Quality of life and health status of hospitalized adults with congenital heart disease in Vietnam: a cross-sectional study.

BACKGROUND: Little is known about the quality of life (QOL) and health status of adults with congenital heart disease (CHD) in developing countries. Therefore, this study aimed to describe the QOL and health status of hospitalized adults with CHD in Vietnam and investigate the association between QOL and their biological-social characteristics. METHODS: A cross-sectional study was conducted with 109 adults with CHD, hospitalized in the Vietnam National Heart Institute, between June and December 2019. Validated instruments to assess QOL and health status describing patient-reported outcomes were used, including the EuroQOL-5 Dimensions-5 Level, Satisfaction with Life Scale, and Hospital Anxiety and Depression Scale. RESULTS: The mean scores on the EuroQOL-descriptive system (EQ-DS) and EuroQOL visual analogue scale (EQ-VAS) were 0.792 (SD = 0.122, 95% confidence interval [CI] 0.769-0.815) and 66.3 (SD = 12.5, 95% CI 63.9-68.7), respectively. A total of 9.2% (n = 9) patients experienced life dissatisfaction. The prevalence of anxiety and depression were 18.7% (n = 20) and 11% (n = 12), respectively. Scores of QOL in patients aged > 30 years were lower than in those aged ≤ 30 years. Stratified multivariate logistic regression revealed that poor QOL related to being unemployed/unstable employment (OR 4.43, 95% CI 1.71-11.47, p = 0.002), life dissatisfaction associated with unmarried status (OR 4.63, 95% CI 1.2-17.86, p = 0.026), anxiety regarding unemployment/unstable employment (OR 3.88, 95% CI 1.27-11.84, p = 0.017) and complex CHD/PAH (OR 4.84, 95% CI 1.33-17.54, p = 0.016), and depression regarding unemployment/unstable employment (OR 4.63, 95% CI 1.22-17.59, p = 0.003). CONCLUSIONS: Reduced QOL and elevated psychological problems were common experiences among hospitalized adults with CHD in Vietnam. Biological-social characteristics such as unmarried status, unemployment/unstable employment, and complex CHD/PAH related to poor QOL, life dissatisfaction, anxiety, and depression.

Genetics, Screening, and Treatment of Familial Hypercholesterolemia: Experience Gained From the Implementation of the Vietnam Familial Hypercholesterolemia Registry.

Familial hypercholesterolemia (FH) is underdiagnosed and undertreated in a majority of the low- and middle-income countries. FH registries could prove useful in bridging the knowledge gaps, supporting genetic and clinical research, and improving health-care planning and patient care. Here, we report the first usage experience of the Vietnam FH (VINAFH) Registry. The VINAFH Registry was established in 2016 as a long-term database for prospective cohorts. FH patients were detected based on the opportunistic and cascade screening. Diagnosis of FH was assessed using the Dutch Lipid Clinic Network criteria, plasma levels of low-density lipoprotein (LDL) cholesterol, and genetic testing. To date, a total of 130 patients with FH have been registered, with 48 index cases and 82 relatives. Of the 130 patients, 8 were homozygous FH patients and 38 were children. Of FH individuals, 46.7% was confirmed by genetic testing: 61 patients (96.8%) carried the LDLR mutation (c.681C > G, c.1427C > G, c.1187-?_2140 ± ?del, c.2529_2530delinsA), and two patients (3.2%) carried the PCSK9 (protein convertase subtilisin/kexin type 9) mutation (c.42_43insTG). The c.2529_2530delinsA mutation detected in this study is novel and reported only in the Vietnamese population. However, only 53.8% of FH patients were followed up post diagnosis, and only 15.3% of these were approved for lipid-lowering therapy and specialized care. Notably, factors such as knowledge about FH in patients and/or guardians of FH children and support of primary care physicians affected patient participation with respect to treatment strategies and follow-up. Genetic identification, screening, and treatment of FH were feasible in Vietnam. The VINAFH Registry significantly contributed to the formation of the government agencies legislative acts that established the importance of FH as a socially and medically important disease requiring appropriate management strategies. Other low- and middle-income countries could, thus, use the VINAFH Registry model as a reference to establish programs for FH management according to the current status.

Homozygous familial hypercholesterolaemia in Vietnam: Case series, genetics and cascade testing of families.

BACKGROUND AND AIMS: Familial hypercholesterolaemia has not been previously described in the Vietnamese population. We aimed to describe the features of patients with homozygous familial hypercholesterolaemia (hoFH) in Vietnam and the outcomes of screening family members using genetic and cholesterol testing. METHODS: Mutation testing by massively parallel sequencing for genes causative of FH was undertaken in five index cases presenting to a single cardiac center with a presumptive diagnosis of hoFH. Cascade testing of all available family members was subsequently undertaken. The number of new cases of FH detected and commenced on lipid-lowering treatment was evaluated. RESULTS: All five index cases had true homozygous mutations in the LDL receptor gene (LDLR). Cascade screening was undertaken in four families. 107 relatives were screened and FH was identified in 56 relatives (52%), including 3 new cases of hoFH. Only 5 FH relatives (9%) were subsequently treated owing to the adverse perceptions and comparative high cost of drug treatment, and lack of awareness of FH among patients and local doctors. CONCLUSIONS: HoFH due to LDLR mutations is a severe disorder in Vietnam that needs early detection and treatment with LDL-cholesterol lowering drugs. Cascade testing of families allows effective detection of new cases of FH that may also benefit from early treatment. However, convincing patients to commence statin treatment is a challenge. Extended education and awareness programs and treatment subsidies are imperative to improve the care of patients and families suffering from FH in Vietnam.