Comparison of obstructive sleep apnoea prevalence and severity across WHO pulmonary hypertension groups.

INTRODUCTION: Pulmonary hypertension is classified into five groups in the WHO classification system. Patients with pulmonary hypertension often have comorbid obstructive sleep apnoea (OSA), yet the prevalence and severity of OSA in each of the WHO pulmonary hypertension groups have not been well established. METHODS: To compare the prevalence and severity of OSA between WHO pulmonary hypertension groups, we performed a retrospective cohort study, including patients who had polysomnography or a home sleep study and confirmed pulmonary hypertension on right heart catheterisation. The primary outcomes of OSA prevalence and severity were measured by median apnoea hypopnea index (AHI) or respiratory event index (REI) and were compared by WHO pulmonary hypertension group. Multivariable negative binomial regression was used to evaluate the association between the outcome of OSA severity by AHI or REI and WHO group. RESULTS: Among the cohort of 132 patients, OSA was common in all WHO pulmonary hypertension groups but was most common and most severe in WHO group II pulmonary hypertension. Median AHI or REI in WHO group II was 12.0 events/hour compared with 2.8 in group I, 3.7 in group III, 10.0 in group IV and 6.4 in group V. Multivariable negative binomial regression showed about a twofold increase in AHI or REI in WHO group II compared with WHO group I pulmonary hypertension. DISCUSSION: Our findings demonstrate that OSA deserves greater consideration as a treatable comorbidity that may affect pulmonary haemodynamics and quality of life in patients with pulmonary hypertension across all WHO groups.

The clinical impact of the Covid-19 pandemic first wave on patients with cystic fibrosis in New York.

BACKGROUND: People with cystic fibrosis (pwCF) may be at risk of complications from COVID-19 but the impact of COVID-19 on pwCF remains unknown. METHODS: We conducted a multicenter retrospective cohort study to assess the impact of the COVID-19 pandemic first wave on pwCF in the New York metropolitan area (NY) from March 1, 2020 to August 31, 2020. Objectives were to determine (1) the prevalence of COVID-19 by PCR and IgG antibody testing, (2) the clinical characteristics of COVID-19, (3) delay in routine outpatient care, and (4) the effect on anxiety and depression in pwCF. RESULTS: There were 26 COVID-19 cases diagnosed by PCR or antibody testing among the study cohort of 810 pwCF. The prevalence of COVID-19 by PCR (1.6%) and IgG antibody (12.2%) testing was low. 58% of cases were asymptomatic and 82% were managed at home. 8% were hospitalized and 1 person died. 89% of pwCF experienced delay in care. The prevalence of anxiety increased from 43% baseline to 58% during the pandemic (P<0.01). In post-hoc analysis, the proportion of patients with diabetes (38% versus 16%, P<0.01) and pancreatic insufficiency (96% versus 66%, P<0.01) were higher while CFTR modulator use was lower (46% versus 65%, P = 0.05) in pwCF who tested positive for COVID-19. CONCLUSIONS: The prevalence of COVID-19 among pwCF in NY during the pandemic first wave was low and most cases were managed at home. CFTR modulators may be protective. PwCF experienced delay in routine care and increased anxiety.

Sleep architecture in patients with interstitial lung disease with and without pulmonary hypertension.

PURPOSE: Sleep disturbance is common in patients with advanced interstitial lung disease (ILD) often complicated by pulmonary hypertension (PH) and may contribute to poor quality of life. The etiology of sleep disturbance and the relationship between PH and sleep architecture in patients with ILD remains unknown. METHODS: We performed a retrospective cohort study comparing sleep architecture on polysomnography in patients with ILD with and without PH, defined as mean pulmonary artery pressure on right heart catheterization ≥ 20 mmHg. We tested the hypothesis that patients with ILD and PH would have increased wake time after sleep onset (WASO) compared to patients with ILD without PH using univariate analysis and multivariable linear regression. RESULTS: In our cohort of patients with ILD who underwent polysomnography (N = 49), patients with PH had lower total diffusion capacity for carbon monoxide (DLCO) (9.28 vs. 12.87 ml/min/mmHg, P = 0.04) and percent DLCO (39% vs. 53%, P = 0.03). On polysomnography, patients with PH had increased percentage of total sleep time with saturation < 90% (T90) (17% vs. 6%, P = 0.03), decreased N2 sleep (181 vs. 233 min, P = 0.03), decreased %N2 sleep (59% vs. 66%, P = 0.04), increased %N1 sleep (22% vs. 14%, P = 0.02), decreased sleep efficiency (62% vs. 72%, P = 0.03), and increased WASO (133 vs. 84 min, P = 0.01). In multivariable analysis, PH was associated with a 43-min increase in WASO (95% CI 6.2-80.2, P = 0.02). CONCLUSION: Patients with ILD and PH have decreased total and %N2 sleep, increased %N1 sleep, decreased sleep efficiency, and increased WASO, likely indicating increased sleep fragmentation.

