RESUMEN
Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI. We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes. The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. Use of fetal echocardiography with measurement of pulmonary artery size combined with prenatal MRI scanning of lung volumes resulted in an improved understanding of this anomaly and directed the management strategy towards a successful Fontan circulation.
Asunto(s)
Anomalía de Ebstein/diagnóstico , Pulmón/embriología , Diagnóstico Prenatal/métodos , Arteria Pulmonar/embriología , Insuficiencia de la Válvula Tricúspide/diagnóstico por imagen , Válvula Tricúspide/anomalías , Adulto , Cardiomegalia/diagnóstico por imagen , Cardiomegalia/embriología , Cardiomegalia/cirugía , Puente Cardiopulmonar , Anomalía de Ebstein/cirugía , Femenino , Enfermedades Fetales/diagnóstico por imagen , Edad Gestacional , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/cirugía , Humanos , Recién Nacido , Pulmón/diagnóstico por imagen , Imagen por Resonancia Magnética , Embarazo , Arteria Pulmonar/cirugía , Válvula Tricúspide/embriología , Válvula Tricúspide/cirugía , Insuficiencia de la Válvula Tricúspide/cirugía , Ultrasonografía PrenatalRESUMEN
OBJECTIVE: To describe the outcome and treatment of two patients with recombinant factor VIIa (rFVIIa) for severe hemorrhage associated with extracorporeal membrane oxygenation (ECMO). DESIGN: Case report. SETTING: A 38-bed pediatric intensive care unit and 20-bed pediatric cardiac intensive care unit at a tertiary care children's hospital. PATIENT: Two patients with life-threatening hemorrhagic complications associated with ECMO requiring massive transfusion of blood products. INTERVENTIONS: Administration of repeated doses of rFVIIa at 90 microg/kg/dose. MEASUREMENT AND MAIN RESULTS: PATIENT 1 was an 11-yr-old male with a dilated cardiomyopathy who had undergone an orthotopic heart transplant treated with venoarterial ECMO postoperatively for right ventricular dysfunction. PATIENT 2 was a 13-yr-old male treated with venoarterial ECMO for cardiopulmonary failure from necrotizing staphylococcal pneumonia. Both patients had severe hemorrhage from the cannulation sites and thoracostomy tubes requiring massive transfusion to maintain intravascular blood volume and replace clotting factors. Both patients were treated with rFVIIa every 2-4 hrs and attained hemostasis. PATIENT 1 was administered three doses and PATIENT 2 was administered ten doses. No evidence of abnormal thrombus formation was noted in their respective ECMO circuits. CONCLUSIONS: The efficacy of rFVIIa in reducing intractable bleeding postcardiac surgery and in other coagulopathic states is being investigated. Despite theoretical concerns of thrombosis, these cases illustrate that there may be a role for the cautious use of rFVIIa in treating severe and intractable hemorrhage associated with ECMO.
Asunto(s)
Oxigenación por Membrana Extracorpórea/efectos adversos , Factor VII/uso terapéutico , Hemorragia/tratamiento farmacológico , Hemostáticos/uso terapéutico , Adolescente , Niño , Factor VIIa , Hemorragia/etiología , Humanos , Unidades de Cuidado Intensivo Pediátrico , Masculino , Proteínas Recombinantes/uso terapéuticoRESUMEN
Septic shock presents a unique challenge in the pediatric patient. Sepsis stimulates the release of inflammatory mediators that can compromise cardiac function. Oxygen extraction abnormalities, diminished responses to adrenergic agonists, and impaired ventricular function often result. After fluid resuscitation and antibiotic therapy, careful cardiovascular assessment is needed to administer appropriate inotropic and vasoactive drugs.
