Definition, clinical classification and initial diagnosis of pulmonary hypertension: Updated recommendations from the Cologne Consensus Conference 2018

Abstract

In the summer of 2016, delegates from the German Society of Cardiology (DGK), the German Respiratory Society (DGP), and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to the definition, clinical classification and initial diagnosis of PH. While the European guidelines provide a detailed clinical classification and a structured approach for diagnostic testing, their application in routine care may be challenging, particularly given the changing phenotype of PH patients who are nowadays often elderly and may present with multiple potential causes of PH, as well as comorbid conditions. Specifically, the working group addresses the thoroughness of diagnostic testing, and the roles of echocardiography, exercise testing, and genetic testing in diagnosing PH. Furthermore, challenges in the diagnostic work-up of patients with various causes of PH including "PAH with comorbidities", CTEPH and coexisting conditions are highlighted, and a modified diagnostic algorithm is provided. The detailed results and recommendations of the working group on definition, clinical classification and initial diagnosis of PH, which were last updated in the spring of 2018, are summarized in this article.

Mildly increased pulmonary arterial pressure: a new disease entity or just a marker of poor prognosis?

“…As there are no published randomized controlled trials for the specific treatment of these subjects with PAH medications, currently no such treatment is generally recommended or approved. Close follow‐up and, if possible, inclusion into clinical trials has been suggested . In specific subgroups, such as symptomatic patients with systemic sclerosis, PAH therapy may be initiated on an individual basis.…”

Lung Disease in Antiphospholipid Syndrome

“…45 Pulmonary arterial hypertension (PAH) is a subgroup of PH, identified as class I. PAH is a primary disease of the blood vessels of the lungs: modifica-tions in the vessels structure and functionality are directly responsible of the elevation in pressure. 46 In healthy individuals, mPAP is around 14 mm Hg, with an upper limit of 20 mm Hg. The clinical significance of mPAP between 21 and 24 mm Hg is still uncertain; it might potentially identify subjects at risk of developing PH.…”

“…mPAP can be directly assessed exclusively by right heart catheterization (RHC), which thus is the gold standard for diagnosis. 46 Transthoracic echocardiography, which is performed as screening in all patients with a clinical suspicion of PH, provides an estimate of systolic PAP based on tricuspid regurgitation peak velocity. In addition, RHC allows differentiating precapillary and postcapillary PH taking into consideration hemodynamic parameters as mean pulmonary-capillary wedge pressure, left ventricular end-diastolic pressure, and pulmonary vascular resistance.…”

Herzinsuffizienz, COPD und Betablocker-Therapie

“…eine rechtsventrikuläre Dysfunktion vorliegt [46]. Koexistieren COPD und Linksherzinsuffizienz, so muss mit einer CpcPH gerechnet werden, die nicht einfach zu diagnostizieren ist [27,42].…”