Riggs Kyle W, Kavoosi Tazheh A, Kroslowitz Benjamin, Zafar Farhan, Heydarian Haleh C, Morales David L S
Division of Cardiac Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Division of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
World J Pediatr Congenit Heart Surg. 2019 Sep;10(5):552-557. doi: 10.1177/2150135119860465.
Hypoplastic left heart disease and its variants complicated by severe semilunar and atrioventricular valve insufficiency are associated with a high mortality risk in utero and postnatally. Through the description of three cases, this report outlines the evolution of a successful strategy of morphological left ventricle isolation (ie, "left-sided Starnes") in caring for this rare cohort of patients with hypoplastic left heart syndrome (HLHS).
This study includes three patients: two patients prenatally diagnosed and one transferred from an outside hospital. All patients presented with complex univentricular heart disease with a nonfunctional left ventricle and severe left-sided atrioventricular and/or semilunar valve insufficiency.
Two patients were delivered with an operating room on standby due to the possibility of emergent surgery being necessary; however, all patients were able to be medically stabilized after birth. Patient 1 underwent successful left ventricle exclusion with a fenestrated polytetrafluoroethylene (PTFE) patch, despite severe mitral valve regurgitation. Patient 2 reinforced the efficacy of PTFE patch occlusion in addition to having aortic valve closure with a small washing jet. Patient 3's clinical course was successfully predicted based on the previous patients and outlined for the family prenatally. All patients are currently growing well at home, awaiting Fontan with marked improvement in cardiac function.
This rare cohort of patients with HLHS having severe left-sided atrioventricular and/or semilunar valve insufficiency appear to survive to birth and can be medically stabilized prior to semiurgent intervention. The Norwood procedure with left ventricle exclusion appears to be a successful strategy for these unique patients.
左心发育不全综合征及其合并严重半月瓣和房室瓣关闭不全的变异型疾病，在子宫内和出生后均具有较高的死亡风险。通过对三例病例的描述，本报告概述了在治疗这一罕见的左心发育不全综合征（HLHS）患者群体时，成功采用形态学左心室隔离策略（即“左侧Starnes手术”）的演变过程。
本研究纳入三名患者：两名产前诊断患者和一名从外院转诊而来的患者。所有患者均表现为复杂的单心室心脏病，左心室无功能，且左侧房室瓣和/或半月瓣严重关闭不全。
两名患者因可能需要急诊手术而在分娩时安排手术室待命；然而，所有患者出生后均能通过药物治疗实现病情稳定。患者1尽管存在严重二尖瓣反流，但采用带孔聚四氟乙烯（PTFE）补片成功进行了左心室隔离。患者2除了通过小冲洗射流关闭主动脉瓣外，还增强了PTFE补片封堵的效果。基于前两名患者的情况，成功预测了患者3的临床病程，并在产前向其家属进行了说明。所有患者目前在家中生长良好，等待进行Fontan手术，心功能有显著改善。
这一罕见的HLHS患者群体，伴有严重的左侧房室瓣和/或半月瓣关闭不全，似乎能够存活至出生，并且在半急诊干预前可通过药物治疗实现病情稳定。采用左心室隔离的Norwood手术似乎是治疗这些特殊患者的成功策略。
