Heinrich Matthys

The Severe Respiratory Insufficiency (SRI) Questionnaire A specific measure of health-related quality of life in patients receiving home mechanical ventilation

BACKGROUND AND OBJECTIVE The objective of this study was to develop a specific instrument for measuring health-related quality of life (HRQL) in patients receiving home mechanical ventilation (HMV). METHODS The Severe Respiratory Insufficiency (SRI) Questionnaire was developed and tested for its psychometric properties following a multicentric clinical trial including 226 patients receiving HMV (mean age 57.3+/-14.0 years). RESULTS Forty-nine items passed the selection process and were allocated to seven subscales: Respiratory Complaints, Physical Functioning, Attendant Symptoms and Sleep, Social Relationships, Anxiety, Psychologic Well-Being, and Social Functioning. Cronbachs alpha was >0.7 in all subscales and >0.8 in four subscales indicating high internal consistency reliability. Construct validity was confirmed by factor analysis, indicating one summary scale that accounts for 59.8% of the variance. Concurrent validity was confirmed by correlating subscales of the SRI and the SF-36 (0.21<r<0.79). Item-scale correlations revealed a high item discriminant validity. In addition, different diagnostic groups could be discriminated by the SRI. Here, the overall best HRQL was measured in the following order: patients with kyphoscoliosis, miscellaneous disorders, neuromuscular diseases, post-tuberculosis, and chronic obstructive pulmonary disease (P<.05). CONCLUSION The SRI is a new multidimensional instrument with high psychometric properties designed to measure specific HRQL in patients with SRI receiving HMV.

Evaluation of health-related quality of life using the MOS 36-Item Short-Form Health Status Survey in patients receiving noninvasive positive pressure ventilation

ObjectiveTo collect benchmark data on the MOS 36-Item Short-Form Health Status Survey (SF-36) in patients receiving noninvasive positive pressure ventilation and to examine whether health-related quality of life is influenced by the underlying disease or by physical parameters.Design and settingMulticentric clinical cross-sectional trial in four general wards specialized in noninvasive positive pressure ventilation.Patients and participants226 patients (78 chronic obstructive pulmonary disease, 57 kyphoscoliosis, 20 posttuberculosis sequelae, 17 Duchenne muscular dystrophy, 13 polyneuropathy, 13 myopathy, 6 amyotrophic lateral sclerosis, 12 obesity-hypoventilation syndrome, 4 poliomyelitis sequelae, 3 phrenic nerve lesion, 3 central hypoventilation syndrome) who used noninvasive positive pressure ventilation for home mechanical ventilation.Measurements and resultsHealth-related quality of life as assessed by the SF-36 was lower than in the general population. Overall the Physical Component Summary (PCS) was significantly lower than the general population norm; the Mental Component Summary (MCS) was also reduced but less markedly. Patients with chronic obstructive pulmonary disease were more impaired in MCS than those with kyphoscoliosis. PCS was significantly associated with age. Gender, lung function, and arterial blood gas values were not significant predictors of health-related quality of life.ConclusionsBenchmark SF-36 data in patients receiving noninvasive positive pressure ventilation are given. Although physical health is significantly impaired in these patients, this does not necessarily lead to mental limitation, and mental health is influenced by the underlying disease, but not by physical parameters.

Effect of Isosorbide Dinitrate on Hemodynamics and Respiration of Patients with Coronary Artery Disease and of Patients with Chronic Cor pulmonale

The influence of isosorbide dinitrate on hemodynamics and gas exchange was studied in 10 patients with coronary artery disease and 10 patients with chronic cor pulmonale. A significant decrease in total pulmonary vascular resistance was found in both groups. This effect seemed mainly to be due to improved conditions for the left ventricle in the patients with coronary heart disease, while it could be explained by vasodilatation of the precapillary pulmonary resistive vessels in the patients with cor pulmonale. In the latter group, there was also a significant increase in the alveolo-arterial PO-2 gradient.

