Structured Evaluation and Management of Patients with COPD in an Accredited Program.

Background: Chronic obstructive pulmonary disease (COPD) is an ambulatory care-sensitive condition. Methods: We compared the impact of care received by patients with COPD at Joint Commission-accredited, disease-specific clinics and primary care clinics at an academic health care systemfrom April 2014 to March 2018. Patients with COPD ≥ 40 years old with ≥ 2 outpatient visits 30 days apart were identified. Baseline demographics, disease-specific performance measures, and health care utilization were compared between groups. Propensity matching was conducted and time to the first emergency department (ED) visit and hospitalization was performed using Cox regression analysis. Results: Of 4646 unique patients with COPD, 1114 were treated at disease-specific clinics and 3532 at primary care clinics. The entire group was predominantly female (58.8 %), non-Hispanic White (74.2 %) with a mean age of 65.4 ± 11.4 years consisting of current (47.6 %) or former smokers (38.4 %). In the disease-specific group, performance measures were performed more frequently, and lower rates of ED visits (hazard ratio [HR]=0.31, 95% confidence interval [CI] 0.18-0.54) and hospitalizations (HR 0.41, 95% CI 0.21-0.79) noted in comparison to the primary care group. Conclusions: In this observational study, the implementation of achronic disease management program through accredited disease-specific clinics for patients with COPD was associated with reduced all-cause ED visits and hospitalizations.

Corticosteroids and Outcomes in Solid Organ Transplant Recipients Infected With Severe Acute Respiratory Syndrome Coronavirus 2.

Objective: To examine outcomes in organ transplant and nontransplant patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection during the initial 22 months of the pandemic. Patients and Methods: We used Optum electronic health records to compare outcomes between an adult transplant group and a propensity-matched nontransplant group that tested positive for SARS-CoV-2 from February 1, 2020, to December 15, 2021. Baseline characteristics, hospitalization, intensive care unit admission, mechanical ventilation, renal replacement therapy, inpatient, and 90-day mortality were compared between the transplant and nontransplant groups and among specific transplant recipients. Cox proportional analysis was used to examine hospitalization and mortality by organ transplant, medical therapy, sex, and the period of the pandemic. Results: We identified 876,959 patients with SARS-CoV-2 infection, of whom 3548 were organ transplant recipients. The transplant recipients had a higher risk of hospitalization (30.6% vs 25%, respectively; P<.001), greater use of mechanical ventilation (7.8% vs 5.6%, respectively; P<.001), and increased inpatient mortality (6.7% vs 4.7%, respectively; P<.001) compared with the nontransplant patients. The initiation of mechanical ventilation was significantly more frequent in the transplant group. After adjustment for baseline characteristics and comorbidities, the transplant group had a higher risk of hospitalization (odds ratio, 1.38; 95% confidence interval, 1.19-1.59), without a difference in mortality. In the transplant group, lung transplant recipients had the highest inpatient mortality (11.6%). Conclusion: Among patients with SARS-CoV-2 infection, the transplant recipients were at a higher risk of hospitalization and inpatient mortality; however, mortality was mainly driven by advanced age and comorbidities rather than by transplant status or immunosuppressive medications. Lung transplant recipients had the greatest inpatient and 90-day mortality.

Acute Respiratory Distress Syndrome in Pregnancy: Updates in Principles and Practice.

Acute respiratory failure occurs in 0.05% to 0.3% of pregnancies and is precipitated by pulmonary and nonpulmonary insults. Acute respiratory distress syndrome (ARDS) is the rapid onset of hypoxemic respiratory failure associated with bilateral pulmonary opacities on chest imaging attributed to noncardiogenic pulmonary edema. The pathophysiological features of ARDS include hypoxemia, diminished lung volumes, and decreased lung compliance. While there is a paucity of data concerning ARDS in the pregnant individual, management principles do not vary significantly between pregnant and nonpregnant patients. The following review will discuss the diagnosis and management of the pregnant patient with ARDS.

