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Aortic dissection is one of the most common causes of death in pregnancy. Most cases are caused by hereditary thoracic aortopathy (HTA), and women are often unaware they are at risk. Awareness, early recognition and involvement of senior clinicians from the expert cardio-obstetric team are essential to facilitate early diagnosis and carefully planned and coordinated antenatal and postnatal care. Frequent imaging antenatally and good blood pressure control are key. Delivery needs particular attention with optimal pain control and techniques to minimise maternal effort. Dissection is most common post partum and a period of close surveillance with meticulous blood pressure control after delivery is crucial, as well as follow-up of the baby in paediatric services. All women with a family history of aortic dissection or sudden death should be investigated. Women with known HTA should be offered specialist individualised pre-pregnancy counselling, including advice about genetics and inheritance, before starting a family. Future research is directed towards more individualised risk assessment, pre-pregnancy advice and pharmacological options to reduce dissection risk.

Transposition of the great arteries (TGA) after atrial switch operation and congenitally corrected TGA (ccTGA) are commonly associated with impaired systemic right ventricular (RV) function and impaired prognosis. We aimed to investigate the value of indices of myocardial deformation on speckle-tracking echocardiography for quantifying ventricular function and their potential role in assessing ventricular-ventricular interaction and outcome in patients with a systemic RV. A total of 129 patients (87 with TGA and atrial switch and 42 with ccTGA, 71 men, age 35 ± 12 years) were investigated, and biventricular myocardial deformation was compared with findings in healthy subjects (n = 38, age 36 ± 10 years). Systemic ventricular longitudinal 2-dimensional (2D) peak systolic strain (RV 2D-LS) was significantly reduced compared with controls (−12.9 ± 3.6 and −15.4 ± 5.1 vs −21.0 ± 5.5 in TGAs, ccTGAs, and controls, P < .0001). Systemic and pulmonary 2D-LS were correlated in patients with TGA (r = 0.46, P < .0001) and ccTGA (r = 0.64, P < .0001), suggesting interventricular interaction, and this was confirmed when ejection fraction on magnetic resonance imaging was assessed (r = 0.53, P < .0001). More importantly, systemic 2D-LS (hazard ratio 1.31, P = .01) was related to adverse clinical outcome (symptomatic progression to New York Heart Association class ≥3, clinically relevant cardiac arrhythmia, or death) in patients with TGA and atrial switch independently of ejection fraction on cardiac magnetic resonance imaging, history of clinically relevant arrhythmia, or functional class. Global longitudinal systolic strain is significantly reduced in patients with a systemic RV, is related to subpulmonary ventricular function, and predicts adverse clinical outcome in adults with atrial switch TGA.

ObjectiveTo assess the relationship between elevated levels of B-type natriuretic peptide (BNP) and outcome in patients with Eisenmenger syndrome.DesignRetrospective study.SettingTertiary centre for adult congenital heart disease.PatientsAll patients with Eisenmenger syndrome (n=181, age 36.9±12.1 years, 31% with Down syndrome) in whom BNP concentrations were measured as part of routine clinical care were included.Main outcome measuresThe study end point was all cause mortality.ResultsDuring a median follow-up period of 3.3 years, 20 patients (7 with Down syndrome) died. Higher BNP concentrations were predictive of all cause mortality on univariate analysis in patients with or without Down syndrome. On multivariable Cox proportional hazard analysis, BNP predicted survival independently of renal function, Down syndrome, or 6 min walk test distance (p=0.004). Temporal increases in BNP concentration were also found to predict mortality. Treatment with disease targeting therapies was associated with a significant reduction in BNP concentrations.ConclusionsBNP concentrations predict outcome in contemporary Eisenmenger patients. Increases in BNP concentrations over time are also of prognostic significance. In addition, disease targeting therapies may help to reduce BNP concentrations in this population, while treatment-naïve patients have static or rising BNP concentrations.

More women with heart disease are reaching reproductive age and will want to embark upon pregnancy. Furthermore, many of these women are delaying pregnancy until later in life when they may be exposed to a greater number of complications from their heart disease. A relatively high proportion of these women will pursue fertility treatment to achieve a pregnancy; consequently, the management of subfertile couples where the woman (or man) has heart disease is of growing importance. In this review, we discuss how fertility investigations and treatment can impact a women with heart disease and how some of the potential complications can be minimized or avoided. We also consider surrogacy, which is an important option when pregnancy is contraindicated.

