Adaptación y validación española del Empowerment of Parents in the Intensive Care-Neonatology (EMPATHIC-N) / Spanish adaptation and validation of the empowerment of parents in the intensive care-neonatology (EMPATHIC-N) questionnaire

Introducción: La satisfacción de los padres de niños en Unidades de Cuidados Intensivos Neonatales (UCIN) apenas se evalúa por la carencia de herramientas específicas. El EMpowerment of PArents in THe Intensive Care Neonatology (EMPATHIC-N) es un cuestionario de satisfacción que evalúa los cuidados centrados en el desarrollo y la familia, validado en varios países pero no en España. Objetivos: Realizar la adaptación y validación del EMPATHIC-N para la evaluación de la satisfacción de los padres de niños ingresados en UCIN. Material y métodos: Primero, se realizó una traducción-retrotraducción y adaptación transcultural siguiendo un procedimiento estandarizado con un comité de expertos, mediante el método Delphi. Posteriormente, se realizó un estudio piloto con ocho padres y un estudio transversal en la unidad neonatal de un hospital terciario para analizar la fiabilidad y la validez convergente del cuestionario. Resultados: La versión española del EMPATHIC-N demostró su comprensibilidad, viabilidad, aplicabilidad y utilidad en el ámbito sanitario infantil, evaluado por 19 profesionales y 60 padres. Su validez de contenido global resultó excelente (0,93). La fiabilidad y validez convergente del EMPHATIC-N se analizaron en 65 encuestas. El α de Cronbach por dominios se situó por encima de 0,7, indicando una alta consistencia interna. La validez fue medida correlacionando los cinco dominios con los cuatro ítems de satisfacción general, demostrando una correlación adecuada (rs: 0,4-0,76; p < 0,01). Conclusiones: La versión española del cuestionario EMPATHIC-N es un instrumento comprensible, viable, aplicable, útil, válido y fiable para medir la satisfacción de los padres en las unidades neonatales. (AU)

Introduction: Parental satisfaction is rarely measured in the neonatal intensive care unit due to a lack of specific assessment tools. The EMpowerment of PArents in THe Intensive Care-Neonatology (EMPATHIC-N) questionnaire is an instrument to assess satisfaction in relation to family-centred care that has been validated in several countries, but not Spain. Objectives: To perform the translation and cultural adaptation of the EMPATHIC-N to Spanish followed by its validation for the purpose of assessing satisfaction in parents with children admitted to the neonatal intensive care unit. Material and methods: The questionnaire first underwent forward and backward translation and transcultural adaptation by a panel of experts through a standardized process based on the Delphi method, followed by a pilot study in 8 parents and then a cross-sectional study in the neonatal intensive care unit of a tertiary care hospital to assess the reliability and converging validity of the Spanish version. Results: The study proved the comprehensibility, validity, feasibility, applicability and usefulness of the Spanish version of the EMPATHIC-N in the field of paediatric health after being evaluated by 19 professionals and 60 parents. The content validity was found to be excellent (0.93). The reliability and convergent validity of the Spanish version of the EMPHATIC-N was analysed in a sample of 65 completed questionnaires. The Cronbach α for each domain was greater than 0.7, indicating a high internal consistency. We assessed validity by analysing the correlation of the 5 domains with the with the 4 general satisfaction items. The validity was found to be adequate (rs, 0.4-0.76; P < .01). Conclusions: The Spanish version of the EMPATHIC-N questionnaire is a comprehensible, useful, valid and reliable instrument to measure satisfaction in the parents of children admitted to neonatal care units. (AU)

Spanish adaptation and validation of the empowerment of parents in the intensive care-neonatology (EMPATHIC-N) questionnaire.

