Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions

Howard Song, Joseph E. Bavaria, Mark W. Kindem, Kathryn Holmes, Dianna M. Milewicz, Cheryl Maslen, Reed E. Pyeritz, Craig T. Basson, Kim Eagle, H. Eser Tolunay, Barbara L. Kroner, Hal Dietz, Victor Menashe, Richard B. Devereux, Patrice Desvigne-Nickens, William Ravekes, Jonathan W. Weinsaft, Donald Brambilla, Mario P. Stylianou, Tabitha Hendershot & 2 others Megan S. Mitchell, Scott A. LeMaire

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Background: Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the National Institutes of Health-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs. Methods: GenTAC's aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from the 606 patients (mean age, 37.5 years) enrolled in GenTAC to date whose clinical data were available. Results: The patients' primary diagnoses included Marfan syndrome (35.8%), bicuspid aortic valve with aneurysm (29.2%), and familial TAAs and dissections (10.7%). Of these, 56.4% had undergone at least one operation; the most common indications were aneurysm (85.7%), valve dysfunction (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for operation, and procedure type were identified. Conclusions: Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgical repair. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for intervention. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs.

Original languageEnglish (US)
Pages (from-to)781-788
Number of pages8
JournalAnnals of Thoracic Surgery
Volume88
Issue number3
DOIs
StatePublished - Sep 2009

Fingerprint

Thoracic Aortic Aneurysm
Registries
Thorax
Aneurysm
Aorta
Therapeutics
Dissection
Marfan Syndrome
Inborn Genetic Diseases
National Institutes of Health (U.S.)
Thoracic Aorta
Aortic Valve
Tertiary Care Centers
Biomarkers
Phenotype

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions. / Song, Howard; Bavaria, Joseph E.; Kindem, Mark W.; Holmes, Kathryn; Milewicz, Dianna M.; Maslen, Cheryl; Pyeritz, Reed E.; Basson, Craig T.; Eagle, Kim; Tolunay, H. Eser; Kroner, Barbara L.; Dietz, Hal; Menashe, Victor; Devereux, Richard B.; Desvigne-Nickens, Patrice; Ravekes, William; Weinsaft, Jonathan W.; Brambilla, Donald; Stylianou, Mario P.; Hendershot, Tabitha; Mitchell, Megan S.; LeMaire, Scott A.

In: Annals of Thoracic Surgery, Vol. 88, No. 3, 09.2009, p. 781-788.

Research output: Contribution to journalArticle

Song, H, Bavaria, JE, Kindem, MW, Holmes, K, Milewicz, DM, Maslen, C, Pyeritz, RE, Basson, CT, Eagle, K, Tolunay, HE, Kroner, BL, Dietz, H, Menashe, V, Devereux, RB, Desvigne-Nickens, P, Ravekes, W, Weinsaft, JW, Brambilla, D, Stylianou, MP, Hendershot, T, Mitchell, MS & LeMaire, SA 2009, 'Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions', Annals of Thoracic Surgery, vol. 88, no. 3, pp. 781-788. https://doi.org/10.1016/j.athoracsur.2009.04.034
Song, Howard ; Bavaria, Joseph E. ; Kindem, Mark W. ; Holmes, Kathryn ; Milewicz, Dianna M. ; Maslen, Cheryl ; Pyeritz, Reed E. ; Basson, Craig T. ; Eagle, Kim ; Tolunay, H. Eser ; Kroner, Barbara L. ; Dietz, Hal ; Menashe, Victor ; Devereux, Richard B. ; Desvigne-Nickens, Patrice ; Ravekes, William ; Weinsaft, Jonathan W. ; Brambilla, Donald ; Stylianou, Mario P. ; Hendershot, Tabitha ; Mitchell, Megan S. ; LeMaire, Scott A. / Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions. In: Annals of Thoracic Surgery. 2009 ; Vol. 88, No. 3. pp. 781-788.
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abstract = "Background: Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the National Institutes of Health-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs. Methods: GenTAC's aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from the 606 patients (mean age, 37.5 years) enrolled in GenTAC to date whose clinical data were available. Results: The patients' primary diagnoses included Marfan syndrome (35.8{\%}), bicuspid aortic valve with aneurysm (29.2{\%}), and familial TAAs and dissections (10.7{\%}). Of these, 56.4{\%} had undergone at least one operation; the most common indications were aneurysm (85.7{\%}), valve dysfunction (65.8{\%}), and dissection (25.4{\%}). Surgical procedures included replacement of the aortic root (50.6{\%}), ascending aorta (64.8{\%}), aortic arch (27.9{\%}), and descending or thoracoabdominal aorta (12.4{\%}). Syndrome-specific differences in age, indications for operation, and procedure type were identified. Conclusions: Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgical repair. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for intervention. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs.",
author = "Howard Song and Bavaria, {Joseph E.} and Kindem, {Mark W.} and Kathryn Holmes and Milewicz, {Dianna M.} and Cheryl Maslen and Pyeritz, {Reed E.} and Basson, {Craig T.} and Kim Eagle and Tolunay, {H. Eser} and Kroner, {Barbara L.} and Hal Dietz and Victor Menashe and Devereux, {Richard B.} and Patrice Desvigne-Nickens and William Ravekes and Weinsaft, {Jonathan W.} and Donald Brambilla and Stylianou, {Mario P.} and Tabitha Hendershot and Mitchell, {Megan S.} and LeMaire, {Scott A.}",
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T1 - Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions

AU - Song, Howard

AU - Bavaria, Joseph E.

AU - Kindem, Mark W.

AU - Holmes, Kathryn

AU - Milewicz, Dianna M.

AU - Maslen, Cheryl

AU - Pyeritz, Reed E.

AU - Basson, Craig T.

AU - Eagle, Kim

AU - Tolunay, H. Eser

AU - Kroner, Barbara L.

AU - Dietz, Hal

AU - Menashe, Victor

AU - Devereux, Richard B.

AU - Desvigne-Nickens, Patrice

AU - Ravekes, William

AU - Weinsaft, Jonathan W.

AU - Brambilla, Donald

AU - Stylianou, Mario P.

AU - Hendershot, Tabitha

AU - Mitchell, Megan S.

AU - LeMaire, Scott A.

PY - 2009/9

Y1 - 2009/9

N2 - Background: Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the National Institutes of Health-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs. Methods: GenTAC's aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from the 606 patients (mean age, 37.5 years) enrolled in GenTAC to date whose clinical data were available. Results: The patients' primary diagnoses included Marfan syndrome (35.8%), bicuspid aortic valve with aneurysm (29.2%), and familial TAAs and dissections (10.7%). Of these, 56.4% had undergone at least one operation; the most common indications were aneurysm (85.7%), valve dysfunction (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for operation, and procedure type were identified. Conclusions: Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgical repair. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for intervention. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs.

AB - Background: Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the National Institutes of Health-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs. Methods: GenTAC's aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from the 606 patients (mean age, 37.5 years) enrolled in GenTAC to date whose clinical data were available. Results: The patients' primary diagnoses included Marfan syndrome (35.8%), bicuspid aortic valve with aneurysm (29.2%), and familial TAAs and dissections (10.7%). Of these, 56.4% had undergone at least one operation; the most common indications were aneurysm (85.7%), valve dysfunction (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for operation, and procedure type were identified. Conclusions: Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgical repair. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for intervention. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs.

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