Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.
Background: In contrast to the adult age group, epidemiologic studies on heart failure (HF) in the pediatric population are lacking. The aim of this prospective study was to analyze the epidemiology of HF during infancy and childhood. Methods: Of the 1,196 children with congenital and acquired heart diseases, primarily diagnosed during a 10-y period in one hospital, we identified those patients who developed HF. Results: Within 10 y, 1,196 children with heart disease were indexed. Heart failure occurred in 124 of these patients (10.4%): 64 out of 1,031 children with congenital heart diseases (6.2%), 13 out of 96 children with rhythm or conduction disturbances (13.5%), 23 out of 39 children with acquired heart diseases (59.0%), and 24 out of 30 children with cardiomyopathies (80.0%). Heart failure occurred in 72 cases (58.1%) during the first year of life. The incidence in infancy was much more pronounced for congenital heart diseases than for other cardiac conditions. The mortality associated with HF and its cause was also lower for children with congenital heart disease (4.7%) than for the other cardiac conditions (8.7%, 23.0%, and 25.0%, for acquired heart diseases, rhythm disturbances, and cardiomyopathies, respectively). Conclusion: Clear differences exist between HF in infants and HF in children. Our study supports the observation that congenital heart disease is the most common causative factor of HF during infancy. Older children with HF are more likely to have acquired heart diseases, cardiomyopathies, and arrhythmias, and these conditions have a considerable mortality.Key words: epidemiology, heart failure, congenital heart disease, arrhythmia, child IntroductionEpidemiological data from the Framingham Study provide insights into the population burden of heart failure (HF), its prognosis, and modifiable risk factors that promote it. In the general population, HF is chiefly the end stage of hypertensive, coronary, and valvular cardiovascular disease. 1 -3 Hypertension has the greatest impact, 4 accounting for 39% of HF events in men and 59% in women, followed by myocardial infarction (34% and 13% of HF events in men and women, respectively), and valvular heart disease (7%-8% of HF events).In contrast to the adult age group, HF in the pediatric population has received little attention. The aim of this prospective study was to analyze the epidemiology of HF during infancy and childhood.
Objective: The aim of this prospective study was to evaluate the proportion of children with delayed recognition of congenital heart disease (CHD). Methods: Of the 744 children with CHD primarily diagnosed during a 10 year period in one hospital, the patients were identified where the diagnosis of CHD was established with a significant delay. Results: Sixty six patients (8.9%) had delayed diagnosis of CHD. Among patients with cyanotic CHD, 10.4% (7 of 67 cases) were referred after they had initially been discharged home from the birth clinic. Among patients with acyanotic CHD, 8.7% (59 of 677) of all children and 35.1% (59 of 168) of the children who required surgery or interventional catheterisation were referred at an age where elective repair should have already been performed or needed immediate treatment because of their haemodynamic status. Of the 66 patients with delayed diagnosis, one infant with cor triatriatum died at admission because of delayed referral and 10 children had severe complications: preoperative cardiogenic shock in seven cases of aortic coarctation and one case of endocardial fibroelastosis, pulmonary hypertensive crisis in one child after delayed repair of a ventricular septal defect, and infectious endocarditis after dental care in a teenager with undiagnosed moderate aortic stenosis, who required Ross operation a few months later. Discussion and conclusion: A substantial proportion of CHD was detected with relevant delay. In all cases of late diagnosis, clinical cardiac findings were present that should have alerted the physician on the possible presence of underlying CHD.
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