INTRODUCTION: Parental satisfaction is rarely measured in the neonatal intensive care unit due to a lack of specific assessment tools. The Empowerment of Parents in The Intensive Care-Neonatology (EMPATHIC-N) questionnaire is an instrument to assess satisfaction in relation to family-centred care that has been validated in several countries, but not Spain. OBJECTIVES: To perform the translation and cultural adaptation of the EMPATHIC-N to Spanish followed by its validation for the purpose of assessing satisfaction in parents with children admitted to the neonatal intensive care unit. MATERIAL AND METHODS: The questionnaire first underwent forward and backward translation and transcultural adaptation by a panel of experts through a standardized process based on the Delphi method, followed by a pilot study in 8 parents and then a cross-sectional study in the neonatal intensive care unit of a tertiary care hospital to assess the reliability and converging validity of the Spanish version. RESULTS: The study proved the comprehensibility, validity, feasibility, applicability and usefulness of the Spanish version of the EMPATHIC-N in the field of paediatric health after being evaluated by 19 professionals and 60 parents. The content validity was found to be excellent (0.93). The reliability and convergent validity of the Spanish version of the EMPHATIC-N was analysed in a sample of 65 completed questionnaires. The Cronbach α for each domain was greater than 0.7, indicating a high internal consistency. We assessed validity by analysing the correlation of the 5 domains with the with the 4 general satisfaction items. The validity was found to be adequate (rs, 0.4-0.76; P < .01). CONCLUSIONS: The Spanish version of the EMPATHIC-N questionnaire is a comprehensible, useful, valid and reliable instrument to measure satisfaction in the parents of children admitted to neonatal care units.

Core components of a rehabilitation program in pediatric cardiac disease.

There is increasing effort in both the inpatient and outpatient setting to improve care, function, and quality of life for children with congenital heart disease, and to decrease complications. As the mortality rates of surgical procedures for congenital heart disease decrease, improvement in perioperative morbidity and quality of life have become key metrics of quality of care. Quality of life and function in patients with congenital heart disease can be affected by multiple factors: the underlying heart condition, cardiac surgery, complications, and medical treatment. Some of the functional areas affected are motor abilities, exercise capacity, feeding, speech, cognition, and psychosocial adjustment. Rehabilitation interventions aim to enhance and restore functional ability and quality of life for those with physical impairments or disabilities. Interventions such as exercise training have been extensively evaluated in adults with acquired heart disease, and rehabilitation interventions for pediatric patients with congenital heart disease have similar potential to improve perioperative morbidity and quality of life. However, literature regarding the pediatric population is limited. We have gathered a multidisciplinary team of experts from major institutions to create evidence- and practice-based guidelines for pediatric cardiac rehabilitation programs in both inpatient and outpatient settings. To improve the quality of life of pediatric patients with congenital heart disease, we propose the use of individualized multidisciplinary rehabilitation programs that include: medical management; neuropsychology; nursing care; rehabilitation equipment; physical, occupational, speech, and feeding therapies; and exercise training.

Acquired Brain Injury in the Pediatric Intensive Care Unit: Special Considerations for Delirium Protocols.

The goal of this article was to highlight the overlapping nature of symptoms of delirium and acquired brain injury (ABI) in children and similarities and differences in treatment, with a focus on literature supporting an adverse effect of antipsychotic medications on recovery from brain injury. An interdisciplinary approach to education regarding overlap between symptoms of delirium and ABI is important for pediatric intensive care settings, particularly at this time when standardized procedures for delirium screening and management are being increasingly employed. Development of treatment protocols specific to children with ABI that combine both nonpharmacologic and pharmacologic strategies will reduce the risk of reliance on treatment strategies that are less preferred and optimize care for this population.

Rehabilitation in Pediatric Heart Failure and Heart Transplant.

Survival of pediatric patients with heart failure has improved due to medical and surgical advances over the past decades. The complexity of pediatric heart transplant patients has increased as medical and surgical management for patients with congenital heart disease continues to improve. Quality of life in patients with heart failure and transplant might be affected by the impact on functional status that heart failure, heart failure complications or treatment might have. Functional areas affected might be motor, exercise capacity, feeding, speech and/or cognition. The goal of rehabilitation is to enhance and restore functional ability and quality of life to those with physical impairments or disabilities. Some of these rehabilitation interventions such as exercise training have been extensively evaluated in adults with heart failure. Literature in the pediatric population is limited yet promising. The use of additional rehabilitation interventions geared toward specific complications experienced by patients with heart failure or heart transplant are potentially helpful. The use of individualized multidisciplinary rehabilitation program that includes medical management, rehabilitation equipment and the use of physical, occupational, speech and feeding therapies can help improve the quality of life of patients with heart failure and transplant.

