Surgical management of aortic dissection in patients with the Marfan syndrome

Julian A. Smith, James I. Fann, D. Craig Miller, Kathleen A. Moore, Abelardo DeAnda, R. Scott Mitchell, Edward B. Stinson, Philip E. Oyer, Bruce A. Reitz, Norman E. Shumway

Research output: Contribution to journalArticle

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Abstract

Background: Aortic dissection is one of the most lethal potential complications in patients with the Marfan syndrome. Methods and Results: Among 360 patients undergoing operative treatment of aortic dissection between 1963 and 1992, 40 had the Marfan syndrome. There were 24 men and 16 women with a mean age of 35 ± 9 years (±1 SD; range, 15 to 54 years). These patients included 16 with acute type A, 2 with acute type B, 18 with chronic type A, and 4 with chronic type B aortic dissections. The aortic arch was involved in 29 cases. Preoperative complications included acute aortic valvular insufficiency in 13 patients, rupture into the pericardial space in 3, and loss of peripheral pulses in 9. The site of primary intimal tear was the ascending aorta in 25 patients, the aortic arch in 2, the descending aorta in 7, and not identified in 6. Operations included ascending aortic and aortic valvular replacement (with or without coronary artery reimplantation) in 22 patients, ascending aortic replacement alone in 5, and descending thoracic aortic replacement in 9. Four operative deaths (10±5% [±70% confidence limits]) occurred in 3 acute patient-years and 1 chronic type A patient-years. Long-term follow-up (216 patient-years; range, 1 month to 22 years; mean, 5.4 years) revealed 15 late deaths, 7 from late aortic sequelae. The overall actuarial survival estimates were 71±8%, 54±10%, and 22±11% at 5, 10, and 15 years, respectively. Twenty late aortic operations were required in 14 patients. Conclusions: Despite satisfactory early results, the long-term survival of patients with the Marfan syndrome was suboptimal (albeit similar to those without the Marfan syndrome). Future progress will pivot on reducing the incidence of aortic dissection in these patients with medical therapy and/or earlier surgical intervention and enhanced postoperative serial imaging surveillance of the entire aorta.

Original languageEnglish (US)
JournalCirculation
Volume90
Issue number5 II
StatePublished - Nov 1994
Externally publishedYes

Fingerprint

Marfan Syndrome
Dissection
Thoracic Aorta
Aorta
Tunica Intima
Survival
Replantation
Pericardium
Rupture
Coronary Vessels
Thorax

Keywords

  • aorta
  • genetic disorders
  • Marfan syndrome
  • surgery, cardiac

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Smith, J. A., Fann, J. I., Miller, D. C., Moore, K. A., DeAnda, A., Mitchell, R. S., ... Shumway, N. E. (1994). Surgical management of aortic dissection in patients with the Marfan syndrome. Circulation, 90(5 II).

Surgical management of aortic dissection in patients with the Marfan syndrome. / Smith, Julian A.; Fann, James I.; Miller, D. Craig; Moore, Kathleen A.; DeAnda, Abelardo; Mitchell, R. Scott; Stinson, Edward B.; Oyer, Philip E.; Reitz, Bruce A.; Shumway, Norman E.

In: Circulation, Vol. 90, No. 5 II, 11.1994.

Research output: Contribution to journalArticle

Smith, JA, Fann, JI, Miller, DC, Moore, KA, DeAnda, A, Mitchell, RS, Stinson, EB, Oyer, PE, Reitz, BA & Shumway, NE 1994, 'Surgical management of aortic dissection in patients with the Marfan syndrome', Circulation, vol. 90, no. 5 II.
Smith JA, Fann JI, Miller DC, Moore KA, DeAnda A, Mitchell RS et al. Surgical management of aortic dissection in patients with the Marfan syndrome. Circulation. 1994 Nov;90(5 II).
Smith, Julian A. ; Fann, James I. ; Miller, D. Craig ; Moore, Kathleen A. ; DeAnda, Abelardo ; Mitchell, R. Scott ; Stinson, Edward B. ; Oyer, Philip E. ; Reitz, Bruce A. ; Shumway, Norman E. / Surgical management of aortic dissection in patients with the Marfan syndrome. In: Circulation. 1994 ; Vol. 90, No. 5 II.
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abstract = "Background: Aortic dissection is one of the most lethal potential complications in patients with the Marfan syndrome. Methods and Results: Among 360 patients undergoing operative treatment of aortic dissection between 1963 and 1992, 40 had the Marfan syndrome. There were 24 men and 16 women with a mean age of 35 ± 9 years (±1 SD; range, 15 to 54 years). These patients included 16 with acute type A, 2 with acute type B, 18 with chronic type A, and 4 with chronic type B aortic dissections. The aortic arch was involved in 29 cases. Preoperative complications included acute aortic valvular insufficiency in 13 patients, rupture into the pericardial space in 3, and loss of peripheral pulses in 9. The site of primary intimal tear was the ascending aorta in 25 patients, the aortic arch in 2, the descending aorta in 7, and not identified in 6. Operations included ascending aortic and aortic valvular replacement (with or without coronary artery reimplantation) in 22 patients, ascending aortic replacement alone in 5, and descending thoracic aortic replacement in 9. Four operative deaths (10±5{\%} [±70{\%} confidence limits]) occurred in 3 acute patient-years and 1 chronic type A patient-years. Long-term follow-up (216 patient-years; range, 1 month to 22 years; mean, 5.4 years) revealed 15 late deaths, 7 from late aortic sequelae. The overall actuarial survival estimates were 71±8{\%}, 54±10{\%}, and 22±11{\%} at 5, 10, and 15 years, respectively. Twenty late aortic operations were required in 14 patients. Conclusions: Despite satisfactory early results, the long-term survival of patients with the Marfan syndrome was suboptimal (albeit similar to those without the Marfan syndrome). Future progress will pivot on reducing the incidence of aortic dissection in these patients with medical therapy and/or earlier surgical intervention and enhanced postoperative serial imaging surveillance of the entire aorta.",
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AU - Fann, James I.

