Resources for Clinicians and Medical Professionals
Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder with multisystem involvement and variability in the type and severity of manifestations. It is best monitored and treated by a multidisciplinary team.
Head-to-Toe of Features Observed in Loeys-Dietz Syndrome
Features are described by body system, symptoms, diagnostic test, treatment, and more.
VASCERN Do’s and Don’ts for Situations Frequently Faced by Loeys-Dietz Syndrome Patients
Discusses pregnancy, delivery, and postpartum care, anaesthesia, aortic dissection, physical activity, retinal detachment, contraindicated medication, emergencies, allergies, and anaphylactic shock.
General Information on Loeys-Dietz Syndrome
Information on our website.
Article: Loeys-Dietz Syndrome (2018), by Dr. Bart Loeys and Dr. Harry Dietz
Discusses clinical characteristics, diagnosis and genetic counselling, management, activities and medication to avoid, risk for family members, and pregnancy.
Cardiovascular Risk Calculators
Indexes and calculators of aortic root z-score, vertebral tortuosity, patient outcomes, and more.
- Parameterz: http://www.parameterz.com/sites/aortic-root
- Pediatric Heart Network Z-Score: http://www.parameterz.com/refs/lopez-circimaging-2017
- Boston Z-Score Calculator: https://marfan.org/dx/zscore-children/
- Parameterz: http://www.parameterz.com/sites/aortic-root
- Boston Z-Score Calculator: https://marfan.org/dx/z-score-adults/
- Maximum aortic size divided by surface area: https://www.annalsthoracicsurgery.org/article/S0003-4975(05)01031-3/fulltext#secd17620610e1700
- Regalado et al.: https://www.jacc.org/doi/10.1016/j.jacc.2022.05.054
- Holmes et al.: https://www.jacc.org/doi/full/10.1016/j.jacc.2022.03.367
- Quezada et al.: https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.37208
- Parameterz: http://www.parameterz.com/refs/quezada-ajmg-2015
2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease
A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.
Guidelines for LDS imaging, medical therapy, surgical thresholds and considerations, pregnancy, cardiac rehabilitation, shared decision making, multidisciplinary teams, and more.
Guidelines for LDS imaging, medical therapy, surgical thresholds and considerations, pregnancy, cardiac rehabilitation, shared decision making, multidisciplinary teams, and more.
UTHealth Genomic Medicine Guidance Application
A point-of-care tool with clinical and genetic information on the following genes: TGFBR1, TGFBR2, TGFB2, SMAD3, ACTA2, PRKG1, MYLK, and MYH11.