Nishant D Patel1, Todd Crawford1, J Trent Magruder1, Diane E Alejo1, Narutoshi Hibino1, James Black2, Harry C Dietz3, Luca A Vricella1, Duke E Cameron4. 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.
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.
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.
Authors: Alexander J Fletcher; Maaz B J Syed; Timothy J Aitman; David E Newby; Niki L Walker Journal: Circulation Date: 2020-05-11 Impact factor: 29.690
Authors: Kristina Mühlstädt; Julie De Backer; Yskert von Kodolitsch; Kerstin Kutsche; Laura Muiño Mosquera; Jens Brickwedel; Evaldas Girdauskas; Thomas S Mir; Adrian Mahlmann; Nikolaos Tsilimparis; Axel Staebler; Lauritz Schoof; Heide Seidel; Jürgen Berger; Alexander M Bernhardt; Stefan Blankenberg; Tilo Kölbel; Christian Detter; Katalin Szöcs; Harald Kaemmerer Journal: J Clin Med Date: 2019-11-29 Impact factor: 4.241