Kumar Vikas, Mahajan Sachin, Jaswal Vivek, Thingnam Shyam Kumar Singh
Department of Cardiothoracic and Vascular Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Eur Heart J Case Rep. 2019 Jun 1;3(2). doi: 10.1093/ehjcr/ytz012.
Supravalvular stenosis of main pulmonary artery is a rare anomaly characterized by the presence of constriction band just above the pulmonary valve. It is mostly acquired after intervention on the pulmonary trunk or less commonly is congenital in origin associated with complex congenital cardiac malformations and very rarely can present as an isolated native congenital supravalvular pulmonary stenosis (SPS).
We present a series of four cases of isolated congenital SPS who underwent surgical correction at our tertiary care institute over 8 years. Mean age of the patients was 2.25 ± 0.96 years with all of them being males. Mean peak systolic gradient across the stenosis was 82 ± 21.48 mmHg ranging from 60 mmHg to 110 mmHg. There was no early and medium-term mortality with 100% survival at mean follow-up of 31 months (range 7-85 months).
Surgical correction of congenital SPS carries excellent early and mid-term results with almost no mortality and very low risk of re-intervention for restenosis.
主肺动脉瓣上狭窄是一种罕见的异常情况，其特征是在肺动脉瓣上方存在缩窄环。它大多在对肺动脉干进行干预后获得，较少情况下为先天性起源，与复杂的先天性心脏畸形相关，并且极少能表现为孤立的先天性原发性瓣上肺动脉狭窄（SPS）。
我们报告了在我们的三级医疗机构8年期间接受手术矫正的一系列4例孤立性先天性SPS病例。患者的平均年龄为2.25±0.96岁，均为男性。狭窄处的平均收缩期峰值压差为82±21.48 mmHg，范围为60 mmHg至110 mmHg。无早期和中期死亡，平均随访31个月（范围7 - 85个月）时生存率为100%。
先天性SPS的手术矫正具有出色的早期和中期结果，几乎无死亡率，再干预治疗再狭窄的风险极低。
