RÉSUMÉ
This meta-analysis aims to comprehensively assess the efficacy and safety of both patent ductus arteriosus (PDA) stents and surgical aortopulmonary shunt (APS) as the initial palliative measures in babies relying on ductal-dependent circulation. This review is essential to compare the outcome of relatively newer catheter-based PDA stent procedure with a surgical APS. By synthesizing existing literature, this review aims to provide insights to inform clinical practice and enhance patient care in this challenging clinical scenario. The methodology involved an extensive search of PubMed and Embase databases using specific keywords and terminology related to mortality, procedural outcomes, and postprocedural complications following PDA stent and APS in cyanotic congenital heart defects (CHD) patients. Six retrospective observational studies met the criteria, with 757 patients included. The analysis showed comparable mortality rates between PDA stents and APS. However, PDA stents were associated with decreased risks of mechanical circulatory support, postprocedural complications, and shorter hospital stays, mechanical ventilation, and intensive care unit stays compared to APS. Notably, patients with pulmonary atresia scheduled for biventricular surgery were more prevalent among those receiving PDA stents. In conclusion, while the risk of mortality is similar between PDA stents and APS, PDA stents offer advantages such as shorter hospital stays and reduced complications. Patient characteristics also vary, with a higher prevalence of intact ventricular septum among those receiving PDA stents.
RÉSUMÉ
ustification: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However,these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required whenrecommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and mayhave co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National ConsensusMeeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of MedicalSciences, New Delhi. The meeting was supported by Children’s HeartLink, a non-governmental organization based in Minnesota, USA.Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heartdiseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.Recommendations: Evidence based recommendations are provided for indications and timing of intervention in common congenitalheart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductusarteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heartdiseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebsteinanomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.
RÉSUMÉ
Introduction A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. Process Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. Objectives The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. Recommendations Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.
RÉSUMÉ
We describe case report of a 45 days old male baby with neonatal lupus erythematosus, who presented with 3rd degree congenital heart block and depigmented skin lesions on face and upper part of body. Diagnosis of the baby was confirmed by anti nuclear levels and skin biopsy.
Sujet(s)
Anticorps antinucléaires/analyse , Bloc cardiaque/congénital , Humains , Nourrisson , Lupus érythémateux cutané/complications , Mâle , Pigmentation de la peau/immunologieRÉSUMÉ
Congenital afibrinogenemia/hypofibrinogenemia is an extremely rare coagulation disorder. We describe a case of congenital hypofibrinogenemia in a 6-year female child, who presented with recurrent ecchymotic spots with no frank bleeding.