肺动脉支架置入术治疗广泛的先天性远端狭窄

Q4 Medicine

JACC. Case reports Pub Date : 2025-10-01 DOI:10.1016/j.jaccas.2025.105311

S. Wiltshire BMed , F. Collins MBBS , E. Kotlyar MBBS, MD, MPVD , K. Kearney MBBS , E. Lau BSc, MBBS, PhD , P. Macdonald MBBS, MD, PhD , D. Boshell MBBS , R. Cordina MBBS (Hons), PhD

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引用次数: 0

摘要

背景：对于远端和广泛的先天性肺动脉狭窄或发育不全的成人，肺动脉支架置入术很少有报道。病例总结：一名35岁女性，患有弥漫性先天性远端肺动脉发育不全并有症状的右心衰，接受了肺动脉支架植入术，临床效果明显改善。一位62岁的女性，由于症状性非持续性室性心动过速和右心室收缩储备不足，广泛的肺动脉远端狭窄接受了支架植入术。她的心律失常消退，右心室收缩储备改善。两个病人都有弹性蛋白基因突变。关于经皮介入治疗远端和广泛的肺动脉狭窄或发育不全的成人的报道有限。我们描述了这一人群中2例成功的肺动脉支架术。远端和广泛的先天性肺动脉狭窄或发育不全可以通过支架置入术治疗，如果由经验丰富的手术医师在正确的临床环境中对精心挑选的患者进行手术。

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Pulmonary Artery Stenting for Widespread Distal Congenital Stenoses

Background

Pulmonary artery stenting is rarely reported in adults with distal and widespread congenital pulmonary artery stenoses or hypoplasia.

Case Summary

A 35-year-old woman with diffuse distal congenital pulmonary artery hypoplasia and symptomatic right heart failure underwent pulmonary artery stenting, experiencing significant clinical improvement. A 62-year-old woman with widespread distal pulmonary artery stenoses underwent stenting owing to symptomatic nonsustained ventricular tachycardia and poor right ventricular contractile reserve. Her arrhythmia resolved and she experienced improvement of right ventricular contractile reserve. Both patients have an elastin gene mutation.

Discussion

There are limited reports of percutaneous intervention for adults with distal and widespread pulmonary artery stenoses or hypoplasia. We describe 2 successful cases of pulmonary artery stenting in this population.

Take-Home Message

Distal and widespread congenital pulmonary artery stenoses or hypoplasia can be treated with stenting when performed in well-selected patients by an experienced proceduralist in the right clinical setting.

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