Law Y M, Mack C L, Sokol R J, Rice M, Parsley L, Ivy D
Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington 98105, USA.
Pediatr Transplant. 2011 Dec;15(8):E162-8. doi: 10.1111/j.1399-3046.2010.01355.x. Epub 2010 Jun 20.
HPS and PPHTN are unusual and challenging pulmonary manifestations of liver disease. We report two pediatric cases in association with heterotaxy polysplenia syndrome and congenital absence of the portal vein. Both patients were symptomatic and hemodynamically compromised and required aggressive medical therapy. One patient with PPHTN alone achieved a successful liver transplant. The second child presented with combined HPS and PPHTN and exhibited a different evolution of pulmonary vascular disease. These cases illustrate associations that must be entertained in the setting of heterotaxy syndrome, cyanosis, or pulmonary hypertension and how strategic medical combined with surgical management can provide a good outcome.
肝肺综合征（HPS）和门静脉高压相关肺动脉高压（PPHTN）是肝病不常见且具有挑战性的肺部表现。我们报告了两例与内脏反位多脾综合征及先天性门静脉缺如相关的儿科病例。两名患者均有症状且血流动力学受损，需要积极的药物治疗。仅患有PPHTN的一名患者成功接受了肝移植。第二个孩子同时患有HPS和PPHTN，表现出不同的肺血管疾病演变过程。这些病例说明了在内脏反位综合征、发绀或肺动脉高压情况下必须考虑的关联，以及如何通过策略性的药物联合手术管理获得良好的治疗效果。
