001), and for the CARPREG risk score 0.57 (p=0.32). All models performed insufficiently in predicting offspring events (AUC⩽0.6). Conclusions The WHO classification is the best available risk assessment model for estimating cardiovascular risk in pregnant women with CHD. None of the offspring prediction models perform adequately in our cohort. [ABSTRACT FROM AUTHOR]

Objective After the arterial switch operation (ASO), disproportional neo-aortic growth during childhood has been reported. Even though it has been suggested neoaortic dilation will stabilise in adulthood, data are lacking. The aim of this study was to assess the change in neo-aortic dimensions, prevalence of neo-aortic dilation >40 mm and long-term outcome in adults who underwent ASO in childhood. Methods All 116 ASO patients operated in a tertiary referral centre and born before 1995 were included. Of these, 83 (72%) survived to adulthood (>17 years) and six were lost to follow-up. Neo-aortic measurements performed in adulthood were collected from available echocardiographic, cardiovascular magnetic resonance and CT images. The time trend was analysed using a mixed model, adjusted for imaging modality. Results Clinical data with at least one measurement of the neo-aortic diameter were available in 77 (93%) adult patients and serial measurements in 65 (78%). At baseline (median age 18.1 years), mean neo-aortic diameter was 36±5 mm. Mean neo-aortic growth was 0.31 mm/year (p<0.001 compared with normal value 0.08 mm/year) and was linear over time. Freedom from neo-aortic dilation beyond a diameter of 40 mm was 23% at 28 years of age. During a mean clinical followup in adulthood of 7.2 years (IQR 4.0 to 10.1), 3 (4%) patients underwent neo-aortic replacement. No other neo-aortic complications occurred. Conclusions In early adulthood, neo-aortic growth was on average linear and did not stabilise over time. [ABSTRACT FROM AUTHOR]

Objective: To study the changes over time in biventricular size and function, and clinical parameters in patients after repair of tetralogy of Fallot (TOF) without subsequent pulmonary valve replacement (PVR). Methods: We prospectively included 78 non-PVR patients (age 20(6–60)years at baseline), who were studied twice with a 5-year interval. Patients underwent magnetic resonance imaging for assessment of biventricular size and function. Exercise testing and electrocardiography were performed to determine peak oxygen uptake (peak VO2) and QRS duration. N-terminal prohormone brain natriuretic peptide (NT-proBNP) was assessed additionally. Results: Pulmonary regurgitation (PR), right ventricular (RV) volumes and QRS duration increased during 5-year follow-up (RV end-diastolic volume (EDV) 130±30ml/m2 to 138±34ml/m2; QRS 132±27msec to 139±27msec); peak VO2 decreased (96±19% to 91±17%). RV ejection fraction, RV effective stroke volume (eff.SV), and NT-proBNP levels remained unchanged. The slope of RVEDV increase was 1.6±3.0ml/m2/year, and depended on RVeff.SV, not on RVEDV, at baseline. Increase in RVEDV correlated with increase in QRS duration over time (r =0.28, p =0.016), and with decrease in RV mass/EDV ratio over time (r =−0.42, p <0.001), not with decrease in peak VO2. In subgroup analysis, patients with larger RVs at baseline showed larger increase in PR during follow-up and larger decrease in NYHA class over time. Conclusions: In TOF patients with moderate RV dilatation, RVEDV increased by 1.6±3.0ml/m2/year, irrespective of RV size at baseline, but depended on RVeff.SV at baseline. Despite limited progression in RV size, unfavourable changes occurred during 5years follow-up, which suggests there is a need for close follow-up. [ABSTRACT FROM AUTHOR]

