Barwad Parag, Prasad Krishna, Vijay Jyothi, Naganur Sanjeev
Department of Cardiology, Advanced Cardiac Centre, Post Graduate Institute of Medical Education & Research, Sector 12, Chandigarh, 160 012, India.
Egypt Heart J. 2020 Sep 29;72(1):64. doi: 10.1186/s43044-020-00097-7.
Hypoplastic right heart syndrome with pulmonary atresia is a rare cyanotic heart disease with poor prognosis requiring urgent intervention to establish the pulmonary blood flow. Pulmonary blood flow is achieved by BT shunt or percutaneous techniques like PDA stenting or pulmonary valve perforation. Various series have shown that early surgical intervention causes high mortality in these patients. Pulmonary valve perforation is a suitable, physiological alternative to surgical techniques in selected patients.
We report a case of hypoplastic right heart syndrome with pulmonary atresia and restrictive VSD presenting with cyanosis from birth and underwent pulmonary valve perforation successfully.
Duct-dependent pulmonary circulation is a pediatric emergency, palliative procedure for establishing adequate pulmonary blood flow is essential early in the life. In the management of duct-dependent pulmonary circulation, RVOT perforation is an effective and safe option in suitable high-risk subgroups. The induced pulmonary regurgitation along with established physiological antegrade flow would be beneficial in the remodeling of tripartite/hypertrophied small RV.
肺闭锁的右心发育不全综合征是一种罕见的青紫型心脏病，预后较差，需要紧急干预以建立肺血流。肺血流可通过体肺分流术或经皮技术如动脉导管未闭支架置入术或肺动脉瓣穿孔术来实现。各种系列研究表明，早期手术干预在这些患者中会导致高死亡率。对于选定的患者，肺动脉瓣穿孔是一种合适的、符合生理的手术技术替代方法。
我们报告一例肺闭锁和限制性室间隔缺损的右心发育不全综合征病例，该患儿自出生起就出现青紫，并成功接受了肺动脉瓣穿孔术。
依赖动脉导管的肺循环是一种儿科急症，早期建立充足的肺血流的姑息性手术至关重要。在依赖动脉导管的肺循环管理中，右室流出道穿孔对于合适的高风险亚组是一种有效且安全的选择。诱导的肺反流以及已建立的生理性顺行血流将有利于三联征/肥厚性小右室的重塑。
