Cohen Meryl S
Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA.
Curr Opin Cardiol. 2018 Jan;33(1):95-100. doi: 10.1097/HCO.0000000000000466.
The purpose of this review is to describe the challenges associated with the diagnosis and treatment of children with borderline ventricles. A borderline ventricle is one in which there is concern that it will not be able to support its circulation. If a biventricular repair is attempted and fails, outcome is often poor. Thus, this early decision is important.
For the borderline right ventricle, options to add an additional source of pulmonary blood flow make the surgical strategy a bit more flexible than for patients with a borderline left ventricle. In general, outcome for a so-called one and one-half ventricle repair are generally good, though the long-term outcome and the effects of this physiology on lifelong exercise performance and quality of life remain to be seen. For the small left ventricle, often multiple surgeries are required to 'force' blood into the left ventricle and potentially help it grow. Though this strategy is successful in some, in others it results in significant residual cardiac issues including pulmonary hypertension.
Determining whether a patient will be better off in the long term with a marginal biventricular repair versus a Fontan circulation remains one of the most difficult problems in the field of pediatric cardiology and cardiac surgery.
本综述旨在描述与临界心室患儿诊断和治疗相关的挑战。临界心室是指担心其无法维持自身循环的心室。如果尝试双心室修复但失败,预后通常较差。因此,这一早期决策很重要。
对于临界右心室,增加额外肺血流来源的选择使手术策略比临界左心室患者更具灵活性。一般来说,所谓的1.5心室修复的预后通常较好,不过长期预后以及这种生理状况对终身运动能力和生活质量的影响仍有待观察。对于小左心室,通常需要多次手术来“迫使”血液进入左心室,并可能帮助其生长。尽管这一策略在一些患者中取得成功,但在另一些患者中会导致包括肺动脉高压在内的严重残余心脏问题。
确定与Fontan循环相比,进行边缘性双心室修复的患者从长期来看是否会更好,仍然是小儿心脏病学和心脏外科领域最困难的问题之一。