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Background Pulmonary Hypertension Association UK (PHA-UK) is the only charity in the UK especially for people affected by pulmonary hypertension (PH). To better understand the impact of PH on patients and carers beyond clinical symptoms, the PHA-UK carried out a cross-sectional survey on the effect of PH on daily living, along with a follow-up survey assessing the financial impact of PH. Methods This is a descriptive cross-sectional survey of adult patients with PH in the UK. A quantitative survey of four key topics (time to diagnosis, quality of life [QoL], financial impact and specialist treatment), was made available to PHA-UK members and patients on PH therapy, with a follow-up financial impact survey sent to those responders who agreed to be contacted further. Data collection was carried out in January and February 2017 for the main survey, and November and December 2017 for the financial impact survey. Results The main survey was completed by 567 individuals, and the financial follow-up survey by 171. Mean age of responders was 69 ± 17 years with 70% female. 60% of respondents said PH had a major impact on their QoL, with 45% reporting that treatment and management improves their QoL ‘a lot’. The time between first experiencing symptoms and diagnosis was ≥1 year for 48% of patients, with 40% seeing 4+ doctors before diagnosis. 63% of patients reported financial worries. Patients in part-time and full-time work reported the greatest financial burden, with a 13 and 33% fall in monthly income respectively. Patients had positive experiences of treatment in specialist centres, with 62% rating their care ‘excellent’, and 92% saying they preferred travelling to a specialist centre rather than seeing a local non-specialist. Conclusions This study reports the largest UK survey exploring issues affecting patients with PH. The study shows that despite the availability of new therapies, patients are still experiencing delays prior to diagnosis, and experiencing both emotional and financial impacts from the disease. By identifying the areas patients find most important in their treatment, this research can inform future care policies and long-term management to support patients living with PH and their families.

Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, with a 1-year survival rate among patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) of 45%. However, more therapies have become available. To investigate the survival and characteristics of all patients diagnosed with CTD-PAH in the U.K. pulmonary hypertension service. National registry of all incident cases of CTD-PAH diagnosed consecutively between January 2001 and June 2006. Patients with CTD-PAH (429; 73% SSc-PAH) were diagnosed by a catheter-based approach. One- and 3-year survival rates were 78 and 47% for patients with isolated SSc-PAH. Survival was worse for those with respiratory disease-associated SSc-PAH (3-yr survival, 28%; P = 0.005) whereas survival among patients with exercise-induced SSc-PAH was superior (3-yr survival, 86%; P = < 0.001). Age, sex, mixed venous oxygen saturation, and World Health Organization functional class were independent predictors of survival in isolated SSc-PAH. Nineteen percent of patients with exercise-induced SSc-PAH and 39% of patients with isolated SSc-PAH who were in functional classes I and II had evidence of disease progression. The prevalence of diagnosed SSc-PAH is 2.93 per 1 million. The 3-year survival rate of 75% for those with pulmonary arterial hypertension associated with systemic lupus erythematosus (SLE-PAH) was significantly better than that for patients with SSc-PAH (P = 0.01). Survival of patients with SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival of patients with respiratory disease-associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism potentially curable by surgery. Perfusion scintigraphy is currently advocated as the imaging modality of choice to exclude CTEPH due to its high sensitivity. We have evaluated the diagnostic utility of lung perfusion MRI. Methods Consecutive patients attending a pulmonary hypertension referral centre undergoing lung perfusion MRI, perfusion scintigraphy, CT pulmonary angiography (CTPA) and right heart catheterisation within 14 days were identified. Results Of 132 patients, 78 were diagnosed as having CTEPH. Lung perfusion MRI correctly identified 76 patients as having CTEPH with an overall sensitivity of 97%, specificity 92%, positive predictive value 95% and negative predictive value 96% compared with perfusion scintigraphy (sensitivity 96%, specificity 90%) and CTPA (sensitivity 94%, specificity 98%). No cases of surgically accessible CTEPH were missed with either modality. Conclusions Lung perfusion MRI has high sensitivity equivalent to perfusion scintigraphy in diagnosing CTEPH but does not require ionising radiation, making it an attractive initial imaging modality to assess patients with suspected CTEPH.

