Adults with congenital heart disease are a growing population. One of the major challenges in the care of these patients is to prevent thromboembolic episodes. Despite relative young age and no typical cardiovascular risk factors, this cohort has a high prevalence of thrombotic events. It is difficult to use treatment algorithms from the general adult population with acquired heart disease in this heterogeneous population due to special conditions such as myocardial scarring after previous surgery, atypical atrial flutter, prothrombotic conditions and the presence of interatrial shunts. Furthermore, there is a lack of scientific evidence regarding how to prevent thromboembolic events with anticoagulation in adults with congenital heart disease. The aim of this paper is to review the current literature pertaining to anticoagulation in adults with congenital heart disease and hence enable recommendations for which patients are likely to benefit from which anticoagulation treatments, when they should be considered and how these would be carried out.
Background: Ruptured aortic aneurysm and aortic dissections are potentially preventable disorders associated with high mortality. Screening of individuals at risk may translate into elective surgical interventions and lowered mortality. It is uncertain if the risk of aortic dilation of varying degrees aggregates within families. Methods: We investigated the risk of having thoracic and abdominal aortic sizes in the highest quartile (measured by computed tomography scans and indexed for body size) if at least one parent did so in the Framingham Heart Study (FHS) cohorts, and estimated the incidence rates and hazards ratio of developing aortic aneurysm or dissection among first-degree relatives of those with aortic aneurysm or dissection, as compared with age- and sex-matched controls (1:10 for aortic aneurysm and 1:100 for aortic dissection) using the Danish nationwide administrative registries. Results: In FHS, offspring (n=235) whose parent(s) had a sex- and age-standardized aortic size in the upper quartile had a multivariable-adjusted ~3-fold increased odds ratio of belonging to the upper quartile themselves. In Denmark, a total of 68,939 individuals (mean age 42 years) had a first-degree relative with aortic aneurysm and 7,209 persons (mean age 39 years) had a firstdegree relative with aortic dissection. During an average follow-up of 7 years, first-degree relatives of patients with aortic aneurysm and dissection had a hazards ratio of 6.70 (95% CI 5.96-7.52) for developing aortic aneurysm and 9.24 (95% CI 5.53-15.44) for dissection, compared to matched controls. These estimates remained unchanged upon adjusting for several comorbidities, including prevalent hypertension, bicuspid aortic valve, and the Marfan syndrome. For both aortic aneurysm and dissections, the absolute event rates approached 1 per 1000 person-years for first-degree relatives versus 11-13 (aortic aneurysm) and 2-3 (aortic dissections) per 100,000 person-years among controls. Conclusions: Increased aortic size, a precursor of aortic aneurysm and a risk factor for dissection, clusters in families. The incidence rates of aortic aneurysm and dissections approach that of other common cardiovascular conditions in first-degree relatives, supporting the use of systematic screening for these conditions.
The contribution of diastolic dysfunction in patients with preserved left ventricular (LV) systolic function to impaired functional status and cardiac mortality in myocardial infarction (MI) is unknown. In the present study, assessment of LV diastolic function was performed by Doppler analysis of the mitral and pulmonary venous flow, and the propagation velocity of early mitral flow by color M-mode Doppler echocardiography in 183 consecutive patients at day 5–7 following their first acute MI. Patients were classified into four groups: group A: preserved LV systolic and diastolic function (n = 73); group B: LV systolic dysfunction with preserved diastolic function (n = 10); group C: LV diastolic dysfunction with preserved systolic function (n = 60); group D: combined LV systolic and diastolic dysfunction (n = 40). The cardiac mortality rate at 1 year was significantly higher in groups C (13%) and D (38%) compared to A (2%) (p < 0.01). Multivariate regression analysis identified LV diastolic dysfunction (p = 0.001), Killip class ≧II (p = 0.006), and age (0.008) as predictors of cardiac death or readmission due to heart failure. The presence of LV diastolic dysfunction with preserved systolic dysfunction is associated with increased morbidity and mortality following acute MI.
Objectives: To test whether an increase in Doppler myocardial performance index (MPI) during dobutamine stress echocardiography, reflecting deterioration of overall left ventricular function, is associated with increased N-terminal pro-brain natriuretic peptide (NT-pro-BNP) concentration and provides prognostic information beyond conventional systolic wall motion analysis after acute myocardial infarction (AMI). Design: Prospective, observational study. Methods: Dobutamine-atropine stress echocardiography (DASE) and NT-pro-BNP were assessed five days after AMI in 109 consecutive patients. MPI was measured at rest and at low-dose (10 mg/kg/min) and peak dobutamine infusion (( 40 mg/kg/min with or without atropine). Main outcome measures: End point was a composite of cardiac death or readmission for heart failure or reinfarction. Results: In 35 patients (32%), MPI increased at low-dose DASE. This was associated with higher NT-pro-BNP concentrations (b = 0.30, p = 0.004). During a mean follow up of 27 (SD 7) months, 8 patients died of cardiac causes and 15 patients were readmitted for heart failure or reinfarction. On Cox regression analysis, an increase in MPI at low-dose DASE (p = 0.02) was an independent predictor of cardiac events. In contrast, traditional wall motion analysis during DASE provided no additional prognostic information. Conclusions: An increase in MPI at low-dose DASE, reflecting early deterioration of overall left ventricular function, is associated with raised NT-pro-BNP concentration and provides prognostic information beyond conventional stress echocardiographic data after AMI. I nducible myocardial ischaemia may be detected and quantified by dobutamine-atropine stress echocardiography (DASE) after acute myocardial infarction (AMI). The detection of ischaemia is based on semiquantitative wall motion analysis, however, which assesses only left ventricular (LV) systolic function. The Doppler echocardiographic myocardial performance index (MPI) is a quantitative measure of combined LV systolic and diastolic function. [1][2][3] Because impaired LV diastolic function precedes LV systolic dysfunction during myocardial ischaemia, 4 quantitative assessment of changes in both systolic and diastolic LV function seems appealing and may potentially increase the clinical yield of DASE after AMI. Data on the changes in MPI during stress echocardiography are limited. Recently, MPI was found to correlate closely with invasive measures of LV systolic function during b adrenergic stimulation. 5 Furthermore, we have previously shown that MPI consistently improves, decreasing its value, during dobutamine stimulation in healthy people, 6 and MPI has been shown to increase (deteriorate) during dobutamine-induced ischaemia in patients with known or suspected coronary artery disease.
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