Abnormal Heart Rate Recovery and Chronotropic Incompetence With Exercise in Patients With Interstitial Lung Disease With and Without Pulmonary Hypertension.

Introduction Chronotropic incompetence (CI) and heart rate (HR) recovery at one minute post-exercise (HRR1) have been proposed as indicators of autonomic imbalance. We retrospectively studied the presence of CI and HRR1 attained on cardiopulmonary exercise testing (CPET) in patients with interstitial lung disease (ILD) and those with interstitial lung disease with pulmonary hypertension (ILD-PHTN). Methods A total of 32 patients (21 had ILD alone; 11 had ILD-PHTN) underwent CPET performed per American Thoracic Society protocol on a manually-braked bicycle. HRR1 was defined as the difference between peak HR and HR after one minute post-exercise. The utilization of HR reserve recovery at peak exercise was expressed as Chronotropic Response Index (CRI) and was calculated as (peak HR-resting HR)/(220-age-resting HR). CI was defined by failure to reach 85% of the age-predicted maximum heart rate (APMHR = 200-Age) and CRI<0.80 (80%). Results VO2max was lower in patients with ILD-PHTN compared to ILD alone (14.15± 5.00 vs. 18.11± 4.48, p<0.05). Mean CRI (0.468± 0.179 versus 0.691± 0.210, p<0.05) and HRR1 (10± 7 versus 18± 9, p<0.05) were lower in patients with ILD-PHTN compared to ILD alone. Twenty out of a total of 32 patients (62.5%) met the criteria for CI. In the ILD group, 10 out of 21 patients (47.62%) and in the ILD-PHTN group 10 of 11 patients (90.90%) had CI. Conclusion Chronotropic Incompetence and abnormal heart rate recovery at one minute post-exercise are notable in patients with ILD and are more severe in patients with ILD-PHTN. These findings may contribute to our understanding of dyspnea due to these conditions.

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.

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.

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.

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.

The Usefulness of Chest CT Imaging in Patients With Suspected or Diagnosed COVID-19: A Review of Literature.

The COVID-19 pandemic has had devastating medical and economic consequences globally. The severity of COVID-19 is related, in a large measure, to the extent of pulmonary involvement. The role of chest CT imaging in the management of patients with COVID-19 has evolved since the onset of the pandemic. Specifically, the description of CT scan findings, use of chest CT imaging in various acute and subacute settings, and its usefulness in predicting chronic disease have been defined better. We performed a review of published data on CT scans in patients with COVID-19. A summary of the range of imaging findings, from typical to less common abnormalities, is provided. Familiarity with these findings may facilitate the diagnosis and management of this disease. A comparison of sensitivity and specificity of chest CT imaging with reverse-transcriptase polymerase chain reaction testing highlights the potential role of CT imaging in difficult-to-diagnose cases of COVID-19. The usefulness of CT imaging to assess prognosis, to guide management, and to identify acute pulmonary complications associated with SARS-CoV-2 infection is highlighted. Beyond the acute stage, it is important for clinicians to recognize pulmonary parenchymal abnormalities, progressive fibrotic lung disease, and vascular changes that may be responsible for persistent respiratory symptoms. A large collection of multi-institutional images were included to elucidate the CT scan findings described.

Pulmonary artery aneurysm: a review.

Pulmonary artery aneurysm is a rare but important entity in the spectrum of pulmonary vascular diseases. The etiologies can be varied and patients can present with non-specific symptoms with the diagnosis being incidental. There is limited consensus regarding the diagnostic criteria and follow-up imaging for patients diagnosed with this entity. Further the management strategies can be variable depending upon underlying disease, etiology, center dependent expertise, and resources available. We review the etiologies, epidemiology, classification, clinical manifestations, and imaging features of pulmonary artery aneurysm. We also review the current management strategies and suggest an algorithmic approach to these patients.

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.

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.

Pulmonary arterial hypertension in the elderly: Clinical perspectives.

