Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results

Nishant D. Patel, Todd Crawford, J. Trent Magruder, Diane E. Alejo, Narutoshi Hibino, James Black, Harry C. Dietz, Luca A. Vricella, Duke E. Cameron

Research output: Research - peer-reviewArticle

Abstract

Objectives Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. Methods We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Results Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Conclusions Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed.

LanguageEnglish (US)
Pages406-412
Number of pages7
JournalJournal of Thoracic and Cardiovascular Surgery
Volume153
Issue number2
DOIs
StatePublished - Feb 1 2017

Fingerprint

Loeys-Dietz Syndrome
Dissection
Aneurysm
Sternotomy
Aortic Aneurysm
Thoracic Aorta
Connective Tissue
Aorta
Rupture
Survival
Mortality
Therapeutics
Bicuspid Aortic Valve

Keywords

  • aortic aneurysm
  • Loeys-Dietz syndrome

ASJC Scopus subject areas

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

Cite this

Cardiovascular operations for Loeys-Dietz syndrome : Intermediate-term results. / Patel, Nishant D.; Crawford, Todd; Magruder, J. Trent; Alejo, Diane E.; Hibino, Narutoshi; Black, James; Dietz, Harry C.; Vricella, Luca A.; Cameron, Duke E.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 153, No. 2, 01.02.2017, p. 406-412.

Research output: Research - peer-reviewArticle

Patel, Nishant D. ; Crawford, Todd ; Magruder, J. Trent ; Alejo, Diane E. ; Hibino, Narutoshi ; Black, James ; Dietz, Harry C. ; Vricella, Luca A. ; Cameron, Duke E./ Cardiovascular operations for Loeys-Dietz syndrome : Intermediate-term results. In: Journal of Thoracic and Cardiovascular Surgery. 2017 ; Vol. 153, No. 2. pp. 406-412
@article{534739f332a842b58340390f5e4e2d76,
title = "Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results",
abstract = "Objectives Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. Methods We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Results Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Conclusions Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed.",
keywords = "aortic aneurysm, Loeys-Dietz syndrome",
author = "Patel, {Nishant D.} and Todd Crawford and Magruder, {J. Trent} and Alejo, {Diane E.} and Narutoshi Hibino and James Black and Dietz, {Harry C.} and Vricella, {Luca A.} and Cameron, {Duke E.}",
year = "2017",
month = "2",
doi = "10.1016/j.jtcvs.2016.10.088",
volume = "153",
pages = "406--412",
journal = "Journal of Thoracic and Cardiovascular Surgery",
issn = "0022-5223",
publisher = "Mosby Inc.",
number = "2",

}

TY - JOUR

T1 - Cardiovascular operations for Loeys-Dietz syndrome

T2 - Journal of Thoracic and Cardiovascular Surgery

AU - Patel,Nishant D.

AU - Crawford,Todd

AU - Magruder,J. Trent

AU - Alejo,Diane E.

AU - Hibino,Narutoshi

AU - Black,James

AU - Dietz,Harry C.

AU - Vricella,Luca A.

AU - Cameron,Duke E.

PY - 2017/2/1

Y1 - 2017/2/1

N2 - Objectives Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. Methods We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Results Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Conclusions Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed.

AB - Objectives Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. Methods We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Results Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Conclusions Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed.

KW - aortic aneurysm

KW - Loeys-Dietz syndrome

UR - http://www.scopus.com/inward/record.url?scp=85008204061&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85008204061&partnerID=8YFLogxK

U2 - 10.1016/j.jtcvs.2016.10.088

DO - 10.1016/j.jtcvs.2016.10.088

M3 - Article

VL - 153

SP - 406

EP - 412

JO - Journal of Thoracic and Cardiovascular Surgery

JF - Journal of Thoracic and Cardiovascular Surgery

SN - 0022-5223

IS - 2

ER -