Ross-kabbani operation in an infant with mitral valve dysplasia.

Background. Mitral valve replacement can be very difficult to obtain in infants because the valve annulus diameter can be smaller than the available prosthesis. Case Report. We describe the case of a 2-month-old female weighing 3.5 kg affected by mitral valve dysplasia leading to severe valve stenosis. Despite full medication, the clinical conditions were critical and surgery was undertaken. The mitral valve was unsuitable for repair and the orifice of mitral anulus was 12 mm, too small for a mechanical prosthesis. Therefore, a Ross-Kabbani operation was undertaken, replacing the mitral valve with the pulmonary autograft and reconstructing the right ventricular outflow tract with an etherograft. Results. The postoperative course was uneventful and the clinical conditions are good at 4-month follow-up. Conclusion. The Ross-Kabbani operation can be an interesting alternative to mitral valve replacement in infants when valve repair is not achievable and there is little space for an intra-annular mechanical prosthesis implant

Role of endovascular repair in the management of late pseudo-aneurysms following open surgery for aortic coarctation.

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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

Difetto del setto interventricolare

Il difetto interventricolare (DIV) è una cardiopatia congenita molto frequente rappresentando, dopo la bicuspidia aortica, il 20% di tutte le cardiopatie congenite nelle varie casistiche chirurgiche (1-38)

Neonatal right Lung Emphysema due to Pulmonary Artery Sling

Pulmonary artery sling is a rare variant of vascular ring where the left pulmonary artery arises from the right and loops behind the trachea or right bronchus causing airway compression. A 40-day-old infant had been mechanically ventilated since birth for severe hypercapnia and right lung emphysema. Left pulmonary artery reimplantation was successfully performed

Coartazione artica e interruzione arco aortico

La coartazione e l\u2019interruzione dell\u2019arco sono patologie ostruttive dell\u2019arco aortico che rappresentano circa il 6-8% di tutte le cardiopatie congenite e si possono presentare isolate o associate ad altre malformazioni. La coartazione aortica \ue8 sicuramente la forma pi\uf9 frequente, si presenta con un\u2019incidenza di 1 caso su 2.500 nati vivi, rappresenta circa il 5-8% di tutte le cardiopatie congenite e nel 60-70% dei casi in cui si manifesta in epoca neonatale si associa ad ipoplasia dell\u2019arco aortic