UNASSIGNED: Arrhythmogenic right @entity296 / @entity1141 (ARVC/D) is a heritable @entity296 characterized by fibro-fatty replacement of right ventricular myocardium. Diagnostic criteria, established in 1994 and modified in 2010, are based on predominately adult manifestations of ARVC/D. The goal of this paper is to review a single-center experience with pediatric ARVC/D and propose modifications of current diagnostic criteria to appropriately include pediatric ARVC/D. We identified 16 pediatric cases of ARVC/D from our tertiary care center. @entity1 demographics, presentation, course, genetic testing, and family history were reviewed. Sixteen @entity1 were diagnosed with ARVC/D through the modified diagnostic criteria, genetic testing, and pathology. Five @entity1 had positive family histories. Five @entity1 presented with @entity1167 , and six were found to have @entity1165 . Two @entity1 presented with @entity712 . Six autopsies, six explanted hearts, and three biopsies found massive fibro-fatty infiltration of the right ventricular wall. Six @entity1 underwent heart transplantation, and two have received automatic implantable cardioverter defibrillator. Two @entity1 had identifiable genetic mutations previously noted in the literature. One @entity1 had a novel mutation of a known ARVC/D gene. Many pediatric @entity1 do not meet the current ARVC/D diagnostic criteria, resulting in delays in diagnosis and treatment. The current criteria need further revision to encompass pediatric manifestations of ARVC/D. In our opinion, pathological and clinical findings alone are sufficient for accurate diagnosis of pediatric ARVC/D. Creating modified pediatric criteria would facilitate prompt diagnosis and management of ARVC/D and facilitate structured research with the goal of improving outcomes.

Arrhythmogenic Right XXXX / @entity1141 (ARVC/D): Review of 16 Pediatric Cases and a Proposal of Modified Pediatric Criteria.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'Patient'] @entity296 :: ('MESH:D009202', 'Disease') :: ['cardiomyopathy', 'ventricular cardiomyopathy'] @entity1165 :: ('MESH:D017180', 'Disease') :: ['ventricular tachycardia'] @entity712 :: ('MESH:D006333', 'Disease') :: ['heart failure'] @entity1167 :: ('MESH:D006323', 'Disease') :: ['cardiac arrest'] @entity1141 :: ('MESH:C535662', 'Disease') :: ['dysplasia']
@entity296 :: (MESH:D009202,Disease) :: ['Ventricular Cardiomyopathy']