Abstract: Background: This study aims to investigate indications and complications of permanent cardiac pacing in adults with congenital heart disease (CHD). Methods and results: Two-hundred and seventy-four CHD patients were identified who underwent permanent pacemaker implantation between 1972 and 2009. The indication for pacing was acquired sinus node or AV node conduction disease (63%), sinus node or AV node conduction disease after cardiac surgery (28%), and drug/arrhythmia-related indications (9%). Patients with complex CHD received a pacemaker at younger age (23 versus 31years, p<0.0001) and more often received an epicardial pacing system (51% versus 23%, p<0.0001) compared to those with simple or moderate CHD. Twenty-nine patients (10.6%) had a periprocedural complication during the primary pacemaker implantation (general population: 5.2%). The most common acute complications were lead dysfunction (4.0%), bleeding (2.6%), pocket infection (1.5%) and pneumothorax (1.5%). During a median follow-up of 12years, pacemaker-related complications requiring intervention occurred in 95 patients (34.6%). The most common late pacemaker-related complications included lead failure (24.8%), pacemaker dysfunction/early battery depletion (5.1%), pacemaker migration (4.7%) and erosion (4.7%). Pacemaker implantation at younger age (<18years) was an independent predictor of late pacemaker-related complication (adjusted hazard ratio 1.68, 95% confidence interval 1.07 to 2.63, p=0.023). Conclusions: The risk of periprocedural complications seems higher in the CHD population compared to the general population and more than one-third of CHD patients encountered a pacemaker-related complication during long-term follow-up. This risk increases for those who receive a pacemaker at younger age. [Copyright &y& Elsevier]

Background: Patients with moderate pulmonary valve restenosis late after relief of severe pulmonary stenosis (PS) may show decreased exercise tolerance. To elucidate the mechanism of decreased exercise tolerance, we evaluated cardiac response to physical and pharmacological stress in these patients and compared results with those of patients with native moderate PS. Methods: Twenty asymptomatic patients with moderate PS were divided into 2 groups: Group I (late after relief of severe PS, n=9), and Group II (no previous intervention, n=11). All patients underwent an exercise test, dobutamine stress (DS) MRI, and delayed contrast enhanced MRI. The response to physical and pharmacological stress was compared between both groups. Results: Group I showed impaired exercise capacity compared to Group II (VO2max=72.8%±3.5% vs. 102.5%±16.3%, p<0.001). During DS-MRI, RV-SV increased in Group II, but not in Group I (+13±8ml, −5±8ml, p<0.001). RV end‐diastolic volume decreased significantly in Group I patients (p=0.006) while it did not significantly change in Group II patients. The amount of RV-SV increase (∆ RV-SV) correlated negatively with the period of moderate PS existence and the current PG in Group I (r=−0.82, p=0.007, and r=−0.68, p=0.04, respectively) but not in Group II (r=0.45, p=0.1, and r=0.40, p=0.2, respectively). Furthermore, ∆ RV-SV correlated negatively with the PG before valvuloplasty (r=−0.76, p=0.02). Conclusion: Impaired exercise capacity in patients with moderate pulmonary restenosis after relief of severe PS is probably caused by inability to increase RV-SV. Disturbed RV filling properties, worsening in time, might play a role. [ABSTRACT FROM AUTHOR]

Purpose: Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA. Methods: 96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥20mmHg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥37mm. Results: All patients (median age 28.0years, range 17–61years; male 57%) were followed with a median follow-up of 7.0years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV). Median AS progression was 1.1mmHg/5years (range — 13–28). Determinants for AS at follow-up were age (ß=0.20, P=0.01), aortic dilatation (ß=4.6, P=0.03), and baseline aortic valve gradient (ß=0.93, P<0.001). BAV was predictive for AR. (ß=0.91, P=0.049). Conclusion: Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to identify which patients with a systemic right ventricle are at risk for clinical events. Background: In patients with congenitally or atrially corrected transposition of the great arteries, worsening of the systemic right ventricle is accompanied by clinical events such as clinical heart failure or the occurrence of arrhythmia. Methods: At baseline, all subjects underwent electrocardiography, echocardiography, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging. Clinical events comprised death, vascular events, tricuspid regurgitation requiring surgery, worsening heart failure, and (supra)ventricular arrhythmia. A Cox proportional hazards analysis was used to assess the most valuable determinants of clinical events. Results: A total of 88 patients with a mean age of 33 years were included in the study. Sixty-five percent were men, and 28% had congenitally corrected transposition of the great arteries. During a follow-up period of 4.3 years, 31 patients (35%) experienced 46 clinical events for an annual risk of 12%. Right ventricular end-diastolic volume index measured by means of cardiovascular magnetic resonance imaging or multirow detector computed tomography (hazard ratio: 1.20; p < 0.01) and peak exercise systolic blood pressure (hazard ratio: 0.86; p = 0.02) were the strongest determinants of clinical events. Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg were most likely to experience clinical events with an annual event rate of 19% versus 0.9% in patients without these risk factors. Conclusions: Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg had a 20-fold higher annual event rate than patients without these risk factors. Regular cardiovascular magnetic resonance imaging and exercise testing are important in the risk assessment of these patients. [Copyright &y& Elsevier]

