Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis

Introduction. Pulmonary Langerhans cell granulomatosis is a rare disease with a variable course. In pulmonary Langerhans cell granulomatosis pulmonary hypertension is frequent and has an independent prognostic impact. A vasculopathy which ist not related to ventilatory disturbance and fibrosis has been identified. An arteriopathy and even a venulopathy have been described. Due to this possible venulopathy vasodilators carry a significant risk for pulmonary congestion and edema. No drugs have been approved until now. Case Presentation. One female with PLCG developed severe PH four years after primary diagnosis of pulmonary Langerhans cell granulomatosis. Retrospective analysis of lung biopsies revealed an arterial vasculopathy at the time of primary diagnosis without clinical signs of PH at this time. Sildenafil led to a sustained improvement of hemodynamic features and exercise capacity. Conclusion. This paper underlines that patients with PLCG with an arterial vasculopathy-related PH might improve under sildenafil. Further trials addressing treatment of PH and vasculopathy are needed

A symptom-related monitoring program following pulmonary embolism for the early detection of CTEPH: a prospective observational registry study

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a long-term complication following an acute pulmonary embolism (PE). It is frequently diagnosed at advanced stages which is concerning as delayed treatment has important implications for favourable clinical outcome. Performing a follow-up examination of patients diagnosed with acute PE regardless of persisting symptoms and using all available technical procedures would be both cost-intensive and possibly ineffective. Focusing diagnostic procedures therefore on only symptomatic patients may be a practical approach for detecting relevant CTEPH. This study aimed to evaluate if a follow-up program for patients with acute PE based on telephone monitoring of symptoms and further examination of only symptomatic patients could detect CTEPH. In addition, we investigated the role of cardiopulmonary exercise testing (CPET) as a diagnostic tool. Methods In a prospective cohort study all consecutive patients with newly diagnosed PE (n=170, 76 males, 94 females within 26 months) were recruited according to the inclusion and exclusion criteria. Patients were contacted via telephone and asked to answer standardized questions relating to symptoms. At the time of the final analysis 130 patients had been contacted. Symptomatic patients underwent a structured evaluation with echocardiography, CPET and complete work-up for CTEPH. Results 37.7%, 25.5% and 29.3% of the patients reported symptoms after three, six, and twelve months respectively. Subsequent clinical evaluation of these symptomatic patients saw 20.4%, 11.5% and 18.8% of patients at the respective three, six and twelve months time points having an echocardiography suggesting pulmonary hypertension (PH). CTEPH with pathological imaging and a mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg at rest was confirmed in eight subjects. Three subjects with mismatch perfusion defects showed an exercise induced increase of PAP without increasing pulmonary artery occlusion pressure (PAOP). Two subjects with pulmonary hypertension at rest and one with an exercise induced increase of mPAP with normal PAOP showed perfusion defects without echocardiographic signs of PH but a suspicious CPET. Conclusion A follow-up program based on telephone monitoring of symptoms and further structured evaluation of symptomatic subjects can detect patients with CTEPH. CPET may serve as a complementary diagnostic tool

Pulmonary and Cardiac Function in Asymptomatic Obese Subjects and Changes following a Structured Weight Reduction Program: A Prospective Observational Study

Background The prevalence of obesity is rising. Obesity can lead to cardiovascular and ventilatory complications through multiple mechanisms. Cardiac and pulmonary function in asymptomatic subjects and the effect of structured dietary programs on cardiac and pulmonary function is unclear. Objective To determine lung and cardiac function in asymptomatic obese adults and to evaluate whether weight loss positively affects functional parameters. Methods We prospectively evaluated bodyplethysmographic and echocardiographic data in asymptomatic subjects undergoing a structured one-year weight reduction program. Results 74 subjects (32 male, 42 female; mean age 42±12 years) with an average BMI 42.5±7.9, body weight 123.7±24.9 kg were enrolled. Body weight correlated negatively with vital capacity (R = −0.42, p<0.001), FEV1 (R = −0.497, p<0.001) and positively with P 0.1 (R = 0.32, p = 0.02) and myocardial mass (R = 0.419, p = 0.002). After 4 months the study subjects had significantly reduced their body weight (−26.0±11.8 kg) and BMI (−8.9±3.8) associated with a significant improvement of lung function (absolute changes: vital capacity +5.5±7.5% pred., p<0.001; FEV1+9.8±8.3% pred., p<0.001, ITGV+16.4±16.0% pred., p<0.001, SR tot −17.4±41.5% pred., p<0.01). Moreover, P0.1/Pimax decreased to 47.7% (p<0.01) indicating a decreased respiratory load. The change of FEV1 correlated significantly with the change of body weight (R = −0.31, p = 0.03). Echocardiography demonstrated reduced myocardial wall thickness (−0.08±0.2 cm, p = 0.02) and improved left ventricular myocardial performance index (−0.16±0.35, p = 0.02). Mitral annular plane systolic excursion (+0.14, p = 0.03) and pulmonary outflow acceleration time (AT +26.65±41.3 ms, p = 0.001) increased. Conclusion Even in asymptomatic individuals obesity is associated with abnormalities in pulmonary and cardiac function and increased myocardial mass. All the abnormalities can be reversed by a weight reduction program

COPD: Rethinking Patient Management - How to Approach a Challenging Patient Group Successfully

In comparison to other chronically ill people, patients suffering from chronic obstructive pulmonary disease (COPD) have many additional difficulties to face and conquer. Due to the contribution of avoidable causes of their illness (smokers' lung), society holds people with COPD responsible for their disease, which in return often leads to stigmatization and social isolation. In addition, COPD patients commonly belong to a less privileged social class, own a low socioeconomic status, and lower education. Their physical symptoms are easily observable and-by employing moderate adherence - treatable. Nonetheless, the influence of COPD on a patient's psyche often plays an overly prominent role during therapy. There is only half a patient laying on the examination table, a revelation that sums up the current state of COPD research and the result of the expert meeting Luftschlosser (castles in the clouds), which took place in spring 2018. Within the limits of the meeting, participants identified practically applicable approaches aiming to enhance the patient management of this challenging patient group. These considerations are supposed to support healthcare professionals in their daily work and aim to improve the therapy as well as the outcome for COPD patients. (C) 2019 S. Karger AG, Base

Correlations of body weight with pulmonary function.

<p>VC = vital capacity. FEV1 = forced expired volume at one second. MEF 25 = Mean expiratory flow 25. P 0.1 = mouth occlusion pressure at 0.1 second.</p

Correlations of changes of pulmonary function with changes of body weight and changes of body fat and change of FEV1/VC from baseline to follow-up.

<p>a–c: Correlations of changes of pulmonary function with changes of body weight and changes of body fat (a–c) d: Change of FEV1/VC (%) ratio from baseline to follow-up (d), (data as mean ± SD), p = 0.006. VC = vital capacity. FEV1 = forced expired volume at one second.</p

Correlations of body weight with cardiac function and myocardial wall thickness.

<p>LVPWd = diastolic left ventricular posterior wall diameter; IVSd = diastolic interventricular septal wall thickness; LIMP = left myocardial performance index; RVOT AccT = acceleration time of flow in the right ventricular outflow tract.</p