Immunohistochemical profile of the pulmonary vasculature in subjects with cirrhosis and histopathologic evidence of pulmonary vascular disease: An autopsy study.

Portopulmonary hypertension (POPH) is a complication of cirrhosis that results in right ventricular failure and death. The objective of this autopsy investigation was to compare pulmonary arterial receptors in subjects with cirrhosis and histopathologic evidence of pulmonary vascular disease (PVD) and control group subjects with cirrhosis lacking evidence of PVD. Autopsy records of 824 subjects with cirrhosis were reviewed to identify pulmonary arterial vasculopathy. Lung sections from paraffin embedded blocks were immunostained for endothelin A (ET-A), endothelin B (ET- B), estrogen α (ER-α), estrogen ß (ER-ß), and vascular endothelial growth factor (VEGF). Subjects with cirrhosis and histopathologic evidence of PVD included 27 individuals with intimal hyperplasia (93%), medial hypertrophy (96%), and plexiform lesions (78%). Immunohistochemical staining for ET-A revealed positive reactivity in 40% of the group with cirrhosis and histopathologic evidence of PVD and 13% of the control group (NS). ET-B reactivity in the pulmonary endothelium and smooth muscle was identified in all subjects with cirrhosis and histopathologic evidence of PVD and control group. VEGF reactivity was identified in the endothelium in all subjects with cirrhosis and histopathologic evidence of PVD compared with 33% of the control group (p = 0.0002). ER-ß reactivity was observed in four subjects (26.6%) with cirrhosis and histopathologic evidence of PVD while none in the control group (NS). Cirrhosis and histopathologic evidence of PVD was found in 3.3% of autopsies with the pulmonary vasculature immunohistochemical profile demonstrating endothelial and smooth muscle reactivity for endothelin, VEGF and ER-ß.

Liver transplant recipient with respiratory failure due to pulmonary fibrosis related to telomere disease requiring lung transplantation.

Short telomere syndrome (STS) is characterized as multiorgan dysfunction presenting with unexplained cytopenias, cryptogenic cirrhosis and pulmonary fibrosis. We present a liver transplant recipient that gradually developed hypoxic respiratory failure attributed to idiopathic pulmonary fibrosis associated telomere disease that culminated in a successful single lung transplantation.

HRCT Diagnosis of Pleuroparenchymal fibroelastosis: Report of two cases.

Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia that is often underdiagnosed on computed tomography scans. The disease process involves a combination of fibrosis involving the visceral pleura and fibroelastic changes within the subpleural lung parenchyma. Although definitive diagnosis is based on pathological evaluation, this is often not feasible and pattern recognition on CT as "definite PPFE" or "consistent with PPFE" is important given that sub group of patients will undergo rapid progression with clinical deterioration.

Non-CF bronchiectasis: Orphan disease no longer.

Bronchiectasis is a complex, chronic respiratory condition, characterized by frequent cough and exertional dyspnea due to a range of conditions that include inherited mucociliary defects, inhalational airway injury, immunodeficiency states and prior respiratory infections. For years, bronchiectasis was classified as either being caused by cystic fibrosis or non-cystic fibrosis. Non-cystic fibrosis bronchiectasis, once considered an orphan disease, is more prevalent worldwide in part due to greater availability of chest computed tomographic imaging. Identification of the cause of non-cystic fibrosis bronchiectasis with the use of chest imaging, laboratory testing, and microbiologic assessment of airway secretions can lead to initiation of specific therapies aimed at slowing disease progression. Nonpharmacologic therapies such as airway clearance techniques and pulmonary rehabilitation improve patient symptoms. Inhaled corticosteroids should not be routinely prescribed unless concomitant asthma or COPD is present. Inhaled antibiotics prescribed to individuals with >3 exacerbations per year are well tolerated, reduce airway bacteria load and may reduce the frequency of exacerbations. Likewise, chronic macrolide therapy reduces the frequency of exacerbations. Medical therapies for cystic fibrosis bronchiectasis may not be effective in treatment of non-cystic fibrosis bronchiectasis.

