Sonu Sahni

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.

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.

Pulmonary rehabilitation and exercise in pulmonary arterial hypertension: An underutilized intervention

Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance which eventually leads to right ventricular failure and death. Early thought process was that exercise and increased physical activity may be detrimental to PAH patients however many small cohort trials have proven otherwise. In addition to the many pharmaceutical options, exercise and pulmonary rehabilitation have also been shown to increase exercise capacity as well as various aspects of psychosomatic health. As pulmonary and exercise rehabilitation become more widely used as an adjuvant therapy patient outcomes improve and physicians should consider this in the therapeutic algorithm along with pharmacotherapy.

Pulmonary manifestations of renal cell carcinoma

Renal cell carcinoma (RCC) accounts for majority of all primary renal neoplasms. Classic manifestations of RCC include the triad of flank pain, hematuria and a palpable renal mass. Patients with RCC can develop various extra renal manifestations including involvements of the lungs, inferior vena cava, liver and the bones. The pulmonary manifestations of renal cell carcinoma include metastatic disease including endobronchial, pleural, parenchymal or lymph node metastasis, pleural effusion or hemothorax. Pulmonary embolism and tumor embolism is another common manifestation of renal cell carcinoma. RCC is a highly vascular tumor and can cause pulmonary arterio-venous fistulas leading to high output failure. Rarely, RCC can also present with paraneoplastic presentations including cough or bilateral diaphragm paralysis. Drugs used to treat RCC have been associated with drug related pneumonitis and form an important differential diagnosis in patients with RCC on therapy presenting with shortness of breath. In this review we discuss the various pulmonary manifestations of RCC. A high index of suspicion with these presentations can lead to an early diagnosis and assist in instituting an appropriate intervention.

Pulmonary arterial hypertension: a current review of pharmacological management

Pulmonary hypertension (PHTN) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance, which eventually leads to right ventricular failure and death. At present there is no cure for pulmonary arterial hypertension (PAH); however over the past decade targeted pharmaceutical options have become available for the treatment of PAH. Prior to evaluation for therapeutic options a definitive diagnosis of pulmonary arterial hypertension must be made via comprehensive physical exam and definitive diagnostic testing. Screening test of choice remains echocardiography and gold standard for definitive diagnosis is right heart catheterization. Once the establishment of a diagnosis of PAH is made therapeutic options may be a possibility based on a diagnostic algorithm and disease severity of the PAH patient. There are different classes of medications available with different mechanisms of actions which net a vasodilatory effect and improve exercise tolerance, quality of life as well and survival.

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.

Health Disparities in Patients with Pulmonary Arterial Hypertension: A Blueprint for Action. An Official American Thoracic Society Statement

Background: Health disparities have a major impact in the quality of life and clinical care received by minorities in the United States. Pulmonary arterial hypertension (PAH) is a rare cardiopulmonary disorder that affects children and adults and that, if untreated, results in premature death. The impact of health disparities in the diagnosis, treatment, and clinical outcome of patients with PAH has not been systematically investigated. Objectives: The specific goals of this research statement were to conduct a critical review of the literature concerning health disparities in PAH, identify major research gaps and prioritize direction for future research. Methods: Literature searches from multiple reference databases were performed using medical subject headings and text words for pulmonary hypertension and health disparities. Members of the committee discussed the evidence and provided recommendations for future research. Results: Few studies were found discussing the impact of health disparities in PAH. Using recent research statements focused on health disparities, the group identified six major study topics that would help address the contribution of health disparities to PAH. Representative studies in each topic were discussed and specific recommendations were made by the group concerning the most urgent questions to address in future research studies. Conclusions: At present, there are few studies that address health disparities in PAH. Given the potential adverse impact of health disparities, we recommend that research efforts be undertaken to address the topics discussed in the document. Awareness of health disparities will likely improve advocacy efforts, public health policy and the quality of care of vulnerable populations with PAH.

Socioeconomic status affects pulmonary hypertension disease severity at time of first evaluation

A low socioeconomic status (SES) has been linked to disproportionate access to health care in many diseases, leading to worse disease severity at initial presentation. There is a paucity of these data in the pulmonary hypertension (PHTN) population. We studied the association of SES, as measured by zip code–based median annual household income, with World Health Organization functional class (WHO-FC) at time of first evaluation in PHTN patients. All patients evaluated at our center with a right heart catheterization revealing a mean pulmonary artery pressure of ≥25 mmHg within 12 months of initial evaluation were considered for the study. Demographics, WHO-FC, and zip codes were obtained from retrospective chart analysis. The 2010 US census was used to obtain zip code–based annual median income. The income groups were divided into quartiles. Patients were categorized by their WHO-FC and zip code–derived median income. Similar analyses were conducted for pulmonary arterial hypertension (PAH) patients. Survival was estimated with the Kaplan-Meier method. Data were analyzed in SAS, and P < 0.05 was considered significant. There were 228 PHTN patients (70 [30.7%] male, 158 [69.3%] female). As median income decreased, the FC at presentation increased, signifying higher disease severity (Spearman correlation: r = −0.161, P < 0.0515). This association between median income groups and WHO-FC at initial evaluation was significant (χ2 test: P < 0.0168). There were 116 PAH patients (32 [27.6%] male, 84 [72.4%] female). There was again a negative relationship between income and initial FC (Spearman correlation: r = −0.0307, P < 0.0007). A lower SES was associated with worse disease, as measured by WHO-FC.

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.

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 patients self-reported fatigue severity. Further studies are necessary to evaluate and design interventions to improve fatigue in in the setting of advanced lung disease.