The Palliative Use of Intrathecal Baclofen in Niemann-Pick Disease Type C.

Niemann-Pick disease type C is a rare progressive genetic disorder that leads to the abnormal accumulation of lipids within various tissues of the body, including brain tissue and liver. There is a rapid progression of the disease, resulting in severe disability in only a few years after the first symptoms, and survival is not much longer. Spasticity, dystonia, and chronic pain are common findings that severely impact quality of life in these patients. Analgesic management with traditional pain medications is not always effective, and the risk for secondary effects in medically complex patients is high. Liver function is also a limiting factor in these patients. This is a case report of a boy with advanced Niemann-Pick disease type C with developmental regression, cataplexia, and seizures. His severe spasticity made positioning and care difficult, and intense pain required multiple hospitalizations. He had unsuccessfully trialed multiple drugs. An intrathecal baclofen pump was placed without surgical complications and resulted in positive clinical effects. Baclofen pumps have classically been used for spasticity management in adults and children with nonprogressive diseases such as cerebral palsy or spinal cord injury with relatively long life expectancies. In adults, they have been used in patients with multiple sclerosis; however, use in pediatric neurodegenerative diseases has scarcely been reported. The use of intrathecal baclofen in palliative settings might provide an additional resource to provide comfort and quality of life for children with neurodegenerative diseases not only at end-of-life stages but also earlier on.

Predictors of Postoperative Rehabilitation Therapy Following Congenital Heart Surgery.

BACKGROUND: Patients with congenital heart disease are at risk of motor, cognitive, speech, and feeding difficulties after cardiac surgery. Rehabilitation therapy could improve functional outcomes in this population if applied in the acute postcardiac surgical in-hospital stay. However, information on the types of acute postcardiac surgery therapy needs in children is scarce. Our goal was to describe rehabilitation therapy following congenital heart surgery and pre/intraoperative factors associated with need for therapy. METHODS AND RESULTS: This is a retrospective cohort study of patients <18 years undergoing heart surgery at our center from January 1, 2013 to January 31, 2015. Demographic, and pre-, intra-, and postoperative clinical and rehabilitation therapy (physical, occupational, speech, feeding therapy, and neurodevelopment intervention) data were collected. Need for rehabilitation therapy in the acute postoperative period, particularly following palliative repair, was the outcome variable in a multivariable logistic regression model to identify independent pre- and intraoperative factors associated with therapy. A total of 586 out of 1415 (41%) subjects received rehabilitation therapy postsurgery. Certain subgroups had increased rehabilitation therapy use such as neonates (80%). On multivariable analysis, palliative repair, prematurity, genetic syndrome, presurgical hospital stay of more than 1 day, and prolonged cardiopulmonary bypass time were independently associated with rehabilitation therapy. CONCLUSIONS: Nearly half of patients who underwent post-congenital heart surgery received rehabilitation therapy. Frequency of use and types of therapy vary according to patient characteristics; however, certain pre- and intraoperative factors are associated with need for rehabilitation therapy, and may aid decision-making for appropriate resource allocation.

A Randomized Trial Comparing Cardiac Rehabilitation to Standard of Care for Adults With Congenital Heart Disease.

BACKGROUND: Cardiac rehabilitation (CR) improves exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. Cardiac rehabilitation has not been extensively studied in adults with congenital heart disease (CHD). METHODS: We performed a prospective, randomized controlled trial (NCT01822769) of a 12-week clinical CR program compared with standard of care (SOC). Participants were ≥16 years old, had moderate or severe CHD, had O2 saturation ≥92%, and had peak O2 consumption ([Formula: see text]) < 80% predicted. We assessed exercise capacity, physical activity, quality of life, self-reported health status, and other variables at baseline and after 12 weeks. The prespecified primary end point was change in [Formula: see text]. RESULTS: We analyzed data on 28 participants (aged 41.1 ± 12.1 years, 50% male), 13 randomized to CR and 15 to SOC. [Formula: see text] averaged 16.8 ± 3.8 mL/kg/min, peak work rate = 95 ± 28 W, and median Minnesota Living with Heart Failure Questionnaire (MLHFQ) score = 27 (interquartile range: 11-44). Cardiac rehabilitation participants were older (48 ± 9 years vs 36 ± 12 years; P = .01), but there were no significant between-group differences in other variables. There were no adverse events related to CR. [Formula: see text] increased in the CR group compared with SOC (+2.2 mL/kg/min, 95% confidence interval: 0.7-3.7; P = .002, age-adjusted +2.7 mL/kg/min; P = .004); there was a nonsignificant improvement in work rate (+8.1 W; P = .13). Among the 25 participants with baseline MLHFQ > 5, there was a clinically important >5-point improvement in 72.7% and 28.6% of CR and SOC participants, respectively ( P = .047). Cardiac rehabilitation was also associated with improved self-assessment of overall health ( P < .04). CONCLUSIONS: Cardiac rehabilitation is safe and is associated with improvement in aerobic capacity and self-reported health status compared with SOC in adults with CHD.