Discordant bilateral bronchoalveolar lavage findings in a patient with acute eosinophilic pneumonia associated with counterfeit tetrahydrocannabinol oil vaping.

Vaping has emerged as a popular alternative form of inhalation of nicotine and marihuana derivates (including Tetrahydrocannabinol, THC) in part due to the avoidance of combustion byproducts. Unfortunately, THC oil (especially that produced by unregulated individuals) may contain dilutants such as propylene glycol, vitamin E, and flavoring ingredients that can lead to adverse respiratory effects. Acute eosinophilic pneumonia (AEP) has been described in association with e-cigarette and vaping associated lung injury (EVALI) but the majority of bronchoalveolar lavage (BAL) samples reported in the literature do not show eosinophils as the predominant cell lineage. Only two other cases of AEP have been published, and here we present the first case reported in the literature of a patient with EVALI with AEP pattern associated with counterfeit tetrahydrocannabinol (THC) oil vaping and discordant bilateral BAL cell count differential.

Trends and Factors Associated with Nebulized Therapy Prescription in Older Adults with Chronic Obstructive Pulmonary Disease from 2008 to 2015.

Background: Medical management of patients with chronic obstructive pulmonary disease (COPD) includes nebulized therapy as an option for inhalational drug delivery. A broad variety of short- and long-acting bronchodilators and inhaled corticosteroids in the nebulized form are available. Despite this, limited information exists on the pattern and predictors of nebulized prescription. We examined the trend and factors associated with prescription of nebulized therapy among Medicare beneficiaries with COPD. Methods: A retrospective cross-sectional study of 5% Medicare beneficiaries with COPD (n = 66,032) who were enrolled in parts A, B, and D and received nebulized prescription from 2008 to 2015 was conducted. This sample has shown to be representative of the entire fee-for-service Medicare population. The primary outcome was a prescription of nebulized medications. Reliever nebulized medications included short-acting beta agonist (SABA), short-acting muscarinic agents (SAMAs), and a combination of SABA and SAMA, while maintenance nebulized medications included long-acting beta agonists, long-acting muscarinic agents, and corticosteroid solutions as well as combinations of these agents. The secondary outcome was prescription of other inhaler respiratory medications not administered with a nebulizer. Results: Overall, 38.9% patients were prescribed nebulized medication and their prescription significantly declined from 42.4% in 2008 to 35.1% in 2015, majority of which was related to decreased prescriptions of nebulized relievers. Factors associated with the prescription of nebulized medications include female gender (odds ratio [OR] = 1.06; 95% confidence interval [CI] = 1.02-1.09), dual eligibility or low-income subsidy beneficiaries (OR = 1.49; CI = 1.44-1.53), hospitalization for COPD in the previous year (OR = 1.29; CI = 1.25-1.34), home oxygen therapy (OR = 2.29; CI = 2.23-2.36), pulmonary specialist visit (OR = 1.24; CI = 1.20-1.27), and moderate (OR = 1.61; CI = 1.57-1.65) or high (OR = 1.52; CI = 1.46-1.59) severity of COPD. Conclusion: Between 2008 and 2015, prescriptions for nebulized therapy for COPD declined among Medicare beneficiaries, probably related to increase in use of maintenance non-nebulized medications.

Spirometry Measurement of Peak Inspiratory Flow Identifies Suboptimal Use of Dry Powder Inhalers in Ambulatory Patients with COPD.

