Developing a shared decision support framework for aortic root surgery in Marfan syndrome

ObjectiveThe study is an early phase of development of a decision support framework for people with Marfan syndrome who are anticipating prophylactic aortic root surgery. Implications of the timing and the nature of the operation chosen were previously elicited in focus groups. In this step, we explored the range of relative values placed by individuals on the implications of decisions made about surgery.MethodsFollowing the principles of the Ottawa Decision Support Framework, eight questions in the general form ‘How important is it to you …’ were framed by a panel. Marfan people, families and specialist doctors answered online. Quantitative and qualitative analyses were performed.ResultsWorldwide, 142 responses were received including 25 specialist doctors. Respondents were 55% female and 46% had previous aortic root surgery. Overall, active lifestyle was more important to males (p=0.03). Patients placed more importance than doctors on not deferring surgery (p=0.04) and on avoidance of anticoagulation in the interests of childbearing (p=0.009). Qualitative analysis showed differing but cogently reasoned values that were sometimes polarised, and mainly driven by the wish to maintain a good quality of life and active lifestyle.ConclusionsGiven the cogency of these viewpoints, people anticipating root replacement surgery should have ample opportunity to express them and to have them acknowledged ahead of a consultation when they can then be fully explored in a mutually informed forum. If they differ from local medical practice, they can then be discussed in the process of reaching shared and individualised decisions.

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Should Pectus Excavatum Alter Aortic Root Surgery in Patients With Marfan Syndrome? A Computed Tomography Scan–Guided Surgical Strategy Through Left Anterior Thoracotomy

Seminars in Thoracic and Cardiovascular Surgery ◽

10.1053/j.semtcvs.2015.08.002 ◽

2015 ◽

Vol 27 (3) ◽

pp. 328-330 ◽

Cited By ~ 1

Author(s):

Giuseppe M. Raffa ◽

Giovanni Gentile ◽

Lavinia De Monte ◽

Michele Pilato

Keyword(s):

Computed Tomography ◽

Marfan Syndrome ◽

Aortic Root ◽

Pectus Excavatum ◽

Surgical Strategy ◽

Computed Tomography Scan ◽

Aortic Root Surgery ◽

Tomography Scan

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Abstract 9979: Health-Related Quality of Life in Children With Marfan Syndrome

Circulation ◽

10.1161/circ.132.suppl_3.9979 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Jill C Handisides ◽

Danielle Hollenbeck-Pringle ◽

Karen Uzark ◽

Felicia L Trachtenberg ◽

Victoria L Pemberton ◽

...

Keyword(s):

Quality Of Life ◽

At Risk ◽

Marfan Syndrome ◽

Aortic Root ◽

Sample Mean ◽

Population Norms ◽

Patient Reported ◽

Health Related ◽

The Impact

Background: Marfan syndrome (MFS) is an autosomal dominant disorder that affects the heart, aorta, eyes, skeleton, lungs, and other organs. Objective: To assess quality of life (QL) in a large multicenter cohort of children with MFS. Methods: The Pediatric Quality of Life Inventory (PedsQL) was administered to 256 subjects with MFS ages 5-18 years as an ancillary study to the Pediatric Heart Network’s Marfan Trial, which compared the effects of atenolol vs. losartan on aortic root growth. PedsQL scores were compared to population norms by one-sample t-tests. Scores > 1 SD below the population sample mean represent at-risk status for impaired health-related QL. The impact of treatment arm (atenolol vs. losartan), severity of clinical features, and patient-reported symptoms on QL was assessed by general linear models. Results: The subjects had a mean age of 11.8±3.9 years and were 62% male, 84% white, and 88% non-Hispanic. Mean PedsQL scores for MFS subjects were significantly lower than population norms. Overall, scores were in the impaired range for physical QL and psychosocial QL in 34% and 27% of subjects, respectively. QL across multiple domains correlated negatively with frequency of patient-reported symptoms (r=0.32-0.40, p<.0001). Subjects with a reported neurodevelopmental disorder (mainly learning disability, attention deficit disorder, and/or hyperactivity) had lower mean QL scores (5.5-7.4 lower, p<.04). There were no significant differences in QL scores between treatment arms. We found no significant association between QL and aortic root z-score, extent of skeletal involvement, or presence of ectopia lentis. Conclusions: Children with MFS are at risk for impaired QL. Higher number of patient-reported symptoms had the greatest negative impact on QL, rather than treatment arm or severity of cardiac, skeletal, or ocular findings. Future interventions to address patient symptoms and neurodevelopmental disorders could improve QL for children with MFS.

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20th Anniversary Update of the Ottawa Decision Support Framework Part 1: A Systematic Review of the Decisional Needs of People Making Health or Social Decisions

Medical Decision Making ◽

10.1177/0272989x20936209 ◽

2020 ◽

Vol 40 (5) ◽

pp. 555-581 ◽

Cited By ~ 3

Author(s):

Lauren Hoefel ◽

Annette M. O’Connor ◽

Krystina B. Lewis ◽

Laura Boland ◽

Lindsey Sikora ◽

...

