房间隔完整的部分性肺静脉异位引流临床特征及预后分析
Clinical characteristics and prognostic analysis of partial anomalous pulmonary venous connection with intact atrial septum
Received date: 2025-01-02
Accepted date: 2025-05-15
Online published: 2025-09-29
目的 分析房间隔完整的部分性肺静脉异位引流（PAPVC-IAS）的临床特征及预后，以提高对该疾病的认识。方法 收集2014年6月至2024年6月就诊于心脏中心，经超声心动图、心脏CTA或外科手术证实为PAPVC-IAS的患儿共16例，分析其临床及影像学特点、预后转归。结果 16例患儿中男7例、女9例，首次确诊年龄为4~101月龄。异位引流路径以心上型最为常见，其中左上肺静脉经过垂直静脉汇入左无名静脉7例（43.7%），右上肺静脉异位引流入上腔静脉3例（18.8%）；其次为心下型，右下肺静脉异位引流至下腔静脉4例（25.0%）；最少见的为心内型，肺静脉直接引流入右房2例（12.5%）。继发表现为右房、右室轻微增大，31.2%（5/16）的患儿合并轻度肺动脉高压。达到手术指征的患儿可给予手术治疗，PAPVC修复术是一种相对安全的手术，结局良好，并发症发生率低。结论 房间隔完整的肺静脉异位引流由于临床症状轻微，早期容易漏诊。若发现不能解释的房室扩大或原因不明的肺动脉高压，应考虑该病的诊断。该病通过手术治疗后预后良好。
关键词： 房间隔完整; 部分性肺静脉异位引流; 心血管畸形
赵小佩 , 肖婷婷 , 徐萌 . 房间隔完整的部分性肺静脉异位引流临床特征及预后分析[J]. 临床儿科杂志, 2025 , 43(10) : 749 -754 . DOI: 10.12372/jcp.2025.24e1413
Objective To analyze the clinical characteristics and prognosis of PAPVC-IAS in order to improve the understanding of the disease. Methods A total of 16 children with PAPVC-IAS confirmed by echocardiography, cardiac CTA or surgery from June 2014 to June 2024 were collected. Their clinical and imaging features, as well as prognosis were analyzed. Results Among the 16 cases, there were 7males and 9 females. The age of the first diagnosis was from 4 to 101months. The most common type of ectopic drainage was the supracardiac type, including 7 cases (43.7%) of left superior pulmonary vein draining into left innominate vein through vertical vein, 3 cases (18.8%) of ectopic drainage of the right upper pulmonary vein into the superior vena cava; followed by infracardiac type, 4 cases (25.0%) of ectopic drainage of right inferior pulmonary vein to inferior vena cava; and the most rare is the intracardiac type, 2 cases (12.5%) of pulmonary vein directly flowing into right atrium. It was reported that the right atrium and right ventricle were slightly enlarged, and 31.2% (5/16) of the patients were associated with mild pulmonary hypertension. Surgical treatment can be given to children with surgical indications. PAPVC repair is a relatively safe operation with good outcomes and few complications. Conclusions PAPVC-IAS is easy to be missed in the early stage because of mild clinical symptoms. If unexplained cardiac enlargement or unexplained pulmonary hypertension is found, the diagnosis of the disease should be considered. The prognosis of the disease is good after surgical treatment.
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