Frailty and Cognitive Function in Middle-Aged and Older Adults With Congenital Heart Disease.

BACKGROUND: Life expectancy of patients with congenital heart disease (CHD) has increased rapidly, resulting in a growing and aging population. Recent studies have shown that older people with CHD have higher morbidity, health care use, and mortality. To maintain longevity and quality of life, understanding their evolving medical and psychosocial challenges is essential. OBJECTIVES: The authors describe the frailty and cognitive profile of middle-aged and older adults with CHD to identify predictor variables and to explore the relationship with hospital admissions and outpatient visits. METHODS: Using a cross-sectional, multicentric design, we included 814 patients aged ≥40 years from 11 countries. Frailty phenotype was determined using the Fried method. Cognitive function was assessed by the Montreal Cognitive Assessment. RESULTS: In this sample, 52.3% of patients were assessed as robust, 41.9% as prefrail, and 5.8% as frail; 38.8% had cognitive dysfunction. Multinomial regression showed that frailty was associated with older age, female sex, higher physiologic class, and comorbidities. Counterintuitively, patients with mild heart defects were more likely than those with complex lesions to be prefrail. Patients from middle-income countries displayed more prefrailty than those from higher-income countries. Logistic regression demonstrated that cognitive dysfunction was related to older age, comorbidities, and lower country-level income. CONCLUSIONS: Approximately one-half of included patients were (pre-)frail, and more than one-third experienced cognitive impairment. Frailty and cognitive dysfunction were identified in patients with mild CHD, indicating that these concerns extend beyond severe CHD. Assessing frailty and cognition routinely could offer valuable insights into this aging population.

Mitral valve replacement in a 3.9-kg infant with fungal endocarditis: A case report.

A three-month-old female infant with a structurally normal heart was diagnosed with fungal endocarditis of the mitral valve with cerebral embolism. After antifungal therapy and a valve-sparing operation with complete removal of the fungal vegetations, a relapse with complete destruction of the valve leaflets and severe mitral regurgitation with decompensated heart failure occurred three months later. A second operation with successful mitral valve replacement was performed. Non-compliance with the anticoagulant treatment with vitamin K antagonist led to thrombosis of the mitral valve prosthesis one year later, and the child died from acute pulmonary edema.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

BACKGROUND: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. METHODS/DESIGN: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DISCUSSION: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

Early extubation strategy after congenital heart surgery: 1-year single-centre experience.

INTRODUCTION: Our aim was to present the initial experience with a protocol-driven early extubation strategy and to identify risk factors associated with failed spontaneous breathing trials within 12 hours after surgery. METHODS: A single institutional retrospective study of children up to 18 years of age was conducted in post-operative cardiac surgical patients over a 1-year period. A daily spontaneous breathing trial protocol was used to assess patients' readiness for extubation. The study population (n = 129) was stratified into two age groups: infants (n = 84) and children (n = 45), and further stratified according to ventilation time: early extubation (ventilation time less than 12 h, n = 86) and deferred extubation (ventilation time more than 12 h, n = 43). Mann-Whitney U-test and binomial logistic regression were used for statistical analysis. RESULTS: Early extubated infants had shorter ICU (4 versus 6 days, p = 0.003) and hospital length of stays (16 versus 19 days, p = 0.006), lower re-intubation rates (1 versus 7 patients, p = 0.003), and lower mortality (0 versus. 4 patients, p = 0.01) than deferred extubated infants. There was no significant difference in the studied outcomes in the children group. Malnourished infants and longer cardiopulmonary bypass times were independently associated with failed spontaneous breathing trials within 12 hours after cardiac surgery. CONCLUSIONS: Early extubated infants after cardiac surgery had shorter ICU and hospital length of stay, without an increase in morbidity and mortality, compared to infants who deferred extubation. Nutritional status and longer cardiopulmonary bypass times were risk factors for failed spontaneous breathing trial.

Differential Molecular Stress Responses to Low Compared to High Doses of Ionizing Radiation in Normal Human Fibroblasts.

Understanding the mechanisms producing low dose ionizing radiation specific biological effects represents one of the major challenges of radiation biology. Although experimental evidence does suggest that various molecular stress response pathways may be involved in the production of low dose effects, much of the detail of those mechanisms remains elusive. We hypothesized that the regulation of various stress response pathways upon irradiation may differ from one another in complex dose-response manners, causing the specific and subtle low dose radiation effects. In the present study, the transcription level of 22 genes involved in stress responses were analyzed using RT-qPCR in normal human fibroblasts exposed to a range of gamma-doses from 1 to 200 cGy. Using the alkali comet assay, we also measured the level of DNA damages in dose-response and time-course experiments. We found non-linear dose responses for the repair of DNA damage after exposure to gamma-radiation. Alterations in gene expression were also not linear with dose for several of the genes examined and did not follow a single pattern. Rather, several patterns could be seen. Our results suggest a complex interplay of various stress response pathways triggered by low radiation doses, with various low dose thresholds for different genes.

[Dilation of atypical native coarctation of aorta with stent implantation]. / Poszerzenie nietypowej koarktacji aorty za pomoca implantacji stentu.

We describe a case of a 21-year-old women with atypical (kinking) native coarctation of aorta. She had arterial hypertension treated pharmacologically. To visualise stenotic segment, standard angiographic planes were not usefull, only right oblique projection showed precisely stenotic segment. Thereafter stent was successfully applied and trans aortic gradient decreased from 30 to 0 mmHg.

[Transcatheter closure of aorto-venous fistulae--is it always necessary?]. / Przezcewnikowe zamykanie przetok aortalno-zylnych--czy zawsze jest konieczne?

We present 2 cases (4 and 17-year-old children) with aorta-vena azygos fistulae. In the fist child spontaneous closure was confirmed by clinical symptoms and aortography, in the second (with significant left-right shunt) successful closure with 10 x 8 mm Amplatzer Duct Occluder was obtained. These case reports show that the strategy of treatment of aorto-venous fistulae must be individualised.