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Introduction: Supraventricular tachycardia is the most common fetal tachyarrhythmia and if persistent often associated with fetal hydrops which can cause intrauterine and neonatal death. Case Presentation: We present a case of early second trimester supraventricular tachycardia in a hydropic fetus, initially refractory to transplacental treatment. Conclusion: The supraventricular tachycardia was successfully treated when supplemented with intraperitoneal flecainide in the fetus.

The semi-allogeneic fetus derives half of its genetic maternal from the mother. The other half, inherited from the father, leads to the expression of proteins that are foreign to the mother. In danger of potential immune recognition and rejection, the fetus is dependent on maternal immune regulation. Multiple mechanisms are in place to ensure the mother and fetus live in harmony during pregnancy, as outlined in Chapter one. This dissertation discusses what happens to some of these mechanisms during fetal intervention in mice. Chapter two focuses on T cell specific mechanisms that prevent maternal T cell activation during pregnancy. These mechanisms include constraint in antigen presentation to maternal T cells, deletion of maternal T cells aware of fetal antigens and the lack of recruitment of maternal T cells to the uterine environment. Fetal intervention in mice results in enhanced antigen presentation with a reduction in apoptosis of activated cells and a more prominent presence of maternal T cells in the uterus. Maternal T cells also play a role in fetal demise (preterm labor) during fetal intervention. Chapter three discusses changes in trafficking maternal cells during fetal intervention. Trafficking of maternal cells into fetal blood during normal pregnancy encourages generation of fetal Tregs that can suppress an anti-maternal T cell response. During fetal intervention, the presence of maternal T cells, maternal microchimerism (MMc), increases and may play a role in limiting engraftment of cells transplanted in utero. Because fetal intervention results in preterm labor, changes in maternal microchimerism during fetal intervention may also play a role in mediating fetal rejection during preterm labor. Using a mouse model of preterm labor, we saw enhanced maternal microchimerism in fetal blood. The contribution of maternal cells in fetal blood to the pathogenesis of preterm labor is an open field for further investigation. Chapter four deals with the role of m

Background: Indications for the ex utero intrapartum therapy (EXIT) procedure have evolved and nowadays in addition to secure the airway, obtain vascular access, administer surfactant and other resuscitation medications, EXIT is used to resect cervical or thoracic masses, for extracorporeal membrane circulation (ECMO) cannulation, as well as to rescue maximum intra-thoracic space for ventilation of the remaining functional lung tissue or in cases in which resuscitation of the neonate may be compromised. EXIT procedure in twin pregnancy has been rarely reported and some doubts have been raised about its strategy and safety in such cases. Methods: We reviewed the medical records of 3 twin pregnancy cases where the EXIT procedure have been performed in our center. Results: The mean gestational age at EXIT procedure was 34 + 4 weeks. In two out the three EXIT procedures, the affected twin was delivered first. The average time on placental bypass was 9 minutes. There were no fetal or maternal complications related to the EXIT procedure. All newborns are currently doing well. Conclusion: In twin pregnancies, prenatal diagnosis combined with the EXIT procedure permits the formulation of a controlled delivery strategy to secure both newborns outcome. In those pregnancies, if intervention can be accomplished without compromise of the normal twin, EXIT can be considered. Our results support that EXIT procedure, if properly planned, safely provides a good outcome for both the fetuses as well as the mother.

Background: Indications for the ex utero intrapartum therapy (EXIT) procedure have evolved and nowadays in addition to secure the airway, obtain vascular access, administer surfactant and other resuscitation medications, EXIT is used to resect cervical or thoracic masses, for extracorporeal membrane circulation (ECMO) cannulation, as well as to rescue maximum intra-thoracic space for ventilation of the remaining functional lung tissue or in cases in which resuscitation of the neonate may be compromised. EXIT procedure in twin pregnancy has been rarely reported and some doubts have been raised about its strategy and safety in such cases. Methods: We reviewed the medical records of 3 twin pregnancy cases where the EXIT procedure have been performed in our center. Results: The mean gestational age at EXIT procedure was 34 + 4 weeks. In two out the three EXIT procedures, the affected twin was delivered first. The average time on placental bypass was 9 minutes. There were no fetal or maternal complications related to the EXIT procedure. All newborns are currently doing well. Conclusion: In twin pregnancies, prenatal diagnosis combined with the EXIT procedure permits the formulation of a controlled delivery strategy to secure both newborns outcome. In those pregnancies, if intervention can be accomplished without compromise of the normal twin, EXIT can be considered. Our results support that EXIT procedure, if properly planned, safely provides a good outcome for both the fetuses as well as the mother.

