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Objectives In the general population, planned caesarean section is thought to be safer in high-risk situations as it avoids the greater risk of an emergency caesarean section. Only limited data exist on the optimal mode of delivery in women with structural heart disease. We investigated the relationship between mode of delivery and pregnancy outcome in women with preexisting heart disease. Methods The Registry on Pregnancy and Cardiac Disease is an on-going, global, prospective observational registry of women with structural heart disease. We report on 1262 deliveries, between January 2007 and June 2011. Results The caesarean section was planned in 393 women (31%): 172 (44%) for cardiac and 221 (56%) for obstetric reasons of whom 53 delivered by emergency caesarean section. Vaginal delivery was planned in 869 (69%) women, of whom 726 (84%) actually delivered vaginally and 143 (16%) had an emergency caesarean section. Perinatal mortality(1.1 vs 2.7, p=0.14) and low apgar score (11.9 vs 10.1, p=0.45) were not significantly different in women who had a caesarean section or vaginal delivery; gestational age(37 vs 38 weeks p=0.003) and birth weight (3073 vs 2870 g p

Objective The objective of this study was to determine outcomes in pregnant women with pre-existing coronary artery disease (CAD) or following an acute coronary syndrome (ACS) including myocardial infarction (MI).Background The physiological changes of pregnancy can contribute to myocardial ischaemia. The pregnancy risk for women with pre-established CAD or a history of ACS/MI is not well studied.Methods This was a retrospective multicentre study. Adverse maternal cardiac, obstetric and fetal/neonatal events were examined. The primary outcome was a composite endpoint of cardiac arrest, ACS/MI, ventricular arrhythmia or congestive heart failure. The prevalence of new or progressive angina during pregnancy was also examined.Results Fifty pregnancies in 43 women (mean age 35 +/- 5 years) were included. Coronary atherosclerosis (40%) and coronary thrombus (36%) were the most common underlying diagnoses. The primary outcome occurred in 10% (5/50) of pregnancies and included one maternal death secondary to cardiac arrest. Other events included ACS/MI (3/50) and heart failure (1/50). New or progressive angina occurred in 18% of pregnancies. Ischaemic complications of any type (new or progressive angina, ACS/MI, ventricular arrhythmia, cardiac arrest) occurred more commonly in women with coronary atherosclerosis compared with those without (50% vs 10%, p=0.003). A high rate of adverse obstetric (16%) and fetal/neonatal (30%) events was observed.Conclusions Pregnant women with pre-existing CAD or ACS/MI before pregnancy are at increased risk of adverse events during pregnancy. Those with coronary atherosclerosis are at highest risk of adverse maternal cardiac events due to myocardial ischaemia during pregnancy.

OBJECTIVES: The surgical approach to repair of tetralogy of Fallot (ToF) has shifted over the years. We aimed to report the long-term follow-up after ToF repair with the transatrial–transpulmonary approach and to determine predictors of long-term outcomes. METHODS: Retrospective analysis of patients operated on in two tertiary referral centres. Primary outcome measures were: death, pulmonary valve replacement (PVR), reintervention for other reasons, internal cardiodefibrillator and/or pacemaker placement. Kaplan– Meier assessment of overall and event-free survival as well as uni- and multivariate analyses of risk factors for outcomes were performed. RESULTS: Four hundred and fifty-three patients were included. Median age at operation was: 0.6 years (range 0–19.6) and median age at the last follow-up was 14.3 years (range 0.1–42.1). Median age at repair decreased from 1.2 years (range 0.6–5.8) (1970–80) to 0.3 years (range 0–4.7) (2000–12). A transannular patch (TP) was used in 65% of all patients. The use of a TP showed a decline from 89% in the initial years of the cohort to 64% in 2000–12. Early mortality was 1.1% (5 patients) for the entire cohort and late mortality 2.4% (11 patients). Overall survival for the entire cohort was 97.3% (95% CI 95.7–98.8) and 91.8% (95% CI 85.9–97.7) at 10 and 25 years, respectively. For patients with a TP (n= 294) vs non-TP (n= 159), this was 97.2% (95% CI 95.2–99.2) vs 97.5% (95% CI 95.1–99.9) at 10-year and 91.0% (95% CI 83.9–98.1) vs 96.3% (95% CI 93.0–99.6) at 25-year follow-up (P= 0.958). Fifty-two patients underwent PVR, and in 5 a pacemaker was inserted. Event-free survival for TP versus non-TP patients was 80.2% (95 CI% 75.5–84.9) vs 81.7% (95% CI 75.2–88.2) at 10-year and 27.9% (95% CI 17.7–38.1) vs 78.5% (95% CI 71.4–85.6) at 25-year follow-up (P= 0.016). In multivariate analysis, both the use of a TP (HR 1.705, 95% CI 1.023–2.842) and the year of surgical repair of tetralogy of Fallot (HR 1.039, 95% CI 1.006–1.073) were associated with a higher probability of an event. CONCLUSIONS: ToF patients corrected with the transatrial–transpulmonary approach have good long-term survival. PVR is a frequent event at longer follow-up, and other events are limited. The use of a TP is a predictor for poorer event-free outcomes, increasing the risk of the composite endpoint 1.7 times.

Background: Adult congenital heart disease (ACHD) patients are at risk of late complications including arrhythmias, heart failure and sudden death. High-sensitive troponin-T (hs-TnT) is the standard for diagnosing acute coronary syndrome, but is also associated with cardiac function and prognosis in other cardiac diseases. We aimed to describe hs-TnT level in ACHD patients, and determine its relationship with cardiac function and other biomarkers. Methods: Consecutive ACHD patients, visiting the outpatient clinic, underwent echocardiography, exercise testing and venipuncture on the same day. Results: In total 587 patients were included (median age 33 [IQR 25-41] years, 58% male, 90% NYHA class I). hs-TnT was above the detection limit of 5 ng/L in 241 patients (41%), of whom 47 (8%) had hs-TnT levels above the 99th percentile of normal of 14 ng/L. hs-TnT levels were highest in patients with a systemic RV or pulmonary hypertension. Patients with normal or non-detectable hs-TnT were younger (32 [IQR 24-40] years) than patient with elevated hs-TnT (42 [IQR 36-60] years, p < 0.001). The prevalence of hs-TnT >= 14 ng/L was higher in patients with NYHA >= II (36%, p < 0.001), systemic systolic dysfunction (38%, p < 0.001), non-sinus rhythm (43%, p < 0.001) and elevated pulmonary pressures (39%, p < 0.001). hs-TnT was correlated with NT-proBNP (r = 0.400, p < 0.001). Conclusions: hs-TnT above the 99th percentile of normal is observed in a non-trivial portion of stable ACHD patients, especially in those with a systemic RV or elevated pulmonary pressures. Since this biomarker of myocardial damage is related to NT-proBNP and ventricular function, its potential predictive value in ACHD patients seems promising and further investigation of underlying mechanisms is warranted. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

