Sendaway capillary NT-proBNP in pulmonary hypertension.

BACKGROUND: N-terminal pro-B-type natriuretic peptide (NT-proBNP) is a biomarker of cardiac ventricular wall stress that is incorporated into pulmonary hypertension (PH) risk stratification models. Sendaway sampling may enable patients to perform NT-proBNP tests remotely. This UK-wide study aimed to assess the agreement of sendaway NT-proBNP with standard venous NT-proBNP and to assess the effect of delayed processing. METHODS: Reference venous NT-proBNP was collected from PH patients. Samples for capillary and venous sendaway tests were collected contemporaneously, mailed to a reference laboratory and processed at 3 and 7 days using a Roche Cobas e411 device. Differences in paired measurements were analysed with Passing-Bablok regression, percentage difference plots and the % difference in risk strata. RESULTS: 113 patients were included in the study. 13% of day 3 capillary samples were insufficient. Day 3 capillary samples were not equivalent to reference samples (Passing Bablok analysis slope of 0.91 (95% CI 0.88 to 0.93) and intercept of 6.0 (95% CI 0.2 to 15.9)). The relative median difference was -7% and there were acceptable limits of agreement. Day 3 capillary NT-proBNP accurately risk stratified patients in 93.5% of cases. By comparison, day 3 venous results accurately risk stratified patients in 90.1% of cases and were equivalent by Passing-Bablok regression. Delayed sampling of sendaway tests led to an unacceptable level of agreement and systematically underestimated NT-proBNP. CONCLUSIONS: Sendaway NT-proBNP sampling may provide an objective measure of right ventricular strain for virtual PH clinics. Results must be interpreted with caution in cases of delayed sampling.

A Health State Utility Study to Elicit Societal Values Associated with Pulmonary Hypertension.

Aim: Pulmonary hypertension (PH) is a rare, severe, and progressive pulmonary vascular disease, which includes five subgroups with similar presentation. Symptoms include dyspnea, and fatigue, and can significantly impact one's health-related quality of life (HRQL). Although treatments are mainly medical, PH group 4, chronic thromboembolic pulmonary hypertension (CTEPH), can be managed with procedures, ie pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA). Currently, drugs in Europe are only approved for pulmonary arterial hypertension (PAH), and CTEPH therefore the aim of this study was to elicit novel societal health state utilities in the UK for PAH and CTEPH based on disease severity, functional class (FC), clinical events, and treatment procedures specifically for CTEPH. Material and Methods: Six health states were defined: World Health Organization (WHO)-FC II, WHO-FC III, and WHO-FC IV [defined by the New York Heart Association (NYHA)]; PH-related hospitalization; and "BPA procedure and recovery" and "PEA surgery and recovery". Health states were based on a targeted literature review and two rounds of interviews with clinical experts (N = 4) and patients (N = 6). Draft health states were validated in cognitive debriefing interviews with clinical experts (N = 3). Health states were valued by the UK general public (n = 200), using a visual analogue scale (VAS) and time trade-off (TTO) assessment with the lead time method. Results: The mean TTO values/utilities were 0.81 (FC II), 0.80 (BPA), 0.78 (PEA), 0.59 (FC III), 0.28 (FC IV), and 0.25 (PH-related hospitalization). Each progression in FC was associated with worse TTO scores. Conclusion: This study reports societal utility values for PAH and CTEPH in the UK. It provides first utility estimates for states such as BPA procedure and recovery, PEA surgery and recovery and PH-related hospitalization for this population. The results show important distinctions between FC, treatment procedures, and hospitalization, and the significant burden of disease on HRQL.

European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology.

