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Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

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Nội dung chi tiết: Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2AHi GhikirciiM Hct»p

|. hut patients with these hearts utilize a disproportionate amount of healthcare resources because of the complexity of management and the need for r Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

epeated interventions and lifelong care [2], Optimal management depends on early recognition and careful observation and planning. Treatment principle

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

s include meticulous protection of the pulmonary vasculature, ongoing surveillance for and rapid treatment of systemic outflow obstruction, and mainte

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2tegies, the long-term outlook for these patients is guarded [3], Palliation of patients with functionally one ventricle is likely to be effective for

only two to four decades |4]. Most, perhaps all. such patients will eventually require an alternative management strategy such as transplantation or m Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

echanical support.Many hearts in this category have only one ventricular sinus or body, anatomically as well as functionally 15.61. The other cham ■ b

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

er usually present in the ventricular mass is an infundibulum or outlet chamber. Ihese include double-inlet left ventricle (D1LV), most double inlet r

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2 but one or both are unsuitable to func tion independently [7]. Most of these arc discussed in Ollier chapters and will only be listed here. Alllwugh

not all cases of these detects have functionally one ventricle, many do and must be identified as soon as possible to maximize outcomes. Included are Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

variants of hypoplastic left heart syndrome (Chapter 20), hypoplastic right heart syndrome (mostly pulmonary atresia with intact ventricular septum) (

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

Chapter 17). straddling mitral valve (Chapter 11). straddling tricuspid valve (Chapter 13). unbalanced common atrioventricular (AV) canal (Chapter 15)

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2(chapter 28), and superiorinferior ventricles (SIV) and criss cross heart.EtiologyThe genetic, and/or environmental causes of these detects are poorly

understood. Most cases appear to he sporadic, although familial occurrences have been reported [8—10]. There arc a few case reports of syndromic asso Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

ciation of TA or other functionally single ventricle hearts [11-18]. The recurrence risk among first degree relatives appears to be in the range assoc

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

iated with polygenic inheritance (2-5%) 119-22].Embryologic developmentConcepts about development of most congenital heart defects are speculative bec

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2known about normal human development and abnormal development in animal models. The following brief description of normal development is provided for

comparison with the proposed abnormal devel opment in subsequent sections.In early looping the heart tube is rather uniform with no clear demarcation Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

of chambers. Cells from the second heart field are added to both ends of the heart tube as it elongates and loops [23], The dorsal niescxardhun, which

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

initially joins the heart lube throughout its length to prepharyngcal mesoderm, degenerates in its mid portion [24] allowing the elongating heart tub

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2e looping heart tube [26], flic AV canal becomes apparent as a constriction between the common atrial chamber and the developing left ventricle and th

e inter ventricular foramen as a constriction between the developingEikữcardiữỊraỹhj in Pediaưic and CenỊcnital I leaf t Diteate: From Fclui tứ Adult. Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

Seccod Edition. Edited by Wymia w. Iji. lac 1- Mertcro. Meryl s. Coben led ĩùl Gevx © 2016 lohn Wiley & Sons. Ud. Published 3>I6 by tohn Wiley & Sone

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

Lid.Companion website: wmchi echocom511512 PartV Miscellaneous Cardiovascular Lesionsventricles. The AV canal is exclusively aligned with the develop

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2eart chambers continue to enlarge, the right Side of the AV canal and the right atrium grow faster than the left, allowing the AV canal to expand abov

e the right ventricle and below the right atrium, creating a right ventricular inflow and establishing alignment of the right atrium with the right ve Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

ntricle through the right side of the AV canal |27|. Swellings or cushions develop in the AV canal as well as the outflow (28). At the same time, sept

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

ation of the common atrium begins with downward growth of septum primum or the primary atrial septum from the superior wall and invasion of the dorsal

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2 atrium, finally fusing With the two main AV cushions. At that point the AV cushions fuse to each other as well, separating the AV canal into right an

d left halves, aligning the right atrium and ventricle and the left atrium and ventricle. respectively. Before completion of atrial septation, the sep Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

tum primum, near its origin from the superior wall, breaks down forming ostium secundum and allowing continued communication between the right and lef

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

t atria. The fused AV cushions then become draped over the muscular inflow ventricular septum that has developed on their ventricular side 130J. Meanw

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2y artery |31J. As this occurs the proximal outflow undergoes counterclockwise rotation, as viewed from the ventricles. so that the aorta which is ante

rior distally comes to lie rightward and posterior proximally, and the pulmonary artery which is posterior distally moves anteriorly and leftward prox Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

imally |321. The outflow septum, which developed by fusion and mus-cularization of the proximal parts of the outflow cushions, then inserts into the l

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

imbs of the interventricular foramen, aligning the aorta with the left ventricle and the pulmonary artery with the right ventricle, and closing the in

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2 myocardium by apoptosis of cardiomyocytes |30|. The semilunar valves develop from the outflow cushions by excavation and thinning that also involves

apoptosis probably initiated by neural crest cells 134).Double-inlet left ventricleDILV includes hearts in which both AV valves are aligned With and c Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

onnected to one ventricular chamber of left ventricular morphology (6) (Figures 27.1-27.6-. Videos 27.1-27.5).Embryologic developmentDILV is easily en

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

visioned as an arrest of development at the stage where the AV canal is completely aligned with the developing leftventricle. Most often DILV is assoc

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2ted with failure of development and growth of the right ventricular sinus. As the AV canal expands toward the inner curvature of the heart tube it bec

omes aligned with the right atrium. However. the expanding edge does not cross the foramen between the left ventricle and outflow as it does in the no Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

rmal heart. It is unclear if confinement of the AV canal to the left ventricle is primary and failure of RV development secondary, or the converse. In

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

either case, atrial septation appears to proceed correctly with the septum primum and dorsal mesenchymal protrusion fusing with and inducing central

PARTVMiscellaneous Cardiovascular LesionsCHAPTER 27Hearts with Functionally One VentricleStephen p. SandersIX-|urlHKiilxIII>rxl( urJui Snryx'T- BittfA

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2underlying muscular ventricular septum over which to drape themselves. The infundibulum or outlet chamber derives from the outflow portion or ascendin

g limb of the heart tube and maintains its primitive connection With the left ventricle, the outflow foramen, which would have been the interventricul Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

ar foramen had the right ventricle developed. In fact, the size of the outlet chain -her is somewhat variable and in some cases it appears that a smal

Ebook Echocardiography in pediatric and congenital heart disease from fetus to adult (2nd edition): Part 2

l portion of right ventricular sinus might be present, especially in hearts where the outlet chamber extends inferiorly toward the diaphragmatic heart

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