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A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure.
We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114).
A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome.
After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.
Cardiac function is critically dependent on the movement of ions across membranes. Contraction of the heart begins when an action potential depolarizes the plasma membrane of the myocyte, the sarcolemma. Depolarization is caused by sequential inward currents of sodium ions and calcium ions. The slow inward calcium current enters through voltagegated channels in the sarcolemma and serves several purposes. First, this current supplies a small amount of the activator calcium for binding to troponin C. Second, the sarcoplasmic reticulum (SR) takes up a considerable portion of this calcium, where it forms part of the internal store for release in subsequent contractions. Finally, this calcium triggers the release of a large amount of calcium from the SR. The released SR calcium initiates contraction by binding to troponin C, resulting in formation of cross-bridges between the myosin head and actin. Relaxation begins with the removal of calcium from the myofilaments, which is mediated primarily by the SR calcium pump but also by the Na+-Ca2+ exchanger. Removal of calcium from the myofilaments results in the breaking of actin-myosin cross-bridges and return to the resting state.
Important changes in the structure and function of cardiac membranes occur during maturation of the mammalian heart (and probably in all vertebrate hearts). These changes result in significant age-related differences in myocardial function. This chapter discusses recent work that has increased our understanding of developmental changes in cardiac membranes and of the impact of these changes on myocardial performance.
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