AIMS: To develop a suite of quality indicators (QIs) for the evaluation of the care and outcomes for adults with pulmonary arterial hypertension (PAH). METHODS AND RESULTS: We followed the European Society of Cardiology (ESC) methodology for the development of QIs. This included (i) the identification of key domains of care for the management of PAH, (ii) the proposal of candidate QIs following systematic review of the literature, and (iii) the selection of a set of QIs using a modified Delphi method. The process was undertaken in parallel with the writing of the 2022 ESC/European Respiratory Society (ERS) guidelines for the diagnosis and treatment of pulmonary hypertension and involved the Task Force chairs, experts in PAH, Heart Failure Association (HFA) members and patient representatives. We identified five domains of care for patients with PAH: structural framework, diagnosis and risk stratification, initial treatment, follow-up, and outcomes. In total, 23 main and one secondary QIs for PAH were selected. CONCLUSION: This document presents the ESC QIs for PAH, describes their development process and offers scientific rationale for their selection. The indicators may be used to quantify and improve adherence to guideline-recommended clinical practice and improve patient outcomes.

Establishing expert consensus for the optimal approach to holistic risk-management in pulmonary arterial hypertension: a Delphi process and narrative review.

Background: Pulmonary arterial hypertension (PAH) is associated with significant morbidity and reduced life expectancy. Various medical therapies, together with non-medical therapies such as exercise training, have been shown to improve outcomes for patients. We performed a Delphi consensus process to establish optimal approaches to optimizing patient care.Methods: A steering group of PAH experts formulated 38 statements grouped into 6 themes: burden of PAH, risk-stratification, the role of clinical phenotyping in the management of PAH, assessing clinical response to treatment, maximizing the medical treatment pathway and the role of other management options. An online survey was sent to PAH health-care professionals throughout the UK to assess consensus with these statements. Consensus was defined as high if ≥70% and very high if ≥90% of the respondents agreed with a statement. A narrative review for each theme was then performedResults: Consensus was very high in 27 (71%) statements, high in 7 (18%) statements and was not achieved in 4 (11%) statements.Conclusions: Based on the consensus scores, the steering group derived 13 recommendations which, if implemented, should result in improved holistic care of patients with PAH.

EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study.

Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified. Correlations with exercise capacity and World Health Organization (WHO) functional class were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-min walk distance (r=-0.546), incremental shuttle walk distance (r=-0.504) and WHO functional class (r=0.497) (all p<0.0001). Distribution of emPHasis-10 score differed significantly between each WHO functional class (all p<0.0001). On multivariate analysis, emPHasis-10 score, but not WHO functional class, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with 1-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO functional class III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 scores had improved exercise capacity (p<0.0001) and patients who transitioned between risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.

The practical management of fluid retention in adults with right heart failure due to pulmonary arterial hypertension.

Our aim with this review is to provide practical advice and management support for nurses and other healthcare practitioners in managing fluid retention in adults with right heart failure (RHF) due to pulmonary arterial hypertension (PAH). Vigilant management of RHF is important for maintaining patient quality of life, as fluid overload can lead to abdominal bloating (ascites) and peripheral oedema, which also has a major impact on patients' morbidity and mortality. Patients with RHF should be assessed regularly for signs of fluid retention. If fluid overload develops, it is important to determine whether it is caused by the progression of PAH, a side effect of PAH-specific treatment, or another drug or comorbid condition, as this affects both the prognosis and the management strategy. Right heart failure can be treated with both pharmacological and non-pharmacological interventions to reduce fluid retention; including altering fluid and salt intake, weight monitoring, and use of diuretics. All patients on diuretics should be regularly monitored for renal dysfunction and electrolyte imbalance and given advice on how to manage the side effects associated with diuretic use. Fluid retention is often assessed and treated in clinical practice by specialist nurses, who act as a key patient contact providing advice and information on symptom management. This review provides an overview of the challenges related to fluid retention, including strategies to help patients manage symptoms and side effects of treatment.

Current organization of specialist pulmonary hypertension clinics: results of an international survey.

