Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome.

Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocardiograms at 0, 12, 24, and 36 months read in the core laboratory of 608 trial subjects, aged 6 months to 25 years, who met original Ghent criteria and had an AoR z-score (AoRz) > 3.

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome.

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed.

Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy.

Background: The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects.

Methods And Results: Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area-adjusted aortic root diameter z-score >3.

Echocardiographic methods, quality review, and measurement accuracy in a randomized multicenter clinical trial of Marfan syndrome.

Background: The Pediatric Heart Network is conducting a large international randomized trial to compare aortic root growth and other cardiovascular outcomes in 608 subjects with Marfan syndrome randomized to receive atenolol or losartan for 3 years. The authors report here the echocardiographic methods and baseline echocardiographic characteristics of the randomized subjects, describe the interobserver agreement of aortic measurements, and identify factors influencing agreement.

Methods: Individuals aged 6 months to 25 years who met the original Ghent criteria and had body surface area-adjusted maximum aortic root diameter (ROOTmax) Z scores > 3 were eligible for inclusion.

Tetralogy of Fallot: anatomic variants and their impact on surgical management.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. In this condition, episodic worsening of hypoxemia results from dynamic shifts in physiology, so-called "Tet spells." The relative frequency of this lesion and the risks of exacerbating "Tet spells" make anesthetic management of this patient population challenging.

Decreased exercise performance with age in children with hypoplastic left heart syndrome.

Objective: Children born with hypoplastic left heart syndrome (HLHS) may experience cardiac dysfunction after staged surgery or transplantation, which may worsen with age. We examined the hypothesis that exercise testing can address cardiovascular capacity and suggest interventions to improve quality of life.

Study Design: Children with HLHS > or = 8 years old performed treadmill or bicycle ergometric testing at 4 centers.

Cardiovascular management of marfan syndrome in the young.

Marfan syndrome is an autosomal-dominant disorder of connective tissue resulting from a mutation in the fibrillin gene. Manifestations of the disorder primarily affect the skeletal, cardiovascular, and ocular systems. The phenotypic manifestations of this disorder can be quite variable.

Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome.

Objective: Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age.

Methods: A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004.

Multi-detector row computed tomographic angiography in pediatric heart transplant recipients: Initial observations.

Background: The purpose of this study was to assess the feasibility and limitation of multi-detector row computed tomographic (MDCT) imaging for evaluating coronary arteries in pediatric heart transplant patients.

Methods: Coronary MDCT angiography was performed in eight pediatric heart transplant recipients. The presence of coronary calcification was evaluated.

Novel use of the Amplatzer duct occluder to close perivalvar leak involving a prosthetic mitral valve.

Perivalvar leak following atrioventricular valve replacement can result in heart failure or hemolytic anemia. These patients may be poor surgical candidates given a complex clinical condition. We describe transcatheter closure of a perivalvar leak in a 3-month infant who had undergone left atrioventricular valve replacement with a St.

Transcatheter occlusion of baffle leaks following atrial switch procedures for transposition of the great vessels (d-TGV).

Baffle-related complications following atrial switch procedures for transposition are relatively common. Transcatheter treatment of baffle stenosis has an established role as a therapeutic modality. However, transcatheter device closure of atrial baffles leaks has rarely been reported.