RESUMO
Chronic lung disease (CLD) is the second leading cause of pulmonary hypertension (PH) and is associated with significant morbidity and mortality. Although PH associated with CLD (PH-CLD) leads to impaired health-related quality of life (HRQOL), there are no validated tools to assess HRQOL in PH-CLD. The Pulmonary Arterial Hypertension-Symptoms and Impact Questionnaire (PAH-SYMPACT) is an HRQOL instrument aimed at assessing the symptoms and impact of PH on overall function and well-being. We performed a single-center prospective cohort study using PAH-SYMPACT scores to compare symptoms, exercise capacity and HRQOL in patients with PAH and PH-CLD. One hundred and twenty-five patients (99 patients with idiopathic/heritable PAH and 26 with PH-CLD) completed the PAH-SYMPACT questionnaire which consists of 22 questions that assess HRQOL across four domains: cardiopulmonary (CP) symptoms, cardiovascular (CV) symptoms, physical impact (PI), and cognitive/emotional (CE) impact. Higher scores indicate worse HRQOL. We compared patients with PAH and PH-CLD using a Wilcoxon rank sum or chi-squared test as appropriate. Multivariate linear regression analysis was used to assess the relationship between PH classification and SYMPACT scores. Compared to PAH, patients with PH-CLD were older, more likely to use oxygen and had worse functional class and exercise capacity. While there was no significant difference between the two groups in CP, CV, or CE domain scores, patients with PH-CLD had significantly worse PI scores by univariate (1.79 vs. 1.13, p < 0.001) and multivariate analysis (1.61 vs. 1.17, p = 0.02) and overall worse SYMPACT scores (1.19 vs. 0.91, p = 0.03). In conclusion, patients with PH-CLD have worse HRQOL as assessed by the PAH-SYMPACT questionnaire versus patients with PAH. Although PAH-SYMPACT has not been validated in PH-CLD, the results of this study can guide clinicians in understanding the symptoms and impact of PH-CLD relative to PAH.
RESUMO
BACKGROUND: The aim of this consensus process was to provide robust national guidelines to assist with decision making with regards to surgical management of patellofemoral instability. METHODS: A national group of surgeons, physiotherapists, basic scientists and a radiologist with expert experience in patellofemoral instability was convened. A formal consensus process was undertaken using validated methodology. They reviewed the existing literature, performed a group consensus meeting to develop recommendations and followed this with a wider consultation meeting with an open invitation for final ratification. The guidelines were reviewed and authorised by the BASK executive committee, followed by the British Orthopaedic Association prior to final publication. RESULTS: The review of the literature confirmed a significant absence of an evidence base to guide clinicians in the surgical management of patellofemoral instability. The consensus process outlined guidance relating to diagnosis and clinical assessment, appropriate imaging modalities, recommendations for surgical options and appropriate post operative physiotherapy. Following wide invitation to contribute to the final document, 102 people responded. Twenty six of those provided additional comments which were blinded, discussed and included or excluded following further discussion amongst the working group. CONCLUSION: Patellofemoral instability is a complex pathology with multiple surgical options available to those who decide surgery is indicated. In the absence of a strong evidence base, these guidelines aim to assist with that decision making process and will pave the way for more robust clinical trials in the future.