Optimal pulmonary hypertension (PH) management relies on a timely, accurate diagnosis and follow-up in specialized clinics by multidisciplinary teams that have clearly defined responsibilities and protocols. Internationally agreed criteria for expert center staff are lacking, particularly with respect to nurses, who often act as a central component of the team. This survey aimed to evaluate the current organization of PH clinics and the role of nurses. The survey (35 questions) was online February-December 2015 and was advertised at international PH nurse meetings and through international PH organizations to their corresponding clinics. In total, 126 healthcare professionals from 32 countries responded. According to respondents, 54% of clinics managed >200 patients, of whom 49% had a pulmonary arterial hypertension (PAH) diagnosis, on average. In terms of staff, 66% had a dedicated program administrator, 35% had one full-time nurse coordinator/practitioner/specialist, and 57% had a nurse attend outpatient clinic alongside a physician. Crucially, not all centers had a nurse in their team. The role of a nurse coordinator/practitioner/specialist varied with 51% taking patient histories/examinations and 66% managing outpatients. In 34% of clinics, nurses were involved in their own research. Protocols were available for PH therapies (81%), management of heart failure (37%) and pain (26%), and referring patients who did not have PAH/chronic thromboembolic PH back to their specialist (62%). Not all clinics are meeting all of the standards outlined in the latest guidelines with key areas of improvement being level of support from/for nurses, clear protocols, and referral pathways.

The patient experience of pulmonary hypertension: a large cross-sectional study of UK patients.

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.

Elicitation of health state utilities associated with the mode of administration of drugs acting on the prostacyclin pathway in pulmonary arterial hypertension.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a rare, incurable disease associated with decreased life expectancy and a marked impact on quality of life (QoL). There are three classes of drugs available for treatment: endothelin receptor antagonists (ERA), drugs acting on nitric oxide pathway (riociguat and phosphodiesterase type 5 inhibitors [PDE5i]), and drugs acting on prostacyclin pathway. The latter have widely different modes of administration - continuous intravenous infusion, continuous subcutaneous infusion, inhaled, and oral - each associated with variable treatment burden, and implications for health economic assessment. This study aimed to establish utility values associated with different modes of administration of drugs acting on the prostacyclin pathway for use in economic evaluations of PAH treatments. METHODS: A UK general public sample completed the EQ-5D-5L and valued four health states in time trade-off interviews. The health states drafted from literature and interviews with PAH experts (n=3) contained identical descriptions of PAH and ERA/PDE5i treatment, but differed in description of administration including oral (tablets), inhaled (nebulizer), continuous subcutaneous infusion, and continuous intravenous infusion. RESULTS: A total of 150 participants (63% female; mean age 37 years) completed interviews. Utilities are presented as values between 0 and 1, with 0 representing the state of being dead and 1 representing being in full health. The mean (SD) utility for oral health state was 0.85 (0.16), while all other health states were significantly lower at 0.74 (0.27) for inhaled (p=0.001), 0.59 (0.31) for subcutaneous (p<0.001) and 0.54 (0.32) for intravenous (p<0.001), indicating that there are disutilities (negative differences) associated with non-oral health states. Disutilities were -0.11 for inhaled, -0.26 for subcutaneous, and -0.31 for intravenous administration. CONCLUSION: The results demonstrate quantifiable QoL differences between modes of administration of drugs acting on the prostacyclin pathway. QoL burden should be considered for economic evaluation of drugs for PAH treatment.

Palliative care in pulmonary arterial hypertension.

PURPOSE OF REVIEW: Developments in the management of pulmonary arterial hypertension have significantly improved prognosis changing this from an acute to a chronic disease. Despite optimal treatment many patients still have a high-symptom burden both because of the disease and the side-effects of therapy, consequently there is an increasing need for a palliative care approach to improve the quality of life for this patient group. This review article will outline the need for palliative care support for patients with pulmonary arterial hypertension, discuss the barriers that currently exist and suggest how this may be improved. RECENT FINDINGS: Studies have been conducted which explore the role of palliative care in pulmonary arterial hypertension including physicians attitudes and the current barriers that exist to prevent its implementation. SUMMARY: Specialist palliative support is utilized in the minority of patients with pulmonary arterial hypertension despite a need for symptom control. Patients may benefit from the introduction of a palliative care approach as part of their standard care, but to achieve this there needs to be a greater understanding of the role of palliative care by both clinicians and patients and more research into the benefits for patients with pulmonary arterial hypertension.

Practical considerations for therapies targeting the prostacyclin pathway.