Long-term survival of a patient with congenital central hypoventilation syndrome despite the lack of continuous ventilatory support

Untreated idiopathic congenital central hypoventilation syndrome (CCHS) is thought to cause infant death within 1–2 months. Here we present an adult patient with CCHS who survived without continuous ventilatory support, despite hypoventilation from early childhood onward. The diagnosis was confirmed at the age of 22 years, when the patient presented with hypoventilation during the night (PaCO2 60 mm Hg, PaO2 56 mm Hg, pH 7.32 HCO–3 30 mmol/l) but hyperventilation when awake (PaCO2 26 mm Hg, PaO2 81 mm Hg, pH 7.56, HCO–3 23 mmol/l). The maximal hematocrit was 77%. Despite mental retardation, noninvasive positive pressure ventilation (NPPV) could be successfully established. NPPV-supported ventilation during the night (PaCO2 36, PaO2 84 mm Hg, pH 7.47, HCO–3 25 mmol/l) reduced hematocrit values (40.6 to 36.8%) over a period of 4 years. In conclusion, long-term survival with CCHS is possible without continuous ventilatory support. Spontaneous improvement of hypoventilation during sleep throughout childhood is possible and hyperventilation during wakefulness may occur in patients with CCHS. CCHS can be managed with NPPV despite mental retardation, even over a long-term period.

Is There a Need for Another Inhalative β2-Agonist besides Formoterol in Patients with Asthma?

Formoterol can substitute the rapid- and short-acting β2-agonists as well as the slow- and long-acting salmeterol. Therefore formoterol in a fixed combination with an inhalant steroid reduces the aerosol devices necessary for asthma control to only one, to be used for regular ‘controller’ and, as needed, ‘rescue therapy’. The side effect profile of formoterol is comparable to the short-acting β2-agonists which makes the combination with a topically active glucocorticoid applicable in patients of any asthma severity as long as they are able to perform an inspiratory vital capacity maneuver

Problems in Evaluating the Effect of Secretolytic Agents on the Mucociliary System by Means of Radioactive Particles

In a double-blind cross-over study the effect of two secretolytic agents on the mucociliary clearance was tested in 8 patients with mild chronic obstructive bronchitis. Clearance was assessed from the removal rate of previously inhaled sulfur colloid particles, tagged with 99Tcm. For the interpreatation of the results, obtained by this method, it is essential to take into account the pattern of particle deposition. For example there was a faster clearance under placebo compared to the secretolytic drugs because of a more central deposition. To overcome this problem different approaches were therefore tested. The following constellations proved to be useful in assessing the effect of secretolytic drugs: (1) change in deposition patter; (2) clearance rate, if no change in deposition takes place; (3) clearance rate from a peripheral area of the lung. An attempt to apply a simple three compartment model proved to be unpracticable probably becuase of the complexity of the mechanism involved. One of these mechanisms could be a reversal in mucus transport, observed at least in one patient, a finding which might be of pathophysiological relevance.

Weaning from mechanical ventilation by long-term nasal positive pressure ventilation in two patients with acute respiratory distress syndrome associated with pneumococcal sepsis

Only few data concerning weaning by nasal positive pressure ventilation (NPPV) are available, and successful weaning by using NPPV in patients with acute respiratory distress syndrome (ARDS) and severe complications has not yet been described. Two cases with ARDS and both preexisting thoracopulmonary disease (infundibulum abnormality and suspected COPD) and associated complications (recurrent sepsis, acute renal failure, need for lobectomy, severe malnutrition) could not be weaned by invasive ventilatory techniques. Both patients presented with rapid shallow breathing and PaCO2 values >60 mm Hg during intermittent trials of spontaneous breathing, although the primary pathology and associated complications had been resolved. Patients were successfully adapted on NPPV in a stepwise approach after 93 days and 67 days of invasive ventilation. In one patient withdrawal from NPPV was possible after 2 months. In the other patient the duration of daily ventilation could be significantly reduced from 18 to 6 h/day after 9 months on NPPV. Therefore, patients with ARDS who cannot be weaned by invasive ventilatory strategies might be removed successfully from invasive mechanical ventilation by using NPPV even when there are preexisting thoracopulmonary disease and major complications during invasive ventilation.