Background: Atrial enlargement may reflect ventricular diastolic dysfunction. Although patients with tetralogy of Fallot (TOF) have been studied extensively, little is known about atrial size and function. We assessed bi-atrial size and function in patients after TOF repair, and related them to biventricular systolic and diastolic function, and clinical parameters. Methods: 51 Patients (21±8years) and 30 healthy controls (31±7years) were included and underwent magnetic resonance imaging to assess bi-atrial and biventricular size, systolic and diastolic function. Patients also underwent exercise testing, and N-terminal prohormone brain natriuretic peptide (NT-proBNP) assessment. Results: In patients, right atrial (RA) minimal volume (34±8ml/m2 vs. 28±8ml/m2, p =0.001) and late emptying fraction were increased; RA early emptying fraction was decreased. Patients had longer right ventricular (RV) deceleration time (0.24±0.10 vs. 0.13±0.04, p <0.001), reflecting impaired RV relaxation, and larger RV volumes. Patients with end-diastolic forward flow (EDFF) had larger RA and RV size, abnormal RA emptying, higher NT-proBNP levels, higher VE/VCO2 slope (ventilatory response to carbon dioxide production), and the most abnormal LV diastolic function (impaired compliance). Patients with abnormal RA emptying (reservoir function <30% and pump function >24%) had higher NT-proBNP levels and worse exercise capacity. RA minimal volume was associated with RV end-diastolic volume (r =0.35, p =0.013). Conclusions: In TOF patients with moderate RV dilatation, abnormal bi-atrial function and biventricular diastolic dysfunction are common. Abnormal RA emptying was associated with signs of impaired clinical condition, as was the presence of EDFF. These parameters, together with RA enlargement, could serve as useful markers for clinically relevant RV diastolic dysfunction. [ABSTRACT FROM AUTHOR]

Aims: Aortic coarctation (CoA) occurs as a “simple” isolated disorder, and in a more “complex” form, combined with associated congenital cardiac abnormalities. Long term outcome of all CoA patients may be complicated by dilatation of the thoracic aorta. The aim of this study was to quantify progressive aortic dilatation, and identify determinants for progressive aortic dilatation. Methods and Results: Cardiovascular Magnetic Resonance Imaging (CMR) and echocardiographic data of 93 CoA patients were analyzed retrospectively on the progression, and determinants, of progressive thoracic aortic dilatation. Outcome of simple- versus complex CoA patients were compared. 93 CoA patients (mean age 39±12years, male 59%) were followed with CMR (follow-up 5.3±1.8years). Twenty-eight patients were classified as simple- and 68 as complex CoA. The mean progression rate of thoracic aortic dilatation was highest in the ascending aorta with 2.2±2.0mm/5years (range 0–7.2mm/5years). History of VSD (β=1.77, P=0.004) and an increased left ventricular mass index (β=0.02, P=0.04) were associated with progressive ascending aortic dilatation. Complex CoA patients show an increased progression rate compared to simple CoA patients with 2.4mm/5years versus 1.5mm/5years respectively. (P=0.03). Conclusion: Adult post-coarctectomy patients show an increased mean progression rate of ascending aortic dilatation with 2.2mm/5years. The progression rate of ascending aortic dilatation is increased in complex CoA patients, as compared to simple CoA patients. These findings point towards a more comprehensive genetic subset of patients with an increased risk for progressive ascending aortic dilatation. [ABSTRACT FROM AUTHOR]