Therapies that target the prostacyclin pathway play a key role in the treatment of both early- and late-stage pulmonary arterial hypertension, and provide significant clinical benefits for patients. A number of agents have been approved, which are administered via intravenous, subcutaneous, inhaled or oral routes. The use of these therapies is associated with practical challenges, relating to the need for up-titration and their routes of administration. We discuss here a number of measures that can be taken to support patients and healthcare professionals in order to address the complexities of using these therapies and to encourage compliance. Providing patients with timely information and education, together with practical advice on managing their medication and associated equipment, assists patients with day-to-day management of therapy. Referral to patient associations and support groups can be of further benefit. With an effective management plan and an experienced multidisciplinary team, the use of therapies that target the prostacyclin pathway can be optimised.

Exercise physiological responses to drug treatments in chronic thromboembolic pulmonary hypertension.

We tested the hypothesis that patients with chronic thromboembolic pulmonary hypertension (CTEPH) that was deemed to be inoperable were more likely to respond to drugs for treating pulmonary arterial hypertension (PAH) by using cardiopulmonary exercise (CPX) testing than those with CTEPH that was deemed to be operable. We analyzed CPX testing data of all patients with CTEPH who were treated with PAH drugs and had undergone CPX testing before and after treatment at a single pulmonary hypertension center between February 2009 and March 2013. Suitability for pulmonary endarterectomy (PEA) was decided by experts in PEA who were associated with a treatment center. The group with inoperable CTEPH included 16 patients, the operable group included 26 patients. There were no differences in demographics and baseline hemodynamic data between the groups. Unlike patients in the operable group, after drug treatment patients with inoperable CTEPH had a significantly higher peak V̇o2 (P < 0.001), work load (P = 0.002), and oxygen pulse (P < 0.001). In terms of gas exchange, there was an overall net trend toward improved V̇e/V̇co2 in the group with inoperable CTEPH, with an increased PaCO2 (P = 0.01), suggesting reduced hyperventilation. No changes were observed in patients with operable CTEPH. In conclusion, treatment with PAH drug therapy reveals important pathophysiological differences between inoperable and operable CTEPH, with significant pulmonary vascular and cardiac responses in inoperable disease. Drug effects on exercise function observed in inoperable CTEPH cannot be translated to all forms of CTEPH.

Left main bronchus compression due to main pulmonary artery dilatation in pulmonary hypertension: two case reports.

Pulmonary arterial dilatation associated with pulmonary hypertension may result in significant compression of local structures. Left main coronary artery and left recurrent laryngeal nerve compression have been described. Tracheobronchial compression from pulmonary arterial dilatation is rare in adults, and there are no reports in the literature of its occurrence in idiopathic pulmonary arterial hypertension. Compression in infants with congenital heart disease has been well described. We report 2 cases of tracheobronchial compression: first, an adult patient with idiopathic pulmonary arterial hypertension who presents with symptomatic left main bronchus compression, and second, an adult patient with Eisenmenger ventricular septal defect and right-sided aortic arch, with progressive intermedius and right middle lobe bronchi compression in association with enlarged pulmonary arteries.

Response to pulmonary arterial hypertension drug therapies in patients with pulmonary arterial hypertension and cardiovascular risk factors.

The age at diagnosis of pulmonary arterial hypertension (PAH) and the prevalence of cardiovascular (CV) risk factors are increasing. We sought to determine whether the response to drug therapy was influenced by CV risk factors in PAH patients. We studied consecutive incident PAH patients (n = 146) between January 1, 2008, and July 15, 2011. Patients were divided into two groups: the PAH-No CV group included patients with no CV risk factors (obesity, systemic hypertension, type 2 diabetes mellitus, permanent atrial fibrillation, mitral and/or aortic valve disease, and coronary artery disease), and the PAH-CV group included patients with at least one. The response to PAH treatment was analyzed in all the patients who received PAH drug therapy. The PAH-No CV group included 43 patients, and the PAH-CV group included 69 patients. Patients in the PAH-No CV group were younger than those in the PAH-CV group (P < 0.0001). In the PAH-No CV group, 16 patients (37%) improved on treatment and 27 (63%) did not improve, compared with 11 (16%) and 58 (84%) in the PAH-CV group, respectively (P = 0.027 after adjustment for age). There was no difference in survival at 30 months (P = 0.218). In conclusion, in addition to older age, CV risk factors may predict a reduced response to PAH drug therapy in patients with PAH.

