Hypersensitivity pneumonitis, allergic bronchopulmonary aspergillosis and other eosinophilic lung diseases.

Hypersensitivity pneumonitis (HP) is traditionally classified into acute, subacute and chronic forms. A high index of suspicion and a detailed investigation into the patient's environment is the key to diagnosis and treatment of HP. Eosinophilic lung diseases can be broadly categorized as idiopathic (acute eosinophilic pneumonia, chronic eosinophilic pneumonia, eosinophilic granulomatosis with polyangiitis and hypereosinophilic syndromes), those with known cause (allergic bronchopulmonary aspergillosis, drugs, parasitic and non-parasitic infections), and those associated with other known lung diseases (asthma, interstitial lung diseases and lung cancers). A detailed review of drug intake, toxin exposures, and travel history is essential in the differential diagnosis of eosinophilic lung diseases.

Counting Steps: A New Way to Monitor Patients with Pulmonary Arterial Hypertension.

RATIONALE: Activity levels in patients with pulmonary arterial hypertension (PAH) have correlated with surrogate markers of disease severity. It is not known whether physical activity measures are useful in monitoring patients with PAH. OBJECTIVES: This pilot study aimed to evaluate whether change in physical activity measured by an accelerometer correlates with changes in six-minute walk distance (6MWD), echocardiographic parameters, NT-proBNP, or health-related quality-of-life measures (HRQOL). METHODS: The study design was a prospective, observational study in subjects with prevalent PAH. Subjects wore a wrist-worn accelerometer (Fitbit Charge HR®) between two outpatient visits. Daily step count and activity levels were recorded, and the change over time was correlated with changes in 6MWD, echocardiographic parameters, HRQOL, and NT-proBNP. MEASUREMENTS AND MAIN RESULTS: 30 subjects were enrolled, of which 20 patients had adequate accelerometer data to be analyzed over the study duration. The mean duration of follow-up was 136.4 ( ± 47.3) days. The change in daily step count correlated with a change in 6MWD (r 0.43, p 0.05). Changes in duration spent in moderately active (r 0.52, p 0.02), lightly active (r 0.48, p 0.05), and sedentary activity levels (r - 0.54, p 0.02) correlated with a change in HRQOL. Changes in activity levels did not correlate with echocardiographic measures or NT-pro BNP. CONCLUSIONS: Changes in daily step count and time spent at fairly active, lightly active, and sedentary activity levels correlate with changes in 6MWD, and HRQOL in subjects with PAH suggesting that accelerometry may be a useful monitoring tool.

Clinical Characteristics and Associated Systemic Diseases in Patients With Esophageal "Absent Contractility"-A Clinical Algorithm.

GOALS: This study was carried out to assess the clinical characteristics and associated systemic diseases seen in patients diagnosed with absent contractility as per the Chicago Classification version 3.0, allowing us to propose a diagnostic algorithm for their etiologic testing. BACKGROUND: The Chicago Classification version 3.0 has redefined major and minor esophageal motility disorders using high-resolution esophageal manometry. There is a dearth of publications based on research on absent contractility, which historically has been associated with myopathic processes such as systemic sclerosis (SSc). STUDY: We conducted a retrospective, multicenter study. Data of patients diagnosed with absent contractility were pooled from Cleveland Clinic, Cleveland, OH (January 2006 to July 2016) and Metrohealth Medical Center, Cleveland, OH (July 2014 to July 2016) and included: age, gender, associated medical conditions, surgical history, medications, and specific antibody testing. RESULTS: A total of 207 patients, including 57 male individuals and 150 female individuals, with mean age of 56.1 and 60.0 years, respectively, were included. Disease distribution was as follows: SSc (diffuse or limited cutaneous) 132, overlap syndromes 7, systemic lupus erythematosus17, Sjögren syndrome 4, polymyositis 3, and dermatomyositis 3. Various other etiologies including gastroesophageal reflux disease, postradiation esophagitis, neuromuscular disorders, and surgical complications were seen in the remaining cohort. CONCLUSIONS: Most practitioners use the term "absent contractility" interchangeably with "scleroderma esophagus"; however, only 63% of patients with absent contractility had SSc. Overall, 20% had another systemic autoimmune rheumatologic disease and 16% had a nonrheumatologic etiology for absent contractility. Therefore, alternate diagnosis must be sought in these patients. We propose an algorithm for their etiologic evaluation.

Six-Minute Walk Test as a Predictor of Diagnosis, Disease Severity, and Clinical Outcomes in Scleroderma-Associated Pulmonary Hypertension: The DIBOSA Study.

