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.

Community-acquired round Pneumonia in a 63-year-old Female.

Round pneumonia is a radiological manifestation of pulmonary lesion. This is found as spherical or oval-shaped radio-opacity on chest X-ray. Round pneumonia has been reported in literature uncommonly. Round pneumonia was first time reported in the radiology literature in 1954 (Wagner et al., 1998). It was first recognized in children. In 1973, Rose and Ward reviewed 21 cases of round pneumonia in children. Radiological findings resembled pulmonary and mediastinal masses. Since then, time and again, round pneumonia has been reported in children; but, this is also found rarely in adults. There are many causes of round pneumonia in adults, for example, infectious and noninfectious. It may mimic pulmonary neoplasms due to its radiological appearance. Hence, the usual diagnostic challenge of round pneumonia is to differentiate pneumonia from bronchogenic carcinoma. Here we present an interesting case of round pneumonia in an adult female.

Dissecting heart failure.

Dissection of ascending aorta is a medical emergency typically presenting with acute chest or back pain and hemodynamic instability. We are reporting a very unusual case of dissection of a large ascending aortic aneurysm presenting as a new onset heart failure. A 46-year-old man presented with gradually increasing dyspnea and orthopnea. His physical examination and laboratory findings were consistent with heart failure. The only unusual feature was a diastolic murmur, which prompted echocardiographic evaluation. Besides left ventricular dilatation, hypertrophy, and severe global hypokinesis, which were expected, we also found severely dilated aortic root with aortic regurgitation and a 8.6×9.7 cm ascending aortic aneurysm with dissection. The patient had a brother who died several years earlier from aortic dissection. Surgical treatment was successful. Type A aortic dissection may rarely present as heart failure. Aortic dissection at young age should prompt screening of first-degree relatives because genetic nature of the disease is very likely.

Effects of pulmonary rehabilitation on Fatigue Severity Scale in patients with lung disease.

INTRODUCTION: Fatigue is a known symptom of advanced lung disease and impacts quality of life and psychological health. Many of these patients undergo pulmonary rehabilitation as part of their therapy. Understanding the effect of pulmonary rehabilitation on fatigue in these patients is important, as one may be able to design more focused rehabilitation programs. The aim of this study is to evaluate the effect of pulmonary rehabilitation on fatigue as measured by the Fatigue Severity Scale (FSS) in patients with advanced lung disease. MATERIAL AND METHODS: Patients were enrolled in a standardized 6 week pulmonary rehabilitation program. They were asked to complete questionnaires to evaluate their self-reported fatigue (FSS), and depression as measured by Geriatric Depression Scale (GDS). The GDS is a self-reported assessment tool used to identify depression in patients. The FSS is a validated instrument that indicates a perception of fatigue that might require medical intervention. Participants completed questionnaires both at baseline and after completing the standardized pulmonary rehabilitation program. Data was analyzed in Statistical Analysis System (SAS). The change in FSS was evaluated using the Wilcoxon signed-rank test. P-values < 0.05 were considered statistically significant. RESULTS: 21 patients (12 females; 9 males; mean age 64.3 ± 11.2 yrs) were considered for the study. Pre-pulmonary rehabilitation FSS scores ranged from 1.6 to 6.7 (mean score of 4.6 ± 1.7). Post pulmonary rehabilitation FSS scores ranged 1.0 to 6.2 (mean score of 3.9 ± 1.6). The median pre-rehabilitation FSS was 5.3 (inter quartile range; Q1-Q3: 3.0-6.1), and median post rehabilitation FSS was 3.9 (inter quartile range; Q1-Q3: 2.6-5.1). There was a significant decrease in FSS scores after completing pulmonary rehabilitation program (p < 0.0208). There was a decrease in GDS (pre-rehabilitation, mean: 5.5 ± 3.6; post-rehabilitation, mean: 4.2 ± 2.9), but this decrease was not statistically significant. The change in GDS correlated with the change in FSS (Spearman Correlation Coefficient 0.525, p < 0.0146). CONCLUSIONS: Patients with advanced lung disease reported a measurable component of fatigue. Participating in pulmonary rehabilitation resulted in significant improvement in patient's self-reported fatigue severity. Further studies are necessary to evaluate and design interventions to improve fatigue in in the setting of advanced lung disease.

A rare case of endobronchial and lung metastasis in basal cell carcinoma.

Basal cell carcinoma (BCC) is the most common skin cancer, but rarely metastasizes. This article describes diagnosis and treatment of an extremely rare case of BCC metastasizing to the lung and endobronchial tissue.

Hirayama Disease: Neutral and Flexion Magnetic Resonance Imaging Manifestations and Single Tertiary Care Center Analysis on 3T Scanner.

