Pulmonary and Critical Care Medicine Division, Henry Ford Hospital, 2799 West Grand Boulevard, K-17, Detroit, MI, 48202, USA,
Lung. 2013 Dec;191(6):593-600. doi: 10.1007/s00408-013-9501-5. Epub 2013 Aug 25.
Portopulmonary hypertension (PoPH) is a subgroup of Group 1 pulmonary arterial hypertension (PAH) with particularly poor prognosis. Delay in initiation of parenteral therapy may be the reason for poor outcome.
We conducted a prospective observational study of all patients with Group 1 PoPH evaluated at Henry Ford Hospital between January 2002 and July 2012. The cohort of the REVEAL Registry patients with PoPH was used as the comparator group. The patient survival rates at 5 years after diagnosis and 2 years after enrollment, treatment trends, and the freedom from all-cause hospitalization rates at 2 years after enrollment were compared using χ (2) analysis.
Twenty-one patients were enrolled in the PH Clinic with PoPH from January 2002 through July 2012. Our patients were significantly more likely to be on prostacyclin IV at 90 days as compared to REVEAL PoPH patients (67 vs. 31 %; p = 0.002). Despite this, early outcomes were not significantly different between the groups: 2-year survival from enrollment (70 and 67 %, respectively; p = 0.77) and 2-year freedom from hospitalization (35 vs. 49 %, respectively; p = 0.29). However, 5-year survival from time of diagnosis was significantly higher in our cohort of PoPH patients (71 and 40 %, respectively; p = 0.02).
Early initiation of parenteral prostacyclin therapy in PoPH patients at a single institution was associated with improved 5-year patient survival from diagnosis as compared to the REVEAL Registry of PoPH patients and allowed for clearance for transplant in 52 % of patients within 1 year.
门脉高压性肺高血压（PoPH）是 1 型肺动脉高压（PAH）的一个亚组，预后特别差。开始使用肠外治疗的延迟可能是预后不良的原因。
我们对 2002 年 1 月至 2012 年 7 月期间在亨利福特医院评估的所有 1 型 PoPH 患者进行了前瞻性观察研究。将 PoPH 患者的 REVEAL 登记处队列用作对照组。使用 χ²检验比较诊断后 5 年和入组后 2 年的患者生存率、治疗趋势以及入组后 2 年的全因住院率无复发生存率。
2002 年 1 月至 2012 年 7 月期间，有 21 例患者在 PH 诊所被诊断为 PoPH。与 REVEAL PoPH 患者相比，我们的患者在 90 天时更有可能使用前列环素 IV（67%比 31%；p=0.002）。尽管如此，两组的早期结果并无显著差异：从入组开始的 2 年生存率（分别为 70%和 67%；p=0.77）和 2 年无住院率（分别为 35%和 49%；p=0.29）。然而，我们的 PoPH 患者队列的 5 年诊断后生存率明显更高（分别为 71%和 40%；p=0.02）。
与 REVEAL 登记处的 PoPH 患者相比，在单一机构中早期开始使用肠外前列环素治疗 PoPH 患者，与诊断后 5 年患者生存率的提高相关，并使 52%的患者在 1 年内获得移植资格。
