Joseph A. Golish

Frequency and Impact of Pulmonary Hypertension in Patients With Obstructive Sleep Apnea Syndrome

The correlates and consequences of pulmonary hypertension (PH) associated with obstructive sleep apnea (OSA) are poorly understood. Patients undergoing pulmonary artery catheterization within 6 months of an overnight polysomnography showing OSA were included in the present analysis. A total of 83 patients with complete data were analyzed (no PH, n = 25 [30%]; PH, 58 [70%]; of these, 18 had a pulmonary capillary wedge pressure of <15 mm Hg). No significant differences were observed between the PH and no PH groups regarding age or apnea-hypopnea index. The correlates of PH were elevated right ventricular systolic pressure (p <0.001), body mass index (p = 0.026), female gender (p = 0.01), nocturnal desaturation (82% vs 18%), and forced vital capacity <70% (p = 0.04) on univariate analysis and female gender (p = 0.03), age <49 years (p = 0.02), body mass index of > or =26 kg/m(2) (p = 0.08), and right ventricular systolic pressure of > or =30 mm Hg (p <0.001) on multivariate analysis. Patients with PH had a lower 6-minute walk distance (285.5 +/- 122 m vs 343 +/- 213 m, p = 0.4). The survival rate at 1, 4, and 8 years for patients with PH was 93%, 75%, and 43% compared to 100%, 90%, and 76% for patients without PH, respectively. Patients with severe PH (n = 27; 33%) had more nocturnal desaturation (p = 0.045), worse pulmonary hemodynamics, and greater mortality (37%) than the groups with mild or moderate PH (16%) or no PH (16%). In conclusion, our results have shown that, although generally mild to moderate, severe PH can occur in patients with OSA. Female gender, younger age, obesity, and nocturnal desaturation were associated with PH. PH can cause functional limitations and increased mortality in patients with OSA.

Sleep-Related Breathing Disorders in Patients with Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic and usually fatal lung disease of unknown etiology. The aim of this study was to describe clinical and polysomnographic features of sleep-related breathing disorders (SRBD) and to identify predictors of obstructive sleep apnea (OSA) in IPF patients. Eight hundred fifty-seven patients with IPF were admitted to the Cleveland Clinic from 2001 to 2005. An all-night polysomnogram (PSG) was performed in 18 of them to investigate complaints suggestive of sleep-disordered breathing. OSA was confirmed in 11 of the 18 IPF patients with complaints suggestive of sleep apnea, while the remain 7 patients had a diagnosis of primary snoring or upper airway resistance syndrome (UARS). All patients showed a reduction in sleep efficiency, REM sleep, and slow wave sleep. The apnea-hypopnea index (AHI) was positively correlated with body mass index (p < 0.0001, r = 0.80). The REM AHI and overall AHI were negatively correlated with FEV1 (p = 0.008, r = −0.59 and p = 0.04, r = −0.49, respectively) and FVC percentages (p = 0.03, r = −0.50 and p = 0.08, r = −0.42, respectively). Our study is the first describing SRBD in IPF patients. An increased BMI and a significant impairment in pulmonary function testing may be predictors of OSA in this population. In the absence of effective treatments for IPF, the diagnosis and treatment of comorbid SRBD may lead to improvements in quality of life.

Health-related quality of life in patients with obstructive sleep apnoea and chronic obstructive pulmonary disease (overlap syndrome).

The association of obstructive sleep apnoea (OSA) and chronic obstructive pulmonary disease (COPD) is not rare as COPD and OSA are both frequent diseases. The aim of this study was to determine the effect of OSA on quality of life (QOL) in patients with overlap syndrome (OVS). Thirty subjects with OVS and 15 control subjects participated. The St Georges Respiratory Questionnaire (SGRQ) was used to determine QOL. The control group included subjects with COPD and no evidence of OSA by overnight polysomnography. All subjects were habitual snorers with normal Epworth Sleepiness Scale scores. Significant differences were found between the groups for the total score and each of the three components of the SGRQ suggesting worse QOL in OVS patients (symptoms 54.9 ± 18.9 vs. 38.2 ± 19.3, p = 0.008; activity 59.2 ± 16.2 vs. 44.4 ± 11.3, p = 0.003; impacts 35.2 ± 23 vs. 20.8 ± 8.7, p = 0.025 and total 45.7 ± 17.7 vs. 30.9 ± 8.7, p = 0.004 in OVS patients and control group, respectively). Obstructive sleep apnoea has a major impact on QOL in patients with OVS and can exist in COPD patients with habitual snoring even in the absence of daytime sleepiness. Further studies are needed to determine the impact of OSA treatment on QOL and morbidity in this population.

Pulmonary Artery Agenesis Presenting as Massive Hemoptysis — A Case Report

Massive hemoptysis is the expectoration of approximately 600 ml of blood in twenty-four hours. Major causes of massive hemoptysis are tuberculosis, bronchiectasis, pulmonary neoplasm, fungus ball, bronchial adenomas, lung abscess, intrabronchial rupture of an aortic aneurysm, cystic fibrosis, pulmonary infarction, and pulmonary trauma. Other, less common causes include Good-pastures syndrome, broncholiths, pulmonary varix, A-V malformation, and bleeding disorders. Agenesis of the pulmonary artery usually occurs in association with congenital cardiac anomalies, and isolated unilateral absence of the pulmonary artery is uncommon. About 10% of the patients with pulmonary artery agenesis develop inconsequential hemoptysis, but massive hemoptysis is a very rare complication of this anomaly. The following is a case report of a twenty-nine-year-old man with agenesis of the left pulmonary artery, who presented with massive hemoptysis requiring embolization and, eventually, pneumonectomy.