Background: Stress is considered a modifiable risk factor for cardiovascular disease. Scalp hair analysis is a tool to assess long-term exposure to the stress hormone cortisol. We aimed to determine the association between hair cortisol concentrations (HCC) and clinical characteristics in patients with structural heart disease. Additionally, we investigated potential predictors for longitudinal change in HCC. Methods: The study consisted of 261 patients with structural heart disease from a randomized controlled trial of mindfulness training. One sample of scalp hair was used to determine HCC both at baseline and at 12-week follow-up. In 151 patients, HCC was available (mean age: 41.3 years, range 18-65). We investigated the association between HCC at baseline and several physiological measures (BMI, blood pressure, heart rate, respiratory rate, 6-minute walk test), as well as psychological parameters (physical and mental component summary measures (SF-36), emotional distress (HADS), and perceived stress). Additionally, we used these clinical parameters to predict HCC change over time. Results: The median HCC was 22.3 pg/mg of hair (23.5 interquartile range). In multivariable linear regression analyses, an association was observed between log-transformed HCC and BMI (beta -0.171, p = 0.037), respiratory rate (beta -0.194, p = 0.016), and the physical summary score (beta-0.163, p = 0.054). Independent predictors of log-transformed HCC change after 12 weeks were mental summary score (beta-0.200, p= 0.019) and diastolic blood pressure (beta -0.171, p = 0.049). Conclusions: In patients with structural heart disease a positive association exists between HCC and BMI. Mental health status may predict a change in long-term cortisol over time. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

Middle-aged and elderly women constitute a large and growing proportion of the population. The peri and postmenopausal period constitutes a challenging transition time for women's health, and menopausal health is a crucial aspect in healthy and successful aging. Currently, no framework for the concept of healthy menopause exists, despite its recognized importance. Therefore, we aimed to: (i) characterize healthy menopause; (ii) identify aspects that contribute to it; and (iii) explore potential approaches to measure it. We propose healthy menopause as a dynamic state, following the permanent loss of ovarian function, which is characterized by self-perceived satisfactory physical, psychological and social functioning, incorporating disease and disability, allowing the attainment of a woman's desired ability to adapt and capacity to self-manage. The concept of healthy menopause applies to all women from the moment they enter the menopausal transition, up until they reach early and late postmenopause and includes women with spontaneous, iatrogenic, and premature menopause. This conceptualization can be considered as a further step in the maintenance and improvement of health in menopausal women from different perspectives, foremost the woman's own perspective, followed by the clinical, public health, and societal perspectives, and can be seen as a further step in delineating lines for future research. Furthermore, it could facilitate the improvement of adequate preventive and treatment strategies, guide scientific efforts, and aid education and communication to health care practitioners and the general public, allowing women the achievement of their potential and the fulfillment of their fundamental role in society. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

ABSTRACT Cardiovascular disease is the most encountered cause of maternal death during pregnancy in the western world and an increase in maternal mortality due to cardiac causes has been observed. More women with congenital or acquired heart disease have the desire to become pregnant. Pregnancy is known to impose a major hemodynamic burden and also has impacts on the coagulation system. The risk of developing complications is clearly increased as compared with the normal population. For optimal management, it is crucial to have information on the effects of cardiac medications on the fetus. The focus of this article is to discuss the management of cardiac disease in pregnancy, as well as the known safety of cardiac medications for the mother and/or fetus.

Unhealthy lifestyle behaviours are considered modifiable risk factors for many diseases. Lifestyle interventions that target these behaviours need rigorous evaluation to assess their effectiveness. The randomized controlled trial is the study design of choice when it comes to the evaluation of interventions. However, lifestyle interventions are often complex and subject to several important issues, such as patient preference and non-adherence, that may threaten the internal and external validity of studies. There is a strong demand for high-quality randomized controlled trials of interventions that promote healthy lifestyle behaviours. With this tutorial we aim to provide guidance in the choice of an optimal randomized controlled trial design in future trials of lifestyle interventions.

Item does not contain fulltext AIMS: The identification of sex differences in the prognosis of adults with a secundum atrial septal defect (ASD2) could help tailor their clinical management, as it has in other cardiovascular diseases. We investigated whether disparity between the sexes exists in long-term outcome of adult ASD2 patients. METHODS AND RESULTS: Patients with ASD2 classified as the primary defect were selected from the Dutch national registry of adult congenital heart disease. Survival stratified by sex was compared with a sex-matched general population. In a total of 2207 adult patients (mean age at inclusion 44.8 years, 33.0% male), 102 deaths occurred during a cumulative follow-up of 13 584 patient-years. Median survival was 79.7 years for men and 85.6 years for women with ASD2. Compared with the age- and sex-matched general population, survival was lower for male, but equal for female patients (P = 0.015 and 0.766, respectively). Logistic regression analyses showed that men had a higher risk of conduction disturbances (OR = 1.63; 95% CI, 1.22-2.17) supraventricular dysrhythmias (OR = 1.41; 1.12-1.77), cerebrovascular thromboembolic events (OR = 1.53; 1.10-2.12), and heart failure (OR = 1.91; 1.06-3.43). CONCLUSION: In contrast to women, adult men with an ASD2 have worse survival than a sex-matched general population. Male patients also have a greater risk of morbidity during adult life. Sex disparity in survival and morbidity suggests the need for a sex-specific clinical approach towards these patients.

The expanding population of adult patients with tetralogy of Fallot (ToF) requires knowledge of its long-term sequelae.The purpose of this study was to examine the coexistence and order of appearance of atrial fibrillation (AF), other supraventricular tachycardias (SVTs), ventricular tachycardia (VT), and ventricular fibrillation (VF), and their impact on survival during long-term follow-up.Adult, corrected ToF patients [N = 225; 128 male; mean ± SD (minimum-maximum) age 41 ± 12 (19-79) years] were included in the study. Medical correspondence, ECGs, and Holter recordings were reviewed for documented AF, other SVTs, VT, and VF.During follow-up of 35 ± 9 (16-64) years, sustained tachyarrhythmias, including SVT (n=50, 22%), AF (n=29, 13%), VT (n=20, 9%), and VF (n=9, 4%), were observed in 71 patients (32%), of whom 27 (38%) had coexistence of different tachyarrhythmias. In 18 patients with coexisting SVT and AF, SVT most often preceded AF in 13 (72%). Age at SVT onset was similar between those with and those without subsequent AF development (40 ± 17 years vs 35 ± 16 years; P = .283). However, age at SVT and AF onset were positively correlated (rho 0.585; P = .011). Prevalence of SVT/AF was associated with VT/VF prevalence (odds ratio [OR] 4.59; P.001). Although 11% of patients with SVT/AF subsequently develop VT/VF, onset of SVT/AF could not predict future VT/VF development (OR 1.81; P = .233). Adult ToF patients are initially at risk for SVT development, followed by AF, VT, and VF at 46 (43-50), 56 (53-59), 57 (54-61), and 62 (61-63) years after ToF correction (P.001), respectively. Survival time decreased when sustained tachyarrhythmias developed (P = .024). Age at onset of SVT, AF, and VT was positively correlated with age at death (SVT: rho 0.734; P = .004; AF: rho 0.783; P = .007, VT: rho 0.755; P = .050).Coexistence of different (supra)ventricular tachyarrhythmias is frequently observed in adult ToF patients. In these patients, a specific order of these tachyarrhythmias was observed. Tachyarrhythmias are associated with decreased survival time, and, more importantly, age at tachyarrhythmia development positively correlates with age at death.

