Rychik J, Rome J J, Collins M H, DeCampli W M, Spray T L
Cardiac Center at The Children's Hospital of Philadelphia, and Department of Pediatrics, The University of Pennsylvania School of Medicine, USA.
J Am Coll Cardiol. 1999 Aug;34(2):554-60. doi: 10.1016/s0735-1097(99)00225-9.
The purpose of this study was to investigate the outcome in infants with hypoplastic left heart syndrome and intact atrial septum and to evaluate the relationship of atrial morphology, left atrial decompression pathway and lung histopathology to outcome.
In the hypoplastic left heart syndrome, severe restriction at the atrial level results in marked systemic hypoxemia after birth. Infants with intact atrial septum may be at high risk for mortality after Norwood operation.
Of 316 infants with hypoplastic left heart syndrome seen at our center over a 6.5-year period, 18 (5.7%) had intact atrial septum. Medical records and echocardiograms were reviewed.
On echocardiography, three types of intact atrial septal morphology were identified: 1) large left atrium, thick prominent septum secondary with thin septum primary adherent (type A, n = 12); 2) small left atrium with thick, muscular atrial septum (type B, n = 4), and 3) giant left atrium, thin atrial septum with severe mitral regurgitation (type C, n = 2). Seven infants had left atrial decompression pathways that were severely obstructed (3/12 type A, 4/4 type B). Norwood operation was performed in 17 infants; one underwent emergency balloon atrial septostomy and died. Of six early survivors, all with type A atrial morphology and unobstructed decompression pathway, three died after subsequent cavopulmonary surgery. Lung histopathology revealed severely dilated lymphatics and "arterialization" of the pulmonary veins in those with the severest degree of obstruction to left atrial egress (type B atrial morphology).
Despite aggressive intervention, outcome for infants born with hypoplastic left heart syndrome and intact atrial septum is poor. Maldevelopment of the pulmonary vasculature contributes to the high mortality seen. Atrial morphology can be used as a marker for the severity of pulmonary vascular disease.
本研究旨在调查房间隔完整的左心发育不全综合征婴儿的预后情况，并评估心房形态、左心房减压途径和肺组织病理学与预后的关系。
在左心发育不全综合征中，心房水平的严重受限导致出生后明显的全身低氧血症。房间隔完整的婴儿在诺伍德手术后可能有较高的死亡风险。
在我们中心6.5年期间收治的316例左心发育不全综合征婴儿中，18例（5.7%）房间隔完整。回顾了病历和超声心动图。
超声心动图检查发现三种类型的房间隔完整形态：1）左心房大，继发隔厚且突出，原发隔薄且附着（A型，n = 12）；2）左心房小，房间隔厚且肌性（B型，n = 4），以及3）左心房巨大，房间隔薄且严重二尖瓣反流（C型，n = 2）。7例婴儿的左心房减压途径严重受阻（A型3/12，B型4/4）。17例婴儿接受了诺伍德手术；1例接受了紧急球囊房间隔造口术并死亡。6例早期存活者均为A型心房形态且减压途径未受阻，其中3例在随后的腔肺分流手术后死亡。肺组织病理学显示，左心房出口梗阻最严重的患者（B型心房形态）存在严重扩张的淋巴管和肺静脉“动脉化”。
尽管进行了积极干预，房间隔完整的左心发育不全综合征婴儿的预后仍然很差。肺血管系统发育不良导致了高死亡率。心房形态可作为肺血管疾病严重程度的标志物。
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