Type B Aortic Dissection in Young Individuals With Confirmed and Presumed Heritable Thoracic Aortic Disease

Research output: Contribution to journalArticle

Authors

  • Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) Consortium

External Institution(s)

  • University of Washington
  • Cornell University
  • University of Michigan, Ann Arbor
  • Baylor College of Medicine
  • Autonomous University of Barcelona

Details

Original languageEnglish (US)
Pages (from-to)534-540
Number of pages7
JournalAnnals of Thoracic Surgery
Volume109
Issue number2
StatusPublished - Feb 2020
Peer-reviewedYes

Abstract

Background: To investigate clinical course of patients with type B aortic dissection (TBAD) occurring at a young age with confirmed or suspected heritable thoracic aortic disease. Methods: Individuals with TBAD occurring at an age <50 years enrolled in the National Registry of the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium were selected for analysis. Three cohorts were compared: Marfan syndrome (MFS TBAD), nonsyndromic familial TBAD (FTBAD), and sporadic TBAD. Demographics, comorbidities, aortic dissection details, and repair were compared. Results: A total of 150 individuals met inclusion criteria (mean age at TBAD, 36.9 ± 9 years): 73 MFS TBAD, 42 FTBAD, and 35 sporadic TBAD. The cohort of sporadic TBAD had more male patients (71.4%) and fewer individuals of European descent (51.4%) compared with MFS TBAD (57.5% male, 84.9% European descent) and FTBAD (59.5% male, 90.5% European descent). There was a stepwise increase in hypertension prevalence across the cohorts (28.8% MFS, 59.5% FTBAD, 71.4% sporadic TBAD, P <.001). Repair of the descending thoracic aorta was performed in 92 cases (67.1% in MFS, 61.9% in FTBAD, and 48.6% sporadic TBAD, P =.18) at a mean of 3.4 ± 5.4 years from TBAD. The repair extent varied. The largest extent of repair was in MFS TBAD, in which thoracoabdominal aortic aneurysm repair was performed in 56.2% compared with 35.7% FTBAD and 17.1% sporadic TBAD (P <.001). Conclusions: Control of hypertension is an essential component of care to decrease the risk of TBAD. Over half of the young individuals with TBAD require aortic repair, and individuals with MFS undergo a larger anatomical extent of repair after TBAD.

Citation formats

APA

Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) Consortium (2020). Type B Aortic Dissection in Young Individuals With Confirmed and Presumed Heritable Thoracic Aortic Disease. Annals of Thoracic Surgery, 109(2), 534-540. https://doi.org/10.1016/j.athoracsur.2019.07.004

Harvard

Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) Consortium 2020, 'Type B Aortic Dissection in Young Individuals With Confirmed and Presumed Heritable Thoracic Aortic Disease', Annals of Thoracic Surgery, vol. 109, no. 2, pp. 534-540. https://doi.org/10.1016/j.athoracsur.2019.07.004