The Connection Between Anatomical Substrate and Clinical Severity in Fetal Ebstein Anomaly.

Autor:	Coacci S; The Cardiac Registry, Departments of Cardiology, Pathology, and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.; Vita-Salute San Raffaele University, IRCCS Policlinico San Donato Hospital, Milan, Italy., Alston ELJ; Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA., Yamasaki T; The Cardiac Registry, Departments of Cardiology, Pathology, and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.; Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine, Mie, Japan., Ronai C; Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.; Department of Pediatrics, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA., Sanders SP; The Cardiac Registry, Departments of Cardiology, Pathology, and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.; Department of Pediatrics, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA., Carreon CK; The Cardiac Registry, Departments of Cardiology, Pathology, and Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.; Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
Jazyk:	angličtina
Zdroj:	Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society [Pediatr Dev Pathol] 2024 Nov-Dec; Vol. 27 (6), pp. 587-591. Date of Electronic Publication: 2024 May 19.
DOI:	10.1177/10935266241250235
Abstrakt:	Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study documents 2 dramatic cases of fetal EA characterized by hydrops and cardiomegaly, leading to intrauterine or early neonatal death. These clinical outcomes were associated with morphological abnormalities including severe tricuspid regurgitation, unguarded TV orifice, pulmonary atresia, and flattened right ventricular myocardium. This study highlights that these adverse anatomical features may result in unfavorable clinical outcomes in fetal EA. While timely identification of such features by prenatal ultrasound is crucial for providing accurate prognostic stratification and guiding treatment decisions, fetopsy may be necessary to discern EA among the spectrum of right-heart anomalies. Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Databáze:	MEDLINE
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