Shifting Risks and Conflicting Outcomes-ECMO for Neonates with Congenital Diaphragmatic Hernia in the Modern Era.

OBJECTIVES: To update previously described trends for neonates with congenital diaphragmatic hernia (CDH) receiving ECMO with changes in recommendations for care, and to determine how recent advancements in respiratory care have affected this patient population. STUDY DESIGN: This study is a retrospective review of more than 2500 neonates with CDH who received ECMO listed in the Extracorporeal Life Support Organization (ELSO) registry. Cochran-Armitage and multivariate regression analyses were used to analyze changes in the patient population over time and in mortality-related risk factors. RESULTS: Almost one-half (48.1%) of the term neonates survived to discharge, representing a 13.8% decline in survival over the past 25 years (P < .0001). Over the past 10 years, the prevalence of respiratory acidosis more than doubled (P < .0001) and the prevalence of major complications increased (P < .001). During the same period, the number of ECMO courses longer than 1 week increased (P < .001), whereas the prevalence of multiple complications (>4) decreased (P < .0001). Surgeries performed on ECMO were associated with worse outcomes than those performed off ECMO. ECMO duration no longer represents a mortality-related risk factor. CONCLUSIONS: Survival rates for neonates with CDH receiving ECMO have continued to drop in the modern era. Although the safety of ECMO has improved over the last decade, the number of patients experiencing significant respiratory acidosis has more than doubled-increasing the risk of intracranial hemorrhage and overall mortality. The evidence for permissive hypercapnia remains mixed; nonetheless, we believe that the risks outweigh the rewards in this patient population.

Supraclavicular Translocation of Aberrant Left Subclavian Artery for Vascular Ring Division and Resection of Kommerell's Diverticulum.

Intrathoracic left subclavian to common carotid artery translocation is advocated for patients undergoing vascular ring division in the setting of an enlarged Kommerell's diverticulum and aberrant left subclavian artery. This approach poses technical challenges in larger patients and patients with unfavorable body habitus. Supraclavicular left subclavian artery to common carotid translocation greatly simplifies this procedure in select patients.

Intrapleural fibrinolysis during pediatric extracorporeal life support.

Pediatric patients who require extracorporeal life support (ECLS) for refractory cardiorespiratory failure are at increased risk for intrapleural hemorrhage due to the effects of systemic anticoagulation and frequent occurrence of pneumothorax. Surgical evacuation is standard therapy for retained hemothorax to prevent secondary empyema, pulmonary compression, and development of fibrothorax. However, surgical interventions during ECLS are hazardous and place patients at increased risk for surgical site bleeding. Intrapleural fibrinolysis with tissue plasminogen activator may be used to facilitate nonsurgical evacuation of retained hemothorax. We present two pediatric patients who were safely and successfully managed with intrapleural fibrinolysis of retained hemothorax during ECLS.

Neoaortic thrombus after Norwood procedure: complication of extracorporeal life support?

Hypoplastic left heart syndrome is the most common diagnosis among infants requiring cardiac extracorporeal life support (ECLS) in the neonatal period, with more than half of patients experiencing an ECLS-related complication. We present a case of nearly obstructing neoaortic thrombus during ECLS occurring in the early postoperative period after modified Norwood palliation of hypoplastic left heart syndrome. Stasis within the neoaortic root due to the increased ventricular afterload associated with venoarterial ECLS may have potentiated this complication. Echocardiographic monitoring and early cardiac catheterization while on ECLS enabled prompt diagnosis and timely surgical correction.

Extracorporeal life support for the neonatal cardiac patient: outcomes and new directions.

Extracorporeal life support is an important therapy for neonates with life-threatening cardiopulmonary failure. Utilization of extracorporeal life support in neonates with congenital heart disease has increased dramatically during the past three decades. Despite increased usage, overall survival in these patients has changed very little and extracorporeal life support-related morbidity, including bleeding, neurologic injury, and renal failure, remains a major problem. Although survival is lower and neurologic complications are higher in premature infants than term infants, cardiac extracorporeal life support including extracorporeal cardiopulmonary resuscitation is effective in preventing death in many of these high-risk patients. Miniaturized ventricular assist devices and compact integrated extracorporeal life support systems are being developed to provide additional therapeutic options for neonates.

Novel titanium constructs for chest wall reconstruction in children.