Hemoglobin Target and Transfusion Modality for Adult Patients With Sickle Cell Disease Acute Chest Syndrome.

BACKGROUND: Despite the importance of transfusion in treating sickle cell disease acute chest syndrome, the target hemoglobin and optimal modality for transfusion remain unknown. OBJECTIVES: To compare hospital length of stay (LOS) in intensive care unit (ICU) patients with acute chest syndrome transfused to hemoglobin ≥ 8 g/dL versus patients transfused to hemoglobin < 8 g/dL; and to compare hospital LOS in acute chest syndrome patients treated with and without exchange transfusion. METHODS: We performed a retrospective cohort study of all acute chest syndrome patients treated in the medical ICU at 2 tertiary care hospitals between January 2011 and August 2016 (n = 82). We compared median hospital LOS in patients transfused to hemoglobin ≥ 8 g/dL by the time of ICU transfer to the medical floor versus patients transfused to hemoglobin < 8 g/dL as well as patients who received exchange transfusion versus no exchange transfusion using Wilcoxon rank-sum tests. We modeled the association between hospital LOS and hemoglobin at ICU transfer to the medical floor using multivariable log-linear regression. RESULTS: Median hospital LOS was about half as long for patients transfused to hemoglobin ≥ 8 g/dL versus hemoglobin < 8 g/dL (8.0 versus 16.5 days, P = 0.008). There was no difference in LOS for patients treated with and without exchange transfusion. On average, a 1 g/dL increase in hemoglobin was associated with a 19.5% decrease (95% CI 10.8-28.2%) in LOS, controlling for possible confounding factors. CONCLUSIONS: Transfusion to a hemoglobin target ≥ 8 g/dL is associated with decreased hospital LOS in patients with acute chest syndrome. There was no difference in LOS between patients who received exchange transfusion and those who did not.

Enlarged pulmonary artery on computed tomography and respiratory failure in sickle cell disease acute chest syndrome.

Predicting the severity of acute chest syndrome is an important research priority in sickle cell disease. In this retrospective study of patients with acute chest syndrome, an enlarged pulmonary artery on computed tomography was associated with severe respiratory failure defined by the need for either noninvasive or mechanical ventilation.

Assessment of patient perception of glaucomatous visual field loss and its association with disease severity using Amsler grid.

