RESUMEN
A 12-year-old male presented with a 6-week history of apparent digital vasculitis with color changes of the distal fingers and erythematous macules of the palms and soles. Physical examination revealed skin findings of Carney complex and an abnormal cardiac examination. Echocardiography demonstrated a large left atrial mass, which caused partial obstruction during diastole and moderate mitral valve insufficiency. Surgical excision and pathological examination of the mass confirmed the presence of a large, peduculated myxoma. This case illustrates the presentation of Carney complex with left atrial myxoma as apparent vasculitis and Raynaud's phenomenon.
Asunto(s)
Atrios Cardíacos , Neoplasias Cardíacas/complicaciones , Mixoma/complicaciones , Síndromes Neoplásicos Hereditarios/complicaciones , Enfermedad de Raynaud/etiología , Enfermedades Cutáneas Vasculares/etiología , Niño , Ecocardiografía , Neoplasias Cardíacas/diagnóstico por imagen , Humanos , Masculino , Mixoma/diagnóstico por imagenRESUMEN
BACKGROUND: Patch enlargement of severe branch pulmonary artery stenosis (PAS) or pulmonary vein ostial stenosis (PVS) can be technically challenging. Recurrences are common and exposure may require long periods of cardiopulmonary bypass (CPB). METHODS: Since 1993, we performed 31 procedures on 27 patients with endovascular stents placed intraoperatively under direct surgical vision: 22 patients with tight PAS and 5 patients with PVS. Selection for intraoperative (vs catheterization laboratory) stent placement was prompted by: (1) the need for a concomitant cardiac surgical procedure (16 cases); (2) limited vascular access for catheterization laboratory stent placement (11 cases); or (3) "rescue" of patients with complications after attempted placement of stents (4 cases). RESULTS: In this group of very complex and challenging patients there were 5 hospital deaths (hospital survival, 81%). Follow-up of survivors has ranged from 1 month to 7 years (mean 2.8 +/- 1.7 years). There have been 3 late deaths (late "series" survival, 70%). No complication or death was related to stent placement. Surviving patients have had significant clinical improvement. Mean pulmonary gradient (postoperative vs preoperative echo) has fallen in all survivors and has decreased from a mean of 66 mm Hg preoperatively to 28 mm Hg postoperatively (p = 0.01). All pulmonary arteries are appreciably enlarged and will be easier to deal with at a later date if necessary. One patient (DORV, HLHS ) with pulmonary vein stents has gone on to a successful Glenn procedure. The other two surviving patients with PV stents have occlusion of their proximal PVs on follow-up catheterization; thus only 1 of 5 patients with stents for PVS has had a successful outcome. Four patients have had repeat surgery. Stents have produced no impediment to subsequent surgical procedures, and the pulmonary arteries were easy to work with. CONCLUSIONS: Intraoperative stenting provides an attractive option for "rehabilitation" of pulmonary vessels. Direct vision insertion on CPB is extremely quick and immediately effective, limiting the CPB exposure required to treat this problem. Once stented, vessels remain open and are amenable to future surgical intervention as necessary. Outcome is better for patients with PAS versus those with PVS.
Asunto(s)
Arteriopatías Oclusivas/cirugía , Cardiopatías Congénitas/cirugía , Arteria Pulmonar/cirugía , Enfermedad Veno-Oclusiva Pulmonar/cirugía , Stents , Adolescente , Arteriopatías Oclusivas/diagnóstico por imagen , Arteriopatías Oclusivas/mortalidad , Niño , Preescolar , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/mortalidad , Mortalidad Hospitalaria , Humanos , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias/mortalidad , Arteria Pulmonar/diagnóstico por imagen , Enfermedad Veno-Oclusiva Pulmonar/diagnóstico por imagen , Enfermedad Veno-Oclusiva Pulmonar/mortalidad , Radiografía , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
OBJECTIVE: The aim of this study is to determine the level of clinical auscultation skills in pediatric residents at Duke University Medical Center. METHODS: Forty-seven residents from pediatrics and joint medicine/pediatrics training programs at Duke University Medical Center were enrolled in this study. They were asked to examine the cardiovascular patient simulator, Harvey, and report their findings. Five common conditions seen in the pediatric population were presented: ventricular septal defect, atrial septal defect, pulmonary valve stenosis, combined aortic valve stenosis and insufficiency, and innocent systolic ejection murmur. The responses were scored by the number of features and diagnoses accurately reported. Five pediatric cardiologists and cardiologists in training were also asked to participate in a manner similar to the trainees. RESULTS: The mean score of features identified for the resident group was 11.4 +/- 2.6 of a possible 19. The diagnostic accuracy was 33%. There was no significant difference between residents by year of training or by type of residency program, although there was a trend toward improved performance with more training. The difference in performance between the pediatric cardiology group and the residents group was striking. The condition that was most frequently misdiagnosed was the innocent systolic ejection murmur. CONCLUSIONS: The clinical auscultation skills of pediatric residents in this study were suboptimal. There was a trend toward improvement as training progressed, although not statistically significant. These skills are likely to improve further with increased exposure to patients with cardiovascular disease especially in the ambulatory care setting.
Asunto(s)
Competencia Clínica , Auscultación Cardíaca , Cardiopatías/diagnóstico , Internado y Residencia , Pediatría/educación , Adulto , Insuficiencia de la Válvula Aórtica/diagnóstico , Estenosis de la Válvula Aórtica/diagnóstico , Niño , Soplos Cardíacos/diagnóstico , Defectos del Tabique Interatrial/diagnóstico , Defectos del Tabique Interventricular/diagnóstico , Humanos , Estenosis de la Válvula Pulmonar/diagnósticoRESUMEN
We report the case of a neonate who developed ventriculitis following insertion of a ventriculoperitoneal shunt. Corynebacterium xerosis was isolated from CSF and from the tip of the catheter after it was removed. The isolate was resistant to multiple antibiotics, but the infant responded to treatment with vancomycin.