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主肺动脉窗:手术治疗后早期和晚期成功的相关因素。

Aortopulmonary window: factors associated with early and late success after surgical treatment.

作者信息

van Son J A, Puga F J, Danielson G K, Seward J B, Mair D D, Schaff H V, Ilstrup D M

机构信息

Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905.

出版信息

Mayo Clin Proc. 1993 Feb;68(2):128-33. doi: 10.1016/s0025-6196(12)60159-1.

DOI:10.1016/s0025-6196(12)60159-1
PMID:8423692
Abstract

Between 1953 and 1990, 19 patients, who were from 7 weeks to 27 years old, underwent surgical correction of aortopulmonary window at the Mayo Clinic. Associated cardiac anomalies were present in nine patients (47%). At operation, extracorporeal circulation was used in all except one patient. In seven patients, division and primary closure were done. In four patients, the defect was closed by direct suture through a transpulmonary approach. In eight patients, the defect was closed with a patch through a transpulmonary or transaortic approach. Four deaths (21%) occurred intraoperatively or immediately postoperatively. All four patients had undergone division of the aortopulmonary window before 1962, and three of them had a pulmonary vascular resistance (Rp) that was 7.3 U.m2 or more and a ratio of Rp to systemic vascular resistance (Rp/Rs) that exceeded 0.6. One patient with an Rp of 11.8 U.m2 and an Rp/Rs of 0.72 died 16 years postoperatively. Statistical analysis of risk factors indicated that early year of operation (P = 0.022), division of the aortopulmonary window versus transaortic or transpulmonary closure (P = 0.009), and a high Rp/Rs (P = 0.021) were significantly associated with perioperative death. All patients with a preoperative Rp of 8 U.m2 or less, an Rp/Rs of less than 0.4, and no major associated cardiac anomalies were in functional class I (New York Heart Association) postoperatively. Our study confirms that infants with aortopulmonary window should undergo operation early, before irreversible pulmonary vascular changes have developed. Patients with an Rp/Rs that exceeds 0.4 should be thoroughly assessed to determine their operability.

摘要

1953年至1990年间,梅奥诊所对19例年龄在7周龄至27岁之间的患者进行了主肺动脉窗手术矫正。9例患者(47%)合并有心脏畸形。手术时,除1例患者外均采用体外循环。7例患者行开窗并一期缝合。4例患者经肺动脉途径直接缝合关闭缺损。8例患者经肺动脉或主动脉途径用补片关闭缺损。4例患者(21%)在术中或术后即刻死亡。这4例患者均在1962年前进行了主肺动脉窗开窗手术,其中3例患者的肺血管阻力(Rp)为7.3U.m²或更高,Rp与体循环血管阻力之比(Rp/Rs)超过0.6。1例Rp为11.8U.m²、Rp/Rs为0.72的患者术后16年死亡。危险因素的统计分析表明,手术年份较早(P = 0.022)、主肺动脉窗开窗术与经主动脉或肺动脉关闭术相比(P = 0.009)以及高Rp/Rs(P = 0.021)与围手术期死亡显著相关。所有术前Rp为8U.m²或更低、Rp/Rs小于0.4且无严重合并心脏畸形的患者术后心功能均为I级(纽约心脏协会分级)。我们的研究证实,主肺动脉窗患儿应在不可逆的肺血管改变出现之前尽早接受手术。Rp/Rs超过0.4的患者应进行全面评估以确定其手术可行性。

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A Case of Aortopulmonary Window: Asymptomatic until the First Pregnancy.一例主肺动脉窗:妊娠前无症状。
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