Atrial fibrillation (AF) is a common co-morbidity in patients with hypertrophic cardiomyopathy (HCM) and it can contribute to poor outcomes. Therefore, identification of safe and effective strategies for controlling AF in HCM are of substantial interest to researchers, clinicians and patients.
In this issue of Heart, Providencia and colleagues (see
Atrial fibrillation (AF) is a well-recognised comorbidity in patients with hypertrophic cardiomyopathy (HCM), occurring in about 25% of patients.
Rhythm control strategies are indicated with symptomatic AF, but the current antiarrhythmic choices are suboptimal. Patients with HCM have a propensity for both atrial and ventricular arrhythmias, and there is little data on the safety of antiarrhythmic therapies in this population. Disopyramide is perhaps the best studied agent and may concurrently help to mitigate outflow obstruction through its negative inotropic...
Down syndrome (DS) is one of the most common chromosomal abnormalities. The incidence of DS at birth in developed countries has remained relatively stable over the last 20 years, at approximately 1 per 1000 live births. DS is associated with a number of congenital defects and is also at increased risk of conditions that may develop later in life, and thus requires multidisciplinary care and close surveillance. Congenital heart disease (CHD) is present in 35%–50% of patients and is haemodynamically significant in two-thirds.
The CHD most commonly encountered in DS is atrioventricular septal defects, ventricular septal defects and atrial septal defects.
There is a growing expectation that information about the performance of clinical services and individual doctors is made publicly available. Patients have a legitimate interest in knowing the care they receive is of high quality, but there are some potential risks in public reporting of these data, not least that of risk-averse behaviour by clinicians concerned about loss of reputation and livelihood that potentially denies patients appropriate treatment. The development of reliable metrics to assess clinical performance is complex, evolving and often controversial. In the USA, the Society of Thoracic Surgeons and the American College of Cardiology (ACC) have published principles for public reporting of outcomes central to which is the use of high-quality, robust and validated clinical data.
Residual right ventricular outflow tract and pulmonary valve disease is common in adults with repaired tetralogy of Fallot. Chronic severe pulmonary regurgitation as a result of surgical repair can lead to myriad complications including right ventricular dysfunction, decreased exercise tolerance, right heart failure and symptomatic arrhythmias. The aim of restoring pulmonary valve integrity is to preserve right ventricular size and function with the intent of mitigating the development of symptoms and poor long-term outcomes. Right ventricular size thresholds by cardiac MRI have emerged beyond which reverse right ventricular remodelling after pulmonary valve replacement is less likely. Though pulmonary valve replacement has been shown to improve right ventricular dimensions and symptoms, no consistent improvement in right ventricular ejection fraction or objective measures of exercise capacity have been demonstrated. Furthermore, there are no long-term studies showing that normalisation of right ventricular size results in improved clinical outcomes. New transcatheter techniques of percutaneous pulmonary valve replacement have emerged with good short-term and mid-term outcomes, further adding to the complexity in determining ‘when’ and ‘how’ right ventricular outflow tract and pulmonary valve intervention should occur. With improved survival of these patients, the trend towards earlier pulmonary valve replacement at smaller right ventricular size and rapidly evolving transcatheter pulmonary valve techniques, the clinician must balance the goals of preserving right ventricular size and function in an attempt to prevent untoward outcomes with risks of multiple interventions in a patient's lifetime.
Acute rheumatic fever (ARF) and the related rheumatic heart disease (RHD) are autoimmune diseases thought to be triggered by group A streptococcal (GAS) pharyngitis. RHD is a leading cause of mortality in the developing world. The strong epidemiological association between GAS throat infection and ARF is highly suggestive of causation, but does not exclude other infections as contributory. There is good evidence of both humoral and cellular autoreactivity and GAS/self cross-reactivity in established RHD. RHD pathogenesis could feasibly be triggered and driven by humoral and/or cellular molecular cross-reactivity between GAS and host cardiac tissues (molecular mimicry). However, good evidence of humoral pathogenicity is lacking and the specific triggering event for RHD remains unknown. It is likely that the critical immunological events leading to ARF/RHD occur at the point of contact between GAS and the immune system in the throat, strongly implicating the mucosal immune system in RHD pathogenesis. Additionally, there is circumstantial evidence that continued live GAS may play a role in ARF/RHD pathogenesis. We suggest that future avenues for study should include the exclusion of GAS components directly contributing to RHD pathogenesis; large genome-wide association studies of patients with RHD looking for candidate genes involved in RHD pathogenesis; genome-wide association studies of GAS from patients with ARF taken at diagnosis to look for characteristics of rheumatogenic strains; and performing case/control studies of GAS pharyngitis/ARF/patients with RHD, and controls to identify microbiological, immunological and environmental differences to elucidate RHD pathogenesis.
