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当代法洛四联症患儿和青年患者手术后的功能预后。

Functional outcome in contemporary children and young adults with tetralogy of Fallot after repair.

机构信息

Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Munchen, Germany.

Institute of Preventive Pediatrics, Technische Universität München, Munich, Germany.

出版信息

Arch Dis Child. 2019 Feb;104(2):129-133. doi: 10.1136/archdischild-2017-314733. Epub 2018 Jul 3.

DOI:10.1136/archdischild-2017-314733
PMID:29970581
Abstract

OBJECTIVE

Functional outcome measures are of growing importance in the aftercare of patients with congenital heart disease. This study addresses the functional status with regard to exercise capacity, health-related physical fitness (HRPF) and arterial stiffness in a recent cohort of children, adolescents and young adults with tetralogy of Fallot (ToF) after repair.

DESIGN

Single-centre, uncontrolled and prospective cohort study.

SETTING

Outpatient department of the German Heart Centre Munich; July 2014-January 2018.

PATIENTS

One hundred and six patients with ToF after repair (13.5±3.7 years, 40 females) were included. Data were compared with a recent cohort of healthy controls (HCs) (n=1700, 12.8±2.6 years, 833 females).

MAIN OUTCOME MEASURES

Patients underwent a symptom-limited cardiopulmonary exercise test, performed an HRPF test (FitnessGram) and had an assessment of their arterial stiffness (Mobil-O-Graph).

RESULTS

Compared with HC, patients with ToF showed lower predicted [Formula: see text]O peak (ToF: 80.4% ± 16.8% vs HC: 102.6% ± 18.1%, p<0.001), impaired ventilatory efficiency (ToF: 29.6 ± 3.6 vs HC: 27.4 ± 2.9, p<0.001), chronotropic incompetence (ToF: 167 ± 17 bpm vs HC: 190 ± 17 bpm, p<0.001) and reduced HRPF (ToF z-score: -0.65 ± 0.87 vs HC z-score: 0.03 ± 0.65, p<0.001). Surrogates of arterial stiffness, central and peripheral systolic blood pressure, did not differ between the two groups.

CONCLUSIONS

Contemporary children, adolescents and young adults with ToF still have functional limitations. How impaired HRPF and limited exercise capacity interact and how they can be modified needs to be evaluated in further intervention studies.

摘要

目的

在先天性心脏病患者的康复治疗中,功能预后指标的重要性日益增加。本研究旨在评估法洛四联症(TOF)患者修复后的近期队列中,在运动能力、与健康相关的身体适应性(HRPF)和动脉僵硬度方面的功能状态。

设计

单中心、非对照、前瞻性队列研究。

地点

德国慕尼黑心脏中心门诊部;2014 年 7 月至 2018 年 1 月。

患者

纳入 106 例修复后 TOF 患者(13.5±3.7 岁,40 名女性)。将数据与最近的健康对照组(HCs)(n=1700,12.8±2.6 岁,833 名女性)进行比较。

主要观察指标

患者进行了症状限制心肺运动试验,进行了 HRPF 测试(FitnessGram),并评估了动脉僵硬度(Mobil-O-Graph)。

结果

与 HC 相比,TOF 患者的预测[Formula: see text]O 峰值较低(TOF:80.4%±16.8% vs HC:102.6%±18.1%,p<0.001),通气效率受损(TOF:29.6±3.6 vs HC:27.4±2.9,p<0.001),变时性功能不全(TOF:167±17 bpm vs HC:190±17 bpm,p<0.001)和 HRPF 降低(TOF z 评分:-0.65±0.87 vs HC z 评分:0.03±0.65,p<0.001)。两组之间中央和外周收缩压等动脉僵硬度的替代指标没有差异。

结论

当代的 TOF 患儿、青少年和年轻成年人仍然存在功能障碍。受损的 HRPF 和有限的运动能力如何相互作用,以及如何进行干预改善,需要在进一步的干预研究中进行评估。

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