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

BACKGROUND: 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. METHODS AND RESULTS: A cross-sectional descriptive study examining 98 stable adult patients with CCHD with a medical questionnaire, blood samples, MRI of the cerebrum (n=72), multidetector CT imaging (MDCT) of the thorax (n=76) and pulmonary scintigraphy (ventilation/perfusion/single-photon emission computerised tomography/CT) (n=66). The prevalence of cerebral infarction and pulmonary thrombosis according to imaging were 47% and 31%, respectively. Comparing the findings with previous medical history revealed a large under-reporting of thrombosis with only 22% of the patients having a clinical history of stroke and 25% of pulmonary thrombosis. There was no association between the degree of erythrocytosis or haemostatic abnormalities and the prevalence of thrombosis. CONCLUSIONS: Patients with CCHD have a prevalence of both cerebral and pulmonary thrombosis of around 30%-40%, which is much higher than that reported previously. Furthermore, there is a large discrepancy between clinical history and imaging findings, suggesting a high prevalence of silent thrombotic events. Neither erythrocytosis nor haemostatic abnormalities were associated with the prevalence of thrombosis in patients with CCHD. TRIAL REGISTRATION NUMBER: http://www.cvk.sum.dk/CVK/Home/English.aspx (H-KF-2006-4068).

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.

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.

Atrial myocardial pathoelectrophysiology in adults with a secundum atrial septal defect is unaffected by closure of the defect. A study using high resolution signal-averaged orthogonal P-wave technique.

BACKGROUND: In patients with atrial septal defect (ASD) the P-wave is prolonged as a marker of delayed atrial conduction which is associated with atrial fibrillation. The study aim was to analyse the impact of ASD closure in adults on P-wave duration and morphology by means of signal-averaged P-waves (PSA-ECG) and to investigate potential mechano-electrical interactions. METHODS: PSA-ECG was obtained before and 8+/-6 months after ASD closure in 35 adult patients (age 53+/-15 years). Heart chamber sizes and pulmonary artery pressure levels were assessed by echoDopplercardiography. RESULTS: P-wave duration and morphology did not change after ASD closure (148+/-16 vs 144+/-16 ms, P=0.07). P-wave duration did not relate to age at repair, preclosure atrial sizes or pulmonary artery pressure. Pre- or postclosure atrial fibrillation propensity was associated with longer P-wave duration both before and after ASD closure. CONCLUSION: Atrial conduction disturbances in middle-aged patients with ASD, manifested as a prolonged P-wave duration, do not change after ASD closure and are not related to the dilatation of the right and left atria. It is suggestive that atrial conduction disturbance associated with ASD develop early and early intervention is required to prevent the development of late atrial fibrillation.

Closure of atrial septal defect in the adult. Cardiac remodeling is an early event.

BACKGROUND: Study aimed to describe the extent and the temporal profile of cardiac remodeling after atrial septal defect closure in the adult. METHODS: Prospective and longitudinal echocardiographic assessment of right and left heart size before and after (1 day-1 week/1/4/12 months) surgical or catheter-based atrial septal closure in 39 adults (age 54+/-15 years). RESULTS: Right ventricular and atrial sizes were markedly reduced, left ventricular size increased and left atrial size remained unchanged after closure. Older age and a history of atrial fibrillation reduced the potential to normalize right and left atrial size after closure. The greater part of the changes occurred very early, in the 1st day/1st week. From then on the speed of change gradually diminished and after 4 months no important changes were observed. The mode of closure did not influence the degree or the pace of the remodeling. CONCLUSION: Cardiac remodeling after atrial septal closure in the adult is a common and early event that seems by and large completed within the first half year after closure. The ventricles seem to have a higher capacity of remodeling than the atria in this setting. The mode of closure does not seem to significantly impact remodeling.

[Adults with congenital heart defects--a growing patient group]. / Vuxna med medfött hjärtfel--en allt större patientgrupp.

The number of grown ups with congenital heart disease (GUCH) increases due to the success story of pediatric cardiology and heart surgery. However, late complications such as arrhythmia, endocarditis and heart failure are common, as are patients requiring reoperation/catheter intervention. In some categories of congenital heart disease, pregnancy may pose a substantial threat. Early information about this is essential. The care of these patients, including heart surgery, should be centralized. A registry of GUCH-patients has been created in Sweden, promoting experience and knowledge concerning a "new" and expanding group of patients in cardiology.