PURPOSE: We have previously reported the use of the vertical expandable prosthetic titanium rib (VEPTR) for treatment of thoracic dystrophy. This report describes our experience with this device and other novel titanium constructs for chest wall reconstruction. METHODS: This is a retrospective chart review of all children and adolescents undergoing chest wall reconstruction with titanium constructs between December 2005 and May 2010. RESULTS: Six patients have undergone chest wall reconstruction with VEPTR or other titanium constructs. Four had chest wall resection for primary malignancy, 1 had metastatic chest wall tumor resection, and 1 had congenital chest wall deformity. There were no immediate complications, and all patients have exhibited excellent respiratory function with no scoliosis. CONCLUSIONS: Chest wall reconstruction after tumor resection or for primary chest wall deformities can be effectively accomplished with VEPTR and other customized titanium constructs. Goals should be durable protection of intrathoracic organs and preservation of thoracic volume and function throughout growth. Careful preoperative evaluation and patient-specific planning are important aspects of successful reconstruction.

Association between peripheral γδ T-cell profile and disease progression in individuals infected with HIV-1 or HIV-2 in West Africa.

BACKGROUND: Human gammadelta (γδ) T cells play an important role in protective immunity in HIV-1 and simian immunodeficiency virus infection; their role in HIV-2 infection is unknown. OBJECTIVE: To determine the role of γδ T cells in control of plasma viral load and CD4 T-cell count in HIV-1 and HIV-2 infections in West Africa. METHODS: Thirty HIV-1 and 25 HIV-2 treatment-naive chronically infected individuals, and 20 HIV-seronegative individuals from Senegal were studied using multiparametric flow cytometry to investigate the frequencies and phenotypes of peripheral γδ T cells. γδ T-cell parameters and correlates of HIV disease progression were assessed. RESULTS: : We observed an expansion of Vδ1 T-cell populations in both HIV-1 and HIV-2 infection. However, unlike HIV-1 infection, no significant contraction of the frequency of total Vδ2 T cells was observed in HIV-2 infection. Significantly lower frequencies of CD4Vδ2 T cells were observed in HIV-2-infected individuals. Furthermore, frequencies of CD28CD45RO and CD27CD28CD45RO Vδ2 T cell were low in HIV-1-infected individuals. Vδ2 T-cell activation levels were elevated in both HIV-1-infected and HIV-2-infected individuals. The frequency of HLA-DRCD38-activated Vδ1 and Vδ2 T cells was associated with a decline in CD4 T-cell counts and increased viral load in both HIV-1 and HIV-2 infection. CONCLUSIONS: Although maintaining the normal frequency of total Vδ2 T cells, HIV-2 infection reduces the frequency of CD4Vδ2 T cells and alters the frequencies of subsets of Vδ1 T cells. Both HIV-1 and HIV-2 infection induce γδ T-cell activation, and this activation is associated with the disease progression.

Role of human immunodeficiency virus (HIV)-specific T-cell immunity in control of dual HIV-1 and HIV-2 infection.

Progressive immune dysfunction and AIDS develop in most cases of human immunodeficiency virus type 1 (HIV-1) infection but in only 25 to 30% of persons with HIV-2 infection. However, the natural history and immunologic responses of individuals with dual HIV-1 and HIV-2 infection are largely undefined. Based on our previous findings, we hypothesized that among patients with dual infection the control of HIV-1 is associated with the ability to respond to HIV-2 Gag epitopes and to maintain HIV-specific CD4(+) T-cell responses. To test this, we compared the HIV-specific ex vivo IFN-gamma enzyme-linked immunospot (ELISPOT) assay responses of 19 dually infected individuals to those of persons infected with HIV-1 or HIV-2 only. Further, we assessed the functional profile of HIV Gag-specific CD4(+) and CD8(+) T cells from nine HIV dually infected patients by using a multicolor intracellular cytokine staining assay. As determined by ELISPOT assay, the magnitude and frequency of IFN-gamma-secreting T-cell responses to gene products of HIV-1 were higher than those to gene products of HIV-2 (2.64 versus 1.53 log(10) IFN-gamma spot-forming cells/10(6) cells [90% versus 63%, respectively].) Further, HIV-1 Env-, Gag-, and Nef- and HIV-2 Gag-specific responses were common; HIV-2 Nef-specific responses were rare. HIV-specific CD4(+) T helper responses were detected in nine of nine dually infected subjects, with the majority of these T cells producing gamma interferon (IFN-gamma) and tumor necrosis factor alpha (TNF-alpha) and, to a lesser extent, interleukin-2. The HIV-1 plasma viral load was inversely correlated with HIV-2 Gag-specific IFN-gamma-/TNF-alpha-secreting CD4(+) and HIV-2 Gag-specific IFN-gamma-secreting CD8(+) T cells. In conclusion, the T-cell memory responses associated with containment of single HIV-1 and HIV-2 infection play a similar significant role in the immune control of dual HIV-1 and HIV-2 infection.