Double venous drainage through the superior vena cava in minimally invasive aortic valve replacement: a retrospective study

Aim To compare the outcomes of patients who underwent upper mini-sternotomy or right mini-thoracotomy and those who underwent full sternotomy and to report a technical improvement in venous drainage by means of double venous cannulation of the superior vena cava (SVC) in mini surgical procedures. Methods We retrospectively analyzed the outcome of 217 patients who underwent aortic valve replacement through upper mini-sternotomy or right mini-thoracotomy at the Department of Cardiovascular Surgery, University Medical Centre Ljubljana, Slovenia from 1996 till 2010. Cannulation of SVC and right atrial appendage was performed in 142/217 (65%) patients, while in the remaining 75 (35%) patients, double cannulation of SVC was used for venous drainage. The results of patients who underwent mini approaches were compared to 236 patients who underwent full sternotomy for the same purpose from 2009 to 2010 at the same center. Results We found a shorter mean length of intensive care unit stay, less volume chest-tube drainage, shorter crossclamp and cardio pulmonary bypass times, and less postoperative permanent pacemaker implantations in the minimally invasive group patients than in full sternotomy group patients. Using double cannulation of the SVC for venous drainage made venous cannulation in mini approaches easier and eliminated the need for obtaining femoral venous access. Conclusion Our study confirmed that even though technically challenging, upper mini-sternotomy and right minithoracotomy approaches for aortic valve replacement have potential advantages over conventional median sternotomy. They were proved to be safe, efficacious, and can significantly reduce surgical trauma and are therefore particularly valuable in some higher risk, obese, diabetic and elderly patients. Using double cannulation of SVC for venous drainage made venous cannulation easier and eliminated the need for obtaining femoral venous access

Mid-Aortic Syndrome in a 3-Year-Old Girl Successfully Treated by Aorto-Aortic Grafting and Renal Artery Implantation Into the Graft

Mid-aortic syndrome, an uncommon acquired or congenital condition characterized by segmental narrowing of the abdominal or distal descending thoracic aorta, is frequently accompanied by ostial stenosis of the aorta\u27s branches. If left untreated, it can result in life-threatening complications secondary to severe hypertension. We report the case of a 3-year-old girl with congenital mid-aortic syndrome, who was diagnosed by chance in the course of a viral illness, and whose high blood pressure values were first dismissed as inaccurate. Attempts to achieve medical or endovascular control of her hypertension were unsuccessful. She was thereafter successfully treated by aorto–aortic bypass grafting, resection of the stenotic segments of both renal arteries, and implantation of the patent arterial segments into the graft

Validation of distinct pathogenic patterns in a cohort of membranoproliferative glomerulonephritis patients by cluster analysis

Background: A novel data-driven cluster analysis identified distinct pathogenic patterns in C3-glomerulopathies and immune complex-mediated membranoproliferative glomerulonephritis. Our aim was to replicate these observations in an independent cohort and elucidate disease pathophysiology with detailed analysis of functional complement markers. ----- Methods: A total of 92 patients with clinical, histological, complement and genetic data were involved in the study, and hierarchical cluster analysis was done by Ward method, where four clusters were generated. ----- Results: High levels of sC5b-9 (soluble membrane attack complex), low serum C3 levels and young age at onset (13 years) were characteristic for Cluster 1 with a high prevalence of likely pathogenic variations (LPVs) and C3 nephritic factor, whereas for Cluster 2-which is not reliable because of the small number of cases-strong immunoglobulin G staining, low C3 levels and high prevalence of nephritic syndrome at disease onset were observed. Low plasma sC5b-9 levels, decreased C3 levels and high prevalence of LPV and sclerotic glomeruli were present in Cluster 3, and patients with late onset of the disease (median: 39.5 years) and near-normal C3 levels in Cluster 4. A significant difference was observed in the incidence of end-stage renal disease during follow-up between the different clusters. Patients in Clusters 3-4 had worse renal survival than patients in Clusters 1-2. ----- Conclusions: Our results confirm the main findings of the original cluster analysis and indicate that the observed, distinct pathogenic patterns are replicated in our cohort. Further investigations are necessary to analyse the distinct biological and pathogenic processes in these patient groups

C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy

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Post-vaccine glomerulonephritis in an infant with hereditary C2 complement deficiency: case study

Abstract We describe a case of a post vaccine immune complex-mediated glomerulonephritis in an infant with compound heterozygous mutations of C2 complement component gene, which is the first such case in the literature. The three and a half months old boy presented with clinical and laboratory signs of nephritic syndrome and was successfully treated with methylprednisolone. An explanation of such a clinical picture may lie in the interaction between C2 deficiency and vaccination

Functional characterization of two novel non-synonymous alterations in CD46 and a Q950H change in factor H found in atypical hemolytic uremic syndrome patients.

Atypical hemolytic uremic syndrome (aHUS) is a disease of complement dysregulation, characterized by hemolytic anemia, thrombocytopenia and acute renal failure. Mutations in complement inhibitors are major risk factors for development of aHUS. The three aHUS patients reported in this study had several previously identified alterations in complement inhibitors; e.g. risk haplotypes in CD46 and factor H but we also identified two novel heterozygous non-synonymous CD46 alterations (p.E142Q and p.G259V). Presence of G259V caused decreased expression of the recombinant mutant CD46 compared to wild type (WT). Western blot analysis showed that the majority of the expressed G259V protein was in the precursor form, suggesting that it is processed less efficiently than WT. Low CD46 expression on the surface of the patient's neutrophils confirmed the in vitro results. Further, G259V had a substantially impaired ability to act as a cofactor to factor I, in the degradation of both C3b and C4b. The E142Q mutant showed neither decreased expression nor impaired function. Two of the patients also had a heterozygous non-synonymous alteration in factor H (p.Q950H), reported previously in aHUS but not functionally tested. This variant showed moderately impaired function in hemolytic assays, both using patient sera and recombinant proteins. The recombinant Q950H also showed a somewhat decreased expression compared to WT but the complement inhibitory function in fluid phase was normal. Taken together, we report a novel CD46 alteration showing both a decreased protein expression and substantially impaired cofactor function (G259V) and another without an effect on expression or cofactor function (E142Q). Moreover, mild consequences of a previously reported aHUS associated rare variant in factor H (Q950H) was also revealed, underlining the clear need for functional characterization of each new aHUS associated mutation