Exercise Pulmonary Hypertension

The clinical value of pulmonary hemodynamics during exercise has not been fully explored. In the last decade, several studies investigated the prognostic and diagnostic relevance of exercise hemodynamics and novel hemodynamic variables including the mean pulmonary arterial pressure (mPAP)/cardiac output (CO) slope and the pulmonary arterial wedge pressure (PAWP)/CO slope have been analyzed. These parameters describe the effects of pulmonary blood flow on the pulmonary pressure and were shown to be of prognostic relevance. In addition, they may also serve as tools to differentiate early forms of pulmonary vascular from left heart diseases. Right heart catheterization remains the gold standard to assess pulmonary hemodynamics both at rest and during exercise, while exercise echocardiography represents a promising non-invasive research tool. In this review, we provide an overview of the growing body of evidence on the clinical relevance of pulmonary hemodynamics during exercise and discuss its potential future role

Volatile Organic Compounds, Bacterial Airway Microbiome, Spirometry and Exercise Performance of Patients after Surgical Repair of Congenital Diaphragmatic Hernia

The aim of this study was to analyze the exhaled volatile organic compounds (VOCs) profile, airway microbiome, lung function and exercise performance in congenital diaphragmatic hernia (CDH) patients compared to healthy age and sex-matched controls. A total of nine patients (median age 9 years, range 6–13 years) treated for CDH were included. Exhaled VOCs were measured by GC–MS. Airway microbiome was determined from deep induced sputum by 16S rRNA gene sequencing. Patients underwent conventional spirometry and exhausting bicycle spiroergometry. The exhaled VOC profile showed significantly higher levels of cyclohexane and significantly lower levels of acetone and 2-methylbutane in CDH patients. Microbiome analysis revealed no significant differences for alpha-diversity, beta-diversity and LefSe analysis. CDH patients had significantly lower relative abundances of Pasteurellales and Pasteurellaceae. CDH patients exhibited a significantly reduced Tiffeneau Index. Spiroergometry showed no significant differences. This is the first study to report the VOCs profile and airway microbiome in patients with CDH. Elevations of cyclohexane observed in the CDH group have also been reported in cases of lung cancer and pneumonia. CDH patients had no signs of impaired physical performance capacity, fueling controversial reports in the literature

Pulmonale Hypertonie assoziiert mit Lungenerkrankungen

Nicht selten sind Lungenerkrankungen und Hypoventilationssyndrome mit einer pulmonalen Hypertonie (PH) assoziiert. In den meisten Fällen liegt eine nicht schwere PH vor. Diese ist hämodynamisch definiert durch einen pulmonalarteriellen Mitteldruck (PAPm) > 20 mmHg, einen pulmonalarteriellen Verschlussdruck (PAWP) ≤ 15 mmHg und einen pulmonal-vaskulären Widerstand von ≤ 5 Wood-Einheiten (WU). Sowohl die nicht schwere (PVR ≤ 5 WU) als auch deutlich ausgeprägter die schwere PH (PVR > 5 WU) sind prognostisch ungünstig. Bei Verdacht auf eine PH wird empfohlen, primär zu prüfen, ob Risikofaktoren für eine pulmonalarterielle Hypertonie (PAH, Gruppe 1 PH) oder eine chronisch thromboembolische pulmonale Hypertonie (CTEPH, Gruppe 4 PH) vorliegen. Falls Risikofaktoren vorliegen oder bei Lungenkranken der Verdacht auf eine schwere PH besteht, wird eine zeitnahe Vorstellung der Patient*innen in einer PH-Ambulanz empfohlen. Bei Patient*innen mit einer schweren PH assoziiert mit Lungenerkrankungen wird eine personalisierte, individuelle Therapie – möglichst im Rahmen von Therapiestudien – empfohlen. Aktuell sollte bei COPD-Patient*innen nur ein Therapieversuch erwogen werden, wenn die assoziierte PH schwergradig und ein „pulmonalvaskulärer“ Phänotyp (schwere präkapilläre PH, aber typischerweise nur milde bis moderate Atemwegsobstruktion, keine oder milde Hyperkapnie und DLCO < 45 % vom Soll) vorliegt. Bei schwerer PH assoziiert mit einer interstitiellen Lungenerkrankung können entsprechend individueller Abwägung Phosphodiesterase-5-Inhibitoren erwogen werden. Inhaliertes Treprostinil kommt bei diesen Patient*innen auch bei nicht schwerer PH in Betracht. Lung diseases and hypoventilation syndromes are often associated with pulmonary hypertension (PH). In most cases, PH is not severe. This is defined hemodynamically by a mean pulmonary arterial pressure (PAPm) > 20 mmHg, a pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance of ≤ 5 Wood units (WU). Both the non-severe (PVR ≤ 5 WU) and much more the severe PH (PVR > 5 WU) have an unfavorable prognosis. If PH is suspected, it is recommended to primarily check whether risk factors for pulmonary arterial hypertension (PAH, group 1 PH) or chronic thromboembolic pulmonary hypertension (CTEPH, group 4 PH) are present. If risk factors are present or there is a suspicion of severe PH in lung patients, it is recommended that the patient should be presented to a PH outpatient clinic promptly. For patients with severe PH associated with lung diseases, personalized, individual therapy is recommended – if possible within the framework of therapy studies. Currently, a therapy attempt with PH specific drugs should only be considered in COPD patients if the associated PH is severe and a “pulmonary vascular” phenotype (severe precapillary PH, but typically only mild to moderate airway obstruction, no or mild hypercapnia and DLCO < 45 % of predicted value) is present. In patients with severe PH associated with interstitial lung disease phosphodiesterase-5-inhibitors may be considered in individual cases. Inhaled treprostinil may be considered also in non-severe PH in this patient population