Andersons pediatric cardiology 1898

“Interstage II”—Beyond the Superior Cavopulmonary Connection In much of North America, concepts and practices specific to interstage management begun by Ghanayem and colleagues at the Children's Hospital of Wisconsin have been widely adopted by the NPC-QIC, a learning collaborative with contributions from more than 60 pediatric centers Improvements accomplished to date have included a significant reduction in interstage mortality for infants awaiting stage II palliation Aggregate interstage mortality decreased from 9.5% to 5.1%, a relative reduction of 46% In addition, there was a significant reduction in the number of infants experiencing interstage growth failure, with a reduction from 18.6% to 13.3%, a relative reduction of 28.4%.44 Family response to their child reaching stage II palliation may vary from excitement due to their child surviving the high-risk interstage period to anxiety related to another cardiac surgery As recovery from stage II palliation progresses and discharge planning begins, families face several care delivery issues First, oversight by the familiar and easily accessible protective interstage care team53 ceases, and comprehensive care is now transferred to the primary/referring cardiologist Alterations in chronic management are inevitable due to changes in circulatory status but may result in confusion and mistrust of the new care team Daily assessments of weights and oxygen saturation is no longer necessary; thus the constant “reassurance” previously provided through daily data trending has ended This is especially important for those infants with noncardiac comorbidities and feeding challenges Despite the aforementioned challenges, the cessation of home monitoring illicits various parent responses, ranging from the realization of how stressful the past months have been, to relief that their child is in a better state, to acknowledging the great amount of energy needed to adjust to their new normal It is currently becoming apparent that the gains realized by protocol-driven monitoring, care, and surveillance can and should continue during “interstage II”—the period in between the superior cavopulmonary connection and total cavopulmonary connection (Fontan) Although the risk of sudden circulatory collapse is reduced after stage II palliation, living with a functionally univentricular heart confers ongoing risk during the second interstage period between the superior cavopulmonary connection and completion Fontan, with a single center report noting 12% mortality during the second interstage period.54 The NPC-QIC launched phase II for interstage care in 2016 with the goal of expanding the scope for ongoing quality improvement and research for infants with hypoplastic left heart syndrome from the time of diagnosis to the first birthday The aim of phase II is to improve outcomes with the focused areas of fetal and perinatal care, surgical and critical care, interstage care, neurodevelopment, nutrition and growth, transparency, and patient and family support Designated working groups in each of these areas will continue to inform and collaboratively improve outcomes and quality of care for children with a functionally univentricular heart during each interstage period At the time of this writing, there is considerable variation in the investigations done during interstage II (e.g., echocardiography, cardiac catheterization, magnetic resonance imaging, laboratory work), as well as the timing of the eventual Fontan operation Patients with a functionally univentricular heart are likely to benefit from the methods learned during interstage I, including less variability in management and consistent surveillance practices Current efforts will redefine “interstage monitoring” to include comprehensive care across the care continuum and, importantly, enrich the lives of children with a functionally univentricular heart and their families References Ghanayem NS, Cava JR, Jaquiss RD, Tweddell JS Home monitoring of infants after stage one palliation for hypoplastic left heart syndrome Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004;7:32–38 Simsic JM, Bradley SM, Stroud MR, Atz AM Risk factors for interstage death after the Norwood procedure Pediatr Cardiol 2005;26(4):400–403 Rudd NA, Frommelt MA, Tweddell JS, et al Improving interstage survival after Norwood operation: outcomes from 10 years of home monitoring J Thorac Cardiovasc Surg 2014;148(4):1540–1547 Siehr SL, Norris JK, Bushnell JA, et al Home monitoring program reduces interstage mortality after the modified Norwood procedure J Thorac Cardiovasc Surg 2014;147(2):718–23 e1 Hansen JH, Furck AK, Petko C, et al Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome Eur J Cardiothorac Surg 2012;41(5):1013–1018 Castellanos DA, Herrington C, Adler S, et al