Double Outlet Right Ventricle

Background. To determine outcomes for the arterial switch operation individualized according to the under- lying anatomy and clinical status. Methods. A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. Results. From July 1993-April 2001, 117 infants under- went an arterial switch operation before 90

Results of the arterial switch operation (ASO) for transposition of the great arteries (TGA) with large ventricular septal defect (VSD) and for Taussig Bing anomaly (TBA) in a tertiary care center of a developing country were retrospectively analyzed. From January 2007 through June 2013, a total of 30 patients with TBA and 54 patients having TGA with large VSD underwent ASO with VSD closure. Age at surgery for TBA was 27 days to 7 years (median age 3 months) and for TGA with VSD it was 1 day to 6 years (median age 2 months). In all, 46.7% of patients with TBA and 30% of patients with TGA/VSD presented with sepsis secondary to pneumonitis and were taken for surgery when the infection was under control. In all, 13% of patients with TBA and 5.5% of patients with TGA/VSD were on intermittent positive pressure ventilation (IPPV) prior to and continuing up to the time of surgery. On multivariate analysis, factors associated with mortality were the presence of preoperative IPPV for pneumon...

In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the "Taussig-Bing anomaly" (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventric...

La venta libre de productos cosméticos con nanopartículas de oro se ha incrementa-do considerablemente en los últimos años. Adicionalmente, las nanopartículas de oro se posicionan como opciones terapéuticas principalmente contra el cáncer. Sin embargo, no se conoce a fondo los posibles efectos tóxicos de estas nanopartículas en células ni organismos sanos, tampoco los posibles efectos teratogénicos durante el desarrollo. El proceso de septación cardiaca, tanto en aves como mamíferos, consiste en pasar de una circulación unidireccional a una bidireccional, evento altamente sensible al estrés oxidante. Razón por la cual nos propusimos conocer los posibles efectos teratogénicos de las nanopartículas de oro durante el desarrollo cardiaco en el embrión de pollo, modelo muy usado que permite la manipulación directa del embrión. En el estudio encontramos: 1. Un incremento de 7.9 veces la tasa de mortalidad por exposición a las nanopartículas de oro contra el grupo control. 2. Defectos en l...

Between 1984 and 1996 five infants underwent surgical repair of pulmonary artery sling associated with severe congenital tracheal stenosis. All infants had symptoms of severe respiratory distress and three of them required ventilator support preoperatively ages ranged from 2 to 11 months (mean age 6 months). Complete tracheal rings were present in all patients as an associated lesion and right upper lobe tracheal bronchus in 3 patients. The length of tracheal stenosis ranged from 18 to 45 mm (median 40 mm). Three had associated intracardiac anomalies (Scimitar syndrome (1), VSD (1), double-outlet right ventricle with VSD (1), double-outlet right ventricle with pulmonary hypertension (1)). Surgical intervention was carried out through a right thoracotomy (1) or median sternotomy (4). Cardiopulmonary bypass (CPB) was used in 3 patients and extracorporeal membrane oxgenator (ECMO). In 1. All infants had reimplantation of the left pulmonary artery into the main pulmonary artery left ant...

Objective: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. Methods: Between January 1987 and December 1997, 23 patients having double-outlet right

Double outlet right ventricle (DORV) was produced experimentally in the chick embryo by preventing the incorporation of the posteromedial conus into the left ventricle. This was accomplished by placing a ligature around the caudal end of the conus at a stage in which it is exclusively continuous with the right ventricle. The embryos were divided into two groups according to

Fontan operation and importance of fenestration in the treatment of unusual and complex forms of double outlet right ventricle (DORV) are well established. Nonetheless, rarely, the creation of fenestration becomes challenging in complex morphologies. We present one such child with situs solitus, dextrocardia, DORV, hypoplastic right ventricle, large ventricular septal defect, severe pulmonic stenosis, extremely small right atrium and left juxtaposed atrial appendages, who underwent Fontan operation. We created an unusual fenestration between left pulmonary artery and juxtaposed right atrial appendage on the left side, due to anatomic complexity. Short-term results are encouraging.

Current MR techniques enable both anatomical and functional evaluations of the heart. Although it is rarely used as a primary diagnostic tool in pediatric cardiology, it can be used as a valuable adjunct to echocardiography and angiography. MRI is particularly useful in clarification of morphology of complex congenital heart diseases. It is the most accurate method of determination of visceral and atrial situs. It is easy to evaluate the systemic and pulmonary venous connections that are very important for the Fontan type of operation, especially in patients with visceral heterotaxy. It facilitates demonstration of diminutive pulmonary arteries in patients with pulmonary atresia. It clearly demonstrates juxtaposition of the atrial appendages that is often missed by echocardiography. The anatomy of the twisted atrioventricular connections is much more clear in MRI than in echocardiography. It enables en face imaging of ventricular septal defect that provides a surgical view. We find ...

Tectocerebellar dysraphia is a rare constellation of malformations comprising of occipital encephalocele, aplasia of the cerebellar vermis and deformity of the tectum. We describe a 7 month old infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.

GATA transcription factors, together with Friend of GATA (FOG) cofactors, are required for the differentiation of diverse cell types. Multiple aspects of hematopoiesis are controlled by the interaction of FOG-1 with the GATA-1/2/3 subfamily. Likewise, FOG-2 is coexpressed with the GATA-4/5/6 subfamily at other sites, including the heart and gonads. FOG-2 and GATA-4 are required for cardiac development. Through transgenic rescue of hematopoietic defects of FOG-1-/- embryos we define an unsuspected role for FOG-1 in heart development. In particular, rescued FOG-1-/- mice die at embryonic day (E) 14.5 with cardiac defects that include double outlet right ventricle and a common atrioventricular valve. Using conditional inactivation of Fog-1 we assign the cell of origin in which FOG-1 function is required. Neural crest cells migrate properly into FOG-1-/- hearts and mice with FOG-1 conditionally excised from neural crest derivatives fail to develop cardiac abnormalities. In contrast, con...

Management of native aortic coarctation during early months of life poses therapeutic challenges, and there is no consensus among medical professionals regarding a management plan. Much can be argued about the benefits, limitations, and/or complications of transcatheter versus surgical intervention in such cases. Occasionally, the complexity of the lesions limits management options. Therefore, each patient requires individual management decisions because there is no one therapeutic plan that satisfies all patients. In this report, four critically ill infants who had complex native coarctation are presented. Surgical repair was not possible because of relative contraindications. The patients underwent transcatheter stent implantation (six procedures and seven stents) as a nondefinitive procedure with acceptable results. Three patients improved. One patient did not survive, mainly due to other major complications. Multiple reexpansions of the stents were carried out when indicated. After a mean follow-up of 45 months (range, 41–49), the three survivors were doing fine and had gained an average weight of 9.7 kg (range, 6.6–13.3). At the time of reporting, the relative contraindications no longer exist and the final surgical repair can be carried out. Our experience suggests that in certain situations and in critically ill infants with complex form of coarctation, stent angioplasty can be used as a life-saving palliative procedure. Further reexpansions can be done when required. This may serve as a bridge to major surgical repair in the future.