Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension

Abstract Background The effects of pulmonary arterial hypertension on cardiovascular and physical function are well documented. Limited information exists regarding the effects of pulmonary arterial hypertension on cognitive function despite patient reports of problems with memory and attention. Our primary purpose was to determine if a prospectively identified cohort of pulmonary arterial hypertension patients had cognitive sequelae. Our secondary purpose was to determine the relationships between cognitive sequelae and neuropsychological test scores with depression, anxiety, and quality of life. Methods Forty-six adults with pulmonary arterial hypertension underwent assessment of cognitive function, depression, anxiety, and quality of life using standardized neuropsychological tests and questionnaires. The patients' scores were compared to normal population data. Medical, affective, neuropsychological, and quality of life data for patients with and without cognitive sequelae were compared using analysis of variance, Chi-square, or Fisher exact tests for categorical data. Correlations assessed relationships between neuropsychological test scores, depression, anxiety, quality of life, and medical data. Results Cognitive sequelae occurred in 58% (27/46) of the pulmonary arterial hypertension patients. Patients with cognitive sequelae had worse verbal learning, delayed verbal memory, executive function, and fine motor scores compared to patients without cognitive sequelae. Twenty-six percent of patients had moderate to severe depression and 19% had moderate to severe anxiety. Depression, anxiety and quality of life were not different for patients with or without cognitive sequelae. Our patients had decreased quality of life, which was associated with worse working memory. Conclusion Patients with pulmonary arterial hypertension have cognitive impairments, depression, anxiety, and decreased quality of life. Depression, anxiety, and quality of life were similar for patients with cognitive sequelae compared to those without cognitive sequelae. Decreased quality of life was associated with worse verbal and working memory. Clinicians should be aware of adverse brain related outcomes in PAH patients. Attention to proximal determinants and possible interventions to prevent or reduce cognitive and emotional morbidity and decreased quality of life are warranted and should be an emphasis in outcomes research.</p

Genetic association of the serotonin transporter in pulmonary arterial hypertension

RATIONALE: The bone morphogenetic receptor type II gene is the major genetic determinant for the inherited form of pulmonary arterial hypertension. However, deleterious mutations of this gene are not observed in the majority of subjects who develop the condition spontaneously and familial disease displays age- and sex-dependent penetrance, indicating the requirement for additional environmental and/or genetic modifiers for disease development. METHODS: We investigated polymorphic variation of the serotonin transporter gene, a biological candidate for predisposition to this vascular disorder. RESULTS: No significant evidence of association between alleles of the serotonin transporter gene and pulmonary hypertension was detected, nor did we observe a relationship with age of onset in familial and idiopathic disease. CONCLUSIONS: Variation of the serotonin transporter gene appears unlikely to confer significant susceptibility to pulmonary arterial hypertension. This study emphasizes the need for adequately powered cohorts for association analyses to identify not only genetic determinants of disease susceptibility but also inherited modifiers for disease development

Serious Bacterial Infections in Young Febrile Infants With Positive Urinalysis Results.

UNLABELLED: It is unknown whether febrile infants 29 to 60 days old with positive urinalysis results require routine lumbar punctures for evaluation of bacterial meningitis. OBJECTIVE: To determine the prevalence of bacteremia and/or bacterial meningitis in febrile infants ≤60 days of age with positive urinalysis (UA) results. METHODS: Secondary analysis of a prospective observational study of noncritical febrile infants ≤60 days between 2011 and 2019 conducted in the Pediatric Emergency Care Applied Research Network emergency departments. Participants had temperatures ≥38°C and were evaluated with blood cultures and had UAs available for analysis. We report the prevalence of bacteremia and bacterial meningitis in those with and without positive UA results. RESULTS: Among 7180 infants, 1090 (15.2%) had positive UA results. The risk of bacteremia was higher in those with positive versus negative UA results (63/1090 [5.8%] vs 69/6090 [1.1%], difference 4.7% [3.3% to 6.1%]). There was no difference in the prevalence of bacterial meningitis in infants ≤28 days of age with positive versus negative UA results (∼1% in both groups). However, among 697 infants aged 29 to 60 days with positive UA results, there were no cases of bacterial meningitis in comparison to 9 of 4153 with negative UA results (0.2%, difference -0.2% [-0.4% to -0.1%]). In addition, there were no cases of bacteremia and/or bacterial meningitis in the 148 infants ≤60 days of age with positive UA results who had the Pediatric Emergency Care Applied Research Network low-risk blood thresholds of absolute neutrophil count &lt;4 × 103 cells/mm3 and procalcitonin &lt;0.5 ng/mL. CONCLUSIONS: Among noncritical febrile infants ≤60 days of age with positive UA results, there were no cases of bacterial meningitis in those aged 29 to 60 days and no cases of bacteremia and/or bacterial meningitis in any low-risk infants based on low-risk blood thresholds in both months of life. These findings can guide lumbar puncture use and other clinical decision making