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

Health‐Related Quality of Life and Its Association With Outcomes in Adults With Congenital Heart Disease and Heart Failure: Insight From FRESH‐ACHD Registry

Background Quality of Life (QoL) is a prognostic factor in heart failure (HF) of patients with acquired cardiac disease. The aim of this study was to determine the predictive value of QoL on outcomes in adults with congenital heart disease (ACHD) and HF. Methods and Results Quality of life of 196 adults with congenital heart disease with clinical heart failure (HF) (mean age: 44.3±13.8 years; 51% male; 56% with complex congenital heart disease; 47% New York Heart Association class III/IV) included in the prospective multicentric registry FRESH‐ACHD (French Survey on Heart Failure−Adult with Congenital Heart Disease) was assessed using the 36‐Item Short Form Survey (SF‐36), a patient‐reported survey. The primary end point was defined by all‐cause death, HF‐related hospitalization, heart transplantation, and mechanical circulatory support. At 12 months, 28 (14%) patients reached the combined end point. Patients with low quality of life experienced major adverse events more frequently (logrank P=0.013). On univariate analysis, lower score at physical functioning (hazard ratio [HR], 0.98 [95% CI, 0.97–0.99]; P=0.008), role limitations related to physical health (HR, 0.98 [95% CI, 0.97–0.99]; P=0.008), and general health dimensions of the SF‐36 (HR, 0.97 [95% CI, 0.95–0.99]; P=0.002) were significantly predictive of cardiovascular events. However, after multivariable analysis, SF‐36 dimensions were no longer significantly associated with the primary end point. Conclusions Patients with congenital heart disease with HF and poor quality of life experience severe events more frequently, making quality of life assessment and rehabilitation programs essential to alter their trajectory

Evaluación del tracto de salida del ventrículo derecho mediante resonancia magnética con flujo 4D en pacientes sometidos a recambio valvular pulmonar transcatéter

Introducción y objetivos La resonancia magnética (RM) con flujo 4D suele utilizarse para evaluar el ventrículo derecho y las arterias pulmonares antes del implante percutáneo de la válvula pulmonar (IPVP). Como el IPVP está limitado por el tamaño del tracto de salida del ventrículo derecho (TSVD), se necesita medirlo con precisión para planificar la intervención. El objetivo del presente estudio es comparar diferentes modalidades de RM con la medición invasiva del TSVD con balón. Métodos Estudio unicéntrico prospectivo de pacientes sometidos a IPVP por insuficiencia pulmonar aislada evaluada mediante RM con flujo 4D, precesión libre en estado estacionario/gradiente eco-3D (3D SSFP/GRE) y angiografía por RM con contraste. La medición con balón se consideró la referencia. Resultados Se incluyó a 23 adultos (media de edad, 38,4 ± 12,5 años). En 18 pacientes el IPVP fue exitoso. El diámetro medio del TSVD más pequeño fue de 25,4 ± 4,3 mm medido con balón y 25,6 ± 3,8 y 21,8 ± 3,6 mm por RM con flujo 4D en sístole y diástole respectivamente. Comparados con los medidos con balón, los diámetros del TSVD se correlacionaron mejor cuando se estimaron mediante RM con flujo 4D sistólico (r = 0,89; p Compared to balloon sizing, RVOT diameters were better correlated when estimated by systolic 4D flow MRI (r = 0.89, P < .001) than by diastolic 4D flow MRI (r = 0.71, P < .001), 3D contrast magnetic resonance angiography (r = 0.73; P < .001) and 3D SSFP/GRE (r = 0.50; P = .04) and was not significantly correlated when estimated by 2D in diastole and systole. The mean difference between systolic 4D flow MRI and balloon sizing was 0.2 mm (95%CI, –3.5 to 3.9 mm), whereas it was wider with other techniques. Conclusions: Beyond the quantification of pulmonary valve regurgitation, 4D flow allows accurate estimation of RVOT diameters, especially in systole, which is fundamental before planning PPVI.Fil: Karsenty, Clément. Hopital Europeen Georges Pompidou; Francia. Children's Hospital; Francia. Institut National de la Santé et de la Recherche Médicale; Francia. Clinique Pasteur; FranciaFil: Alattar, Yousef. Hopital Europeen Georges Pompidou; FranciaFil: Mousseaux, Elie. Hopital Europeen Georges Pompidou; Francia. Institut National de la Santé et de la Recherche Médicale; FranciaFil: Marcilhacy, Gabrielle. Hopital Europeen Georges Pompidou; FranciaFil: Gencer, Umit. Institut National de la Santé et de la Recherche Médicale; FranciaFil: Craiem, Damian. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; ArgentinaFil: Iserin, Laurence. Hopital Europeen Georges Pompidou; FranciaFil: ladouceur, Magalie. Hopital Europeen Georges Pompidou; Francia. Institut National de la Santé et de la Recherche Médicale; FranciaFil: Legendre, Antoine. Hopital Europeen Georges Pompidou; FranciaFil: Laredo, Mikael. Hopital Europeen Georges Pompidou; Francia. Assistance Publique Hôpitaux de Paris; FranciaFil: Bonnet, Damien. Universite de Paris; Francia. Hôpital Universitaire Necker-Enfants malades; FranciaFil: Malekzadeh Milani, Sophie. Hopital Europeen Georges Pompidou; Francia. Hôpital Universitaire Necker-Enfants malades; FranciaFil: Soulat, Gilles. Hopital Europeen Georges Pompidou; Francia. Institut National de la Santé et de la Recherche Médicale; Franci