PURPOSE: To investigate patients' perception of glaucomatous VF loss and its association with glaucoma severity using the Amsler grid test. METHODS: In this prospective cross-sectional study, glaucoma patients with abnormal 10-2 Humphrey Swedish Interactive Threshold Algorithm-standard VF tests were enrolled consecutively. All patients underwent a black-on-white Amsler grid test for each eligible eye. They were asked to outline any perceived scotomas (areas with abnormal grid lines) on the grid and then describe verbally their perception of the scotomas. Examiners asked patients to clarify their descriptions. All descriptions used by patients were recorded in their own words, which were then sorted into descriptor categories according to similar themes. The number of descriptor categories was counted for each eye. 10-2 VF mean deviation (MD) was compared among eyes that reported different number of descriptor categories. The mean 10-2 VF MD values were compared among different descriptor categories. RESULTS: Fifty glaucoma patients (88 eyes) were included. Patients used a total of 44 different descriptors for their scotomas. Patients' descriptors were classified into categories that incorporated similar themes, resulting in 4 overarching descriptor categories: Missing/White, Blurry/Gray, Black, and Not Aware. Fifty-two eyes reported one descriptor category and 19 eyes reported two descriptor categories (mean number of descriptor categories = 1.27±0.45). Eyes that reported two descriptor categories had worse VF MD than those that reported one (-17.86±10.31 dB vs. -12.08±7.53 dB; p = 0.012). When eyes were organized according to its combination of descriptor categories, each eye naturally sorted into one of the following 5 groups, in frequency order: Missing/White (27 eyes; 31%), Blurry/Gray (21 eyes; 24%), combined Missing/White and Blurry/Gray (19 eyes; 21%), Not Aware (17 eyes; 19%), and Black (4 eyes; 5%). The mean 10-2 VF MD severity order was Black (-21.18±10.59 dB), combined Missing/White and Blurry/Gray (-17.86±10.31 dB), Missing/White (-11.92±6.76 dB), Blurry/Gray (-10.55±7.03 dB), and Not Aware (-3.91±4.05 dB) (p<0.001). CONCLUSION: Paracentral vision loss in glaucoma is perceived by patients. As the perception of scotomas and the variety of terms to describe scotomas are related to glaucoma severity, clinicians should pay attention to patients' subjective descriptions of their glaucomatous VF loss. The historical notion that glaucoma patients lose their peripheral vision first and eventually look through a black tunnel needs to be updated to reflect the true perception of glaucoma.

Efficacy of the Amsler Grid Test in Evaluating Glaucomatous Central Visual Field Defects.

PURPOSE: To investigate the efficacy of the Amsler grid test in detecting central visual field (VF) defects in glaucoma. DESIGN: Prospective, cross-sectional study. PARTICIPANTS: Patients with glaucoma with reliable Humphrey 10-2 Swedish Interactive Threshold Algorithm standard VF on the date of enrollment or within the previous 3 months. METHODS: Amsler grid tests were performed for each eye and were considered "abnormal" if there was any perceived scotoma with missing or blurry grid lines within the central 10 degrees ("Amsler grid scotoma"). An abnormal 10-2 VF was defined as ≥3 adjacent points at P < 0.01 with at least 1 point at P < 0.005 in the same hemifield on the pattern deviation plot. Sensitivity, specificity, and positive and negative predictive values of the Amsler grid scotoma area were calculated with the 10-2 VF as the clinical reference standard. Among eyes with an abnormal 10-2 VF, regression analyses were performed between the Amsler grid scotoma area and the 10-2 VF parameters (mean deviation [MD], scotoma extent [number of test points with P < 0.01 in total deviation map] and scotoma mean depth [mean sensitivity of test points with P < 0.01 in total deviation map]). MAIN OUTCOME MEASURES: Sensitivity, specificity, and positive and negative predictive values of the Amsler grid scotoma area. RESULTS: A total of 106 eyes (53 patients) were included (mean ± standard deviation age, 24-2 MD and 10-2 MD = 66±12 years, -9.61±8.64 decibels [dB] and -9.75±9.00 dB, respectively). Sensitivity, specificity, and positive and negative predictive values of the Amsler grid test were 68%, 92%, 97%, and 46%, respectively. Sensitivity was 40% in eyes with 10-2 MD better than -6 dB, 58% in eyes with 10-2 MD between -12 and -6 dB, and 92% in eyes with 10-2 MD worse than -12 dB. The area under the receiver operating characteristic curve of the Amsler grid scotoma area was 0.810 (95% confidence interval, 0.723-0.880, P < 0.001). The Amsler grid scotoma area had the strongest relationship with 10-2 MD (quadratic R(2)=0.681), followed by 10-2 scotoma extent (quadratic R(2)=0.611) and 10-2 scotoma mean depth (quadratic R(2)=0.299) (all P < 0.001). CONCLUSIONS: The Amsler grid can be used to screen for moderate to severe central vision loss from glaucoma.

Defining 10-2 visual field progression criteria: exploratory and confirmatory factor analysis using pointwise linear regression.

