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

doi:10.1016/j.jtcvs.2016.10.088

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

J Thorac Cardiovasc Surg. 2017 Feb;153(2):406-412. doi: 10.1016/j.jtcvs.2016.10.088. Epub 2016 Nov 16.

Authors

Nishant D Patel¹, Todd Crawford¹, J Trent Magruder¹, Diane E Alejo¹, Narutoshi Hibino¹, James Black², Harry C Dietz³, Luca A Vricella¹, Duke E Cameron⁴

Affiliations

¹ Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
² Division of Vascular Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md.
³ Department of Medicine, The McKusick-Nathans Institute of Genetic Medicine, The Johns Hopkins Medical Institutions, Baltimore, Md.
⁴ Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md. Electronic address: npatel4@jhmi.edu.

PMID: 27955909
DOI: 10.1016/j.jtcvs.2016.10.088

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: Loeys-Dietz syndrome; aortic aneurysm.

MeSH terms

Adolescent
Adult
Aorta / surgery*
Aortic Valve / abnormalities*
Aortic Valve / surgery
Bicuspid Aortic Valve Disease
Cardiac Surgical Procedures / methods*
Female
Follow-Up Studies
Heart Valve Diseases / surgery*
Humans
Loeys-Dietz Syndrome / surgery*
Male
Retrospective Studies
Time Factors
Treatment Outcome
Vascular Surgical Procedures / methods*
Young Adult