The aetiology of cyanosis could be unclear in children, even for specialised paediatricians. Two cases were reported: first, a 6-year-old child with features of left isomerism and Fallot was ...fortuitously diagnosed with anomalous hepatic venous drainage before complete repair. Second, a newborn with an antenatal diagnosis of ductus venosus agenesis had an isolated intermittent right-to-left atrial shunt when upright, with favourable outcome, in contrast to the association with significant heart malformations including inferior caval vein interruption. Multimodality imaging and 3D printing helped to rule out extracardiac causes of persistent cyanosis and plan surgical repair.
•IE is a feared complication of TPVI that affects valve durability and outcomes.•IE following TPVI in CHD exhibits several distinctive features.•Several risk factors are associated with IE.•Patient ...and parent education on IE prevention should be provided.
The introduction of transcatheter pulmonary valve implantation (TPVI) has greatly benefited the management of right ventricular outflow tract dysfunction. Infective endocarditis (IE) is a feared complication of TPVI that affects valve durability and patient outcomes. Current recommendations provide only limited guidance on the management of IE after TPVI (TPVI-IE). This article, by a group of experts in congenital heart disease in children and adults, interventional cardiology, infectious diseases including IE, and microbiology, provides a comprehensive review of the current evidence on TPVI-IE, including its incidence, risk factors, causative organisms, diagnosis, and treatment. The incidence of TPVI-IE varies from 13–91/1000 person-years for Melody valves to 8–17/1000 person-years for SAPIEN valves. Risk factors include history of IE, DiGeorge syndrome, immunosuppression, male sex, high residual transpulmonary gradient and portal of bacteria entry. Staphylococci and streptococci are the most common culprits, whereas Staphylococcus aureus is associated with the most severe disease. In addition to the modified Duke criteria, a high residual gradient warrants a strong suspicion. Imaging studies are helpful for the diagnosis. Intravenous antibiotics guided by blood culture results are the mainstay of treatment. Invasive re-intervention may be required. TPVI-IE in patients with congenital heart disease exhibits several distinctive features. Whether specific valve types are associated with a higher risk of TPVI-IE requires further investigation. Patient and parent education regarding IE prevention may have a role to play and should be offered to all patients.
The indications for percutaneous pulmonary valve implantation (PPVI) have been extended to include large dysfunctional right ventricular outflow tracts (RVOTs). Prestenting of the RVOT is commonly ...performed before PPVI in order to ensure a stable landing zone. The AndraStent XXL (AndraMed GmbH, Reutlingen, Germany), a cobalt-chromium stent with semi-open cell design, has unique mechanical properties in this indication but is no longer available in France.
To assess the efficiency of AndraStent XXL before PPVI.
In this retrospective multicentre cohort study, 86 AndraStents XXL were implanted in 77 patients in 6 centres.
PPVI was indicated mainly for pulmonary regurgitation (75.3%) in native or patched RVOT (88.3%). The stents were manually mounted on balloon catheters and delivered through sheaths using a conventional femoral approach. PPVI was performed successfully in 97.4% of patients after successful prestenting, generally during the same procedure (77.9%). There were no deaths associated with stent implantation, and four patients experienced five complications, mainly stent embolization, including one requiring surgery. Neither stent fracture nor dysfunction were observed in any patient during a mean follow-up of 19.2±8.7months. Stent analysis showed an excellent maximal stent expansion (97.1%) regardless of balloon size. A 22.3%±3.4 stent shortening with a 30mm balloon was observed.
Implantation of large cobalt-chromium AndraStent XXL stents is efficient for prestenting before PPVI.
Les indications de revalvulation pulmonaire percutanée ont été étendues aux larges voies de sortie du ventricule droit. La revalulvation pulmonaire percutanée est généralement précédée d’un prestenting de la voie de sortie du ventricule droit afin d’assurer une zone de largage stable. L’AndraStent XXL (AndraMed GmbH, Reutlingen, Allemagne), une endoprothèse en cobalt et chrome à mailles semi-ouvertes, possède des propriétés mécaniques uniques dans cette indication mais n’est plus disponible en France.