Cardiovascular Magnetic Resonance (CMR) imaging is accurate and reproducible for the assessment of right ventricular (RV) morphology and function. However, the diagnostic accuracy of CMR derived RV measurements for the detection of pulmonary hypertension (PH) in the assessment of patients with suspected PH in the clinic setting is not well described. We retrospectively studied 233 consecutive treatment naïve patients with suspected PH including 39 patients with no PH who underwent CMR and right heart catheterisation (RHC) within 48hours. The diagnostic accuracy of multiple CMR measurements for the detection of mPAP ≥ 25 mmHg was assessed using Fisher's exact test and receiver operating characteristic (ROC) analysis. Ventricular mass index (VMI) was the CMR measurement with the strongest correlation with mPAP (r = 0.78) and the highest diagnostic accuracy for the detection of PH (area under the ROC curve of 0.91) compared to an ROC of 0.88 for echocardiography calculated mPAP. Late gadolinium enhancement, VMI ≥ 0.4, retrograde flow ≥ 0.3 L/min/m2 and PA relative area change ≤ 15% predicted the presence of PH with a high degree of diagnostic certainty with a positive predictive value of 98%, 97%, 95% and 94% respectively. No single CMR parameter could confidently exclude the presence of PH. CMR is a useful alternative to echocardiography in the evaluation of suspected PH. This study supports a role for the routine measurement of ventricular mass index, late gadolinium enhancement and the use of phase contrast imaging in addition to right heart functional indices in patients undergoing diagnostic CMR evaluation for suspected pulmonary hypertension.

ObjectivesPulmonary hypertension is a life-shortening disease that has a considerable impact on quality of life. Improving our understanding of how individuals are affected and cope with the disease will help to improve services and outcomes. This review synthesises the published qualitative research that has listened to adults discuss their experiences of living with the disease.DesignA comprehensive systematic search of four databases was conducted in May 2020: Web of Science, PubMed, PsycINFO and Cochrane Library. Suitable studies were evaluated using the Critical Appraisal Skills programme. Findings from the studies were extracted and subjected to a thematic synthesis.ResultsNineteen articles were identified reflecting the experiences of over 1900 individuals impacted by pulmonary hypertension from Europe, North and South America and Asia. Ten studies did not report participant’s WHO functional class of pulmonary hypertension, which resulted in comparing experiences between different severity difficult. All studies met the majority of the quality assessment items. Six descriptive themes emerged discussing participant’s experiences of diagnosis, treatment, prognosis, healthcare professionals, impact and coping with pulmonary hypertension. Four higher order analytical themes were developed from the descriptive themes, reflecting: (i) uncertainties and anxiety that participants encountered related to pulmonary hypertension; (ii) lack of recognition of the impact of the condition; (iii) frustration at the paucity of awareness of pulmonary hypertension in society and healthcare settings and (iv) participant’s accounts of transitioning through different stages of living with the disease.ConclusionsThese findings form the first synthesis of experiences of life in individuals impacted by pulmonary hypertension and illustrate the multifaceted impact of the condition. The voices of numerous groups are missing from the literature highlighting the need for additional research. The results have implications for clinical practice emphasising the role of educational and psychological therapies to support those with the disease.