Prevalence and characteristics of restless legs syndrome in patients with pulmonary hypertension.

BACKGROUND Patients with pulmonary hypertension (PH) have an increased prevalence of risk factors for restless legs syndrome (RLS). We performed a cross-sectional study to determine the prevalence and characteristics of RLS in this population. METHODS Patients filled out two questionnaires during a visit: (1) a diagnostic tool for RLS, based on the core clinical features; and (2) a 10-question rating scale used to assess severity. Data were obtained by medical record review with regard to demographics, characteristics of PH and known RLS risk factors. RESULTS Restless legs syndrome was found in 43.6% (24 of 55) (mean age +/- SD: 49 +/- 14 years; 41 women, 14 men) of patients and 54% of these had moderate or severe RLS. Patients with RLS were younger but gender differences were not appreciated. Presence of RLS did not correlate with measures of PH severity; however, patients with RLS were more likely to have a better 6-minute walk distance (p = 0.015) and lower BNP level (p = 0.07) and less likely to be WHO Class IV or require oxygen during the 6-minute walk test. Patients with a history of hypothyroidism (67%; p = 0.04) and those on opioids for relief of leg pain (69%) were more likely to have RLS. CONCLUSIONS Patients with PH had a very high prevalence of RLS and most had moderate or severe symptoms. RLS was more common in more active patients and those who were hypothyroid or on opioids for relief of leg pain. Patients with PH should be screened for RLS because good treatment options are available.

An arrhythmia detector and heart rate estimator for overnight polysomnography studies

We present an algorithm for automatic on-line analysis of the ECG channel acquired during overnight polysomnography (PSG) studies. The system is independent of ECG morphology, requires no manual initialization, and operates automatically throughout the night. It highlights likely occurrences of arrhythmias and intervals of bad signal quality while outputting a continual estimate of heart rate. Algorithm performance is validated against standard ECG databases and PSG data. Results demonstrate a minimal false negative rate and a low false positive rate for arrhythmia detection, and robustness over a wide range of noise contamination.

Treatment of bronchospastic disorders in the 1990s : what does the future hold ?

The management of asthma is a common clinical problem for not only the pulmonologist, but the internist and paediatrician as well. Studies estimate that asthma may effect as many as 5% of individuals in Western societies (Fleming & Crombie 1987). A many faceted condition, it ranges in severity from a mild intermittent affliction to a disabling, sometimes life-threatening disease. Despite enormous expenditures on research and treatment, asthma continues to be a major cause of morbidity and mortality. Indeed, the incidence of asthma-related mortality may actually be increasing (Benatar 1986). Treatment of bronchospastic disorders follows close on the heels of new developments in our understanding of the pathogenesis and biochemistry of the disorder itself. The clinician is faced with the dazzling array of agents. These range from improved versions of older drugs to radically new classes of agents based on a new, more sophisticated understanding of the pathophysiology of bronchospastic disorders. One convenient way of viewing new pharmacological modalities is as a spectrum. We may begin with new formulations and delivery systems of previously available drugs, progressing through new applications of agents not traditionally used in asthma therapy, eventually arriving at entirely new classes of drugs the development of which comes about through research of the physiology of asthma. 1. Existing Asthma Therapy

Comparison of Epworth Sleepiness Scale scores by patients with obstructive sleep apnea and their bed partners.

OBJECTIVES In some patients with obstructive sleep apnea (OSA), Epworth Sleepiness Scale scores (ESS) do not reflect the severity of disease. In many cases, bed partners (BPs) report more severe hypersomnia on the part of the patient than the patient him/herself. The purpose of this study was to assess the agreement between patients and BPs on ESS scores and to compare patients and BPs on the correlation between ESS and the severity of OSA. METHODS ESS scores were estimated by patients and their BPs and their scores were compared. The correlation between the ESS and the apnea-hypopnea index, arousal index, minimum oxygen desaturation, and body mass index of the patient was estimated and compared between the patient and BP. RESULTS ESS scores of the patient and BP were similar in their correlation with polysomnographic variables. BPs tended to give slightly higher ESS scores than the patient. CONCLUSIONS ESS scores as estimated by BPs of patients with OSA did not correlate more positively with polysomnographic variables of OSA severity than scores estimated by affected patients.

Clinical management of asthma in the 1990s : Current therapy and new directions

SummaryAsthma is a serious global health problem affecting nearly 100 million people worldwide. Its rising prevalence and associated morbidity and mortality are of increasing concern. Traditionally, symptomatic control of bronchoconstriction with β2 agonists and theophylline has been the mainstay of therapy. However, during recent years, inflammation has been recognised as the predominant cause of reversible airway obstruction and airway hyperreactivity. As a result, the emphasis in treatment has shifted to the early use of inhaled corticosteroids to control airway inflammation. β2 agonists are best used on an as-needed basis for the relief of acute bronchoconstriction and for the prevention of exercise-induced asthma. Sustained release theophylline or an inhaled long-acting β2 agonist may effectively control nocturnal symptoms. Preliminary studies involving agents active in the 5-lipoxygenase pathway as preventive therapy are encouraging. Further studies are needed to define their role in the management of asthma.

The immotile cilia syndrome: explanation for many a clinical mystery.

With the advent of electron microscopy, ciliary abnormalities could be detected, analyzed, and correlated with clinical problems. Kartageners syndrome, identified over 40 years ago as a triad of sinusitis, bronchiectasis, and situs inversus, was found to actually be a subset of a broader category of diseases: the immotile cilia syndrome. Two other subsets involving multiple organ systems have also been recognized.