Atrial fibrillation (AF) is common in hypertrophic cardiomyopathy (HCM) and is associated with a high risk of stroke. The efficacy and safety of catheter ablation in this setting is poorly characterised. We aimed to systematically review the existing literature and to perform a meta-analysis to determine the efficacy and safety of catheter ablation of AF in patients with HCM.
Random-effects meta-analysis of studies comparing HCM versus non-HCM controls. The outcomes of freedom from AF/atrial tachycardia, and acute procedure-related complications were assessed. Studies were searched on MEDLINE, EMBASE, COCHRANE and clinicaltrials.gov.
Fourteen studies were considered eligible for the systematic review, of which five were included in the meta-analysis. Freedom from AF/atrial tachycardia relapse was higher in patients without HCM (after a single procedure: 38.7% HCM vs 49.8% controls, OR=2.25, 95% CI 1.09 to 4.64, p=0.03; after ≥1 procedure: 51.8% HCM vs 71.2% controls, OR=2.62, 95% CI 1.52 to 4.51, p=0.0006; I2=33% and 26%, respectively). Risk of procedure-related adverse events was low. Repeat procedures (mean difference=0.16, 95% CI 0.0 to 0.32, p=0.05, I2=53%) and antiarrhythmic drugs (OR=4.70, 95% CI 2.31 to 9.55, p<0.0001, I2=0%) are more frequently needed in patients with HCM to prevent arrhythmia relapse. Sensitivity analyses suggested that the outcome in patients with HCM with less dilated atria and paroxysmal AF may be more comparable to the general population.
The observed complication rate of catheter ablation of AF in patients with HCM was low. Even though the risk of relapse is twofold higher, catheter ablation can be effective in patients with HCM and AF, particularly in patients with paroxysmal AF and smaller atria.
Based on previous experiences, the Food and Drug Administration and the European Medicines Agency recommend that clinical trials for novel antidiabetic drugs are powered to detect increased cardiovascular risk. In this context, data concerning licensed drugs such as metformin and sulfonylureas are conflicting. The influence of baseline cardiovascular risk on any treatment effect appears obvious but has not been formally proven. We therefore evaluated association of metformin and sulfonylureas with cardiovascular events in patients with different cardiovascular risk profiles indicated by N-terminal of the prohormone brain natriuretic peptide (NT-proBNP) levels.
2024 patients with diabetes mellitus were included in this observational study. The primary endpoint was defined as a combination of cardiovascular events and death. Association of metformin and sulfonylureas was assessed using Cox regression models. Possible differences of these associations in patients with different NT-proBNP levels were studied by stratifying and through interaction analysis.
During a median follow-up of 60 months, the primary endpoint occurred in 522 (26%) of patients. The median age was 63 years. A Cox regression analysis was adjusted for site of treatment, concomitant medication, age, gender, body mass index, glycated haemoglobin, duration of diabetes, glomerular filtration rate, cholesterol, and history of smoking and cardiac disease. Metformin was associated with a decreased risk in the cohort with elevated NT-proBNP ≥300 pg/mL (HR 0.70, p=0.014) and a similar association was found for the interaction between metformin and NT-proBNP (p=0.001). There was neither an association for sulfonylureas nor a significant interaction between sulfonylureas and NT-proBNP.
Metformin is associated with beneficial cardiovascular outcomes in patients with diabetes only when (sub)clinical cardiovascular risk defined by NT-proBNP levels is present.
A young man attended the adult congenital heart disease clinic for routine follow-up. He had a diagnosis of transposition of the great arteries (TGA) and underwent a Senning procedure at 10 months of age. He had remained well over the intervening years, was fully saturated, with a normal exercise tolerance, no cardiovascular symptoms and no history of arrhythmia or symptoms suggesting thromboembolic events. Transthoracic echocardiogram is shown in
Which of the following is the most likely diagnosis? Intact atrial pathways Secundum atrial septal defect Baffle leak Primum atrial septal defect Baffle obstruction
Intact atrial pathways
Secundum atrial septal defect
Primum atrial septal defect
To characterise patients with trisomy 21 (Down syndrome, DS) based on the data of the German National Register for Congenital Heart Defects, to identify changes in the availability of surgical therapy over time and to analyse the impact of these changes on developing Eisenmenger syndrome (ES) as well as survival.
