2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Abstract

PRESCRIBING IN PRACTICE ■ P ulmonary hypertension refers to increased pressure in the pulmonary arterial circulation. The pulmonary circulation has to accommodate the entire cardiac output in each cardiac cycle, and evolution has adapted to this by making it a low-pressure high-flow system. However, pathology can affect both the arterial and venous components of this system. Pulmonary venous hypertension mainly refers to diseases that result in elevated venous pressure and occurs mainly from mitral valve and left-sided heart disease; these will not be discussed in this article. As a result of greater understanding of the molecular and cellular pathways involved in the pathobiology of pulmonary arterial hypertension (PAH), novel and exciting treatments have become available to treat this condition. These new drugs represent a huge step forward in the treatment of this universally fatal disease in that they allow improvement in quality of life and survival; however, they do not as yet offer a cure. Definition PAH is defined by consensus as a mean pulmonary artery pressure of above 25mmHg in the setting of a normal or reduced cardiac output, with a normal pulmonary capillary wedge pressure (PCWP) and elevated pulmonary vascular resistance (PVR). The normal PCWP is required to exclude the presence of significant left heart disease and pulmonary venous hypertension. As a result, the diagnosis of pulmonary hypertension requires invasive right-heart catheterisation. Classification Pulmonary hypertension is an umbrella term that refers simply to elevated pressure in the pulmonary vasculature. There are a wide variety of causes of this and the 2008 WHO conference in Dana Point attempted to clarify and organise these into a more defined classification (see Figure 2). 1 This article will focus on PAH (group 1).

Lack of a Tricuspid Regurgitation Doppler Signal and Pulmonary Hypertension by Invasive Measurement

“…Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) ≥25 mm Hg and is associated with increased morbidity and mortality in a variety of chronic diseases 1, 2. According to current guidelines and consensus statements, the recommended initial noninvasive method to screen for PH is transthoracic echocardiography (TTE) 1, 3.…”

“…According to current guidelines and consensus statements, the recommended initial noninvasive method to screen for PH is transthoracic echocardiography (TTE) 1, 3. Pulmonary pressure is estimated on TTE by interrogation of the tricuspid regurgitation velocity (TRV) using continuous‐wave Doppler 4, 5.…”

“…Pulmonary pressure is estimated on TTE by interrogation of the tricuspid regurgitation velocity (TRV) using continuous‐wave Doppler 4, 5. By use of this method, an estimated pulmonary artery systolic pressure of >40 mm Hg5 or a TRV of >2.8 m/s1 in the absence of an obvious cause of PH should prompt further evaluation with invasive right heart catheterization (RHC).…”

Lung perfusion: MRI vs. SPECT for screening in suspected chronic thromboembolic pulmonary hypertension

“…The case that was missed by SPECT was in a patient with coexistent lung pathology, which caused a defect that was not typical of embolic disease on the SPECT image; the anatomical information available on the nonsubtracted MRI database meant that was less of an issue for the MR perfusion scan. It should be noted that CTEPH and CTED may be missed on CTPA by radiologists not experienced in the assessment of pulmonary vascular disease, leading to the recommendation in the latest international guidelines that SPECT Q is preferred to CTPA when screening for CTEPH 14. Given the similarities between the images obtained by DCE‐MRI and SPECT Q, it is anticipated that DCE‐MRI would have similar diagnostic performance in the hands of a general radiologist.…”

“…The images were qualitatively assessed as either positive or negative for chronic thromboembolic disease. On both DCE perfusion MRI and perfusion SPECT, the presence of one or more segmental or subsegmental perfusion defects was considered positive for pulmonary embolic disease, as per recognized clinical guidelines 14. Figure 1 gives an example of a normal and positive SPECT and DCE‐MRI scan.…”