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Background: In acquired heart disease, brain natriuretic peptide (BNP) and N-Teminal pro-brain natriuretic peptide (NT-proBNP) are increasingly used as diagnostic and prognostic markers. In adult Congenital heart disease, the abnormal anatomy and physiology complicate assessment of cardiac function. We Studied the clinical correlates of measurement of natriuretic peptides (NP) in adults with a right ventricle in the systemic position or with Fontan-type physiology. Methods: A prospective longitudinal study (follow up time 23 +/- 13 months, mean +/- S.D.) was conducted in a specialised centre on 61 patients (age 26 +/- 8 years; NYHA class 1.5 +/- 0.6) including Senning/Mustard corrected transposition, congenitally corrected transposition and Fontan/ total cavopulmonary connection. Plasma NP concentration was compared with NYHA class, exercise capacity and echocardiographically determined systemic systolic ventricular function. Results: Neurohormone concentrations were generally elevated (mean=290% of upper reference limit) and related to NYHA class (P < 0.001, NYHA I vs. II-IV). No clinically significant relationship to ventricular function or exercise capacity was found however. An NP measurement could not predict the future Course of the disease in terms of functional status or ventricular function. Conclusion: In contrast to patients with acquired heart disease, measurement of NP seems to have low clinical value in adults with a right ventricle in the systemic position or with Fontan-type physiology.

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. (c) 2005 Elsevier Ireland Ltd. All rights reserved.

Aims To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period. Methods and results Data collected as part of the Euro Heart Survey on adult CHD was analysed. This entailed information transcribed from the files of 4110 patients diagnosed with one of eight congenital heart conditions (‘defects'), who consecutively visited the outpatient clinics of one of the participating centres in 1998. The patients were included retrospectively and followed until the end of 2003 for a median follow-up of 5.1 years. Notwithstanding their overall relatively good functional class and low mortality over the follow-up period, a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. There were major differences between the eight defects, both in morbidity and regarding specific characteristics. Outcomes were worst in cyanotic defects and in the Fontan circulation, but a considerable proportion of the other patients also suffer from cardiac symptoms. In particular, arrhythmias are common. Conclusion The spectrum of adult CHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period

Aims To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period. Methods and results Data collected as part of the Euro Heart Survey on adult CHD was analysed. This entailed information transcribed from the files of 4110 patients diagnosed with one of eight congenital heart conditions (‘defects'), who consecutively visited the outpatient clinics of one of the participating centres in 1998. The patients were included retrospectively and followed until the end of 2003 for a median follow-up of 5.1 years. Notwithstanding their overall relatively good functional class and low mortality over the follow-up period, a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. There were major differences between the eight defects, both in morbidity and regarding specific characteristics. Outcomes were worst in cyanotic defects and in the Fontan circulation, but a considerable proportion of the other patients also suffer from cardiac symptoms. In particular, arrhythmias are common. Conclusion The spectrum of adult CHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period

Aims To investigate the role of guidelines in structuring the clinical care for adult patients with congenital heart disease (CHD), and to assess adherence to the guidelines in Europe. Methods and results A selected number of current guidelines were chosen pertaining to operative procedures, investigations, and the use of medication (‘interventions'). The source for this analysis was the database of the Euro Heart Survey on adult CHD, which contains retrospectively collected data on 4110 patients followed-up for a median of 5.1 years. For each guideline investigated, patients were selected from the database for whom the particular guideline was relevant. The selected cases were classified according to two criteria: was there an indication for the particular intervention and did the intervention take place? In this manner, cases of ‘undue treatment' and ‘insufficient treatment' were identified. Adherence to guidelines was found to be good in the case of operative procedures and prophylactic drug treatment. However, regarding diagnostic procedures there had been adherence to guidelines in only slightly more than half of the cases. Conclusion Guidelines have an important role in the actual clinical care of adults with CHD. However, large outcome studies are needed to develop more precise guidelines