Severe aortic root dilatation in infantile Marfan syndrome.

Cardiovascular manifestations of Marfan syndrome are associated with increased mortality, especially in the pediatric population. Early recognition is critical to long-term management. We present two cases of genetically defined "classical" Marfan syndrome presenting with severe infantile aortic root dilatation among siblings and discuss options for therapy.

Optimum position of transthoracic intracardiac line following cardiac surgery.

Objectives: The transthoracic intracardiac line placed in the right atrium provides a convenient access to the central venous system following cardiac surgery. However, it is associated with risks such as migration and bleeding. We conducted a retrospective study to determine whether position of transthoracic line with respect to site of exit from the chest makes a difference in the rate of complications.

Effect of thyroid hormone on cardiac function following orthotopic heart transplantation in piglets.

Studies in adult HT have demonstrated improved cardiac function in the recipient following administration of T3 to the donor. The purpose of this experiment was to assess the effects of T3 on the function of the immature donor heart following HT in a piglet model. A total of 32 piglets were divided into 16 donors and 16 recipients.

Clinical evaluation of a radio-protective cream for the hands of the pediatric interventional cardiologist.

Objectives: The aim of this study was to evaluate the effectiveness of UltraBLOX™ radiation attenuating hand cream during lengthy cardiac catheterization procedures in children.

Background: The hands of interventional cardiologists receive high doses of radiation due to their proximity to the X-ray beam. Radiation attenuating gloves have about a 26% attenuation rate, but reduce dexterity and tactile sensation.

Feasibility and Safety of Unzipping Small Diameter Stents in the Blood Vessels of Piglets.

Objectives: This study sought to determine the feasibility and safety of unzipping small-diameter stents (SDS) in a growing animal model.

Background: SDS implanted to relieve stenosis of blood vessels in infants may result in refractory stenosis as the child grows. If stents can be longitudinally fractured-unzipped-then the target vessel can potentially be redilated to the eventual adult vessel diameter.

Superior Vena Cava Reconstruction for Treatment of Chylothorax Resulting From Thrombosis of Superior Vena Cava in Young Infants.

Background: Thrombosis and occlusion of the superior vena cava (SVC) can cause massive chylothorax resulting in significant morbidity and mortality among young infants. Medical therapy is often unsuccessful. We report a new surgical technique that entails open thrombectomy and reconstruction of the SVC and innominate vein to treat this condition.

Unzipping of small diameter stents: an in vitro study.

Objective: The purpose of this study was to determine if small-diameter stents can be unzipped in vitro.

Background: Small-diameter stents can relieve stenosis in infant blood vessels. As the child grows, refractory stenosis may result.

17Beta-estradiol confers a protective effect against transforming growth factor-beta2-induced cataracts in female but not male lenses.

Transforming growth factor-beta2 (TGF-beta2) induces anterior subcapsular cataracts, with a marked increase in cytoskeletal and extracellular matrix proteins, such as alpha-smooth muscle actin (alphaSMA). It has been shown that 17beta-estradiol (E2) can prevent TGF-beta2-induced cataracts in lenses from ovariectomized female rats. The purpose of the current study was to extend this finding by testing whether E2 can prevent TGF-beta2-induced cataracts and inhibit the induction of alphaSMA gene expression in normal male and normal, non-ovariectomized female rats.