Pulse oximetry screening for critical congenital heart disease in planned out of hospital births and the incidence of critical congenital heart disease in the Plain community.

OBJECTIVE: This study evaluated pulse oximetry screening (POS) for critical congenital heart disease (CCHD) in planned out of hospital births with special attention to births in Plain communities (Amish, Mennonite and similar). STUDY DESIGN: Wisconsin out of hospital births in 2013 and 2014 were evaluated. Care providers were supplied with and trained in the use of pulse oximeters for CCHD screening. State records were reviewed to identify deaths and hospital admissions due to CCHD in this population. RESULTS: Detailed information on POS was available in 1616 planned out of hospital births. Seven hundred and ninety-nine were from the Plain community. In total, 1584 babies (98%) passed their POS, 16 infants (1%) failed and 16 (1%) were not screened. Five infants from the Plain community had CCHD and three were detected by POS. CONCLUSION: POS for CCHD can be successfully implemented outside the hospital setting and plays a particularly important role in communities with high rates of CCHD and where formal prenatal screening is uncommon.

Pulse oximetry screening for critical congenital heart disease in the neonatal intensive care unit.

OBJECTIVE: Pulse oximetry screening (POS) is an effective tool to detect critical congenital heart disease (CCHD) in asymptomatic term infants, but its value in the neonatal intensive care unit (NICU) requires further clarification. STUDY DESIGN: A retrospective review of 1005 babies without previously diagnosed CCHD admitted to a level III NICU was performed to assess the risk for missed CCHD and performance of POS. RESULT: Of the 1005 NICU patients, 812 had documented POS and none failed POS. In 812 patients, 547 had delayed POS because of the use of supplemental oxygen. In 259/812 patients, POS was delayed until the baby was >2 weeks old. CCHD was excluded by echocardiography, irrespective of POS, in 287/1005 patients. CONCLUSION: POS can be performed in the NICU with minimal adverse effects. However, in many NICU patients CCHD is confirmed or excluded before POS, and POS will frequently be performed after CCHD would have been expected to become symptomatic.

Care everywhere, a point-to-point HIE tool: utilization and impact on patient care in the ED.

BACKGROUND: Emergency departments (EDs) routinely struggle with gaps in information when providing patient care. A point to point health information exchange (HIE) model has the potential to effectively fill those gaps. OBJECTIVE: To examine the utility, perceived and actual, of a point-to-point HIE tool called Care Everywhere (CE) and its impact on patient care in the ED. METHODS: This mixed methods study was performed at four large hospital EDs between January 2012 and November 2012. Retrospective data was extracted from the electronic health record (EHR) to evaluate CE utilization since implementation. ED notes data were extracted from ED visits occurring between January 2012 and June 2012 and were reviewed to evaluate the impact of exchanged information on patient care. RESULTS: Per focus group discussions, physicians thought the information received via CE was of value to patient care, particularly laboratory results, imaging, medication lists, discharge summaries and ECG interpretations. They feel the greatest impact of HIE is the avoidance of duplicative diagnostic testing and the identification of drug-seeking behavior. Nursing and ancillary staff expressed somewhat less enthusiasm but still felt HIE positively impacted patient care. Over a period of six months, CE was used in approximately 1.46% of ED encounters. A review of ED provider notes over that time period revealed CE use resulted in 560 duplicate diagnostic procedures being avoided and 28 cases of drug seeking behavior identified. CONCLUSION: Our study provides insight into the perceived value of HIE from the point of view of our ED physicians and staff. It also demonstrates that a point-to-point HIE tool such as Epic System's Care Everywhere has the potential to generate greater efficiencies within the ED and impact to patient care through elimination of duplicative diagnostic imaging or testing and resource utilization associated with those procedures.

22q11.2 microdeletions in adults with familial tetralogy of Fallot.

PURPOSE: To determine the incidence of 22q11.2 microdeletions in the adult survivors of correction of tetralogy of Fallot who have familial congenital heart disease. METHODS: Patients who had survived a correction of tetralogy of Fallot between 1954 and 1974 and had affected family members were identified during a study of these long-term survivors. Fluorescence in situ hybridization analysis was performed using both the N 25 (Oncor) and TUPLE1(VYSIS) probes, mapped to 22q11.2. RESULTS: One of 18 (5.6%) patients had a microdeletion within 22q11.2, including both N25 and TUPLE1. CONCLUSION: 22q11.2 microdeletions involving TUPLE1 and/or N25 are present in a minority of adults with familial tetralogy of Fallot.