Aims An endothelin-1 receptor blocker, shown to be effective in patients with pulmonary arterial hypertension, might decrease pulmonary vascular resistance to increase cardiac filling and consequently improve exercise capacity in Fontan patients. Methods and results This was a prospective, multicentre randomized open label trial in Fontan patients. One group received bosentan for 6 months. The other group did not receive study medication for the first 3 months, followed by bosentan for 6 months. The primary endpoint was exercise capacity, and secondary endpoints were NT-proBNP level, cardiac output, SF-36 (Short Form-36) quality of life (QoL), and NYHA class. Forty-two adults (median age 29 (range 18–56) years, 52% male, 88% NYHA class I–II) from five tertiary referral centres participated in the study. Ten patients were on diuretics. Ten patients were not motivated to finish the study. Analysis of all 32 patients who finished the study at 6 months of treatment showed that mean peak V'O2 (24 vs. 25 mL/kg/min), median SQUASH score (6614 vs. 6390), median NT-proBNP (314 vs. 274 ng/L), and mental QoL (50 vs. 51) remained unchanged as compared with baseline (P = NS, for all). After treatment, NYHA class had improved in 6 (19%), was unchanged in 24 (75%), and declined in 2 (6%) patients. Subgroup analysis on age, ventricular morphology, type of Fontan circulation, or baseline NT-proBNP level did not reveal efficacy of bosentan. Six transient adverse effects were reported. Conclusion An increased NT-proBNP level was present in the majority of Fontan patients. Six months of bosentan treatment was not beneficial. Trial registration NTR1557 [ABSTRACT FROM AUTHOR]

Background-The role of angiotensin II receptor blockers in patients with a systemic right ventricle has not been elucidated. Methods and Results-We conducted a multicenter, double-blind, parallel, randomized controlled trial of angiotensin II receptor blocker valsartan 160 mg twice daily compared with placebo in patients with a systemic right ventricle caused by congenitally or surgically corrected transposition of the great arteries. The primary end point was change in right ventricular ejection fraction during 3-year follow-up, determined by cardiovascular magnetic resonance imaging or, in patients with contraindication for magnetic resonance imaging, multirow detector computed tomography. Secondary end points were change in right ventricular volumes and mass, ̇vo2peak, and quality of life. Primary analyses were performed on an intention-to-treat basis. A total of 88 patients (valsartan, n=44; placebo, n=44) were enrolled in the trial. No serious adverse effects occurred in either group. There was no significant effect of 3-year valsartan therapy on systemic right ventricular ejection fraction (treatment effect, 1.3%; 95% confidence interval, -1.3% to 3.9%; P=0.34), maximum exercise capacity, or quality of life. There was a larger increase in right ventricular end-diastolic volume (15 mL; 95% confidence interval, 3-28 mL; P<0.01) and mass (8 g; 95% confidence interval, 2-14 g; P=0.01) in the placebo group than in the valsartan group. Conclusions-There was no significant treatment effect of valsartan on right ventricular ejection fraction, exercise capacity, or quality of life. Valsartan was associated with a similar frequency of significant clinical events as placebo. Small but significant differences between valsartan and placebo were present for change in right ventricular volumes and mass. [ABSTRACT FROM AUTHOR]

Background: Type D personality, characterized by high levels of negative affectivity and social inhibition, is related to mortality, morbidity, poor health status, quality of life (QoL) and less healthcare utilization in various cardiovascular patient groups. To date, studies in patients with congenital heart disease (CHD) are lacking. Aims: (1) To examine the prevalence of Type D personality in CHD patients; (2) to compare Type D to non-Type D patients with regard to disease severity, functional status, health status and QoL; and (3) to examine the extent to which Type D personality is independently related to healthcare utilization. Methods: A total of 1109 adult CHD patients were included in a questionnaire survey. Due to missing data, 302 patients were excluded. Results: The prevalence of Type D personality was 20.4%. Type D patients reported a poorer functional status, health status and QoL than non-Type D patients (p <0.05). Type D patients reported less healthcare use than non-Type D patients (primary and cardiac outpatient healthcare: adjusted OR=0.56, 95% CI=0.35–0.90; inpatient healthcare: adjusted OR=0.38, 95% CI=0.17–0.83). Results of a post-hoc analysis showed a high prevalence of Type D personality in patients with a poor functional status who did not consult their cardiologist. Conclusion: Type D patients report a poorer functional status, health status and QoL, but less healthcare utilization. In clinical practice, patients should be screened for Type D personality, since social inhibition may prevent them from contacting a healthcare provider in the event of symptom aggravation. [ABSTRACT FROM AUTHOR]

