Branco L M, Agapito A F, Oliveira J A, Gonçalves J M, Quininha J, Velho H V, Pereira A J, Ferreira M L, Antunes A M, Bento R
Serviço de Cardiologia do Hospital de Santa Marta.
Rev Port Cardiol. 1990 Mar;9(3):221-5.
We report the case of a 23 year old patient in whom heart disease was detected in childhood. He began to have suddenly fever and symptoms of right side heart failure. There were signs of aortic regurgitation, a systodiastolic murmur in the left parasternal region and a diastolic murmur in the left 4th intercostal space. The bidimensional and Doppler Echocardiogram showed a saccular dilatation in the right ventricular outflow tract. He was submitted to catheterisation which confirmed aortic regurgitation and detected ventricular septal defect and pulmonary hypertension. During surgery, a subaortic ventricular septal defect was seen as well as a dilatation of the right coronary aortic cusp which prolapsed through the defect to the right ventricular outflow tract. Vegetations were seen in the pulmonary valve. The aortic valve and a pulmonary cusp were excised and a Björk Shiley aortic prosthetic valve was inserted. The septal defect was closed. The patient is still alive, symptomless, with 40 months of follow up. We discuss some problems related to this case, particularly the surgical indication and the rarity of the morphology discovered.
我们报告一例23岁患者,其心脏病于儿童期被发现。他开始突然发热并出现右侧心力衰竭症状。有主动脉反流的体征,胸骨左缘有全舒张期杂音,在左第4肋间有舒张期杂音。二维及多普勒超声心动图显示右心室流出道呈囊状扩张。他接受了心导管检查,证实有主动脉反流,并检测到室间隔缺损和肺动脉高压。手术中,可见主动脉瓣下室间隔缺损以及右冠状动脉主动脉瓣叶扩张,该瓣叶通过缺损脱垂至右心室流出道。在肺动脉瓣可见赘生物。切除主动脉瓣和一个肺动脉瓣叶,并植入了一个比约克·希利主动脉人工瓣膜。关闭了室间隔缺损。患者仍然存活,无症状,已随访40个月。我们讨论了与该病例相关的一些问题,特别是手术指征以及所发现形态的罕见性。