RESUMEN
We present the successful perioperative management of an adult patient with Ebstein's anomaly for abdominal rectopexy surgery. The patient developed mild hypotension and a fall in peripheral oxygen saturation (SpO 2 ) after administration of a graded epidural block. Correction of the fall in the blood pressure; however, did not improve the SpO 2 . The patient was administered an intravenous infusion of dopamine to improve the cardiac output and this led to improvement in the SpO 2 .
Asunto(s)
Cardiotónicos/administración & dosificación , Dopamina/administración & dosificación , Anomalía de Ebstein/sangre , Defectos del Tabique Interatrial/sangre , Oxígeno/sangre , Anomalía de Ebstein/complicaciones , Anomalía de Ebstein/fisiopatología , Femenino , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/fisiopatología , Humanos , Infusiones Intravenosas , Persona de Mediana EdadRESUMEN
AIMS: Patients with Ebstein's anomaly of the tricuspid valve may have right-to-left shunt at atrial level resulting in hypoxaemia, high haematocrit and hyperviscosity syndrome. The purpose of this study was to assess the results of percutaneous closure of atrial right-to-left shunt in patients with Ebstein's anomaly. METHODS AND RESULTS: Records of patients treated between January 2002 and June 2010 were reviewed. Their condition before and after shunt closure (clinical data, oxygen saturation and haematocrit) were studied. During this period nine selected patients with Ebstein's anomaly and right-to-left shunt at atrial level were treated. Ages ranged from six to 67 years; seven were male. Mean pulmonary artery pressures were under 25 mmHg in all. Three patients had previous episodes of stroke and three had very high haematocrit, two of whom required therapeutic phlebotomies. Test occlusion of the shunt was performed in all patients with a balloon catheter, revealing an increase in systemic oxygen saturation, with right atrial pressures remaining <18 mmHg in all. Percutaneous closure of atrial shunt was achieved in all. There were no major complications. Arterial oxygen saturations increased in all patients from 85.0 ± 4.5% to 96.7 ± 1.5% (mean ± standard deviation). Medium follow-up was five years. The three patients with very high haematocrit levels had a decrease in its values from 62.9 ± 2.8% to 45.5 ± 3.9% after device occlusion. Both therapeutic phlebotomy programs were discontinued. All patients reported a marked improvement in effort tolerance. CONCLUSIONS: Percutaneous closure of atrial right-to-left shunt in selected patients with Ebstein's anomaly offers significant improvement, abolishing hypoxaemia and hyperviscosity and preventing paradoxical embolisation.
Asunto(s)
Función Atrial , Cateterismo Cardíaco , Anomalía de Ebstein/complicaciones , Foramen Oval Permeable/terapia , Defectos del Tabique Interatrial/terapia , Válvula Tricúspide/anomalías , Adolescente , Adulto , Anciano , Presión Sanguínea , Viscosidad Sanguínea , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Niño , Anomalía de Ebstein/sangre , Anomalía de Ebstein/fisiopatología , Embolia Paradójica/etiología , Embolia Paradójica/terapia , Femenino , Foramen Oval Permeable/sangre , Foramen Oval Permeable/complicaciones , Foramen Oval Permeable/fisiopatología , Defectos del Tabique Interatrial/sangre , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/fisiopatología , Hematócrito , Humanos , Hipoxia/etiología , Hipoxia/terapia , Masculino , Persona de Mediana Edad , Oxígeno/sangre , Portugal , Diseño de Prótesis , Estudios Retrospectivos , Dispositivo Oclusor Septal , Factores de Tiempo , Resultado del Tratamiento , Válvula Tricúspide/fisiopatología , Adulto JovenRESUMEN
Ebstein's anomaly is a rare congenital heart defect. Associated lesions are uncommon, and the mortality rates can be as high as 54% during the first month of life. Two cases of severe Ebstein's anomaly with ventricular septal defect are described. It is speculated that this rare association, allowing adequate forward pulmonary blood flow in the neonate, permitted the reported patients to survive the neonatal period, which is the most life-threatening time. The authors propose that the presence of a small ventricular septal defect can be beneficial for such patients, averting the need for surgery during early infancy when the risk is highest.
