Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age.

No abstract

A new mortality model based on a mixture distribution function is proposed. We mix a half-normal distribution with a generalization of the skew-normal distribution. As a result, we get a six-parameter distribution function that has a good fit with a wide variety of mortality patterns. This mixture model is fitted to several mortality data schedules and compared with the Siler (five-parameter) and Heligman-Pollard (eight-parameter) models. Our proposal serves as a convenient compromise between the Heligman-Pollard model (which ensures a good fit with data but is often overparameterized) and the Siler model (which is more compact but fails to capture 'accident humps').

Premature mortality is often a neglected component of overall deaths, and the most difficult to identify. However, it is important to estimate its prevalence. Following Pearson's theory about mortality components, a definition of premature deaths and a parametric model to study its transformations are introduced. The model is a mixture of three distributions: a Half Normal for the first part of the death curve and two Skew Normals to fit the remaining pieces. One advantage of the model is the possibility of obtaining an explicit equation to compute life expectancy at birth and to break it down into mortality components. We estimated the mixture model for Sweden, France, East Germany and Czech Republic. In addition, to the well-known reduction in infant deaths, and compression and shifting trend of adult mortality, we were able to study the trend of the central part of the distribution of deaths in detail. In general, a right shift of the modal age at death for young adults is observed; in some cases, it is also accompanied by an increase in the number of deaths at these ages: in particular for France, in the last twenty years, premature mortality increases.

No abstract

Adults with congenital heart disease (ACHD) represent a growing population. To evaluate frequency, type and outcomes of cardiac surgery in ACHD, we gathered data from the European Congenital Heart Surgeons Association Database of 20,602 adult patients (≥18 years) with a diagnosis of congenital heart disease who underwent cardiac surgery, between January 1997 and December 2017. We demonstrated that overall surgical workload (as absolute frequencies of surgical procedures per year) for this specific subset of patients increased steadily during the study period. The most common procedural groups included septal defects repair (n = 5740, 28%), right-heart lesions repair (n = 5542, 27%) and left-heart lesions repair (n = 4566, 22%); almost one-third of the procedures were re-operations (n = 5509, 27%). When considering the year-by-year relative frequencies of the main procedural groups, we observed a variation of the surgical scenario during the last two decades, characterized by a significant increase over time for right and left-heart lesions repair (p < 0.0001, both); while a significant decrease was seen for septal defects repair (p < 0.0001) and transplant (p = 0.03). Overall hospital mortality was 3% (n = 622/20,602 patients) and was stable over time. An inverse relationship between mortality and the number of patients operated in each center (p < 0.0001) was observed.

No abstract

Objectives: We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). Methods: Eleven centers belonging to the European Congenital Heart SurgeonsAssociation contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). Results:The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death