Jaiswal P K, Balakrishnan K G, Saha A, Venkitachalam C G, Tharakan J, Titus T
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
Int J Cardiol. 1994 Sep;46(2):113-9. doi: 10.1016/0167-5273(94)90031-0.
There were 63 patients of Ebstein's anomaly of tricuspid valve encountered from 1976 to 1991; 28 (44.4%) were male and 35 (55.6%) female. Their age at presentation ranged from 3 months to 51 years. Five (7.9%) patients were asymptomatic, 48 (76.2%) had class II-III exertional dyspnoea, palpitation or both. Thirty patients (47.6%) had cyanosis. Electrocardiogram showed paroxysmal atrial fibrillation in two, chronic atrial fibrillation in four (6.3%), paroxysmal supraventricular tachycardia in seven, atrial or ventricular ectopic beats in five (7.9%), 2:1 atrioventricular block in one (1.6%), complete atrioventricular block in two (3.2%) and type B WPW syndrome in nine patients (14.3%). Chest X-ray showed diminished vascularity in 22 (34.9%). Diagnosis was established by cardiac catheterization and or echocardiography. Atrialized right ventricular chamber was demonstrated in 51 (80.9%) by angiography and in 40 (63.5%) by electrophysiology. Patients were followed up for 1-172 months. Seventeen patients (26.9%) required surgery. Three patients (4.8%) died during medical follow-up, and five (7.9%) died following surgery. Survival probability for 46 medical patients was 88.9% at 172 months. Factors affecting survival were pulmonary blood flow, cyanosis, clubbing and systemic arterial oxygen saturation.
1976年至1991年间共收治63例三尖瓣埃布斯坦畸形患者；男性28例（44.4%），女性35例（55.6%）。就诊时年龄从3个月至51岁不等。5例（7.9%）患者无症状，48例（76.2%）有Ⅱ-Ⅲ级劳力性呼吸困难、心悸或两者皆有。30例（47.6%）有发绀。心电图显示2例阵发性心房颤动，4例慢性心房颤动（6.3%），7例阵发性室上性心动过速，5例房性或室性早搏（7.9%），1例2:1房室传导阻滞（1.6%），2例完全性房室传导阻滞（3.2%），9例患者有B型预激综合征（14.3%）。胸部X线显示22例（34.9%）血管纹理减少。通过心导管检查和/或超声心动图确诊。血管造影显示51例（80.9%）有房化右心室腔，电生理检查显示40例（63.5%）有房化右心室腔。对患者进行了1至172个月的随访。17例（26.9%）患者需要手术治疗。3例（4.8%）患者在药物随访期间死亡，5例（7.9%）患者术后死亡。46例接受药物治疗患者在172个月时的生存概率为88.9%。影响生存的因素有肺血流量、发绀、杵状指和体动脉血氧饱和度。