Aims We report the maternal and foetal outcomes at birth and after 6 months in a cohort of pregnant women with hypertrophic cardiomyopathy (HCM). Although most women with HCM tolerate pregnancy well, there is an increased risk of obstetric and cardiovascular complications.Methods and results All pregnant women with HCM entered into the prospective worldwide Registry of Pregnancy and Cardiac disease (ROPAC) were included in this analysis. The primary endpoint was a major adverse cardiovascular event (MACE), which included death, heart failure (HF), thrombo-embolic event, and arrhythmia. Baseline and outcome data were analysed and compared for patients with MACE vs. without MACE and for patients with obstructive HCM vs. non-obstructive HCM. Sixty pregnant women (mean age 30.4 +/- 6.0 years) with HCM (41.7% obstructive) were included. No maternal mortality occurred in this cohort. In 14 (23%) patients at least one MACE occurred: 9 (15.0%) HF and 7 (12%) an arrhythmia (6 ventricular and 1 atrial fibrillation). MACE occurred most commonly during the 3rd trimester and postpartum period. In total, 3 (5.0%) women experienced foetal loss. Women with MACE had a higher rate of emergency Caesarean delivery for cardiac reasons (21.4% vs. 0%, P = 0.01). No significant differences in pregnancy outcome were found between women with obstructive and non-obstructive HCM. NYHA functional class of >= II and signs of HF before pregnancy, were associated with MACE.Conclusion Although most women with HCM tolerated pregnancy well, cardiovascular complications were not uncommon and predicted by pre-pregnancy status facilitating pre-pregnancy counselling and targeted antenatal care.

Atrial fibrillation (AF) and other supraventricular tachycardias (SVTs) are known complications after surgical repair of atrial septal defect (ASD), but sinus node dysfunction (SND) and complete atrioventricular conduction block (cAVB) may also occur.The aim of this study was to examine time course and interrelationship of various dysrhythmias in patients with ASD.Adult patients (N = 95) with surgically repaired secundum ASD (n = 40), partial atrioventricular septal defect (n = 37) or sinus venosus defect (n = 18), and documented SND, cAVB, AF, and/or other SVT were included. The median age at repair was 13 years (interquartile range [IQR] 6-45 years), and patients were followed for 26 years (IQR 15-37 years) after ASD repair.SND was observed in 34 patients (36%), cAVB in 14 (14%), AF in 48 (49%), and SVT in 44 (45%); 37 patients (39%) had ≥2 dysrhythmias. All dysrhythmias presented most often after ASD repair (P.01), with a median duration of 12 years (IQR 17 days - 32 years) to 16 years (IQR 4 - 28 years) between repair and onset. Development of SND and cAVB late after ASD repair was not related to a redo procedure in 100% and 60% of patients, respectively. SND preceded atrial tachyarrhythmias in 50% (P = .31) and SVT preceded AF in 68% (P = .09) of patients with both dysrhythmias.A substantial number of dysrhythmias presented (very) late after ASD repair. In most patients, development of late SND and cAVB was not related to redo procedures. In patients with multiple dysrhythmias, a specific order of appearance was not observed.

The Loeys–Dietz syndrome (LDS) is a connective tissue disorder affecting the cardiovascular, skeletal, and ocular system. Most typically, LDS patients present with aortic aneurysms and arterial tortuosity, hypertelorism, and bifid/broad uvula or cleft palate. Initially, mutations in transforming growth factor-β (TGF-β) receptors (TGFBR1 and TGFBR2) were described to cause LDS, hereby leading to impaired TGF-β signaling. More recently, TGF-β ligands, TGFB2 and TGFB3, as well as intracellular downstream effectors of the TGF-β pathway, SMAD2 and SMAD3, were shown to be involved in LDS. This emphasizes the role of disturbed TGF-β signaling in LDS pathogenesis. Since most literature so far has focused on TGFBR1/2, we provide a comprehensive review on the known and some novel TGFB2/3 and SMAD2/3 mutations. For TGFB2 and SMAD3, the clinical manifestations, both of the patients previously described in the literature and our newly reported patients, are summarized in detail. This clearly indicates that LDS concerns a disorder with a broad phenotypical spectrum that is still emerging as more patients will be identified. All mutations described here are present in the corresponding Leiden Open Variant Database., 0, SCOPUS: ar.j, info:eu-repo/semantics/published

There is increasing evidence that mindfulness can reduce stress, and thereby affect other psychological and physiological outcomes as well. Earlier, we reported the direct 3-month results of an online modified mindfulness-based stress reduction training in patients with heart disease, and now we evaluate the effect at 12-month follow-up. 324 patients (mean age 43.2 years, 53.7% male) were randomized in a 2:1 ratio to additional 3-month online mindfulness training or to usual care alone. The primary outcome was exercise capacity measured with the 6 minute walk test (6MWT). Secondary outcomes were blood pressure, heart rate, respiratory rate, NT-proBNP, cortisol levels (scalp hair sample), mental and physical functioning (SF-36), anxiety and depression (HADS), perceived stress (PSS), and social support (PSSS12). Differences between groups on the repeated outcome measures were analyzed with linear mixed models. At 12-months follow-up, participants showed a trend significant improvement exercise capacity (6MWT: 17.9 meters, p = 0.055) compared to UC. Cohen's D showed significant but small improvement on exercise capacity (d = 0.22; 95%CI 0.05 to 0.39), systolic blood pressure (d = 0.19; 95%CI 0.03 to 0.36), mental functioning (d = 0.22; 95%CI 0.05 to 0.38) and depressive symptomatology (d = 0.18; 95%CI 0.02 to 0.35). All other outcome measures did not change statistically significantly. In the as-treated analysis, systolic blood pressure decreased significantly with 5.5 mmHg (p = 0.045; d = 0.23 (95%CI 0.05-0.41)). Online mindfulness training shows favorable albeit small long-term effects on exercise capacity, systolic blood pressure, mental functioning, and depressive symptomatology in patients with heart disease and might therefore be a beneficial addition to current clinical care. Trial registration www.trialregister.nl NTR3453.

Aims: Assessment of feasibility, accuracy and applicability in clinical practice of real-time three-dimensional echocardiography (RT-3DE) in children with congenital heart defects. Methods and results: From September 2004 to June 2005, 100 consecutive children (57 infants, 43 children > 1 year of age), who were scheduled for corrective intracardiac surgery, were enrolled in this study. RT-3DE was performed with Philips Sonos 7500 echo-system and off-line analysis with TomTec Echoview© software. Quantitative and qualitative assessments of the region of interest were performed on the 3D reconstruction, comparing these results with the anatomic findings and measurements performed during intracardiac surgery. Acquisition of RT-3DE datasets was feasible in 92 of the 100 (92%) patients and acquisition time was 6 ± 3 minutes. The overall quantitative analyses showed an excellent correlation (r < 0.90) between RT-3De and surgery. Also the qualitative analyses were accurate compared with surgical findings, in all patients in which RT-3DE was feasible. Conclusion: This study shows that RT-3DE can be used in the clinical practice for the assessment of intracardiac anatomy in children with congenital heart disease. The information derived from the 3D reconstructions can be taken into consideration in the preoperative planning and management regarding interventional or surgical therapy.

