Modelling Survival and Mortality Risk to 15 Years of Age for a National Cohort of Children with Serious Congenital Heart Defects Diagnosed in Infancy

Congenital heart defects (CHDs) are a significant cause of death in infancy. Although contemporary management ensures that 80% of affected children reach adulthood, post-infant mortality and factors associated with death during childhood are not well-characterised. Using data from a UK-wide multicentre birth cohort of children with serious CHDs, we observed survival and investigated independent predictors of mortality up to age 15 years. Methods Data were extracted retrospectively from hospital records and death certificates of 3,897 children (57% boys) in a prospectively identified cohort, born 1992–1995 with CHDs requiring intervention or resulting in death before age one year. A discrete-time survival model accounted for time-varying predictors; hazards ratios were estimated for mortality. Incomplete data were addressed through multilevel multiple imputation. Findings By age 15 years, 932 children had died; 144 died without any procedure. Survival to one year was 79.8% (95% confidence intervals [CI] 78.5, 81.1%) and to 15 years was 71.7% (63.9, 73.4%), with variation by cardiac diagnosis. Importantly, 20% of cohort deaths occurred after age one year. Models using imputed data (including all children from birth) demonstrated higher mortality risk as independently associated with cardiac diagnosis, female sex, preterm birth, having additional cardiac defects or non-cardiac malformations. In models excluding children who had no procedure, additional predictors of higher mortality were younger age at first procedure, lower weight or height, longer cardiopulmonary bypass or circulatory arrest duration, and peri-procedural complications; non-cardiac malformations were no longer significant. Interpretation We confirm the high mortality risk associated with CHDs in the first year of life and demonstrate an important persisting risk of death throughout childhood. Late mortality may be underestimated by procedure-based audit focusing on shorter-term surgical outcomes. National monitoring systems should emphasise the importance of routinely capturing longer-term survival and exploring the mechanismsThis work was supported by a British Heart Foundation project grant (reference PG/02/065/13934). RLK was awarded an MRC Special Training Fellowship in Health of the Public and Health Services Research (reference G106/1083). HG and the Centre for Paediatric Epidemiology and Biostatistics benefited from Medical Research Council funding support to the MRC Centre of Epidemiology for Child Health (reference G04005546). Great Ormond St Hospital for Children NHS Trust and the UCL Institute of Child Health receives a proportion of funding from the Department of Health's NIHR Biomedical Research Centres schem

Pulmonary embolism in neurosurgical patients

We report the outcome of a retrospective study on the frequency of pulmonary embolism during the hospital stay in a series of 7,250 neurosurgical patients. Of 4,500 patients who underwent surgery 25 (0.55%) developed pulmonary embolism at some point after the operation while 5 of the 2,750 patients not operated on (0.18%) developed a fatal pulmonary embolism. We analyze the general risk factors--age, sex, length of stay and paralysis of the limbs. Meningioma was the most frequent intracranial tumor to be affected by this complication. We discuss the connection between thromboembolism and meningioma

Intra-extracranial thrombosis of the internal carotid artery associated with meningioma

Cases of internal carotid artery occlusion associated with an intracranial tumor are rare. The authors report two cases of complete occlusion of the internal carotid artery. In one case the thrombosis was probably caused by direct compression of the carotid siphon by a large meningioma arising from the inner third of the sphenoid wing. In the other it was associated with multiple meningiomatosis although there was no contiguity between the intracranial tumors and the occluded carotid artery. After a review of literature we think they are of some interest, and we have found an interesting incidence of association between thrombosis and meningiomaCases of internal carotid artery occlusion associated with an intracranial tumor are rare. The authors report two cases of complete occlusion of the internal carotid artery. In one case the thrombosis was probably caused by direct compression of the carotid siphon by a large meningioma arising from the inner third of the sphenoid wing. In the other it was associated with multiple meningiomatosis although there was no contiguity between the intra cranial tumors and the occluded carotid artery. After a review of literature we think they are of some interest . and we have found an interesting incidence of association between thrombosis and meningioma

Clinical significance of family history and bicuspid aortic valve in children and young adult patients with Marfan syndrome

Background: Marfan syndrome is an autosomal dominant disorder of the connective tissue, whose cardinal features affect eyes, musculoskeletal, and cardiovascular system. Despite prevalence and natural history of cardiovascular manifestation are well known in adults, little is known about children and young adult patients. The aim of this study was to describe a well-characterised cohort of consecutive children and young patients with marfan syndrome, looking at the impact of family history and presence of bicuspid aortic valve on disease severity.Methods: A total of 30 consecutive children and young patients with Marfan syndrome were evaluated. All patients underwent a comprehensive clinical-instrumental-genetic evaluation. Particular attention was posed to identify differences in prevalence of cardiovascular abnormalities between patients with and without family history of Marfan syndrome or bicuspid aortic valve.Results: Of these 30 patients, family history of Marfan syndrome and bicuspid aortic valve were present in 76 and 13%, respectively. Compared to patients with family history of Marfan syndrome, those without showed higher prevalence of aortic sinus dilation (87 versus 32%, p-value = 0.009), greater aortic sinus diameters (4.2 ± 2.1 versus 1.9 ± 1.1 z score, p-value = 0.002), and higher rate of aortic surgery during follow-up (37 versus 0%, p-value = 0.002). Compared to patients with tricuspid aortic valve, those with bicuspid aortic valve were younger (3.2 ± 4.3 versus 10.7 ± 6.8 years old, p-value = 0.043), showed greater aortic sinus diameters (4.2 ± 0.9 versus 2.2 ± 1.6 z score, p-value = 0.033), and underwent more frequently aortic root replacement (50 versus 4%, p-value = 0.004).Conclusions: In our cohort of patients with Marfan syndrome, the absence of family history and the presence of bicuspid aortic valve were associated to severe aortic phenotype and worse prognosis