The association of clinical outcome with right atrial and ventricular remodelling in patients with pulmonary arterial hypertension: study with real-time three-dimensional echocardiography.

AIMS: Right atrial (RA) dilatation may be important for patients' outcome in pulmonary arterial hypertension (PAH). The aim of this study was to examine the longitudinal RA and right ventricular (RV) remodelling in PAH patients using real-time three-dimensional echocardiography (3DE) and their relation to clinical outcome. METHODS AND RESULTS: Sixty-two consecutive PAH patients were studied and compared with a control group of 30 healthy volunteers. RA and RV sphericity indices were measured with 3DE. RV ejection fraction (RVEF), RA volume (RAvol), and the quantification of jet area of tricuspid regurgitation (TR) were measured. Two observers were used for reproducibility assessment. The geometrical change of RA and RV was assessed in relation to clinical outcome, as defined by the increase of functional class or admission to the hospital due to right heart failure. Over 1 year of follow-up, there was significant increase of RA sphericity index (0.85±0.16 vs. 1.2±0.24, P<0.01), RV dilatation (RV sphericity index 0.71±0.07 vs. 0.98±0.04, P<0.01), as well as deterioration of RV systolic function (RVEF 33±8.2 vs. 28±7.6%, P<0.01). Twenty-three patients (37%) had a clinical deterioration within 1 year. An increase of RA sphericity index>0.24 predicted clinical deterioration with a sensitivity of 96% and a specificity of 90% [area under the curve (AUC) 0.97]. RV sphericity index was less sensitive (70%) and specific (62%) in predicting clinical deterioration (AUC 0.649). The deterioration in RVEF had a sensitivity of 91.1% and a specificity of 35.3% (AUC 0.479) in predicting clinical deterioration. The dilatation of RA>14 mL over 1 year had high sensitivity at 82.6% but low specificity at 30.8% in predicting clinical deterioration. CONCLUSION: PAH leads to RA and RV dilatation and functional deterioration which are linked to an adverse clinical outcome. 3DE measurement of RA sphericity index may be a suitable index in predicting clinical deterioration of PAH patients.

Diagnosing and managing pulmonary hypertension.

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure > or = 25 mmHg at rest as assessed invasively by right heart catheterisation. It can affect patients at any age and presents with non-specific symptoms. Accurate diagnosis is important as while PH is a potentially lethal disease it is treatable. Identification of the cause of PH is crucial to ensure that the patient receives appropriate management. The most common causes are heart and lung disease; specialist drug therapies for PH are contraindicated in these patients. However, PH is also associated with a diverse group of diseases. The most common symptom of PH is breathlessness on exertion. Syncope may be the presenting symptom especially in children. Other PH symptoms include: angina pectoris, palpitations, dry cough, exertional nausea and vomiting. Lack of response to conventional treatment for dyspnoea should alert the GP to search for another cause of the patient's symptoms, one of which could be PH. Patients should have an ECG, a chest radiograph and a full blood count, renal and liver function as well as thyroid function tests. When PH is suspected, referral to the local cardiology or respiratory department is recommended. Referral should be urgent if the patient is syncopal, has rapidly progressing symptoms or is in heart failure. Echocardiogram is the investigation of choice for detecting elevated pulmonary pressures. If the echocardiogram shows signs of PH then the patient should be referred to a designated PH centre if pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension are suspected or if PH is of uncertain cause.

Pulmonary arterial hypertension: a multidisciplinary approach to care.

Pulmonary arterial hypertension (PAH) is a devastating disease that has a considerable effect on quality of life and eventually leads to right heart failure. The past ten years have seen improvements in drug therapy and patient care, and the prospects for patients with PAH are now considerably better. Although new drugs have played a major role in this improvement, the focus is on improving the patient care aspect of PAH management. Specialist and general nurses are expected to play a key role in patient care, and this article summarises how changes in the management of PAH can be implemented, with special emphasis on the role of the multidisciplinary team. Lessons learned from this specialty may be effectively transferred to similar rare chronic disease areas.

Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension.