Out of 1549 patients with DS with congenital heart disease in the National Register for Congenital Heart Defects, 894 patients (55% female, mean age 17.5 years) had a post-tricuspid shunt lesion (atrioventricular septal defect 69.5%, ventricular septal defect 27.7%, patent arterial duct 2.6%) and were included in the current study.
The likelihood of being treated interventionally or surgically before the age of 1 year increased significantly over time. In parallel, the likelihood of developing ES decreased over time (53% birth cohort during 1950s/1960s vs 0.5% birth cohort during 2000–2009, p<0.0001). Overall survival after 1, 10, 20 and 40 years was 96.8%, 94.1%, 92.6% and 75.5%, respectively. Patients with ES had a significantly worse survival compared with those without ES (HR 18.1; 95% CI 7.2 to 45.4; p<0.0001).
The availability of surgical correction was associated with a decrease in the likelihood of developing ES. Patients with DS still have reduced survival prospects compared with the general population, but this effect is largely driven by patients developing ES who still have a very poor prognosis.
Hypothermia can induce ECG J waves. Recent studies suggest that J waves may be associated with ventricular fibrillation (VF) in patients with structurally normal hearts. However, little is known about the ECG features, clinical significance or arrhythmogenic potentials of therapeutic hypothermia (TH)-induced J waves.
We analysed ECGs from 240 patients who underwent TH at six major university hospitals in Korea between August 2010 and December 2013. The prevalence, amplitudes and distributions of the J waves and the development of malignant arrhythmia were analysed.
The average patient body temperature was 33.5±1.0°C during TH. J waves were observed in 98 patients (40.8%). They were newly developed in 91 cases, and pre-existing J waves were augmented in seven patients. J waves during TH were primarily observed in leads II, III, aVF and V4–6. The average amplitude of the J waves was 0.239±0.152 mV. There were four VF events during TH. These events occurred in three patients who were finally diagnosed with Brugada syndrome, idiopathic VF or early repolarisation syndrome, respectively, and in one patient with non-cardiac aetiology (asphyxia).
J waves were recorded in about 40% of the patients who received TH. They were most frequently observed in the inferior limb leads or lateral precordial leads. Life-threatening VF occurred only rarely (1.7%) during TH and were mainly observed in patients with primary arrhythmic disorder. Although a causal relationship between TH-induced J waves and VF remains unknown, administering TH to this potentially susceptible, high-risk population may require careful attention.
The electrocardiogram (ECG) is the most commonly used tool to screen for left ventricular hypertrophy (LVH), and yet current diagnostic criteria are insensitive in modern increasingly overweight society. We propose a simple adjustment to improve diagnostic accuracy in different body weights and improve the sensitivity of this universally available technique.
Overall, 1295 participants were included—821 with a wide range of body mass index (BMI 17.1–53.3 kg/m2) initially underwent cardiac magnetic resonance evaluation of anatomical left ventricular (LV) axis, LV mass and 12-lead surface ECG in order to generate an adjustment factor applied to the Sokolow–Lyon criteria. This factor was then validated in a second cohort (n=520, BMI 15.9–63.2 kg/m2).
When matched for LV mass, the combination of leftward anatomical axis deviation and increased BMI resulted in a reduction of the Sokolow–Lyon index, by 4 mm in overweight and 8 mm in obesity. After adjusting for this in the initial cohort, the sensitivity of the Sokolow–Lyon index increased (overweight: 12.8% to 30.8%, obese: 3.1% to 27.2%) approaching that seen in normal weight (37.8%). Similar results were achieved in the validation cohort (specificity increased in overweight: 8.3% to 39.1%, obese: 9.4% to 25.0%) again approaching normal weight (39.0%). Importantly, specificity remained excellent (>93.1%).
Adjusting the Sokolow–Lyon index for BMI (overweight +4 mm, obesity +8 mm) improves the diagnostic accuracy for detecting LVH. As the ECG, worldwide, remains the most widely used screening tool for LVH, implementing these findings should translate into significant clinical benefit.
Histological analyses of debris captured by a cerebral protection system (CPS) during transcatheter valve-in-valve (VIV) procedures have not been reported.
Fifteen consecutive patients with stenotic aortic (n=13) or mitral (n=2) surgical or transcatheter bioprostheses were treated with implantation of a transcatheter heart valve (THV) in the presence of a dual-filter CPS. Mean patient age was 75 years; mean logistic EuroSCORE was 31%. Filters were collected and histological assessment of debris was performed. Patients were followed clinically until discharge.
