PDF(Pubmed)
Abstract：
BACKGROUND: Absent Pulmonary Valve Syndrome (APVS) is a rare birth defect where the pulmonary valve is missing or underdeveloped. APVS often occurs alongside Tetralogy of Fallot, (TOF) another heart defect.
METHODS: A 33-year-old woman gave birth to a male infant with severe pulmonary stenosis (PS) and a large ventricular septal defect (VSD). The infant underwent surgery to close the VSD and resect the stenotic ring. Two years later, he remained asymptomatic with a closed VSD and no pulmonary valve gradient.
CONCLUSIONS: Despite high mortality rates, long-term survival has improved with advancements in surgical repair. This case underscores the significance of early detection and personalized surgical strategies for complex congenital heart defects.
CONCLUSIONS: Early identification of subtle symptoms is crucial for timely intervention, while individualized surgical strategies optimize outcomes. Further research is needed to understand the complex interplay of cardiac anomalies in APVS, particularly the absence of a patent ductus arteriosus in this case.
METHODS: A 33-year-old woman gave birth to a male infant with severe pulmonary stenosis (PS) and a large ventricular septal defect (VSD). The infant underwent surgery to close the VSD and resect the stenotic ring. Two years later, he remained asymptomatic with a closed VSD and no pulmonary valve gradient.
CONCLUSIONS: Despite high mortality rates, long-term survival has improved with advancements in surgical repair. This case underscores the significance of early detection and personalized surgical strategies for complex congenital heart defects.
CONCLUSIONS: Early identification of subtle symptoms is crucial for timely intervention, while individualized surgical strategies optimize outcomes. Further research is needed to understand the complex interplay of cardiac anomalies in APVS, particularly the absence of a patent ductus arteriosus in this case.
摘要：
背景：肺动脉瓣缺失综合征（APVS）是一种罕见的出生缺陷，其中肺动脉瓣缺失或发育不全。APVS通常与法洛四联症同时发生，（TOF）另一个心脏缺陷。
方法：一名33岁女性生下一名男性婴儿，患有严重的肺动脉狭窄（PS）和巨大的室间隔缺损（VSD）。婴儿接受手术以关闭VSD并切除狭窄环。两年后,他仍然无症状，闭合性VSD和无肺动脉瓣梯度。
结论：尽管死亡率很高，随着外科修复技术的进步,长期生存率得到改善.此病例强调了复杂先天性心脏病早期发现和个性化手术策略的重要性。
结论：早期发现细微症状对于及时干预至关重要，而个性化的手术策略可以优化结果。需要进一步的研究来了解APVS中心脏异常的复杂相互作用，特别是在这种情况下没有动脉导管未闭。
方法：一名33岁女性生下一名男性婴儿，患有严重的肺动脉狭窄（PS）和巨大的室间隔缺损（VSD）。婴儿接受手术以关闭VSD并切除狭窄环。两年后,他仍然无症状，闭合性VSD和无肺动脉瓣梯度。
结论：尽管死亡率很高，随着外科修复技术的进步,长期生存率得到改善.此病例强调了复杂先天性心脏病早期发现和个性化手术策略的重要性。
结论：早期发现细微症状对于及时干预至关重要，而个性化的手术策略可以优化结果。需要进一步的研究来了解APVS中心脏异常的复杂相互作用，特别是在这种情况下没有动脉导管未闭。