Abstract: Background: We hypothesized 1) that long-term ventricular outcome and exercise capacity would be better in patients with isolated pulmonary valve stenosis (PS) treated with balloon pulmonary valvuloplasty (BPV) than in patients operated for tetralogy of Fallot (TOF), and 2) that ventricular outcome and exercise capacity would not be different in PS patients and healthy controls. Methods: We included 21 PS patients after BPV (16.2±5.2years) and 21 patients operated for TOF (16.6±5.6years), matching them for gender, age at treatment, and age at study. Patients underwent cardiovascular magnetic resonance (CMR) imaging, exercise testing, 12-lead ECG and 24-hour Holter monitoring for assessment of right ventricular (RV) size and function, pulmonary regurgitation (PR), exercise capacity and electrocardiographic status. Healthy controls for CMR imaging and exercise testing were matched for gender and age at study. Results: RV volumes and PR percentage were significantly larger in TOF patients than in PS patients; biventricular ejection fraction (EF) was not different. PR was mild in most PS patients. RV end-systolic volume was significantly larger in PS patients than in healthy controls; RVEF was significantly lower. Both patient groups had similar exercise test results. Peak workload and VO2 max. were significantly lower in PS patients than in healthy controls. Conclusions: Longstanding mild PR in PS patients can lead to an enlarged RV, reduced RV function and reduced exercise capacity. Despite more PR and larger RV volumes in TOF patients, exercise capacity and biventricular function are similar in both patient groups. [Copyright &y& Elsevier]

Atrial arrhythmia (AA) is common in adult patients with congenital heart disease (CHD). To enable the prevention of AA or its complications, timely identification of adult patients with CHD at risk of AA is crucial. Long total atrial activation times have been related to AA. Tissue Doppler imaging (TDI) permits noninvasive evaluation of the total atrial conduction time (PA-TDI duration). The present study evaluated the association between the PA-TDI duration and the development of AA in adult patients with CHD. A total of 223 adult patients with CHD were followed up for the occurrence of AA after PA-TDI duration assessment. The PA-TDI duration was defined as the interval from the onset of the P wave on the electrocardiogram to the peak of the A′ wave at the lateral atrial wall on TDI tracings. Among the various clinical and echocardiographic parameters, the association between the PA-TDI duration and AA occurrence was investigated. The median follow-up was 39 months (interquartile range 21 to 57). A PA-TDI duration of ≥126 ms was associated with AA during follow-up (log-rank, p <0.001). On multivariate analysis, a PA-TDI duration >126 ms (hazard ratio 2.25, 95% confidence interval 1.21 to 4.19) and history of AA (hazard ratio 4.89, 95% confidence interval 2.75 to 8.71) were independently associated with the occurrence of AA. In conclusion, PA-TDI duration and a history of AA were independently associated with the occurrence of AA in adult patients with CHD. The PA-TDI duration is a useful tool to identify patients with CHD at risk of AA during follow-up. [ABSTRACT FROM AUTHOR]

Abstract: Background: HMG-coA-reductase-inhibitors (statins) have been proven to reduce atherosclerosis progression as observed by carotid intima-media thickness in patients with known coronary heart disease, independent of lipid lowering. Cardiovascular complications are common in patients after successful coarctation repair. The effect of statins on cardiovascular risk in adults after successful coarctation repair has not yet been established. Methods: We designed a multicentre, prospective, randomised, open label trial to evaluate the effect of the HMGcoA-reductase-inhibitor (Atorvastatin) on atherosclerotic progression in adult post-coarctectomy patients. The primary endpoint in this study is the carotid intima-media thickness as measured by Bmode ultrasonography of the carotid arteries. Conclusion: This large prospective, randomised, open label trial will establish the effect of HMG-coA-reductase inhibitors (Atorvastatin) on cardiovascular risk in adult patients after successful coarctation repair. [Copyright &y& Elsevier]