Pulmonary hypertension (PH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance, which eventually leads to right ventric-ular failure and death. Pulmonary arterial hypertension (PAH) (World Health Organization Group I), a subset of PH, and may be idiopathic in nature or associated with other systemic conditions and is thought to most commonly effect women, the majority of whom are of childbearing age. However, PAH in the elderly population is being increasingly diagnosed creating clinical considerations that had once not been considered. Often in an elderly population the diagnosis of PAH may be delayed due to chronic comorbid conditions such as coronary artery disease or other dyspneic conditions. Though survival and clinical outcomes have improved, the elderly population continues to have disproportionately lower survival rates. High clinical suspicion of PAH warrants a complete diagnostic workup with right heart catheterization. Upon diagnosis, PAH specific therapy should be initiated with possible drug interactions in mind. Adjuvant pulmonary rehabilitation should be considered as a conservative measure with definitive results. Finally, psychosomatic aspects of the disease should also be considered in elderly populations.

Acute Opiate Overdose: An Update on Management Strategies in Emergency Department and Critical Care Unit.

BACKGROUND: Opioids are natural, semisynthetic, or synthetic substances that act on opioid receptors in the central nervous system. Clinically, they are prescribed for pain management. Opioid overdose (OOD) occurs when the central nervous system and respiratory drive are suppressed because of excessive consumption of the drug. Symptoms of OOD include drowsiness, slow breathing, pinpoint pupils, cyanosis, loss of consciousness, and death. Due to their addictive potential and easy accessibility opioid addiction is a growing problem worldwide. Emergency medical services and the emergency department often perform initial management of OOD. Thereafter, some patients require intensive care management because of respiratory failure, metabolic encephalopathy, acute kidney injury, and other organ failure. AREAS OF UNCERTAINTY: We sought to review the literature and present the most up-to-date treatment strategies of patients with acute OOD requiring critical care management. DATA SOURCES: A PubMed search was conducted to review all articles between 1950 and 2017 and the relevant articles were cited. RESULTS & CONCLUSIONS: Worldwide, approximately 69,000 people die of OOD each year, and approximately 15 million people have opioid addiction. In the United States, death from OOD has increased almost 5-fold from 2001 to 2013. OOD leading to intensive care unit admission has increased by 50% from 2009 to 2015. At the same time, the mortality associated with these admissions has doubled. The management strategies include airway management, use of reversal agents, assessing and treating coingestions and associated complications, treatment of opioid withdrawal with alpha-agonists, and psychosocial support to help with opiate addiction and withdrawal. This warrants awareness among clinicians regarding the adverse effects associated with opioid use, management strategies, and calls for a multidisciplinary approach to treating these patients.

Successful pulmonary thromboendarterectomy in a patient with sickle cell disease and associated resolution of a leg ulcer.

Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH. We present such a case of a patient with SCD diagnosed with severe PH who was found to have CTEPH and successfully underwent a thromboendarterectomy with resolution of his symptoms such as reduction of his oxygen requirements and healing of chronic leg ulcer. This case illustrates the importance of screening patients with SCD and elevated pulmonary artery pressures for CTEPH as this would offer possible treatment options such as pulmonary thromboendarterectomy and/or riociguat in this subset of 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.

Diagnosing and managing scleroderma-related pulmonary arterial hypertension.

Scleroderma is an uncommon autoimmune disease of unknown cause that may affect any organ system in the body. Patients with scleroderma are prone to developing pulmonary complications, including pulmonary arterial hypertension (PAH), that are the leading cause of death in this population. This article describes scleroderma-related PAH and its diagnosis and management.

Pulmonary hypertension associated with antiphospholipid antibody: Call for a screening tool?

