scholarly journals Outcome of Antibody‐Mediated Fetal Heart Disease With Standardized Anti‐Inflammatory Transplacental Treatment

2022 ◽  
Author(s):  
Wadi Mawad ◽  
Lisa Hornberger ◽  
Bettina Cuneo ◽  
Marie‐Josée Raboisson ◽  
Anita J. Moon‐Grady ◽  
...  
Keyword(s):  
Heart Disease ◽  
Immune Globulin ◽  
Atrioventricular Block ◽  
Fetal Heart ◽  
Survival Rates ◽  
Intravenous Immune Globulin ◽  
Ventricular Rate ◽  
Endocardial Fibroelastosis ◽  
Immune Mediated ◽  
The Impact

Background Transplacental fetal treatment of immune‐mediated fetal heart disease, including third‐degree atrioventricular block (AVB III) and endocardial fibroelastosis, is controversial. Methods and Results To study the impact of routine transplacental fetal treatment, we reviewed 130 consecutive cases, including 108 with AVB III and 22 with other diagnoses (first‐degree/second‐degree atrioventricular block [n=10]; isolated endocardial fibroelastosis [n=9]; atrial bradycardia [n=3]). Dexamethasone was started at a median of 22.4 gestational weeks. Additional treatment for AVB III included the use of a β‐agonist (n=47) and intravenous immune globulin (n=34). Fetal, neonatal, and 1‐year survival rates with AVB III were 95%, 93%, and 89%, respectively. Variables present at diagnosis that were associated with perinatal death included an atrial rate <90 beats per minute (odds ratio [OR], 258.4; 95% CI, 11.5–5798.9; P <0.001), endocardial fibroelastosis (OR, 28.9; 95% CI, 1.6–521.7; P <0.001), fetal hydrops (OR, 25.5; 95% CI, 4.4–145.3; P <0.001), ventricular dysfunction (OR, 7.6; 95% CI, 1.5–39.4; P =0.03), and a ventricular rate <45 beats per minute (OR, 12.9; 95% CI, 1.75–95.8; P =0.034). At a median follow‐up of 5.9 years, 85 of 100 neonatal survivors were paced, and 1 required a heart transplant for dilated cardiomyopathy. Cotreatment with intravenous immune globulin was used in 16 of 22 fetuses with diagnoses other than AVB III. Neonatal and 1‐year survival rates of this cohort were 100% and 95%, respectively. At a median age of 3.1 years, 5 of 21 children were paced, and all had normal ventricular function. Conclusions Our findings reveal a low risk of perinatal mortality and postnatal cardiomyopathy in fetuses that received transplacental dexamethasone±other treatment from the time of a new diagnosis of immune‐mediated heart disease.

Congenital atrioventricular block: clinical presentation, clinical evaluation, and management

ESC CardioMed ◽  
2018 ◽  
pp. 1966-1968
Author(s):  
Drago Fabrizio ◽  
Battipaglia Irma
Keyword(s):  
Heart Disease ◽  
Atrioventricular Block ◽  
Pacemaker Implantation ◽  
Cardiac Conduction ◽  
Ventricular Rate ◽  
Postnatal Life ◽  
Av Block ◽  
Cardiac Stimulation ◽  
Immune Mediated ◽  
Congenital Atrioventricular Block

Congenital atrioventricular block (CAVB) is a cardiac conduction disorder that is diagnosed in utero, at birth, or within the first month of life. When it is diagnosed between the first month and the 18th year of life, it is defined as childhood atrioventricular (AV) block. CAVB may occur in association with concomitant congenital heart disease, or be isolated, in a structurally normal heart (e.g. immune-mediated, inherited, or idiopathic CAVB). The majority of isolated CAVB is an immune-mediated AV block, due to transplacental passage of maternal autoantibodies, damaging the fetal cardiac conduction system. Only in a third of infants with immune-mediated CAVB is a well-defined autoimmune disease known in the mother. During fetal life, fetal echocardiography still represents the gold standard for the diagnosis of CAVB. Two major negative prognostic markers are low ventricular rate and the appearance of foetal hydrops. As regards prognosis, a risk for heart failure, syncope, and sudden death is present both during fetal and postnatal life. Dilated cardiomyopathy represents another complication in CAVB history, with different possible aetiologies (right ventricular permanent pacing, reactivation of autoimmune myocarditis). The indications for pacemaker implantation in patients with CAVB are similar to those for acquired heart disease, with some special technical considerations due to young age (epicardial versus endocardial systems, pacing site, etc.). As a future perspective, leadless cardiac stimulation in children with CAVB may represent a definitive solution and an answer to many questions.