PURPOSE: To test different visual field progression criteria using trend analysis in a glaucoma population followed with long sequences of 10-2 tests as a first attempt to understand and document rates of progression in the central field. DESIGN: Retrospective cohort study. PARTICIPANTS: We included 146 eyes of 146 patients with established glaucoma. METHODS: Pointwise linear regression analysis using the methods of ordinary least squares was performed on the 68 test locations of the 10-2 visual field sequences. Threshold sensitivities at each test location were plotted as the dependent variable against follow-up time as the independent variable. Statistically significant progression or improvement of a visual field test point was defined if its regression slope measured ≤-1.0 dB/year or ≥+1.0 dB/year, respectively, at P<0.01. We explored sets of criteria to define visual field progression, generating a hypothetical sensitivity (progression), specificity (improvement), and progression-to-improvement ratio (PIR) for each criterion. The criterion with the highest PIR was deemed the one with best performance. Latent class analysis (LCA) was used to determine visual field sectors with highest inter-correlation. MAIN OUTCOME MEASURES: The performance of different visual field progression criteria to detect fast rates of mean deviation (MD) change. RESULTS: Median baseline 10-2 MD value was -12.0 dB (interquartile range [IQR], -6.7 to -17.8 dB), and the median rate of 10-2 MD change over time was -0.38 dB/year (IQR, -0.07 to -0.77 dB/year). The highest PIR was obtained with the progression criterion requiring at least 3 test points located in the same LCA-derived 10-2 visual field sector progressing faster than -1.0 dB/year at P<0.01. This criterion was further validated for content and convergence. CONCLUSIONS: This is the first study to investigate progression criteria for 10-2 visual fields using rates of change and to test their performance and validity. These findings may be useful to improve the monitoring of patients with glaucoma at different levels of functional loss and to develop new perimetric algorithms that scrutinize specific visual field locations for a more accurate detection of progression.

Factors associated with focal lamina cribrosa defects in glaucoma.

PURPOSE: To assess factors associated with focal lamina cribrosa (LC) defects in glaucoma. METHODS: Serial enhanced depth imaging (EDI) optical coherence tomography (OCT) B-scans of the optic nerve head were obtained from glaucoma patients. EDI OCT scans were reviewed for focal LC defects (laminar holes or disinsertions). Evaluated clinical factors included age, central corneal thickness, visual field (VF) mean deviation (MD), exfoliation syndrome, normal-tension glaucoma (NTG), disc hemorrhage, and intraocular pressure (IOP) during past follow-up. RESULTS: One hundred forty-eight glaucomatous eyes (148 patients; mean age, 68 ± 12 years; mean VF MD, -11.63 ± 6.96 dB) were included. Sixty-seven (45%) eyes had focal LC defects and 81 (55%) did not. Eyes with focal LC defects had a higher prevalence of both disc hemorrhage (25% vs. 6%) and NTG (33% vs. 9%) and worse VF MD (-14.12 vs. -9.58 dB) than those without focal LC defects (P = 0.002, P < 0.001, and P < 0.001, respectively). In the multivariate logistic regression analysis, higher frequency of disc hemorrhage detection (odds ratio [OR], 3.63; P = 0.032), a diagnosis of NTG (OR, 4.23; P = 0.005), and worse VF MD (OR, 1.11; P < 0.001) were significant factors associated with the presence of focal LC defects. Disc hemorrhage developed in the same half of the disc as the largest or the second largest focal LC defect in 15 of 17 eyes (88.2%). CONCLUSIONS: Disc hemorrhage, a diagnosis of NTG, and more advanced glaucoma status are associated with focal LC defects. Future studies are needed to elucidate the cause-and-effect relationships between focal LC defects and these factors.

Parafoveal scotoma progression in glaucoma: humphrey 10-2 versus 24-2 visual field analysis.

