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弥漫性肺动静脉畸形(PAVM)的手术切除

Surgical resection of diffuse pulmonary arteriovenous malformations (PAVMs).

作者信息

Falk Aden R, Nitsche Lindsay J, Bontrager Colleen E, Bond Sarah, Beslow Lauren A, Borst Alexandra J, Pogoriler Jennifer, Devlin Paul J, Goldmuntz Elizabeth, Singhal Sunil, Trerotola Scott O, Fuller Stephanie M

机构信息

Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa.

Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, Pa.

出版信息

JTCVS Open. 2024 Nov 16;23:309-317. doi: 10.1016/j.xjon.2024.11.002. eCollection 2025 Feb.

Abstract

OBJECTIVE

Patients with pulmonary arteriovenous malformations (PAVM) can have significant morbidity and mortality. Surgical resection in isolation or with embolization is reported to treat diffuse-type PAVMs. Herein, we describe outcomes for children and adults for whom PAVMs were managed with elective surgical resection.

METHODS

This retrospective analysis includes all patients treated with surgical resection for PAVM from August 1, 2009, to July 20, 2023. Demographic, diagnostic, treatment, and follow-up information were abstracted from medical records. Descriptive statistics were used.

RESULTS

Among 18 patients who underwent surgical resection of PAVMs, 12 had hereditary hemorrhagic telangiectasia. Primary indications for surgery included hemoptysis (n = 4), dyspnea (n = 8), persistence of PAVM following embolotherapy (n = 5), and stroke (n = 1). Selected PAVMs were diffuse-type (n = 14) or highly complex (n = 4). Eight patients underwent embolotherapy before surgery. Most resections were performed via thoracotomy (16/18), with 2 video-assisted thoracoscopic surgeries. Resection consisted of lobectomy (n = 14), segmentectomy (n = 3), or pneumectomy (n = 1). Median oxygen saturation improved from 90% preoperatively to 97% postoperatively. The majority (17/18) of patients were extubated in the operating room, with no major complications. The median hospital length of stay was 4.5 days (range, 2-9 days), with a median of 1 intensive care unit day (range, 1-5 days). At median follow-up of 16 months (range, 6 months-12.1 years), median oxygen saturation was 98%, no bleeding recurred, and 100% survived.

CONCLUSIONS

Although embolization has been the main therapy for most PAVMs, surgical resection of diffuse-type PAVMs is safe and effective. Outcomes were excellent with improvement of oxygen saturation and functional status.

摘要

目的

肺动静脉畸形(PAVM)患者可出现严重的发病率和死亡率。据报道,单独手术切除或联合栓塞治疗弥漫型PAVM。在此,我们描述了接受择期手术切除治疗PAVM的儿童和成人的治疗结果。

方法

这项回顾性分析纳入了2009年8月1日至2023年7月20日期间所有接受PAVM手术切除治疗的患者。从病历中提取人口统计学、诊断、治疗和随访信息,并进行描述性统计分析。

结果

在18例行PAVM手术切除的患者中,12例患有遗传性出血性毛细血管扩张症。手术的主要指征包括咯血(n = 4)、呼吸困难(n = 8)、栓塞治疗后PAVM持续存在(n = 5)和中风(n = 1)。所选的PAVM为弥漫型(n = 14)或高度复杂型(n = 4)。8例患者在手术前接受了栓塞治疗。大多数切除手术通过开胸手术进行(16/18),2例为电视辅助胸腔镜手术。切除方式包括肺叶切除术(n = 14)、肺段切除术(n = 3)或全肺切除术(n = 1)。平均血氧饱和度从术前的90%提高到术后的97%。大多数患者(17/18)在手术室拔管,无重大并发症。中位住院时间为4.5天(范围2 - 9天),中位重症监护病房住院时间为1天(范围1 - 5天)。中位随访16个月(范围6个月至12.1年)时,平均血氧饱和度为98%,无出血复发,100%存活。

结论

尽管栓塞一直是大多数PAVM的主要治疗方法,但弥漫型PAVM的手术切除是安全有效的。血氧饱和度和功能状态得到改善,治疗效果良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6dd7/11883706/dc481db12be0/ga1.jpg

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