Wawrzyńska Liliana, Remiszewski Paweł, Kurzyna Marcin, Fijałkowska Anna, Burakowski Janusz, Dabrowski Marek, Orłowski Tadeusz, Roszkowski Kazimierz, Dłutek Piotr, Klepetko Walter, Torbicki Adam
Klinika Chorób Wewnetrznych Klatki Piersiowej Instytutu Gruźlicy i Chorób Płuc.
Pol Arch Med Wewn. 2006 Jun;115(6):565-71.
We describe a case of 29 year old man, a first Polish patient with idiopathic arterial hypertension (IPAH) listed from Poland and successfully treated with lung transplantation in Vienna. Time from diagnosis to lung transplant was merely 11 months. Rapid clinical deterioration required treatment with most of currently approved or emerging methods, including oral and parenteral prostacyclin analogues administration by inhalation and chronic subcutaneous infusion. Atrial balloon septostomy was used to bridge the patient to transplant. We describe multiple problems in providing pharmacotherapy and in arranging logistics for lung transplantation. Peri- and multiple post-transplantation complications including dehiscence of right main bronchial anastomosis and its successful therapy are also presented. We consider good long term outcome as assessed 26 months post transplantation as an encouragement for other attempts at lung transplantation in patients with IPAH and for development of this method of therapy in Poland.
我们描述了一例29岁男性患者,他是首位来自波兰的特发性肺动脉高压(IPAH)患者,从波兰转诊而来,并在维也纳成功接受了肺移植手术。从诊断到肺移植的时间仅为11个月。快速的临床恶化使得患者需要接受目前大多数已批准或新出现的治疗方法,包括吸入和慢性皮下输注口服及胃肠外前列环素类似物。心房球囊造口术被用于使患者过渡到移植阶段。我们描述了在提供药物治疗和安排肺移植后勤保障方面的多个问题。还介绍了围手术期和多次移植后并发症,包括右主支气管吻合口裂开及其成功治疗。我们认为,移植后26个月评估的良好长期结果,对波兰IPAH患者进行其他肺移植尝试以及发展这种治疗方法是一种鼓舞。