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Andersons pediatric cardiology 1897

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changes between scheduled clinic visits and provide an opportunity for identifying problems before serious deterioration.47 Use of web-based telemedicine has been incorporated into the management of interstage infants at some centers A tablet or device with video display functions provides the potential benefit of real-time observation of physical status without the need for the infant being transported to local medical provider or cardiology clinic site.48,49 High-risk specialty clinics during the interstage period provide care continuity across the phases of inpatient and outpatient care These interstage clinics ideally provide multidisciplinary care through engagement of nutrition, speech therapy, social work, and psychology services in addition to the cardiac care team at weekly or biweekly intervals Proximity to the high-risk interstage clinic may be prohibitive for some patients and thus require closer collaboration between the referring cardiologist and the interstage care team In some circumstances, discharge to a temporary residence in close proximity to the surgical site during the interstage period is preferable due to the remote access to health care from the family's permanent residence Progression Through Stage II Palliation Timing of Stage II Palliation Progression to the cavopulmonary anastomosis at stage II palliation reduces both wall stress on the single systemic ventricle, may reduce atrioventricular valve insufficiency, increases diastolic pressure with improved coronary artery perfusion, and leads to more efficient circulation Prior to institution of home monitoring, timing of stage II palliation was delayed until 6 months of age In the initial series of home-monitored patients, those who breached home monitoring criteria had successful stage II palliation at 3.6 ± 1 months, with similar somatic growth to those patients who did not breach home monitoring criteria and had stage II palliation at 5.6 ± 2.1 months.1 The success of early cavopulmonary anastomosis in these patients deemed at greatest risk for interstage mortality has modified practice at some centers in that stage II palliation is electively performed at 4 months of age or earlier if necessary Shortening the period of risk linked to the inefficient dual-distribution circulation after stage I palliation was postulated to improve survival Data from 31 centers participating in the NPC-QIC registry demonstrated less interstage mortality in centers that performed stage II palliation less than 5 months (5.7 vs 9.9 months), with similar survival to hospital discharge and hospital length of stay following stage II palliation between groups.50 The Congenital Heart Surgeons’ Society identified that optimal timing of stage II palliation differed across patient risk groups Specifically, low- and intermediate-risk patients had similar operative survival when stage II palliation was performed at 4, 6, or 8 months However, stage II palliation at 3 months of age was associated with maximal 2-year survival Younger age at stage II palliation for high-risk patients did not exhibit a similar survival advantage with earlier timing of stage II palliation High-risk patients were those who had moderate-severe right ventricle dysfunction just prior to stage II palliation, required extracorporeal membrane oxygenation after stage I palliation, and had lower weight-for-age z-score at the pre–stage II palliation catheterization.51 Similar findings were noted in the Pediatric Heart Network Single Ventricle Reconstruction evaluation for optimal timing of stage II palliation.52 A strategy that uses inpatient management throughout the initial interstage period for high-risk patients does not mitigate the greater mortality beyond stage II palliation when compared with those patients deemed suitable for home monitoring after stage I palliation (Fig 72.7).3 FIG 72.7 Actuarial survival for home-monitored patients with events, home-monitored patients without events, and interstage inpatients Survival for interstage inpatients is significantly lower than patients discharged with home monitoring Survival was similar for outpatients with and without home-monitored events CPB, Cardiopulmonary bypass; ECMO, extracorporeal membrane oxygenation; S1P, stage 1 palliation; S2P, stage 2 palliation (From 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.) ... oxygenation after stage I palliation, and had lower weight-for-age z-score at the pre–stage II palliation catheterization.51 Similar findings were noted in the Pediatric Heart Network Single Ventricle Reconstruction evaluation for optimal timing of stage II palliation.52 A strategy that uses inpatient management

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    Section 6 Functionally Univentricular Heart

    Progression Through Stage II Palliation

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