Évaluer l’efficacité de l’AndraStent XXL avant revalvulation pulmonaire percutanée.
Dans cette étude de cohorte rétrospective multicentrique, 86 AndraStents XXL ont été implantés chez 77 patients dans 6 centres.
La fuite pulmonaire etait l’indication principale de revalvulation (75,3 %) principalement dans des voies natives ou patchées (88,3 %). Les endoprothèses ont été sertis manuellement sur des ballons et implantées à travers des gaines en utilisant une approche fémorale conventionnelle. La revalvulation a été réalisée avec succès chez 97,4 % des patients lorsque l’endoprothèse avait été implantée avec succès, généralement au cours de la même procédure (77,9 %). Aucun décès n’a été associé à l’implantation d’une endoprothèse et quatre patients ont présenté cinq complications, principalement une embolisation de l’endoprothèse, dont une nécessitant une intervention chirurgicale. Aucune fracture ni dysfunction de l’endoprothèse n’ont été observés chez aucun patient au cours d’un suivi moyen de 19,2±8,7 mois. L’analyse des endoprothèses a montré une excellente expansion maximale de l’endoprothèse (97,1 %) quelle que soit la taille du ballon. Serti sur un ballon de 30mm, le raccourcissement de l’endoprothèse etait en moyenne de 22,3 %±3,4.
L’implantation d’une endoprothèse en chrome-cobalt AndraStent XXL est efficace pour pour créer une zone de largage avant revalvulation pulmonaire percutanée.
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•Hypertrophic cardiomyopathy (HCM) is rare in children, and sudden cardiac death (SCD) is difficult to predict.•HCM Risk-Kids and PRIMaCY score have been developed to predict 5-year ...risk of arrhythmic events.•We tested these scores in a real-life French paediatric HCM cohort.•Positive predictive values were poor, hence additional factors should be considered.
Hypertrophic cardiomyopathy (HCM) is rare in children, and sudden cardiac death (SCD) is difficult to predict. Two prognostic scores – HCM Risk-Kids and Precision Medicine for Cardiomyopathy (PRIMaCY) – were developed to assess the risk of SCD in the next 5 years in children with HCM.
To test the ability of these scores to predict SCD in children with HCM. Also, to identify factors associated with a severe cardiac rhythmic event (SCRE) (ventricular fibrillation, sustained ventricular tachycardia, heart transplant for rhythmic reasons or SCD).
Retrospective, multicentre, observational study at 10 medical centres in the Nord-Pas-de-Calais region, France.
This study included 72 paediatric patients with HCM during 2009–2019 who were followed for a median (interquartile range IQR) of 8.5 (5.0–16.2) years. Eleven patients (15.3%) presented with SCRE. HCM Risk-Kids was high, with a median (IQR) score of 6.2% (2.1–12.8%; significant threshold≥6.0%) and the PRIMaCY median (IQR) score was 7.1% (2.6–15.0%; significant threshold≥8.3%). The positive predictive value was only 27.1% (95% confidence interval CI 21.5–32.5%) for HCM Risk-Kids (with a threshold of≥6.0%) and 33.2% (95% CI 27.1–38.9%) for the PRIMaCY score (with a threshold of≥8.3%). The negative predictive values were 95.4% (95% CI 92.3–97.7%) and 93.0% (95% CI 89.8–96.2%), respectively. Three of 28 patients with an implantable cardioverter defibrillator (ICD) experienced complications (including inappropriate shocks).
HCM Risk-Kids and the PRIMaCY score have low positive predictive values to predict SCD in paediatric patients. If used alone, they could increase the rate of ICD implantation and thus ICD complications. Therefore, the scores should be used in combination with other data (genetic and magnetic resonance imaging results).
Percutaneous pulmonary valve implantation (PPVI) with Sapien 3 valve is effective to treat right ventricle outflow (RVOT) dysfunction. Issues related to presenting and tricuspid valve injuries were ...reported with the initial implantation method. A modified technique was developed without pre-stenting and with a protective delivery method for valve implantation.