Antiphospholipid (aPL) antibodies are antibodies specific for anionic phospholipids. They are immunoglobulins that attack phospholipids, phospholipid-binding proteins, or phospholipid-protein complexes and are detected in anticardiolipin and lupus anticoagulant assays. aPL antibodies are often associated with antiphospholipid syndrome (APS) which can be idiopathic or from secondary causes such as systemic lupus erythematosus (SLE), infection or drugs. They have also been shown to be associated with Pulmonary Hypertension. We conducted a review of the literature that included all articles on PubMed with keywords 'antiphospholipid antibody' and 'pulmonary hypertension' between January 1980 and July 2017 and identified 217 articles. A total of 47 articles were found to be relevant to the topic and included as references. We ascertained that aPL antibodies have been implicated in the development of both idiopathic pulmonary arterial hypertension (PAH) and PAH associated with connective tissue disease (CTD). aPL antibodies were also noted to be associated with left-sided valvular heart disease that can lead to pulmonary venous hypertension (PVH). Patients with anitiphospholipid antibody syndrome (Diagnostic criteria incudes +aPL antibodies) were noted to have a high risk of developing chronic thromboembolic pulmonary hypertension (CTEPH). A recent study also found a positive association of aPL antibodies with ILD and PH in patients with systemic sclerosis. While association between autoimmune thyroid disease and PH (Group V PH), and autoimmune thyroid disease and aPL antibodies is established, no studies linked these three phenomena together. Thus, aPL antibodies had an association with all WHO groups of Pulmonary hypertension (PH). In this review article, we study the association and discuss the need for screening for PH in patients with positive aPL antibodies.

The multiple dimensions of Platypnea-Orthodeoxia syndrome: A review.

Platypnea-Orthodeoxia syndrome (POS) is a rare clinical entity characterized by dyspnea and arterial desaturation while in the upright position. The various pathophysiologic mechanisms leading to POS has puzzled clinicians for years. The hypoxia in POS has been attributed to the mixing of the deoxygenated venous blood with the oxygenated arterial blood via a shunt. The primary mechanisms of POS in these patients can be broadly classified based on intracardiac abnormalities, extracardiac abnormalities and miscellaneous etiologies. A Patent Foramen Ovale (PFO) was the most common reported site of an intracardiac shunt. In addition to PFO, intracardiac shunt leading to POS has been reported from either an Atrial Septal Defect (ASD) or an Atrial Septal Aneurysm (ASA). Most patients with an intracardiac shunt also demonstrated a secondary anatomic or a functional defect. Extracardiac causes of POS included intra-pulmonary arteriovenous malformations and lung parenchymal diseases. A systematic evaluation is necessary to identify the underlying cause and institute an appropriate intervention. We conducted a review of literature and reviewed 239 cases of POS. In this article, we review the etiology and pathophysiology of POS and also summarize the diagnostic algorithms and treatment modalities available for early diagnosis and prompt treatment of patients presenting with symptoms of platypnea and/or orthodeoxia.

Pulmonary manifestations of urothelial carcinoma of the bladder.

Urothelial carcinoma (Transitional cell carcinoma) of the bladder is the pre-dominant histological type of bladder cancer in the United States and Europe. Patients with bladder cancer usually present with painless hematuria. The diagnosis is often delayed, as the symptoms are similar to various other benign conditions such as urinary tract infection, prostatitis or renal calculi. In some patients, the metastatic lesions will cause the initial presenting symptoms. We conducted a MedLine/PubMED search identifying all relevant articles with "pulmonary manifestations", "urothelial bladder cancer", "manifestations of bladder cancer" or a combination of these terms in the title. The pulmonary manifestations of urothelial carcinoma of the bladder include metastatic disease including cavitary lesions, endobronchial, pleural, or lymph node metastasis pleural effusion and chylothorax. Pulmonary embolism and tumor embolism is another manifestation of this cancer. Intravesical Bacillus Calmette-Gurin therapy for bladder cancer has been associated with a range of adverse effects including the systemic spread of Bacilli Calmette-Guérin immunotherapy affecting the lungs. Other drugs used to treat bladder cancer can be associated with drug-related pneumonitis. Other rare manifestations include a sarcoid like reaction and systemic granulomatous disease to Bacilli Calmette-Guérin therapy. In this review we discuss the various pulmonary manifestations of urothelial carcinoma of the bladder. A high index of suspicion with these presentations can lead to an early diagnosis and assist in instituting an appropriate intervention.