Division of Pediatric Cardiac Surgery, Fondazione G. Monasterio, Massa, Italy.
Institute of Clinical Physiology, National Research Council (CNR), Via Aurelia Sud, 54100, Massa, Italy.
Cardiovasc Ultrasound. 2021 Jun 19;19(1):23. doi: 10.1186/s12947-021-00249-y.
In patients with repaired Fallot, subsequent surgical or interventional procedures and adverse cardiac events are frequent. We aimed to evaluate the impact of a simple pre-operative anatomic classification based on the size of the pulmonary valve (PV) annulus and branches on future therapeutic requirements and outcomes.
This is a single-center retrospective analysis of patients operated for Fallot before the age of 2 years, from January 1990. Pre-operative anatomy, surgical and interventional procedures and adverse events were extrapolated from clinical records.
Among the 312 patients, a description of the PV and pulmonary arteries (PAs) native anatomy was known in 239 patients (male:147, 61.5%), which were divided in the following 3 groups: group 1 (65 patients) with normal size of both PV and PAs; group 2 (108 patients) with PV hypoplasia but normal size PAs; group 3 (66 patients) with concomitant hypoplasia of the PV and PAs. During the 12.7 years (IQR 6.7-17) follow-up time, 23% of patients required at least one surgical or interventional procedure. At Kaplan-Meier analysis, there was a significant difference in requirement of future surgical or interventional procedures among the 3 groups (p < 0,001). At multivariate Cox regression analysis, hypoplasia of PV and PAs was an independent predictor of subsequent procedures (HR:3.1,CI:1.06-9.1, p = 0.03).
Native anatomy in Tetralogy of Fallot patients affects surgical strategy and follow-up. It would be therefore advisable to tailor patient's counseling and follow-up according to native anatomy, rather than following a standardized protocol.
在法洛四联症患者中，随后的手术或介入治疗以及心脏不良事件较为常见。我们旨在评估一种简单的术前解剖分类方法对未来治疗需求和结果的影响，这种方法基于肺动脉瓣环和分支的大小。
这是一项单中心回顾性分析，纳入了 1990 年 1 月前接受法洛四联症手术的 2 岁以下患者。从临床记录中推断出术前解剖结构、手术和介入治疗程序以及不良事件。
在 312 名患者中，有 239 名（男性 147 名，61.5%）患者的肺动脉瓣（PV）和肺动（PAs）的解剖结构描述已知，将其分为以下 3 组：组 1（65 名）患者的 PV 和 PAs 大小正常；组 2（108 名）患者的 PV 发育不良但 PAs 大小正常；组 3（66 名）患者的 PV 和 PAs 同时发育不良。在 12.7 年（IQR 6.7-17）的随访期间，23%的患者至少需要一次手术或介入治疗。在 Kaplan-Meier 分析中，3 组患者之间对未来手术或介入治疗的需求存在显著差异（p<0.001）。在多变量 Cox 回归分析中，PV 和 PAs 发育不良是随后进行介入治疗的独立预测因子（HR：3.1，CI：1.06-9.1，p=0.03）。
法洛四联症患者的原生解剖结构影响手术策略和随访。因此，根据原生解剖结构来调整患者的咨询和随访可能会更好，而不是遵循标准化的方案。