Congenital atrioventricular block: clinical presentation, clinical evaluation, and management

ESC CardioMed ◽  
2018 ◽  
pp. 1966-1968
Author(s):  
Drago Fabrizio ◽  
Battipaglia Irma
Keyword(s):  
Heart Disease ◽  
Atrioventricular Block ◽  
Pacemaker Implantation ◽  
Cardiac Conduction ◽  
Ventricular Rate ◽  
Postnatal Life ◽  
Av Block ◽  
Cardiac Stimulation ◽  
Immune Mediated ◽  
Congenital Atrioventricular Block

Congenital atrioventricular block (CAVB) is a cardiac conduction disorder that is diagnosed in utero, at birth, or within the first month of life. When it is diagnosed between the first month and the 18th year of life, it is defined as childhood atrioventricular (AV) block. CAVB may occur in association with concomitant congenital heart disease, or be isolated, in a structurally normal heart (e.g. immune-mediated, inherited, or idiopathic CAVB). The majority of isolated CAVB is an immune-mediated AV block, due to transplacental passage of maternal autoantibodies, damaging the fetal cardiac conduction system. Only in a third of infants with immune-mediated CAVB is a well-defined autoimmune disease known in the mother. During fetal life, fetal echocardiography still represents the gold standard for the diagnosis of CAVB. Two major negative prognostic markers are low ventricular rate and the appearance of fetal hydrops. As regards prognosis, a risk for heart failure, syncope, and sudden death is present both during fetal and postnatal life. Dilated cardiomyopathy represents another complication in CAVB history, with different possible aetiologies (right ventricular permanent pacing, reactivation of autoimmune myocarditis). The indications for pacemaker implantation in patients with CAVB are similar to those for acquired heart disease, with some special technical considerations due to young age (epicardial versus endocardial systems, pacing site, etc.). As a future perspective, leadless cardiac stimulation in children with CAVB may represent a definitive solution and an answer to many questions.

scholarly journals OC21.04: Risk based prenatal monitoring for immune mediated fetal heart disease: a prospective 5-year experience

2015 ◽  
Vol 46 ◽  
pp. 46-47
Author(s):  
E. Jaeggi ◽  
F. Golding ◽  
C. Laskin ◽  
J.C. Kingdom ◽  
E. Silverman ◽  
...  
Keyword(s):  
Heart Disease ◽  
Fetal Heart ◽  
Immune Mediated

scholarly journals Promoting Resilience in Families with Children with Congenital Heart Disease

2019 ◽  
Vol 4 (1) ◽  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Cognitive Functioning ◽  
Congenital Heart ◽  
Wide Spectrum ◽  
Heart Defects ◽  
Survival Rates ◽  
Intervention Strategy ◽  
Treatment Protocols ◽  
The Impact

Congenital heart disease (CHD) is the most common birth defect, affecting approximately 40,000 infants annually in the US [1]. CHD involves a variety of heart defects, with a wide spectrum from simple to moderate to complex. Due to advances in pediatric cardiology and cardiac surgery, life expectancy in these children has increased drastically over the past decades. Now 90% of infants diagnosed with CHD live well into adulthood [2]. Due to the increase in survival rates, attention has shifted towards the impact of CHD on psychological and cognitive functioning [3]. Children with CHD are at risk for neurodevelopmental and psychosocial problems related to operative factors. Children with CHD display lower cognitive functioning, and higher rates in behavioral, attentional and emotional problems. Increased parental stress also adds to the psychosocial issues experienced by these children. For this reason, psychological care needed to promote resiliency in this population. Incorporating psychological services into treatment protocols with children with CHD is beneficial and will improve behavioral and emotional functioning across their lifespan [4]. This paper will review the definitions of resiliency and Congenital Heart Disease, as well as the professional roles of people treating infants with CHD, the risk factors deterring resiliency, and the developmental outcomes in children with CHD. It will then examine some of the most common psychological interventions for children with CHD and chronic illness, including CBT and psychoeducational interventions, and family-based interventions that also include a psychoeducational component. I will conclude by discussing the value of this multidimensional approach and why it is the most effective intervention strategy to promote resiliency in children with CHD.

scholarly journals Natural Antibodies and Alloreactive T Cells Long after Kidney Transplantation

2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Nicole M. van Besouw ◽  
Aleixandra Mendoza Rojas ◽  
Sarah B. See ◽  
Ronella de Kuiper ◽  
Marjolein Dieterich ◽  
...  
Keyword(s):  
T Cells ◽  
Immune Globulin ◽  
Natural Antibodies ◽  
Intravenous Immune Globulin ◽  
Effector Memory ◽  
Kidney Transplant Recipients ◽  
Positive Correlation ◽  
Alloreactive T Cells ◽  
Ifn Γ ◽  
The Impact