OBJECTIVES: Determine the prevalence of suboptimal peak inspiratory flow rate (PIFR) and associated patient characteristics and compare PIFR measurements obtained with spirometry and In-Check DIAL® device in ambulatory patients with COPD. METHODS: Patients underwent PIFR measurement with In-Check DIAL® device and pulmonary function testing with calibrated equipment. Group characteristics and lung function were compared for patients with suboptimal (≤ 60 L/min) and optimal (> 60 L/min) PIFR. Receiver operating curve analysis determined the best maximal forced inspiratory flow (FIF max) value in identifying optimal PIFR by gender and height. RESULTS: From July 1, 2016 to January 31, 2018, a total of 303 patients with chronic obstructive pulmonary disease (COPD) had PIFR and pulmonary function measurements. Group mean age was 65.5 ± 11.3 years with equal gender distribution. Suboptimal PIFR was observed in 61 (20.1%) patients. A significant correlation was observed between PIFR and FIF max, inspiratory capacity and residual volume (RV) to total lung capacity (TLC) ratio. In the suboptimal PIFR group, mean FIF max measured by spirometry was significantly less compared with the optimal PIFR group; 178.5 ± 56.9 L/min and 263.4 ± 89.9 L/min, respectively (p<0.0001). Receiver operator curve analysis of FIF max to identify an optimal PIFR yielded an area under the curve of 0.79. Males < 65 inches had a suboptimal PIFR in 16.7 % of the male cohort, while females < 65 inches had a suboptimal PIFR in 27.4 % of the women. CONCLUSIONS: Suboptimal PIFR was present in 1 in 5 stable patients with COPD and was more frequent in short statured females. Spirometry determined FIF max was associated with PIFR based on gender and height.

Validation of claims-based algorithms for pulmonary arterial hypertension.

Administrative claims studies do not adequately distinguish pulmonary arterial hypertension (PAH) from other forms of pulmonary hypertension (PH). Our aim is to develop and validate a set of algorithms using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes and electronic medical records (EMR), to identify patients with PAH. From January 2012 to August 2015, the EMRs of patients with ICD-9-CM codes for PH with an outpatient visit at the University of Texas Medical Branch were reviewed. Patients were divided into PAH or non-PAH groups according to EMR encounter diagnosis. Patient demographics, echocardiography, right heart catheterization (RHC) results, and PAH-specific therapies were assessed. RHC measurements were reviewed to categorize cases as hemodynamically determined PAH or not PAH. Weighted sensitivity, specificity, and positive and negative predictive values were calculated for the developed algorithms. A logistic regression analysis was conducted to determine how well the algorithms performed. External validation was performed at the University of Virginia Health System. The cohort for the development algorithms consisted of 683 patients with PH, PAH group (n = 191) and non-PAH group (n = 492). A hemodynamic diagnosis of PAH determined by RHC was recorded in the PAH (26%) and non-PAH (3%) groups. The positive predictive value for the algorithm that included ICD-9-CM and PAH-specific medications was 66.9% and sensitivity was 28.2% with a c-statistic of 0.66. The positive predictive value for the EMR-based algorithm that included ICD-9-CM, EMR encounter diagnosis, echocardiography, RHC, and PAH-specific medication was 69.4% and a c-statistic of 0.87. A validation cohort of 177 patients with PH examined from August 2015 to August 2016 using EMR-based algorithms yielded a similar positive predictive value of 62.5%. In conclusion, claims-based algorithms that included ICD-9-CM codes, EMR encounter diagnosis, echocardiography, RHC, and PAH-specific medications better-identified patients with PAH than ICD-9-CM codes alone.

Pulmonary arterial hypertension associated with interferon therapy: a population-based study.

BACKGROUND: Isolated cases of pulmonary arterial hypertension (PAH) with interferon α or ß therapy have been reported, but no population-based estimates of the incidence of the disease after interferon exposure are available. The aim of this study was to determine the incidence of PAH after initiation of interferon therapy, using a large commercial insurance database. METHODS: Using National Drug Codes (NDCs) and Healthcare Common Procedure Coding System (HCPCS) codes, we utilized the Clinformatics™ Data Mart (CDM) database to identify subjects between 20 and 65 years old who received α or ß interferon therapy between April 2001 and December 2012. Patients were followed from one year prior to the first medication claim for interferon to the first diagnosis of pulmonary hypertension using ICD-9-CM codes 416.0 and 416.8, or disenrollment. In those subjects diagnosed with pulmonary hypertension, a prescription for PAH-specific medications was used as a surrogate endpoint. RESULTS: We identified 20,113 subjects who received interferon therapy during the study period. The median follow-up was 20 months. Pulmonary hypertension occurred in 71 subjects, and PAH-specific medications were prescribed to 7 of these subjects. CONCLUSION: Although our analysis showed that the development of PAH is a rare event with interferon therapy, the risk of developing the disease is several fold higher than that for the general population.

