Fatigue in patients with syndromic heritable thoracic aortic disease: a systematic review of the literature and a qualitative study of patients’ experiences and perceptions

Systematic review resultsSearch results

The search strategy is presented in the flow chart in Fig. 1. A total of 867 articles were identified, 251 were read full text, of these 33 articles satisfied the eligibility criteria and were included in this review. There were 30 primary and three secondary (review) studies.

Fig. 1figure 1

Flowchart of search, screening and inclusion process of the systematic review

Included secondary studies: review articles

Three review articles fulfilled our inclusion criteria [19, 20, 51], but in no reviews the primary outcome was fatigue. Two review articles dealt with psychosocial aspects of MFS. One [20] included 11 articles dealing with fatigue, and the other [19] four articles. The last review article [51], addressing quality of life (QoL) in people with HTADs, included five articles of fatigue. All three review articles indicated that fatigue appears to be prevalent in patients with sHTADs, but none reported the estimated prevalence of fatigue of included studies. Nevertheless, all reported that fatigue in patients with MFS seems to have a huge impact on their QoL and daily life.

Included primary studies: fatigue in sHTADs

Of the 30 primary studies dealing with fatigue in different sHTADs, 25 dealt with adults while five with children/adolescents. Twenty-one articles on patients with MFS (17 on adults and 4 on children), one article on MFS and EDS, two on EDS, three on vEDS/LDS, and three on different sHTADs (two on adults/one on children). One third (n = 10) of the studies [52,53,54,55,56,57,58,59,60,61] did not describe if they included patients with verified diagnoses. The diagnoses were either self-reported or the confirmation of diagnoses was not reported in the study. The rest of the studies either included patients diagnosed according to the Ghent Nosology for MFS, or genetic verified diagnoses for LDS, vEDS and the other sHTADs. Eighty-three percent of the articles were published from 2010 to October 2022, with 70% published from 2015 onward. Twenty-three articles (77%) were from Europe, six (20%) from USA, and one (3%) from Korea (Table 2).

Table 2 Data extraction of included studies of patients with sHTADsMethodological appraisal of primary studies

Study design and level of focus on fatigue: Of the 30 primary articles 22 studies were cross-sectional quantitative questionnaire studies [11, 13, 16, 17, 31, 32, 52,53,54, 56,57,58, 61,62,63,64,65,66,67,68,69,70]. Four were prospective: one pilot rehabilitation intervention study [71], two experimental studies [55, 72] and one longitudinal study [73]. Four studies were qualitative and used different methods: individual interviews [74], focus groups [12], document analyses [59] and combining individual interviews with focus groups [60]. Three of the qualitative studies dealt with children/adolescents [59, 60, 74]. No randomized controlled studies were identified.

In only eight [16, 17, 31, 32, 55, 57, 61, 68] of the 30 articles the primary outcome was to investigate fatigue. All these were cross-sectional quantitative studies. Four were on MFS, two on EDS, one on vEDS/LDS, and one on children with different sHTADs (MFS, vEDS) (Table 3).

Table 3 Quality assessment om included studies; quantitative studies, qualitative studies and review studies

Recruitment and sample sizes: In most papers [11,12,13, 16, 17, 31, 32, 52, 55, 58, 60,61,62,63, 65,66,67,68,69,70, 72,73,74] the participants were recruited from the clinic where the researchers worked, and in some [54, 56, 57, 59, 64] from the patient organization, or from both [53, 71]. Approximately, a total of 2,479 adults were included in the articles, with a variation of 16 [31] to 318 [54] respondents (mean 94/median 64), and in most studies the response rate was low. Four studies had more than 200 participants [54, 57, 63, 64], but the sample size of these studies probably represents a small percentage of the estimated national patient populations. In the largest study [54], the diagnosis was self-reported and the link to the survey was sent to 13,280 persons, of whom 318 completed the survey, indicating a response rate of 2% of the study population.

Instruments for assessing fatigue and vitality

Ten studies [11, 16, 17, 31, 63, 66, 67, 69, 71, 72] used Fatigue Severity Scale (FSS) two [55, 68] used Multidimensional Fatigue Inventory (MFI-20), two [

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