BACKGROUND: Hirayama disease (HD) is a rare benign type of cervical cord myelopathy occurring commonly in young males as unilateral or bilateral asymmetrical amyotrophy of the hand and forearm muscles in C8-T1 distribution. Magnetic resonance imaging (MRI) is the best technique for the evaluation and imaging of this entity. MATERIALS AND METHODS: This is a retrospective review of cervical magnetic resonance images of patients that were taken for clinically suspected and diagnosed HD on 3T MRI in postcontrast neutral and flexion (30°-40°) positions from July 2019 to January 2024 at Shri Ram Murti Smarak Institute of Medical Sciences, Bareilly. RESULTS: Fourteen patients included in the study were males less than 34 years of age. MRI findings of cord atrophy in the lower cervical region/cervico-dorsal junction, abnormal cervical curvature, loss of attachment of the dorsal dural sac and subjacent laminae with anterior displacement, and a prominent intense enhancing posterior epidural space were observed in all 14 patients. The minimum anteroposterior cord diameters in the neutral and flexion positions were 2.9 and 2.8 mm, respectively (mean thickness of laminodural space on flexion - 5.2 mm). Other MRI findings showed variable representations. CONCLUSIONS: Flexion-position MRI has emerged as the gold standard for establishing and validating the diagnosis of HD in clinically suspected cases and should be an essential part of the protocol for the screening of clinically suspected cases of HD to aid in early treatment and therapeutic intervention. Complimentary newer sequences such as the Three-dimensional (3D)-Constructive interference in Steady State (CISS)/Fast Imaging Employing Steady-state Acquisition Cycled Phases (FIESTA-C) may reinforce better appreciation of epidural flow voids.

Ortner's syndrome: A systematic review of presentation, diagnosis and management.

Ortner's syndrome (OS), also called cardiovocal syndrome, is a rare condition hallmarked by left recurrent laryngeal nerve palsy due to underlying cardiopulmonary disease. The purpose of this review is to systemically analyze the existing literature for cases of OS to outline typical presentation, methods of diagnosis, and management of these patients. Case reports, case series, and cohort studies describing OS between 1955 and 2021 were identified. Individual manuscripts were reviewed for clinical features, presentation, and management. A total of 117 patient cases were gathered from 92 published articles. Common symptoms included hoarseness, dyspnea, cough, and dysphagia. The most common associated comorbidity was aortic aneurysm (41%), followed by pulmonary hypertension (35%), mitral stenosis (17%), and hypertension (12%). Among those who were managed via surgical intervention, 85.4% reported improvement in their hoarseness. While historically OS was associated with mitral stenosis, in recent decades, aortic aneurysms and dilation of the pulmonary artery from pulmonary hypertension have emerged as primary etiologies of OS. Therefore, OS should be considered in any patient presenting with hoarseness and history of cardiopulmonary disease. Surgical intervention in appropriate candidates resolves OS in most cases.

Right side of heart failure.

The function of the right ventricle (RV) in heart failure (HF) has been mostly ignored until recently. A 2006 report of the National Heart, Lung, and Blood Institute identified a gap between RV research efforts and its clinical importance compared with that of the left ventricle. This recent shift in paradigm is fueled by the prognostic value ascribed to RV failure in HF and morbidity/mortality after myocardial infarction and surgery. In this review, we examine the significance of RV failure in the HF setting, its clinical presentation and pathophysiology, and ways to evaluate RV function using echocardiographic measurements. Furthermore, we discuss the medical management of RV failure including traditional therapies like beta-blockers and newer options like nitric oxide, phosphodiesterase inhibitors, and calcium sensitizers. Mechanical support is also examined. Finally, this review places an emphasis on RV failure in the setting of left ventricular assist devices and heart transplantation.

Obstructive lung disease secondary to compression of the bronchus by an enlarged pulmonary artery.

In clinical practice, the presence of wheezing generally indicates an airway disease. In rare circumstances, adjacent mediastinal structures may compress the tracheobronchial tree leading to obstructive physiology. Compression of the tracheobronchial region by an enlarged pulmonary artery (PA) is exceedingly rare. We present here a case of pulmonary hypertension, where the enlarged PA resulted in obstructive lung physiology with a relevant review of the literature.

The uses of overnight pulse oximetry.

Overnight pulse oximetry (OPO) has proven to be an effective and beneficial technique to determine the cardiorespiratory status of patients in both the inpatient and outpatient settings. It is a cheap, safe, reliable, simple, and accurate method of patient monitoring as compared to the expensive and labor-intensive method of multichannel polysomnography for detecting sleep-disordered breathing. It provides accurate information about patient's oxygenation status and also helps in monitoring the response to continuous positive airway pressure and in the surgical treatment of obstructive sleep apnea (OSA). Nocturnal hypoxemia portends a poor prognosis in patients of chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and neuromuscular diseases. OPO can help its early detection and management.