Post-transplant native pneumonectomy for interstitial fibrosis and small cell lung cancer.

Lung transplantation is a definitive treatment for select patients with end-stage pulmonary disease. Following transplantation, the reported rate of lung cancer is between 1-9% and is associated with a variety of risk factors, including smoking history and chronic immunosuppression. The majority of post-transplant lung cancer reported in the literature is histologically classified as non-small cell lung carcinoma (NSCLC). We report a unique case of early stage small cell lung carcinoma (SCLC) identified in the native lung following single lung transplantation.

Incidence of right heart catheterization in patients initiated on pulmonary arterial hypertension therapies: A population-based study.

BACKGROUND: Pulmonary hypertension represents a heterogeneous collection of conditions classified into 5 groups according to pathology, pathophysiology and response to treatment. Right heart catheterization is required to classify patients and before initiation of specific therapy for treatment of pulmonary arterial hypertension. The aim of this study was to determine performance of right heart catheterization in patients prescribed pulmonary arterial hypertension (PAH)-specific medications. METHODS: A retrospective review of administrative claims was performed using the Clinformatics Data Mart database. Individuals with an encounter diagnosis of pulmonary hypertension were identified by ICD-9 codes. An initial encounter diagnosis was defined as continuous enrollment for 12 months without a previous ICD-9 diagnosis of pulmonary hypertension. These individuals were followed for 15 months after initial encounter diagnosis. Individuals prescribed PAH-specific medications were assessed for characteristics, comorbidities and performance of echocardiography and right heart catheterization. RESULTS: From 2002 to 2011, 15,772 patients had an outpatient visit with a diagnosis of pulmonary hypertension. From the initial group, 969 (6.1%) patients were prescribed PAH-specific medications within 1 year of encounter diagnosis. Oral PAH-specific medications were prescribed to 94.2% of patients. In patients prescribed PAH-specific medications, 91% had an echocardiogram within 1 year of encounter diagnosis. Cardiac catheterization was performed in 407 patients (42%) within 3 months of initial prescription and in 583 patients (60.2%) during the entire study period. CONCLUSION: Performance of right heart catheterization was low in this population-based study of patients with an ICD-9 diagnosis code of pulmonary hypertension and taking PAH-specific medications.

Successful treatment of aortic root abscess in a patient with pulmonary arterial hypertension.

Continuous prostanoid infusion is an established treatment for pulmonary arterial hypertension that has led to improvements in symptoms, exercise tolerance, and survival. Patients with pulmonary arterial hypertension (PAH) who develop sepsis frequently experience clinical and hemodynamic deterioration associated with poor outcomes. Successful management of sepsis involves identification of the source of infection, early antimicrobial administration, judicious fluid resuscitation, and continuation of specific PAH therapies. We describe successful management of a patient with idiopathic PAH receiving chronic intravenous prostacyclin therapy who developed an aortic root abscess due to Clostridium perfringens requiring emergent aortic root repair. Management involved imaging studies, removal of potential sources with administration of intravenous antibiotics, and cautious fluid administration with hemodynamic monitoring. A multidisciplinary group led by a PAH specialist worked cohesively before, during, and after surgical intervention and achieved a successful outcome.

Burn injury associated with home oxygen use in patients with chronic obstructive pulmonary disease.