Asunto(s)
Gasto Cardíaco Bajo/tratamiento farmacológico , Cardiomiopatías/tratamiento farmacológico , Cardiotónicos/uso terapéutico , Choque Séptico/complicaciones , Agonistas Adrenérgicos beta/uso terapéutico , Gasto Cardíaco Bajo/diagnóstico , Gasto Cardíaco Bajo/etiología , Cardiomiopatías/diagnóstico , Cardiomiopatías/etiología , Niño , Humanos , Inhibidores de Fosfodiesterasa/uso terapéutico , Choque Séptico/fisiopatología , Vasoconstrictores/uso terapéuticoRESUMEN
BACKGROUND: Management strategies for preoperative infants with hypoplastic left heart syndrome (HLHS) include increased inspired nitrogen (hypoxia) and increased inspired carbon dioxide (hypercarbia). There are no studies directly comparing these 2 therapies in humans. This study compares the impact of hypoxia versus hypercarbia on oxygen delivery, under conditions of fixed minute ventilation. METHODS AND RESULTS: Ten anesthetized and paralyzed preoperative infants with HLHS were evaluated in a prospective, randomized, crossover trial comparing hypoxia (17% FIO(2)) with hypercarbia (2.7% FICO(2)). Each patient was treated in a random order (10 minutes per condition) with a recovery period (15 to 20 minutes) in room air. Arterial (SaO(2)) and superior vena caval (SvO(2)) co-oximetry and cerebral oxygen saturation (ScO(2)) measurements were made at the end of each condition and recovery period. ScO(2) was measured by near infrared spectroscopy. Hypoxia significantly decreased both SaO(2) (-5.2+/-1.1%, P=0.0014) and SvO(2) (-5.6+/-1.7%, P=0.009) compared with baseline, but arteriovenous oxygen saturation (AVO(2)) difference (SaO(2)-SvO(2)) and ScO(2) remained unchanged. Hypercarbia decreased SaO(2) (-2.6+/-0.6%, P=0.002) compared with baseline but increased both ScO(2) (9.6+/-1.8%, P=0.0001) and SvO(2) (6+/-2.2%, P=0.022) and narrowed the AVO(2) difference (-8.5+/-2.3%, P=0.005). Both hypoxia and hypercarbia decreased the balance between pulmonary and systemic blood flow (Qp:Qs) compared with baseline. CONCLUSIONS: In preoperative infants with HLHS, under conditions of anesthesia and paralysis, although Qp:Qs falls in both conditions, oxygen delivery is unchanged during hypoxia and increased during hypercarbia. These data cannot differentiate cerebral from systemic oxygen delivery.
Asunto(s)
Dióxido de Carbono/administración & dosificación , Síndrome del Corazón Izquierdo Hipoplásico/terapia , Nitrógeno/administración & dosificación , Oxígeno/administración & dosificación , Cuidados Preoperatorios , Respiración Artificial/métodos , Administración por Inhalación , Anestesia , Arterias/fisiopatología , Monitoreo de Gas Sanguíneo Transcutáneo/instrumentación , Encéfalo/metabolismo , Estudios Cruzados , Hemodinámica/efectos de los fármacos , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Hipoxia/sangre , Hipoxia/inducido químicamente , Recién Nacido , Monitoreo Fisiológico , Oxígeno/análisis , Oxígeno/metabolismo , Estudios Prospectivos , Espectroscopía Infrarroja CortaRESUMEN
BACKGROUND: Cardiopulmonary bypass (CPB) and hypothermic circulatory arrest (HCA) are important components of congenital cardiac surgery. Ischemia/reperfusion injury and inflammatory cascade activation result in endothelial damage and vascular leak which are clinically manifested as pulmonary edema and low cardiac output postoperatively. Newborns are particularly susceptible. Subtraction cloning is a useful method of isolating induced genes and can be applied to CPB/HCA. MATERIALS AND METHODS: We used a newborn lamb model replicating infant CPB with HCA to obtain tissues during various periods of reperfusion. We utilized subtraction cloning to identify mRNA induced in lung following CPB/HCA and reperfusion. Ribonuclease protection was used to quantify mRNA levels. RESULTS: We isolated a cDNA encoding ovine aquaporin-1 in a subtracted cDNA screen comparing control lung with lung exposed to CPB/HCA and reperfusion. Aquaporin-1 mRNA levels increased 3-fold in lung (p = .006) exposed to CPB/HCA and 6 hr of reperfusion. No induction was observed immediately following bypass or after 3 hr of reperfusion. We found no significant induction of aquaporin-1 mRNA following bypass, arrest, and reperfusion in other tissues surveyed, including ventricle, atrium, skeletal muscle, kidney, brain, and liver. CONCLUSIONS: Our finding that aquaporin-1 mRNA is reproducibly induced in lung following CPB/HCA with 6 hr of reperfusion suggests an important role for the water channel in the setting of pulmonary edema. Induction of Aquaporin-1 is late compared with other inflammatory mediators (ICAM-1, E-selectin, IL-8). Further studies are needed to determine if aquaporin-1 contributes to the disease process or if it is part of the recovery phase.
