Table 2 Main findings of PROMs.
Study ID | Sample | Measure | Measure Outcome | Overall Findings | Findings physical health component (PCS) |
|---|---|---|---|---|---|
Cox 1997 | HCM; N = 137 | SF-36 | GENERIC | - Symptom pattern strongly associated with HRQoL and wellbeing; - Patients with dyspnoe reported poorer HRQoL. - HRQoL also associated with patterns of chest pain. | - Patients who experienced both atypical and exertional pain had the lowest scores on physical functioning, role limitations due to physical factors, general health perceptions, vitality and bodily pain. - Syncope →significantly lower PCS and bodily pain scales |
Steptoe 2000 | DCM; N = 60 | SF-36 | GENERIC | - Pronounced restrictions in QoL and wellbeing; - Major limitations on all SF-36 dimensions except bodily pain; - In comparision with HCM pop: DCM sign greater restrictions in social functioning; HCM experience sign higher levels of pain - Several aspects of impaired HRQoL are not simple functions of disease severity, but determined by factors related to psychological adjustment as well. | - Older patients experience more vitality; female sign poorer HRQoL than male in terms of physical funtioning; perc gen health and bodily pain; pat with lower shortening fractions had poorer HRQoL PCS - DCM fam reported better phys funct, fewer role lim. |
Christiaans 2009 | HCM; N = 228 | SF-36 | GENERIC | - Carriers HCM report ↓HRQoL | - Carriers HCM: ↓ general health perception; ↓vitality, ↓bodily pain - Subgroup carriers with manifest HCM sign worse physical prediction |
Hamang 2010 | LQTS, HCM + AR LQTS, AR HCM; N = 127 | SF-36 | GENERIC | - Manifest HCM: scored lower on all health domains compared to patients with LQTS-syndrome or with patients at risk. - ↑ Manifestations/symptoms to affect health | - Physical health differed according to disease status - Manifest HCM, LQTS report poorer physical health - AR patients reported better phys health |
Pedrosa 2010 | N = 126; HCM (n = 84); asymptomatic controls (n = 42) | MLHFQ | DISEASE-SPECIFIC | - Poor HRQoL was independently associated with poor sleep quality, NYHA classification and diuretic therapy. - Congestive heart failure symptoms ass worse HRQoL | - No results on physical health |
Hamang 2011 | LQTS, HCM + AR LQTS, AR HCM; N = 126 | SF-36 | GENERIC | N/A | - ↑ Manifestations/symptoms to affect health |
Ingles 2012 | ICC (33) + AR ICC(21); N = 54 | SF-36 | GENERIC | - No change HRQoL, - No change in health status outcome due to genetic testing | - HCM, DCM, CPVT → ↓ PCS - AR HCM and carriers HCM; AR LQTS; AR DCM→ ↑ PCS |
Ingles 2015 | HCM; N = 486 | SF-36 | GENERIC | - Predictive genetic test for adult onset disease does not lead to long term impairment of HRQoL | - ↑ comorbidities; ↓ income; ↓ specialized clinic |
Brouwers 2015 | NCCM(45), FH(43), acq DCM (42); N = 130 | SF-12 | GENERIC | - Stronger adverse eff on health status in patients with manifest HCM LQTS versus AR patients without symptoms | - ↑ comorbidity, ↑ ICD implant ↓ age, ↓ education |
Richardson 2018 | CPVT; N = 54 | SF-36 | GENERIC | - Vitality (energy and fatigue)domain: worse than gen pop | - No results on physical health |
Hamang 2012 | LQTS, HCM + AR LQTS, AR HCM; N = 173 | SF-36 | GENERIC | N/A | - Physical health differed according to disease status. - Patients with clinical LQTS or clinical HCM reported poorer physical health - Higher avoidance (measured with HFA) is strongly related to poorer physical health |
Huff 2013 | HCM; N = 24 | KCCQ | DISEASE-SPECIFIC | - KCCQ results demonstrated moderate reductions in all domains, with greatest reduction in HRQoL-domain | - KCCQ (physical functioning and overall scores) has moderate correlation with NYHA class and cardiopulmonary exercise test |
McGorrian 2013 | AR ICC; N = 316 | SF-12 | GENERIC | - No association with a difference in HRQoL found when comparing different patients groups (e.g., SADS vs channelopathy vs cardiomyopathy) | - PCS-12 decreased with age (p = 0.01), indicating worsening physical health |
Ingles 2013 | ICC; N = 409 | SF-36 | GENERIC | - HCM, DCM, CPVT→ ↓ PCS - AR HCM and carriers HCM; AR LQTS; AR DCM→ ↑ PCS - Presence and severity of symptoms; activity restrictions; adverse medication side effects have negative effect on health status, anxiety, and depression | - Women; comorbidity; NYHA functional class]≈ impaired physical score - ↓PCS score: physical disability due to lifestyle modification recommendations - HCM, DCM, CPVT: ↓PCS vs AR HCM; AR LQTS - AR DCM and carriers HCM: ↑PCS |
Hickey 2014 | LQTS, BrS, HCM, DCM; N = 58 | SF-36 | GENERIC | - Similar to other studies. - No LT negative consequences of undergoing gen test - ICD-shock→ ↓ HRQoL | - Individuals with positive genetic cardiac test scored significantly lower on the bodily pain dimension |
Capota 2020 | HCM; N = 91 | KCCQ | DISEASE-SPECIFIC | - Sign worse HRQoL in the HCM population as compared to matched patients from the general public. - Pulmonary hypertension significantly impact global HRQoL. | - Important gender differences in global KCCQ, poorer and higher symptom burden among women - Similar results as Hamang 2010 |
Brothers 2021 | AR ARVC; N = 73 | SF-36 | GENERIC | - No significant differences in the scores between baseline, 6 M, 1Y | - No results on physical health |
