Laohachai Karina, Winlaw David, Sholler Gary, Veerappan Sundar, Cole Andrew, Ayer Julian
The Heart Centre for Children, The Children'S Hospital at Westmead, Locked Bag 4001, Sydney, NSW, 2145, Australia.
The University of Sydney, Sydney, Australia.
Pediatr Cardiol. 2019 Jun;40(5):1035-1040. doi: 10.1007/s00246-019-02111-5. Epub 2019 May 7.
Tricuspid valve regurgitation (TR) increases adverse outcomes in children with hypoplastic left heart syndrome (HLHS). Changes in tricuspid valve (TV) annulus and leaflet geometry have been described but the underlying causes for TR in HLHS remain uncertain. We aimed to examine the association between left ventricular (LV) size and TR in infants with HLHS as right ventricular (RV)-LV interactions may be important in TR development. Echocardiograms of 50 infants with HLHS were reviewed. LV size, RV function, TR grade, TV annulus z-score, and aortic arch obstruction were examined at birth and 1 year of age (or the latest study post-bidirectional Glenn anastomosis if the patient was < 1 year of age). 24/50 (48%) had severe LV hypoplasia and 26/50 (52%) had mild/moderate LV hypoplasia. At 1 year, 10/24 (42%) with severe LV hypoplasia had moderate/severe TR versus 0/26 in the mild/moderate LV hypoplasia group (p = 0.0002). TR progressed ( ≥ 1 grade) in 14/24(58%) with severe LV hypoplasia versus 5/26 (19%) with mild/moderate LV hypoplasia (p = 0.008). In this cohort, no association was found between the degree of TR and either RV function, TV annular z-score, or arch obstruction; or between the degree of LV hypoplasia and either RV function or TV annular z-score. In infants with HLHS, the severity and progression of TR is associated with the severity of LV hypoplasia. The mechanism for this association needs further exploration but suggests a role for RV-LV interactions in the development of TR.
三尖瓣反流（TR）会增加左心发育不全综合征（HLHS）患儿出现不良结局的风险。虽然已有关于三尖瓣（TV）瓣环和瓣叶几何形状变化的描述，但HLHS中TR的潜在病因仍不明确。我们旨在研究HLHS婴儿左心室（LV）大小与TR之间的关联，因为右心室（RV）-LV相互作用可能在TR的发生发展中起重要作用。回顾了50例HLHS婴儿的超声心动图。在出生时和1岁时（如果患者年龄小于1岁，则在双向格林吻合术后的最新研究时）检查LV大小、RV功能、TR分级、TV瓣环z评分和主动脉弓梗阻情况。50例中有24例（48%）有严重的LV发育不全，26例（52%）有轻度/中度LV发育不全。1岁时，24例严重LV发育不全的患儿中有10例（42%）出现中度/重度TR，而轻度/中度LV发育不全组中26例无一例出现（p = 0.0002）。24例严重LV发育不全的患儿中有14例（58%）TR进展（≥1级），而轻度/中度LV发育不全的患儿中有5例（19%）出现TR进展（p = 0.008）。在该队列中，未发现TR程度与RV功能、TV瓣环z评分或弓部梗阻之间存在关联；也未发现LV发育不全程度与RV功能或TV瓣环z评分之间存在关联。在HLHS婴儿中，TR的严重程度和进展与LV发育不全的严重程度相关。这种关联的机制需要进一步探索，但提示RV-LV相互作用在TR的发生发展中起作用。