Die mucociliare Clearance der Lunge; untersucht mit radioaktiv markiertem Schwefelkolloid

The mucociliary system of the lung was studied in eight normal subjects and ten patients with chronic bronchitis, using sulfur colloids, labelled with 99mTechnetium. By recording the radioactive impulses over the lung with a scintillation camera and analysing the data with a computer, we were able to exclude extrapulmonary artefacts and to select central areas of the tracheobronchial tree. Time activity curves obtained over the central airways were interpreted by means of a model. In bronchitics the disappearance rate of the radioactive particles was found to depend upon the pattern of deposition and the degress of airway obstruction. Because of the good reproducibility in one and the same subject, the effect of drugs, influencing the mucociliary system, can be tested. We used a newly developed vagolytic substance which did not influence the mucociliary system and which reduced little the airway resistance in patients with chronic bronchitis at the administered dose.SummaryThe mucociliary system of the lung was studied in eight normal subjects and ten patients with chronic bronchitis, using sulfur colloids, labelled with99mTechnetium. By recording the radioactive impulses over the lung with a scintillation camera and analysing the data with a computer, we were able to exclude extrapulmonary artefacts and to select central areas of the tracheobronchial tree. Time activity curves obtained over the central airways were interpreted by means of a model. In bronchitics the disappearance rate of the radioactive particles was found to depend upon the pattern of deposition and the degree of airway obstruction. Because of the good reproducibility in one and the same subject, the effect of drugs, influencing the mucociliary system, can be tested. We used a newly developed vagolytic substance which did not influence the mucociliary system and which reduced little the airway resistance in patients with chronic bronchitis at the administered dose.ZusammenfassungDas Mucociliarsystem der Lunge von 8 Normalpersonen und 10 Patienten mit chronischer Bronchitis wurde mit Hilfe von radioaktiv markiertem99mTechnetium-Schwefelkolloid untersucht. Registrierung der vom Inhalt ausgesandten Impulse mit der Anger-Kamera und Auswertung durch den Computer ermöglichten die Ausblendung extrapulmonaler Störfaktoren und die Erfassung wichtiger Teilbereiche des Bronchialsystems. Der Verlauf von Zeit-Aktivitätskurven wurde über den zentralen Luftwegen an Hand eines Modells interpretiert. In der Patientengruppe ist die Clearance der Testsubstanz vom Depositionsmuster und vom Obstruktionsgrad abhängig. Die gute Reproduzierbarkeit der Eliminationskurve beim einzelnen eröffnet eine Methode, die Wirkung von Medikamenten auf das Mucociliarsystem zu untersuchen. Im Doppelblindversuch wurde ein neu entwickeltes Parasympathikolytikum getestet. Es zeigte in der verwandten Konzentration bei leichter antiobstruktiver Wirkung keinen signifikanten Einfiuß auf die mucociliare Klärfunktion.

[Pulmonary hypertension (author's transl)].

SummaryDefinitions of the different forms of pulmonary hypertension are given. The pathophysiological mechanisms are briefly reviewed. The total and regional blood flow distribution pattern measured by133Xenon are correlated with the mean pulmonary artery pressure in patients with Cor pulmonale of different etiology and degree. A comparison between clinical, radiological, ECG and haemodynamic findings at rest and during exercise is then made for patients with various degrees of pulmonary hypertension.ZusammenfassungEinleitend werden die verschiedenen Formen der pulmonalen Hypertonie erwähnt und definiert, sowie die pathophysiologischen Grundlagen kurz diskutiert. Typische Verteilungsmuster der regionalen und globalen Lungenperfusion gemessen mit133Xenon werden in Beziehung zum Grad der pulmonalen Hypertonie gebracht. Abschließend findet ein Vergleich der klinischen, radiologischen, elektrokardiographischen und hämodynamischen Diagnosemöglichkeiten bei verschiedenen Schweregraden der pulmonalen Hypertonie in Ruhe und unter ergometrischer Belastung statt.

Quantitative Measurement of Lung Volumes Using133 Xe

A steady-state technique for measurement of lung volumes, using 133Xe, a scintillation camera, and a data processing system, is described. Good agreement with the results, obtained by whole-body plethysmography was found except for patients with airway obstruction and/or trapped air. In this group, valuable physiological information about uneven ventilation, volume distribution and trapped air can be obtained, if one compares the results with the findings of whole-body plethysmography and single breath techniques