OBJECTIVE: To compare the performance of 10-2 versus 24-2 visual fields (VFs) in detecting progression of initial parafoveal scotoma (IPFS) in glaucomatous eyes. DESIGN: Retrospective, observational study. PARTICIPANTS: Glaucoma patients with the following criteria: (1) an IPFS (≥ 3 adjacent points with P<0.05 within the central 10° degrees of fixation, 1 point or more with P<0.01 lying at the innermost paracentral points, and no scotoma outside the central 10°) in either hemifield based on 2 reliable Humphrey 24-2 Swedish interactive threshold algorithm standard VFs, and (2) 5 or more 10-2 and 24-2 VFs. METHODS: Based on threshold map sensitivities, VF progression, defined as having 1 or more significantly progressing point(s) with a slope of sensitivity of less than -1.0 dB/year at P<0.01, was evaluated using pointwise linear regression. MAIN OUTCOME MEASURES: The number of progressing eyes in 10-2 and 24-2 VF analyses. RESULTS: Fifty eyes (50 patients) were included (mean age ± standard deviation, 62 ± 9 years). Mean follow-up period (5.7 vs. 5.6 years) and number of VFs (7.6 vs. 7.8) were similar between 10-2 and 24-2 analyses (all P>0.3). Significantly more progressing eyes were detected in 10-2 than in 24-2 analyses (24 vs. 11 eyes; P = 0.007). This difference became greater within the central 10° (24 vs. 4 eyes; P<0.001). Four of the 11 progressing eyes in 24-2 analysis were missed in 10-2 analysis, whereas 17 of the 24 progressing eyes in 10-2 analysis were missed in 24-2 analysis. The 4 progressing eyes missed in 10-2 analysis had progressing point(s) only outside the central 10° in 24-2 analysis. The other 3 eyes with progressing point(s) only outside the central 10° in 24-2 analysis were detected as progressing in 10-2 analysis. Similar results were obtained when more stringent criteria (at least 2 significantly progressing points within the same hemifield) were used for VF progression. CONCLUSIONS: The 10-2 VF detects more progressing eyes than the 24-2 VF in glaucoma patients with IPFS, suggesting that closer surveillance of the central VF using testing algorithms with closely spaced grids is warranted in eyes with parafoveal scotomas. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Progression pattern of initial parafoveal scotomas in glaucoma.

OBJECTIVE: To characterize the progression pattern of initial parafoveal scotomas (IPFSs) using cross-sectional and longitudinal 10-2 visual field (VF) data. DESIGN: Retrospective, observational study. PARTICIPANTS: Glaucoma patients with an IPFS in either hemifield based on 2 reliable 24-2 Swedish interactive threshold algorithm standard VFs (≥3 adjacent points with P<0.05 within the central 10° of fixation, 1 point or more with P<0.01 lying at the innermost paracentral points, and no scotoma outside the central 10°) and at least 2 10-2 VFs (first and last VFs 1 year or more apart). METHODS: To simulate a cohort with an extended follow-up, eyes with an IPFS were divided into subgroups based on the severity of glaucoma using their 10-2 VF pattern standard deviation (PSD). Cross-sectional data were used to create an average pattern deviation map that was generated by averaging pattern deviation map values of 10-2 VF point-by-point within each subgroup. Longitudinal data (eyes with 5 or more 10-2 VFs) was used to perform pointwise linear regression analysis of pattern deviation values. Patterns of IPFS progression were identified from these cross-sectional and longitudinal assessments. MAIN OUTCOME MEASURES: Average pattern deviation maps (cross-sectional) and maps of progression rates (longitudinal) in different disease severity subgroups. RESULTS: Eighty eyes (80 patients) and 40 eyes (40 patients) with an IPFS were included for cross-sectional and longitudinal analyses, respectively. The mean age ± standard deviation, 24-2 VF mean deviation, and 24-2 VF PSD for all eyes were 63±10 years, -3.27±2.18 dB, and 5.46±2.40 dB, respectively. Based on maps generated in both cross-sectional and longitudinal analyses, IPFS in the superior hemifield had an arcuate pattern initially that later deepened approximately 3° to 5° above fixation. The scotoma then elongated toward the physiologic blind spot and spread toward the nasal periphery, sparing the area corresponding to the papillomacular bundle. The IPFS in the inferior hemifield had a similar pattern, but was slightly farther from fixation. CONCLUSIONS: Superior and inferior IPFS have a similar characteristic pattern of progression, although the latter tend to be farther from fixation. Understanding these patterns should help in the management of such patients and in improving VF testing algorithms.