Relationship between patient understanding and timeliness of penicillin prophylaxis in rheumatic heart disease prevention programmes in American Samoa.

AIM: While mostly eradicated in developed nations, rheumatic heart disease (RHD) is still the leading cause of preventable cardiovascular disease in children. RHD and its antecedent acute rheumatic fever remain endemic in many low to middle income countries, as well as in vulnerable communities in wealthy ones. Evidence-based interventions are particularly important in resource-poor settings. We sought to determine if efforts directed at patient and family education impact degree of participation in community-based prevention measures, and with short-term disease progression. METHODS: We performed an observational, cross-sectional study of children with RHD aged 5-19 years, along with their parents, in American Samoa. A survey was administered in November 2016 to assess patient and parent knowledge of RHD. Scores were compared to percent timeliness of penicillin prophylaxis via chart review. RESULTS: We collected a total of 70 surveys of child-parent dyads with a patient mean age of 14.28 years ±2.71. An increased knowledge score was predictive of increased penicillin compliance for both children (12.70% increase in compliance per 1-unit increase in score (P = 0.0004)) and parents (10.10% increase in compliance per 1-unit increase in score (P = 0.0012)). CONCLUSIONS: A clear relationship exists between patient and parent knowledge of RHD and timeliness of penicillin prophylaxis doses. This study was the first to link patient understanding of RHD to engagement with preventative measures.

Surgical Quality Predicts Length of Stay in Patients with Congenital Heart Disease.

Historically, the primary marker of quality for congenital cardiac surgery has been postoperative mortality. The purpose of this study was to determine whether additional markers (10 surgical metrics) independently predict length of stay (LOS), thereby providing specific targets for quality improvement. Ten metrics (unplanned ECMO, unplanned cardiac catheterization, revision of primary repair, delayed closure, mediastinitis, reexploration for bleeding, complete heart block, vocal cord paralysis, diaphragm paralysis, and change in preoperative diagnosis) were defined in 2008 and subsequently collected from 1024 consecutive index congenital cardiac cases, yielding 990 cases. Four patient characteristics and 22 case characteristics were used for risk adjustment. Univariate and multivariable analyses were used to determine independent associations between each metric and postoperative LOS. Increased LOS was independently associated with revision of the primary repair (p = 0.014), postoperative complete heart block requiring a permanent pacemaker (p = 0.001), diaphragm paralysis requiring plication (p < 0.001), and unplanned postoperative cardiac catheterization (p < 0.001). Compared with patients without each metric, LOS was 1.6 (95 % CI 1.1-2.2, p = 0.014), 1.7 (95 % CI 1.2-2.3, p = 0.001), 1.8 (95 % CI 1.4-2.3, p < 0.001), and 2.0 (95 % CI 1.7-2.4, p < 0.001) times as long, respectively. These effects equated to an additional 4.5-7.8 days in hospital, depending on the metric. The other 6 metrics were not independently associated with increased LOS. The quality of surgery during repair of congenital heart disease affects outcomes. Reducing the incidence of these 4 specific surgical metrics may significantly decrease LOS in this population.

A defined management strategy improves early outcomes after the Fontan procedure: the PORTLAND protocol.

BACKGROUND: Patients undergoing the Fontan procedure may have extended hospital stay due to various postoperative factors including prolonged chest tube drainage. Our aim was to determine the efficacy of our Fontan management protocol in reducing chest tube drainage and length of stay. METHODS: Patients who underwent a Fontan procedure at our institution from June 2008 to September 2013 were analyzed (n = 42). We currently manage our patients according to the PORTLAND protocol: Peripheral vasodilation, Oxygen, Restriction of fluids, Technique of surgery, Low-fat diet, Anticoagulation (including antithrombin III management), No ventilator, and Diuretics. Group A (n = 28) had surgery prior to initiation of this protocol; group B (n = 14) had surgery during the current protocol era. RESULTS: The median number of chest tube days was lower in group B (6 vs 11 days, p < 0.001) as was the total indexed drainage (126 vs 259 mL/kg, p < 0.001). Patients in group B had shorter intensive care unit length of stay (4 vs 7 days, p = 0.004) and hospital length of stay (8 vs 13 days, p = 0.001). Group B had higher preoperative common atrial pressures (7.0 vs 5.8 mm Hg, p = 0.017), end-diastolic pressures (9 vs 7 mm Hg, p = 0.026), and trended toward higher pulmonary artery pressures (11.5 vs 9.5 mm Hg, p = 0.077). There was no statistically significant difference in age, weight, transpulmonary gradient, or pulmonary vascular resistance between groups. CONCLUSIONS: The PORTLAND protocol has improved early outcomes after the Fontan procedure. Chest tube drainage and duration, and both intensive care unit and hospital length of stay have been reduced since initiation of this protocol.

