Iain Armstrong

Improved Outcomes in Medically and Surgically Treated Chronic Thromboembolic Pulmonary Hypertension

RATIONALE 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. OBJECTIVES To investigate the prognosis of CTEPH in a national setting during recent years. METHODS 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. MEASUREMENTS AND MAIN RESULTS 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%. CONCLUSIONS 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.

Aspire Registry: assessing the spectrum of pulmonary hypertension identified at a referral centre

Pulmonary hypertension (PH) is a heterogeneous condition. To date, no registry data exists reflecting the spectrum of disease across the five diagnostic groups encountered in a specialist referral centre. Data was retrieved for consecutive, treatment-naïve cases diagnosed between 2001 and 2010 using a catheter-based approach. 1,344 patients were enrolled, with a mean follow-up of 2.9 yrs. The 3-yr survival was 68% for pulmonary arterial hypertension (PAH), 73% for PH associated with left heart disease, 44% for PH associated with lung disease (PH-lung), 71% for chronic thromboembolic PH (CTEPH) and 59% for miscellaneous PH. Compared with PAH, survival was inferior in PH-lung and superior in CTEPH (p<0.05). Multivariate analysis demonstrated that diagnostic group independently predicted survival. Within PAH, Eisenmenger’s survival was superior to idiopathic PAH, which was superior to PAH associated with systemic sclerosis (p<0.005). Within PH-lung, 3-yr survival in sleep disorders/alveolar hypoventilation (90%) was superior to PH-lung with chronic obstructive pulmonary disease (41%) and interstitial lung disease (16%) (p<0.05). In CTEPH, long-term survival was best in patients with surgically accessible disease undergoing pulmonary endarterectomy. In this large registry of consecutive, treatment-naïve patients identified at a specialist PH centre, outcomes and characteristics differed between and within PH groups. The current system of classification of PH has prognostic value even when adjusted for age and disease severity, emphasising the importance of systematic evaluation and precise classification.

Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach

Please cite this paper as: Kiely D, Condliffe R, Webster V, Mills G, Wrench I, Gandhi S, Selby K, Armstrong I, Martin L, Howarth E, Bu’Lock F, Stewart P, Elliot C. Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach. BJOG 2010;117:565–574.

Pulmonary hypertension in COPD: results from the ASPIRE registry

The phenotype and outcome of severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is described in small numbers, and predictors of survival are unknown. Data was retrieved for 101 consecutive, treatment-naïve cases of pulmonary hypertension in COPD. Mean±sd follow-up was 2.3±1.9 years. 59 patients with COPD and severe pulmonary hypertension, defined by catheter mean pulmonary artery pressure ≥40 mmHg, had significantly lower carbon monoxide diffusion, less severe airflow obstruction but not significantly different emphysema scores on computed tomography compared to 42 patients with mild–moderate pulmonary hypertension. 1- and 3-year survival for severe pulmonary hypertension, at 70% and 33%, respectively, was inferior to 83% and 55%, respectively, for mild–moderate pulmonary hypertension. Mixed venous oxygen saturation, carbon monoxide diffusion, World Health Organization functional class and age, but not severity of airflow obstruction, were independent predictors of outcome. Compassionate treatment with targeted therapies in 43 patients with severe pulmonary hypertension was not associated with a survival benefit, although improvement in functional class and/or fall in pulmonary vascular resistance >20% following treatment identified patients with improved survival. Standard prognostic markers in COPD have limited value in patients with pulmonary hypertension. This study identifies variables that predict outcome in this phenotype. Despite poor prognosis, our data suggest that further evaluation of targeted therapies is warranted.

The use of iloprost in early pregnancy in patients with pulmonary arterial hypertension

In patients with pulmonary hypertension, pregnancy is associated with a high risk of maternal death. Such patients are counselled to avoid pregnancy, or if it occurs, are offered early interruption. Some patients, however, decide to continue with their pregnancy and others may present with symptoms for the first time whilst pregnant. Pulmonary vasodilator therapy provides a treatment option for these high-risk patients. The present study describes three patients with pulmonary arterial hypertension of various aetiologies who were treated with the prostacyclin analogue iloprost during pregnancy, and the post-partum period. Nebulised iloprost commenced as early as 8 weeks of gestation and patients were admitted to hospital between 24–36 weeks of gestation. All pregnancies were completed with a duration of between 25–36 weeks and all deliveries were by caesarean section under local anaesthetic. All patients delivered children free from congenital abnormalities, and there was no post-partum maternal or infant mortality. In conclusion, although pregnancy is strongly advised against in those with pulmonary hypertension, the current authors have achieved a successful outcome for mother and foetus with a multidisciplinary approach and targeted pulmonary vascular therapy.