We aimed to compare procedural results in the modified technique group (MTG) to matched patients in a conventional technique group (CTG). All consecutive procedures performed in the MTG over 9months were matched based on RVOT type and size to consecutive procedures performed before.
Twenty-seven patients were included in each group. Size of the Sapien 3 valves were 23mm (n=9), 26mm (n=9), 29mm (n=36). The two groups were similar regarding demographic data, RVOT type, pre-procedure hemodynamics (Table 1). PPVI was performed in a single procedure in all patients of the MTG, whereas six (22.2%) patients of the CTG group had pre-stenting as a first step and valve implantation later (P=0.02). Procedures were successful in all cases. Stent embolization was reported in two patients (7.4%) in the CTG, which were impacted in pulmonary arteries. Instability of a valve was observed in one case (3.7%) in the MTG, stabilized with two stents and an additional valve-in-valve implantation. Hemodynamics results were good in all cases without significant difference among the two groups (Table 2).
Procedure duration and fluoroscopy times were significantly reduced in the MTG (48.1 versus 82.6minutes, P<0.0001; 15.2 versus 29.8minutes, P=0.0002).
During follow-up, no stent fracture or valve dysfunction was noticed in both groups.
PPVI without pre-stenting and with a protective delivery method of the Sapien 3 valve reduces significantly the procedure complexity, procedure duration and irradiation, while maintaining excellent hemodynamics results.
Percutaneous pulmonary valve implantation (PPVI) is effective to treat right ventricle outflow (RVOT) dysfunction. Long term Results and risk factors of valve dysfunction remain little known.
We ...aimed to study long term Results of PPVI. All consecutive patients who had successful PPVI between January 2008 and January 2020 in one single tertiary center were prospectively included. Follow-up was updated in 2020.
Two hundred and twelve patients were included (Melody Valve, n=32 (15.1%); Sapien XT Valve, n=47 (22.2%); Sapien 3 valve, n=133 (62.7%). Median age was 28 years old (range 10-81). Patched native pulmonary artery was the most frequent RVOT type (44.4%). Mean follow-up was 2.8 years (range 3 months to 11.4 years). Pulmonary valve replacement (PVR) was performed in 23 cases (10.8%), related to stenotic degeneration (n=22 cases, 95%) and severe regurgitation (n=1, 4.3%). PVR was performed in 17 Melody valves (53.1%) and 6 Sapien valves (3.3%), with an incidence of 7.6% per patient-year and 1.3% per patient-year respectively (P=0.05). Mean interval between PPVI and PVR was 4.7 years (range 1.1–10.1 year) (Fig. 1). Infective endocarditis (IE) was reported in 13 patients (Melody valve, n=12, 37.5%; Sapien valve, n=1, 0.5%) with an incidence of 5.5% per patient-year and 0.2% per patient-year respectively (P<0.0001) (Fig. 2). In an univariate Cox regression analysis, risk factors of PVR were RV obstruction before PPVI (P=0.04), transpulmonary maximal velocity >2.7m/s after PPVI (P=0.05), tricuspid regurgitation doppler maximal gradient >50mmHg after PPVI (P<0.001), valve diameter <22mm (P<0.003), IE (P<0.0001) and age <25 (P=0.04). Risk factors of IE were Melody valve (P<0.0001), RV obstruction before (P=0.03) or after PPVI (P=0.05), valve diameter <22mm (P<0.03), and younger age (P=0.02).
PPVI is effective to treat RVOT dysfunction. Implanted Valve durability is shorter when residual RV obstruction is observed, in smaller RVOT, and when IE occurs.
Abstract A 50‐year‐old female patient was readmitted with refractory systemic right ventricular failure. The patient underwent a Mustard procedure during childhood for transposition of the great ...arteries. A significant residual ventricular septal defect was present, which represents a major risk factor of death following ventricular assist device. We describe the combination of ventricular assist device implantation preceded by hybrid closure of ventricular septal defect.
