Right Ventricular Outflow Tract Stenting as an Option to Blalock-Taussig Shunt for Tetralogy of Fallot

J. Sandoval, C. Zabal
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Abstract

Tetralogy of Fallot (TOF) is a complex cardiac condition that includes a wide anatomical spectrum. Surgical repair may have an uneventful outcome between 6 months and the first year of life in most patients with favorable anatomy (e.g. adequate size of branch pulmonary arteries). However, management remains a challenge in symptomatic patients that present early in life, often due to a more severe involvement of right sided obstruction. In this setting, cyanosis becomes evident as a consequence of the progressive narrowing of the pulmonary infundibulum as well as an increased right ventricular stroke volume that faces a semi-fixed resistance and directs flow towards the systemic circulation. In addition, these patients often show underdevelopment of right-sided structures, including a smaller pulmonary annulus, a short and tapered main pulmonary artery, as well as variable degree of diffuse hypoplasia and/or stenosis of the pulmonary arteries. (1) Thus, it is not surprising that most of these children exhibit early progressive cyanosis and are at potential risk of “hypoxic spells” that require early medical stabilization with prostaglandin infusion to maintain arterial duct patency until a more definitive therapy can be established. Today, although some selected centers advocate for early surgical repair within the first 3 months of life (provided anatomical features are considered favorable), many countries (including Latin America) continue to support an initial palliative approach. Creation of a systemic-to-pulmonary shunt, usually a modified BlalockTaussig type (MBTS) followed by complete repair at a later stage is still the most adopted strategy in many centers around the world. Far from being considered an ideal palliative measure, a MBTS can result in disproportionate growth of the pulmonary arteries or stenosis of the involved pulmonary branch that may hinder results at the time of complete repair. (2, 3) Furthermore, according to a recent publication, shunt thrombosis or pulmonary
法洛四联症右心室流出道支架术作为Blalock-Taussig分流术的选择
法洛四联症(TOF)是一种复杂的心脏疾病,包括广泛的解剖谱。在大多数解剖结构良好(如肺动脉分支足够大小)的患者中,手术修复在6个月至1岁之间可能有一个平稳的结果。然而,对于早期出现症状的患者,治疗仍然是一个挑战,通常是由于更严重的右侧梗阻。在这种情况下,紫绀变得明显,这是肺大管进行性变窄的结果,同时右心室卒中容积增加,面临半固定阻力,并将血流导向体循环。此外,这些患者常表现为右侧结构发育不全,包括较小的肺环,短而变细的肺动脉,以及不同程度的弥漫性发育不全和/或肺动脉狭窄。(1)因此,大多数儿童表现出早期进行性紫绀,并有“缺氧期”的潜在风险,这并不奇怪,需要早期通过前列腺素输注来维持动脉导管通畅,直到确定更明确的治疗方法。今天,虽然一些选定的中心提倡在生命的前3个月内进行早期手术修复(如果解剖特征被认为是有利的),但许多国家(包括拉丁美洲)继续支持最初的姑息治疗方法。创建系统到肺分流,通常是改良的BlalockTaussig型(MBTS),然后在后期进行完全修复,仍然是世界上许多中心最采用的策略。MBTS远不是一种理想的缓解措施,它可能导致肺动脉不成比例的生长或受累肺分支的狭窄,这可能会影响完全修复时的效果。(2,3)此外,根据最近的出版物,分流血栓形成或肺动脉栓塞
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