Noninvasive cardiac output estimation by inert gas rebreathing in pediatric and congenital heart disease.

BACKGROUND: Inert gas rebreathing (IGR) techniques provide rapid, reliable estimates of cardiac output in adults with structurally normal hearts. Data on IGR reliability in pediatric and congenital heart disease populations are lacking. Our objective was to validate pulmonary blood flow (Qp) measurement by IGR compared with clinical reference tests, cardiovascular magnetic resonance (CMR), and indirect Fick. METHODS: Pulmonary blood flow was measured by IGR and CMR or indirect Fick in 80 patients grouped by presence and type of shunt lesion. Inert gas rebreathing precision was assessed using Bland-Altman analysis, repeatability coefficient, intraclass correlation, and coefficient of error. Agreement with the reference tests was assessed with Bland-Altman plots. For comparison, agreement between the 2 reference tests, CMR and indirect Fick, was assessed in 34 contemporary patients. RESULTS: Subjects were aged 7-78 years and had a wide range of cardiac diagnoses. Inert gas rebreathing Qp showed good repeatability (95% limits of agreement for 2 trials = ±22%, repeatability coefficient = 1.2 L/min, intraclass correlation = 0.92, and coefficient of error = 5%). In the absence of left-to-right shunting (n = 67), IGR Qp estimates agreed with CMR and indirect Fick Qp estimates, and the reference tests agreed with each other, with mean bias ≤10% (≤0.5 L/min) and 95% limits of agreement ±33%-38%. Conversely, IGR was unreliable in patients with left-to-right shunt (n = 14), with large bias (-58%, -4.0 L/min) and wide limits of agreement (±76%). CONCLUSIONS: Inert gas rebreathing reliably estimates Qp in children and adults with congenital heart disease in the absence of left-to-right shunting, with agreement comparable to that seen between CMR and indirect Fick estimates.

Exercise oscillatory ventilation in patients with Fontan physiology.

BACKGROUND: Exercise oscillatory ventilation (EOV) refers to regular oscillations in minute ventilation (VE) during exercise. Its presence correlates with heart failure severity and worse prognosis in adults with acquired heart failure. We evaluated the prevalence and predictive value of EOV in patients with single ventricle Fontan physiology. METHODS AND RESULTS: We performed a cross-sectional analysis and prospective survival analysis of patients who had undergone a Fontan procedure and subsequent cardiopulmonary exercise test. Data were reviewed for baseline characteristics and incident mortality, heart transplant, or nonelective cardiovascular hospitalization. EOV was defined as regular oscillations for >60% of exercise duration with amplitude >15% of average VE. Survival analysis was performed using Cox regression. Among 253 subjects, EOV was present in 37.5%. Patients with EOV were younger (18.8±9.0 versus 21.7±10.1 years; P=0.02). EOV was associated with higher New York Heart Association functional class (P=0.02) and VE/VCO2 slope (36.8±6.9 versus 33.7±5.7; P=0.0002), but not with peak VO2 (59.7±14.3 versus 61.0±16.0% predicted; P=0.52) or noninvasive measures of cardiac function. The presence of EOV was associated with slightly lower mean cardiac index but other invasive hemodynamic variables were similar. During a median follow-up of 5.5 years, 22 patients underwent transplant or died (n=19 primary deaths, 3 transplants with 2 subsequent deaths). EOV was associated with increased risk of death or transplant (hazard ratio, 3.9; 95% confidence interval, 1.5-10.0; P=0.002) and also predicted the combined outcome of death, transplant, or nonelective cardiovascular hospitalization after adjusting for New York Heart Association functional class, peak VO2, and other covariates (multivariable hazard ratio, 2.0; 95% confidence interval, 1.2-3.6; P=0.01). CONCLUSIONS: EOV is common in the Fontan population and strongly predicts lower transplant-free survival.

