Influence of socioeconomic factors on pregnancy outcome in women with structural heart disease

OBJECTIVE: Cardiac disease is the leading cause of indirect maternal mortality. The aim of this study was to analyse to what extent socioeconomic factors influence the outcome of pregnancy in women with heart disease.  METHODS: The Registry of Pregnancy and Cardiac disease is a global prospective registry. For this analysis, countries that enrolled ≥10 patients were included. A combined cardiac endpoint included maternal cardiac death, arrhythmia requiring treatment, heart failure, thromboembolic event, aortic dissection, endocarditis, acute coronary syndrome, hospitalisation for cardiac reason or intervention. Associations between patient characteristics, country characteristics (income inequality expressed as Gini coefficient, health expenditure, schooling, gross domestic product, birth rate and hospital beds) and cardiac endpoints were checked in a three-level model (patient-centre-country).  RESULTS: A total of 30 countries enrolled 2924 patients from 89 centres. At least one endpoint occurred in 645 women (22.1%). Maternal age, New York Heart Association classification and modified WHO risk classification were associated with the combined endpoint and explained 37% of variance in outcome. Gini coefficient and country-specific birth rate explained an additional 4%. There were large differences between the individual countries, but the need for multilevel modelling to account for these differences disappeared after adjustment for patient characteristics, Gini and country-specific birth rate.  CONCLUSION: While there are definite interregional differences in pregnancy outcome in women with cardiac disease, these differences seem to be mainly driven by individual patient characteristics. Adjustment for country characteristics refined the results to a limited extent, but maternal condition seems to be the main determinant of outcome

The haematocrit – an important factor causing impaired haemostasis in patients with cyanotic congenital heart disease

Background: Patients with cyanotic congenital heart disease(CCHD) have haemostatic abnormalities, which result in an increased risk of bleeding. The cause is unknown, but recent studies have indicated that an elevated haematocrit, which is present in cyanotic patients, could be an important factor. The aim of this study was to characterize the haemostatic profile, examine how changes in haematocrit affect the haemostatic profile, and whether a haematocrit reduction could terminate bleeding in CCHD patients. Methods: This was a prospective, multicenter study. The haemostatic profile consisting of haematocrit, platelet count and thrombelastography(TEG) was characterized in ninety-eight CCHD patients. To evaluate the influence of haematocrit on the haemostatic profile, 21 of the patients underwent phlebotomy and 16 patients received treatment with an iron supplement. Furthermore ten patients with haemoptysis underwent phlebotomy. The haemostatic profile was reevaluated after interventions. Results: TEG revealed that patients with CCHD and elevated haematocrit were hypocoagulable due to reduced clot formation and strength. Furthermore a positive correlation between elevated haematocrit and hypocoagulability was present. Interventions such as phlebotomy and treatment with supplemental iron causing significant haematocrit changes confirmed the correlation between haematocrit and the haemostatic profile. Finally a haematocrit reduction by phlebotomy successfully terminated haemoptysis in ten CCHD patients. Conclusion: Patients with CCHD and elevated haematocrit are hypocoagulable. The hypocoagulable haemostatic profile is positively correlated to increasing haematocrit. An intervention, which increases or decreases haematocrit, changes the haemostatic profile. A haematocrit reduction seems to improve the haemostatic profile, and may thereby terminate bleeding. However, these results warrant further studies. (C) 2012 Elsevier Ireland Ltd. All rights reserved

Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease

Background: Patients with cyanotic congenital heart disease (CCHD) have haemostatic abnormities associated with bleeding and thrombo-embolic events. The haemostatic abnormalities are not fully understood, but recent studies indicate that elevated haematocrit and fibrinogen function may be of importance. The aim of this study was to characterise the haemostatic profile and examine the potential role of haematocrit on clot formation and strength in CCHD patients. Furthermore to examine whether CCHD patients with history of haemoptysis have diminished fibrinogen function compared to those without haemoptysis. Methods: In a prospective study 75 adult CCHD patients had haematocrit, platelet count, and plasma fibrinogen concentration examined. Furthermore thrombelastography(TEG) as well as TEG Functional Fibrinogen(TEG FF) assay evaluating fibrinogen function(FLEV) was performed. Data were compared with historical data regarding previous haemoptysis in CCHD patients. Results: Haematocrit was 57 +/- 8% and platelet counts in the lower normal range. TEG revealed a hypocoagulable condition with impaired clot formation. TEG values were correlated to haematocrit, indicating that elevated haematocrit causes impaired clot formation and strength. Despite high levels of plasma fibrinogen, TEG FF demonstrated that FLEV was diminished and negatively correlated to haematocrit. Furthermore CCHD patients with previous history of haemoptysis had significantly lower FLEV compared to CCHD patients without haemoptysis. Conclusion: Patients with CCHD are hypocoagulable mainly due to impaired fibrinogen function. Despite a low platelet count, platelet function does not seem to be severely affected in CCHD patients. Haemostasis, and especially fibrinogen function, is negatively affected by elevated haematocrit, and fibrinogen function is diminished in CCHD patients with haemoptysis. (C) 2012 Elsevier Ireland Ltd. All rights reserved