Keyword(s):

Systematic Review ◽

Decision Support ◽

Family Involvement ◽

Information Overload ◽

Decision Makers ◽

Difficult Decision ◽

Social Decisions ◽

Patient Decision ◽

Decision Support Framework ◽

Ottawa Decision Support Framework

Background. The Ottawa Decision Support Framework (ODSF) has been used for 20 years to assess and address people’s decisional needs. The evidence regarding ODSF decisional needs has not been synthesized. Objectives. To synthesize evidence from ODSF-based decisional needs studies, identify new decisional needs, and validate current ODSF decisional needs. Methods. A mixed-studies systematic review. Nine electronic databases were searched. Inclusion criteria: studies of people’s decisional needs when making health or social decisions for themselves, a child, or a mentally incapable person, as reported by themselves, families, or practitioners. Two independent authors screened eligibility, extracted data, and quality appraised studies using the Mixed Methods Appraisal Tool. Data were analyzed using narrative synthesis. Results. Of 4532 citations, 45 studies from 7 countries were eligible. People’s needs for 101 unique decisions (85 health, 16 social) were reported by 2857 patient decision makers ( n = 36 studies), 92 parent decision makers ( n = 6), 81 family members ( n = 5), and 523 practitioners ( n = 21). Current ODSF decisional needs were reported in 2 to 40 studies. For 6 decisional needs, there were 11 new (manifestations): 1) information (overload, inadequacy regarding others’ experiences with options), 2) difficult decisional roles (practitioner, family involvement, or deliberations), 3) unrealistic expectations (difficulty believing outcome probabilities apply to them), 4) personal needs (religion/spirituality), 5) difficult decision timing (unpredictable), and 6) unreceptive decisional stage (difficulty accepting condition/need for treatment, powerful emotions limiting information processing, lacking motivation to consider delayed/unpredictable decisions). Limitations. Possible publication bias (only peer-reviewed journals included). Possible missed needs (non-ODSF studies, patient decision aid development studies, 3 ODSF needs added in 2006). Conclusion. We validated current decisional needs, identified 11 new manifestations of 6 decisional needs, and recommended ODSF revisions.

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20th Anniversary Update of the Ottawa Decision Support Framework: Part 2 Subanalysis of a Systematic Review of Patient Decision Aids

Medical Decision Making ◽

10.1177/0272989x20924645 ◽

2020 ◽

Vol 40 (4) ◽

pp. 522-539 ◽

Cited By ~ 1

Author(s):

Lauren Hoefel ◽

Krystina B Lewis ◽

Annette O’Connor ◽

Dawn Stacey

Keyword(s):

Decision Support ◽

Usual Care ◽

Risk Perceptions ◽

Decision Aids ◽

Cochrane Review ◽

Decision Quality ◽

Patient Decision Aids ◽

Patient Decision ◽

Decision Support Framework ◽

Ottawa Decision Support Framework

Background. The Ottawa Decision Support Framework (ODSF) has guided the development of patient decision aids (PtDAs) for 20 years and needs updating across a range of decisions and hypothesized outcomes. Purpose. To determine the effectiveness of ODSF-developed PtDAs on hypothesized outcomes and to recommend framework changes. Data Source. A subanalysis of randomized controlled trials included in the 2017 Cochrane review of PtDAs comparing PtDAs to usual care in adults considering health treatment or screening decisions (searched to 2015). Study Selection. Trials in the original review that evaluated ODSF-developed PtDAs. Data Synthesis. Meta-analyses of ODSF outcomes with similar measurements and descriptions of other reported outcomes. Results. Of 105 trials, 24 evaluated ODSF-developed PtDAs. Compared with usual care, ODSF PtDAs improved knowledge (mean difference [MD] 13.85; 95% confidence interval [CI] 10.32−17.37; 14 trials), increased accurate risk perceptions (risk ratio [RR] 2.41; 95% CI 1.66−3.48; 7 trials), and increased congruence between informed values and chosen options (RR 1.32; 95% CI 1.09−1.59; 4 trials). They reduced perceived decisional needs as measured using the Decisional Conflict Scale (MD −5.92; 95% CI −8.58 to −3.26; 15 trials) and the proportion of undecided patients (RR 0.65; 95% CI 0.50−0.83; 13 trials). Non-ODSF PtDAs, designed with or without a specific framework, also outperformed usual care. Few ODSF trials measured secondary outcomes. Limitations. The included trials had heterogeneity. Conclusion. ODSF PtDAs address decisional needs and improve decision quality; the best indicator of addressing perceived uncertainty is “proportion undecided.” Secondary ODSF outcomes should be reduced to adherence to one’s chosen option and use/costs of health services, which warrant further research.

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