Exercise tolerance improves after pulmonary rehabilitation in pulmonary hypertension patients.

Pulmonary rehabilitation (PR) is part of the recommended management plan of pulmonary hypertension (PHTN) and is important to better quality of life and exercise tolerance. This study aimed at determining effectiveness of PR on exercise capacity. Retrospective chart analysis was conducted on patients referred to our PHTN clinic for PR. Patients who had PHTN diagnosed on right heart catheterization (defined by mean pulmonary artery pressure>25 mmHg) and completed a standardized 12-week PR program were considered for the study. Patients' baseline exercise tolerance was recorded as speed attainable on a treadmill and duration of exercise in minutes. Demographics, age, sex, and oxygen use were obtained from chart review. Eighteen PHTN patients (5 male, 13 female; mean age 67.7±11.6 years) were considered for the study (six World Health Organization [WHO] group 1 pulmonary arterial hypertension [33.33%], eight WHO group III PHTH [44.44%], two WHO group IV and two WHO V PHTN [11.11%]). Treadmill speed improved following rehabilitation (1.3 mph [interquartile range {IQR}, 1.0-1.8 mph] to 2.2 mph [IQR, 1.3-2.8 mph]; P<0.0001, Wilcoxon signed rank test). Median exercise time improved (27 min (IQR, 22-30 min) to 30 min (IQR, 24-30 min); not significant. Improvement was defined only as an increase in speed or duration, or both. Sixteem of 18 participants improved (88.9% [95% exact binomial confidence interval, 65.3%-98.6%). Patients with PHTN benefit from a structured PR program to improve their exercise capacity and should be enrolled in PR programs as part of their management.

Depression in pulmonary arterial hypertension: An undertreated comorbidity.

Pulmonary arterial hypertension (PAH) is a debilitating condition leading to progressive decline in functional capacity. As a result, PAH can lead to psychological impairment that can impact the overall disease status. The medical community has developed several screening questionnaires in order to assess depression in their patients allowing physicians to be at the forefront of recognizing clinical depression. There is a suggestion that depression symptomatology is more prevalent in the PAH population. The aim of this article is to review the current thought process about diagnosis and management of depression in PAH patients.

Posterior capsule rupture with herniation of lens fragment following blunt ocular trauma.

Posterior capsule rupture with herniated lens fragment in the vitreous cavity on magnetic resonance imaging has not been reported in India until now; however, it has been reported in other countries. Therefore, this study reports the case of a 15-year-old boy presenting with posttraumatic loss of vision in the right eye due to posterior capsular rupture and herniation of lens material into the vitreous cavity, which was detected by B-scan ultrasonography and magnetic resonance imaging as no ophthalmic examination was possible due to the posttraumatic cataract. The patient was treated by lens aspiration with anterior chamber vitrectomy and placement of posterior chamber intraocular lens, with the patient achieving 6/6 visual acuity postoperatively. This case is unusual due to the rarity of the findings, and it highlights the crucial role of imaging in achieving timely diagnosis and surgery to restore vision in the affected eye.

Caregiver's burden in pulmonary arterial hypertension: a clinical review.

Caregiver's burden is a multidimensional phenomenon affecting care-givers physically, emotionally and socially. It is critical to examine the burden of caregivers, because of the complex responsibility they have with their partners. There are relatively few studies that have examined factors linked with psychological burden amongst caregivers of pulmonary arterial hypertension (PAH) patients. Hence, it is pertinent to develop a good understanding of these factors and develop appropriate management strategies, modified to assist PAH caregivers.

Dyspnea, depression and health related quality of life in pulmonary arterial hypertension patients.

Pulmonary arterial hypertension (PAH) is a rare and devastating disease which is characterized by worsening dyspnea and exercise tolerance. These patients are often found to have concomitant, depression, anxiety and impaired health-related quality of life (HRQOL). The interrelationship of dyspnea, depression and HRQOL in these patients is not well studied. Retrospective analysis was performed on 46 PAH patients (mean age 51.73). Patients completed Medical Outcomes Study Short - Form 36 V2 (SF-36) to measure HRQOL, Modified Medical Research Council (mMRC) Dyspnea Scale and Zung Depression Scale (ZDS). Physical Health Composite Scores (PCS) and Mental Health Composite Scores (MCS) were derived from SF-36. Spearman's correlation was computed to determine degree of correlation between pairs of scales. 46 patients (12 males, 34 females; median age 51.4 yr) with confirmed PAH were considered for the study of which 36 patients (9 males, 27 females, median age 50.1 yr), were eligible for further analysis. MMRC Dyspnea Scale Score was 1.0 (Q1 to Q3:1.0 to 2.0). Median MCS was 52.1 (Q1 to Q3:41.7 to 57.1) and PCS was 37.9 (Q1 to Q3: 30.7 to 49.6). There was a significant negative correlation between dyspnea and PCS (r =-0.660, P<0.0001) and MCS (r =-0.342, P<0.0411). The ZDS was available for 17 of these patients; their median score was 42.0 (Q1 to Q3: 33.0 to 46.0). There was a significant correlation between the ZDS and PCS (r =-0.578, P<0.0150,) MCS (r =-0.752, P<0.0005). Patients with PAH suffer from diminished HRQOL correlating with their dyspnea and underlying depression.