The semi-allogeneic fetus derives half of its genetic maternal from the mother. The other half, inherited from the father, leads to the expression of proteins that are foreign to the mother. In danger of potential immune recognition and rejection, the fetus is dependent on maternal immune regulation. Multiple mechanisms are in place to ensure the mother and fetus live in harmony during pregnancy, as outlined in Chapter one. This dissertation discusses what happens to some of these mechanisms during fetal intervention in mice. Chapter two focuses on T cell specific mechanisms that prevent maternal T cell activation during pregnancy. These mechanisms include constraint in antigen presentation to maternal T cells, deletion of maternal T cells aware of fetal antigens and the lack of recruitment of maternal T cells to the uterine environment. Fetal intervention in mice results in enhanced antigen presentation with a reduction in apoptosis of activated cells and a more prominent presence of maternal T cells in the uterus. Maternal T cells also play a role in fetal demise (preterm labor) during fetal intervention. Chapter three discusses changes in trafficking maternal cells during fetal intervention. Trafficking of maternal cells into fetal blood during normal pregnancy encourages generation of fetal Tregs that can suppress an anti-maternal T cell response. During fetal intervention, the presence of maternal T cells, maternal microchimerism (MMc), increases and may play a role in limiting engraftment of cells transplanted in utero. Because fetal intervention results in preterm labor, changes in maternal microchimerism during fetal intervention may also play a role in mediating fetal rejection during preterm labor. Using a mouse model of preterm labor, we saw enhanced maternal microchimerism in fetal blood. The contribution of maternal cells in fetal blood to the pathogenesis of preterm labor is an open field for further investigation. Chapter four deals with the role of m

Background: Indications for the ex utero intrapartum therapy (EXIT) procedure have evolved and nowadays in addition to secure the airway, obtain vascular access, administer surfactant and other resuscitation medications, EXIT is used to resect cervical or thoracic masses, for extracorporeal membrane circulation (ECMO) cannulation, as well as to rescue maximum intra-thoracic space for ventilation of the remaining functional lung tissue or in cases in which resuscitation of the neonate may be compromised. EXIT procedure in twin pregnancy has been rarely reported and some doubts have been raised about its strategy and safety in such cases. Methods: We reviewed the medical records of 3 twin pregnancy cases where the EXIT procedure have been performed in our center. Results: The mean gestational age at EXIT procedure was 34 + 4 weeks. In two out the three EXIT procedures, the affected twin was delivered first. The average time on placental bypass was 9 minutes. There were no fetal or maternal complications related to the EXIT procedure. All newborns are currently doing well. Conclusion: In twin pregnancies, prenatal diagnosis combined with the EXIT procedure permits the formulation of a controlled delivery strategy to secure both newborns outcome. In those pregnancies, if intervention can be accomplished without compromise of the normal twin, EXIT can be considered. Our results support that EXIT procedure, if properly planned, safely provides a good outcome for both the fetuses as well as the mother.

Background: Indications for the ex utero intrapartum therapy (EXIT) procedure have evolved and nowadays in addition to secure the airway, obtain vascular access, administer surfactant and other resuscitation medications, EXIT is used to resect cervical or thoracic masses, for extracorporeal membrane circulation (ECMO) cannulation, as well as to rescue maximum intra-thoracic space for ventilation of the remaining functional lung tissue or in cases in which resuscitation of the neonate may be compromised. EXIT procedure in twin pregnancy has been rarely reported and some doubts have been raised about its strategy and safety in such cases. Methods: We reviewed the medical records of 3 twin pregnancy cases where the EXIT procedure have been performed in our center. Results: The mean gestational age at EXIT procedure was 34 + 4 weeks. In two out the three EXIT procedures, the affected twin was delivered first. The average time on placental bypass was 9 minutes. There were no fetal or maternal complications related to the EXIT procedure. All newborns are currently doing well. Conclusion: In twin pregnancies, prenatal diagnosis combined with the EXIT procedure permits the formulation of a controlled delivery strategy to secure both newborns outcome. In those pregnancies, if intervention can be accomplished without compromise of the normal twin, EXIT can be considered. Our results support that EXIT procedure, if properly planned, safely provides a good outcome for both the fetuses as well as the mother.

Background: Indications for the ex utero intrapartum therapy (EXIT) procedure have evolved and nowadays in addition to secure the airway, obtain vascular access, administer surfactant and other resuscitation medications, EXIT is used to resect cervical or thoracic masses, for extracorporeal membrane circulation (ECMO) cannulation, as well as to rescue maximum intra-thoracic space for ventilation of the remaining functional lung tissue or in cases in which resuscitation of the neonate may be compromised. EXIT procedure in twin pregnancy has been rarely reported and some doubts have been raised about its strategy and safety in such cases. Methods: We reviewed the medical records of 3 twin pregnancy cases where the EXIT procedure have been performed in our center. Results: The mean gestational age at EXIT procedure was 34 + 4 weeks. In two out the three EXIT procedures, the affected twin was delivered first. The average time on placental bypass was 9 minutes. There were no fetal or maternal complications related to the EXIT procedure. All newborns are currently doing well. Conclusion: In twin pregnancies, prenatal diagnosis combined with the EXIT procedure permits the formulation of a controlled delivery strategy to secure both newborns outcome. In those pregnancies, if intervention can be accomplished without compromise of the normal twin, EXIT can be considered. Our results support that EXIT procedure, if properly planned, safely provides a good outcome for both the fetuses as well as the mother.

We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction.

We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction.