Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK.
Department of Pediatrics, Division of Pediatric Cardiology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, Netherlands.
Cardiol Young. 2020 Apr;30(4):511-520. doi: 10.1017/S1047951120000463. Epub 2020 Mar 16.
Infants with isolated atrial septal defects are usually minimally symptomatic, and repair is typically performed after infancy. Early repair may be considered if there is high pulmonary blood flow and reduced respiratory reserve or early signs of pulmonary hypertension. Our aim was to review the characteristics and outcomes of a cohort of patients who underwent infant repair at our institute.
The study included 56 infants (28 female, 19 trisomy 21) with isolated atrial septal defect (age: 8 months (1.5-12), weight: 6 kg (2.8-7.5), echo Qp/Qs: 1.9 ± 0.1) who underwent surgical closure (20 fenestrated). Three groups were identified: 1) chronic lung disease and pulmonary hypertension (group A: n = 28%); 2) acutely unwell infants with pulmonary hypertension but no chronic lung disease (group B: n = 20, 36%); and 3) infants with refractory congestive heart failure without either pulmonary hypertension or chronic lung disease (group C: n = 9, 16%).
Post-operatively, pulmonary hypertension infants (47/56) showed improvement in tricuspid annular plane systolic excursion z-score (p < 0.001) and right ventricular systolic/diastolic duration ratio (p < 0.05). All ventilator (14.3%) or oxygen-dependent (31.6%) infants could be weaned within 2 weeks after repair. One year later, weight z-score increased in all patients and by +1 in group A, +1.3 in group B and +2 in group C. Over a median follow-up of 1.4 years, three patients died, four patients continued to have pulmonary hypertension evidence and two remained on targeted pulmonary hypertension therapy.
Atrial septal defect repair within the first year may improve the clinical status and growth in infants with early signs of pulmonary hypertension or those requiring respiratory support and facilitate respiratory management.
孤立性房间隔缺损的婴儿通常症状轻微，通常在婴儿期后进行修复。如果存在高肺血流量和呼吸储备减少或早期肺动脉高压迹象，可考虑早期修复。我们的目的是回顾在我们研究所接受婴儿修复的患者队列的特征和结果。
本研究纳入了 56 名孤立性房间隔缺损婴儿（28 名女性，19 名 21 三体）（年龄：8 个月（1.5-12），体重：6 公斤（2.8-7.5），超声 Qp/Qs：1.9 ± 0.1），行外科闭合术（20 例有孔）。分为三组：1）慢性肺部疾病和肺动脉高压（A 组：n = 28%）；2）患有肺动脉高压但无慢性肺部疾病的急性不适婴儿（B 组：n = 20，36%）；3）患有难治性充血性心力衰竭而无肺动脉高压或慢性肺部疾病的婴儿（C 组：n = 9，16%）。
术后，肺动脉高压婴儿（47/56）三尖瓣环平面收缩期位移 z 评分（p < 0.001）和右心室收缩/舒张期比值（p < 0.05）均有改善。所有需要呼吸机（14.3%）或氧气依赖（31.6%）的婴儿在修复后 2 周内均可脱机。1 年后，所有患者体重 z 评分增加，A 组增加 1，B 组增加 1.3，C 组增加 2。中位随访 1.4 年后，3 例患者死亡，4 例患者继续存在肺动脉高压证据，2 例患者仍接受靶向肺动脉高压治疗。
对于有早期肺动脉高压迹象或需要呼吸支持的婴儿，在 1 年内进行房间隔缺损修复可能会改善其临床状况和生长情况，并有助于呼吸管理。