Prevalence of cerebral and pulmonary thrombosis in patients with cyanotic congenital heart disease

Patients with cyanotic congenital heart disease (CCHD) have a high prevalence of thrombosis, the most frequently described locations being the cerebral and pulmonary vessels. The reported prevalence of both cerebral infarction and pulmonary thrombosis has been highly variable. The aim of this study was to examine the prevalence of both cerebral and pulmonary thrombosis in CCHD according to medical history and imaging. In addition, the role of known erythrocytosis and haemostatic abnormalities as risk factors was evaluated

Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicentre study

BACKGROUND: -Eisenmenger syndrome (ES) is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of ES patients. METHODS: -We identified in a multicentre approach adults with ES under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic and laboratory parameters. RESULTS: -We studied 1098 patients (median age 34.4years, range 16.1-84.4years, 65.1% female, 31.9% with Down syndrome). The majority had a post-tricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pre-tricuspid lesion (n=, 12.7%). Over a median follow-up of 3.1years [IQR 1.4-5.9], allowing for 4361.6 patient-years observation, 278 patients died and six and six underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis only age (HR 1.41/10years, 95%CI 1.24-1.59, P<0.001), pre-tricuspid shunt (HR 1.56, 95%CI 1.02-2.39, P=0.041), oxygen saturation at rest (HR 0.53/10%, 95%CI 0.43-0.65, P<0.001), presence of sinus rhythm (HR 0.53, 95%CI 0.32-0.88, P=0.013) and presence of pericardial effusion (HR 2.41, 95%CI 1.59-3.66, P<0.001) remained significant predictors of death. CONCLUSIONS: -There is significant premature mortality amongst contemporary adults with ES. We report, herewith a multivariable mortality risk stratification model based on five simple, non-invasive predictors of death in this population

Transcatheter device closure of atrial septal defects in patients aged 40 years and older

Objective To evaluate the safety and effect of transcatheter device closure in ostium secundum atrial septal defects (ASD II) in patients aged 40 years and older Methods Retrospective single-centre study concerning 47 consecutive ASD transcatheter occlusion procedures performed between January 1999 and December 2008 Electrocardiography, echocardiography and clinical assessments of the patients were conducted pre- and post-intervention and at follow-up Results Of the 130 patients who were referred for interventional ASD closure, 47 were 40 years and older and all of them actually had the device inserted There were no major complications during the intervention Mean follow-up time was 15 15 months During follow-up, three patients needed surgical reintervention because of device embohsation (n=2) or dislocation (n=1) Of the patients with severe right ventricular (RV) dilatation, more than half (58%) had no or mild dilatation at last follow-up Reduction of RV dilatation was not related to age Pulmonary hypertension was present in 63% before the procedure and was reduced to 38% at follow-up NYHA class improved in all age groups, also in patients over 60 years of age In two of the three patients who died during follow-up, no cause of death could be established, but both had responded well to treatment regarding the echocardiographic and clinical findings Conclusion Transcatheter device closure of ASD is a successful and effective treatment, also for patients aged 40 years and older Patients showed regression of right ventricular enlargement and an improvement in functional class (Neth Heart J 2010,18 537-42

Immunoreactive trypsin(Ogen) in the sera of children with recent-onset insulin-dependent diabetes and matched controls

To evaluate the exocrine pancreatic function at the time of diagnosis of insulin-dependent diabetes mellitus, we determined immunoreactive an-odal and cathodal trypsin(ogen) levels in sera from almost all children (n = 375) 0-14 years of age in Sweden in whom diabetes developed during 1 year, and in sex-, age-, and geographically matched control subjects (n = 312). The median level of anodal trypsin(ogen) was 5 (quartile range, 3-7) µg/L in children with newly diagnosed diabetes, compared with a median level of 7 (quartile range, 4-8) µg/L in control subjects (p < 0.0001). Similarly, the median level of cathodal trypsin(ogen) was 8 (quartile range, 4-10) µg/L in children with diabetes, compared with a median level of 11 (quartile range, 7-15) µg/L in control subjects (p < 0.0001). The median of the individual ratios between cathodal and anodal trypsin(ogen) was 1.4 in the diabetic patients and 1.7 in the control children (p < 0.001). In a multivariate test, however, only the decrease in cathodal trypsin(ogen) concentration was associated with diabetes. The levels of trypsin(ogen)s did not correlate with levels of islet cell antibodies, present in 81% of the diabetic children. Several mechanisms may explain our findings, for example, similar pathogenetic factors may affect both the endocrine and exocrine pancreas simultaneously, a failing local trophic stimulation by insulin on the exocrine cells may decrease the trypsinogen production, and there may be an increased elimination of trypsin(ogen) because of higher filtration through the kidneys in the hyperglycemic state