Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association.

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for discharge, caregiver support and education, healthcare teams and resources, surveillance strategies and practices, national quality improvement efforts, interstage outcomes, and future areas for research. The statement is directed toward pediatric cardiologists, primary care providers, subspecialists, advanced practice providers, nurses, and those caring for infants undergoing staged surgical palliation for single ventricle heart disease.

Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies.

Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period. A crucial focus of care is the comprehensive family training in the use of home surveillance monitoring of oxygen saturation, enteral intake, weight, and the early recognition of "red flag" symptoms indicating potential cardiopulmonary or nutritional decompensation. Beginning with admission to the intensive care unit of the newborn with hypoplastic left heart syndrome, nurses provide critical care and education to prepare the family for interstage home care. This article presents detailed nursing guidelines for educating families on the home care of their medically fragile infant with single-ventricle circulation.

Interstage feeding and weight gain in infants following the Norwood operation: can we change the outcome?

BACKGROUND: Poor weight gain is common in infants after Stage I Norwood operation and can negatively impact outcomes. OBJECTIVES: The purpose of this study was to examine the impact of feeding strategy on interstage weight gain. METHODS: In a multi-centre study, 158 infants discharged following the Norwood operation were enrolled prospectively. Weight and feeding data were obtained at 2-week intervals. Differences between feeding regimens in average daily weight gain and change in weight-for-age z-score between Stage I discharge and Stage II surgery were examined. RESULTS: Discharge feeding regimens were oral only in 52%, oral with tube supplementation in 33%, and by nasogastric/gastrostomy tube only in 15%. There were significant differences in the average daily interstage weight gain among the feeding groups - oral only 25.0 grams per day, oral/tube 21.4 grams per day, and tube only 22.3 grams per day - p = 0.019. Tube-only-fed infants were significantly older at Stage II (p = 0.004) and had a significantly greater change in weight-for-age z-score (p = 0.007). The overall rate of weight gain was 16-32 grams per day, similar to infant norms. The rate of weight gain declined over time, with earlier decline observed for oral- and oral/tube-fed infants (less than 15 grams per day at 5.4 months) in comparison with tube-only-fed infants (less than 15 grams per day at 8.6 months). CONCLUSION: Following Stage I Norwood, infants discharged on oral feeding had better average daily weight gain than infants with tube-assisted feeding. The overall weight gain was within the normal limits in all feeding groups, but the rate of weight gain decreased over time, with an earlier decline in infants fed orally.

Parental knowledge regarding lifelong congenital cardiac care.

OBJECTIVE: To assess parental knowledge regarding lifelong congenital cardiac care (LLCCC). BACKGROUND: National guidelines recommend that nearly 50% of adult survivors with congenital heart disease (CHD) receive LLCCC; the number of adults who receive such care seems far less. Inadequate parental knowledge of LLCCC might contribute to care interruption. METHODS: In this multicenter study, we administered a questionnaire to parents of children with moderate and complex CHD to assess knowledge of LLCCC. RESULTS: A total of 500 parents participated; the median age of their children was 10 years (range: 2-18 years). Most parents (81%) understood that their child would need LLCCC, but only 44% recognized that their child's cardiology care should be guided by an adult congenital heart specialist in adulthood. More than half (59%) of the parents stated that their current cardiology team had never spoken to them about LLCCC, but 96% wished to learn more. Variables associated with parental LLCCC knowledge included previous discussions regarding LLCCC, underlying cardiac surgical diagnosis, and level of parental education. CONCLUSIONS: A substantial number of parents of children with moderate and complex CHD lack knowledge about LLCCC, but almost all of them have a desire to learn more about the care their child will need as an adult.

New approach to interstage care for palliated high-risk patients with congenital heart disease.