Ventricular mass index correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis-associated pulmonary arterial hypertension

OBJECTIVE The ventricular mass index (VMI) has been proposed as a diagnostic tool for the assessment of patients with suspected pulmonary hypertension (PH). We hypothesized that in patients with SSc it may predict the presence or absence of PH. METHODS Details of all consecutive SSc patients undergoing MRI and right heart catheterization were collected prospectively. Subsequently, the VMI for all patients was calculated, and further baseline data were collected. RESULTS Data for 40 patients, 28 of whom were diagnosed with PH at rest (PH(REST)), were analysed. VMI correlated strongly with mean pulmonary artery pressure (mPAP; r = 0.79). Using a VMI threshold of 0.56, positive predictive value (PPV) for PH(REST) was 88% and negative predictive value (NPV) was 100%. Using a threshold of 0.7, PPV was found to be 100% and NPV 53%. Echocardiographically obtained tricuspid gradient (TG) also demonstrated a strong correlation with mPAP. Two-year survival in patients with VMI <0.7 and > or =0.7 was 91 and 43%, respectively (P < 0.001). CONCLUSION VMI correlates well with mPAP in patients with SSc and may have a role in non-invasively excluding clinically significant PH in breathless SSc patients in whom echocardiographic screening has failed. Further study in larger groups of patients is justified.

Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives

Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH on patients and carers, beyond the clinical symptoms, are still limited. In order to explore this, a large-scale international survey investigated four key areas affected by PAH (physical and practical, emotional, social, and information needs) and provides new insight into patients’ and carers’ experiences of living with the disease. The results from the survey highlight not only the limited ability of patients to carry out everyday tasks, but also the financial impact and social isolation experienced by both patients and carers. The study confirmed that a decline in a patient’s World Health Organization functional class, which indicates an increase in clinical severity of the disease, is associated with greater limitations. Results from the survey demonstrate the need for multidisciplinary PAH management and a comprehensive standard of care to assess and improve all aspects of well-being for both patients and carers. In addition, they underline the need for updated PAH guidelines that address these needs.

emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension

The aim of this study was to develop a measure of the impact of pulmonary hypertension (PH) on health-related quality of life (HRQoL) as there is a need for a short, validated instrument that can be used in routine clinical practice. Interviews were conducted with 30 PH patients to derive 32 statements, which were presented as a semantic differential six-point scale (0–5), with contrasting adjectives at each end. This item list was completed by patients attending PH clinics across the UK and Ireland. Rasch analysis was applied to identify items fitting a uni-dimensional model. 226 patients (mean age 55.6±14 years; 70% female) with PH (82% had pulmonary arterial hypertension) completed the study questionnaires. 10 of the 32 items demonstrated fit to the Rasch model (Chi-squared 16; p>0.05) and generated the emPHasis-10 questionnaire. Test–retest (intraclass correlation coefficient 0.95, n=33) and internal consistency (Chronbach’s &agr;=0.9) were strong. emPHasis-10 scores correlated consistently with other relevant measures and discriminated subgroups of patients stratified by World Health Organization functional class (ANOVA F=1.73; p<0.001). The emPHasis-10 is a short questionnaire for assessing HRQoL in pulmonary arterial hypertension. It has excellent measurement properties and is sensitive to differences in relevant clinical parameters. It is freely available for clinical and academic use. emPHasis-10 is a short, valid tool for routine assessment of health-related quality of life in pulmonary hypertension http://ow.ly/qv75v

The assessment of breathlessness in pulmonary arterial hypertension: Reliability and validity of the Dyspnoea-12

Breathlessness is a cardinal symptom of pulmonary arterial hypertension (PAH); yet no breathlessness instrument has been previously tested for reliability and validity for this population. Using a cross-sectional design, we tested the psychometric properties of the Dyspnoea-12 (D-12), for the assessment of breathlessness in PAH. Pearson’s correlations with World Health Organization functional class (WHO FC), Minnesota Living with Heart failure – pulmonary hypertension modified version (MLHF-PH), Hospital Anxiety and Depression scale (HADS) and 6-minute walk distance test (6MWD) were conducted. Participants (n = 176) were mostly female (70.5%), mean age 54.3±14 years; diagnosed with idiopathic PAH (48.9%), congenital heart disease (27.8%) and connective tissue disease (23.3%); and most were WHO FC II (32.4%) and III (52.3%). The D-12 showed excellent internal consistency for the total and two-component scores for physical and emotional (Cronbach’s α 0.95, 0.93 and 0.94, respectively). D-12 total score was significantly associated with MLHF-PH (r = 0.70), HADS (anxiety r = 0.54 and depression r = 0.68), WHO FC (r = 0.49), and 6MWD (r = -0.26). In patients with PAH, the D-12 - a short patient reported measure of breathlessness severity that taps the physical and emotional components, is a reliable and valid instrument.

A review of the management of pulmonary arterial hypertension associated with congenital heart disease.

Approximately 5–10% of adolescent and adult patients with congenital heart disease (CHD) will develop pulmonary arterial hypertension (PAH). Patients with PAH associated with CHD (PAH–CHD) exhibit several similarities to those with idiopathic and other associated forms of PAH, especially with regards to their non-specific, cardinal symptoms. The development of PAH–CHD can lead to lifelong impairment although, paradoxically, survival may be better versus idiopathic PAH. Patients with PAH–CHD may experience social limitations, and emotional and psychological issues, arising from their disease burden. Nurses and other allied healthcare professionals are well placed to deliver the individually-tailored care that patients with PAH–CHD require. Activities known to be of particular benefit include patient engagement and education, patient empowerment, colleague training, and ensuring effective communication across the multidisciplinary team. Recent developments in the management of PAH–CHD have led to changes in the medical needs and optimal care of this patient population. This review aims to provide an overview of the natural course, diagnosis, symptoms and impact of PAH–CHD. We also aim to communicate the current standards in management of patients with PAH–CHD, and how their outlook can be improved in the future.