AU - Miller, D. Craig

AU - Moore, Kathleen A.

AU - DeAnda, Abelardo

AU - Mitchell, R. Scott

AU - Stinson, Edward B.

AU - Oyer, Philip E.

AU - Reitz, Bruce A.

AU - Shumway, Norman E.

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N2 - Background: Aortic dissection is one of the most lethal potential complications in patients with the Marfan syndrome. Methods and Results: Among 360 patients undergoing operative treatment of aortic dissection between 1963 and 1992, 40 had the Marfan syndrome. There were 24 men and 16 women with a mean age of 35 ± 9 years (±1 SD; range, 15 to 54 years). These patients included 16 with acute type A, 2 with acute type B, 18 with chronic type A, and 4 with chronic type B aortic dissections. The aortic arch was involved in 29 cases. Preoperative complications included acute aortic valvular insufficiency in 13 patients, rupture into the pericardial space in 3, and loss of peripheral pulses in 9. The site of primary intimal tear was the ascending aorta in 25 patients, the aortic arch in 2, the descending aorta in 7, and not identified in 6. Operations included ascending aortic and aortic valvular replacement (with or without coronary artery reimplantation) in 22 patients, ascending aortic replacement alone in 5, and descending thoracic aortic replacement in 9. Four operative deaths (10±5% [±70% confidence limits]) occurred in 3 acute patient-years and 1 chronic type A patient-years. Long-term follow-up (216 patient-years; range, 1 month to 22 years; mean, 5.4 years) revealed 15 late deaths, 7 from late aortic sequelae. The overall actuarial survival estimates were 71±8%, 54±10%, and 22±11% at 5, 10, and 15 years, respectively. Twenty late aortic operations were required in 14 patients. Conclusions: Despite satisfactory early results, the long-term survival of patients with the Marfan syndrome was suboptimal (albeit similar to those without the Marfan syndrome). Future progress will pivot on reducing the incidence of aortic dissection in these patients with medical therapy and/or earlier surgical intervention and enhanced postoperative serial imaging surveillance of the entire aorta.

AB - Background: Aortic dissection is one of the most lethal potential complications in patients with the Marfan syndrome. Methods and Results: Among 360 patients undergoing operative treatment of aortic dissection between 1963 and 1992, 40 had the Marfan syndrome. There were 24 men and 16 women with a mean age of 35 ± 9 years (±1 SD; range, 15 to 54 years). These patients included 16 with acute type A, 2 with acute type B, 18 with chronic type A, and 4 with chronic type B aortic dissections. The aortic arch was involved in 29 cases. Preoperative complications included acute aortic valvular insufficiency in 13 patients, rupture into the pericardial space in 3, and loss of peripheral pulses in 9. The site of primary intimal tear was the ascending aorta in 25 patients, the aortic arch in 2, the descending aorta in 7, and not identified in 6. Operations included ascending aortic and aortic valvular replacement (with or without coronary artery reimplantation) in 22 patients, ascending aortic replacement alone in 5, and descending thoracic aortic replacement in 9. Four operative deaths (10±5% [±70% confidence limits]) occurred in 3 acute patient-years and 1 chronic type A patient-years. Long-term follow-up (216 patient-years; range, 1 month to 22 years; mean, 5.4 years) revealed 15 late deaths, 7 from late aortic sequelae. The overall actuarial survival estimates were 71±8%, 54±10%, and 22±11% at 5, 10, and 15 years, respectively. Twenty late aortic operations were required in 14 patients. Conclusions: Despite satisfactory early results, the long-term survival of patients with the Marfan syndrome was suboptimal (albeit similar to those without the Marfan syndrome). Future progress will pivot on reducing the incidence of aortic dissection in these patients with medical therapy and/or earlier surgical intervention and enhanced postoperative serial imaging surveillance of the entire aorta.

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KW - genetic disorders

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