Asunto(s)
Anomalía de Ebstein/epidemiología , Defectos del Tabique Interventricular/epidemiología , Comorbilidad , Circulación Coronaria , Anomalía de Ebstein/sangre , Anomalía de Ebstein/diagnóstico por imagen , Anomalía de Ebstein/fisiopatología , Electrocardiografía , Femenino , Defectos del Tabique Interventricular/sangre , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/fisiopatología , Humanos , Lactante , Oxígeno/sangre , UltrasonografíaRESUMEN
INTRODUCTION: Ebstein's anomaly is defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle. The aim of the study was to evaluate exercise capacity with cardiopulmonary stress testing and to determine plasma BNP levels in adults with Ebstein's anomaly, and to establish their relation with echocardiogaphic grading of the lesion severity. MATERIALS AND METHODS: Study group consisted of 21 patients (16 males, aged 40.3+/-11.5 years). The control group: 19 healthy individuals (13 males, aged mean 39.9+/-9.3 years). On echocardiography the grade of the lesion severity was calculated (EGE) and used to define the following four groups: I < 0.5, II: 0.5-0.9, III: 1.0-1.49, IV > 1.5. The forced vital capacity (FVC), first second forced expiratory volume (FEV1), peak oxygen uptake (peak VO2), and VE/VCO2 slope were assessed with cardiopulmonary stress test and plasma BNP levels measured with radioimmunometric assay. RESULTS: In the studied group VO2 was lower than in control (21.9+/-5.4 vs. 33.6+/-8.3 mL/kg/min [p = 0.00001]), VE/VCO2 slope was higher in Ebstein's group (40.1+/-8.1, p = 0.00001). BNP levels were higher in the Ebstein group then in controls (35.9+/-25.0 vs. 17.2+/-9.9 pg/mL [p = 0.0002]) and did not differ significantly between EGE groups. PeakVO2 of 24.5+/-3.9 in patients from II EGE group were higher than in patients from EGE groups: III (17.2+/-5.2 p = 0.007) and IV (22.9+/-4.7 p = 0.05). CONCLUSIONS: Exercise capacity of adults with Ebstein's anomaly is significantly reduced and plasma BNP levels are higher compared to healthy individuals. Exercise capacity in patients with Ebstein's anomaly becomes gradually lower alongside the EGE severity; however, BNP levels do not correlate significantly with this parameter.
Asunto(s)
Anomalía de Ebstein/sangre , Anomalía de Ebstein/fisiopatología , Prueba de Esfuerzo , Péptido Natriurético Encefálico/sangre , Adulto , Tolerancia al Ejercicio , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
In young patients with Ebstein's anomaly and symptoms of congestive heart failure or severe cyanosis tricuspid valve repair may be unsuccessful and prosthetic valve replacement is needed, which is associated with a high operative mortality. This report describes the implantation of a valved aortic homograft as a conduit between right atrium and pulmonary artery to at least postpone valve surgery in three symptomatic children (4.1, 8.3, and 10 years of age) with an arterial oxygen saturation of 75 to 82%. The intracardiac anatomy was left untouched except for closure of the associated secundum atrial septal defect. One patient with stable postoperative hemodynamics died of severe endobronchial bleeding on the 2nd postoperative day. Due to the increase of antegrade pulmonary blood flow via the conduit the arterial oxygen saturation rose to 92% in the two surviving patients. Postoperative hemodynamic evaluation revealed low right atrial and mean pulmonary artery pressures (12 mmHg). Pulsed Doppler ultrasound sampling of flow in the conduit demonstrated antegrade flow during atrial and ventricular systole. In symptomatic patients with severe malformation of the tricuspid valve a partial modified Fontan procedure offers a promising palliation avoiding tricuspid valve surgery in early childhood.
Asunto(s)
Válvula Aórtica/trasplante , Anomalía de Ebstein/cirugía , Procedimiento de Fontan/métodos , Niño , Preescolar , Anomalía de Ebstein/sangre , Anomalía de Ebstein/diagnóstico por imagen , Estudios de Seguimiento , Procedimiento de Fontan/mortalidad , Hemodinámica , Humanos , Trasplante Homólogo , Resultado del Tratamiento , UltrasonografíaRESUMEN
For chronic fetal hypoxia due to maternal Ebstein's anomaly, oxygen was administered daily to the mother by mask for 105 days. At 20 weeks of gestation, umbilical venous blood gases in room air showed pH 7.42, PO2 25.7 mm Hg, PCO2 33.7 mm Hg and O2 saturation 48.7%, and changed to 7.45, 39.1 mm Hg, 25.9 mm Hg and 77.4% on 3 liters/min of oxygen inhalation by mask, respectively. The PO2 of the maternal arterial blood gases increased to 30 mm Hg on oxygen administration at 15 weeks of gestation, but at 25 weeks of gestation the PO2 increased by only about 10 mm Hg. At 30 weeks, intrauterine growth retardation was suspected. Just after the second puncture of the umbilical cord at 31 weeks and 3 days of gestation, 80 bpm fetal bradycardia occurred for several minutes without recovery and emergency cesarean section was done under the general anesthesia.