The management of chronic thromboembolic pulmonary hypertension (CTEPH) has changed over recent years with the growth of pulmonary endarterectomy surgery and the availability of disease-modifying therapies. To investigate the prognosis of CTEPH in a national setting during recent years. All incident cases diagnosed in one of the five pulmonary hypertension centers in the United Kingdom between January 2001 and June 2006 were identified prospectively. Information regarding baseline characteristics, treatment, and follow-up was subsequently collected from hospital records. A total of 469 patients received a diagnosis, of whom 148 (32%) had distal, nonsurgical disease. One- and three-year survival from diagnosis was 82 and 70% for patients with nonsurgical disease and 88 and 76% for those treated surgically (P = 0.023). Initial functional improvement in patients with nonsurgical disease was noted but did not persist at 2 years. Significant functional and hemodynamic improvements were seen in surgically treated patients with an increase in six-minute-walk distance of 105 m (P < 0.001) at 3 months. Five-year survival from surgery in the 35% of patients who survived to 3 months but had persistent pulmonary hypertension was 94%. The prognosis in nonsurgical disease has improved. We have confirmed the previously described good outcome in surgically treated disease. However, we have also demonstrated that the long-term prognosis for patients who have persistent pulmonary hypertension at 3 months after surgery is good. The observed improvements in outcome during the modern treatment era reinforce the importance of identifying patients with this increasingly treatable condition.

Background Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are severe, progressive diseases characterized by key symptoms such as dyspnea and fatigue. These symptoms impair physical functioning, with patients struggling to perform their daily activities. One traditional measure of physical functioning and exercise capacity is the 6-minute walk test (6MWT). Actigraphy represents a promising tool to complement the 6MWT and provide a holistic picture of physical performance in patients with PAH or CTEPH. However, the current literature holds limited evidence on content validity of actigraphy in these populations, as reported by patients themselves. The primary objective of this study was to understand which physical functioning concepts are most meaningful to patients with PAH or CTEPH and identify relevant actigraphy variables and appropriate timeframes for their measurement. Methods This was a cross-sectional, qualitative study in adults with a confirmed diagnosis of PAH or CTEPH. Participants from the UK and USA were interviewed one-on-one via a web-based platform, with interviewers using a semi-structured discussion guide that included concept elicitation and cognitive debriefing sections. Data within the anonymized interview transcripts were coded and thematically analyzed. Results Concept elicitation identified the physical functioning concepts most meaningful to patients with PAH or CTEPH and generated a combined conceptual model of physical functioning, which strongly aligned with previous literature. During cognitive debriefing, of the four actigraphy variables debriefed in relation to these physical functioning concepts, study participants highly valued time spent in non-sedentary physical activity and time spent in moderate to vigorous activity, while step count and walking speed emerged as less relevant. Participants indicated four alternative variables as relevant: walking distance, walking up hills or inclines, duration of continuous walking bouts, and time spent walking. Regardless of the variable, participants suggested a timeframe of approximately 10 or 12 h/day over a minimum of 14 days for measuring physical functioning. Conclusions By demonstrating the content validity of actigraphy measures of physical functioning, this qualitative study begins to address the evidence gaps identified by the regulatory requirements for using actigraphy endpoints in future PAH and CTEPH clinical trials.

