Article
Cardiac & Cardiovascular Systems
Yoshimasa Seike, Koki Yokawa, Shigeki Koizumi, Kenta Masada, Yosuke Inoue, Hiroko Morisaki, Takayuki Morisaki, Hiroaki Sasaki, Hitoshi Matsuda
Summary: This study evaluated the surgical outcomes of valve-sparing root replacement in patients with Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS). The results showed that patients with MFS and LDS had similar incidences of aortic valve insufficiency and aortic valve reoperation. However, patients with LDS had a higher risk of aortic dissection and aortic reoperation after the initial operation.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2022)
Article
Genetics & Heredity
Daniel Maghsoudi, Thomas R. W. Nixon, Martin P. Snead
Summary: Loeys-Dietz syndrome is a connective tissue disorder similar to Marfan syndrome, but retinal detachment is rarely reported. This study reports a 5-generation family affected by LDS, where 6 eyes of 4 individuals had retinal detachment. The study suggests that ophthalmic examination should be added to the initial assessment of LDS patients and patients should be informed of the early warning symptoms of retinal detachment.
AMERICAN JOURNAL OF MEDICAL GENETICS PART A
(2023)
Editorial Material
Cardiac & Cardiovascular Systems
Shinichiro Oda, Satoshi Fujita, Hideki Tatewaki, Akira Shiose
Summary: This article reports a case of coronary artery compression associated with aortic root dilation in a patient with congenital heart disease, and introduces an effective method of treatment using valve-sparing aortic root replacement.
ANNALS OF THORACIC SURGERY
(2022)
Article
Cardiac & Cardiovascular Systems
Reilly D. Hobbs, Megan L. Schultz, Megan L. Loney, Sonaal Verma, Julia Xiong, Richard G. Ohye, Edward Bove, Ming-Sing Si
Summary: This study aimed to describe the experience of valve-sparing aortic root replacement in children with prior Ross procedures. Results showed that reimplantation offers better durability compared with the traditional remodeling procedure. Patients with moderate or severe postoperative neoaortic insufficiency were more likely to require subsequent valve replacement.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Yoshimasa Seike, Hitoshi Matsuda, Yosuke Inoue, Hiroaki Sasaki, Hiroko Morisaki, Takayuki Morisaki, Junjiro Kobayashi
Summary: This study compares the surgical outcomes between Marfan syndrome with mutations in the fibrillin gene and Loeys-Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2. The results show that Loeys-Dietz syndrome patients have higher rates of reoperation, particularly for aortic arch. The study suggests that aggressive arch surgery should be recommended in the initial operation for Loeys-Dietz syndrome patients to prevent additional aortic events.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2022)
Article
Cardiac & Cardiovascular Systems
Sebastian Freilinger, Mathieu N. Suleiman, Gert Bischoff, Peter Ewert, Annika Freiberger, Michael Huntgeburth, Ann-Sophie Kaemmerer, Judith Schopen, Christian Meierhofer, Nicole Nagdyman, Harald Kaemmerer, Michael Weyand, Frank Harig
Summary: In this study, the body composition of patients with MFS/LDS was assessed using bioelectrical impedance analysis. It was found that many patients with MFS/LDS are obese, which is often overlooked. BIA was shown to be a superior method for evaluating body composition compared to routine anthropometric parameters. These findings highlight the importance of assessing obesity in MFS/LDS patients for vascular risk evaluation.
REVIEWS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Joseph S. Coselli, Irina V. Volguina, Lynna Nguyen, Susan Y. Green, Scott A. LeMaire, Marc R. Moon
Summary: A study conducted by Joseph S. Coselli and several other researchers is described. The significance of the study is unknown.
ANNALS OF CARDIOTHORACIC SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Monica Chivulescu, Kirsten Krohg-Sorensen, Esther Scheirlynck, Beate R. Lindberg, Lars A. Dejgaard, Oyvind H. Lie, Thomas Helle-Valle, Eystein T. Skjolsvik, Mette E. Estensen, Thor Edvardsen, Per S. Lingaas, Kristina H. Haugaa
Summary: The study found a high prevalence of MAD in patients with MFS and LDS, and MAD was closely associated with aortic events and the need for mitral valve surgery, suggesting that MAD may be a marker of severe disease in MFS and LDS patients.
EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING
(2021)
Article
Cardiac & Cardiovascular Systems
Shuichi Shiraishi, Yutaka Okita, Maya Watanabe, Masanori Tsuchida
Summary: A case of a 2-year-old girl with Loeys-Dietz syndrome complicated by aortic root dilatation and aortic regurgitation is reported. Valve-sparing aortic root replacement with reimplantation technique and aortic valve repair were performed to treat the patient. The latest echocardiography after 5 years showed a stable condition with trivial aortic insufficiency.
INTERACTIVE CARDIOVASCULAR AND THORACIC SURGERY
(2022)
Article
Genetics & Heredity
Stefano Nistri, Rosina De Cario, Elena Sticchi, Gaia Spaziani, Matteo Della Monica, Sabrina Giglio, Silvia Favilli, Betti Giusti, Pierluigi Stefano, Guglielmina Pepe
Summary: Marfan syndrome (MFS) and Loeys-Dietz syndrome type 4 (LDS4) are two hereditary connective tissue disorders with overlapping clinical manifestations but different characteristics, and age plays a role in the onset of symptoms.
Article
Cardiac & Cardiovascular Systems
Albert J. Pedroza, Paul Cheng, Alex R. Dalal, Kathrin Baeumler, Aya Kino, Emily Tognozzi, Rohan Shad, Nobu Yokoyama, Ken Nakamura, Olivia Mitchel, William Hiesinger, Elena Gallo Macfarlane, Dominik Fleischmann, Y. Joseph Woo, Thomas Quertermous, Michael P. Fischbein
Summary: Patients with Loeys-Dietz syndrome who undergo prophylactic aortic arch replacement during valve-sparing aortic root replacement demonstrate excellent surgical outcomes without overt downstream flow or shear stress disturbances. Abnormal smooth muscle cell-mediated aortic remodeling occurs within the normal diameter, clinically at-risk Loeys-Dietz syndrome arch segment.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Aroa Ruiz-Munoz, Andrea Guala, Jose Rodriguez-Palomares, Lydia Dux-Santoy, Luz Servato, Angela Lopez-Sainz, Lucia La Mura, Chiara Granato, Javier Limeres, Teresa Gonzalez-Alujas, Laura Galian-Gay, Laura Gutierrez, Kevin Johnson, Oliver Wieben, Augusto Sao-Aviles, Ignacio Ferreira-Gonzalez, Arturo Evangelista, Gisela Teixido-Tura
Summary: Aortic stiffness is higher in Loeys-Dietz syndrome (LDS) patients, with reduced rotational flow and abnormal wall shear stress compared to healthy volunteers (HV). However, LDS patients showed similar aortic stiffness and overlapping aortic flow features when compared to Marfan syndrome (MFS) patients.
EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING
(2022)
Article
Cardiac & Cardiovascular Systems
Dov Levine, Parth Patel, Yanling Zhao, Patra Childress, Megan Chung, Bradley G. Leshnower, Paul Kurlansky, Craig R. Smith, Edward P. Chen, Hiroo Takayama
Summary: Long-term outcomes of David V valve-sparing aortic root replacement (VSARR) in patients with bicuspid aortic valve (BAV) were compared to those with tricuspid aortic valve (TAV). The survival and reoperation rates after VSARR were similar between BAV and TAV patients, but a higher incidence of reoperation was observed in BAV patients in the late years postoperatively.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Peter Ewert, Frank Harig
Summary: The aim of the study was to explore the relationship between aortic size and body composition in Marfan syndrome and Loeys-Dietz syndrome patients. The results revealed that many patients did not fit the expected slender phenotype and were obese, which was associated with a more severe aortic phenotype.
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2022)
Article
Cardiac & Cardiovascular Systems
Malakh Shrestha, Dietmar Boethig, Heike Kruger, Tim Kaufeld, Andreas Martens, Axel Haverich, Erik Beckmann
Summary: This article presents a 25-year experience with valve-sparing aortic root replacement using a straight tube graft. The procedure was found to have low perioperative morbidity and mortality rates, and the short- and long-term results were excellent.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)