Relationship between Exercise Parameters and Noninvasive Indices of Right Ventricular Function in Patients with Biventricular Circulation and Systemic Right Ventricle.

OBJECTIVES: In patients with systemic right ventricles (RVs) in a biventricular circulation, exercise capacity and RV function often deteriorate over time and echocardiographic assessment of systemic RV function is difficult. The purpose of this study was to examine the relationship between exercise capacity and RV function and to determine which noninvasive imaging parameters correlate most closely with exercise capacity. DESIGN: Patients with a systemic RV (D-loop transposition of the great arteries [TGA] after atrial switch procedure or physiologically "corrected" TGA) who underwent cardiopulmonary exercise testing (CPX) and noninvasive imaging (cardiac magnetic resonance [CMR] and echocardiography [echo]) within 1 year of CPX were identified. Regression analysis was used to evaluate the relationship between exercise variables and noninvasive indices of ventricular function. RESULTS: We identified 92 patients with 149 encounters (mean age 31.0 years, 61% men, 70% D-loop TGA) meeting inclusion criteria. Statistically significant correlations between % predicted peak oxygen uptake (%pVO2 ) and RV ejection fraction (EF) (r = 0.29, P = .0007), indexed RV end-systolic volume (r = -0.25, P = .002), and Tei index (r = -0.22, P = .03) were found. In patients without additional hemodynamically significant lesions, the correlations between %pVO2 and RV EF (r = 0.37, P = .0007) and the Tei index (r = -0.28, P = .03) strengthened and a correlation emerged between %pVO2 and dP/dtic (r = 0.31, P = .007). On multivariable analysis, Tei index was the only statistically significant correlate of %pVO2 (P = .04). CONCLUSIONS: In patients with systemic RVs in a biventricular circulation, CMR-derived RVEF and echo-derived Tei index correlate with %pVO2 . On multivariable analysis, the Tei index was the strongest predictor of peak %pVO2 response.

Effect of inhaled iloprost on the exercise function of Fontan patients: a demonstration of concept.

BACKGROUND: Exercise capacity following Fontan surgery is often depressed. An inability to reduce pulmonary vascular resistance appropriately during exercise may contribute to this phenomenon. The aim of this study was to determine whether administration of iloprost, a selective pulmonary vasodilator, would improve exercise function after Fontan procedure. METHODS: Double-blind, randomized, placebo controlled, crossover trial. Patients performed two cardiopulmonary exercise tests (CPX) separated by <1 month. A single nebulizer treatment (iloprost or placebo) was administered before each CPX. RESULTS: 18 patients aged 12-49 (median 17) years were recruited. Mild throat discomfort developed in 10/18 patients during iloprost administration; all but 1 were able to complete treatment. No symptoms developed during placebo treatments (p<0.001). Two additional patients did not complete CPX: one with atrial flutter; another with developmental issues that precluded adequate CPX. In the 15 remaining subjects oxygen pulse (a surrogate for forward stroke volume) at peak exercise was higher following iloprost (median increase 1.2 ml/beat; p<0.001). Peak VO2 also rose (median increase 1.3 ml/kg/min; p<0.04). Nine patients had peak VO2 <30 ml/kg/min; each of these patients had higher peak VO2 following iloprost. Only 3/6 patients with peak VO2 >30 ml/kg/min had higher peak VO2 following iloprost (p<0.04). CONCLUSIONS: Iloprost improves the peak oxygen pulse and peak VO2 of patients with Fontan physiology and appears to be particularly beneficial among patients with impaired exercise function. Treatment is associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan patients with limited functional capacity.

[Functional outcomes after resection of hip arthroplasty (Girdlestone technique)]. / Situación funcional de los pacientes tras resección de artroplastia de cadera (Girdlestone).

