Lessons learned from a thorny case

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Pumpless Lung Assist as a Bridge to Medical Therapy in a Teenager With Pulmonary Arterial Hypertension and Partial Anomalous Pulmonary Venous Return

International audienceHeart failure is the main cause of death in patients with pulmonary arterial hypertension and congenital heart disease. We used an original approach in a 15-year-old girl with rapidly progressive right heart failure secondary to severe pulmonary arterial hypertension and partial anomalous pulmonary venous return. After surgical congenital heart defect repair on cardiopulmonary bypass, she was weaned off bypass using a central Novalung for 11 days, then started on triple specific pulmonary vasodilator therapy

Right ventricular remodelling in CHD-PAH patients using 3D speckle tracking

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Right ventricular remodelling in CHD-PAH patients using 3D speckle tracking

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Repeat mitral valve repair for haemolysis in children

SummaryBackgroundSevere haemolysis is a rare complication after mitral valve repair in congenital heart disease.AimWe describe four children with severe mitral regurgitation who underwent valve repair and subsequently developed profound haemolytic anaemia.MethodsClinical, echocardiographic and surgical data were collected retrospectively from a surgical centre in France during a 5-year period.ResultsTwo patients had atrioventricular septal defects, one patient had congenital mitral dysplasia and one had anomalous left coronary artery from the pulmonary artery with mitral regurgitation. Haemolysis was diagnosed 20 to 75days after surgery, as a result of clinical and biological examination; it was severe, and blood transfusion support was necessary in all cases. Haemolysis was always associated with eccentric mitral regurgitation with a variable degree of severity (from low to severe). After exclusion of other haemolysis aetiology, redo mitral repair surgery was performed successfully in all cases. The haemolysis was considered to be mechanical in origin, caused by regurgitation of blood through the residual mitral regurgitation and stitches.ConclusionSevere haemolysis is a rare complication that can occur after mitral valve repair in congenital heart disease. All patients underwent successful redo repair and had resolution of haemolysis after surgery. Even if redo surgery is required, iterative mitral repair is possible

A piglet model of iatrogenic rectosigmoid hypoganglionosis reveals the impact of the enteric nervous system on gut barrier function and microbiota postnatal development

International audienceBackground - Hirschsprung-associated enterocolitis physiopathology likely involves disturbed interactions between gut microbes and the host during the early neonatal period. Our objective was to create a neonatal porcine model of iatrogenic aganglionosis to evaluate the impact of the enteric nervous system (ENS) on microbiota and intestinal barrier postnatal development. Methods - Under general anesthesia, the rectosigmoid serosa of 5-day-old suckling piglets was exposed to 0.5% benzalkonium chloride solution (BAC, n = 7) or saline (SHAM, n = 5) for 1 h. After surgery, animals returned to their home-cage with the sow and littermates and were studied 21 days later. Results - BAC treatment induced partial aganglionosis with absence of myenteric plexus and reduced surface area of submucosal plexus ganglia (-58%, P < 0.05) in one third of the rectosigmoid circumference. Epithelial permeability of this zone was increased (conductance +63%, FITC-dextran flux +386%, horseradish-peroxidase flux +563%, P < 0.05). Tight junction protein remodeling was observed with decreased ZO-1 (-95%, P < 0.05) and increased claudin-3 and e-cadherin expressions (+197% and 61%, P < 0.05 and P = 0.06, respectively). BAC piglets harbored greater abundance of proinflammatory bacteria (Bilophila, Fusobacterium) compared to SHAM in the rectosigmoid lumen. Conclusions - This large animal model demonstrates that hypoganglionosis is associated with dramatic defects of gut barrier function and establishment of proinflammatory bacteria. Level of evidence

3D modeling and printing in large native right ventricle outflow tract to plan complex percutaneous pulmonary valve implantation

Objective: Investigating accuracy of cardiac tomography (CT) derived post-processing3D reconstruction (CT-PPR) and 3D printing to predict percutaneous pulmonary valve implantation (PPVI) feasibility. Background: PPVI feasibility remains challenging in large native regurgitant right ventricle outflow tract (RVOT). Methods: Fifteen patients with large native RVOT were investigated. CT-PPR consisted in RVOT long-axis curvilinear reconstruction (LACR) to measure the landing zone (LZ), and 3D volume rendering for morphological evaluation. A STL was generated to create 3D printed model (flexible resin). Balloon sizing was subsequently performed to measure LZ diameter (3D-MBD), compared to invasive balloon diameter (IBD) during catheterization, considered as the Gold Standard. Two operators predicted the feasibility of PPVI using CT-PPR and 3D printed models independently and blinded to outcome. Results: On 3D printed models, RVOT shape was tubular in 5 patients, divergent in 5 patients, concave in 4 patients and convergent in one. Agreement with CT-PPR RVOT shape morphology assessment was observed in 93% of cases (Kappa coefficient 0.91, p < 0.0001). Minimal IBD was 26.0 [24.4–27.9] mm. Minimal LZ LACR diameters were well correlated to IBD (Spearman rho = 0.67, p = 0.007; r2 = 0.55, p = 0.002) with a mean underestimation bias of 2.8 mm. Minimal 3D-MBD was correlated to IBD (Spearman rho = 0.55, p = 0.04, r2 = 0.50, p = 0.003) with mean underestimation bias of 0.9 mm.PPVI was successful and uneventful in 11 patients, challenging in one and non-feasible in 3. Using CT-PPR and 3D printed models, interventionists predictions agreement with outcome was 93% and 87% (Kappa coefficient = 0.86, p = 0.0001, and Kappa coefficient = 0.65, p = 0.0007 respectively). Conclusion: RVOT 3D CT-PPR and printing allow reliable assessment of RVOT shape and LZ diameter prior to PPVI. Prediction of PPVI feasibility in challenging cases is facilitated

A modified procedure for percutaneous pulmonary valve implantation of the Edwards SAPIEN 3 valve

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