Background-Transposition of the great arteries (TGA) is a life-threatening malformation in neonates, but it is amenable to complete repair. Prenatal detection, diagnosis, and early management may modify neonatal mortality and mortality. Methods and Results-Preoperative and postoperative morbidity and mortality were compared in 68 neonates with prenatal diagnosis and in 250 neonates with a postnatal diagnosis of TGA over a period of 10 years. The delay between birth and admission was 2Ϯ2.8 hours in the prenatal group and 73Ϯ210 hours in the neonatal group (PϽ0.01). Clinical condition at arrival, including metabolic acidosis and multiorgan failure, was worse in the neonatal group (PϽ0.01). Once in the pediatric cardiology unit, the management was identical in the 2 groups (atrioseptostomy, PGE 1 infusion, operation date). Preoperative mortality was 15 of 250 (6%; 95% CI, 3% to 9%) in the neonatal group and 0 of 68 in the prenatal group (PϽ0.05). Postoperative morbidity was not different (25 of 235 versus 6 of 68), but hospital stay was longer in the neonatal group (30Ϯ17 versus 24Ϯ11 days, PϽ0.01). In addition, postoperative mortality was significantly higher in the neonatal group (20 of 235 versus 0 of 68, PϽ0.01); however, the known risk factors for operative mortality were identical in the 2 groups. Conclusions-Prenatal diagnosis reduces mortality and morbidity in TGA. Prenatal detection of this cardiac defect must be increased to improve early neonatal management. In utero transfer of fetuses with prenatal diagnosis of TGA in an appropriate unit is mandatory. (Circulation. 1999;99:916-918.)

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Congenital ventricular diverticulum and aneurysm are two distinct entities, with different histologic and morphologic characteristics and outcomes. Assessment of these differential characteristics is of importance for prenatal counseling.

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Non-surgical insertion of the PV is possible without any major complications. This new technique may have an important role in the management of conduit obstructions and pulmonary regurgitation.

Background-Although prenatal diagnosis of transposition of the great arteries (TGA) reduces neonatal mortality, the preoperative course can be complicated in infants with a restrictive foramen ovale (FO) or a ductus arteriosus (DA) constriction. We sought to determine the specificity and sensitivity of prenatal features of physiological shunts in predicting postnatal clinical status in prenatally diagnosed TGA in babies delivered in a tertiary care center providing all facilities for neonatal urgent care. Methods and Results-The outcomes of 130 fetuses with TGA were reviewed over a period of 5.5 years. Restriction of the FO and/or constriction of the DA could be analyzed in 119/130 fetuses at 36Ϯ2.7 weeks of gestation. Twenty-four out of 119 had at least 1 abnormal shunt (23 FO, 5 DA, and 4 both). Thirteen of 130 neonates had profound hypoxemia (PaO 2 Ͻ25 mm Hg) and metabolic acidosis (pH Ͻ7.15) in the first 30 minutes and required immediate balloon atrioseptostomy. Two who had abnormal FO and DA died despite aggressive resuscitation. The specificity and sensitivity of the fetal echo in predicting neonatal emergency were 84% and 54%, respectively. The specificity and sensitivity of a combination of restrictive FO and DA constriction were 100% and 31%, respectively. Conclusions-Restriction of the FO and/or of the DA has a high specificity to predict the need for emergency neonatal care in fetuses with TGA, but the sensitivity is too low to detect all high-risk fetuses. Exceptional procedures should be considered for fetuses that have a combination of restrictive FO and DA constriction. Key Words: heart defects, congenital Ⅲ transposition of great vessels Ⅲ echocardiography P renatal detection of transposition of the great arteries (TGA) has been well established. [1][2][3][4] Of those neonates admitted with TGA, mortality is estimated around 4% and is mostly due to inadequate interatrial mixing despite prostaglandin E1 infusion. 5 We have previously reported that prenatal diagnosis of TGA reduced neonatal mortality and morbidity. 3 However, early demise of neonates with TGA has been reported even after a prenatal diagnosis, suggesting that there was no site available for mixing immediately after birth. 3,[5][6][7][8] Indeed, antenatal restriction of the foramen ovale and/or the ductus arteriosus were found predictive of significant neonatal morbidity and mortality. 9 Because the surgical mortality for the arterial switch operation is as low as 2% in many institutions, 10 -13 the preoperative mortality is a major issue in the management and outcome of infants with TGA. Here, we reviewed the prenatal features of the foramen ovale and of the ductus arteriosus in all our cases of prenatally diagnosed TGA to address the relation between prenatal echo features and need for urgent intervention. Methods PopulationOne hundred thirty consecutive patients with a prenatal diagnosis of TGA were identified over a period of 5.5 years (November 1, 1997 to February 28, 2003. TGA was suspected during routine prenatal ultrasonogra...

B-lymphoproliferative disorder (BLPD) is a rare but severe complication of organ and bone marrow transplantation (BMT). Profound cytotoxic T-cell deficiency is thought to allow the outgrowth of Epstein-Barr virus–transformed B cells. When possible, reduction of immunosuppressive treatment or surgery for localized disease may cure BLPD. Therapeutic approaches using chemotherapy or antiviral drugs have limited effects on survival. Adoptive immunotherapy with donor T-cell infusions has given promising results in BMT recipients. We previously reported that administration of two monoclonal anti–B-cell antibodies (anti-CD21 and anti-CD24) could contribute to the control of oligoclonal BLPD. Here we report the long-term results of treatment with these monoclonal anti–B-cell antibodies for cases of severe BLPD. In an open multicenter trial, 58 patients in whom aggressive B-cell lymphoproliferative disorder developed after BMT (n = 27) or organ (n = 31) transplantation received 0.2 mg/kg/d of specific anti-CD21 and anti-CD24 murine monoclonal antibodies (MoAbs) for 10 days. The treatment was well tolerated. Thirty-six of the 59 episodes of BLPD in the 58 patients presented complete remission (61%). The relapse rate was low (3 of 36, 8%). Multivariate analysis identified the following risk factors for partial or no response to anti–B-cell MoAb therapy: multivisceral disease (P ≤ .005), central nervous system involvement (P ≤ .05), and late onset of BLPD (P ≤ .005). The overall long-term survival was 46% (median follow-up, 61 months); it was lower among BMT patients (35%) than organ transplant patients (55%). None of the patients who had received BMT for hematological malignancy survived for 1 year. Eight of these 11 patients presented monoclonal BLPD. Tumor burden was the only other variable that contributed significantly to poor survival. Thus, as assessed from this long-term study, the use of anti–B-cell MoAbs therefore appears to be a safe and relatively effective therapy for severe posttransplant BLPD. © 1998 by The American Society of Hematology.

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