Asunto(s)
Acuaporinas , Puente Cardiopulmonar , Regulación de la Expresión Génica , Canales Iónicos/genética , Pulmón/metabolismo , Daño por Reperfusión Miocárdica/genética , Secuencia de Aminoácidos , Animales , Animales Recién Nacidos , Acuaporina 1 , Clonación Molecular , ADN Complementario , Paro Cardíaco Inducido , Hemodinámica , Datos de Secuencia Molecular , ARN Mensajero/genética , ARN Mensajero/metabolismo , Ovinos , Agua/metabolismoRESUMEN
OBJECTIVE: The purpose of this study was to assess morbidity and mortality associated with delayed sternal closure after pediatric cardiac operations. METHODS: Hospital records were reviewed of all patients with an open sternum after a cardiac operation at Children's Hospital, Boston, from January 1992 to December 1995. RESULTS: A total of 178 patients had delayed sternal closure with an overall mortality rate of 19%. The most common diagnosis of patients with delayed sternal closure was hypoplastic left heart syndrome (29%). Although myocardial distention or chest wall edema (n = 47) was a common indication to delay sternal closure, in many patients (n = 47) the sternum was left open electively to avoid postoperative cardiac or respiratory compromise. Successful sternal closure was achieved in 158 patients (89%) at a mean of 3.4 +/- 1.8 days after opening. There were significant increases in left atrial pressure (7.7 +/- 3.4 to 9.8 +/- 4.1 mm Hg, p = 0.00001) and right atrial pressure (8 +/- 3.2 to 10.1 +/- 3.3 mm Hg, p = 0.00001) with sternal closure. There was a small but statistically significant drop in pH (7.44 +/- 0.05 to 7.41 +/- 0.08, p < 0.0001) during sternal closure. The peak inspiratory pressure, delivered breaths per minute, and fraction of inspired oxygen all significantly increased during sternal closure. Clinical evidence of surgical site infection occurred in 12 (6.7%) of the patients with delayed sternal closure; mediastinitis developed in 7 (3.9%) patients. CONCLUSIONS: Although delayed sternal closure after complex operations for congenital heart disease is often necessary in the operating room because of edema, unstable hemodynamic conditions, or bleeding, it can also be used electively to aid in hemodynamic and respiratory stability in the initial postoperative period. Our review supports a low morbidity associated with delayed sternal closure in a pediatric population.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías Congénitas/cirugía , Complicaciones Posoperatorias/prevención & control , Esternón/cirugía , Vendajes , Edema/etiología , Edema/prevención & control , Cardiopatías Congénitas/fisiopatología , Hemodinámica , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Recién Nacido , Morbilidad , Periodo Posoperatorio , Estudios Retrospectivos , Infección de la Herida Quirúrgica/prevención & control , Factores de TiempoRESUMEN
Techniques of transcatheter device placement for treatment of pediatric congenital heart disease have developed substantially since their introduction 20 years ago. Patent ductus arteriosus occlusion can be accomplished by umbrella deployment or coil placement. Intracardiac defects can be closed with umbrella or buttoned devices. Stenoses of vessels or conduits that are only temporarily relieved with balloon dilation can be effectively expanded with intravascular stents. Recent procedural modifications have been introduced in an attempt to minimize the size of the delivery sheath and reduce complications that can arise from device embolization. Transcatheter device placement can be an important adjunct to surgery for correction or palliation of congenital heart lesions.
Asunto(s)
Cateterismo Cardíaco/métodos , Conducto Arterioso Permeable/terapia , Cardiopatías Congénitas/terapia , Prótesis e Implantes , Cateterismo , Ecocardiografía Transesofágica , Defectos del Tabique Interventricular/terapia , Humanos , StentsRESUMEN
Phospholipid antibodies (lupus anticoagulant, cardiolipin) are associated with a syndrome of repeated fetal loss. Mothers with phospholipid antibodies are currently being treated with either prednisone, aspirin, or heparin to prevent fetal death. We describe a neonate whose mother had cardiolipin antibody and recurrent fetal loss and was treated with prednisone and aspirin. Thrombosis was noted in placental fetal vessels. Thromboses developed in the infant's aorta, left renal artery, middle cerebral artery, and superior sagittal sinus. Infants of phospholipid-positive mothers may have vascular thrombosis and should be carefully monitored for signs of thromboembolism.