Due to the enormous improvements in the treatment of children with congenital heart disease, an increasing number of patients reach adulthood nowadays. To achieve the aim of excellent care concerning pregnancy in this patient population, a pro-active discussion of pregnancy with all women in this patient population is essential. Several risk stratifi cation models have been developed in order to identify high risk pregnancies and better inform patients about these risks. Counselling and dedicated care are needed to optimise both maternal and fetal outcomes. Better access to health care systems contributes to further improvements in mortality and morbidity. Furthermore a well-developed, multidisciplinary plan for each individual patient concerning pregnancy, delivery and follow-up is essential.

Objective: To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS). Methods: Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years. Results: Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (

Background: Three-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF). Objectives: To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Methods: The study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years). They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men). Results: Increased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls. Conclusions: Significant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair. Resumo Fundamento: Ecocardiografia tridimensional (3D) acoplada à técnica de speckle-tracking (3DSTE) é uma nova metodologia útil para a avaliação de volumes e propriedades funcionais do átrio esquerdo (AE). Há crescente interesse científico na análise da deformação miocárdica em adultos com tetralogia de Fallot corrigida (cTOF). Objetivos: Comparar os volumes de AE, propriedades funcionais baseadas no volume e parâmetros de strain entre pacientes com cTOF e controles saudáveis pareados por idade e sexo. Métodos: A população do estudo consistiu em 19 adultos com cTOF consecutivos, em ritmo sinusal, acompanhados na Universidade Szeged, Hungria (idade média: 37,9 ± 11,3 anos; 8 homens; com correção cirúrgica aos 4,1 ± 2,5 anos de idade). Todos foram submetidos a ecocardiografia transtorácica bidimensional com Doppler padrão e 3DSTE. Os resultados foram comparados aos de 23 controles saudáveis pareados por idade e sexo (idade média: 39,2 ± 10,6 anos; 14 homens). Resultados: Aumento dos volumes de AE e redução das frações de esvaziamento de AE em relação ao ciclo cardíaco foram demonstrados em pacientes com cTOF em comparação aos dos controles. Os volumes de ejeção de AE caracterizando todas as funções do AE não diferiram entre os dois grupos. Strains de AE global e segmentar médio uni- e multidimensional, caracterizando função de reservatório de AE, estavam diminuídos em adultos com cTOF em comparação aos de controles. À semelhança dos strains de pico, reduzidos strains de AE global e segmentar médio na contração atrial, caracterizando função de bomba atrial, foram demonstrados em pacientes com cTOF em comparação aos de controles. Conclusões: Demonstrou-se significativa deterioração das funções de AE em adultos com cTOF em fase tardia após correção.

Background— A Dutch online patient decision aid to support prosthetic heart valve selection was recently developed. A multicenter randomized controlled trial was conducted to assess whether use of the patient decision aid results in optimization of shared decision making in prosthetic heart valve selection. Methods and Results— In a 5-center randomized controlled trial, patients were allocated to receive either standard preoperative care (control group) or additional access to the patient decision aid (intervention group). Legally capable adult patients accepted for elective isolated or combined aortic and mitral valve replacement were included. Primary outcome was preoperative decisional conflict (Decisional Conflict Scale); secondary outcomes included patient knowledge, involvement in valve selection, anxiety and depression, (valve-specific) quality of life, and regret. Out of 306 eligible patients, 155 were randomized (78 control and 77 intervention). Preoperative decisional conflict did not differ between the groups (34% versus 33%; P =0.834). Intervention patients felt better informed (median Decisional Conflict Scale informed subscore: 8 versus 17; P =0.046) and had a better knowledge of prosthetic valves (85% versus 68%; P =0.004). Intervention patients experienced less anxiety and depression (median Hospital Anxiety and Depression Scale score: 6 versus 9; P =0.015) and better mental well-being (mean Short Form Health Survey score: 54 versus 50; P =0.032). Three months postoperatively, valve-specific quality of life and regret did not differ between the groups. Conclusions— A patient decision aid to support shared decision making in prosthetic heart valve selection does not lower decisional conflict. It does result in more knowledgeable, better informed, and less anxious and depressed patients, with a better mental well-being. Clinical Trial Registration— http://www.trialregister.nl . Unique identifier: NTR4350.

Item does not contain fulltext Fragmented QRS complexes (fQRS) on 12-lead electrocardiogram are known predictors of ventricular tachyarrhythmia (VTA) in patients with coronary artery disease. There is limited knowledge of the clinical implications of fQRS in patients with congenital heart defects (CHD). Aims of this study were to examine (1) the occurrence of fQRS in patients with various types of CHD and (2) whether fQRS is associated with development of VTA. This study was designed as retrospective case-control study. Patients with CHD with VTA were included and matched with control patients of the same age, gender, and CHD type. Clinical data and fQRS were analyzed and compared. The initial VTA episode developed in 139 patients with CHD at a mean age of 39 +/- 14 years. Compared with controls (n = 219, age 38 +/- 13 years), QRS duration was longer in patients with VTA (110 vs 100 ms; p

Objective: Women with repaired coarctation of the aorta (rCoA) are at risk of hypertensive disorders and other complications during pregnancy. Hypertensive disorders in pregnant women are associated with inadequate uteroplacental flow, which is related to adverse offspring outcome. The aim of this study was to investigate the relationship of maternal cardiac function, placental function and pregnancy complications in women with rCoA.Methods: We included 49 pregnant women with rCoA and 69 controls from the prospective ZAHARA-studies (Zwangerschap bij Aangeboren HARtAfwijkingen, pregnancy in congenital heart disease). Clinical evaluation, echocardiography and uteroplacental Doppler flow (UDF) measurements were performed at 20 and 32 weeks gestation. Univariable regression analysis was performed.Results: Comparison of rCoA and healthy women. In women with rCoA, tricuspid annular plane systolic excursion (TAPSE) decreased during pregnancy (25.7 mm to 22.8 mm, P = 0.006). UDF indices and pregnancy complication rates were similar in both groups. Offspring of rCoA women had lower birth weight (3233 g versus 3578 g, P = 0.001), which was associated with beta-blocker use during pregnancy (beta = -418.0, P = 0.01).Association of cardiac function and UDF. Right ventricular (RV) function before pregnancy (TAPSE) and at 20 weeks gestation (TAPSE and RV fractional area change) were associated with impaired UDF indices (umbilical artery pulsatility index at 20 weeks beta = -0.02, P = 0.01, resistance index at 20 and 32 weeks beta = -0.01, P = 0.02 and beta = -0.02, P = 0.01 and uterine artery pulsatility and resistance index at 20 weeks gestation beta = -0.02, P = 0.05 and beta = -0.01, P = 0.02).Conclusions: Women with rCoA tolerate pregnancy well. However, RV function is altered and is associated with impaired placentation.