Chronic hypertension is a major concern in adults who have undergone resection of coarctation of the aorta (CoA) in childhood. In otherwise healthy subjects, exercise-induced hypertension is prognostic for chronic hypertension; however, the prognostic value in patients with CoA remains unknown. The aim of the present study was to evaluate the predictive value of exercise-induced hypertension for chronic hypertension in these patients. In the present prospective follow-up study, 74 patients with CoA (58% men, age 30.9 ± 9.5 years) underwent ambulatory blood pressure (BP) monitoring and exercise testing twice from 2001 to 2009 with a follow-up period of 6.3 ± 0.8 years. Hypertension was defined as a mean systolic BP ≥140 mm Hg and/or mean diastolic BP ≥90 mm Hg or the need for antihypertensive treatment. Exercise-induced hypertension was defined as a mean systolic BP of <140 mm Hg and peak exercise systolic BP of ≥200 mm Hg. At baseline, 27 patients (36%) were hypertensive, 11 (15%) had exercise-induced hypertension, and 36 (49%) were normotensive. At follow-up, all 27 hypertensive patients remained hypertensive. Of the 11 with exercise-induced hypertension, 7 (64%) had developed chronic hypertension, and 4 (36%) continued to have exercise-induced hypertension. Of the 36 normotensive patients, 7 (19%) had developed hypertension, 12 (33%) had developed exercise-induced hypertension, and 17 (47%) remained normotensive. On multivariate analysis, baseline maximum exercise systolic BP was independently associated with the mean systolic BP at follow-up (β = 0.13, p = 0.005). In conclusion, the maximum exercise systolic BP was a predictor for chronic hypertension in patients with CoA. These findings demonstrate the clinical importance of exercise-induced hypertension and warrant additional study into the long-term consequences of exercise-induced hypertension and the potential beneficial role of early antihypertensive treatment in adult patients after CoA repair with exercise-induced hypertension. [Copyright &y& Elsevier]

Abstract: Background: The Fontan circulation is a palliative procedure performed in patients with complex congenital heart disease (CHD), making transpulmonary blood flow dependent on the systemic venous pressure. In a Fontan circulation a low pulmonary vascular resistance (PVR) is crucial, as is epitomized by the observation that a high PVR is a strong predictor of mortality. Long-term follow-up has shown that PVR may rise many years after the Fontan procedure has been performed, possibly due to micro-emboli from a dilated right atrium or from the venous system. Other mechanisms of increased PVR might be aging, obstructed airways caused by lymphatic dysfunction, lack of pulsatile pulmonary flow causing a release of endothelium-derived vasoactive molecules, and prolonged overexpression of vasoconstrictors such as endothelin-1. Mean plasma level of endothelin-1 has been shown to be significantly higher in Fontan patients compared to healthy controls. In patients with pulmonary arterial hypertension (PAH), therapy with bosentan, an endothelin-1 receptor antagonist, has demonstrated to improve exercise capacity and to reduce the elevated PVR. In addition, reduction of PVR is shown early and late after the Fontan procedure on treatment with exogenous NO, another advanced PAH therapy. However, the long term effect of reducing the PVR by bosentan treatment on exercise capacity in Fontan patients is still unknown. Methods: We designed a prospective, multicenter, randomized open label trial to study the effect of bosentan in Fontan patients. The primary endpoint will be the change in maximum exercise capacity (peak V''O2). Conclusion: We hypothesize that treatment with bosentan, an endothelin-1 receptor antagonist, improves maximum exercise capacity and functional capacity in adult Fontan patients. [Copyright &y& Elsevier]

The article discusses the conducted study to evaluate the quality of life (QoL) of patients with a history of acute pulmonary embolism (PE). QoL refers on the effect of a disease, as well as its treatment on the physical, psychologic and social function and well being of the patients. In the study, univariate analysis and linear regression analysis were used to analyze and identify the independent determinants of QoL.