OBJECTIVE: Home surveillance monitoring might identify patients at risk for interstage death after stage 1 palliation for hypoplastic left heart syndrome. We sought to identify the effect that a high-risk program might have on interstage mortality and identification of residual/recurrent lesions after neonatal palliative operations. METHODS: Between January 2006 to January 2010, newborns after stage 1 palliation for hypoplastic left heart syndrome or shunt placement were invited to participate in our high-risk program. Patients enrolled in our high-risk program comprise the study group. Patients who had similar operations between January 2002 and December 2005 comprise the control group. Comparisons are made between the 2 groups with respect to interstage mortality and the frequency and timing of interstage admissions requiring medical, catheter, or surgical treatment. RESULTS: Seventy-two patients met the criteria for our high-risk program. Fifty-nine (82%) of 72 patients were enrolled. Among 19 patients with hypoplastic left heart syndrome in our high-risk program, outpatient interstage mortality was zero. Outpatient interstage mortality for the 36 control subjects with hypoplastic left heart syndrome was 6%. Among 40 patients with shunts in the study group, there was 1 outpatient interstage death compared with 4 (6%) deaths in 68 subjects in the control group. Significant residual/recurrent lesions were identified with similar frequency between the 2 groups. However, after shunt operations, these lesions were detected and treated at significantly younger mean ages for patients followed in the high-risk program (P < .005). CONCLUSIONS: Initiation of a high-risk program might decrease interstage mortality after high-risk neonatal palliative operations. Such an approach might contribute to earlier detection of significant residual/recurrent lesions amenable to therapy.

Nursing considerations in the care of patients with pulmonary hypertension.

Pulmonary hypertension is a potentially lethal condition that may be encountered during the entire life span of patients with many forms of congenital or acquired heart disease, pulmonary disorders, and other diseases. Each pulmonary hypertensive patient requires anticipatory interventions geared to prevent severe exacerbations of the pulmonary hypertensive condition, promote pulmonary vasodilation, and optimize ventricular function. Patients with pulmonary hypertension are at higher risk for developing pulmonary hypertensive episodes in the immediate postoperative period after cardiac surgery, as well as during nonsurgical admissions. Nurses are in a critical position to provide anticipatory care to prevent the development of pulmonary hypertensive events. Nurses can be instrumental in optimizing outcomes for patients with pulmonary hypertension by providing immediate care upon the development of a pulmonary hypertension event and by monitoring ongoing responses to adjustments in therapeutic interventions.

Frequency and indications for tracheostomy and gastrostomy after congenital heart surgery.

Patients undergoing congenital heart surgery may occasionally require additional surgical procedures in the form of tracheostomy and gastrostomy. These procedures are often performed in an attempt to diminish hospital morbidity and length of stay. We reviewed the Web-based medical records of all patients undergoing congenital heart surgery at Miami Children's Hospital from February 2002 through August 2007. Patients who were deemed preterm and had undergone closure of a patent ductus arteriosis were eliminated. The records of all other patients were queried for the terms gastrostomy, g-tube, Nissan, fundal plication, tracheostomy, or tracheotomy. Patients' medical records in which these terms appeared in any portion were completely reviewed. There were 1660 congenital heart operations performed in the study period. There were 592 operations performed on patients whose age ranged from 1 month to 1 year and 441 neonatal operations. Mortality was 2%. Median postoperative stay was 8 days (range, 1-191 days), 12 days for neonates (range, 3-142 days), and 19 days for neonates undergoing RACHS-1 category 6 operations (range, 4-142 days). Tracheostomies were performed in four patients (0.2%). Gastrostomies were performed on eight patients (0.4%), representing 0.8% of patients <1 year of age, 1.4% of neonates, and 2.4% of patients undergoing RACHS-1 category 6 operations. The rate of patients undergoing either tracheostomy or gastrostomy after congenital heart surgery at our institution was quite low. Avoidance of either of these two procedures was achieved without increased morbidity or length of stay. The rate at which these procedures need to be performed may reflect the magnitude of the patients' lifetime trauma related to their underlying condition and acute and total surgical experiences.