Time-related arterial changes after allogeneic hematopoietic stem cell transplantation in children.

BACKGROUND: Metabolic syndrome is a frequent late effect in young adults after hematopoietic stem cell transplantation (HSCT) performed in childhood. METHODS: To further study the signs of cardiovascular changes in HSCT patients, we performed noninvasive vascular ultrasonic measurements of arterial stiffness and endothelial function in 25 children (median age: 11.2 y) and in 22 healthy matched controls. RESULTS: The distensibility of the common carotid artery (CCA) was significantly lower in the patients than in the controls (mean = 0.48, SD = 0.19 vs. mean = 0.64 mm Hg(-1) × 10(-2), SD = 0.28; P = 0.024). The distensibility decreased with time passed after HSCT (P = 0.009). The compliance of the CCA was decreased (mean = 0.10, SD = 0.04 vs. mean = 0.13 mm(2) × mm Hg(-1), SD = 0.05; P = 0.041), and the incremental elastic modulus (E inc) was higher in the patients than in the controls (mean = 2.05, SD = 0.7 vs. mean = 1.6 mm × 10(3), SD = 0.6; P = 0.019). E inc was associated with time passed after HSCT (P = 0.036). The size of the brachial artery and flow-mediated dilation did not differ between the groups. CONCLUSION: Early mechanical changes of the arterial wall were found at young age after HSCT. Ultrasonography may offer a noninvasive method to find early alterations of the vascular bed and to optimize prevention of atherosclerosis in HSCT patients.

Morbidity after paediatric cardiac surgery assessed with usage of medicines: a population-based registry study.

OBJECTIVE: To examine the overall morbidity of patients who underwent surgery for congenital cardiac defect during childhood. BACKGROUND: A congenital cardiac defect treated with surgery is seldom totally cured. The incidence of residua, sequelae, and comorbidity is quite high. The morbidity has not been thoroughly examined. METHODS AND PATIENTS: Medication was used as an indicator of morbidity. Data from the Finnish Research Registry of Paediatric Cardiac Surgery were linked to data from the medication registry of Finland's Social Insurance Institution. This study includes 5116 patients with a mean age of 33.5 (ranged from 14.7 to 64.8) years, who had undergone surgery for congenital cardiac defect between 1953 and 1989. The use of medicines among patients in 2004 was compared with 10232 age- and sex-matched control subjects. RESULTS: The overall use of medicines was frequent; 62% of patients and 53% of controls had purchased at least one prescribed medicine (risk ratio: 1.2, 95% confidence interval: 1.1-1.2). The number of patients using cardiovascular medicines (17%) and anti-thrombotic agents (5%) was higher than that of control subjects (risk ratio: 2.2 and 8.4). In addition, the patients needed medicinal care for epilepsy (3%), asthma (7%), and psychiatric diseases (10%) more often than did controls (risk ratio: 2.2, 1.5, and 1.3, respectively). CONCLUSION: Patients operated on for congenital cardiac defect had more chronic diseases and used more medicines than did controls.

Causes of late deaths after pediatric cardiac surgery: a population-based study.

OBJECTIVES: We examined the causes and modes of late death after pediatric cardiac surgery. BACKGROUND: The late mortality of patients operated on for congenital heart defect (CHD) is comprehensively unexamined. In this study, the causes of death were examined to obtain further knowledge of the morbidity of the patients. METHODS: We studied all late deaths of patients operated on for CHD in Finland during the years 1953 to 1989. We calculated the survival of patients, identified the causes of deaths from death certificates, and examined the modes of CHD-related deaths. We compared the survival and the causes of non-CHD-related deaths to those of the general population. RESULTS: Of the 6,024 patients who survived their first operation, 592 (9%) died during the 45-year follow-up period. The progress of treatment was seen in the survival of the patients operated on in different decades. The cause of death was confirmed with postmortem examination in 474 (81%) cases. The majority of patients (397, 67%) died owing to the CHD. Furthermore, non-CHD-related mortality was twice as high (risk ratio 1.9, 95% confidence interval 1.5 to 2.4) as expected. The main mode for CHD-related death was heart failure (40%). Other modes included perioperative (26%), sudden (22%), and cardiovascular (12%) deaths. The number of deaths caused by neurological and respiratory diseases was higher and the number of accidental deaths was lower than expected. CONCLUSIONS: The survival of patients was lower than that of the general population (relative 45-year survival 89%). Most patients died owing to CHDs, but non-CHD-related mortality was also high.

Late results and quality of life after pediatric cardiac surgery in Finland: a population-based study of 6,461 patients with follow-up extending up to 45 years.

This population-based study characterizes the history and progress of pediatric cardiac surgery in Finland. For the first time in the literature, all defects and procedures are included in an outcome study, reflecting the true effectiveness of operative treatment. All the patients operated on since the beginning of pediatric cardiac surgery in Finland in 1953 and through the end of 1989 were included in this population-based, long-term outcome study. The survival was defined from the Finnish Population Registry Center. The survival rates were compared with those of an age- and sex-matched general population. During the study period, 6,461 patients underwent surgery; 96% of them were traced. A questionnaire was used to study the quality of life. The 45-year survival of patients was 15% less than that of the general population. The survival and the number of necessary operations varied widely with the defect. The patients coped well with their defects when compared with the general population. The education level was similar, and the employment level was higher than expected. Patients were living in a steady relationship as often as the general population, but parenthood was less frequent than expected. The incidence of congenital heart disease among the children of the patients was 2.4%. The overall survival of children operated on for heart defects is fairly good. The patients, especially those with simpler defects, do not have increased risk of death years after successful operation. The majority of patients are feeling well, and their life situation is similar to the general population.

Recurrent pericarditis in children and adolescents: report of 15 cases.

OBJECTIVES: The aim of this study was to analyze the clinical findings, course, and treatment of recurrent pericarditis (RP) in patients with onset in childhood and adolescence. BACKGROUND: Recurrent pericarditis is a chronic condition that has presented problems in management. Knowledge about this disease is based on observations in adults, and no series of children has previously been published. METHODS: Fifteen children (nine males, six females) in whom pericarditis had recurred at least twice were encountered in the period 1985 to 1998. Their age at onset was 6.5 to 16.8 years (mean 11.6 years), and the follow-up was 4.0 to 16.2 years (mean 8.0 years). RESULTS: Recurrent pericarditis was preceded by open-heart surgery by 1 month to 5 years earlier in 7 of 15 patients. The six children with an atrial septal defect (ASD) had an operation at an older age (mean 9.9 years) than usual (mean 4.8 years). The risk of RP in children operated on for ASD at the age of six years or later was 5%. An initial attack of pericarditis was associated with pleuritis and/or pneumonia in 10 of 15 patients and with colitis in 2 of 15 patients During follow-up, the patients had 2 to 30 recurrences (mean 9.9). Later attacks tended to be milder. At the end of follow-up, 7 patients had been without attacks for >or=4 years, whereas after 4 to 16 years, the remaining patients still had active disease. No instance of constriction was found. Altogether, 11 of 15 patients were treated with corticosteroids. However, corticosteroids, whether alone or with methotrexate (n = 5), azathioprine (n = 1), cyclosporine (n = 1), or colchicine (n = 4) did not prevent recurrences. CONCLUSIONS: The most frequent background for RP in children was the closure of ASD after the age of six years. Its course was unpredictable and often chronic, irrespective of the underlying cause or the therapy given. Colchicine did not prevent relapses.