Scimitar syndrome.

Clin Pediatr (Phila)

Published: June 1982

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http://dx.doi.org/10.1177/000992288202100605DOI Listing

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A Curious Case of Scimitar Syndrome That Defies Embryology.

Ann Thorac Surg Short Rep

December 2024

Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, Ohio.

Scimitar syndrome is a rare condition described by unique anatomic features that consist mainly of an abnormal connection of the right pulmonary veins to the inferior vena cava and right atrial junction, as well as an anomalous systemic arterial supply to the right lung. We present the case of a 60-year-old man with an atypical variant of scimitar syndrome that was embryologically perplexing and anatomically challenging to correct. We highlight key surgical and procedural considerations for a patient with scimitar syndrome presenting with this complex surgical anatomy.

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The objective of this study is to evaluate the clinical application and primary outcome of transcatheter embolization using Amplatzerâ„¢ Vascular Plug (AVP) Type 2 and Type 4 in different congenital cardiovascular malformations. This is a single-center retrospective observational cohort study. We analyzed clinical and imaging data of 36 patients retrospectively who received transcatheter embolizations of the following malformations using AVP: systemic-to-pulmonary collateral arteries (SPCA), patent ductus arteriosus (PDA), ventricular septal defects (VSD), and aberrant pulmonary sequestration arteries (PSA).

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Aim: This study investigated the major factors contributing to the missed diagnosis of total anomalous pulmonary venous connection (TAPVC) in fetal echocardiography.

Methods: We retrospectively analyzed the prenatal ultrasonic images of 32 fetuses with missed diagnoses of TAPVC, compared them with autopsy and postnatal surgical records, and summarized the most likely reasons leading to the missed diagnoses.

Results: We studied a total of 157 fetuses with TAPVC, 32 (20.

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Article Synopsis
  • Total anomalous pulmonary venous return (TAPVR) can cause severe issues shortly after birth, making early detection crucial for better outcomes.
  • Despite being a serious congenital heart condition, TAPVR is often not diagnosed before birth, even though there are known ultrasound markers.
  • This commentary introduces a new color Doppler fetal sonographic marker that could help identify TAPVR prenatally, potentially preventing life-threatening complications.
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Hypogenetic lung syndrome, commonly known as Scimitar syndrome, is a rare cardiopulmonary anomaly characterized primarily by anomalous pulmonary venous return and lung hypoplasia. While it is most frequently diagnosed in infancy or early childhood, cases in adulthood are exceedingly rare. We present a case of a middle-aged female who was diagnosed with Scimitar syndrome.

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