Background. The relationship between circulating effector memory T and B cells long after transplantation and their susceptibility to immunosuppression are unknown. To investigate the impact of antirejection therapy on T cell-B cell coordinated immune responses, we assessed IFN-γ-producing memory cells and natural antibodies (nAbs) that potentially bind to autoantigens on the graft. Methods. Plasma levels of IgG nAbs to malondialdehyde (MDA) were measured in 145 kidney transplant recipients at 5–7 years after transplantation. In 54 of these patients, the number of donor-reactive IFN-γ-producing cells was determined. 35/145 patients experienced rejection, 18 of which occurred within 1 year after transplantation. Results. The number of donor-reactive IFN-γ-producing cells and the levels of nAbs were comparable between rejectors and nonrejectors. The nAbs levels were positively correlated with the number of donor-reactive IFN-γ-producing cells (rs = 0.39, p = 0.004 ). The positive correlation was only observed in rejectors (rs = 0.53, p = 0.003 ; nonrejectors: rs = 0.24, p = 0.23 ). Moreover, we observed that intravenous immune globulin treatment affected the level of nAbs and this effect was found in patients who experienced a late ca-ABMR compared to nonrejectors ( p = 0.008 ). Conclusion. The positive correlation found between alloreactive T cells and nAbs in rejectors suggests an intricate role for both components of the immune response in the rejection process. Treatment with intravenous immune globulin impacted nAbs.

Abstract 169: Impact of Resistance to Intravenous Immune Globulin on Changes of Left Ventricular Myocardial Deformation Over Time in Children with Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Soo Jung Kang ◽  
Dong Keon Yon
Keyword(s):  
Kawasaki Disease ◽  
Immune Globulin ◽  
Acute Phase ◽  
Left Ventricular ◽  
Myocardial Deformation ◽  
Intravenous Immune Globulin ◽  
Myocardial Mechanics ◽  
Convalescent Phase ◽  
The Impact ◽  
Over Time

Introduction: This study aimed to assess the impact of resistance to intravenous immune globulin(IVIG) on left ventricular(LV) myocardial deformation in children with Kawasaki disease(KD) during the acute and convalescent phases of illness. Few studies have elucidated the impact of resistance to IVIG on the progressive changes of myocardial mechanics over time in patients with KD. Methods: We studied 26 patients with KD and 8 normal control subjects. Of the 26 patients, 16 were IVIG non-responders. Echocardiograms were obtained during the acute and convalescent phases of KD. Standard echocardiographic data and peak systolic global LV longitudinal strain[strain(ε)] were obtained using vector velocity imaging. Results: During the acute phase of KD, peak systolic global LV longitudinal ε decreased significantly in both IVIG non-responders(-21.18 % ± 3.97) and responders(-20.94 % ± 3.15) compared to controls(-24.99 % ± 2.23). Although in the acute phase, LV ejection fraction(EF) was significantly higher in the IVIG non-responders(55.38 % ± 5.14) compared to the responders(50.2 % ± 6.53), peak systolic global LV longitudinal ε was not significantly higher in the IVIG non-responders compared to the responders. During the convalescent phase, peak systolic global LV longitudinal ε tended to increase in non-responders(-21.62% ± 3.98) and responders(-21.83% ± 2.61) compared to the acute phase, but remained significantly decreased in both groups compared to controls. The increment of peak systolic global LV longitudinal ε from acute to convalescent phase tended to be smaller in the IVIG non-responders compared to the responders. The proportion of patients with coronary dilatations in both IVIG non-responders(4 of 16) and IVIG responders(2 of 10) did not differ significantly. Conclusions: The increment of peak systolic global LV longitudinal ε over time tended to be smaller in the IVIG non-responders compared to the IVIG responders. Resistance to IVIG may delay normalization of myocardial mechanics in IVIG non-responders. Further studies with larger number of patients, as well as long-term follow-up of myocardial deformation in KD are necessary.

scholarly journals Mechanisms of action of intravenous immune globulin in immune-mediated diseases

1996 ◽  
Vol 104 ◽  
pp. 3-9 ◽  
Author(s):  
L. Mouthon ◽  
S. V. Kaveri ◽  
S. H. Spalter ◽  
S. Lacroix-Desmazes ◽  
C. Lefranc ◽  
...  
Keyword(s):  
Immune Globulin ◽  
Mechanisms Of Action ◽  
Intravenous Immune Globulin ◽  
Immune Mediated

scholarly journals Severe Hyponatremia as the Initial Sign Preceding Guillain-Barré Syndrome, an Acute Inflammatory Demyelinating Polyneuropathy: A Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Benjamin Kloesel ◽  
LaTonya J. Hickson
Keyword(s):  
Immune Globulin ◽  
Guillain Barre Syndrome ◽  
Intravenous Immune Globulin ◽  
Severe Hyponatremia ◽  
Acute Inflammatory Demyelinating Polyneuropathy ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Guillain Barré Syndrome ◽  
Initial Sign ◽  
Guillain Barré

Guillain-Barré syndrome is an immune-mediated polyneuropathy that frequently presents with progressive muscle weakness. Hyponatremia has recently been described as a feature of this condition, generally appearing over the course of the illness and following the diagnosis of this demyelinating process. We report a case of Guillain-Barré syndrome presenting with severe hyponatremia that is further exacerbated by intravenous immune globulin therapy. Awareness should be raised for consideration of both Guillain-Barré syndrome and its treatment with intravenous immune globulin therapy as the cause of hyponatremia.

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