Armengol Rofes A J, Serrano Durán M, Albert Brotons D C, Sánchez López C, Casaldáliga Ferrer J, Girona Comas J M
Unidad de Cardiología Pediátrica, Hospital Universitario Materno-Infantil Vall d'Hebron, Barcelona.
An Esp Pediatr. 1996 Feb;44(2):139-44.
Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect which consists of an apical displacement of the valvar annulus with a deformity of the septal and posterior leaflets. We report 35 cases studied from 1969 to 1993 (45% males and 55% females). One patient presented a polymalformation syndrome and another had a case of Ebstein's anomaly amongst his siblings. We performed an echocardiography in 31 patients and a catheterization in 15. The mean follow-up was 7.6 years (range: 1 day to 16.1 years). The diagnosis was established during the neonatal period in 66.7%. We found other associated cardiac anomalies in 57.1%, with radiologic cardiomegaly in 80.7%. Upon analysis of the EKGs, the P wave was higher than 0.3 mV in 48.4% and 16.1% showed Wolff-Parkinson-White syndrome. Paroxysmal supraventricular tachycardia developed in 12.9%. Long term cardiotonic treatment was needed in 27.3%. In 24.2% cardiac surgery was performed, including one heart transplantation. The mortality rate was 27.3%. The severity of Ebstein's anomaly is extremely variable, not only in its anatomy, but also in the clinical presentation. The severe neonatal form, the associated cardiac defects and heart failure worsen the prognosis.
埃布斯坦畸形（三尖瓣下移畸形）是一种相对罕见的先天性心脏缺陷，其特征为瓣环向心尖移位，伴有隔叶和后叶畸形。我们报告了1969年至1993年期间研究的35例病例（男性占45%，女性占55%）。1例患者伴有多种畸形综合征，另1例患者的兄弟姐妹中有1例患有埃布斯坦畸形。我们对31例患者进行了超声心动图检查，对15例患者进行了心导管检查。平均随访时间为7.6年（范围：1天至16.1年）。66.7%的病例在新生儿期确诊。我们发现57.1%的患者伴有其他心脏畸形，80.7%的患者有心脏扩大的影像学表现。分析心电图发现，48.4%的患者P波高于0.3mV，16.1%的患者表现为预激综合征。12.9%的患者发生阵发性室上性心动过速。27.3%的患者需要长期强心治疗。24.2%的患者接受了心脏手术，其中包括1例心脏移植。死亡率为27.3%。埃布斯坦畸形的严重程度差异极大，不仅在解剖结构上，临床表现也各不相同。严重的新生儿型、相关的心脏缺陷和心力衰竭会使预后恶化。
