Flemming Larsen

OBJECTIVES: The Swedish Pulmonary Arterial Hypertension Register (SPAHR) is an open continuous register, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients from 2000 and onwards. We hereby launch the first data from SPAHR, defining baseline characteristics and survival of Swedish PAH and CTEPH patients. DESIGN: Incident PAH and CTEPH patients 2008-2014 from all seven Swedish PAH-centres were specifically reviewed. RESULTS: There were 457 PAH (median age: 67 years, 64% female) and 183 CTEPH (median age: 70 years, 50% female) patients, whereof 77 and 81%, respectively, were in functional class III-IV at diagnosis. Systemic hypertension, diabetes, ischaemic heart disease and atrial fibrillation were common comorbidities, particularly in those >65 years. One-, 3- and 5-year survival was 85%, 71% and 59% for PAH patients. Corresponding numbers for CTEPH patients with versus without pulmonary endarterectomy were 96%, 89% and 86% versus 91%, 75% and 69%, respectively. In 2014, the incidence of IPAH/HPAH, associated PAH and CTEPH was 5, 3 and 2 per million inhabitants and year, and the prevalence was 25, 24 and 19 per million inhabitants. CONCLUSION: The majority of the PAH and CTEPH patients were diagnosed at age >65 years, in functional class III-IV, and exhibiting several comorbidities. PAH survival in SPAHR was similar to other registers.

BACKGROUND: A high survival rate can be achieved in patients with severe acute respiratory distress syndrome (ARDS) using extracorporeal membrane oxygenation (ECMO). The technique and the costs are, however, debated and follow-up studies in survivors are few. The aim of this study was to evaluate long-term pulmonary health after ECMO and severe ARDS. METHODS: Twenty-one long-term survivors of severe ARDS and ECMO were studied in a follow-up program including high-resolution computed tomography (HRCT) of the lungs, extensive pulmonary function tests, pulmonary scintigraphy and the pulmonary disease-specific St George's Respiratory Questionnaire (SGRQ). RESULTS: The majority of patients had residual lung parenchymal changes on HRCT suggestive of fibrosis, but the extension of morphologic abnormalities was limited and without the typical anterior localization presumed to indicate ventilator-associated lung injury. Pulmonary function tests revealed good restitution with mean values in the lower normal range, while T(1/2) for outwash of inhaled isotope was abnormal in all patients consistent with subclinical obstructivity. Most patients had reduced health-related quality of life (HRQoL), according to the SGRQ, but were stating less respiratory symptoms than conventionally treated ARDS patients in previous studies. The majority were integrated in normal work. CONCLUSION: The majority of ECMO-treated ARDS patients have good physical and social functioning. However, lung parenchymal changes on HRCT suggestive of fibrosis and minor pulmonary function abnormalities remain common and can be detected more than 1 year after ECMO. Furthermore, most patients experience a reduction in HRQoL due to the pulmonary sequelae.

OBJECTIVE: To compare the effectiveness of exercise therapy aimed at restoring neuromuscular control mechanisms at the shoulder with other conservative interventions for the treatment of chronic shoulder pain with and without accompanying stiffness. DESIGN: Randomized control trial. PATIENTS: A total of 138 volunteers with unilateral shoulder pain of local mechanical origin. METHODS: Subjects were randomly allocated to receive exercise therapy aimed at restoring dynamic stabilizing mechanisms and muscle co-ordination at the shoulder; or subacromial corticosteroid injection; or a combination of physical modalities and range of motion exercises. Outcome measurements of pain intensity, functional impairment, active range of motion, isometric muscle force and self-assessed improvement were taken at baseline and after 5 weeks of treatment. RESULTS: The mean/median changes in all outcome measurements at 5 weeks indicated that subjects in each treatment group, improved significantly with no difference between the treatment groups. Comparison with an earlier no-treatment trial would suggest that this improvement is greater than that which could be expected by natural recovery. CONCLUSION: Exercise therapy aimed at restoring neuromuscular control, corticosteroid injection and multiple physical modalities and range of motion exercises are equally effective in the short-term treatment of shoulder pain, with exercise therapy and corticosteroid injection being less costly to administer.

OBJECTIVES: To elucidate whether cardiac magnetic resonance imaging (MRI) could be useful in disclosing structural changes in the myocardium in sarcoidosis patients and to relate echo-Doppler derived indices of left ventricular function to electrocardiogram (ECG) findings. DESIGN: The MRI was performed in 18 consecutive patients with sarcoidosis. Left ventricular ejection fraction (LVEF), i.e. systolic function, was estimated echocardiographically by Simpson's two-dimensional method (n = 16). Diastolic function was estimated by age-corrected Doppler-derived indices: isovolumetric relaxation time (IVRT), deceleration time (DT) and early filling/atrial contraction ratio (E/A ratio). RESULTS: Eleven patients had conduction defects or dysrhythmias (ECG+) whilst seven patients had a normal ECG (ECG-). In two patients, high signalling, contrast-enhanced, isolated regions, suggestive of deposits, were seen in the left ventricular myocardium on MRI. Both these patients had abnormal ECGs and signs of systolic and/or diastolic dysfunction on echocardiography. LVEF was subnormal in seven of 10 of the ECG+ patients and in two of six of the ECG-. Signs of diastolic dysfunction were found in 59% and 56% of the measurements in the ECG+ and ECG- patients, respectively. CONCLUSION: We conclude (i) that myocardial deposits on MRI in sarcoidosis patients have a high specificity for cardiac involvement but a rather low sensitivity; (ii) that a substantial proportion of sarcoidosis patients with abnormal ECGs have echocardiographic signs of systolic and/or diastolic dysfunction.