Transcatheter closure of post-infarction ventricular septal defect with the Amplatzer Septal Occluder device.

There is an 80-90% mortality rate within the first 2 months of the occurrence of a post-infarction ventricular septal defect (VSD) with medical treatment alone. The muscular VSD presents a technical problem for the surgeon. Surgical treatment was unsuccessful in two patients. They were treated successfully using the Amplatzer Septal Occluder, with improvement in their condition.

Mitroflow pericardial bioprosthesis in the aortic position. Low incidence of structural valve deterioration in elderly patients during an 11-year follow-up.

Aortic valve replacement with Mitroflow pericardial bioprosthesis, with or without concomitant bypass surgery was performed on 403 consecutive patients between March 1984 and December 1994. The mean age was 74 (range 10-92) years, with male/female ratio 183/220. Early mortality was 3.5% (14/403) and late mortality 25% (99/389). Actuarial survival was 35.4%+/-9.4% at 10 years and freedom from valve-related mortality 86.2%+/-9.6% at 8 years (total follow-up 1 270 years). The thromboembolic rate was 6.5% (fatal 0.7%)/patient year. The incidence of prosthetic valve endocarditis was 0.6% (fatal 0.3%)/patient year. Structural valve deterioration was found in 0.8%/patient year (no fatality). Actuarial freedom from all reoperations at 8 years was 92.4+/-8%. The Mitroflow pericardial heart valve is proposed as a good choice for aortic valve replacement in elderly patients, in view of the low rates of reoperation, anticoagulant treatment and valve-related thromboembolism.

Atrial septal defect in adults. Thirty-eight-year follow-up of a surgically and a conservatively managed group.

This report describes the long-term (up to 38 years) outcome, in terms of mortality and cardiovascular morbidity, in a non-randomized clinical observation study of a surgically and a conservatively managed group of adult patients with shunt lesions at atrial level. Haemodynamic status was assessed at baseline and at repeat examination. Twelve patients underwent surgical repair of the lesion between these two heart catheterizations, resulting in a marked reduction in heart size and right ventricular systolic pressure. These changes were associated with improvement in functional class and a trend towards less risk of cerebrovascular incidents, but not atrial fibrillation, during follow-up. Among 12 patients not operated on between catheterizations, symptomatic deterioration was common, often necessitating later surgical repair. The results support the assumption that early surgery should be recommended for adults with a haemodynamically significant lesion, to reduce the risk of mortality and prevent symptomatic deterioration.

Left-to-right cardiac shunts: comparison of measurements obtained with MR velocity mapping and with radionuclide angiography.

PURPOSE: To investigate the agreement between two noninvasive methods, magnetic resonance (MR) velocity mapping and first-pass radionuclide angiography, to quantify the pulmonary-to-systemic blood flow ratio (QP/QS) in adults, adolescents, and children with left-to-right cardiac shunts. MATERIALS AND METHODS: The accuracy and precision of MR velocity mapping were studied in 12 control subjects (six men, six women) and in a phantom. MR velocity mapping and radionuclide angiography were performed on the same day in 24 patients (16 adults, two adolescents, six children; five male patients, 19 female patients). RESULTS: The mean error in QP/QS at MR velocity mapping in phantom experiments was -1% +/- 1 (mean +/- SD). In control subjects, QP/QS at MR velocity mapping was 1.03 +/- 0.03, and the cardiac index was 3.1 L/min/m2 +/- 0.2 and 3.2 L/min/m2 +/- 0.3 for women and men, respectively. In patients, QP/QS at radionuclide angiography was 14% +/- 13, higher than at MR velocity mapping. Interobserver variability was four times higher for radionuclide angiography compared with MR velocity mapping, 0% + 16 versus 0% +/- 4 (n = 12). The difference between repeated MR flow measurements in the same vessel was -1% +/- 5 (n = 36). CONCLUSION: The data suggest that MR velocity mapping is accurate and precise for measurements of shunt size over the whole range of possible QP/QS values.

Functional evaluation of extracardiac ventriculopulmonary conduits and of the right ventricle with magnetic resonance imaging and velocity mapping.

