Hosseinpour Amir-Reza, Perez Marie-Hélène, Longchamp David, Cotting Jacques, Sekarski Nicole, Hurni Michel, Prêtre René, Di Bernardo Stefano
Department of Cardiac Surgery, University Hospital of Vaud, Lausanne, Switzerland.
Pediatric Intensive Care, University Hospital of Vaud, Lausanne, Switzerland.
Congenit Heart Dis. 2018 Mar;13(2):210-216. doi: 10.1111/chd.12545. Epub 2017 Oct 25.
Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.
A retrospective observational study. We defined "late" as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such malformations referred to us from 2004 untill 2015.
Department of Pediatric Cardiology and Cardiac Surgery, University Hospital of Vaud, Lausanne, Switzerland.
There were 39 patients, aged 2-35 years (median: 5 years), without chromosomal abnormalities. All had malformations amenable to biventricular repair, and all had high systolic right ventricular pressures by echocardiography prior to referral.
All patients underwent catheterization for assessment of pulmonary hypertension. If this was reversible, surgical correction was offered.
(1) Operability based on reversibility of pulmonary hypertension. (2) When surgery was offered, mortality and evidence of persisting postoperative pulmonary hypertension were examined.
Eighteen patients had no pulmonary hypertension, 5 of variable ages were inoperable due to irreversible pulmonary hypertension, and 16 had reversible pulmonary hypertension. Therefore, 34 patients underwent corrective surgery, with no immediate or late mortality. Pulmonary arterial and right ventricular pressures decreased noticeably in all operated patients. This is sustained to date; they are all asymptomatic with no echocardiographic evidence of pulmonary hypertension at a median follow-up of 7 years (range 2-13 years).
Pulmonary hypertension may still be reversible in many surprisingly old patients with left-to-right shunt lesions, who may therefore still be operable.
由于左向右分流导致肺血流量和压力增加的先天性心脏畸形，通常在婴儿早期进行修复，这既能早期缓解心力衰竭，又能避免因担心伴随的肺动脉高压可能变得不可逆，除非在早期纠正这些缺陷。然而，根据我们的经验，年龄一直不是肺动脉高压不可逆性的良好预测指标，本文将介绍这一经验。
一项回顾性观察研究。我们将“晚期”定义为年龄≥2岁。我们检查了2004年至2015年转诊至我们这里的所有年龄≥2岁的此类畸形患者的临床、超声心动图和血流动力学数据。
瑞士洛桑沃州大学医院儿科心脏病学和心脏外科。
39例患者，年龄2至35岁（中位数：5岁），无染色体异常。所有患者均有适合双心室修复的畸形，且在转诊前超声心动图显示右心室收缩压均较高。
所有患者均接受导管检查以评估肺动脉高压。如果肺动脉高压可逆，则进行手术矫正。
（1）基于肺动脉高压可逆性的可手术性。（2）当提供手术时，检查死亡率和术后持续存在肺动脉高压的证据。
18例患者无肺动脉高压，5例因肺动脉高压不可逆而无法手术，年龄各异，16例有可逆性肺动脉高压。因此，34例患者接受了矫正手术，无即刻或晚期死亡。所有接受手术的患者肺动脉和右心室压力均显著下降。这种情况一直持续到现在；在中位随访7年（范围2至13年）时，他们均无症状，超声心动图也无肺动脉高压的证据。
许多年龄出人意料大的左向右分流病变患者的肺动脉高压仍可能是可逆的，因此这些患者仍可能适合手术。
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