A new publication, co-authored by Prof Alessandro Pini (VASCERN eHealth and Training & Education Chair and HTAD-WG and MSA-WG member), entitled “Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome” is now available.
This study focuses on a cohort of children and adolescents with Marfan syndrome (MFS), a rare vascular disease that affects the body’s connective tissue and that causes aortic root dilation that, if not treated and managed early, can lead to a life-threatening aortic dissection.
The main findings of this paper are that central pulse pressure (CPP; difference between the systolic and diastolic blood pressure in the proximal aorta) and Pulse Pressure Amplification (PPA), which are associated with aortic root diameter (currently the only validated indicator of aortic dissection risk), are impaired in pediatric MFS patients and an increased CPP was reported in patients whose aortic dilation worsened at 1-year follow-up.
This indicates that certain central blood pressure parameters (i.e. CPP and PPA) may be able to detect the earliest hemodynamic abnormalities in pediatric MFS patients, however further longitudinal studies are still needed in order to confirm the predictive ability of these measurements before they can be used with certainty in the assessment of aortic dissection risk in MFS patients.
You can access the full article here
Reference: J Am Heart Assoc. 2017 Nov 7;6(11). pii: e006815. doi: 10.1161/JAHA.117.006815.
Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome.
Grillo A1,2, Salvi P3, Marelli S4, Gao L5, Salvi L6, Faini A1, Trifirò G7, Carretta R8, Pini A4, Parati G1,2.