The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

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.

Adults with Loeys–Dietz syndrome and vascular Ehlers–Danlos syndrome: A cross‐sectional study of health burden perspectives

“…We speculate that the latter group might not yet suffer from severe cardiovascular symptoms or findings. However, annual examination of the cardiologic status has been recommended as a minimum from the time of diagnosis for all these groups (Byers et al, ; Loeys & Dietz, ; von Kodolitsch et al, ). Our findings also indicate that acute or prophylactic cardiac surgeries are common in LDS, and previous studies have found that prophylactic aortic root surgery is safe and durable (Byers et al, ; Krohg‐Sorensen et al, ).…”

“…This means more individuals will be diagnosed early in life, often without any or with few clinical symptoms. Interdisciplinary medical follow‐up programs are highly recommended, and many patients need specialized aortic surgical treatment prophylactic or acute events (Hiratzka et al, ; Milewicz & Regalado, ; Pepin, Murray, & Byers, ; von Kodolitsch et al, ).…”

Inpatient rehabilitation for adult patients with Marfan syndrome: an observational pilot study

“…However sparse studies and case reports emphasize the need for psychosocial support of affected persons [3–5]. All authors agree on the crucial value of a multidisciplinary approach to rehabilitation for MFS [2, 6]. …”

Features of Marfan syndrome not listed in the Ghent nosology – the dark side of the disease

“…Ideally, a central physician/ caregiver, aware of the many possible features should coordinate the care and relay to appropriate specialists whenever needed. The important role of multidisciplinary teams in the management and treatment of Marfan syndrome has been described previously [381].…”