In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.
The pericardial fluid found in CVC-associated PCE is consistent with the infusate. We speculate that there are several mechanisms, ranging from frank perforation that seals spontaneously to CVC tip adhesion to the myocardium with diffusion into the pericardial space. Routine radiography should be performed, and the CVC tip should be readily identifiable. The CVC tip should remain outside the cardiac silhouette but still within the vena cavae (approximately 1 cm outside the cardiac silhouette in premature infants and 2 cm in term infants). A change in cardiothoracic ratio may be diagnostic of a PCE, and pericardiocentesis is associated with significantly reduced mortality. Increased awareness of this complication may decrease the mortality associated with CVC-related PCE.
This study characterized fetal growth differences among control infants (n= 276) and infants with d-transposition of the great arteries (TGA) (n = 69), tetralogy of Fallot (n = 66), hypoplastic left heart syndrome (n = 51), and coarctation of the aorta (n = 65), thus permitting assessment of competing theories about the relation between these cardiovascular malformations and fetal growth disturbance. Subjects were liveborn singletons without genetic or extra-cardiovascular structural abnormalities sampled from the Baltimore-Washington Infant Study. Multivariate analysis of covariance was performed: birth weight, birth length, newborn head circumference, and two nonlinear functions of these measures were regressed jointly on a diagnostic class variable and covariates. Differences in the vectors of dependent variable means across diagnostic groups were striking (p < 0.0001). Infants with TGA had normal birth weight, but lesser head volume relative to birth weight. Infants with tetralogy of Fallot were smaller in all measured dimensions, but they were shaped normally. Infants with hypoplastic left heart syndrome were smaller in all measured dimensions, and head volume was disproportionately small relative to birth weight. Infants with coarctation of the aorta had lower birth weight, shorter birth length, and greater head volume relative to birth weight. These findings suggest that fetal circulatory abnormalities may predict abnormal patterns of fetal growth.
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