Takayasu S, Obunai Y, Konno S
Am Heart J. 1978 Feb;95(2):154-62. doi: 10.1016/0002-8703(78)90458-1.
Twenty-six patients with Ebstein's anomaly were classified into three types according to their clinical features, heart catheterization data, angiocardiographic and anatomical findings which were obtained on surgery or autopsy. The hemodynamics in each type were discussed. 1. Tricuspid Stenosis Dominant Type. Eight patients, who were cyanotic and had severe symptoms, mild to moderate cardiomegaly, and the "double-ball sign" on angiocardiography were classified into this type. A pressure gradient across the tricuspid valve was demonstrated in 5 patients. 2. Tricuspid Insufficiency Dominant Type. Four cyanotic patients, who had mild symptoms despite the severe cardiomegaly were grouped into this type. The "double-ball sign" was found in all. In three patients, incompetent tricuspid valve was observed. Tricuspid insufficiency necessitates the volume overwork of the right atrium and the functioning right ventricle, resulting in severe dilatation. 3. Mild Type. Fourteen patients who showed no or mild cyanosis, no or mild symptoms, and mild to moderate cardiomegaly, were classified into this type. It is considered that the adequate cardiac output in these patients is attributable to the good function of the tricuspid valve.
26例埃布斯坦畸形患者根据其临床特征、心导管检查数据、心血管造影及手术或尸检时获得的解剖学发现分为三种类型。对各类型的血流动力学进行了讨论。1. 三尖瓣狭窄为主型。8例患者，有紫绀且症状严重，有轻至中度心脏扩大，心血管造影显示“双球征”，归入此型。5例患者显示三尖瓣跨瓣压差。2. 三尖瓣关闭不全为主型。4例紫绀患者，尽管心脏扩大严重但症状轻微，归入此型。所有患者均发现“双球征”。3例患者观察到三尖瓣关闭不全。三尖瓣关闭不全使右心房和功能正常的右心室容量负荷过重，导致严重扩张。3. 轻型。14例患者无或有轻度紫绀，无或有轻度症状，有轻至中度心脏扩大，归入此型。认为这些患者的心输出量充足归因于三尖瓣的良好功能。