Extracardiac ventriculopulmonary conduits tend to deteriorate over time, developing both obstruction and regurgitation. In this prospective study, magnetic resonance imaging (MRI) was compared with Doppler echocardiography to determine whether MRI improves the noninvasive evaluation of conduit patients. Twenty-five patients (median age 10 years, range 2.5 to 32) were investigated 27 times with Doppler echocardiography and an MRI protocol with spin echo sequences for morphology, velocity mapping, and multislice gradient echo technique for right ventricular volume measuring. Cardiac catheterization data were available in 6 patients. Echocardiography could assess the morphology of the conduits in 6 patients, whereas MRI demonstrated all conduits efficiently. Doppler echocardiography could evaluate the occurrence of regurgitation in 18 patients and could quantify peak velocity in 20 of the patients. A technically adequate MRI velocity mapping was obtained in 25 patients. There was good agreement between MRI and Doppler echocardiography in establishing or not establishing regurgitation, but Doppler echocardiography was less reliable in evaluating the degree of regurgitation. The correlation between peak velocity determined with Doppler and magnetic resonance imaging was r = 0.63 [corrected]. Correlations between catheterization pressure gradients and noninvasive techniques were r = 0.97 for magnetic resonance imaging [corrected] versus catheterization, and r = 0.86 [corrected] for Doppler versus catheterization. MRI can provide complete information on the morphology and function of extracardiac ventriculopulmonary conduits, as well as of the right ventricle. If the results of MRI and echocardiography with Doppler are in agreement, heart catheterization and angiography can be avoided, even in patients considered for conduit replacement.

Does the risk of infective endarteritis justify routine patent ductus arteriosus closure?

OBJECTIVES: The risk of infective endarteritis in patients with patent ductus arteriosus seems to have declined during the last 30-40 years, and cases of patent ductus arteriosus complicated by infective endarteritis are now very rare. Moreover, with the introduction of antibiotics, there has been a dramatic reduction in mortality from infective endocarditis. Despite these changes, however, the prevention of infective endarteritis has remained the principal indication, apart from haemodynamic reasons, for surgical patent ductus arteriosus closure. The aim of this study was to ascertain whether prevention of infective endarteritis is still a justifiable indication for routine closure in all cases of patient ductus arterious. METHODS: The records of 270 paediatric and adult cases of patent ductus arteriosus was reviewed with respect to infective endarteritis. All Swedish death certificates issued during the period 1960-93 were checked for the occurrence of patent ductus arteriosus in combination with infective endarteritis. RESULTS: There had been no cases of infective endarteritis over an aggregate of 1196 years at risk. Of nearly three million deaths in Sweden during the period 1960-93 two cases were due to infective endarteritis as a complication of patent ductus arteriosus. CONCLUSION: The present findings suggest routine closure of a patent ductus arteriosus, for the sole purpose of eliminating the risk of infective endarteritis, is unnecessary.

Pregnancy and heart disease: a review.

About one per cent of all pregnancies are affected by maternal heart disease, which may have significant influence on the maternal and foetal outcome. In this context rheumatic heart disease is declining and congenital heart disease is becoming the dominant category. Our experience of pregnancy in the new groups of patients created by paediatric heart surgery is scant and a continuous update is required.

Antihypertensive effect of felodipine combined with beta-blockade. A comparison between 2 and 3 daily dosages.

24 hypertensive patients, who were not satisfactorily controlled (diastolic blood pressure greater than 95 mm Hg) with beta-blockers alone were randomised to 2 treatment groups where felodipine was administered for 2 weeks in a total daily dose of 15 mg divided in 2 or 3 doses. Following a 2-week placebo washout period, the patients were switched to the alternative dose regimen in a double-blind crossover manner. Blood pressure was measured with standard techniques and was also non-invasively monitored for 24 hours at the end of each dose regimen period and at the end of the intermediate placebo period. Mean arterial blood pressure at the end of the placebo run-in period was 169/105 mm Hg. Felodipine 5 mg thrice daily reduced blood pressure by 20/9 mm Hg and felodipine 7.5 mg twice daily by 17/9 mm Hg (p less than 0.05). The difference between the 2 dose regimens was not statistically significant. When 24-hour blood pressure measurements for the 2 dose regimens were compared, there were no statistically significant differences. Both regimens reduced the 24-hour blood pressure significantly compared with placebo. Two patients were withdrawn during the study, 1 before felodipine treatment started and the other due to diarrhoea and flushing related to felodipine. Otherwise felodipine was generally well tolerated.