OBJECTIVES: To quantify the risk of burn injury associated with home oxygen use and to examine the risk factors associated with the development of this injury. PATIENTS AND METHODS: We used a nested case-control and a retrospective cohort design to study enrollment and claims data from a national sample of Medicare beneficiaries 66 years and older with a diagnosis of chronic obstructive pulmonary disease (COPD) from January 1, 2001, through December 31, 2010. The primary outcome was burn injury in patients with COPD prescribed home oxygen. RESULTS: In the nested case-control method, patients with burn injury were twice (odds ratio, 2.43; 95% CI, 1.57-3.78) as likely to be prescribed oxygen in the preceding 90 days compared with those without burn injury. In the retrospective cohort study, the absolute risk of burn injury in patients prescribed oxygen therapy was 2.98 per 1000 patients compared with 1.69 per 1000 patients not prescribed oxygen during a 22-month period. The excess risk of a burn injury associated with oxygen was 0.704 per 1000 patients per year, and the number needed to harm was 1421. In multivariable analysis, factors associated with burn injury included male sex, low socioeconomic status, oxygen therapy use, and the presence of 3 or more comorbidities. CONCLUSION: The benefits of oxygen therapy in patients with COPD outweigh the modest risk of burn injury associated with home oxygen use. However, with the increasing number of patients being prescribed oxygen, health care professionals must educate and counsel patients regarding the potential risk of burn injury.

ARDS in pregnancy.

Acute respiratory distress syndrome (ARDS) is an uncommon condition in pregnant patients. The causes of ARDS are associated with obstetric causes such as amniotic fluid embolism, preeclampsia, septic abortion, and retained products of conception or nonobstetric causes that include sepsis, aspiration pneumonitis, influenza pneumonia, blood transfusions, and trauma. An essential component in management of ARDS involves good communication between the obstetrics team and critical care specialist and a fundamental understanding of mechanical ventilatory support. Medical therapies such as nitric oxide and corticosteroids play a complimentary role. Extracorporeal life support is beneficial in the management of the parturient with severe ARDS.

Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare disease with a predilection for young women that is associated with right ventricular failure and premature death. PAH can complicate pregnancy with hemodynamic instability or sudden death during parturition and postpartum. Our aim was to examine the impact of PAH on pregnancy outcomes in the modern era. METHODS: We conducted a retrospective evaluation of pregnant patients with PAH managed between 1999 and 2009 at five US medical centers. Patient demographics, medical therapies, hemodynamic measurements, manner of delivery, anesthetic administration, and outcomes were assessed. RESULTS: Among 18 patients with PAH, 12 continued pregnancy and six underwent pregnancy termination. Right ventricular systolic pressure in patients managed to parturition was 82 ± 5 mm Hg and in patients with pregnancy termination was 90 ± 16 mm Hg. Six patients underwent pregnancy termination at mean gestational age of 13 ± 1.0 weeks with no maternal deaths or complications. Twelve patients elected to continue their pregnancy and were hospitalized at 29 ± 1.4 weeks. PAH-specific therapy was administered to nine (75%) at time of delivery consisting of sildenafil, IV prostanoids, or combination therapy. All parturients underwent Cesarean section at 34 weeks with one in-hospital death and one additional death 2 months postpartum for maternal mortality of 16.7%. CONCLUSIONS: Compared with earlier reports, maternal morbidity and mortality among pregnant women with PAH was reduced, yet maternal complications remain significant and patients should continue to be counseled to avoid pregnancy.

Chronic pulmonary artery dissection associated with pulmonary arterial hypertension.

Abstract Pulmonary artery dissection is a complication associated with pulmonary arterial hypertension. This complication is described as acute in onset and is frequently fatal without intervention. We describe a patient with idiopathic pulmonary arterial hypertension and chest pain found to have an unsuspected chronic pulmonary artery dissection on postmortem examination. Chronic pulmonary artery dissection should be considered in patients with chest pain and worsening dyspnea, as the frequency this condition may be underestimated.