Single-stage arterial switch operation conversion with dextro-transposition of the great arteries and pulmonary arterial hypertension due to baffle leak after a Senning procedure.

Unlabelled: Patients who have undergone an atrial switch operation for dextro-transposition of the great arteries (dTGA) sometimes suffer from right ventricular dysfunction, tricuspid regurgitation, arrhythmias, or baffle leaks. We report the first case of single-stage arterial switch operation conversion in an adult patient with dTGA and pulmonary arterial hypertension (PAH) due to a baffle leak after a Senning procedure. Perioperative mechanical circulatory support was required for 3 weeks.

Novel electrocardiographic criteria for short QT syndrome in children and adolescents.

Aims: Although shortening of the corrected QT interval (QTc) is a key finding in the diagnosis of short QT syndrome (SQTS), there may be overlap of the QTc between SQTS patients and normal subjects in childhood and adolescence. We aimed to investigate electrocardiographic findings for differentiation of SQTS patients.

Methods And Results: The SQTS group comprised 34 SQTS patients <20 years old, including 9 from our institutions and 25 from previous reports.

A Nonsense SMAD3 Mutation in a Girl with Familial Thoracic Aortic Aneurysm and Dissection without Joint Abnormality.

Introduction: Thoracic aortic aneurysms and dissections (TAAD) are rare in children and often associated with underlying genetic disorders accompanied with other systemic manifestations, including connective or osteo-articular tissue diseases.

Case Presentation: We report the case of a 10-year-old girl with a novel nonsense SMAD3 mutation, p.Glu102X, who presented with familial TAAD without any signs of osteoarthritis.