Post splenectomy related pulmonary hypertension.

Splenectomy predisposes patients to a slew of infectious and non-infectious complications including pulmonary vascular disease. Patients are at increased risk for venous thromboembolic events due to various mechanisms that may lead to chronic thromboembolic pulmonary hypertension (CTEPH). The development of CTEPH and pulmonary vasculopathy after splenectomy involves complex pathophysiologic mechanisms, some of which remain unclear. This review attempts congregate the current evidence behind our understanding about the etio-pathogenesis of pulmonary vascular disease related to splenectomy and highlight the controversies that surround its management.

Depression in pulmonary arterial hypertension and interstitial lung diseases.

Advanced lung diseases such as pulmonary arterial hypertension (PAH) and interstitial lung diseases (ILD) are chronic diseases that cause significantly high morbidity and mortality. As a result, patients can undergo some psychological changes leading to a poor quality of life and depression. Diagnosis of depression is often obscured because fatigue and apathy, two common symptoms of depression, frequently overlap with PAH and ILD. Healthcare providers are sometimes reluctant to ask or mistakenly believe that these symptoms are part of the ongoing disease process, rather than a serious condition like depression. Screening tools are available for physicians to be well positioned in recognizing clinical depression in PAH and ILD. A MedLine/PubMED search was performed identifying all relevant articles with "PAH", "ILD", "screening tools" and/or "Depression" in the title. The aim of this review is to provide a brief description of some of the instruments used to screen patients and classes of psychotropic medications accessible to physicians. While pulmonary rehabilitation programs can have a positive impact on patients, physicians should also utilize cognitive behavioral therapy (CBT) as part of regular care.

Association between weight loss and improvement of ventricular systolic function in advanced heart failure.

In heart failure (HF), longitudinal changes in ventricular ejection fraction are poorly studied. The authors' objective was to document the dynamic changes in systolic function of both ventricles during acute HF and after a 3-month follow-up period, and to identify factors associated with ventricular improvement. A limited access dataset from the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) trial provided by The National Heart, Lung, and Blood Institute was analyzed. In patients admitted to the hospital with acute HF, both ventricles were evaluated by echocardiography on admission, at discharge, and at 3 months. From baseline to 3 months, left ventricular ejection fraction (LVEF) increased in 43.4% of patients, decreased in 23.9%, and remained unchanged in 32.7%. Similarly, right ventricular systolic function improved in 40.9%, deteriorated in 25.0%, and remained stable in 34.1%. Weight loss during index admission and cardiac index/cardiac output increase were the only factors associated with LVEF improvement from baseline to month 3. There was no difference between ischemic and nonischemic etiology. In acute HF, ischemic or nonischemic, systolic function of the ventricles improved during 3 months in about 40% of patients and remained unchanged or deteriorated in the rest. Weight loss and increase in cardiac index/output during hospitalization was associated with improvement in LVEF.

Spontaneous pneumomediastinum: time for consensus.

Pneumomediastinum (PM) is defined as the presence of free air in the mediastinal cavity. It is often regarded as a revealing sign of a more serious medical condition. PM is broken down into two categories, one, with an instigating event, referred to as secondary PM. The other is when free air is discovered in the mediastinal cavity without a clear etiology, referred to as spontaneous pneumomediastinum (SPM). Often misdiagnosed due to the vague nature of presenting symptoms, SPM must be part of the differential diagnosis of a chest pain patient to expedite discovery and if necessary, management. A MedLine/PubMED search was performed identifying all relevant articles with "SPM" in the title. Six case series were reviewed to determine what clinical scenario constitutes a possible case of SPM. Results showed that almost all patients with SPM exhibited some chest pain, but Hamman's crunch was present in only one-fifth of patients. Patients with certain pre-existing pulmonary diseases showed a greater propensity for the presence of free air in the mediastinal cavity. SPM must be diagnosed and managed promptly due to rare, but serious complications and any chest pain with an unknown etiology should contain SPM in the differential diagnosis.