Influence of Antegrade Pulmonary Blood Flow on Outcomes of Superior Cavopulmonary Connection.

BACKGROUND: We sought to characterize short- and long-term outcomes after superior cavopulmonary connection (SCPC) in children eligible for inclusion of antegrade pulmonary blood flow (APBF) in the SCPC circuit, exploring whether maintaining APBF was associated with outcomes. METHODS: This was a retrospective cohort study of patients with single-ventricle heart disease and APBF who underwent SCPC at our center between January 1, 2000, and September 30, 2017. Patients were divided into 2 groups: APBF eliminated (APBF-), and APBF maintained (APBF+) at the time of SCPC. RESULTS: Of 149 patients, 108 (72.5%) were in APBF- and 41 (27.5%) were in APBF+. Of those in APBF+, 5 (12.2%) subsequently had APBF eliminated after SCPC. Patients in APBF+ had a higher prevalence of chest tube duration >10 days and underwent more interventions during the post-SCPC hospitalization (1.9% vs 12%; P = .008 for both) but had shorter surgical support times at SCPC (P < .0001). There were no differences in post-SCPC intensive care unit or hospital length of stay. During the study period, 82 patients (76%) in APBF- and 22 patients (54%) in APBF+ underwent Fontan completion. Patients in APBF+ had a greater weight gain from SCPC to Fontan (6.7 [1.8-22] kg vs 8.15 [4.4-20.6] kg; P = .012) and a shorter hospital length of stay after Fontan (9 [4-107] days vs 7.5 [4-14] days; P = .044). CONCLUSIONS: Short-term morbidity associated with maintaining APBF at the time of SCPC is modest, but longer term outcomes suggest potential benefits in those in whom APBF can be successfully maintained.

Cardiac resource utilization in adults at a freestanding children's hospital.

OBJECTIVE: This article aims to give a comprehensive description of cardiac resource utilization in adults at a freestanding children's hospital. DESIGN: Retrospective chart review. SETTING: Large, tertiary, freestanding children's hospital. PATIENTS: Adults (18 years of age and older) seen within the heart center or evaluated by heart center personnel from January 1, 2006 through December 31, 2010. RESULTS: There were 2794 adults that utilized cardiology services during the study period. The mean patient age was 23.5 ± 8.4 years (18-70.5). The 1748 (62.6%) patients had a diagnosis of congenital heart disease. There were a total of 525 catheterization laboratory encounters and 104 cardiac surgical procedures performed on adult patients during the 5-year study period. The in-hospital mortality for all invasive encounters was 0%. The minor adverse event rate for all invasive encounters was 6.7% and 4.8%, respectively. Also, the overall major adverse event rates were 1.9% and 4.8%, respectively. There were 162 cardiology evaluations performed on adults in the emergency room. There were a total of 5489 adult cardiology clinic visits. The most common congenital heart disease diagnoses were: tetralogy of Fallot: 228 (13%), D-transposition of the great arteries: 208 (11.9%), single ventricle physiology: 187 (10.7%), atrial septal defect 128: (7.3%), and ventricular septal defect: 117 (6.7%). Overall, the complexity of congenital heart disease was: simple: 41%, moderate: 37%, and complex: 22%. CONCLUSIONS: A significant and growing number of adult patients are currently being cared for at pediatric institutions. Excellent outcomes have been achieved at these centers with dedicated adult congenital heart disease services, consisting of both cardiologists and other faculty trained in adult medicine. Others with training in adult medicine, whether they be staff or contracted consulting services, are also required to help manage adult comorbidities.

Surgical pulmonary valve replacement: a benchmark for outcomes comparisons.

BACKGROUND: Patients with right heart obstructive lesions develop residual or recurrent right ventricle outflow tract pathology as a result of native or implanted pulmonary valve (PV) dysfunction. Until recently, the standard of care has been surgical placement of a PV or valved right ventricle to pulmonary artery conduit. Catheter-based options are being increasingly applied in patients with PV dysfunction. The purpose of our study was to evaluate outcomes of surgical pulmonary valve/conduit replacement (PVR) at a large pediatric hospital to provide contemporary benchmark data for comparison with developing technologies. METHODS: Retrospective review of patients undergoing PVR not associated with complex concomitant procedures from July 1995 to December 2010 was completed. Inclusion criteria were designed to generally match those applied to patients promoted for catheter-based valve replacement based on age and weight (age≥5 years and weight≥30 kg). RESULTS: There were 148 PVRs with all patients having undergone ≥1 previous interventions (tetralogy of Fallot [53%] and pulmonary atresia [17%]). Surgical indications were PV insufficiency (60%), PV stenosis (26%), and both (13%). Valves used included bioprosthetic (n=108; 73%) and homografts (n=40; 27%). Time-to-extubation, intensive care unit stay, and hospital length of stay were <1 day (interquartile range, 0-1 day), 2 days (interquartile range, 1-2 days), and 5 days (interquartile range, 4-6 days), respectively, with no hospital deaths. Freedom from PV reintervention at 1, 3, and 5 years was 99%, 99%, and 94%, respectively. Multivariable analysis showed age<13 years (P=.003), and smaller valve size (P=.025) were associated with increased risk of valve reintervention. Patient survival at follow-up (mean, 5.0±3.9 years) was 99%. CONCLUSIONS: Surgical PVR is safe with low in-hospital and midterm follow-up mortality and reoperation rates. These outcomes provide a useful benchmark for treatment strategy comparisons.