Sherene Shalhub1, Mary J Roman2, Kim A Eagle3, Scott A LeMaire4, Qianzi Zhang4, Artur Evangelista5, Dianna M Milewicz6. 1. Division of Vascular Surgery, Department of Surgery, The University of Washington, Seattle, Washington. Electronic address: shalhub@uw.edu. 2. Division of Cardiology, Weill Cornell Medical Center, New York, New York. 3. University of Michigan Frankel Cardiovascular Center, University of Michigan Health System, Ann Arbor, Michigan. 4. Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas. 5. Department of Cardiology, University Hospital Vall d'Hebron, Barcelona, Spain. 6. Division of Medical Genetics, Department of Internal Medicine, The University of Texas Health Science Center at Houston, Houston, Texas.
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.
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.
Authors: Anita Kaw; Kaveeta Kaw; Ellen M Hostetler; Ana Beleza-Meireles; Adam Smith-Collins; Catherine Armstrong; Ingrid Scurr; Timothy Cotts; Rajani Aatre; Michael J Bamshad; Dawn Earl; Abraham Groner; Katherine Agre; Yehuda Raveh; Callie S Kwartler; Dianna M Milewicz Journal: Am J Med Genet A Date: 2022-05-14 Impact factor: 2.578
Authors: Kathryn W Holmes; Sheila Markwardt; Kim A Eagle; Richard B Devereux; Jonathan W Weinsaft; Federico M Asch; Scott A LeMaire; Cheryl L Maslen; Howard K Song; Dianna M Milewicz; Siddharth K Prakash; Dongchuan Guo; Shaine A Morris; Reed E Pyeritz; Rita C Milewski; William J Ravekes; H C Dietz; Ralph V Shohet; Michael Silberbach; Mary J Roman Journal: J Am Coll Cardiol Date: 2022-05-31 Impact factor: 27.203
Authors: Dianna M Milewicz; Alan C Braverman; Julie De Backer; Shaine A Morris; Catherine Boileau; Irene H Maumenee; Guillaume Jondeau; Arturo Evangelista; Reed E Pyeritz Journal: Nat Rev Dis Primers Date: 2021-09-02 Impact factor: 65.038