Mechanical Support of the Failing Fontan Circulation. 2021

Christopher E Mascio
Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address: mascioc1@email.chop.edu.

The Fontan is the final common pathway for most single ventricle lesions. It is not a durable circulation and many Fontan patients eventually present for advanced heart failure therapies including mechanical circulatory support. While there have been advancements in available durable devices, many pediatric patients have size, anatomical, and physiologic constraints that make placement of adult ventricular assist devices challenging. And, Fontan failure comes in different varieties, including failure with preserved systolic function. This subset of patients requires either a subpulmonary assist device or a total artificial heart. Unique operative maneuvers, including atrial cannulation and atrioventricular valve manipulation have allowed many failing Fontan patients to be bridged to transplant after recovery of end-organ dysfunction and improved nutrition. The Advanced Cardiac Therapies Improving Outcomes Network is a multicenter learning network of pediatric hospitals implanting, managing, and studying ventricular assist devices in children and adults with congenital heart disease. This network is advancing the knowledge of outcomes and approaches to mechanical circulatory support in those with congenital heart disease, including Fontans. The congenital heart disease community continues to work toward and hope for a smaller durable device that will help this complex cohort of patients.

UI MeSH Term Description Entries
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006330 Heart Defects, Congenital Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life. Congenital Heart Disease,Heart Abnormalities,Abnormality, Heart,Congenital Heart Defect,Congenital Heart Defects,Defects, Congenital Heart,Heart Defect, Congenital,Heart, Malformation Of,Congenital Heart Diseases,Defect, Congenital Heart,Disease, Congenital Heart,Heart Abnormality,Heart Disease, Congenital,Malformation Of Heart,Malformation Of Hearts
D006333 Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION. Cardiac Failure,Heart Decompensation,Congestive Heart Failure,Heart Failure, Congestive,Heart Failure, Left-Sided,Heart Failure, Right-Sided,Left-Sided Heart Failure,Myocardial Failure,Right-Sided Heart Failure,Decompensation, Heart,Heart Failure, Left Sided,Heart Failure, Right Sided,Left Sided Heart Failure,Right Sided Heart Failure
D006353 Heart-Assist Devices Small pumps, often implantable, designed for temporarily assisting the heart, usually the LEFT VENTRICLE, to pump blood. They consist of a pumping chamber and a power source, which may be partially or totally external to the body and activated by electromagnetic motors. Artificial Ventricle,Heart Assist Device,Heart Ventricle, Artificial,Pumps, Heart-Assist,Vascular-Assist Device,Vascular-Assist Devices,Ventricle-Assist Device,Ventricular Assist Device,Artificial Heart Ventricle,Artificial Heart Ventricles,Artificial Ventricles,Assist Device, Heart,Assist Device, Ventricular,Assist Devices, Heart,Assist Devices, Ventricular,Device, Heart Assist,Device, Heart-Assist,Device, Vascular-Assist,Device, Ventricle-Assist,Device, Ventricular Assist,Devices, Heart Assist,Devices, Heart-Assist,Devices, Vascular-Assist,Devices, Ventricle-Assist,Devices, Ventricular Assist,Heart Assist Devices,Heart Ventricles, Artificial,Heart-Assist Device,Heart-Assist Pump,Heart-Assist Pumps,Pump, Heart-Assist,Pumps, Heart Assist,Vascular Assist Device,Vascular Assist Devices,Ventricle Assist Device,Ventricle, Artificial,Ventricle, Artificial Heart,Ventricle-Assist Devices,Ventricles, Artificial,Ventricles, Artificial Heart,Ventricular Assist Devices
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D018729 Fontan Procedure A procedure in which total right atrial or total caval blood flow is channeled directly into the pulmonary artery or into a small right ventricle that serves only as a conduit. The principal congenital malformations for which this operation is useful are TRICUSPID ATRESIA and single ventricle with pulmonary stenosis. Fontan Circuit,Fontan Circulation,Fontan Operation,Fontan Palliation,Hemi-Fontan Procedure,Bidirectional Cavopulmonary Shunt,Bidirectional Glenn Procedure,Bidirectional Glenn Shunt,Norwood Procedure, Stage 2,Norwood Procedure, Stage 3,Norwood Procedure, Stage II,Norwood Procedure, Stage III,Stage 2 Norwood Procedure,Stage 3 Norwood Procedure,Stage II Norwood Procedure,Stage III Norwood Procedure,Bidirectional Cavopulmonary Shunts,Bidirectional Glenn Procedures,Bidirectional Glenn Shunts,Cavopulmonary Shunt, Bidirectional,Circuit, Fontan,Circulation, Fontan,Glenn Procedure, Bidirectional,Glenn Shunt, Bidirectional,Hemi Fontan Procedure,Operation, Fontan,Palliation, Fontan,Procedure, Bidirectional Glenn,Procedure, Fontan,Procedure, Hemi-Fontan,Shunt, Bidirectional Cavopulmonary

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