Devaney Eric J, Chang Andrew C, Ohye Richard G, Bove Edward L
Division of Pediatric Cardiac Surgery, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.
Ann Thorac Surg. 2006 Mar;81(3):992-5; discussion 995-6. doi: 10.1016/j.athoracsur.2005.08.020.
Pulmonary vein (PV) stenosis, whether congenital or after repair of total anomalous pulmonary venous connection (TAPVC), continues to carry a poor prognosis.
A retrospective review identified 36 patients who underwent repair of PV stenosis between December 1989 and June 2003. Fourteen with congenital PV stenosis underwent scar excision and primary repair (n = 2), intraoperative stent placement (n = 4), or sutureless pericardial marsupialization (n = 8). Twenty-two with acquired PV stenosis after TAPVC repair underwent anastomotic revision and/or vein repair (n = 11) or sutureless pericardial marsupialization (n = 11). Follow-up ranged from 1 month to 14 years (median, 30 months).
Among the 14 patients with congenital PV stenosis, 8 died (3 early deaths, 4 late deaths with restenosis, and 1 late noncardiac death). Among the six survivors, five (4 after marsupialization) have not developed restenosis. Among 11 of 22 patients with acquired PV stenosis undergoing anastomotic revision or vein repair, there were 5 deaths (2 early, 2 late with restenosis, and 1 late noncardiac death) and 1 of the six survivors has developed restenosis. Of the remaining 11 undergoing marsupialization, there was one late death (with restenosis) and 10 survivors have no restenosis. Congenital etiology, use of marsupialization technique, presence of associated defect, and extent of disease were identified as risk factors for poor outcome.
Patients with pulmonary vein stenosis continue to have a guarded prognosis. Sutureless pericardial marsupialization was associated with satisfactory midterm results and appears superior to other conventional techniques.
肺静脉（PV）狭窄，无论是先天性的还是在完全性肺静脉异位连接（TAPVC）修复术后，预后仍然很差。
一项回顾性研究确定了1989年12月至2003年6月期间接受PV狭窄修复术的36例患者。14例先天性PV狭窄患者接受了瘢痕切除及一期修复（n = 2）、术中支架置入（n = 4）或无缝合心包开窗术（n = 8）。22例TAPVC修复术后获得性PV狭窄患者接受了吻合口修复和/或静脉修复（n = 11）或无缝合心包开窗术（n = 11）。随访时间为1个月至14年（中位数为30个月）。
在14例先天性PV狭窄患者中，8例死亡（3例早期死亡，4例晚期死亡伴再狭窄，1例晚期非心脏性死亡）。在6例幸存者中，5例（4例在开窗术后）未发生再狭窄。在22例接受吻合口修复或静脉修复的获得性PV狭窄患者中，11例中有5例死亡（2例早期死亡，2例晚期死亡伴再狭窄，1例晚期非心脏性死亡），6例幸存者中有1例发生再狭窄。其余11例接受开窗术的患者中，有1例晚期死亡（伴再狭窄），10例幸存者无再狭窄。先天性病因、开窗术技术的应用、相关缺陷的存在以及疾病的程度被确定为预后不良的危险因素。
肺静脉狭窄患者的预后仍然不佳。无缝合心包开窗术的中期结果令人满意，似乎优于其他传统技术。