L’adaptation à la vie extra-utérine requiert la résorption rapide du liquide pulmonaire, la création de la capacité fonctionnelle résiduelle (FRC), ainsi que l’augmentation du débit pulmonaire. Bien ...que ces mécanismes aient été largement étudiés, leurs cinétiques restent mal connues faute de méthode d’étude non invasive in vivo. L’échec de l’adaptation cardiorespiratoire à la naissance est la principale cause de mortalité et de morbidité de la période néonatale, malgré l’utilisation de stratégie préventive comme la corticothérapie anténatale, et l’amélioration des techniques de réanimation néonatale. Les acides gras polyinsaturés oméga 3 (AGPI n3) que l’on retrouve dans les huiles de poisson ont des effets cardiovasculaires et respiratoires qui pourraient améliorer la transition périnatale. Par ailleurs, leurs propriétés anti-inflammatoires peuvent réduire les conséquences des effets iatrogènes des techniques de réanimation dont la mise en oeuvre est nécessaire en cas de maladaptation cardiorespiratoire à la naissance. Les objectifs de ce travail ont été de : Mettre au point et valider une méthode d’exploration de la cinétique de réabsorption du liquide pulmonaire à la naissance par résonnance magnétique nucléaire Etudier les effets des AGPI n3 sur : l’adaptation ventilatoire à la naissance la circulation pulmonaire périnatale la prévention des lésions pulmonaires induites par une hyperoxie prolongée Les études ont été réalisées sur les modèles expérimentaux de foetus de brebis et de ratons nouveau-nés exposés à une hyperoxie prolongée. Nous montrons que la technique par IRM permet d’évaluer la cinétique de clairance du liquide pulmonaire in vivo en situations physiologique et pathologique. Alors que les AGPI n3 améliorent la circulation pulmonaire, ils n’ont pas d’effet sur la clairance du liquide pulmonaire. Par contre, ils réduisent les lésions pulmonaires induites par l’oxygène. Ces résultats expérimentaux suggèrent que les AGPI n3 pourraient prévenir les échecs d’adaptation cardiorespiratoire à la naissance et ses conséquences à long terme.
Perinatal transition requires clearance of pulmonary fluid, creation of functional respiratory capacity (FRC), and the multiplication of pulmonary blood flow by ten, to ensure gas exchange and blood oxygenation. Physiology of perinatal transition has been broadly studied but the kinetics of its evolution is less known. The aim of our study was to assess the kinetics of the respiratory transition. Besides, we supposed that polyunsaturated fatty-acids omega 3 (n3 PUFA) would improve this transition. To study the kinetic of respiratory transition, we used the MRI in 3 groups of lambs: premature group (Preterm) with a term of 122-124 days, two groups of late preterm lambs (134-136 days), including one group who received antenatal steroids (Late preterm + steroids), and a group without steroids (Late preterm). Surprisingly, we observed a rapid increase of lung fluid in preterm lambs reaching 30% at the end of the experimentation. Creation of FRC was also impaired in this group. In the Late preterm group, clearance of pulmonary fluid was slowed down with a moderate fall of 10%, whereas, in the Late preterm + steroids group, lung fluid dropped by 50% (p<0.05). Similarly, FRC in the Late preterm + steroids group was higher than the other 2 groups (p <0.05). Respiratory functions were closely related to lung fluid content and to FRC. We hypothesized that n3 PUFA would improve perinatal transition using two models: - An acute model: in a chronically instrumented model, catheters were introduced in the jugular and the carotid vessels of lamb fetuses at 122 days of gestation. Lamb fetuses received either Omegaven (n3 PUFA), or Intralipide (n6 PUFA), or isotonique physiological serum (SSI) for 4 days. Respiratory transition was explored by MRI. There was no significant difference between these three groups. Total lung fluid increased by about 20% in Omegaven group and 18% in SSI group (p> 0.05). - A model of Bronchopulmonary dysplasia: pups were exposed to hyperoxia for 10 days, and feeding rats received supplementation by either n3 PUFA (Omacor), n6 