Patent foramen ovale closure in children without cardiopathy: Child-PFO study

International audienc

Intradetrusor Injections of Botulinum Toxin Type A in Children With Spina Bifida: A Multicenter Study

International audienceOBJECTIVE:To assess the effectiveness of intradetrusor injections of botulinum toxin type A (IDBTX-A) in children with spina bifida.METHODS:All patients aged less than 16 years old who underwent IDBTX-A between 2002 and 2016 at 6 institutions were included in a retrospective study. Our primary endpoint was the success rate of IDBTX-A defined as both clinical improvement (no incontinence episodes between clean intermittent catheterizations [CICs], absence of urgency, and less than 8 CICs per day) and urodynamic improvement (resolution of detrusor overactivity and normal bladder compliance for age) lasting ≥12 weeks. Predictive factors of success were assessed through univariate analysis.RESULTS:Fifty-three patients with a mean age of 8.5 years were included. All patients were under CIC and 88.7% had received anticholinergics with either poor efficacy or bothersome adverse events. The global success rate of the first injection (clinical and urodynamic) was 30%. Patients with closed spinal dysraphism had a significantly better success rate than patients with myelomeningocele (P = .002). The clinical success rate was 66% and was significantly associated with maximum urethral closure pressure (34 cm H2O vs 54.4 cm H2O, P = .02). The urodynamic success rate was 34%. Maximum cystometric capacity (P <.0001) and compliance (P = .01) significantly improved after the first IDBTX-A and maximum detrusor pressure tended to decrease (P = .09) except in the subgroup of patients with poor compliance. After a mean follow-up of 3.7 years, 23 patients (43.4%) required augmentation cystoplasty. Excluding 6 patients who were lost to follow-up, 38.3% of patients were still undergoing botulinum toxin injections at last follow-up.CONCLUSION:In this series, despite the fact that IDBTX-A enabled clinical improvement in 66% patients, urodynamic outcomes were poor resulting in a low global success rate (30%)

Implication of heterozygous variants in genes of the leptin-melanocortin pathway in severe obesity

International audienceContext: Unlike homozygous variants, the implication of heterozygous variants on the leptin-melanocortin pathway in severe obesity has not been established.Objective: To describe the frequency, the phenotype, and the genotype-phenotype relationship for heterozygous variants in LEP, LEPR, POMC, and PCSK1 in severe obesity.Methods: In this retrospective study, genotyping was performed on at least one of the LEP, LEPR, POMC, and PCSK1 genes in 1,486 probands with severe obesity (600 children, 886 adults). The phenotype was collected in 60 subjects with heterozygous variants and 16 with homozygous variants. We analyzed variant frequency, Body Mass Index (BMI), age of obesity onset, food impulsivity, and endocrine abnormalities.Results: The frequency of subjects with homozygous variants was 1.7% (n=26), and 6.7% (n=100) with heterozygous variants. Adults with homozygous variants had a higher BMI (66 versus 53 kg/m 2, p=0.015), an earlier onset of obesity (0.4 versus 5.4 years, p<0.001), more often food impulsivity (83% versus 42%, p=0.04), and endocrine abnormalities (75% versus 26%, p<0.01). The BMI was higher for subjects with high-impact heterozygous variants (61 versus 50 kg/m², p=0.045) and those with a second heterozygous variant on the pathway (65 versus 49 kg/m², p<0.01). In children, no significant differences were found for the age of obesity onset and BMI.Conclusions: Heterozygous variants in LEP, LEPR, POMC, and PCSK1 are frequent in severe obesity and sometimes associated with a phenotype close to that of homozygotes. These data suggest a systematic search for variants in severe early-onset obesity, to discuss therapy that targets this key pathway

Cardiogenic shock in adults with congenital heart disease: Insights from the FRENSHOCK registry

International audienceBackground: Data on cardiogenic shock in adults with congenital heart disease (ACHD) are scarce.Aim:We sought to describe cardiogenic shock in ACHD patients in a nationwide cardiogenic shock registry.Methods: From the multicentric FRENSHOCK registry (772 patients with cardiogenic shock from 49 French centres between April and October 2016), ACHD patients were compared with adults without congenital heart disease (non-ACHD). The primary outcome was defined by all-cause mortality, chronic ventricular assist device or heart transplantation at 1 year.Results: Out of the 772 patients, seven (0.9%) were ACHD, who were younger (median age: 56 vs. 67 years), had fewer cardiovascular risk factors, such as hypertension (14.3% vs. 47.5%) and diabetes (14.3% vs. 28.3%), and no previous ischaemic cardiopathy (0 vs. 61.5%). Right heart catheterization (57.1% vs. 15.4%), pacemakers (28.6% vs. 4.6%) and implantable cardioverter-defibrillators (28.6% vs. 4.8%) were indicated more frequently in the management of ACHD patients compared with non-ACHD patients, whereas temporary mechanical circulatory support (0 vs. 18.7%) and invasive mechanical ventilation (14.3% vs. 38.1%) were less likely to be used in ACHD patients. At 1 year, the primary outcome occurred in 85.7% (95% confidence interval: 42.1–99.6) ACHD patients and 52.3% (95% confidence interval: 48.7–55.9) non-ACHD patients. Although 1-year mortality was not significantly different between ACHD patients (42.9%) and non-ACHD patients (45.4%), ventricular assist devices and heart transplantation tended to be more frequent in the ACHD group.Conclusions: Cardiogenic shock in ACHD patients is rare, accounting for only 0.9% of an unselected cardiogenic shock population. Despite being younger and having fewer co-morbidities, the prognosis of ACHD patients with cardiogenic shock remains severe, and is similar to that of other patients