Association of Acute Anti-inflammatory Treatment With Medium-term Outcomes for Coronary Artery Aneurysms in Kawasaki Disease.

Background: The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown.

Methods: Using data from the International KD Registry in patients with ≥ medium CAA we evaluate associations of treatment with outcomes and major adverse cardiac events (MACE).

Results: Medium or large CAA was present in 527 (32%) patients.

A direct comparison of next generation sequencing enrichment methods using an aortopathy gene panel- clinical diagnostics perspective.

Background: Aortopathies are a group of disorders characterized by aneurysms, dilation, and tortuosity of the aorta. Because of the phenotypic overlap and genetic heterogeneity of diseases featuring aortopathy, molecular testing is often required for timely and correct diagnosis of affected individuals. In this setting next generation sequencing (NGS) offers several advantages over traditional molecular techniques.

Characterization of large genomic deletions in the FBN1 gene using multiplex ligation-dependent probe amplification.

Background: Connective tissue diseases characterized by aortic aneurysm, such as Marfan syndrome, Loeys-Dietz syndrome and Ehlers Danlos syndrome type IV are heterogeneous and despite overlapping phenotypes, the natural history, clinical manifestations and interventional course for each diagnosis can be quite unique. The majority of mutations involved in the etiology of these disorders are missense and nonsense mutations. However, large deletions and duplications undetected by sequencing may be implicated in their pathogenesis, and may explain the apparent lack of genotype-phenotype correlation in a subset of patients.

Pediatric emergency department use by adults with chronic pediatric disorders.

Objective: To describe pediatric emergency department use by adults with chronic pediatric disorders, known as transition patients.

Design: Retrospective descriptive study.

Setting: The pediatric emergency department of a tertiary care pediatric hospital during calendar year 2005.

Stentless porcine valves in the right ventricular outflow tract: improved durability?

Objective: Stentless porcine valves are commonly used for aortic valve replacement in adults, yet their long-term performance in the right ventricular (RV) outflow tract is unknown. We evaluated intermediate-term performance of stentless porcine valves in the RV outflow tract in 150 children and adults over a 10-year period.

Methods: We retrospectively reviewed data on all patients undergoing placement of a pulmonary valve or RV-PA conduit with a stentless porcine prosthesis (>/=19 mm) from 1998 to 2008.

Clinical stress testing in the pediatric age group: a statement from the American Heart Association Council on Cardiovascular Disease in the Young, Committee on Atherosclerosis, Hypertension, and Obesity in Youth.

This statement is an updated report of the American Heart Association's previous publications on exercise in children. In this statement, exercise laboratory requirements for environment, equipment, staffing, and procedures are presented. Indications and contraindications to stress testing are discussed, as are types of testing protocols and the use of pharmacological stress protocols.