UNLABELLED: Pulmonary hypertension (PH) is a progressive disease with a poor prognosis. Identifying chronic thromboembolic pulmonary disease as a cause of PH has major clinical implications as these patients could be potentially offered a surgical cure. Ventilation-perfusion (V/Q) scintigraphy has a high sensitivity to detect embolic disease but its value has been challenged with the emergence of multidetector CT pulmonary angiography (CTPA). We compared the value of V/Q scintigraphy with CTPA in detecting chronic thromboembolic pulmonary disease. METHODS: We retrospectively reviewed the results of V/Q scintigraphy and CTPA performed on patients who had been referred to the Pulmonary Hypertension Service at Hammersmith Hospital between 2000 and 2005. A total of 227 patients (85 males, 142 females; age range, 18-81 y; mean age, 42 y) had all tests done at Hammersmith Hospital and were included in the study. Interpretation of scans was according to the modified PIOPED (Prospective Investigation of Pulmonary Embolism Diagnosis) criteria. CTPA was considered as suggestive of chronic thromboembolic pulmonary disease if it showed visualization of the thrombus or webs, recanalization, perfusion abnormalities, stenosis, or strictures. Standard pulmonary angiography was performed via femoral approach. In 90% of the cases, CTPA and V/Q scintigraphy were performed within 10 d. RESULTS: Seventy-eight patients (group A) had a final diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) and 149 (group B) had non-CTEPH etiology. Among group A, V/Q scintigraphy was reported as high probability in 75 patients, intermediate probability in 1 patient, and low probability in 2 patients. CTPA was positive in 40 patients and negative in 38 patients. Among group B, V/Q scintigraphy was reported as low probability in 134, intermediate probability in 7, and high probability in 8 patients. CTPA was negative in 148 patients and false-positive in 1 patient. Statistical analysis showed V/Q scintigraphy to have a sensitivity of 96%-97.4% and a specificity of 90%-95%. CTPA showed a sensitivity of 51% and a specificity of 99%. CONCLUSION: Our results demonstrate that V/Q scintigraphy has a higher sensitivity than CTPA in detecting CTEPH as a potential curable cause of PH.

Sildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) study.

RATIONALE: Phosphodiesterase type 5 (PDE5) inhibition has been proposed for the treatment for pulmonary arterial hypertension (PAH). OBJECTIVE: This study compared adding sildenafil, a PDE5 inhibitor, to conventional treatment with the current practice of adding bosentan, an endothelin receptor antagonist. METHODS: Twenty-six patients with PAH, idiopathic or associated with connective tissue disease, World Health Organization (WHO) functional class III, were randomized in a double-blind fashion to receive sildenafil (50 mg twice daily for 4 weeks, then 50 mg three times daily) or bosentan (62.5 mg twice daily for 4 weeks, then 125 mg twice daily) over 16 weeks. MEASUREMENTS: Changes in right ventricular (RV) mass (using cardiovascular magnetic resonance), 6-minute walk distance, cardiac function, brain natriuretic peptide, and Borg dyspnea index. MAIN RESULTS: When analyzed by intention to treat, there were no significant differences between the two treatment groups. One patient on sildenafil died suddenly. Patients on sildenafil who completed the protocol showed significant changes from baseline, namely, reductions in RV mass (-8.8 g; 95% confidence interval [CI], -2, -16; n = 13, p = 0.015) and plasma brain natriuretic peptide levels (-19.4 fmol x ml(-1); 95% CI, -5, -34; p = 0.014) and improvements in 6-minute walk distance (114 m; 95% CI, 67, 160; p = 0.0002), cardiac index (0.3 L x min(-1) x m(-2); 95% CI, 0.1, 0.4; p = 0.008), and systolic left ventricular eccentricity index (-0.2; 95% CI, -0.02, -0.37; p = 0.031). Bosentan improved 6-minute walk distance (59 m; 95% CI, 29, 89; n = 12, p = 0.001) and cardiac index (0.3; 95% CI, 0.1, 0.4; p = 0.008). CONCLUSIONS: Sildenafil added to conventional treatment reduces RV mass and improves cardiac function and exercise capacity in patients with PAH, WHO functional class III. Safety monitoring is important until more experience is obtained.