[一系列心脏矫正大动脉转位(双不协调)患者的诊断和治疗]。

Jesús De Rubens-Figueroa, Paulina Villamar-García, Diana G Ruiz-Meléndez, Carlos González-Rebeles, Alexis Palacios-Macedo
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摘要

目的:心房-心室和心室-动脉双不协调(DD)或大动脉转位矫正术是一种罕见的心脏疾病,每1000个活产新生儿中就有0.02-0.07个患有此病。本研究旨在描述一系列 DD 患者的诊断、治疗和演变情况:方法:对过去 22 年中诊断出的 DD 患者的病历进行回顾性和描述性研究。研究采用了描述性统计方法。数字变量采用均值和标准差,分类变量采用频率和百分比:22 年中研究了 30 名患者,男性与女性的比例为 1.5:1,平均年龄为 20 个月。24/29(82.7%)名患者的病位为室间隔缺损。室间隔缺损是最常见的病变,占 25/29 例(86.2%),三尖瓣关闭不全占 70%。四名患者被诊断为肺动脉高压。20%的患者患有房室传导阻滞。一名患者患有沃尔夫-帕金森-怀特综合征。70%的患者接受了手术治疗。其中8人接受了格伦手术(26.6%),4人接受了丰坦手术(13.3%)。随访时间从1个月到17年不等。5人死亡(16.6%)。在随访的25名患者中,18名患者(72%)心室功能正常,5名患者为二级Ross分级(20%),2名患者为三级(8%):结论:这些患者的生活质量正在改善,但文献中对实施最合适手术的理想时间仍存在争议。
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[Diagnosis and treatment in a series of patients with cardiac corrected transposition of the great arteries (double discordance)].

Objective: The atrio-ventricular and ventricle-arterial double discordance (DD) or corrected transposition of the great arteries is a rare heart disease, it occurs in 0.02-0.07 of every 1,000 live newborns. The objective of the study is to describe the diagnosis, treatment and evolution of a series of patients with DD.

Method: A retrospective and descriptive study was carried out, reviewing the records of patients diagnosed with DD in the last 22 years. Descriptive statistics were performed. Numerical variables were obtained using means and standard deviation and categorical variables using frequencies and percentages.

Results: Thirty patients were studied in 22 years with a ratio of 1.5:1 for men, with a mean age of 20 months. The situs was solitus in 24/29 patients (82.7%). Ventricular septal defect was the most frequent lesion in 25/29 (86.2%) Tricuspid insufficiency in 70%. Four patients diagnosed with pulmonary atrial hypertension. With atrio-ventricular block 20%. One with Wolff-Parkinson-White syndrome. Surgical treatment was carried out in 70% of patients. Eight with Glenn procedure (26.6%) and 4 with Fontan surgery (13.3%). Follow-up ranged from 1 month to 17 years. Five died (16.6%). Of the 25 patients in follow-up, 18 patients (72%) had normal ventricular function, 5 with Grade II Ross classification (20%) and 2 in Grade III (8%).

Conclusions: The quality of life of these patients is improving and there is still controversy in the literature about the ideal time to perform the most appropriate surgical procedure.

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