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姑息性森宁手术治疗重度肺动脉高压的先天性心脏病

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension.

作者信息

Penha Juliano Gomes da, Zorzanelli Leina, Barbosa-Lopes Antonio Augusto, Atik Edimar, Miana Leonardo Augusto, Tanamati Carla, Caneo Luiz Fernando, Miura Nana, Aiello Vera Demarchi, Jatene Marcelo Biscegli

机构信息

Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

出版信息

Arq Bras Cardiol. 2015 Oct;105(4):353-61. doi: 10.5935/abc.20150097. Epub 2015 Aug 18.

Abstract

BACKGROUND

Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.

OBJECTIVE

Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.

METHODS

Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.

RESULTS

After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.

CONCLUSION

The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

摘要

背景

大动脉转位(TGA)是最常见的青紫型心脏病,发病率为每1000例活产儿中有0.2至0.4例。许多在生命最初几个月未接受治疗的患者可能会进展为严重的肺血管疾病。这些患者的治疗可能包括在心房水平重新引导血流的姑息性手术。

目的

报告我们机构对诊断为TGA和伴有严重肺血管疾病的右心室双出口患儿进行姑息性森宁手术的经验,并评估姑息性森宁手术的早期和晚期临床进展。

方法

基于对1991年至2014年期间病历的评估进行回顾性研究。仅纳入因肺压力升高而无明确心脏病手术治疗指征的患者。

结果

随访一年后,动脉血氧饱和度平均从62.1%升至92.5%,血细胞比容平均从49.4%降至36.3%。16例患者可行肺组织学分析。8例患者证实有3级和4级肺活检表现。

结论

姑息性森宁手术改善了动脉血氧饱和度,减轻了红细胞增多症,并为患有室间隔缺损、严重肺动脉高压且预后不良的TGA患者提供了更好的生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8423/4632999/e095d0937cfd/abc-105-04-0353-g01.jpg

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