Contains fulltext : 176974.pdf (Publisher’s version ) (Open Access) BACKGROUND: Reproductive disorders, such as polycystic ovary syndrome (PCOS), primary ovarian insufficiency (POI) and hypertensive pregnancy disorders (HPD) like pre-eclampsia (PE), are associated with an increased risk of cardiovascular disease (CVD). Detection of early signs of cardiovascular disease (CVD), as well as identification of risk factors among women of reproductive age which improve cardiovascular risk prediction, is a challenge and current models might underestimate long-term health risks. The aim of this study is to assess cardiovascular disease in patients with a history of a reproductive disorder by low-dose computed tomography (CT). METHODS: Women of 45 - 55 years, who experienced a reproductive disorder (PCOS, POI, HPD), are invited to participate in this multicenter, prospective, cohort study. Women will be recruited after regular cardiovascular screening, including assessment of classical cardiovascular risk factors. CT of the coronary arteries (both coronary artery calcium scoring (CACS), and contrast-enhanced coronary CT angiography (CCTA)) and carotid siphon calcium scoring (CSC) is planned in 300 women with HPD and 300 women with PCOS or POI. In addition, arterial stiffness (non-invasive pulse wave velocity (PWV)) measurement and cell-based biomarkers (inflammatory circulating cells) will be obtained. DISCUSSION: Initial inclusion is focused on women of 45 - 55 years. However, the age range (40 - 45 years and/or >/= 55 years) and group composition may be adjusted based on the findings of the interim analysis. Participants can potentially benefit from information obtained in this study concerning their current cardiovascular health and expected future risk of cardiovascular events. The results of this study will provide insights in the development of CVD in women with a history of reproductive disorders. Ultimately, this study may lead to improved cardiovascular prediction models and will provide an opportunity for timely adjustment of preventive strategies. Limitations of this study include the possibility of overdiagnosis and the average radiation dose of 3.5 mSv during coronary and carotid siphon CT, although the increased lifetime malignancy risk is negligible. TRIAL REGISTRATION: Netherlands Trial Register, NTR5531 . Date registered: October 21st, 2015.

Contains fulltext : 169883.pdf (Publisher’s version ) (Open Access) INTRODUCTION: Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to investigate the wish to conceive and the concerns about cardiovascular complications during pregnancy in women with TS. METHODS: The patient association for TS invited all members of >/=18 years old (n = 344) to complete a specifically developed, disease-specific questionnaire, including questions about fertility, wish to conceive, attempts and concerns. Results were compared with previously published results of this questionnaire in women with congenital heart disease. RESULTS: The questionnaire was completed by 89 women (median age 30.1 years, Q1-Q3 = 22.9-39.4). Of them, 51% had 45, X0-monosomy and 38% had >/=1 cardiac abnormality. Seventeen women (19%) had attempted to become pregnant and 12 of them succeeded to become pregnant. Women who had not undertaken attempts to conceive (81%), considered themselves mainly too young or had no partner. Of the total sample, 58% were concerned about the influence of pregnancy on their cardiovascular status. This was higher (75%) in the sample of women with TS and cardiac abnormalities, than in women with congenital heart disease from a previously published cohort (21%), (p < .001). There were no differences in concerns about pregnancy complications between women with TS who respectively had or had not attempted to become pregnant. DISCUSSION: Women with TS, especially those with cardiac abnormalities, show serious concerns about the risks pregnancy may have. Patients should be timely counseled and specifically asked about their concerns. Psychosocial care should be provided when necessary.

For the first time in 2017 adults with CHD was included in the World Congress of Pediatric Cardiology & Cardiac Surgery. With growing numbers and more complex patients reaching adulthood, there is a growing need for more attention to this subspecialty. Although survival is excellent and now over 90% of patients reach adulthood, many have residual problems and complications. Heart failure and arrhythmias are most commonly encountered. Life-expectancy is nearly normal for mild lesions, but remains reduced in moderate-complex lesions and re-interventions are often needed. As most patients want to live a normal life, sports participation and pregnancy become very important issues. Finally, although innovative treatments are being developed, including for end-stage heart failure, we have to refine strategies for optimal care, including during the end-stage lives of our patients. This article provides an overview of a selection of topics in the field of adults with CHD presented during the 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS) in Barcelona.

Background: Data on pharmacological management during pregnancy are scarce. The aim of this study was to describe the type and frequency of cardiac medication used in pregnancy in patients with cardiovascular disease and to assess the relationship between medication use and fetal outcome. Methods and results: Between 2007 and 2011 sixty hospitals in 28 countries enrolled 1321 pregnant women. All patients had structural heart disease (congenital 66%, valvular 25% or cardiomyopathy 7% or ischemic 2%). Medication was used by 424 patients (32%) at some time during pregnancy: 22% used beta-blockers, 8% antiplatelet agents, 7% diuretics, 2.8% ACE inhibitors and 0.5% statins. Compared to those who did not take medication, patients taking medication were older, more likely to be parous, have valvular heart disease and were less often in sinus rhythm. The odds ratio of fetal adverse events in users versus non-users of medication was 2.6 (95% CI 2.0-3.4) and after adjustment for cardiac and obstetric parameter was 2.0 (95% CI 1.4-2.7). Babies of patients treated with beta-blockers had a significantly lower adjusted birth weight (3140 versus 3240 g, p = 0.002). The highest rate of fetal malformation was found in patients taking ACE inhibitors (8%). Conclusion: One third of pregnant women with heart disease used cardiac medication during their pregnancy, which was associated with an increased rate of adverse fetal events. Birth weightwas significantly lower in children of patients taking beta-blockers. A randomized trial is needed to distinguish the effects of the medication from the effects of the underlying maternal cardiac condition. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

Background Lack of evidence-based data on the spectrum of cardiovascular disease (CVD) in pregnancy or in the postpartum period, as well as on maternal and fetal outcome, provides challenges for treating physicians, particularly in areas of low resources. The objectives of this study were to investigate the spectrum of disease, mode of presentation and maternal and fetal outcome of patients referred to a dedicated Cardiac Disease and Maternity Clinic (CDM). Methods The prospective cohort study was conducted at a single tertiary care centre in South Africa. Two hundred and twenty-five women presenting with CVD in pregnancy, or within 6 months postpartum, were studied over a period of 2 years. Clinical assessment, echocardiography and laboratory tests were performed at baseline and follow-up visits. Prepartum, peripartum and postpartum complications were grouped into cardiac, neonatal and obstetric events. Results Ethnicity was black African (45%), mixed ethnicity (32%), white (15%), Indian/others (8%) and 12% were HIV positive. Of the 225 consecutive women (mean age 28.8+/-6.4), 196 (86.7%) presented prepartum and 73 in modified WHO class I. The 152 women presenting in a higher risk group (modified WHO class II-IV) were offered close follow-up at the CDM clinic and were diagnosed with congenital heart disease (32%, 15 operated previously), valvular heart disease (26%, 15 operated previously), cardiomyopathy (27%) and other (15%). Women presenting with symptoms of CVD or heart failure postpartum (n=30) presented in a higher New York Heart Association, had higher heart rates (p42 days postpartum. Perinatal death occurred in 1/152 (0.7%)-translating to a perinatal mortality rate of 7/1000 live births. Conclusions Disease patterns were markedly different to that seen in the developed world. However, joint obstetric-cardiac care in the low-resource cohort was associated with excellent survival outcome rates of pregnant mothers (even with complex diseases) and their offspring and was similar to that seen in the western world. Mortality typically occurred in the postpartum period, beyond the standard date of recording maternal death.

