RESUMEN
Children with trisomy 13 and 18 (previously deemed "incompatible with life") are living longer, warranting a comprehensive overview of their unique comorbidities and complex care needs. This Review Article provides a summation of the recent literature, informed by the study team's Interdisciplinary Trisomy Translational Program consisting of representatives from: cardiology, cardiothoracic surgery, neonatology, otolaryngology, intensive care, neurology, social work, chaplaincy, nursing, and palliative care. Medical interventions are discussed in the context of decisional-paradigms and whole-family considerations. The communication format, educational endeavors, and lessons learned from the study team's interdisciplinary care processes are shared with recognition of the potential for replication and implementation in other care settings.
Asunto(s)
Cromosomas Humanos Par 18 , Cuidados Paliativos/organización & administración , Grupo de Atención al Paciente , Síndrome de la Trisomía 13 , Trisomía , Defensa del Niño , Toma de Decisiones Clínicas , Discapacidades del Desarrollo/genética , Discapacidades del Desarrollo/terapia , Nutrición Enteral , Femenino , Monitoreo Fetal , Cardiopatías Congénitas/genética , Cardiopatías Congénitas/terapia , Humanos , Alimentos Infantiles , Trastornos de la Nutrición del Lactante/prevención & control , Recién Nacido , Cuidado Intensivo Neonatal/métodos , Comunicación Interdisciplinaria , Esperanza de Vida , Masculino , Hipotonía Muscular/genética , Hipotonía Muscular/terapia , Neoplasias/complicaciones , Diagnóstico Prenatal , Relaciones Profesional-Familia , Síndrome de la Trisomía 13/diagnóstico , Síndrome de la Trisomía 13/embriología , Síndrome de la Trisomía 13/terapiaRESUMEN
OBJECTIVES: Children with congenital heart disease may require long-term central venous access for intensive care management; however, central venous access must also be preserved for future surgical and catheterization procedures. Transhepatic venous catheters may be an useful alternative. The objective of this study was to compare transhepatic venous catheters with traditional central venous catheters regarding complication rate and duration of catheter service. DESIGN: Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters. SETTING: Single freestanding pediatric hospital located in the central United States. PATIENTS: Pediatric patients with congenital heart disease who underwent placement of transhepatic venous catheter. INTERVENTIONS: Cohort's central venous catheter complication rates and duration of catheter service were compared with transhepatic venous catheter data. MEASUREMENTS AND MAIN RESULTS: Twelve patients had a total of 19 transhepatic venous lines. Transhepatic venous lines had a significantly longer duration of service than central venous lines (p = 0.001). No difference between the two groups was found in the number of documented thrombi, thrombolytic burden, or catheter sites requiring wound care consultation. A higher frequency of infection in transhepatic venous lines versus central venous lines was found, isolated to four transhepatic venous lines that had a total of nine infections. All but one was successfully managed without catheter removal. The difference in the proportion of infections to catheters in transhepatic venous lines versus central venous lines was significant (p = 0.0001), but no difference in the rate of infection-related catheter removal was found. CONCLUSIONS: Without compromising future central venous access sites, transhepatic venous lines had superior duration of service without increased thrombosis, thrombolytic use, or insertion site complications relative to central venous lines. Transhepatic venous catheters had a higher infection rate, and further investigation into the etiology is warranted.