The outcome of patients with right atrial isomerism is poor.

Right-atrial isomerism (RAI) is a heterotaxy syndrome with disturbances of left-right axis development resulting in complex heart malformations and anomalies of the thoracic and abdominal organs. To study the outcome of RAI, all data from patients diagnosed with this syndrome at Helsinki University Hospital between January 1976 and December of 2010 were reviewed. The outcomes were studied for 32 patients (38 % girls). The overall survival was 22 % at a median follow-up time of 13.8 years (range 0.1-33). Extracardiac malformations, mostly asplenic, occurred in 91 % of patients. Cardiac defects included dextrocardia in 44 % and common atrioventricular valve in 100 % of patients. Ventriculoarterial discordance or double-outlet ventricle was seen in 56 and 44 % of patients, respectively. Total anomalous pulmonary venous drainage occurred in 75 % and partially anomalous venous drainage in 13 % of patients. Pulmonary outflow-tract obstruction was identified in 91 % of patients. Cardiac arrhythmias were noted in nine patients (28 %), two of them with atrioventricular block. Cardiovascular surgery was performed in 71 % patients (N = 25), seven patients were inoperable. Biventricular repair was not possible in any of the patients. During long-term follow-up there was no significant difference between the patients with total, normal, or partially anomalous pulmonary venous drainage (P = 0.5). In conclusion, RAI is one of the most severe forms of congenital cardiac diseases. The prognosis remains poor despite modern surgical techniques. When RAI is identified during pregnancy, prenatal counseling, termination, or planning for prompt cardiac treatment after the birth is necessary.

Lowered levels of serum albumin and HDL-cholesterol in children with a recent mild infection.

BACKGROUND: Infections are associated with atherogenic changes in serum.AIM. To elucidate effects of recent infections on risk factors for coronary heart disease in children. SUBJECTS AND METHODS: In 1983 and again 3 years later, 2458 individuals aged 9, 12, 15, 18 and 21 years were investigated. In 1986, 106 subjects had symptoms of infection during the past 2 weeks before their follow-up visit. Their serum albumin and lipid concentrations were compared to those in 1983 when these individuals probably were healthy. An age- and sex-matched healthy control group from the cohort 1986 was chosen for comparison. For cholesterol age, sex and body mass index specific Z-scores in addition to actual values were used in statistical comparisons. RESULTS: Serum albumin was 42 g/L in subjects with positive history of infection and 46 g/L in healthy controls (P<0.0001). HDL-cholesterol and the ratio of HDL- to total cholesterol were lower with increasing evidence of infection. Elevated serum C-reactive protein (CRP) or orosomucoid grouped the subjects with high and low serum HDL-cholesterol concentrations better than history of infection alone. CONCLUSION: A mild infection lowers serum HDL-cholesterol and serum albumin concentrations, which both favour atherogenesis.