Right atrial (RA) area predicts mortality in patients with pulmonary hypertension, and is recommended by the European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines. The advent of deep learning may allow more reliable measurement of RA areas to improve clinical assessments. The aim of this study was to automate cardiovascular magnetic resonance (CMR) RA area measurements and evaluate the clinical utility by assessing repeatability, correlation with invasive haemodynamics and prognostic value. A deep learning RA area CMR contouring model was trained in a multicentre cohort of 365 patients with pulmonary hypertension, left ventricular pathology and healthy subjects. Inter-study repeatability (intraclass correlation coefficient (ICC)) and agreement of contours (DICE similarity coefficient (DSC)) were assessed in a prospective cohort (n = 36). Clinical testing and mortality prediction was performed in n = 400 patients that were not used in the training nor prospective cohort, and the correlation of automatic and manual RA measurements with invasive haemodynamics assessed in n = 212/400. Radiologist quality control (QC) was performed in the ASPIRE registry, n = 3795 patients. The primary QC observer evaluated all the segmentations and recorded them as satisfactory, suboptimal or failure. A second QC observer analysed a random subcohort to assess QC agreement (n = 1018). All deep learning RA measurements showed higher interstudy repeatability (ICC 0.91 to 0.95) compared to manual RA measurements (1st observer ICC 0.82 to 0.88, 2nd observer ICC 0.88 to 0.91). DSC showed high agreement comparing automatic artificial intelligence and manual CMR readers. Maximal RA area mean and standard deviation (SD) DSC metric for observer 1 vs observer 2, automatic measurements vs observer 1 and automatic measurements vs observer 2 is 92.4 ± 3.5 cm2, 91.2 ± 4.5 cm2 and 93.2 ± 3.2 cm2, respectively. Minimal RA area mean and SD DSC metric for observer 1 vs observer 2, automatic measurements vs observer 1 and automatic measurements vs observer 2 was 89.8 ± 3.9 cm2, 87.0 ± 5.8 cm2 and 91.8 ± 4.8 cm2. Automatic RA area measurements all showed moderate correlation with invasive parameters (r = 0.45 to 0.66), manual (r = 0.36 to 0.57). Maximal RA area could accurately predict elevated mean RA pressure low and high-risk thresholds (area under the receiver operating characteristic curve artificial intelligence = 0.82/0.87 vs manual = 0.78/0.83), and predicted mortality similar to manual measurements, both p < 0.01. In the QC evaluation, artificial intelligence segmentations were suboptimal at 108/3795 and a low failure rate of 16/3795. In a subcohort (n = 1018), agreement by two QC observers was excellent, kappa 0.84. Automatic artificial intelligence CMR derived RA size and function are accurate, have excellent repeatability, moderate associations with invasive haemodynamics and predict mortality.

Follow-up after acute pulmonary embolism (PE) is important to assess recovery, consider the need for on-going anticoagulation and identify chronic thromboembolic pulmonary hypertension (CTEPH), a rare but serious complication of PE. 16 dilemmas in the follow-up of acute PE were identified by a steering committee of four pulmonologists and a haematologist with interest in PE and/or CTEPH. Current literature was reviewed and a practical approach suggested based on expert consensus. Dilemmas discussed included: (1) how to manage a breathless patient; (2) what to do if CTEPH is suspected; (3) the difference between CTEPH and post-PE syndrome, (4) testing for thrombophilia, (5) when to investigate for cancer, (6) anticoagulation duration and dose, (7) approaches to discussions and decision-making with respect to anticoagulation, (8) use of aspirin and whether antiplatelet therapy should be stopped during anticoagulation and (9) advice for patients on discharge from hospital at 3 months and information for first-degree relatives. Given the occurrence of complications that may require assessment, follow-up of patients post-PE should be systematic and consider the individual needs of the patient.

Background Aldosterone is a mineralocorticoid hormone critically involved in arterial blood pressure regulation. Although pharmacological aldosterone antagonism reduces mortality and morbidity among patients with severe left-sided heart failure, the contribution of aldosterone to the pathobiology of pulmonary arterial hypertension (PAH) and right ventricular (RV) heart failure is not fully understood. Methods The effects of Eplerenone (0.1% Inspra® mixed in chow) on pulmonary vascular and RV remodeling were evaluated in mice with pulmonary hypertension (PH) caused by Sugen5416 injection with concomitant chronic hypoxia (SuHx) and in a second animal model with established RV dysfunction independent from lung remodeling through surgical pulmonary artery banding. Results Preventive Eplerenone administration attenuated the development of PH and pathological remodeling of pulmonary arterioles. Therapeutic aldosterone antagonism – starting when RV dysfunction was established - normalized mineralocorticoid receptor gene expression in the right ventricle without direct effects on either RV structure (Cardiomyocyte hypertrophy, Fibrosis) or function (assessed by non-invasive echocardiography along with intra-cardiac pressure volume measurements), but significantly lowered systemic blood pressure. Conclusions Our data indicate that aldosterone antagonism with Eplerenone attenuates pulmonary vascular rather than RV remodeling in PAH.