BackgroundSystemic right ventricle (sRV) patients are at an increased risk of developing heart failure. Sodium-glucose cotransporter 2 inhibitors (SGLT2i) could be a valuable treatment option. This study investigated the changes in ventricular function in sRV failure patients in the first year after starting SGLT2i.MethodsAdult sRV patients from the international, real-world ACHIEVE-SGLT2i registry were included if they had a clinical diagnosis of sRV failure, a transthoracic echocardiogram before starting SGLT2i, and at least one in the first year after starting available for analysis. The primary outcomes were changes in sRV global longitudinal strain (GLS) and fractional area change (FAC). Longitudinal changes were evaluated using linear mixed models.ResultsThirty-nine sRV failure patients (46±9.3 years old, 41% female) were included. Twenty-five (64%) had transposition of the great arteries after an atrial switch procedure and 14 (36%) had congenitally corrected transposition. sRV GLS improved significantly in the first 50 days (−1.4%-point per month, p<0.001) and stabilised afterwards (<0.1%-point per month, p=0.520). Though age had a significant overall negative effect on sRV GLS (0.1%-point per year of age, p=0.049), it did not influence the longitudinal changes after starting SGLT2i. sRV FAC also improved in the first 50 days (3.2%-point per month, p=0.002), after which sRV FAC deteriorated in patients with subpulmonary left ventricular pacing (−0.9%-point per month, p=0.012) while it stabilised in patients without pacing (0.1%-point per month, p=0.573). In the first 50 days, tricuspid annular plane systolic excursion also improved significantly in all patients (1.2 mm per month, p=0.006), and stabilised afterwards (p=0.721).ConclusionsSGLT2i therapy is associated with improvements in systolic ventricular function in sRV failure patients. Despite early improvement in sRV FAC, there was a negative longer term correlation with subpulmonary left ventricular pacing, potentially reflecting adverse effects of subpulmonary ventricular pacing on sRV function.

ObjectiveInfective endocarditis (IE) is associated with significant morbidity and mortality. Patients with adult congenital heart disease (ACHD) have an increased risk of developing IE. The aim of this study is to describe the incidence, predictors of outcome and mortality associated with IE in ACHD in a contemporary cohort.MethodsAll episodes of IE in adults with congenital heart disease referred to our tertiary centre between 1999 and 2013 were included in the study. Patients were identified from the hospital database. The diagnosis of endocarditis was established according to the modified Duke criteria. The primary endpoint of the study was endocarditis-associated mortality.ResultsThere were 164 episodes of IE in 144 patients (male 102, 70.8%). Mean age at presentation was 32.3±22.7 years. Out of these, 43% had a simple, 23% a moderate and 32% a complex lesion. It was at least the second bout of IE in 37 episodes (23%). A predisposing event could be identified in only 26.2% of episodes. Surgical intervention during the same admission was performed in 61 episodes (37.2%). During a median follow-up of 6.7 years (IQR 2.9–11.4), 28 (19.4%) patients died. Out of these, 10 deaths were related to IE (IE mortality 6.9%). On unvariate regression analysis, the development of an abscess (OR: 7.23; 95% CI 1.81 to 28.94, p<0.01) and age (OR: 1.05; 95% CI 1.01 to 1.10, p=0.03) were the only predictors of IE-associated mortality. There was no increase in IE cases at our centre during the period of the study.ConclusionsIE-associated morbidity and mortality in a contemporary cohort of ACHD patients is still high in the current era.

Abstract Aims The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality. Methods and results This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P < 0.0001). Although a large number of ‘asymptomatic’ (NYHA class 1) patients did not achieve a ‘normal’ peak VO2, the NYHA class was a strong predictor of mortality, with an 8.7-fold increased mortality risk in class 3 compared with class 1 (hazard ratio 8.68, 95% confidence interval: 5.26–14.35, P < 0.0001). Conclusion Despite underestimating the degree of limitation in some ACHD patients, NYHA classification remains a valuable clinical tool. It correlates with objective measures of exercise and the severity of underlying cardiac disease, as well as mid- to long-term mortality and should, thus, be into incorporated the routine assessment and risk stratification of these patients.

ObjectivesApproximately 5%–10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain.MethodsAll adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis.ResultsA total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, p<0.0001).ConclusionsArrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.

BackgroundIncreased longevity in patients with congenital heart disease (CHD) is associated with late complications, mainly heart failure, which may not be amenable to redo surgery and become refractory to medical therapy and so, trigger referral for transplantation. We assessed the current role and future prospects of heart and heart-lung transplantation for patients with CHD in England.MethodsWe performed a retrospective analysis of hospital episodes for England for 1997–2015, identifying patients with a CHD code (ICD-10 ‘Q2xx.x’), who underwent heart or heart-lung transplantation.ResultsIn total, 469 transplants (82.2% heart and 17.8% heart-lung) were performed in 444 patients. Half of patients transplanted had mild or moderate CHD complexity, this percentage increased with time (p=0.001). While overall, more transplantations were performed over the years, the proportion of heart-lung transplants declined (p<0.0001), whereas the proportion of transplants performed in adults remained static. Mortality was high during the first year, especially after heart-lung transplantation, but remained relatively low thereafter. Older age and heart-lung transplantation were strong predictors of death. While an increase in CHD transplants is anticipated, actual numbers in England seem to lag behind the increase in CHD patients with advanced heart failure.ConclusionsThe current and future predicted increase in the numbers of CHD transplants does not appear to parallel the expansion of the CHD population, especially in adults. Further investment and changes in policy should be made to enhance the number of donors and increase CHD transplant capacity to address the increasing numbers of potential CHD recipients and optimise transplantation outcomes in this growing population.