Aims To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair. Methods and results This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age

Aims The aim of this study was to assess the value of two-dimensional (2D) transthoracic simultaneous multiplane imaging (SMPI) in the evaluation of suitability for percutaneous atrial septal secundum defect (ASD) closure compared with the golden standard 2D transoesophageal echocardiography (TEE). Methods and results Twenty-nine patients with an ASD underwent both SMPI and TEE. Ten patients (34%) were male (age 41 +/- 18 years, range 20-74). SMPI assessment of ASD size and rims included xPlane and I-rotate modes. Rims were defined as suitable for ASD percutaneous closure using a cut-off value of 5 mm. There were no significant differences between SMPI in xPlane mode and TEE regarding the sizes of the anterior-posterior dimension (13.7 +/- 4.5 vs. 14.5 +/- 5.2 mm) and superior-inferior dimension (13.5 +/- 3.9 vs. 14.1 +/- 5.0 mm, respectively). Agreement for the aortic, atrioventricular, inferior, right upper pulmonary vein, and superior rims was 100, 100, 100, 96, and 96%, respectively. Conclusion The SMPI technique can reliably assess the dimensions and rim size of a secundum ASD for pre-interventional selection when compared with TEE and has thus the potential to replace TEE.

Aims Left ventricular (LV) dysfunction is a major determinant of late adverse clinical outcome in adult patients with tetralogy of Fallot (ToF). Therefore, early detection is important. Speckle-tracking echocardiography (STE) has emerged as a quantitative technique to assess LV function. The aim of this study was to evaluate LV rotation and twist with STE in adult ToF patients and their association with right ventricular (RV) and LV dimensions and function, exercise capacity, and NT-proBNP level. Methods Eighty-two ToF patients and 56 healthy controls matched for age and gender underwent echocardiography, electrocardiography, cardiac magnetic resonance imaging (CMR), bicycle ergometry, and NT-proBNP measurement. For STE, short-axis parasternal views were obtained at the LV base and apex. We analysed LV apical and basal rotation curves and calculated LV twist. Results Of the 82 ToF patients (55% male, age 33 ± 10 years, 98% NYHA I), 58 (71%) had normal twist, but lower than the controls [12.5 (IQR: 6.6) vs. 16.9 (IQR: 8.2) degrees, P = 0.002] mainly due to decreased apical rotation. Twenty-one (26%) patients had abnormal apical rotation which was associated with larger LV dimensions and decreased systolic biventricular function. Multivariable regression analyses showed positive relations of LV twist with biventricular systolic function measured with echocardiography as well as CMR. Conclusion The majority of adults with corrected ToF show a reduced LV twist. Strikingly, one-quarter of these patients have an abnormal apical rotation which is associated with decreased systolic LV and RV function. These findings suggest that abnormal apical rotation is a new objective diagnostic criterion for detection of ventricular dysfunction.

In addition to the haemodynamic changes in pregnancy, hormones also induce changes in the aortic wall. Women with diseases like Marfan syndrome, Ehlers-Danlo syndrome, or other aortic abnormalities, have an increased risk of complications during pregnancy. Counselling and risk assessment before pregnancy is mandatory for all women with known aortic disease. Proper information should be provided about the risks of morbidity and mortality during pregnancy and information on the risks for the fetus, including the potential recurrence of disease in the offspring. Evaluation of past medical and family history, the aortic size before conception, and any increase in size before and during pregnancy, is essential to try and estimate the risk of aortic dissection. If the aorta is dilated, prophylactic repair before pregnancy may be indicated. In some cases, elective surgery during pregnancy may be warranted. In women with a severely dilated ascending aorta, caesarean section is, at present, the advised mode of delivery. (C) 2014 Elsevier Ltd. All rights reserved.

Objective The objective of our study was to report on the total experience in thoracic aortic surgery over a 40-year time period for a single institution. Methods All 1075 patients who underwent surgery for thoracic aortic pathology from 1972 to 2011 (n = 1159) were included. Patient, procedural, and follow-up information was obtained from hospital records and the civil registry. Patients were grouped into 4 categories: acute type A dissection (n = 261), other ascending aortic/arch surgery (n = 626), descending aortic surgery (n = 175), and thoracoabdominal surgery (n = 97). Risk factors for early and late mortality and the incidence of reoperations were analyzed. Results The annual number of operations increased significantly over time. In all 4 patient groups, early mortality (in hospital or within 30 days of operation) decreased significantly over time to 15.3% in group 1, 1.9% in group 2, 0% in group 3, and 10.5% in group 4 during the contemporary time period 2007 to 2011. Overall actuarial survival was 54.3% (95% confidence interval, 50.7-57.9) after 10 years and 27.8% (95% confidence interval, 26.4-38.3) after 20 years. Late survival improved over time, but was reduced compared with the general population and was related predominantly to preexisting risk factors. In 80 patients, 111 reoperations were necessary, most frequently in group 1 patients and in patients with connective tissue disease. Conclusions Thoracic aortic operations were performed increasingly during a 40-year time period. Early mortality decreased and late survival increased significantly in all patient groups. A significant proportion of patients required multiple operations.

A 35-year-old female patient was admitted to the hospital after an accidental car crash. Medical history noted anal surgery for rectovaginal fistula at young age. She complained of dyspnea on exertion for several years. On physical examination, a diastolic murmur at the left second intercostal space was noted. No relevant abnormalities were seen in blood results, ECG, and x-ray. Echocardiography demonstrated a normal left ventricular function with severe aortic regurgitation and mild dilatation of the ascending aorta (39 mm). Transesophageal echocardiography confirmed a quadricuspid aortic valve (Figure 1A–1C). A contrast computed tomography scan revealed a double vena cava inferior system, hemiazygos, persisting vena cava superior, and aneurysmatic coronary sinus with a quadricuspid aortic valve (Figure 2A–2D). She was referred to the clinical geneticist for syndrome diagnosis. On physical examination, she showed bilateral preauricular pits and an asymmetric face. The combination of external ear abnormalities, anal surgery, and a congenital heart defect suggested the diagnosis of cat-eye syndrome. Single-nucleotide polymorphism array analysis confirmed a nonmosaic 22q11.1q11.21 quadrisomy in leukocytes. The patient underwent aortic valve replacement (Figure …

Background To investigate the overall sexual functioning and disease specific sexual problems in congenital heart disease (ConHD) patients, for both genders and different cardiac diagnostic groups, and compare these with Dutch normative data. Also disease specific sexual problems were investigated. Methods From a longitudinal cohort of patients, operated for ConHD between 1968 and 1980, 254 patients (median age: 40, 53.4% male) were included in this study: atrial septal defect (n=72), ventricular septal defect (n=71), pulmonary stenosis (n=30), tetralogy of Fallot (n=53) and transposition of the great arteries (n=28). Patients completed internationally validated, generic questionnaires and also disease specific instruments on sexual functioning. Results Patients showed a delay in starting sexual activities compared with peers. Females with ConHD scored significantly worse compared with normative data on all scales of sexual functioning, indicating a broad range of sexual problems and 15% showed clinical levels of sexual dysfunction. Of the males, 14% suffered from erectile dysfunction. Males with ConHD scored worse on erectile function, orgasmic function and satisfaction regarding their sexual life compared with normative data. No differences were found between the different cardiac diagnoses. The majority of patients reported disease specific worries and fears about the use of contraceptives, heredity, pregnancy and delivery. Patients indicated to have been suboptimally informed about sexuality in early adolescence. Conclusions This study shows that sexual functioning is impaired in adults with ConHD. Providing information to patients about sexuality, pregnancy, delivery and heredity should be improved, and given at young age.

