Aim: Ebstein's anomaly is a rare congenital cardiac abnormality, associated with cyanosis and arrhythmia. Patients often reach childbearing age and pregnant women pose a challenge to the treating physician. We describe the course and outcome of pregnancy in women with Ebstein's anomaly and discuss the related management issues.
Methods: Analysis of pregnancies in four women with Ebstein's anomaly was carried out in a referral institute in northern India. Data of two women were analyzed retrospectively and the other two women were prospectively followed in their pregnancies during the study period. The course of the pregnancy, disease and perinatal outcome in each woman were analyzed.
Results: Four women had eight pregnancies all resulting in vaginal deliveries. There were two premature deliveries. The mean birth weight was 2.54 + or - 0.88 kg. Of the eight babies, six did not have any cardiac anomalies; one was an unexplained neonatal death and for another, no data was available. Pregnancy was well tolerated in two patients, while one had right heart failure during early pregnancy, and one had arrhythmia during labor, which was managed medically; one patient had arrhythmia in the postpartum period, which was managed medically.
Conclusion: When a woman with Ebstein's anomaly reaches childbearing age, fertility is not affected, even in cyanotic women. Under close supervision by the woman's obstetrician and cardiologist, the pregnancy outcome is usually favorable.
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http://dx.doi.org/10.1111/j.1447-0756.2009.01130.x | DOI Listing |
Case Rep Cardiol
December 2024
Department of Anesthesiology and Pain Management, Cleveland Clinic, Cleveland, Ohio, USA.
Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome.
View Article and Find Full Text PDFJ Cardiothorac Vasc Anesth
December 2024
Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Oman.
A circular shunt, initially described by Shone et al. in 1962, refers to abnormal blood recirculation through complete intracardiac or intra- and extracardiac communications, bypassing the capillary beds. This pathophysiological condition is most commonly associated with complex congenital heart defects, such as Ebstein's malformation, pulmonary atresia, Gerbode defect, and so on.
View Article and Find Full Text PDFAm J Med Genet A
December 2024
Division of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, Japan.
Ann Pediatr Cardiol
November 2024
Department of CVTS, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Association of Ebstein's anomaly of tricuspid valve (TV) with tetralogy of Fallot (TOF) is extremely rare. Their coexistence is unique as they modify each other's physiology. We report a case of a girl child having this rare combination along with Diamond-Blackfan syndrome awaiting a bone marrow transplant.
View Article and Find Full Text PDFAnn Pediatr Cardiol
November 2024
Department of Surgery, Pediatric and Congenital Heart Surgery Unit, National Cardiovascular Center Harapan Kita, West Jakarta, Indonesia.
Background: Surgical intervention is the definitive treatment for Ebstein anomaly, offering both biventricular and nonbiventricular repair options. The objective of this study is to identify a specific cutoff value for tricuspid septal leaflet displacement, which will be a crucial factor in determining the selection of a surgical approach with lower mortality risk in biventricular repair.
Methods And Results: This is a retrospective cohort study of consecutive patients with Ebstein anomaly undergoing surgical intervention at the National Cardiovascular Center Harapan Kita from January 2010 to December 2023.
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