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.

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.

The aim of this study is to review the role and technique of renal artery embolisation (RAE), and assess its effectiveness in the palliative treatment of unresectable or inoperable renal cell carcinoma (RCC) in our institution. The study group consisted of 19 consecutive patients (16 male, 3 female; age range 47-87 years) who underwent palliative RAE for the treatment of renal carcinoma between January 2000 and December 2005. Unresectable disease was present in 11 patients (3 stage IVa, 8 stage IVb). Potentially resectable disease was present in 8 patients (4 stage II, 1 stage IIIa, 1 stage IIIb, 2 stage IIIc); however, these patients were unfit for surgery for other reasons. 13 patients presented with haematuria, which was gross in 7 patients. Nine patients complained of flank pain. RAE was performed using polyvinyl alcohol or embosphere particles, metallic coils and, in some cases, absolute alcohol was necessary. At the time of analysis, 12 patients had died while 7 patients were still alive, with an overall median survival for the study group of 6 months. In the 7 patients with transfusion dependant gross haematuria, there was stabilization of the haemoglobin level post-embolisation. In the 9 patients who presented with flank pain, symptoms improved or resolved in 8 patients. The median length of hospital stay for the 18 patients who were discharged was 5.0 days. RAE is a safe and tolerable management option for patients with inoperable or unresectable renal carcinoma as a means of palliation of local symptoms and improving clinical status, with low morbidity and shorter hospital stay.

Patients with pulmonary hypertension (PH) can be extremely challenging to manage in the critical care setting. In this article we review the classification, diagnosis, and chronic management of PH. An approach to the management of the critically unwell PH patient is discussed. Initial management involves treating underlying precipitants of deterioration and optimizing right ventricular (RV) preload. Reduction of RV afterload with pulmonary vasodilators is also required. Augmentation of cardiac function and perfusion pressures with inotropes and vasopressors may additionally be needed. Advanced renal and respiratory support may be appropriate depending on the clinical context. Patients with known PH who have undergone major surgery or who are in the immediate postpartum period are also at significant risk of deterioration and require management in the critical care setting. Although pulmonary vasodilators are associated with improvements in pulmonary haemodynamics and oxygenation in patients with acute respiratory distress syndrome or after cardiac surgery, there is currently no evidence demonstrating improved outcomes. Key points • Current classification of pulmonary hypertension comprised five diagnostic groups based on shared pathophysiology. • Diagnostic approach includes echocardiography, the exclusion of thromboembolic disease, and right heart catheterization. • The majority of evidence for the use of pulmonary vasodilators is in patients with pulmonary arterial hypertension. • Critical care management of the acutely unwell patient should involve: a. treating precipitating factors. b. optimizing right ventricular preload and afterload. c. maintaining perfusion pressures. d. increasing right ventricular contractility.