This study highlights the influence of age on phenotypes of incident pulmonary arterial hypertension and has shown the changes in demographics and epidemiology over the past decade in a national setting. The results suggest that there may be two subtypes of patients: the younger subtype with more severe hemodynamic impairment but better survival, compared with the older subtype who has more comorbidities.

Objective Pregnancy in women with pulmonary hypertension (PH) is reported to carry a maternal mortality rate of 30-56%. We report our experience of the management of pregnancies using a strategy of early introduction of targeted pulmonary vascular therapy and early planned delivery under regional anaesthesia.Design Retrospective observational study.Setting Specialist quaternary referral pulmonary vascular unit.Population Nine women with PH who chose to proceed with ten pregnancies.Methods A retrospective review of the management of all women who chose to continue with their pregnancy in our unit during 2002-2009. Main outcome measures Maternal and fetal survival.Results All women commenced nebulised targeted therapy at 8-34 weeks of gestation. Four women required additional treatment or conversion to intravenous prostanoid therapy. All women were delivered between 26 and 37 weeks of gestation. Delivery was by planned caesarean section in nine cases. All women received regional anaesthesia and were monitored during the peripartum period in a critical care setting. There was no maternal mortality during pregnancy and all infants were free from congenital abnormalities. One woman died 4 weeks after delivery following patient-initiated discontinuation of therapy. All remaining women and infants were alive after a median of 3.2 years (range, 0.8-6.5 years) of follow-up.Conclusion Although the risk of mortality in pregnant women with PH remains significant, we describe improved outcomes in fully counselled women who chose to continue with pregnancy and were managed with a tailored multiprofessional approach involving early introduction of targeted therapy, early planned delivery and regional anaesthetic techniques.

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.

Vasopressin is a peptide synthesized in the hypothalamus whose primary role is in fluid homeostasis. It has recently gained interest as a potential agent in the treatment of cardiorespiratory arrest. Initial human studies have shown benefits with vasopressin in patients with out of hospital ventricular fibrillation and asystolic cardiac arrest. One subgroup of patients not included in these trials is patients with pulmonary hypertension, who have a five-year mortality rate of 50%. Animal studies have shown vasopressin to be a vasodilator in the pulmonary vascular system of rats, under normoxic and hypoxic conditions, with conflicting results in canines. Human studies have shown conflicting results with increases, decreases and no changes seen in pulmonary artery pressures of patients with a variety of clinical conditions. Research needs to be done in patients with pulmonary hypertension regarding the potential role of vasopressin during cardiac arrest in this subgroup.

malignancy at the time of the CT scan compared to patients with known malignancy (69.4% vs 46.0%, p = 0.0046), or if metastases were present at the time of CT scan (58.3% vs 26.7%, p = 0.0012).There were 86 (53.8%) central (main or lobar pulmonary arteries), 60 (37.5%) segmental, and 14 (8.8%) subsegmental pulmonary emboli. No significant mortality difference was observed between these radiological features. Conclusion This study has assessed potential poor prognostic features in patients with cancer and iPE. Despite the vast majority receiving therapeutic anticoagulation, there is a high 30-day and 6-month mortality. The benefits of conventional treatment in this clinical situation are as yet unclear. Rationale There is limited data on predictors of survival in patients with Pulmonary Hypertension (PH) in the context of Heart Failure and Preserved Ejection Fraction (HF-pEF). Simple non-invasive tests to aid the physician in prognostication would be valuable. The aim of this study was to examine demographic and non-invasive predictors of outcome in PH-HF-pEF in a large well phenotyped PH registry. Method In the ASPIRE Registry (Hurdman J et al Eur Resp J, 2012), 1737 consecutive, incident, treatment-naıve patients with suspected PH underwent diagnostic evaluation between February 2001 and 2010. Patients were diagnosed as PH-HF-pEF if no other causes of PH could be identified and they fulfilled the following criteria: signs and symptoms of heart failure; mean pulmonary artery pressure ≥25 mmHg at rest and pulmonary arterial wedge pressure >15 mmHg by RHC; preserved left ventricular systolic function (ejection fraction ≥50%) by echocardiography or CMR. Predictors of survival were assessed using forward stepwise Cox regression analysis. Variables with a p-value Results 98 patients who fulfilled the diagnostic criteria for PH-HF-pEF were identified. Maximum duration of follow-up was 10 years with a mean follow up 4.9 ± 2.3 years, during which 33 (34%) patients died. After multivariate analysis, only ISWT distance HR 0.99 CI (0.99-1.00) and TLCO HR 0.96 CI(0.94-0.98) at baseline, were predictors of outcome (p < 0.01). Median predicted TLCO in the PH-HF-pEF population was 65%. The 5-year survival in those with a TLCO <65% predicted was 60%, compared with 85% in those whose TLCO was ≥65% (p < 0.01). Conclusions Simple non-invasive testing such as TLCO and exercise capacity measured by the ISWT predict outcome in patients with PH-HF-pEF.