Gastric pseudoaneurysm in the setting of Loey’s Dietz Syndrome

Loey’s Dietz syndrome is a disorder of connective tissue caused by a mutation in the genes that encode transforming growth factor (TGF) beta receptor 1 and 2. It is an autosomal dominant disorder similar to Marfan’s syndrome but with a more aggressive clinical course. Patients with Loey’s-Dietz syndrome have progressive dilatation of the aortic root that can lead to aortic dissection and rupture. The location of non-aortic arterial aneurysms may be wide spread but often occur in the head and neck vessels.peer-reviewe

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation

ObjectiveTo develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).DesignUnder the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RANDâ UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an â open comment period.â Final approval of each QM was provided by a vote of the ACC ACPC Council.PatientsPatients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded.ResultsTwelve candidate QMs were generated. Seven metrics passed the RANDâ UCLA process. Four passed the â open comment periodâ and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age.ConclusionsApplication of the RANDâ UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/142334/1/chd12540_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/142334/2/chd12540.pd

Pulmonary Atresia with Intact Ventricular Septum Causing Severe Left Ventricular Outflow Tract Obstruction

We describe an infant with pulmonary atresia with intact ventricular septum (PAIVS) and severe left ventricular outflow tract (LVOT) obstruction secondary to a suprasystemic right ventricle causing leftward displacement of the interventricular septum. Imaging demonstrated an aneurysmal dilation at the base of the proximal main pulmonary artery (MPA) with no forward flow from the right ventricle. During transannular patch and central shunt placement, the communication between the pulmonary artery and the right ventricle was enlarged to ensure adequate decompression. We report this successful palliation and resulting complete elimination of the LVOT obstruction in a very unique presentation of PAIVS in a newborn