Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry.

BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service. METHODS: A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010. RESULTS: Patients with PoPH (n = 110) had survival rates of 85%, 60%, and 35% at 1, 3, and 5 years. The prevalence of PoPH was 0.85 cases/1 million. Mean age at diagnosis was 53 ± 12 years, with a balanced distribution in gender. Alcohol (n = 57) and hepatitis C (n = 10) were the most common causes of portal hypertension. Phosphodiesterase V inhibitors were the most frequently used targeted therapy, in 63.6% (n = 70) of patients, endothelin receptor antagonists were used in 10% (n = 11) and prostacyclin analogs in 12.7% (n = 14). Univariate and multivariate analysis of baseline characteristics did not demonstrate a significant influence of severity of portal hypertension or liver cirrhosis, World Health Organization Functional Class, cardiopulmonary hemodynamics, or year of diagnosis on survival. CONCLUSIONS: Survival of patients with PoPH remains poor despite targeted therapy and worse than patients with idiopathic PAH. The benefit of PAH therapies in PoPH on long-term morbidity and mortality outcomes needs further consideration and study.

Observational study of lung transplant recipients surviving 20 years.

BACKGROUND: Lung transplant recipients have reduced long-term survival compared with other solid organ recipients. There is a lack of published data on the characteristics of very long term survivors. METHODS: We describe the demographics, clinical history and post-procedure function of all lung transplant recipients who have survived greater than 20 years at our centre. RESULTS: At the time of analysis there were 21 (16.4%) of 128 patients who survived over 20 years. The mean age at transplantation was 31.8 ± 9.9 years. Five of 21 had undergone single-lung, eight double-lung and eight heart-lung transplant procedures. At the last evaluation, mean percentage predicted FEV1 in recipients of single and double lung were 51.3% and 57.9% respectively. By 20 years, 19 (90.5%) patients had developed bronchiolitis obliterans syndrome (BOS) with three (14%) BOS 1, six (29%) BOS 2 and 10 (48%) BOS 3 and two (9.5%) free from BOS. The median time to onset of BOS was 9.7 years (range 1.6-17.9). Of eight patients (38%) who required renal replacement, four (19%) had successfully undergone renal transplantation and four (19%) were on haemodialysis. Only one patient (5%) had symptomatic osteoporosis. Nineteen patients (90%) were treated for hypertension. Five patients (24%) had diabetes, all with an underlying diagnosis of cystic fibrosis and four of them developing diabetes post operatively. CONCLUSIONS: In our experience, 20-year survivors of lung transplantation had a delayed onset of BOS and morbidities due to immunosuppression that can be appropriately managed leading to long-term survival.