PUFA (sunflower oil), or SSI, with control groups that received the same feeding supply but which were exposed to air. Histological studies showed bronchopulmonary dysplasia lesions in the Hyperoxia groups, characterized by decrease of alveolar number, decrease in the number of secondary alveolar septa, and widening of the interstitial space. These lesions were similar in SSI and n6 PUFA groups, whereas n3 PUFA improved these lesions (p <0.05). To assess the effects of n3 PUFA in pulmonary vascular resistances (PVR), we studied their effects on fetal circulation characterized by elevated RVP using a chronically instrumented experimental model of lamb fetus. Lamb fetuses received either n3 PUFA (Omegaven), or n6 PUFA (Intralipid). We then explored the action mechanisms of n3 PUFA: 1) NO pathway using L-Nitro Argenine (LNA), 2) potassium channels pathway using Tetraethylamonium (TEA) and 3) epoxides produced by cytochrome P450 using MS-PPOH. Finally, we investigated the effect of Eicosapentaenoic Acid (EPA) perfusion, or Docosahexaenoic Acid (DHA) perfusion in pulmonary circulation. We showed that, unlike Intralipid, Omegaven induced pulmonary vasodilation, and an increase in pulmonary flow. An identical effect was reproduced by EPA infusion, while DHA did not modify PVR. This response was not modified by LNA. But it was reduced by MS-PPOH, and abolished by TEA. These results indicate that n3 PUFA induced pulmonary vasodilation, mediated by epoxides which act on potassium channels. To conclude, we demonstrated that respiratory transition in preterm lambs even in late preterm ones is altered with the presence of pulmonary edema. n3 PUFA did not improve lung fluid clearance but should be considered in the tretment of pulmonary hypertension, and in the prevention of brochopulmonary dysplasia of the newborns.
Although evidence exists that n-3 polyunsaturated fatty acids may improve the outcome in patients with severe respiratory failure, little is known regarding their pulmonary circulatory effects. This ...question is clinically relevant in respiratory failure associated with pulmonary hypertension, in particular in newborn infants with persistent pulmonary hypertension. The objective of this study was to investigate the effects of n-3 polyunsaturated fatty acids on the fetal pulmonary circulation.
Randomized, placebo-controlled comparative laboratory investigation.
University research facility.
Fifty-two chronically prepared lamb fetuses.
Catheters and ultrasonic flow transducer were placed through a left thoracotomy in the lamb fetus to determine aortic, pulmonary, and left atrial pressures and left pulmonary artery blood flow.
We compared the pulmonary vascular responses to 120 mins of Omegaven (lipid emulsions enriched in n-3 polyunsaturated fatty acids) or Intralipide (lipid emulsions enriched in n-6 polyunsaturated fatty acids) infusion. Then we investigated the effects of Omegaven on the pulmonary circulation after nitric oxide synthase inhibition by L-nitro-arginine, potassium channel blockade by tetraethylammonium, and cytochrome P450 epoxygenase inhibition by (methylsulfonyl)-2-(2-propynyloxy)-benzenehexanamide. Pulmonary artery and aortic pressures as well as blood gases and plasma lactate concentrations did not change during either fat emulsion infusion. Left pulmonary blood flow increased by 30% and pulmonary vascular resistance decreased by 28% during Omegaven infusion, whereas left pulmonary blood flow and pulmonary vascular resistance did not change during Intralipide infusion. This pulmonary vascular response to Omegaven was not altered by l-nitro-arginine. At the opposite, Omegaven induced pulmonary vasodilatation was abolished by tetraethylammonium and markedly attenuated by (methylsulfonyl)-2-(2-propynyloxy)-benzenehexanamide.
Lipid emulsion containing n-3 polyunsaturated fatty acids may induce a potent and sustained vasodilatation in the fetal lung. This pulmonary vasodilator response is mediated through production of vasoactive mediators by cytochrome P450 epoxygenase and through activation of potassium channels.
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