@article{9d8fa75988234c16aacb09fe7dc7e23a,
title = "Open Thoracoabdominal Aortic Repair in Patients With Heritable Aortic Disease in the GenTAC Registry",
abstract = "Background: Although patients with various types of heritable aortopathy often require distal aortic repair, data are limited regarding the most extensive operations—open thoracoabdominal aortic aneurysm (TAAA) repairs. The objective of this multicenter registry study was to characterize TAAA repairs in a large cohort of patients with different heritable aortic diseases. Methods: From the 3699 patients enrolled at 8 participating centers in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) Registry, we identified 155 open TAAA repairs in 142 unique patients. We examined data related to clinical characteristics, surgical techniques, and outcomes. Results: The primary diagnoses included Marfan syndrome (n = 76; 54%), familial thoracic aortic aneurysm and dissections (n = 31; 22%), and Loeys-Dietz syndrome (n = 10; 7%). Most repairs were performed for aneurysms associated with aortic dissection (n = 110; 71%). The most common repairs involved the entire descending thoracic aorta with distal extension (21% Crawford extent I and 36% extent II). Adjuncts used during repair varied substantially. The operative mortality rate was 1.3%. Other complications included paraplegia (4%), acute renal failure (5%), and vocal cord paralysis (21%). Reoperation after TAAA repair was required in a subset of cases for early bleeding (n = 15; 10%) and late repair failure (n = 7; 5%). Conclusions: Open TAAA repairs are necessary in a variety of heritable aortic diseases. These patients often require extensive surgical repair, and a variety of adjunctive techniques are utilized. The risk of repair failure and the need for reoperation in a subset of patients support the need for vigilant long-term surveillance after repair.",
author = "{GenTAC Investigators} and Frankel, {William C.} and Song, {Howard K.} and Milewski, {Rita K.} and Sherene Shalhub and Pugh, {Norma L.} and Eagle, {Kim A.} and Roman, {Mary J.} and Pyeritz, {Reed E.} and Maslen, {Cheryl L.} and Ravekes, {William J.} and Milewicz, {Dianna M.} and Coselli, {Joseph S.} and LeMaire, {Scott A.} and Federico Asch and Joseph Bavaria and Patrice Desvigne-Nickens and Richard Devereux and Harry Dietz and Kim Eagle and Jennifer Habashi and Kathryn Holmes and Barbara Kroner and Nazli McDonnell and Cheryl Maslen and Dianna Milewicz and Rita Milewski and Shaine Morris and Siddharth Prakash and Reed Pyeritz and William Ravekes and Mary Roman and Ralph Shohet and Silberbach, {G. Michael} and Howard Song and Tolunay, {H. Eser} and Hung Tseng and Jonathan Weinsaft",
note = "Funding Information: The GenTAC Registry has been supported by US Federal Government contracts HHSN268200648199C and HHSN268201000048C from the National Heart Lung and Blood Institute and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (Bethesda, MD). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Dr LeMaire's work is supported in part by the Jimmy and Roberta Howell Professorship in Cardiovascular Surgery at Baylor College of Medicine. The authors gratefully acknowledge Scott Weldon, MA, CMI, of Baylor College of Medicine, for creating the illustration. The GenTAC Registry Investigators are: Federico Asch, MD; Joseph Bavaria, MD; Patrice Desvigne-Nickens, MD; Richard Devereux, MD, PhD; Harry Dietz, MD, PhD; Kim Eagle, MD; Jennifer Habashi, MD; Kathryn Holmes, MD; Barbara Kroner, PhD; Scott LeMaire, MD; Nazli McDonnell, MD; Cheryl Maslen, PhD; Dianna Milewicz, MD, PhD; Rita Milewski, MD, PhD; Shaine Morris, MD, MPH; Siddharth Prakash, MD, PhD; Reed Pyeritz, MD, PhD; William Ravekes, MD; Mary Roman, MD; Ralph Shohet, MD; G. Michael Silberbach, MD; Howard Song, MD, PhD; H. Eser Tolunay, PhD; Hung Tseng, PhD; and Jonathan Weinsaft, MD. Funding Information: The GenTAC Registry has been supported by US Federal Government contracts HHSN268200648199C and HHSN268201000048C from the National Heart Lung and Blood Institute and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (Bethesda, MD). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Dr LeMaire{\textquoteright}s work is supported in part by the Jimmy and Roberta Howell Professorship in Cardiovascular Surgery at Baylor College of Medicine . The authors gratefully acknowledge Scott Weldon, MA, CMI, of Baylor College of Medicine, for creating the illustration. The GenTAC Registry Investigators are: Federico Asch, MD; Joseph Bavaria, MD; Patrice Desvigne-Nickens, MD; Richard Devereux, MD, PhD; Harry Dietz, MD, PhD; Kim Eagle, MD; Jennifer Habashi, MD; Kathryn Holmes, MD; Barbara Kroner, PhD; Scott LeMaire, MD; Nazli McDonnell, MD; Cheryl Maslen, PhD; Dianna Milewicz, MD, PhD; Rita Milewski, MD, PhD; Shaine Morris, MD, MPH; Siddharth Prakash, MD, PhD; Reed Pyeritz, MD, PhD; William Ravekes, MD; Mary Roman, MD; Ralph Shohet, MD; G. Michael Silberbach, MD; Howard Song, MD, PhD; H. Eser Tolunay, PhD; Hung Tseng, PhD; and Jonathan Weinsaft, MD. Publisher Copyright: {\textcopyright} 2020 The Society of Thoracic Surgeons",
year = "2020",
month = may,
doi = "10.1016/j.athoracsur.2019.08.047",
language = "English (US)",
volume = "109",
pages = "1378--1384",
journal = "Annals of Thoracic Surgery",
issn = "0003-4975",
publisher = "Elsevier USA",
number = "5",
}