BACKGROUND: Pulmonary hypertension (PH) is a common complication of scleroderma (SSc) and is a leading cause of morbidity and mortality. OBJECTIVES: To explore the utility of the 6MWT in the prediction of SSc-PH and to assess its prognostic implications. METHODS: A retrospective review of SSc patients from 2003 to 2013, with 6MWT and echocardiogram (n = 286), was conducted. Presence of PH was defined by right heart catheterization. Patients were randomized into development and validation cohorts. Using regression techniques, we developed a scoring system to predict the presence of SSc-PH and tested it in our validation cohort. Trends of mortality and disease severity were studied for incremental scores. RESULTS: The DIBOSA scoring system includes DIstance walked in 6 min, BOrg dyspnea index, and SAturation of oxygen at 6 min. The DIBOSA score in the development cohort ranged from 0 to 3, resulting in an area of 0.858 (P < 0.0001) under the ROC curve. A score of 0 had a NPV of 100% and a score of 3 had a PPV of 86.58%. The validation cohort had an area under the ROC curve of 0.842. The DIBOSA score correlated with both pulmonary artery pressures and mortality. The 3-year survival rates for DIBOSA scores of 0, 1, 2, and 3 were 100, 100, 87.67, and 66.67%, respectively (HR = 3.92, P < 0.0001). CONCLUSIONS: DIBOSA score is a sensitive tool for the prediction of SSc-PH. The DIBOSA score is a direct predictor of mortality in SSc-PH and strongly correlates with pulmonary pressures. 6MWT can be used to predict clinical outcomes in SSc-PH.

Cardiac Implantable Electronic Device Infections: Added Complexity and Suboptimal Outcomes With Previously Abandoned Leads.

OBJECTIVES: This study sought to assess the impact of previously abandoned leads on the clinical management of cardiac device infections, notably transvenous lead extraction and subsequent clinical course. BACKGROUND: The population of patients with cardiac implantable electronic devices continues to grow with a disproportionate increase in device infections, which are invariably life threatening. A potentially complicating issue is the widely practiced strategy of device lead abandonment at the time of system revision, change, or upgrade, which is affecting an increasing number of patients. METHODS: The study assessed the impact of previously abandoned leads in a prospectively maintained registry of consecutive patients undergoing percutaneous extraction of infected cardiac devices at the Cleveland Clinic between August 1996 and September 2012. The primary clinical endpoint was complete procedural and clinical success defined as the successful removal of the device and all lead material from the vascular space, in the absence of a major complication. RESULTS: Of 1,386 patients with infected cardiac devices, 323 (23.3%) had previously abandoned leads. Failure to achieve the primary endpoint occurred more frequently in patients with abandoned leads (13.0% vs. 3.7%; p < 0.0001). This was primarily due to retention of lead material (11.5% vs. 2.9%; p < 0.0001), which was associated with poor clinical outcomes including higher rates of 1-month mortality (7.4% vs. 3.5% in those without lead remnants). Lead extraction procedures in patients with previously abandoned leads were longer (p < 0.0001), with longer fluoroscopy times (p < 0.0001), and more likely to require specialized extraction tools (94.4% vs. 81.8%; p < 0.0001) or adjunctive rescue femoral workstations (14.9% vs. 2.9%; p < 0.0001). Procedural complications occurred more frequently in patients with previously abandoned leads (11.5% vs. 5.6%; p = 0.0003), which was true for both major (3.7% vs. 1.4%; p = 0.009) and minor complications (7.7% vs. 4.4%; p = 0.02). CONCLUSIONS: Previously abandoned leads complicate the management of cardiac device infections, leading to worse clinical outcomes.

Safety of Catheter Ablation for Atrial Fibrillation in Patients With Prior Cerebrovascular Events.

OBJECTIVES: This study sought to report on the safety of catheter ablation for atrial fibrillation (AF) in patients with prior cerebrovascular events (CVEs), at a large-volume tertiary care center over the course of the past 15 years. BACKGROUND: Many patients with drug-refractory AF have a history of a prior CVE. These patients are considered to be at high procedural risk for catheter ablation but data are scant. METHODS: All consecutive patients undergoing AF ablation at the Cleveland Clinic were enrolled in a prospectively maintained data registry, which was used to identify patients with a prior CVE. Strict periprocedural anticoagulation protocols were in place. Extreme care was taken with sheath and catheter manipulation to prevent thrombus formation or air embolism. All thromboembolic and hemorrhagic events occurring periprocedurally and up to 3 months of follow-up were identified. RESULTS: Of 9,413 consecutive patients who underwent AF ablation, 247 patients with a prior CVE were identified (median age, 64 years; 40.1% female; median CHA2DS2-VASC score, 4). Anticoagulants used were warfarin (n = 192), dabigatran (n = 32), rivaroxaban (n = 15), and apixaban (n = 8). All patients received intravenous heparin before transseptal access (activated clotting time target during procedure, 350 to 400 seconds). The energy source was radiofrequency in 242 patients and cryoenergy in 5 patients. Acute procedural complications included 5 groin hematomas (1 requiring transfusion), 5 pericardial effusions with associated tamponade physiology in 2 (1 required pericardiocenthesis, 1 required surgery), and 1 arteriovenous fistula (managed conservatively). Importantly, none of the patients had a periprocedural thromboembolic event. CONCLUSIONS: Patients with a prior history of cerebrovascular events do not seem to be predisposed to a significant risk of clinical CVE recurrence when undergoing catheter ablation for AF without interruption of therapeutic anticoagulation.