Objectives: The aim of this study was to analyze whether QRS duration, before and after pulmonary valve replacement (PVR), is related to long-term outcome in patients with tetralogy of Fallot (TOF). Background: Key factors that determine outcome after PVR in adult TOF patients are largely unknown. Recognition of such factors assists the identification of patients at increased risk of adverse events. Methods: Adults who previously underwent total correction for TOF (n = 90; age 31.4 ± 10.3 years) and required PVR for pulmonary regurgitation were included. The QRS duration was measured pre-operatively and 6 months after PVR. The post-operative changes in QRS duration were calculated. Adverse events (death, re-PVR, ventricular tachycardia, and symptomatic heart failure) were noted during follow-up. Results: During 5.5 ± 3.5 years of follow-up, 13 adverse events occurred. The 5-year event-free survival rate was 76% for patients with a pre-operative QRS duration >180 ms and 90% in patients with a QRS duration ≤180 ms (p = 0.037). For patients with a post-operative QRS duration >180 ms, 5-year event-free survival was 71%, whereas it was 91% for patients with a post-operative QRS duration ≤180 ms (p = 0.004). After multivariate correction, a post-operative QRS duration >180 ms (hazard ratio: 3.685, 95% confidence interval: 1.104 to 12.304, p < 0.05) and the absence of a reduction in QRS duration post-PVR (hazard ratio: 6.767, 95% confidence interval: 1.704 to 26.878, p < 0.01), was significantly associated with adverse outcome. Conclusions: Severe QRS prolongation, before or after PVR, and the absence of a reduction in QRS duration after PVR, are major determinants of adverse outcome during long-term follow-up of patients with TOF. [ABSTRACT FROM AUTHOR]

Heart rate (HR) at rest is an important marker of prognosis in heart failure, but has not been addressed in pulmonary arterial hypertension (PAH). To determine the prognostic value of HR at rest in patients with PAH, we retrospectively analyzed 140 consecutive patients with idiopathic PAH. Electrocardiogram (ECG)-derived HR at rest was evaluated as a potential predictor of adverse prognosis (death or lung transplantation), in addition to World Health Organization functional class, 6-minute walk distance, and hemodynamics before and approximately 1 year and 2 years after initiation of PAH treatment. During follow-up, 49 patients (35%) died, and 5 patients (4%) underwent lung transplantation. Before treatment initiation and after 1 year and 2 years of treatment, respectively, a higher HR at rest was an independent predictor of adverse prognosis (hazard ratios per 10-beats/min increase 1.76, 95% confidence interval 1.42 to 2.18, 2.31, 95% confidence interval 1.58 to 3.38, 2.1, 95% confidence interval 1.39 to 3.19, respectively, p <0.001 for all). Change in HR between the first and last ECG also independently predicted prognosis (hazard ratio per 1-beat/min increase 1.03, 95% confidence interval 1.01 to 1.06). In conclusion, a higher HR at rest and an important increase in HR at rest during follow-up signify a considerable risk of death in patients with PAH. ECG-derived HR at rest is an important marker of prognosis and should be assessed before and at frequent intervals after initiation of treatment for PAH. [Copyright &y& Elsevier]

The article assesses whether the electrocardiogram (ECG) is useful for monitoring treatment response based on changes in pulmonary vascular resistance (PVR) in patients with pulmomary arterial hypertension (PAH). An ECG was recorded in 81 PAH patients at the time of diagnostic right heart catheterization and a year after treatment. Results conclude that routine ECG evaluation can be a vital contribution to assess the treatment response in PAH patients.

Abstract: Background: Pregnancy in women with congenital aortic stenosis (AS) is associated with increased cardiac complications. Data on non-cardiac complications are limited, and this information is crucial for prenatal counselling and perinatal care. The aim of this study was to present the maternal and perinatal outcome of pregnancy in women with congenital AS. Methods: By review of the Dutch CONCOR national registry and a local Belgian tertiary care centre database, 35 women with congenital AS with a history of completed pregnancy before aortic valve replacement were enrolled in this study. Medical history and maternal and perinatal outcome were determined. Results: Thirty-five women had 58 pregnancies resulting in 53 successful pregnancies, three miscarriages, and two abortions. The most serious cardiac complications were heart failure (n =2, 3.8%) and atrial arrhythmia (n =3, 5.7%). Although cardiac complications were present (9.4%), obstetric (22.6%) and perinatal (24.5%) complications were observed more often. A total of six pregnancies (11.3%) were complicated by hypertension-related disorders, including one case of eclampsia. Furthermore, 7 premature births (13.2%) and 7 small-for-gestational-age births (13.2%) were encountered. Pregnancy in women with severe AS was characterized by an increased incidence of heart failure and premature labour, and shorter pregnancy duration. Older women (>30 years) were at increased risk of perinatal events (odds ratio 4.38, 95% confidence interval 1.02 to 18.81). Conclusions: Pregnancy is generally well tolerated in women with congenital AS. Importantly, an excess of obstetric and perinatal complications was found, requiring more meticulous attention. [Copyright &y& Elsevier]