INTRODUCTION: the complications of hip arthroplasty in elderly patients can lead to resection of the prosthesis and cement, also known as the Girdlestone technique. This kind of surgery causes serious dysmetria and alters gait dynamics, thus affecting the patient's functional ability. The aim of this study was to describe the functional outcome, 1 year after surgery, in a group of patients that underwent surgery with this technique. MATERIAL AND METHODS: we performed a descriptive study of 48 patients aged more than 65 years old who underwent surgery with the Girdlestone technique from 1999 to 2005. Twenty-one patients completed the study. Functional outcome and pain were measured with the Harris scale, and gait with the Functional Ambulatory Classification (FAC) scale 1 year after surgery. RESULTS: the mean age was 79.71 years. The most frequent causes of exeresis were periprosthetic infection in 42% and recurrent hip dislocation in 36%. Of the 21 patients assessed 1 year after the intervention, 42.8% were unable to walk and needed a wheelchair and only 28.5% were able to walk with the aid of one or two crutches. Pain was moderate in 23.8% of the patients and was mild in the remainder. The mean score on the Harris scale was 51 out of 100 points. CONCLUSIONS: failure of hip arthroplasty requiring Girdlestone resection is very damaging to functional outcome in elderly patients.

Situación funcional de los pacientes tras resección de artroplastia de cadera (Girdlestone) / Functional outcomes after resection of hip arthroplasty (Girdlestone technique)

Introducción: las complicaciones de las artroplastias de cadera en pacientes ancianos pueden obligar a la resección de la prótesis y del cemento, o técnica de Girdlestone. Esa intervención produce una grave dismetría y una alteración de la dinámica de la marcha que repercute en la capacidad funcional de los pacientes. En este trabajo queremos describir la situación funcional, a partir del año de la intervención, de un grupo de pacientes intervenidos mediante esta técnica. Material y métodos: estudio descriptivo de 48 pacientes mayores de 65 años, intervenidos de artroplastia de resección de Girdlestone, durante los años 1999-2005. Se completó el estudio en 21 pacientes. La situación funcional y el dolor se midieron mediante la escala de valoración de Harris y la capacidad de marcha mediante el Functional Ambulatory Classification (FAC), a partir del año de la intervención. Resultados: la edad media fue de 79,71 años. Las causas más frecuentes de la exéresis fueron: el 42% infección periprotésica y el 36% la luxación recidivante. De los 21 pacientes valorados un año después de la intervención, el 42,8% no podía caminar y precisaba una silla de ruedas, y tan sólo el 28,5% eran independientes para la marcha, con ayuda de uno o dos bastones. El 23,8% de los pacientes tenía dolor moderado; en el resto el dolor era leve. El valor medio de la escala de Harris fue de 51/100 puntos. Conclusiones: el fracaso de las artroplastias de cadera que precisan de una resección de Girdlestone supone un grave deterioro de la capacidad funcional de los pacientes ancianos

Introduction: the complications of hip arthroplasty in elderly patients can lead to resection of the prothesis and cement, also known as the Girdlestone technique. This kind of surgery causes serious dysmetria and alters gait dynamics, thus affecting the patient’s functional ability. The aim of this study was to describe the functional outcome, 1 year after surgery, in a group of patients that underwent surgery with this technique. Material and methods: we performed a descriptive study of 48 patients aged more than 65 years old who underwent surgery with the Girdlestone technique from 1999 to 2005. Twenty-one patients completed the study. Functional outcome and pain were measured with the Harris scale, and gait with the Functional Ambulatory Classification (FAC) scale 1 year after surgery. Results: the mean age was 79.71 years. The most frequent causes of exeresis were periprosthetic infection in 42% and recurrent hip dislocation in 36%. Of the 21 patients assessed 1 year after the intervention, 42.8% were unable to walk and needed a wheelchair and only 28.5% were able to walk with the aid of one or two crutches. Pain was moderate in 23.8% of the patients and was mild in the remainder. The mean score on the Harris scale was 51 out of 100 points. Conclusions: failure of hip arthroplasty requiring Girdlestone resection is very damaging to functional outcome in elderly patients (AU)