Although its prevalence is relatively low in pregnant women, heart disease is the most important cause of maternal mortality. Problems may arise due to hemodynamic burden and the hypercoagulable state of pregnancy. Heart disease may be congenital or acquired. In developed countries, the former composes the biggest part of women with heart disease. Patients with unrepaired lesions, cyanotic lesions, diminished systemic ventricular function, complex congenital heart disease, left ventricular outflow tract obstruction, pulmonary hypertension, or mechanical valves are at highest risk of developing complications during pregnancy. All patients with known cardiac disease should preferably be counseled before conception. Pre-pregnancy evaluation should include risk assessment for the mother and fetus, including medication use and information on heredity of the cardiac lesion. Management of pregnancy and delivery should be planned accordingly on individual bases. The types of complications are related to the cardiac diagnosis, with arrhythmias and heart failure being most common. Treatment options should be discussed with the future parents, as they may affect both mother and child. In general, the preferred route of delivery is vaginal. The optimal care for pregnant women with heart disease requires multidisciplinary involvement and is best concentrated in tertiary centers.

Aim.: The study aims to describe the long-term cardiological and psychological results of our first surgical cohort of arterial switch operation (ASO) patients and compare the results with our earlier series of Mustard patients. Methods.: Twenty-four survivors of ASO operated in our center (1985-1990) were evaluated by electrocardiography, echocardiography, magnetic resonance imaging, exercise testing, 24-hour Holter-monitoring, and health-related quality of life questionnaire. The results were compared with 58 adult Mustard patients who were evaluated in 2001 using the same study protocol. Results.: Arterial switch operation was performed at a median age of 13 days and Mustard operation at 2 years. Median follow-up was 22 years (range 20-25) and 25 years (22-29), respectively. After ASO, survival was better (P =04). The event-free survival after 22 years was 77% after ASO vs. 44% after Mustard (P =03). Good systemic ventricular function was present in 93% after ASO vs. 6% after Mustard (P

Item does not contain fulltext AIMS: Discrete subaortic stenosis (DSS) is often diagnosed early in life and known for its sometimes rapid haemodynamic progression in childhood and strong association with aortic regurgitation (AR). However, data about the evolution of DSS in adulthood are scarce. Therefore, we aimed to evaluate the natural history of DSS, and identify risk factors for the progression of DSS, AR, and intervention-free survival. METHODS AND RESULTS: Conservatively managed adult DSS patients were included in this retrospective multicentre cohort study. Mixed-effects and joint models were used to assess the progression of DSS and AR, and intervention-free survival. Longitudinal natural history data were available for 149 patients [age 20 (IQR: 18-34) years, 48% male]. Sixty patients (40.3%) had associated congenital heart defects (CHDs). The median follow-up duration was 6.3 (IQR: 3.0-12.4) years. The baseline peak left ventricular outflow tract (LVOT) gradient was 32.3 +/- 17.0 mmHg and increased by 0.8 +/- 0.1 mmHg/year. While the baseline LVOT gradient (P = 0.891) or age (P = 0.421) did not influence the progression rate, the presence of associated CHD was associated with faster progression (P = 0.005). Mild AR was common (58%), but did not significantly progress over time (P = 0.701). The median intervention-free survival was 16 years and associated with the baseline LVOT gradient [hazard ratio (HR) = 3.9 (95% CI: 2.0-7.6)], DSS progression [HR = 2.6 (95% CI: 2.0-3.5)], and AR [HR = 6.4 (95% CI 2.6-15.6)]. CONCLUSION: In contrast to children, DSS progresses slowly in adulthood. In particular, patients with associated CHD are at risk for faster progression and should be monitored cautiously. Discrete subaortic stenosis progression is not influenced by the baseline LVOT gradient or age. Mild AR is common, but non-progressive over time.

Thoracic aortic aneurysms and dissections (TAAD) is a serious condition with high morbidity and mortality. It is estimated that 20% of non-syndromic TAAD cases are inherited in an autosomal-dominant pattern with variable expression and reduced penetrance. Mutations in myosin heavy chain 11 (MYH11), one of several identified TAAD genes, were shown to simultaneously cause TAAD and patent ductus arteriosus (PDA). We identified two large Dutch families with TAAD/PDA and detected two different novel heterozygote MYH11 variants in the probands. These variants, a heterozygote missense variant and a heterozygote in-frame deletion, were predicted to have damaging effects on protein structure and function. However, these novel alterations did not segregate with the TAAD/PDA in 3 out of 11 cases in family TAAD01 and in 2 out of 6 cases of family TAAD02. No mutation was detected in other known TAAD genes. Thus, it is expected that within these families other genetic factors contribute to the disease either by themselves or by interacting with the MYH11 variants. Such an oligogenic model for TAAD would explain the variable onset and progression of the disorder and its reduced penetrance in general. We conclude that in familial TAAD/PDA with an MYH11 variant in the index case caution should be exercised upon counseling family members. Specialized surveillance should still be offered to the non-carriers to prevent catastrophic aortic dissections or ruptures. Furthermore, our study underscores that segregation analysis remains very important in clinical genetics. Prediction programs and mutation evaluation algorithms need to be interpreted with caution.

Aims: The twisting motion of the heart has an important role in the function of the left ventricle. Speckle tracking echocardiography is able to quantify left ventricular (LV) rotation and twist. So far this new technique has not been used in congenital heart disease patients. The aim of our study was to investigate the feasibility and the intra- and inter-observer reproducibility of LV rotation parameters in adult patients with congenital heart disease. Methods and Results: The study population consisted of 66 consecutive patients seen in the outpatient clinic (67% male, mean age 31 7.7 years, NYHA class 1 0.3) with a variety of congenital heart disease. First, feasibility was assessed in all patients. Intraand inter-observer reproducibility was assessed for the patients in which speckle tracking echocardiography was feasible. Adequate image quality, for performing speckle echocardiography, was found in 80% of patients. The bias for the intra-observer reproducibility of the LV twist was 0.0°, with 95% limits of agreement of 2.5° and 2.5° and for interobserver reproducibility the bias was 0.0°, with 95% limits of agreement of 3.0° and 3.0°. Intra- and inter-observer measurements showed a strong correlation (0.86 and 0.79, respectively). Also a good repeatability was seen. The mean time to complete full analysis per subject for the first and second measurement was 9 and 5 minutes, respectively. Conclusion: Speckle tracking echocardiography is feasible in 80% of adult patients with congenital heart disease and shows excellent intra- and inter-observer reproducibility. (Echocardiography 2013;30:961-966)

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 VO 2 ) 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/m 2 to 138±34ml/m 2 ; QRS 132±27msec to 139±27msec); peak VO 2 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/m 2 /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 2 . 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/m 2 /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.

