The COMPERA 2.0 model has been validated as a novel risk stratification system in pulmonary arterial hypertension (PAH), yet awaits validation in other causes of pulmonary hypertension [1,2]. This study aimed to validate this model in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who were either inoperable or had residual CTEPH following intervention.A retrospective analysis was undertaken of all records of patients who were diagnosed with CTEPH at the Scottish Pulmonary Vascular Unit between 1 January 2010 and 31 December 2020. Patients were included if they met the following criteria: 1) age ⩾18 years; 2) diagnosis of CTEPH according to standard diagnostic criteria [3,4] and based on multidisciplinary consensus; 3) incident diagnosis of treatment-naïve CTEPH, and did not have pulmonary endarterectomy (PEA) or balloon angioplasty (BPA) during follow-up; or 4) had residual pulmonary hypertension after PEA or BPA, included at the point of right heart catheterisation confirming residual pulmonary hypertension (mean pulmonary artery pressure ⩾25 mmHg). Patients were excluded if they were missing any of: baseline World Health Organization functional class (WHO FC), 6-min walk distance (6MWD) or N-terminal prohormone brain natriuretic peptide (NT-proBNP). Diagnostic admission, including right heart catheterisation, served as the baseline visit. Risk according to the three-stratum method and four-stratum method was performed as described by HOEPER et al. [1]. Repeat risk stratification was performed at first follow-up.The primary outcome was all-cause mortality with survival time calculated from the date of diagnosis until death, truncated at 5 years. Survival analysis was performed with Kaplan-Meier analysis with the log-rank test. Cox proportional hazard ratios (HRs) were calculated in reference to the high risk category and Harrell's C-statistic was used to compare Cox models for mortality. Significance was set at the p<0.05 level. GraphPad Prism (v9.3.0, San Diego, CA, USA) was used for analysis. Approval was obtained from London-Riverside Research Ethics Committee (21/PR/1607).218 patients were diagnosed with CTEPH in the study period. 20 patients were excluded due to missing baseline data and 70 patients had no residual pulmonary hypertension following PEA. 128 patients were therefore included in the baseline cohort, of whom 52 (40.6%) were surgically inoperable, 26 (20.3%) were medically inoperable, 23 (17.9%) declined PEA, 19 (14.8%) had residual pulmonary hypertension following PEA or BPA and seven (5.4%) died whilst PEA was being considered. At baseline, 53.9% were male, 18% were WHO FC II, 77.3% FC III and 4% FC IV. The mean age was 69.4 years, mean NT-proBNP was 2244 pg•mL −1 and mean 6MWD was 262 m. At baseline, using the four-stratum model, eight (6.3%) patients were low risk, 32 (25%) patients were intermediate-low risk, 63 (49.2%) patients were intermediate-high risk and 25 (19.5%) patients were high risk. Using the three-stratum model, 10 (7.8%) patients were low risk, 93 (72.7%) ...

The right ventricle (RV) is an important structure which serves a multitude of vital physiological functions in health. For many years, the left ventricle has dominated the focus of understanding in both biology and pathophysiology and the RV was felt to be more of a passive structure which rarely had an effect on disease states. However, it is increasingly recognised that the RV is essential to the homoeostasis of normal physiology and disturbances in RV structure and function have a substantial effect on patient outcomes. Indeed, the prognosis of diseases of lung diseases affecting the pulmonary vasculature and left heart disease is intimately linked to the function of the right ventricle. This review sets out to describe the developmental and anatomical complexities of the right ventricle while exploring the modern techniques employed to image and understand its function from a clinical perspective.

Pulmonary hypertension can be very difficult to identify and patients often experience a long delay before diagnosis. This article aims to raise awareness of pulmonary hypertension in the primary care setting, and discusses the role of the GP in diagnosis and management, with a focus on pulmonary arterial hypertension.

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