Debris captured by either or both filters was detected in all patients. Acute thrombus was the most common type of debris, found in all patients, followed in frequency by arterial wall tissue (n=12 patients (80%)), calcification (n=11 (73%)) and valve tissue (n=9 (60%)). Less frequently found were organised thrombus (n=5 (30%)), foreign material (n=4 (27%)) and myocardium (n=2 (13%)). A median of 123 debris particles per patient was detected, with a trend towards a greater median number of particles collected in proximal filters (78 vs 39, p=0.065). The average maximum particle diameter was 88 (range 56–175) µm, with a median of 20 particles ≥150 µm. No stroke or transient ischaemic attack (TIA) had occurred by the time of discharge (mean 8 days).
Transcatheter VIV procedures were associated with the release of particulate debris into the cerebral circulation in all patients. The type of debris suggests that debris originates predominantly from arterial and valvular passage of the THV.
To analyse time to cardiovascular events and mortality in patients with type 2 diabetes (T2D) who received treatment intensification with insulin or a glucagon-like peptide-1 (GLP-1ar) analogue following dual therapy failure with metformin (MET) and sulphonylurea (SU).
A retrospective cohort study was conducted in 2003 patients who were newly treated with a GLP-1ar or insulin following dual therapy (MET+SU) failure between 2006 and 2014. Data were sourced from The Health Improvement Network database. Risks of major adverse cardiovascular events (MACE) (non-fatal myocardial infarction, non-fatal stroke and all-cause mortality) were compared between MET+SU+insulin (N=1584) versus MET+SU+GLP-1ar (N=419). Follow-up was for 5 years (6614 person-years). Propensity score matching analysis and Cox proportional hazard models were employed.
Mean age was 52.8±14.1 years. Overall, the number of MACE was 231 vs 11 for patients who added insulin versus GLP-1ar, respectively (44.5 vs 7.7 per 1000-person-years adjusted HR (aHR): 0.27; 95% CI 0.14 to 0.53; p<0.0001). Insulin was associated with significant increase in weight compared with GLP-1ar (1.78 vs –3.93 kg; p<0.0001) but haemoglobin A1c reduction was similar between both treatment groups (–1.29 vs –0.98; p=0.156). In a subgroup analysis of obese patients (body mass index >30 kg/m2) there were 84 vs 11 composite outcomes (38.6 vs 8.1 per 1000 person-years; aHR: 0.31; 95% CI 0.16 to 0.61; p=0.001) in the insulin and GLP-1ar groups, respectively.
In this cohort of obese people with T2DM, intensification of dual oral therapy by adding GLP-1ar analogue is associated with a lower MACE outcome in routine clinical practice, compared with adding insulin therapy as the third glucose-lowering agent.
Implementation of statin therapy in practice for primary prevention of cardiovascular disease is controversial due to concerns over costs and side-effects with broader use and uncertainty regarding LDL goals in the primary prevention population. Previous primary prevention trials suggest a reduction in cardiovascular outcomes in largely white patients with significant risk factors for coronary disease. The HOPE-3 trial randomized a diverse population of 12,000 individuals over 55 years of age (women over 60) with 1–2 relatively modest risk factors for cardiovascular disease (annual risk ~1%) but otherwise no indication for statin therapy to 10 mg of rosuvastatin daily vs placebo. The composite primary outcome was death from cardiovascular causes, non- fatal MI or stroke. Nearly 13% of patients were excluded following roll-in based on side-effects or lab abnormalities. For remaining patients, over a median follow of 5.6 years there was a...
Learning objectives To understand basic cardiovascular magnetic resonance (CMR) physics and to appreciate CMR safety issues, especially in relation to implanted medical devices and contrast agents. To describe the common indications for performing a clinical CMR study. To become familiar with the diagnostic ability of CMR and its influence on patient management.
To understand basic cardiovascular magnetic resonance (CMR) physics and to appreciate CMR safety issues, especially in relation to implanted medical devices and contrast agents.
To describe the common indications for performing a clinical CMR study.
To become familiar with the diagnostic ability of CMR and its influence on patient management.
Recent technical developments in cardiovascular imaging have led to a number of options for the non-invasive investigation of cardiovascular disease. Often, the choice of imaging modality comes down to local availability and expertise, as in many clinical scenarios there is not one clearly superior test. This is reflected in current international guidelines and recommendations. The relative advantages and disadvantages of each modality are already well published,