Newly Published
Images in Anesthesiology  |   February 2020
Cardiovascular Risks in Patients with Loeys–Dietz Syndrome
Author Notes
  • From the Department of Anesthesiology and Intensive Care Medicine, University Hospital Tuebingen, Tuebingen, Germany.
  • Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are available in both the HTML and PDF versions of this article. Links to the digital files are provided in the HTML text of this article on the Journal’s Web site (www.anesthesiology.org).
    Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are available in both the HTML and PDF versions of this article. Links to the digital files are provided in the HTML text of this article on the Journal’s Web site (www.anesthesiology.org).×
  • Correspondence: Address correspondence to Dr. Fideler: frank.fideler@med.uni-tuebingen.de
Article Information
Images in Anesthesiology / Cardiovascular Anesthesia / Pediatric Anesthesia
Images in Anesthesiology   |   February 2020
Cardiovascular Risks in Patients with Loeys–Dietz Syndrome
Anesthesiology Newly Published on February 10, 2020. doi:https://doi.org/10.1097/ALN.0000000000003190
Anesthesiology Newly Published on February 10, 2020. doi:https://doi.org/10.1097/ALN.0000000000003190
Compression of the left atrium is well described in cases of mediastinal masses, pericardial effusion, or aortic aneurysms. Here, we present a transthoracic apical four-chamber view of a patient with Loeys–Dietz syndrome that displays a severe slit-like compression of the left atrium between the sternum and the thoracic spine (Supplemental Digital Content, http://links.lww.com/ALN/C263). Loeys–Dietz syndrome is a Marfan-like autosomal dominant connective tissue disorder which typically includes aneurysmal aorta or adjacent arterial branches.1  The vascular course is aggressive, with complications developing at smaller aortic dimensions compared to Marfan syndrome. Many patients die in their 20s due to aortic dissection. Up to 60% of patients with Loeys–Dietz syndrome show scoliosis, which can compress the heart, particularly when manifesting with concomitant pectus excavatum.2  Apart from cardiovascular risks, airway management can be complicated in these patients by craniosynostosis, cleft palate, retrogenia, and cervical spine instability. Cervical spine manipulation should be minimized by using video laryngoscopy or fiberoptic intubation for airway management.3