Denby, Kara J; Clark, Daniel E; Markham, Larry W
doi: 10.1136/heartjnl-2017-311774pmid: 28751537
Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at <5 years of age, many adult cardiologists are unfamiliar with the pathophysiology of this disease. This vasculitis has a predilection for coronary arteries with a high complication rate across the lifespan for those with medium to large coronary artery aneurysms. An inflammatory cascade produces endothelial dysfunction and damage to the vascular wall, leading to aneurysmal dilatation. Later, pseudonormalisation of the vascular lumen occurs through vascular remodelling and layering thrombus, but this does not necessarily indicate resolution of disease or reduction of risk for future complications. There is a growing prevalence of Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood.
D’Amario, Domenico; Amodeo, Antonio; Adorisio, Rachele; Tiziano, Francesco Danilo; Leone, Antonio Maria; Perri, Gianluigi; Bruno, Piergiorgio; Massetti, Massimo; Ferlini, Alessandra; Pane, Marika; Niccoli, Giampaolo; Porto, Italo; D’Angelo, Gianluca A; Borovac, Josip Anđelo;
Carlson, Selma D; Steinberg, Zachary L; Krieger, Eric V
doi: 10.1136/heartjnl-2017-312174pmid: 28954831
Clinical introductionA 28-year-old woman with a history of critical pulmonic stenosis, status postsurgical valvotomy and subsequent pulmonary valve replacement, presented to the cardiology clinic with 1 year of progressive exertional dyspnoea. She has a heart rate of 75 bpm and blood pressure of 110/55 mm Hg. Cardiac auscultation reveals a 1/6 systolic ejection murmur along the left sternum and an early 3/6 diastolic decrescendo murmur. A transthoracic echocardiogram is obtained (figure 1).QuestionsWhich of the following would be most likely found during right heart catheterisation?Ratio of pulmonary to systemic blood flow (Qp:Qs) >1.5Pulmonary vascular resistance >3 Wood unitsRight atrial pressure >10mm HgPulmonary artery systolic pressure >45mm Hg E. Pulmonary artery diastolic pressure <10mm Hg
Crump, Casey; Sundquist, Jan; Winkleby, Marilyn A; Sundquist, Kristina
doi: 10.1136/heartjnl-2016-310716pmid: 28500243
ObjectiveLow physical fitness and obesity have been associated with higher risk of developing heart failure (HF), but their interactive effects are unknown. Elucidation of interactions among these common modifiable factors may help facilitate more effective primary prevention.MethodsWe conducted a national cohort study to examine the interactive effects of aerobic fitness, muscular strength and body mass index (BMI) among 1 330 610 military conscripts in Sweden during 1969–1997 (97%–98% of all 18-year-old men) on risk of HF identified from inpatient and outpatient diagnoses through 2012 (maximum age 62 years).ResultsThere were 11 711 men diagnosed with HF in 37.8 million person-years of follow-up. Low aerobic fitness, low muscular strength and obesity were independently associated with higher risk of HF, after adjusting for each other, socioeconomic factors, other chronic diseases and family history of HF. The combination of low aerobic fitness and low muscular strength (lowest vs highest tertiles) was associated with a 1.7-fold risk of HF (95% CI 1.6 to 1.9; p<0.001; incidence rates per 100 000 person-years, 43.2 vs 10.8). These factors had positive additive and multiplicative interactions (p<0.001) and were associated with increased risk of HF even among men with normal BMI.ConclusionsLow aerobic fitness, low muscular strength and obesity at the age of 18 years were independently associated with higher risk of HF in adulthood, with interactive effects between aerobic fitness and muscular strength. These findings suggest that early-life interventions may help reduce the long-term risk of HF and should include both aerobic fitness and muscular strength, even among persons with normal BMI.
Wang, Min; Long, Weiqing; Li, Di; Wang, Duan; Zhong, Yuan; Mu, Di; Song, Jiayi; Xia, Min
doi: 10.1136/heartjnl-2016-310914pmid: 28490621
Objective7-Ketocholesterol (7-KC), a major oxidation product of cholesterol, is found in human atherosclerotic plaque and more atherogenic than cholesterol in animal models. This study was designed to investigate the association of plasma 7-KC level with the incident cardiovascular disease (CVD) events in general population.MethodsWe measured plasma 7-KC concentrations at baseline in 1944 participants free from CVD in a community-based cohort study. The primary endpoint was incident of a major adverse cardiovascular event. A Cox proportional hazards model was used to calculate the HRs with 95% CI.ResultsA total of 101 incident CVD events were recorded during the 5.2 year median follow-up. The baseline plasma 7-KC levels were associated with a higher risk of incident CVD events; compared with quartile 1, participants in quartile 4 had an unadjusted HR of 2.38 (2.03–2.85, p<0.001) and an adjusted HR of 1.70 (1.45–1.91, p=0.004) after adjusting for traditional risk factors. Plasma 7-KC levels improved all of the metrics of discrimination and reclassification when added to the intima–media thickness (C-statistic: p=0.002; net reclassification improvement (NRI): p<0.001; integrated discrimination improvement (IDI): p<0.001), family history of myocardial infarction (C-statistic: p=0.011; NRI: p=0.004; IDI: p=0.003) and elevated high-sensitivity C reactive protein (C-statistic: p=0.008; NRI: p=0.015; IDI: p=0.009).ConclusionsElevated plasma 7-KC levels are associated with the incident CVD events in a population-based cohort. Further studies are needed to confirm this observation.
Showing 1 to 10 of 16 Articles
doi: 10.1136/heartjnl-2017-311269pmid: 28668906
Duchenne muscular dystrophy (DMD) is a genetic, progressive neuromuscular condition that is marked by the long-term muscle deterioration with significant implications of pulmonary and cardiac dysfunction. As such, end-stage heart failure (HF) in DMD is increasingly becoming the main cause of death in this population. The early detection of cardiomyopathy is often challenging, due to a long subclinical phase of ventricular dysfunction and difficulties in assessment of cardiovascular symptomatology in these patients who usually loose ambulation during the early adolescence. However, an early diagnosis of cardiovascular disease in patients with DMD is decisive since it allows a timely initiation of cardioprotective therapies that can mitigate HF symptoms and delay detrimental heart muscle remodelling. Echocardiography and ECG are standardly used for screening and detection of cardiovascular abnormalities in these patients, although these tools are not always adequate to detect an early, clinically asymptomatic phases of disease progression. In this regard, cardiovascular magnetic resonance (CMR) with late gadolinium enhancement is emerging as a promising method for the detection of early cardiac involvement in patients with DMD. The early detection of cardiac dysfunction allows the therapeutic institution of various classes of drugs such as corticosteroids, beta-blockers, ACE inhibitors, antimineralocorticoid diuretics and novel pharmacological and surgical solutions in the multimodal and multidisciplinary care for this group of patients. This review will focus on these challenges and available options for HF in patients with DMD.