Emergent use of extracorporeal membrane oxygenation during pediatric cardiac catheterization.

OBJECTIVES: The goal of this study was to evaluate the utility of extracorporeal membrane oxygenation (ECMO) to resuscitate patients following critical cardiac events in the catheterization laboratory. DESIGN: Retrospective review of medical records. SETTING: Cardiac intensive care unit and cardiac catheterization laboratory at a tertiary care children's hospital. PATIENTS: Pediatric patients cannulated emergently for ECMO in the cardiac catheterization laboratory (n = 22). INTERVENTIONS: ECMO was initiated emergently in the cardiac catheterization laboratory for progressive hemodynamic deterioration due to low cardiac output syndrome or catheter-induced complications. MEASUREMENTS AND MAIN RESULTS: Twenty-two patients were cannulated for ECMO in the catheterization laboratory between 1996 and 2004. Median age was 33 months (range 0-192), median weight 14.8 kg (2.4-75), and median duration of ECMO 84 hrs (2-343). Indications included catheter-induced complication (n = 14), severe low cardiac output syndrome (n = 7), and hypoxemia (n = 1). Three patients (14%) were cannulated in the catheterization laboratory before catheterization for low cardiac output or hypoxemia. During cannulation, 19 patients (86%) were receiving chest compressions; median duration of cardiopulmonary resuscitation was 29 mins (20-57). Eighteen patients (82%) survived to discharge (five of whom underwent cardiac transplantation) and four (18%) died. Of 19 patients who received cardiopulmonary resuscitation during cannulation, 15 (79%) survived to discharge and nine (47%) sustained neurologic injury. There was no significant difference between survivors and nonsurvivors in age, weight, duration of cardiopulmonary resuscitation or ECMO support, pH, or lactate levels. CONCLUSIONS: ECMO is a technically feasible and highly successful tool in the resuscitation of pediatric patients following critical events in the cardiac catheterization laboratory.

Management of coronary artery fistulae. Patient selection and results of transcatheter closure.

OBJECTIVES: We report short-term findings in 33 patients after transcatheter closure (TCC) of coronary artery fistulae (CAF) and compare our results with those reported in the recent transcatheter and surgical literature. BACKGROUND: Transcatheter closure of CAF has been advocated as a minimally invasive alternative to surgery. METHODS: We reviewed all patients presenting with significant CAF between January 1988 and August 2000. Those with additional complex cardiac disease requiring surgical management were excluded. RESULTS: Of 39 patients considered for TCC, occlusion devices were placed in 33 patients (85%) at 35 procedures and included coils in 28, umbrella devices in 6 and a Grifka vascular occlusion device in 1. Post-deployment angiograms demonstrated complete occlusion in 19, trace in 11, or small residual flow in 5. Follow-up echocardiograms (median, 2.8 years) in 27 patients showed no flow in 22 or small residual flow in 5. Of the 6 patients without follow-up imaging, immediate post-deployment angiograms showed complete occlusion in 5 or small residual flow in 1. Thus, complete occlusion was accomplished in 27 patients (82%). Early complications included transient ST-T wave changes in 5, transient arrhythmias in 4 and single instances of distal coronary artery spasm, fistula dissection and unretrieved coil embolization. There were no deaths or long-term morbidity. Device placement was not attempted in 6 patients (15%), because of multiple fistula drainage sites in 4, extreme vessel tortuosity in 1 and an intracardiac hemangioma in 1. CONCLUSIONS: A comparison of our results with those in the recent transcatheter and surgical literature shows similar early effectiveness, morbidity and mortality. From data available, TCC of CAF is an acceptable alternative to surgery in most patients.