Health-Related Quality of Life in Children and Young Adults with Marfan Syndrome.

OBJECTIVE: To assess health-related quality of life (HRQOL) in a large multicenter cohort of children and young adults with Marfan syndrome participating in the Pediatric Heart Network Marfan Trial. STUDY DESIGN: The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales were administered to 321 subjects with Marfan syndrome (5-25 years). PedsQL scores were compared with healthy population norms. The impact of treatment arm (atenolol vs losartan), severity of clinical features, and number of patient-reported symptoms on HRQOL was assessed by general linear models. RESULTS: Mean PedsQL scores in children (5-18 years) with Marfan syndrome were lower than healthy population norms for physical (P ≤ .003) and psychosocial (P < .001) domains; mean psychosocial scores for adults (19-25 years) were greater than healthy norms (P < .001). HRQOL across multiple domains correlated inversely with frequency of patient-reported symptoms (r = 0.30-0.38, P < .0001). Those <18 years of age with neurodevelopmental disorders (mainly learning disability, attention-deficit/hyperactivity disorder) had lower mean PedsQL scores (5.5-7.4 lower, P < .04). A multivariable model found age, sex, patient-reported symptoms, and neurodevelopmental disorder to be independent predictors of HRQOL. There were no differences in HRQOL scores by treatment arm, aortic root z score, number of skeletal features, or presence of ectopia lentis. CONCLUSIONS: Children and adolescents with Marfan syndrome were at high risk for impaired HRQOL. Patient-reported symptoms and neurodevelopmental disorder, but not treatment arm or severity of Marfan syndrome-related physical findings, were associated with lower HRQOL.

Baccalaureate Minority Nursing Students Perceived Barriers and Facilitators to Clinical Education Practices: An Integrative Review.

AIM: This integrative review synthesized baccalaureate minority nursing students' perceptions of their clinical experiences. BACKGROUND: The diversity of the nursing workforce does not mirror the United States population. Attrition rates of minority nursing students remain higher than rates for White students. Literature examining facilitators and barriers to minority student success predominantly focuses on academic factors, excluding those relevant to clinical education. METHOD: An integrative review using literature from nursing and education. FINDINGS: Three common perceived barriers were identified: discrimination from faculty, peers, nursing staff, and patients; bias in faculty grading practices; and isolation. CONCLUSION: Although little is known about the relationship between clinical failures and overall attrition, this review provides evidence that minority students encounter significant barriers in clinical education. To increase the diversity of the nursing workforce, faculty must address these issues and make modifications to ensure an equal opportunity at a quality education for all students.

Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial.

BACKGROUND: Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass. METHODS AND RESULTS: In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3 ± 1.1 (mean ± SD) months. Mean PDI (74 ± 19) and MDI (89 ± 18) scores were lower than normative means (each P<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R(2)=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R(2)=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy. CONCLUSIONS: Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Patient outcomes after transcatheter and surgical pulmonary valve replacement for pulmonary regurgitation in patients with repaired tetralogy of Fallot: A quasi-meta-analysis.

BACKGROUND: Individuals with repaired tetralogy of Fallot develop pulmonary regurgitation that may cause symptoms (dyspnea, chest pain, palpitations, fatigue, presyncope, and syncope), impair functional capacity, and may affect health-related quality of life. Surgical pulmonary valve replacement is the gold standard of treatment although transcatheter pulmonary valve replacement is becoming more common. Patients want to know whether less invasive options are as good. AIMS: This analysis aimed to examine the differences in surgical versus transcatheter pulmonary valve replacement effects in terms of physiological/biological variables, symptoms, functional status and health-related quality of life. METHODS: This quasi-meta-analysis included 85 surgical and 47 transcatheter pulmonary valve replacement studies published between 1995-2016. RESULTS: In terms of physiological/biological variables, both surgical and transcatheter pulmonary valve replacement improved pulmonary regurgitation and systolic and diastolic right ventricular volume indices but not heart function. In the left heart, only surgical pulmonary valve replacement improved heart function. Only transcatheter pulmonary valve replacement improved left ventricular end-diastolic indices and neither improved endsystolic indices. Only surgery has been demonstrated to decrease QRS duration but there is little evidence of arrhythmia reduction. Symptom change is poorly documented. Functional class improves but exercise capacity generally does not. Some aspects of health-related quality of life improve with surgery and in one small transcatheter pulmonary valve replacement study. CONCLUSION: Transcatheter and surgical pulmonary valve replacement compare favorably for heart remodeling. Exercise capacity does not change with either technique. Health-related quality of life improves after surgical pulmonary valve replacement. There are numerous gaps in documentation of changes in arrhythmias and symptoms.

Perceptions of academic health science research center personnel regarding informed consent processes and therapeutic misconception.

INTRODUCTION: Instrumentation exists to measure voluntariness and misunderstanding in informed consent processes. However, research personnel's perspectives about using instrumentation to measure therapeutic misconceptions in research participants has not been reported. We designed a workshop to promote research personnel knowledge of emerging instrumentation and to study the perceptions of research personnel regarding such instruments. METHODS AND FINDINGS: Two nationally recognized experts who have developed psychometric instruments to measure aspects of informed consent presented their recent findings to research personnel of the Medical University of South Carolina at a one-day workshop. Following the presentations, workshop attendees divided into two focus groups and shared their perceptions regarding the presentation content. Inductive thematic analysis detected themes related to informed consent processes including: investigator/provider role clarity; investigator transparency; therapeutic misconception; and screening subjects for understanding. CONCLUSION: Our findings suggest future directions in applied, proactive empirical research to better understand investigator perceptions and practices related to transparency in research, and to develop instrumentation to detect risks to the integrity of informed consent in order to promote voluntariness and autonomy and minimize therapeutic misconception in research practices.