Significance of early transient complete heart block as a predictor of sudden death late after operative correction of tetralogy of Fallot.

We evaluated the long-term outcome and incidence of sudden death in the 288 patients discharged after corrective operation for tetralogy of Fallot at the University of Minnesota between 1954 and 1974. The perioperative characteristics of these patients were evaluated with respect to their long-term outcome. A health status survey was sent to each survivor and their functional status was evaluated with respect to the perioperative variables. Current follow-up was available in 279 of 288 of the postoperative survivors (97%); the mean duration of follow-up was 28 years. The 40-year survival rate was 76% and 154 of 163 of survey responders (95%) were in New York Heart Association class I or II. Twenty-six of the 288 died suddenly (9%). Those with transient complete heart block that persisted beyond the third postoperative day had a lower long-term survival rate (p <0.001) and a higher incidence of late sudden death (p <0.001). Patients aged >12 years at operation had a lower long-term survival rate (p <0.001) and less favorable long-term functional status. The use of a transannular patch was associated with increased late morbidity but not mortality. Thus, the very long-term outcome after operative correction of tetralogy of Fallot is excellent. Late sudden death remains a concern for operative survivors, occurring in 9% of these patients. Late sudden death is strongly associated with transient complete heart block that persisted beyond the third postoperative day.

Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.

BACKGROUND: Ventricular arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot are devastating complications in adult survivors of early surgery, but their prediction remains difficult. METHODS: We examined surgical, electrocardiographic, and late haemodynamic data, and their relation to clinical arrhythmia and sudden death occurring over 10 years, in a multicentre cohort of patients with repaired tetralogy, who were alive in 1985. RESULTS: Of 793 patients (mean age at repair 8.2 years [SD 8], mean time from repair 21.1 years [8.7]) who entered the study, 33 patients developed sustained monomorphic ventricular tachycardia, 16 died suddenly, and 29 had new-onset sustained atrial flutter or fibrillation. Electrocardiographic markers (QRS duration, QRS rate of change between 1985 and 1995) were significantly greater in the ventricular tachycardia and sudden-death groups. Older age at repair was associated with a higher risk of sudden death and atrial tachyarrhythmia. Pulmonary regurgitation was the main underlying haemodynamic lesion for patients with ventricular tachycardia and sudden death, whereas tricuspid regurgitation was for those with atrial flutter/fibrillation. Despite adverse haemodynamics, no patient who died suddenly had undergone late reoperation. CONCLUSION: Arrhythmia and sudden death are important late sequelae for patients after repair of tetralogy of Fallot. The electrophysiological and haemodynamic substrate of sudden death resembled that of sustained ventricular tachycardia, with pulmonary regurgitation being the predominant haemodynamic lesion. Preservation or restoration of pulmonary valve function may thus reduce the risk of sudden death. Furthermore, electrocardiographic markers can help to identify patients at risk.

Adults with tetralogy of Fallot: long-term follow-up.

Tetralogy of Fallot is the most common cyanotic heart lesion and one of the most commonly seen by cardiologists caring for adults with congenital heart disease. In the last 50 years, the outlook for these patients has changed dramatically, and most children with tetralogy of Fallot will live well into adulthood. Although most will have undergone a corrective operation, an important minority of patients with tetralogy of Fallot will have had only a previous palliative procedure. Rarely, the clinician may encounter an adult with unoperated tetralogy of Fallot. With surgical correction, the very long-term outcome is excellent, and most patients lead active and productive lives. However, certain issues must be considered for the long-term care of patients with tetralogy of Fallot. These individuals have an important incidence of residual or recurrent lesions as well as an increased risk for late sudden death. The exact mechanisms for late sudden death are incompletely defined but probably include both the residual hemodynamic and electrophysiologic substrates that act in concert.

Self-administration in rats allowed unlimited access to nicotine.

The purpose of the present study was to develop an animal model of nicotine self-administration that more closely approximates the conditions of human nicotine use than do existing models. In most nicotine self-administration models, rats acquire self-administration during brief daily sessions in which rapid injections of a relatively high dose of the drug, 0.03 mg/kg, serve as the reinforcer. The present study examined nicotine self-administration in rats that acquired the behavior while having virtually unlimited access to injections of a relatively low dose of the drug; the rats did not have any prior operant training or shaping. Under these conditions, rats readily acquire nicotine self-administration at doses at least as low as 0.00375 mg/kg per injection, and they self-administer throughout the active portion of their light cycle. The daily nicotine intake of rats, which ranged from 0.18 to 1.38 mg/kg per day, appears to be comparable to that of human smokers.