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Pediatric Cardiology Oct 2022Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve... (Review)
Review
Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Ebstein's anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. The most symptomatic patients need intensive care support in the neonatal period. This article summarizes and analyzes the literature on Ebstein's anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. The surgical options available for patients with Ebstein's anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. The review also assesses the effects of pregnancy on the Ebstein's circulation, and vice versa, the effects of Ebstein's on pregnancy outcomes. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.
Topics: Adult; Ebstein Anomaly; Echocardiography; Fetus; Heart Defects, Congenital; Humans; Infant, Newborn; Patient Care; Tricuspid Valve
PubMed: 35460366
DOI: 10.1007/s00246-022-02908-x -
Echocardiography (Mount Kisco, N.Y.) Dec 2020Atrial septal defects (ASD) are among the most common congenital heart diseases encountered in adulthood. Patent foramen ovale (PFO) is present in up to 25% of the... (Review)
Review
Atrial septal defects (ASD) are among the most common congenital heart diseases encountered in adulthood. Patent foramen ovale (PFO) is present in up to 25% of the population. ASD could present as isolated lesion or in association with more complex congenital heart disease form as tetralogy of Fallot, or Ebstein's anomaly of tricuspid valve. There is a wide range of clinical presentation ranging from asymptomatic subjects surviving to adulthood undiagnosed to subjects presenting with right heart failure and severe pulmonary vascular disease (Eisenmenger syndrome). This manuscript is an in depth review of the complex atrial septation, the variable clinical presentation of ASD and PFO, and its clinical and therapeutic implications.
Topics: Adult; Foramen Ovale, Patent; Heart Septal Defects, Atrial; Humans
PubMed: 33368546
DOI: 10.1111/echo.14646 -
Journal of Women's Health (2002) Mar 2020Psychiatric illnesses are common in women of childbearing age. The perinatal period is a particularly high-risk time for depression, bipolar, and anxiety disorders.... (Review)
Review
Psychiatric illnesses are common in women of childbearing age. The perinatal period is a particularly high-risk time for depression, bipolar, and anxiety disorders. The scope of the public health problem of perinatal mental disorders is discussed followed by an examination of the specific research methods utilized for the study of birth and developmental outcomes associated with maternal mental illness and its treatment. The evidence on exposure to common psychotropics during pregnancy and breastfeeding is reviewed. Selective serotonin reuptake inhibitors or serotonin-norepinephrine reuptake inhibitor medications are not associated with higher rates of birth defects or long-term changes in mental development after adjustment for confounding factors associated with underlying psychiatric illness. Lithium exposure is associated with an increased risk for fetal cardiac malformations, but this risk is lower than previously thought (absolute risk of Ebstein's anomaly 6/1,000). Antipsychotics, other than risperidone and potentially paliperidone, have not been associated with an increase in birth defects; olanzapine and quetiapine have been linked with an elevated risk of gestational diabetes. Due to the dramatic physiological changes of pregnancy and enhanced hepatic metabolism, drug doses may need to be adjusted during pregnancy to sustain efficacy. Untreated maternal psychiatric illness also carries substantial risks for the mother, fetus, infant, and family. The goal of perinatal mental health treatment is to optimally provide pharmacotherapy to mitigate the somatic and psychosocial burdens of maternal psychiatric disorders. Regular symptom monitoring during pregnancy and postpartum and medication dose adjustments to sustain efficacy constitutes good practice.
Topics: Antipsychotic Agents; Breast Feeding; Female; Humans; Infant, Newborn; Mental Disorders; Postpartum Period; Pregnancy; Pregnancy Complications; Psychotropic Drugs; Selective Serotonin Reuptake Inhibitors
PubMed: 31800350
DOI: 10.1089/jwh.2019.7781 -
Journal of the American College of... Jun 2023
Topics: Humans; Ebstein Anomaly; Risk Assessment
PubMed: 37344045
DOI: 10.1016/j.jacc.2023.05.009 -
Mayo Clinic Proceedings Apr 2023
Topics: Humans; Ebstein Anomaly
PubMed: 37019511
DOI: 10.1016/j.mayocp.2023.02.014 -
The Canadian Journal of Cardiology Jul 2022Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To...
Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.
Topics: Adult; Aortic Coarctation; Canada; Ebstein Anomaly; Fontan Procedure; Heart Defects, Congenital; Humans; United States
PubMed: 35460862
DOI: 10.1016/j.cjca.2022.03.021 -
Journal of Cardiothoracic and Vascular... Dec 2022
Topics: Humans; Ebstein Anomaly; Tricuspid Valve; Tricuspid Valve Insufficiency
PubMed: 36163155
DOI: 10.1053/j.jvca.2022.08.019