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Yskert von Kodolitsch,1 Meike Rybczynski,1 Marina Vogler,2 Thomas S Mir,3 Helke Schüler,1 Kerstin Kutsche,4 Georg Rosenberger,4 Christian Detter,5 Alexander M Bernhardt,5 Axel Larena-Avellaneda,6 Tilo Kölbel,6 E Sebastian Debus,6 Malte Schroeder,7,8 Stephan J Linke,9,10 Bettina Fuisting,9 Barbara Napp,1 Anna Lena Kammal,11 Klaus Püschel,11 Peter Bannas,12 Boris A Hoffmann,13 Nele Gessler,13 Eva Vahle-Hinz,14 Bärbel Kahl-Nieke,14 Götz Thomalla,15 Christina Weiler-Normann,16 Gunda Ohm,17 Stefan Neumann,18 Dieter Benninghoven,19 Stefan Blankenberg,1 Reed E Pyeritz20 1Clinic of Cardiology, University Heart Centre, 2Marfan Hilfe Deutschland e.V., Zentrumsehstärke, 3Clinic for Pediatric Cardiology, University Heart Centre, 4Institute of Human Genetics, 5Clinic of Cardiovascular Surgery, University Heart Centre, 6Clinic of Vascular Medicine, University Heart Centre, 7Department of Trauma, Hand, and Reconstructive Surgery, 8Department of Orthopedics, 9Clinic of Ophthalmology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany; 10Smilow Center for Translational Research, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA; 11Department of Legal Medicine, 12Diagnostic and Interventional Radiology Department and Clinic, 13Clinic of Electrophysiology, University Heart Centre, 14Department of Orthodontics, Center for Dental and Oral Medicine, 15Clinic of Neurology, 16Martin Zeitz Center for Rare Diseases, 17Strategic Business Development, 18Business Unit Quality Management, University Medical Center Hamburg-Eppendorf, 19Mühlenberg-Clinic for Rehabilitation, Bad Malente-Gremsmühlen, 20Zentrumsehstärke, Hamburg, Germany Abstract: Marfan syndrome (MFS) is a rare, severe, chronic, life-threatening disease with multiorgan involvement that requires optimal multidisciplinary care to normalize both prognosis and quality of life. In this article, each key team member of all the medical disciplines of a multidisciplinary health care team at the Hamburg Marfan center gives a personal account of his or her contribution in the management of patients with MFS. The authors show how, with the support of health care managers, key team members organize themselves in an organizational structure to create a common meaning, to maximize therapeutic success for patients with MFS. First, we show how the initiative and collaboration of patient representatives, scientists, and physicians resulted in the foundation of Marfan centers, initially in the US and later in Germany, and how and why such centers evolved over time. Then, we elucidate the three main structural elements; a team of coordinators, core disciplines, and auxiliary disciplines of health care. Moreover, we explain how a multidisciplinary health care team integrates into many other health care structures of a university medical center, including external quality assurance; quality management system; clinical risk management; center for rare diseases; aorta center; health care teams for pregnancy, for neonates, and for rehabilitation; and in structures for patient centeredness. We provide accounts of medical goals and standards for each core discipline, including pediatricians, pediatric cardiologists, cardiologists, human geneticists, heart surgeons, vascular surgeons, vascular interventionists, orthopedic surgeons, ophthalmologists, and nurses; and of auxiliary disciplines including forensic pathologists, radiologists, rhythmologists, pulmonologists, sleep specialists, orthodontists, dentists, neurologists, obstetric surgeons, psychiatrist/psychologist, and rehabilitation specialists. We conclude that a multidisciplinary health care team is a means to maximize therapeutic success. Keywords: multidisciplinary, Marfan syndrome, health care, team, profession, sociology, management

Marfan syndrome (MFS) is the exemplar of chronic genetic disorders that require multiorgan system management by health care providers (HCPs) and lifelong self-management by affected individuals. This article describes the ways to facilitate transition to self-management (TSM) by the adolescent with MFS. This thematic content analysis uses data collected for a larger grounded theory investigation of TSM of a chronic genetic disorder in adolescents and focuses on the system issues related to transition. A total sample of 107 included three groups of participants: parents ( n = 39), adolescents ( n = 37, ages 14–21 years) and young adults ( n = 16, ages 22–34 years) with MFS, and HCPs ( n = 15), including physicians, genetic counselors, and nurses. Data were derived from 180 transcripts of audiotaped interviews and a sociodemographic survey. Frames of mind that are antecedent to transition were belief in the diagnosis, wanting to understand and appreciate the cause and effect of MFS, and willing to share responsibility in problem solving. These frames of mind occurred primarily within the context of the family relationship. Parents taught children self-surveillance as ‘listening to one’s body’. The parent’s fears and need to stay involved in the child’s health care slowed the child’s independent work on self-management responsibilities. A systematic, institutionalized transition program for adolescents might involve parents and the child soon after diagnosis and incrementally build acknowledgment, understanding, and rapport. [ABSTRACT FROM AUTHOR]

Circulatory vessels are subject to diverse pathologic processes that depend on the properties of the vessels. Vascular problems such as dilatation, dissection, and rupture can occur as a result of many disorders. Conditions that affect vessels include inherited disorders such as Marfan syndrome and the Ehlers-Danlos syndromes and poorly understood congenital disorders such as arteriovenous malformations and capillary fragility. Depending on which condition patients have, they must be given proper counseling about activity and options for management. For some patients, strenuous exercise, pregnancy, and competitive sports are prohibited, but for others with milder disorders, participation in selected sports is permitted. [ABSTRACT FROM AUTHOR]