Project details

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The outcomes of ten year follow-up of relatives at risk for sudden death due to dilatation/ dissection of the aorta: time for improvement?

Keywords:
Outcomes Relatives Aorta

Researchers:
Prof. I.M. van Langen
drs. K. Nieuwhof
Dr. E. Birnie

Nature of the research:
This is a retrospective cohort study on the uptake of recommended aortic screening, follow-up and outcomes of relatives at risk for aortic dilatation and dissection. The index patients and their relatives visited the Departments of Genetics and Cardiology at the University Medical Center Groningen (UMCG) or one of the outpatient clinics between January 2004 and January 2014. In this study data are collected retrospectively from patient files.

Fields of study:
cardiology and thoracic surgery genetics

Background / introduction
Dilatation of the ascending aorta is often hereditary. Without timely treatment (replacement of the dilated part of the aorta) young patients may die from aortic dissection. Therefore regular cardiologic screening of relatives is important and recommended (Erbel et al., 2014), especially in families of index patients in whom Familial Thoracic Aortic Aneurysms and Dissections (FTAAD), Marfan Syndrome (MFS) or Loeys‚ÄźDietz Syndrome (LDS) is diagnosed. Literature shows that participation in the screening and timely detection and treatment of aorta dilatation/ dissection can avoid disease-related morbidity and mortality in patients and their relatives (Erbel et al., 2014). In the past decade our index patients have therefore been advised to inform their relatives about their risk of aorta dilatation/ dissection and to recommend participation in the screening. Follow up of these relatives could shed light on the uptake of screening, follow-up and outcomes and the possible reasons to reject screening.
In this retrospective cohort study we describe to what degree these advices were followed and what resulting outcomes of screening and follow-up of these (500) relatives are. Eventually we will investigate if these advices indeed led to health benefits for these relatives.
Research question / problem definition
To describe (1) the dissemination of screening advices in families at risk of sudden death due to aortic dissection, (2) the adherence to these recommendations and (3) the outcomes in these families.
The results will be used to evaluate the existing guidelines for relatives at risk for dilatation/ dissection of the aorta; to give recommendations on the optimal strategy to disseminate these advices within families at risk; and to formulate topics for future research.
Workplan
We already selected the 500 eligible relatives of index patients that attended our outpatient clinic in the past decade. We still have to collect data of approximately 350 relatives and enter these data in the database. The next step will be to analyze and publish these data. This project is part of PhD study.
References
Erbel R, Aboyans V, Boileau C et al. ESC Guidelines on the diagnosis and treatment of aortic
diseases. The Task Force for the Diagnosis and Treatment of Aortic diseases of the European
Society of Cardiology (ESC). 2014.
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