Shyu K G, Tseng C D, Chiu I S, Hung C R, Chu S H, Lue H C, Tseng Y Z, Lien W P
Department of Internal Medicine, National Taiwan University Hospital, Taipei, ROC.
Int J Cardiol. 1993 Sep;41(2):115-21. doi: 10.1016/0167-5273(93)90150-f.
Infundibular pulmonic stenosis with intact ventricular septum of primary origin is an uncommon condition. We report 15 such patients (nine males and six females, aged 7-36 years) who had undergone surgical correction for the anomaly during the period between 1975 and 1992. The occurrence of this clinical setting represents 0.19% (15/7826) of all cardiac operations and 0.46% (15/3222) of congenital heart diseases undergoing surgical correction during that period of time. The lesion was of discrete fibromuscular hypertrophy of the infundibulum in all 15 patients. The presenting symptoms of most patients were exertional dyspnea and syncope; however, five patients with severe obstruction were asymptomatic. The peak systolic pressure gradient across the infundibulum ranged from 71 to 230 mmHg. There was only one operative death; the remainder had remained well following the surgery over a mean follow-up period of 35 months. Surgical correction for infundibular pulmonic stenosis is rewarding in the absence of heart failure.
原发性起源且室间隔完整的漏斗部肺动脉狭窄是一种罕见疾病。我们报告了15例此类患者（9例男性，6例女性，年龄7至36岁），他们于1975年至1992年期间接受了该畸形的手术矫正。这种临床情况的发生率占所有心脏手术的0.19%（15/7826），占该时期接受手术矫正的先天性心脏病的0.46%（15/3222）。所有15例患者的病变均为漏斗部离散性纤维肌性肥厚。大多数患者的主要症状为劳力性呼吸困难和晕厥；然而，5例严重梗阻患者无症状。漏斗部收缩期峰值压力阶差范围为71至230 mmHg。仅1例手术死亡；其余患者术后平均随访35个月情况良好。在无心力衰竭的情况下，漏斗部肺动脉狭窄的手术矫正效果良好。
