Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey.

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

Application of pediatric appropriate use criteria for initial outpatient evaluation of syncope.

BACKGROUND: Syncope is a common reason for outpatient transthoracic echocardiography (TTE). We studied the applicability of pediatric appropriate use criteria (AUC) on initial outpatient evaluation of children (≤18 years) with syncope. METHODS: Data were obtained before (Phase I, April-September 2014) and after (Phase II, January-April 2015) the release of the AUC document from six participating pediatric cardiology centers. Site investigators determined the indication for TTE and assigned appropriateness rating based on the AUC document: Appropriate (A), May Be Appropriate (M), Rarely Appropriate (R), or "unclassifiable" (U) if it did not fit any scenario in the AUC document. RESULTS: Of the total 4562 TTEs, 310 (6.8%) were performed for syncope: 174/2655 (6.6%) Phase I and 136/1907 (7.1%) Phase II, P=.44. Overall, 168 (50.5%) were for indications rated A, 63 (18.9%) for M, 79 (23.7%) for R, and 23 (6.9%) for U. Release of AUC did not change the appropriateness of TTEs [A=51.6% vs 49.0%, P=.63, R=20.2% vs 28.3%, P=.09]. Overall syncope-related R indications formed 15.7% of R indications for all the echocardiograms performed in the entire Pediatric Appropriate Use (PAUSE) study (11.9% Phase I and 22.4% Phase II, P=.002). TTEs were normal in majority of the patients except 7 that had incidental findings. CONCLUSIONS: In conclusion, syncope is a common reason for indications rated R and release of the AUC document did not improve appropriate utilization of TTE in syncope. Targeted educational interventions are needed to reduce unnecessary TTEs in children with syncope.

Syncope Best Practices: A Syncope Clinical Practice Guideline to Improve Quality.

OBJECTIVE: To determine whether implementation of a standardized clinical practice guideline (CPG) for the evaluation of syncope would decrease practice variability and resource utilization. DESIGN: A retrospective review of medical records of patients presenting to our practice for outpatient evaluation of syncope before and after implementation of the CPG. The guideline included elements of history, physical exam, electrocardiogram, and "red flags" for further testing. SETTING: Outpatient pediatric cardiology offices of a large pediatric cardiology practice. PATIENTS: All new patients between 3 and 21 years old, who presented to cardiology clinic with a chief complaint of syncope. INTERVENTIONS: The CPG for the evaluation of pediatric syncope was presented to the providers. OUTCOME MEASURES: Resource utilization was determined by the tests ordered by individual physicians before and after initiation of the CPG. Patient final diagnoses were recorded and the medical records were subsequently reviewed to determine if any patients, who presented again to the system, were ultimately diagnosed with cardiac disease. RESULTS: Of the 1496 patients with an initial visit for syncope, there was no significant difference in the diagnosis of cardiac disease before or after initiation of the CPG: (0.6% vs. 0.4%, P = .55). Electrocardiography provides the highest yield in the evaluation of pediatric syncope. Despite high compliance (86.9%), there were no overall changes in costs ($346.31 vs. $348.53, P = .85) or in resource utilization. There was, however, a decrease in the variability of ordering of echocardiograms among physicians, particularly among those at the extremes of utilization. CONCLUSIONS: Although the CPG did not decrease already low costs, it did decrease the wide variability in echo utilization. Evaluation beyond detailed history, physical exam, and electrocardiography provides no additional benefit in the evaluations of pediatric patients presenting with syncope.

Postoperative cerebral oxygenation in hypoplastic left heart syndrome after the Norwood procedure.

BACKGROUND: Cerebral near-infrared spectroscopy (NIRS) is being used with increasing frequency in the care of pediatric patients after surgery for congenital heart disease. Near-infrared spectroscopy provides a means of evaluating regional cerebral oxygen saturation (cSaO(2)) noninvasively, with correlations to cardiac output and central venous saturation. Prior studies have demonstrated that systemic venous saturation can predict outcome after the Norwood procedure. With this in mind, we sought to determine whether regional cSaO(2) by NIRS technology could predict risk of adverse outcome after the Norwood procedure. METHODS: We reviewed the first 48 hours of postoperative hemodynamic data on 50 patients with hypoplastic left heart syndrome at our institution who underwent the Norwood procedure. Cerebral oxygen saturation data within 48 hours of surgery were analyzed for association with subsequent adverse outcome, which was defined as intensive care unit length of stay greater than 30 days, need for extracorporeal membrane oxygenation, or hospital death after 48 hours. RESULTS: There were 18 adverse events among the 50 subjects. The mean cSaO(2) for the entire cohort at 1 hour, 4 hours, and 48 hours after surgery was 51% +/- 7.5%, 50% +/- 9.4%, and 59% +/- 8.1%, respectively. Mean cSaO(2) for the first 48 postoperative hours of less than 56% was a risk factor for subsequent adverse outcome (odds ratio 11.9, 95% confidence interval: 2.5 to 55.8). Mean cerebral NIRs of less than 56% over the first 48 hours after surgery yielded a sensitivity of 75.0% and a specificity of 79.4% to predict those at risk for subsequent adverse events. CONCLUSIONS: Low regional cerebral oxygen saturation by NIRS in the first 48 hours after the Norwood procedure has a strong association with subsequent adverse outcome. Monitoring of cerebral saturation can serve as a valuable monitoring tool and can identify patients at risk for poor outcome.

Near infrared spectroscopy changes with pericardial tamponade.

OBJECTIVE: Report on the use of near infrared spectroscopy (NIRS) for the recognition of pericardial tamponade after neonatal congenital heart surgery. DESIGN: Case report. SETTING: Pediatric cardiac intensive care unit. PATIENT: Seven-day-old term infant with double inlet left ventricle, hypoplastic aortic arch, and ascending aorta, postoperative day (POD) 3 from the Norwood procedure. INTERVENTION: After an initial stable early postoperative course, the patient's cerebral saturation decreased from the 50s on POD 2, to the 40s in the early morning hours of POD 3. By 9 am on POD 3, the cerebral saturation decreased further to the mid 30s. No change in pulsoximetry, arterial blood gas acid base balance, or blood pressure occurred. An echocardiogram was performed due to the progressive decline in cerebral saturation values. A 10-mm circumferential pericardial effusion was diagnosed. The effusion was drained without incident. Cerebral saturation returned to the 50s, the patient had no further complications and was discharged to home on POD 15. CONCLUSION: Pericardial tamponade is a well-known complication of open heart surgery in children, and early recognition of this can be difficult. This case report demonstrated cerebral saturation to be decreased as impending pericardial tamponade developed. Along with the commonly used markers of tamponade, near infrared spectroscopy measurement of cerebral saturation may also be of benefit in recognizing this life-threatening condition.