Background— Pregnant women with congenital heart disease (CHD) are susceptible to cardiovascular, obstetric, and offspring complications. In women with CHD, cardiac dysfunction may compromise uteroplacental flow and contribute to the increased incidence of obstetric and offspring events. Methods and Results— We performed a prospective multicenter cohort study of pregnant women with CHD and healthy pregnant women. We compared clinical, laboratory, echocardiographic, and uteroplacental Doppler flow (UDF) parameters at 20 and 32 weeks gestation, and pregnancy outcome. We related cardiovascular parameters to UDF parameters and pregnancy outcome in women with CHD. We included 209 women with CHD and 70 healthy women. Cardiovascular parameters (N-terminal pro-B-type natriuretic peptide, left and right ventricular function) differed between both groups. UDF parameters were impaired in CHD women (umbilical artery pulsatility and resistance index at 32 weeks in CHD versus healthy women, P =0.0085 and P =0.017). The following cardiovascular parameters prepregnancy and at 20 weeks gestation were associated with UDF (umbilical artery resistance index) at 32 weeks at multivariable analysis: (1) right ventricular function (tricuspid annular plane systolic excursion) ( P =0.002), (2) high N-terminal pro-B-type natriuretic peptide ( P =0.085), (3) systemic ( P =0.001), and (4) pulmonary ( P =0.045) atrioventricular valve regurgitation. Women with CHD had more obstetric (58.9% versus 32.9%, P P =0.008) than healthy women. Impaired UDF was associated with adverse obstetric and offspring outcome. Conclusions— UDF parameters are abnormal in pregnant women with CHD. Cardiovascular function is associated with an abnormal pattern of UDF. Compromised UDF may be a key factor in the high incidence of offspring and obstetric complications in this population.

Item does not contain fulltext 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

Background— Discrete subaortic stenosis is notable for its unpredictable hemodynamic progression in childhood and high reoperation rate; however, data about adulthood are scarce. Methods and Results— Adult patients who previously underwent surgery for discrete subaortic stenosis were included in this retrospective multicenter cohort study. Mixed-effects and joint models were used to assess the postoperative progression of discrete subaortic stenosis and aortic regurgitation, as well as reoperation. A total of 313 patients at 4 centers were included (age at baseline, 20.2 years [25th–75th percentile, 18.4–31.0 years]; 52% male). Median follow-up duration was 12.9 years (25th–75th percentile, 6.2–20.1 years), yielding 5617 patient-years. The peak instantaneous left ventricular outflow tract gradient decreased from 75.7±28.0 mm Hg preoperatively to 15.1±14.1 mm Hg postoperatively ( P P =0.001). Mild aortic regurgitation was present in 68% but generally did not progress over time ( P =0.76). A preoperative left ventricular outflow tract gradient ≥80 mm Hg was a predictor for progression to moderate aortic regurgitation postoperatively. Eighty patients required at least 1 reoperation (1.8% per patient-year). Predictors for reoperation included female sex (hazard ratio, 1.53; 95% confidence interval, 1.02–2.30) and left ventricular outflow tract gradient progression (hazard ratio, 1.45; 95% confidence interval, 1.31–1.62). Additional myectomy did not reduce the risk for reoperation ( P =0.92) but significantly increased the risk of a complete heart block requiring pacemaker implantation (8.1% versus 1.7%; P =0.005). Conclusions— Survival is excellent after surgery for discrete subaortic stenosis; however, reoperation for recurrent discrete subaortic stenosis is not uncommon. Over time, the left ventricular outflow tract gradient slowly increases and mild aortic regurgitation is common, although generally nonprogressive over time. Myectomy does not show additional advantages, and because it is associated with an increased risk of complete heart block, it should not be performed routinely.

To describe the very long-term outcome after surgical closure of an atrial septal defect (ASD). Longitudinal cohort study of 135 consecutive patients who underwent surgical ASD repair at age

ObjectivesThe aim of this study was to determine the value of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in adults with congenital heart disease (CHD) and investigate its relationship with ventricular function and exercise capacity.BackgroundNT-proBNP may detect early deterioration in cardiac function.MethodsIn this cross-sectional study, extensive echocardiography, exercise testing, and NT-proBNP measurements were performed on the same day in consecutive adult patients with CHD.ResultsIn total, 475 patients were included in this study (mean age of 34 ± 12 years, 57% male, 90% New York Heart Association class I). The median NT-proBNP level was 15.1 pmol/l (interquartile range [IQR]: 7.1 to 31.3 pmol/l), and the NT-proBNP level was >14 pmol/l in 53% of patients. The highest NT-proBNP levels were observed in patients with Fontan circulation (36.1 pmol/l [IQR: 14.4 to 103.8 pmol/l]) and a systemic right ventricle (RV) (31.1 pmol/l [IQR: 21.8 to 56.0 pmol/l]), and the lowest values were seen in patients with aortic coarctation (7.3 pmol/l [IQR: 2.8 to 19.5 pmol/l]). NT-proBNP levels correlated with age (r = 0.39, p < 0.001) and were higher in women (median of 21.7 vs. 10.4 pmol/l; p < 0.001). In patients with aortic stenosis or aortic coarctation, NT-proBNP levels correlated with diastolic function parameters of E/E′ ratio (r = 0.40, p < 0.001) and left atrial dimension (r = 0.36, p < 0.001). In patients with a systemic RV, NT-proBNP levels correlated with RV annulus diameter (r = 0.31, p = 0.024). In patients with tetralogy of Fallot, the strongest correlations were observed with left atrial dimension (r = 0.46, p < 0.001) and left ventricular ejection fraction (r = 0.37, p

BackgroundAtrial 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.Methods51 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.ResultsIn 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, p24%) 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).ConclusionsIn 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.

Aims The purpose of this study is to describe disease presentation, co-morbidities, diagnosis and initial therapeutic management of patients with peripartum cardiomyopathy (PPCM) living in countries belonging to the European Society of Cardiology (ESC) vs. non-ESC countries. Methods and results Out of 500 patients with PPCM entered by 31 March 2016, we report on data of the first 411 patients with completed case record forms (from 43 countries) entered into this ongoing registry. There were marked differences in socio-demographic parameters such as Human Development Index, GINI index on inequality, and Health Expenditure in PPCM patients from ESC vs. non-ESC countries (P < 0.001 each). Ethnicity was Caucasian (34%), Black African (25.8%), Asian (21.8%), and Middle Eastern backgrounds (16.4%). Despite the huge disparities in socio-demographic factors and ethnic backgrounds, baseline characteristics are remarkably similar. Drug therapy initiated post-partum included ACE inhibitors/ARBs and mineralocorticoid receptor antagonists with identical frequencies in ESC vs. non-ESC countries. However, in non-ESC countries, there was significantly less use of beta-blockers (70.3% vs. 91.9%) and ivabradine (1.4% vs. 17.1%), but more use of diuretics (91.3% vs. 68.8%), digoxin (37.0% vs. 18.0%), and bromocriptine (32.6% vs. 7.1%) (P < 0.001). More patients in non-ESC vs. ESC countries continued to have symptomatic heart failure after 1 month (92.3% vs. 81.3%, P < 0.001). Venous thrombo-embolic events, arterial embolizations, and cerebrovascular accidents were documented in 28 of 411 patients (6.8%). Neonatal death rate was 3.1%. Conclusion PPCM occurs in women from different ethnic backgrounds globally. Despite marked differences in socio-economic background, mode of presentation was largely similar. Embolic events and persistent heart failure were common within 1 month post-diagnosis and required intensive, multidisciplinary management.

Women with genetic aortic disease, such as Marfan syndrome, are at increased risk of pregnancy complications, in particular those with aortic dissection [(1)][1]. Aneurysms-osteoarthritis syndrome (AOS) is an autosomal-dominant syndrome caused by SMAD3 mutations, and is characterized by arterial